ILEANfl ZOLOG

MfiRIE-JEAHnE KOOS MIHHEA MUKTEAHU

OPHTHALMOLOGY

Editura EUROSTAMPA
Timisoara, B-dul Revolutiei din 1989 nr. 26
Tel./fax: 056-20.48.16

I.S.B.N.: 973-8244-71-4 * ** * *
Editura* Eurostampa
Tipar executat la Tipogrufia Eurostampa
TIMI§OARA, 2001
■‘W

ILEANA ZOLOG
HIARIE-JEAAGE HOOS MIHHEft MUIGEAnU
*
J
?
I

t
OPHTHOOLOGY
 Contents

1. ANATOMY

2. LIDS & LACRIMAL APPARATUS

THE LIDS
Infections & Inflammations of the Lids.
Positional Defects of the Lids
Anatomic Deformities of the Lids
Blepharospasm (Tic)..
Ptosis
Pseudoptosis

THE LACRIMAL APPARATUS

Infections of the Lacrimal Apparatus
v/ Dacryocystitis.
•. / Canaliculitis...
Dacryoadenitis

3. TEARS

Source & Function of the Tears

Composition of the Tears
Layers of the Preocular Tear Film
Dry Eye Syndrome (Keratoconjunctivitis Sicca; KCS) ...
Other Disorders of the Lacrimal System

4. CONJUNCTIVA

DISEASES OF THE CONJUNCTIVA

CONJUNCTIVITIS
y Bacterial Conjunctivitis
y Conjunctivitis Cue to Chlamydiae ..
5
 • ^ZViral Conjunctivitis Neurotrophic Corneal Ulcers .....57
41| / Exposure Keratitis 58
J >> Allergic ^Conjunctivitis 42|
i ij j ChemicPI Conjunctivitis Mooren’s Ulcer.........................................................................................58
44*
Fungal Conjunctivitis 451 DEGENERATIVE CORNEAL CONDITIONS 59
Conjunctivitis due to Animal Parasites... 45 J xKeratoconus 59
. Conjunctivitis of unknown Etiology 47/
J Chronic; Follicular Conjunctivitis Corneal Dystrophy or Degeneration 59
471 Hereditary Corneal Dystrophies 60
47 I Arcus Senilis 62
i Conjunctival Folliculosis........................ 47 I;
'Ocular Rosacea Miscellaneous Corneal Disorders ............................. 62
Benign Mucous Membrane Pemphigoid Sclerokeratitis (Sclerosing Keratitis) .................................... 62
47 V Thygeson's Superficial Punctate Keratitis 62
(Ocular Pemphigus)
hi - Erythema Multiforme Bullosum Corneal Pigmentation 63
38 p
f:j (Stevens-Johnson Syndrome)
‘ Dermatitis Herpetiformis & 48 |
SCLERA 63
.48 f
48 r ^•Episcleritis.............................................................................................. 64
Hi Epidermolysis Bullosa
48 1
Hl :Erythema Nodosum............................... Staphyloma .................................................. 64
.49 Scleritis 65
J
.p Psoriasis................................................. Hyaline Degeneration 65
Ip :• Reiter’s Disease Scleromalacia Perforans 65
.49 *
•Superior-Limbic Keratoconjunctivitis .49 J
I I ’
H; REGENERATIVE DISEASES 6. UVEAL TRACT 66
HPinguecula
-Pterygium . SPECIFIC TYPES OF UVEITIS 71
.49 j
• ' I •• • x'Uveitis associated with joint Disease 71
.49 |
MISCELLANEOUS DISORDERS OF ^Heterochromic Uveitis 72
' THE CONJUNCTIVA .50 !
*-*Lens-induced Uveitis -............................ ....73
;j; Lymphangiectasis .50 i
Chronic Cyclitis (Pars Planitis) .............................................................. 73
.50
Hl Congenital Conjunctival Lymphedema. ^Sympathetic Ophthalmia (Sympathetic Uveitis) 73
Subconjunctival Hemorrhage Tuberculous Uveitis................................................................................. 74
Hi
SfCORNEAOphthalmia Neonatorum
& SCLERA....................................................... Sarcoidosis (Boeck’s Sarcoid) 74
P|i Oculoglahdular Diseases Toxoplasmic Uveitis 75
CORNEA................ 51

CORNEAL ULCERS 7. RETINA 76

52
-*T Bacterial Corneal Ulcers ....................... 53
-U-Viral Corneal Ulcers.............................................. PHYSIOLOGY 77
54
Fungal Corneal Ulcers ........ 56
R Corneal Ulcers due to Hypersensitivity Reactions 56 DISEASES OF THE RETINA
Corneal Ulcer due to Vitamin A Deficiency 57 ,^/Retinal Artery Occlusion
7
6
 Retinal Vein Occlusion........................................................................... “Flashing Lights”..................................................................................... 100
Diabetic Retinopathy.............................................................................. “Vitreous Floaters”......................................................................... 10,1
Edema of the Retina............................................................................... Asteroid Hyalosis......................................................... ................... 10:1
Retrolental Fibroplasia........................................................................... Synchysis Scintillans................... ......................................................... 102
Retinitis Pigmentosa............................................................................... Massive Vitreous Retraction......................................................... J...... 102
Paving Stone Degeneration of the Retina.............................................. Injury to the Vitreous.......... ........................................................... ■...... 102
Lattice Degeneration............................................................................... Vitreous Inflammation..................................................................... :...... 103
Vitreous Hemorrhage............................................................................. 103
I
Retinal Tears & Holes.............................................................................
Retinal Detachment.......................................................... ..................... Vitreous Surgery.............................................................................*...... 10,3
y
M
THE MACULA.........................................................................................
Central Serous Detachment of the Retina.............................................. F.
10. ORBIT.................................... ~..................................... ...... 1d5J b.
Detachment of Pigment Epithelium........................................................ i •:
New Vessels beneath the Pigment Epithelium..................................... Exophthalmos (Proptosis)............................................................... 1Q6;:
Senile Degeneration of Bruch’s Membrane.......................................... Enophthalmos................................................................................. i-......... 1081;
Disciform Degeneration of the Macula Inflammatory Orbital Diseases...................................................... 1......... 108;;
(Kuhnt-Junius Disease).......................................................................... Orbital Cellulitis.............................................................................. i....... 108 i’
Angioid Streaks....................................................................................... Orbital Periostitis.................................................................... 109!:
Hereditary Diseases of the Macula............... 4........................................ Cavernous Sinus Thrombosis.......... ............................................•-....... 109.'
Color Vision & Color Blindness............................................................. Pseudotumor of Orbit....................... X.......109
i
i
I

8. LENS........................................................................... 11. NEURO - OPHTHALMOLOGY...................................... 110

CATARACT DISEASES OF THE OPTICNERVE..............................................«......112
Senile Cataract........................................................................................ Opitic Neuritis................................................................................ <....... 112i
Congenital Cataract................................................................................ Retrobulbar Neuritis....................................................................... 1....... 114
Traumatic Cataract...................................................................................
Cataract secondary to Intraocular Disease DEMYELINATING DISEASES.................................................... i....... ljl4;
(“Complicated Cataract”)......................................................................... Multiple Sclerosis....................................................................... ........... ljl4-
Cataract associated with systemic Disease.......................................... Neuromyelitis Optica (Devic’s Disease)..................................... !....... 1^15;
Toxic Cataract....................................................... Diffuse Periaxial Encephalitis (Schilder’s Disease)................... •....... 1jl5
437666305419600
Dislocated Lens (Ectopia Lentis)........................................................... 1j15;
PAPILLEDEMA & OPTIC ATROPHY..
Papilledema (Choked Disk).....-.......... 115
Optic Nerve Atrophy............................
9. VITREOUS............................................................................. Genetically Determined Optic Atrophy

Anatomy & Function ............................................................................... DISEASES OF THE OPTIC CHIASM

Composition.................................................... ....................................... . Lesions affecting the Optic Chiasm
Intrasellar Pituitary Tumors......................................................... .;........ 120
Examination of the Vitreous................................................................. '
Aging of the Vitreous............................................................................. ' Craniopharyngioma (Rathke Pouch Tumor)........................................ 1*20
i

8 9
 •1 *
i Suprasellar Meningiomas 12V
i Glioma of the Optic Chiasm 12f HEAD TRAUMA
; A
Subdural Hemorrhage 136
i /THE OPTIC PATHWAY 12I
Subarachnoid Hemorrhage ................................................ 136
’ -i i
! iTHE PUPILLARY PATHWAYS 12^! INTRACRANIAL INFECTIONS OF OPHTHALMOLOGIC
' j /Argyll Robertson Pupil 123: IMPORTANCE 137
; Tonic Pupil 123j Meningitis 137
: Horner’s Syndrome 123; Brain Abscess ............................. 138
■ ; j
[ ’i EXTRAOCULAR MOVEMENTS 125? THE PHAKOMATOSES .............................138
■Supranuclear Pathways 125<
: Lesions of Supranuclear Pathways ,...................... 125' CEREBROMACULAR DEGENERATION :........................... 139
• » 'Parinaud's Syndrome 1261
\ /Lesions of the Brain Stem 126? NIEMANN-PICK DISEASE ...............140
■ j Oculogyric Crisis... 1261
> j ' Supranuclear Syndromes involving MISCELLANEOUS DISEASES OF
•: • ^disjunctive Ocular Movements 126; NEURO-OPHTHALMOLOGIC IMPORTANCE 140
i 5Supranuclear Lesions & Strabismus 1271 Amblyopia due to Methanol Poisoning 140
| Nuclear & Intranuclear Connections 127. Nutritional Amblyopia (Tobacco-Alcohol Amblyopia) 141
^'Summary of Disorders of Cranial Nerves III, IV, & V! 128- Amblyopia due to Quinine & related Compounds ..................... 142
Jxl Syndromes Affecting Cranial Nerves III, IV, & VI ............................ 129 Amblyopia due to Salicylates............................................................... .142
y Herpes Zoster (Shingles) 142
; | NYSTAGMUS 130 y Neuroparalytic Keratitis 143
I ! Physiologic Nystagmus 131 Duane's Syndrome .................... 143
! -Pathologic Nystagmus ....................... 131 Migraine ............................. 143
j ’ ’ Congenital Nystagmus 131 Craniosynostosis (Oxycephaly) .................. 143
’ | /Spasmus Nutans 132 Mandibulofacial Dysostosis 144
. j :• Nystagmus due to Neurologic Disturbances 132 Waardenburg’s Syndrome ....... 144
5 j /.Vestibular Nystagmus 132 Myasthenia Gravis 144
! ! i Gaze Nystagmus .......................... 133 Central Nervous System Complications of Use
i "Voluntary & Hysterical Nystagmus .......................... 133 of oral Contraceptives .................................................... 145

I CENTRAL NERVOUS SYSTEM TUMORS OF

; j NEURO-OPHTHALMOLOGIC IMPORTANCE 133
j Cerebral Tumors ............................... 133 12. STRABISMUS ................................................... 146
: Cerebellar Tumors 134
Brain Stem Tumors 134 Anatomy 146
• Other Intracranial Tumors of Neuro-Ophthalmologic ^/Definitions ............................... 147
j / Importance 134 Physiology: Motor Aspects 148
Physiology: Sensory Aspects 149
■ ! ^CEREBROVASCULAR LESIONS OF OPHTHALMOLOGIC Objectives & Principles of Therapy of Strabismus ............................ 150
■IMPORTANCE 135 / Classification of Strabismus 151
10 11
 Esotropia INTRAOCULAR TUMORS 178
Nonparalytic Esotropia 15^ Primary benign intraocular Tumors 178
Nonaccommodative Esotropia 15? > Primary malignant Tumors of the Intraocular Structures 179
^Accommodative Esotropia...................... 15® i-
^^Paretic (Noncomitant) Esotropia 15«| ORBITAL TUMORS....................................................... 181
Exotropia (divergent Strabismus) 1555 Primary Orbital Tumors 18&
intermittent Exotropia............................. 15f£ Secondary Orbital Tumors & Metastatic Tumors from C
Constant Exotropia 156/ distant Sites ............................. .185
Hypertropia............................................. 157-
Heterophoria 15$
Discussion & Treatment of Heterophoria 1G0’ 15. TRAUMA.............................................................................. 185;
1 ' ’ I. ••

i x Nonpenetrating Injuries of the Eyeball..................... ............................ 1851

13. GLAUCOMA 161] Penetrating Injuries to the Eye .............................187 j
i injuries to the Lids 189\
Physiology of Glaucoma 162- Injuries involving the Orbit & its Contents 189 i
Special Diagnostic Technics 163/ f ’
Open-Angle Glaucoma 165? i i
Angle-Closure Glaucoma (Acute Glaucoma )
16. OPTICS & REFRACTION
Primary Congenital or Infantile Glaucoma
Secondary Glaucoma 168';
PHYSIOLOGIC ASPECTS OF OPTICS . 191:
1691
Glaucoma Secondary to Changes in the Lens............ 1701
Glaucoma Secondary to Changes in the Uveal Tract. REFRACTIVE ERRORS 192
170]
Glaucoma Secondary to Trauma 170j ^/Hyperopia 193
Glaucoma Secondary to Rubeossis lridis -^Simple Myopia......................................... 194
-> Glaucoma Secondary to the Use of topical 17lj Pseudomyopia 195
^Degenerative Myopia I. i
^Corticosteroids .1^5
(Malignant Myopia, Progressive Myopia)
^Acquired Myopia . .196
172; .196
14. TUMORS ...................................................... ^•Astigmatism
Presbyopia (Old Sight) ............ .197
1:
172
LID TUMORS ...................................................... 172
Benign Tumors of the Lids 173
✓ Primary malignant Tumors of the Lids 17. PREVENTIVE OPHTHALMOLOGY 199
J ’ ' =’
175.
CONJUNCTIVAL TUMORS 175 Glaucoma 199: I
Primary benign Tumors of the Conjunctiva 177 Amblyopia Ex Anopsia (“Lazy Eye”) 199 s
’ Primary malignant Tumors of the Bulbar Conjunctiva Corneal or Intraocular Infection 200 : ■
177 Ophthalmia Neonatorum ............... 206 ;• ’
CORNEAL TUMORS ................ 177 Ocular Injuries................................................... 20. ■;
Primary malignant Tumors of the Cornea Intraocular Foreign Bodies............................... ■ 201: ’

12 13
 ■ i’
i Radiation Injury
f | Blowout Fracture of the Orbit
i. J Xerophthalmia
Anatom
J Exposure Keratitis
i; | Visual loss due to Drugs & Other Iatrogenic Factors
Tarsal section of lid

18.’SPECIAL. SUBJECTS OF PEDIATRIC INTEREST

The Normal Eye in Infants & Children

: Congenital Eye Defects
» ' ^Developmental Body Defects associated with
jpcular Defects..........
[postnatal Problems....
•; iTests for Visual Acuity

SELECTED REFERENCE BOOKS

INDEX

i I
i■

Medial canthus

FIG 1-1. External landmarks ofthe eye. The sclera is covered by

transparent conjunctiva.

15
14
 PLICA SEMILUNARIS
SCLERA LACRIMAL CARUNCLE

LACRIMAL LAKE

CD

LIMBUS ORIFICES OF MEIBOMIAN

GLANDS
ANTERIOR POLE OF CORNEA
POSTERIOR LID MARGIN

FIG 1-2. External landmarks of the eye. The sclera is covered by transparent conjunctiva.

□ £
£%
s§ &*
-
J
c •e
F IG 1-3. In te rn a l stru ctu res o f th e h u m an eye.

o’
n
—
Q>
cr

3
a
I
w s OK'SIS ■sg^7 a
»»*3C “
 Superior sagittal sinus
Sinus cavemosus
Inferior sagittal sinus
\ Superior ophthalmic vuin
fr&vrttn u o'? mernb rjww? CcAmtft wdothelfosin
rontel vein
Corneal eplttefium .. . p««X-in'i&rs msnsterariB
Pigment
Carnert I serarna Nasofrontal vain

Angular vein
Ep*scteral
Inferior ophthalmic vein
Cana-1 ol Schlemm, Occipital
diploid vein
Anierfctrc&t&v
Scher-ijl spur. Posterior
Seitans auricular vein-
Superficial
temporal vein
Occipital vein Pterygoid plexus
Zonular fibers^ Anterlurfacial vein
empty External jugular vein
ClEiarV bpltW»Hum
Posterior facial vein
1
Stroma of iris Internal jugular vein
FIG1-4. Anterior chamber angle and surrounding structure. / x Submental vein

Soporipr obkqu-s X.-Z

FIG 1-6. Venous drainage system of the eye.

Superior rectus muscle^

VSuperlor palpebral muscle
Vortex
Vitreous

i Frontal nerve
Short paEtflrior
: cilioryarturisE Trochlear
; and elscrft
; dlijity nerve j, Oculomotor nerve

Lacrimal narve= - Anterior chamber

Long posterior
ciliary artery ,-ir>d Ahducens narve* Cornea
: Inferiorobhquh *5^.3 ciliary nerve
I Nasociliary nervs-^/JJJjfc

Maxillary nerve-"*. gJu/Optic nerve

f7 Inferior rectus \
Pterygopalatine ganglion-
Vortex '''■ muscle •
' Infraorbital nerve .
vain
Inferior oblique muscla

FIG 1-7. Lateral view of eye and surrounding structures.

FIG ] -5. Posterior view of left eye.

18 19
 Major arterial circle of iris

Conjunctival !

Vessels of
Anterior

vessels

Choroidal vessels

Retinal

Vortex vein

Long posterior ciliary artery

posterior ciliary arteries

Central FIG 1-9. Vascular supply to the anterior segment I ’!
Dural vessels • H
of rwina
i :l
vessels

FIG 1-8 Vascular supply to the eye. AH arterial branches originate with the ophtalmic artery.
Venous drainage is trough the cavernous sinus and the pterygoid plexus.

20 21
 45

T
■i- in the lower lid, appearing as yellow vertical streaks INFECTIONS & INFLAMMATIONS OF THE ^4-
V: deep to the conjunctiva. The meibomian glands LIDS
£
LIDS & LACRIMAL produce a sebaceous substance which creates an
oi I layer on the surface of the tear film. This helps
to prevent rapid evaporation of the normal tear
APPARATUS Jayer. The glands of Zeis are smaller, modified HQBnggLUM
.^x<Ac^cWj - Sebaceous glands which are connected with the
follicles of the eyelashes. The sweat glands of
*; Moll are onbranched sinuous tubules which begin Hordeolum is a common staphylococcal
in a simple spiral and not in a glomerulus as do infection of the lid glands which is characterized
ordinary sweat glands. by a localized red, swollen, and acutely tender
There is a gray line (mucocutaneous border)
QV • kn the margins of both the upper and the lower
"dj /til,.UriL-
— ”nr (-eyelids, if an incision is made along this line, the
The orbicularis oculi muscle, which is supplied |cxT
by the seventh cranial nerve, is roughly circular. Its lid can be cleanly split into a posterior portion,
X Anatomy
function is to close the lids. The levator palpcbrac containing the tarsal plate and conjunctiva, and
J U -i The- -•
j rrheheyelids are movable folds of tissue an anterior portion, containing the orbicularis oculi
muscle, supplied by the third nerve, inserts into the
which seirve to protect the eye. The skin of the tarsal plate and the skin and serves to elevate the lid. muscle, skin, and hair follicles.
lids is loose and elastic, permitting extreme The blood supply to the Lids is derived
The superior tarsal muscle (of Muller), supplied by
swelling and subsequent return to normal shape mainly from the ophthalmic and lacrimal arteries.
sympathetic nerves, originates in the levator muscle
and size.’ The tarsal plates consist of dense The lymphatics drain into the preauricular,
and inserts at the superior edge of the tarsus,
fibrous tissue with some elastic tissue. They are coursing deep to the levator aponeurosis. parotid, and submaxillary lymph glands.
lin^d posteriorly by conjunctiva and fuse medially
The 3 types of glands in the lid are the
and laterally to form the medial and lateral meibomian glands and the glands of Moll and Physiology of Symptoms
paipebrdjl tendons (ligaments), which attach to Lid disorders are among the most common
Zeis. The meibomian glands are long sebaceous
thcfjorbilaj bones. The orbital septum is the fascia of all ocular problems. The patient with
glands in the tarsal plate.
lying posterior to the orbicularis oculi muscle and They do not communicate with the hair disorders of the eyelids will have varied FIG 2-2. Internal hordeolum, left upper eyelid,
is the barrier between, the lid and the orbit. follicles. There are about 25 in the upper lid and 20 complaints. There are many pain fibers in the pointing on skin side. This should be opened by
tissues near the lid margins. Consequently, if a horizontal skin incision.
(here is inflammation with stretching of tissues,
as in hordeolum, the patient complains of It is essentially an abscess, as there is pus
1 moderately severe pain. In marginal blepharitis
*T<a.^(s5CS'\Sr .(^c,VvP U-.' formation within the lumen ofthe affected gland.
there is no pain but the patient complains of When it affects the meibomian glands it is
\ d * •^^Lator pajpebree musete-
J red-rimmed eyes; because of the proximity of relatively large and is known as an internal
Orbital fat
the lid margins to the conjunctiva, frequent hordeolum. The smaller and more superficial
Gland of Krouse^ ------ Orbicularis oculi muscle attacks of conjunctivitis are a common external hordeolum (sty) is an infection of Zeis’s
Gbnd of Wo"r,n” Orbital septum complaint. or Moll’s glands. Pain is the primary symptom,
Levatdr palpebrae aponeurosis Jf the patient has a foreign body sensation, and the intensity of the pain is in direct proportion
— Superior lersal
jrsal muscle i ."V*" entropion, with eyelashes rubbing on the to the amount of lid swelling. An internal
✓

Meibomian glond
cornea, should be ruled out. If the lid falls away hordeolum may point to the skin or to the
Conjuiicliva _ In larsal plate from the eyeball, as in ectropion, tearing will be conjunctival side of the lid; cxlenal hordeolum
. Eyelash the chief complaint, since the tears do not have always points to the skiti side of the lid margin.
access to the lower punctum. Exposure keratitis Treatment of both internal and external
Lower eyelid feiraclors may also occur with ectropion. hordeolum is with warm compresses for 10 -15
f.
minutes 3 times a day, and incision und drainage
Technic orUpperUd Eversion of the purulent* material if the process does not
Have the patient look down. Grasp the lashes begin to resolve within 48 hours. An ophthalmic
gently and exert pressure posteriorly and medially antibacterial instilled into the conjunctival sac
on the upper lid at the upper tarsal border with a every 3 hours is beneficial. A large internal
FJG 2-1. Cross section of the upper lid. cotton applicator. hordeolum can be complicated by cellulitis of the
- Wvzsckj-VK ..
' - CCVc’^\td<fV^<^Vfe'X,A L— v ■ t CH Ct \
 / n*c’r ' " M
J 3 ...........
j

.CaLclC 5
•T* I 7 L ■
CHALAZION Staphylococcal blepharitis is usually ulcerative. .■x- MEIBOMIAN ITIS Senile entropion is dne
cine to a.clegOncr
a.cegt&cfoftjpn
tazliXl f \ Seborrheic blepharitis (non-ulcerative) is usually of fascial attachments in the Jowerjeycfi-d,
associated with the presence of Pityrosporum j
including a dehiscenceof the ioWr eyciid
Chalazion is a sterile granulomatous ovale, although this organism has not been shown Bilateral, chronic inflammation of the aponeurosis (retractors) from the Iowcf~eye|.id
inflammation ofa meibomian gland, of unknown to be the etiologic factor. As a rule, both types are meibomian glands is an uncommon disease of tarsus, This allows preseptal orbiculaf.ishio
cause, characterized by localized swelling in the present (mixed infection). Seborrhea of the scalp, unknown etiology which occurs during or after override preLarsal orbicularis and rotate the I)d
upper or lower eyelid. It may begin with the middle years of life. It is generally preceded
margin inward. Cicatricial entropion is duelko
brows, and ears is frequently associated with
$
inflammation and tenderness similar to a seborrheic blepharitis. y'-dl^O by or associated with blepharitis. scarring of the tarsus palpebrarunL. and pis
hordeolum and develop over a period of weeks. The chief symptoms are irritation, burning, The patient complains of chronically red and therefore comnionTn trachoma.^jichiasis
The majority point toward the conjunctival side and itching of the lid margins. The eyes are “red- 'i irritated eyes and a slight but continuous (turning inwarcTof tiieTasKes~so that They rub
ofthe lid. When tire lid is everted, the conjunctiva rimmed.” Many scales or “granulations” can be discharge. The meibomian glands are prominent, on tlTT cornea) is a serious complication yof
over the chalazion is seen to be reddened and 4 entropion. It causes corneal irritation and-.mhy
seen clinging to the lashes of both the upper and the lid margins are red, and there is a frothy
r elevated. lower lids. In the staphylococcal type, the scales conjunctival discharge. A soft, cheesy yellow encourage corneal ulceration. i* |!
If sufficiently large, a chalazion may press are dry, the lids are red, tiny ulcerated areas are material which contains no organisms can be Surgery to evert the lid is effective in the
upon the eyeball and cause astigmatism. found along the lid margins, and the lashes tend expressed from the glands. An irritative treatment of both types of entropion. A tiseful
In general, a fully developed chalazion is to fall out. In the seborrheic type the scales are conjunctivitis due to contact with the meibomian temporary measure is to tape the lower lid|,to
differentiated from hordeolum by the absence of greasy, ulceration does not occur, and the lid secretion is a frequent complication. the cheek with tension temporally and ifrfciijjofjly.
acute inflammatory signs. margins are less red. In the more common mixed The only treatment is repeated expression of
type, both dry and greasy scales are present and the meibomian glands. However, because this
the Lid margins are red and may be ulcerated. treatment never produces dramatic results, the ECTROPION
Staphylococcus aureus and Pityrosporum ovale patient usually neglects to do it or have it done, J
can be seen together or singly in stained material and the disease process continues indefinitely with
scraped from the lid margins. a slight tendency to become worse. Ectropion (sagging and eversion ofiihe Jowcr
Conjunctivitis, superficial keratitis of the lid), usually bilateral, is a frequent finding in,oilier
lower third of the cornea, and chronic persons. Ectropion may be caused by T^larcafloh
meibomianitis are common complications of of the orbicularis oculi muscle, either as parfof
staphylococcal blepharitis. Seborrheic blepharitis POSITIONAL DEFECTS the aging process or following Bell’s palsy! The
is occasionally complicated by a mild keratitis. Or THE LIDS symptoms are tearing and irritation."Exposure
Persons with staphylococcal blepharitis are prone keratitis may occur.
to develop chalazions and hordeola.
FIG 2_~3.£?halazion, right lower.eyelid. The scalp, eyebrows, and fid margins must
teoA <4 cV\m. V-S i X entropion
be kept clean, particularly in the seborrheic type,
Chalazion seldom subsides spontaneously; by means of soap and water shampoo. Scales must ■■ ■
if it is large enough to distort vision or to be a be removed from the lid margins daily with a damp
cosmetic blemish, excision is indicated. catton applicator. Entropion (turning inward
IkJ of the 1IU./ usually
utlG lid)
. Pathologically, there is proliferation of the Staphylococcal blepharitis is treated with affects the lower lid but may £affect the upper lid. \
endothelium of the acinus and a granulomatous antislaphylococcal antibiotic or sulfonamide eye It seldom occurs in persons under 40 yenrs.o£age
n der dftygars nfage. /
inflammatory response, including some Langhans ointment applied on a cotton applicator once daily The 2 conimqn~tVDes are senil ' ■■■* and_
"e .(spastic) %
type giant cells. to the lid margins. / <] hO»T cicatricial.
Meibomianitis cannot be curra permanently]
although expression of the material from the glands
1
may give temporary relief. Staphylococcal FIG 2-5. Ectropion.
MARGINAL BLEPHARITIS* conjunctivitis or keratitis usually disappears
•( 1 . (Granulated Eyelids) promptly following local antistaphylococcal Marked ectropion is treated by su'rgical
medication. X CyC ) shortening of the lower lid-in a horizontal
The seborrheic And staphylococcal types direction. Minor degrees of ectropion qanPbe
Blepharitis is a common chronic bilateral usually become mixed, and may run a chronic treated by several fairly deep clecttocdutery
inflammation of the fid margins. There are 2 course over a period of months or years if not. penetrations through the conjunctiva 4-5 mm
main types: staphylococcal and seborrheic. treated adequately. Tronfthe fid margins at the inferior aspect ofjhe
tarsal plate. The fibroLic reaction which follows

-o
wifi frequently i?{W the lid up to its nprrhal
r 1G 2-4. Entropion position.
J
 DEFORMITIES OF THE LIDS EPICANTHUS f short time, the prognosis for cessation of the muscle al any level from the oculomotor nucleus
blepharospasm is good. Long-standing cases (midbrain) to the myoneural junction.
jf feLEPHAROCHALASIS & Epicanthus is characterized by vertical folds tend to persist regard!less of treatment.
of skin over the medial canthi. It is present to some t? Clinical Findings
i ; DERMATOCHALAS1S degree in most children. The skin fold is often Congenita] ptosis is evident at birtli and is
large enough to cover part of the nasal sclera and ? PTOSIS often observed to be associated with weakness ot
cause “pseudoesotropia,” as the eyes appear to other extraocular, muscles.
IM;; . ■
. Blepharochalasis is redundancy of the skin Drooping of the lids when the eyes are open The affected lid has a smooth, flat
be crossed when a normal amount of medial sclera ’
of tlid pipper or lower lids associated with is not visible. Prominent epicanthal folds in L may be unilateral or bilateral and constant or appearance, and the tarsal fold caused by the pul!
lympiiH^etha and a.defect in the orbital septum. children gradually decrease as the child grows L intermittent. of a normal levatormuscle is absent. This is more
It ofte^f occurs in young adults, and a familial older and are seldom apparent by school age. £ noticeable on upward gaze, when the lid fails to
tendency is often present. Treatment is surgical, Etiology refract asj.be eye moves~upwajd-
but th (^condition may recur. A. Congenitak If theTicTdroops enough to partially occlude
Congential ptosis is usually the result of the pupil, the child usually attempts to compensate
developmental failure of the levator muscleofthe by elevating the brow with the frontalis muscle.
liHTalone or jnassocigton with anomalies of the This produces a marked Wrinkling ofthe forehead,
superior rectus muscle (most frequent) or most evident when the condition is unilateral.
complete external ophthalmoplegia (rare). It may If the pupil is completely occluded,
be transmitted as a dofmriant characteristic. amblyopia ex anopsia may occur.
The ptosis ofmuscular dystrophy progresses
B. Acquired: Acquired ptosis can be very slowly and insidiously but finally becomes
considered in 3 main categories: complete.
1. Mechanical factors-Abnormal weight of Ptosis in rnyasthenja_giayjs is gradual in
the lids may make it difficult for a normaTTevator onset, characteristically appearing in the evening,
FIG 2-8. Epicanthus. fnuscle to elevate fully. This may be due to acute wjtlvfatigue, and improving overnight Later it
■ EKr^rtf. Blcpharochalasis. or_chronic inflammatory edema or swelling, becomes permanent. In over 80% ofcases, some
’ i'l! ■« tumor, or an extra fold of fatty material, as in degree of transient orngrrnanenLophthalmoDlegia
i P4nriatochalasis is redundancy of upper or BLEPHAROSPASM (Tic) \ xanthelasma. follows and produces diplopia. A diagnostic test
lower lid tissues due to aging processes. It may 2. Myogenic factors (eg, muscular of injection of neostigmine (Prostigmin) or
also, p'jptijr following repeated bouts of Blepharospasm is persistent or repetitive ! dystrophy, myasthenia gravis)-Ptbsis of one or edrophoniumfTensi Ion) usuallygives a dramatic
angioneurotic ederqa with consequent stretching r involuntary contraction of the orbicularis oculi • both lids is often the first sign of myasthenia response by temporarily abolishing the ptosis.
of the sjjim ijExcisiori of a portion of the skin of the muscle. It is usually bilateral and is more common ; gravis and occurs eventually in over 95% of cases. Neurogenic pinsis_presents different clinical
lids is Required if vision is affected or for cosmetic in older persons. The cause is not known. It may i The essential defect in myasthenia gravis seems pictures according to the level of the pathway
reaspn&j Prolapsing orbital fat may be removed at be due to irritative Jesions of the cornea and ( to be in the Immoral transmission at the myoneural affected. 'hre^fmBiftgs^off^refTmrtions of a
the sametime. ? conjunctiva or, of--the seventh cranial nerve. • junction. neurologic syndrome usually help establish the
Hi if 8 r-motioharstresTand fatigue make it worse. The Neurogenic (paralytic) factors- diagnosis.
eyes sho’uTe be examined carefully to rule out i Interference with the pathways of the portion of
irritative lesions such as corneal foreign body, i the third cranial nerve sup: lying the levatqr
' meibomianitis, and trichiasis/
Treatment consists of removal of the !
causative factor if possible. If the specific etiology
is not known, explanation and reassurance are in ?
order. Intractable cases may require alcohol I
injection of the orbicularis oculi muscle to
produce temporary paralysis?^eventh nerve.block
with a long-actingloCaTanesthetic should be tried »
before alcohol injection is resorted to. Plastic
surgery designed to weaken'orBicularis function
FIG pcrniatqchalasis of upper lids and and selective removal of seventh nerve branches i
hcn.iialjipri of orbital fat of lower lids without have been successful in some cases-. FIG 2-9. Surgical correction of ptosis. Left: Before operation, ptosis of the upper lid was present.
bl cph arocli alasis. if the condition has been present for only a Right: After the operation (levatorresection), the ptosis was well corrected and a natural-appearing
upper lid fold produced.
26 27
 2_
TYcatmcnt moistening the surfaces of these structures. They g the canaliculi and the nasolacrimal ducts and by Clinical Findings
If the condition is so slight that no cosmetic drain into the lacrimal canaliculi through theS palpation of the lacrimal gland. If the complaint is
deformity is present and there is no interference ] acrimal puncta. round apertures about CTT of “dry eyes”, he also approximates the quantity The chief symptoms ate tearing |anil
with visual acuity, it is best left alone. in diameter on the medial aspect of both thejmper V of tear production (Schinner test). discharge. In. the acute form, infiammatiojnj pain,
Myasthenia gravis should be treated as and lower lid margins. There are many causes of tearing, eg, swelling, and tenderness are present,in.(ear
indicated (with neostigmine or a similar drug). conjunctivitis, keratitis, iritis, and foreign bodies. sac area; purulent material can be expressed from
Some relief may be obtained by wearing However, if tearing is the only symptom, the cause the tear sac. In the chronic form, tearing
special spectacle frames which have a posteriorly in the great majority of cases will be found in the the only sign; mucoid material can usually be
attached wire crutch that suspends or elevates the lacrimal drainage apparatus. expressed from the tear sac. | |i; ?
lid by traction. This may be indicated in temporary' The infectious agent can be identified
paresis or in those who are not good candidates microscopically by staining a conjunctival sijr.car
for surgery. INFECTIONS of the lacrimal taken after expression of the tear sac. «' !. • ;
Surgery is the treatment of choice for APPARATUS Corneal ulcer occasionally occurs friliovJing
cosmetic reasons, and should be performed early minor corneal trauma in the preseinbe| of
if vision is occluded. The type of surgery varies pneumococcal dacryocystitis. Perforation bfjlhe
with the cause: 1. DACRYOCYSTITIS skin and fistula formation may also occur.’. If a
(1) If the levator muscle is not completely comeal ulcer occurs, vigorous ld.cal andsysteknic
paralyzed, resection or shortening of the muscle Infection of the lacrimal sac is a common treatment is indicated and a dacryocystectojiijy or
is the procedure of choice. FIG 2-10. ThE lacrimal drainage system. | acute or chronic disease which usually occurs in dacryocystorhinostomy should -be done xvjfltput
(2) If the levator muscle has no action, the infants or in persons over 40 (about 9 out of 10 delay. ! ■ ?d; ’
elevating effect of the frontalis muscle may be The canaliculi are about 1 mm in diameter chrome gtlult cases occur in menopausal women);
utilized by passing some type of traction device and 8 mm long, and join to form a common { it is uncommon in the intermediate age groups. It Treatment
(wire, fascia, or suture) subcutaneously from the caflaliculus justKefore opening into the lacrimal ( is most often unilateral and is always secondary
frontalis to the tissue of the eyelid (tarsus), so that sac. Diverticuli may be a part of the normal j to obstruction of the nasolacrimal duct. In many A. Adult Dacryocystitis: Warm compresses
when the brow is raised the lid will be elevated. structure and are susceptible to fungal infection. adult cases the etiology of the obstruction remains to the affected eye at frequent intervals during
The advantage of this procedure is that no tissue The lacrimal sac is the dilated portion of the ‘1 unknown, but there may be a history of severe the acute stage. |i j|iji I
is destroyed and the traction material may be lacrimal drainage system which lies in the bony trauma to the nose. Acute cases are often 1. Acute-Specific treatpienl fof qidute
withdrawn and reinserted if desired. The risk of lacrimal fossa. preceded by chronic dacryocystitis: some cases staphylococcal or pneumococcal dacryqcyMitis
infection is great unless the patient’s own fascia The nasolacrimal duct is the downward i are preceded by chronic conjunctivitis (eg. consists of penicillin, 1 million units daily pvl|until
lata is used. continuation of the lacrimal sac. It opens into the trachoma). In acute dacryocystitis the usual the inflammation subsides. •' ; ; i
inferior meatus lateral to the inferior turbinate. infectious agent is one of the staphylococci; in 2. Chronic-Since an obstruction* b-|jl:he
All of the passages of the lacrimal drainage | chronic dacryocystitis, Diplococcuspneumoniae nasolacrimal duct is the basic cauisjjb, of
PSEUDOPTOSIS system are lined with epithelium. The tears pass or occasionally Haemophilus influenzae. dacryocystitis, the disease is usually perslsteru
into the puncta by capillary attraction. The ; Candida albicans can cause chronic until the obstruction is relieved. However,'probing
Pseudoptosis may occur when the upper lid combined forces of the capillary attraction in the dacryocystitis and may form a complete cast of the is notably unsuccessful in adults,''and
lacks its normal support, as yritfra shrunken or canaliculi, gravity, and the pumping action of the nasolacrimal duct. (Mixed infections do not occur). dacryocystorhinostomy is usually necessary if
orbicularis oculi muscle on the lacrimal sac tend symptoms are severe. If chronic tearing is the-dnlj'
to continue the flow of tears down the I symptom, many patients prefer the tejanii|j to
nasolacrimal duct into the nose and naso-pharynx. surgery. ' |H?M
i ’ r i *>1 ; !

U, THE LACRIMAL APPARATUS Physiology of Symptoms B. In fan tile Dacryocystitis: Normall^ the
Patients with disorders of the lacrimal nasolacrimal ducts open spontaneously fJhriiig
apparatus complain of tearing or “dry eyes.” In the first month of life. Occasionally onej^f the
Anatomy the event of "tearing” without associated ducts fails to canalize and a seeoji'daTy
Tbe lacrimal apparatus consists of the symptoms, the pathology is in the lacrimal pneumococcal dacryocystitis develops. Wheiithis
Iacrifgai_ gjanj, accessor/ glands, canaliculLte^_ drainage system or the result of hypersecretion. Fig 2-11. Acute dacryocystitis. happens, forceful massage of die tear sac is indicated,
' Sac, and naso-lacrimai duct. The lacrimal gland Paradoxic lacrimation (an occasional late and antibiotic or sulfonamide1 drops should be
isqjear-secreting gland located in the anterior complication of seventh nerve palsy) is a It is curious that dacryocystitis is instilled in theconjunctival sac 4-5 times dailyJ|tftHis
superior temporaTportion of the orbit. Several condition in .which salivary gland fibers innervate seldom complicated by conjunctivitis even is not successful after a few weeks probing' of the
secretory ductsconnecl the gland toTHc superior die lacrimal gland. If the eyes are dry, there is faulty though the conjunctival sac is constantly nasolacrimal duct is indicated • regardless jdf the
corijunctival fornix. The tears pass.down. over the production, of tears. The physician investigates being bathed with pus exuding through the inlands age. The tearsac is ini gated freelyjust bifore
cornea^OuIHariand pajpeftfal”conjttncliva, the symptoms by irrigation to test the patency of lacrimal puncta. probing. One probing is effective in about 75% of
29
cases’ in (he remainder, cure can almost always be 3. DACRYOADENITIS
achieved by repeated probings or by a temporary
silicohe lacrimal drainage splint. Acute inflammation of the lacrimal gland isa'-X
3
Course & Prognosis
rare unilateral condition which may be seen ia:.;i
children as a complication of mumps, measles /J TEARS
I Acute adult dacryocystitis responds well to or influenza, and in adults in association with,*
sysfefriici antibiotic therapy. Recurrences are gonorrhea. It may also develop following
coninaon if the nasolacrimal duct obstruction is not perforating injury to the lacrimal gland.
remoyed.-The chronic form can be kept latent by In the acute type there is considerable-/,
using antibiotic eye drops, but relief of the swelling, pain, and injection over the upper'I
obstruction is the only cure. temporal aspect ofthe eye. This is treated by warm h
compresses.
= •; If bacterial infection is present, antibiotics/ SOUROE & FUN<: TION OF THE TEARS Although lysozyme is known to have a lytic
H ’ 2. CANALICULITIS are given systemically. Incision may be necessary / effect on certain bacteria, its absence does not
;‘.i
if pus collects in the gland under tension. necessarily increase the risk of infection.
The tears are a mixture of secretions from the
’’Ipanaliculitis is an uncommon chronic Chronic dacryoadenitis is occasionally seen t
major and minor (accessory) lacrimal glands, the Reduction in tear lysozyme concentration usually
unilateral condition caused by infection with bilaterally as one manifestation of sarcoidosis. *
goblet cells, and the meibomian glands. Under occurs early in the course of Sjogren’s syndrome
Actinomyces israelii, Candida albicans, or This condition (which, with lacrimal gland g:
normal circumstances, the tear fluid forms a thin and is considered helpful in the diagnosis of that
Aspergillus species, it affects the lower canaliculus swelling, is called Mikulicz’s syndrome) usually |
morejaften than the upper, occurs exclusively in layer approximately 7-10 mm thick that covers disorder. Lysozyme in tears can be measured by
occurs in blacks and is self-limited. The prognosis turbidimetric assays utilizing the microorganism
the corneal and conjunctivaL epithelium. The
adults, and causes a secondary purulent is good. Chronic dacryoadenitis may also occur £
functions of this ultrathin layer are (I) to make Micrococcus lysodeiktikus (heat-killed) as the
conjunctivitis which frequently escapes etiologic in tuberculosis, lymphatic leukemia, and ?
the cornea a smooth optical surface by abolishing substrate. This can be performed on tear samples
diagnosis! lymphosarcoma. 4
minute surface irregularities ofits epithelium; (2) collected on regular Schirmer strips.
The patient complains of a mildly red and
to wet the surface of the corneal and conjunctival The average’glucose concentration of the
irridjtcd eye with a slight discharge. The organism
epithelium, preventing damage to the epithelial tears is 2.5 mgZ 100 ml. An approximate
can be seen microscopically on a direct smear taken
cells; and (3) to inhibit the growth of .determination of the amount in patients with
frorii the canaliculus.
microorganisms on the conjunctiva and cornea hyperglycemia caii be made with a commercially
■ Curpttage of the necrotic material in the
by mechanical flushing and the antimicrobial available colorimetric paper test strip (Clinistix) '
involvedfcanaliculus, followed by irrigation, is
action of the tear fluid. thathas been moistened with tears. This is a useful
usually effective. Canaliculotomy is sometimes
test in comatose patients.
necejsparyl Tincture of iodine should be applied to
The average tear urea level is 0.04 mg/ 100
the lifting ofthe canaliculus alter canaliculotomy.
COMPOSITION OF THE TEARS ml. Changes in the blood concentrations of both
glucose and urea parallel changes in the tear
The normal tear volume is estimated to be glucose and tear urea levels.
about 6 ml in each eye, and the average rate of K\ Na+, and Cl- occur in higher
turnover about 1.2 ml permmute. When collected concentrations in tears than in plasma. The
with minimal trauma, tear fluid contains a high average pH of tears is 7.35. Under normal
concentration of proteins. Three fractions are conditions, tear fluid is isotonic.
demonstrable by paper electrophoresis: albumin, If collection of the tear fluid is traumatic, the
globulins, and lysozyme. The antimicrobial normal constituents of the tears may be altered and
activity of the tears is m the gamma globulin and there may be transudation of substances from the
lysozyme fractions. blood. In certain inflammatory conditions of die
The gamma globulins found in the normal conjunctiva, there is marked transudation of
tear fluid are IgA, IgG, and IgE. The IgA immunoglobulins directly from the blood to the tear
predominates and is .similar to the IgA found in fluid.
other body secretions bathing mucous membrane
left eye. surfaces such as saliva and the bronchial, nasal,
and gastrointestinal secretions. The IgA found in LAYERS OFTHE PREOCULARTEAR FILM
tears differs fromsenim IgA, however, and is more
concentrated. In certain allergic conditions such The tear film covering the corneal and
as vernal conjunctivitis, the IgE concentration of conjunctival epithelium (pieocular tear film) is
tear fluid increases. composed of 3 layers (Fig 3-1): (1) The superficial
30
31
 $
'A to the corneal surface, caused by corneal corneal epithelium and the other end moving
lipid layer is a monomolecular layer derived from DRY EYE SYNDROME scarring and loss of epithelial microvilli, or freely.
the secretions of the meibomian glands and (Keratoconjunctivitis Sicca: KGS) 2 to deficient blinking. Three types of corneal filaments have be en
thought to retard evaporation of the aqueous layer. ;-a recognized: (1) filaments consisting entirely of
(2) The middle aqueous layer is elaborated by the Deficiency in any of the tear film components'^ mucus, (2) filaments consisting entirely of
major and minor lacrimal glands and contains may lead to loss of the film’s stability. This will# epithelial cells, and (3) filaments consisting O.f
Clinical Findings
water-soluble substances (salts and proteins). cause rapid break-up ofthe tear film, and dry spots-- epithelial cells and mucus. •;
(3) The deep mucinous layer is composed of will appear on the cornea] and conjunctival®
A. Symptoms and Signs: Patients with dry In patients with Sjogren’s syndrome,
glycoprotein mucin and overlies the corneal and epithelium. Dryness ofthe eye may therefore result? conjunctival scrapings may show increased
eyes complain most frequently of a scratchy or
conjunctival epithelial cells. The epithelial cell from any disease associated with deficiency ofh numbers of goblet cells. f j ’
sandy (foreign body) sensation. Other common
. membranes are composed of lipoproteins and are the tear film components (aqueous, mucin, orf; »•
symptoms are itching, excessive mucus secretion,
therefore relatively hydrophobic. Such a surface lipid). Although there are many forms of KCS,;: B. Schirmer Test: The use of wettab Ife filter
inability to produce tears, a burning sensation,
cannot be wetted with an aqueous solution alone. those connected with rheumatoid arthritis or other 1 ’ photosensitivity, redness, pain, and difficulty in paper strips as a device for measuring tear
Mucin (glycoprotein) plays an important role in connective tissue diseases are commonly referred £ secretion was first described by Koster iii 1900
moving the lids. In most patients, the most
.wetting this surface. It is partly adsorbed onto to as Sjogren’s syndrome. remarkable feature of the eye examination is the in connection with a study of facial-'Tierye
the corneal epithelial cell membranes and is paralysis. Koster placed one end of a fillejr paper
grossly normal appearance of the eye.
anchored by the microvilli of the surface epithelial Etiology Lacrimal gland enlargement occurs strip 1 cm wide and 20 cm long in the conjiinctival
cells. This provides a new hydrophilic surface for Conditions predisposing to or characterized uncommonly in patients with Sjogren’s syndrome. sac in each eye and noted the extent of Wetting
the aqueous tears to spread on, arid the surface is by dryness of the eye axe the following: I The most characteristic feature on slitlamp by the tear fluid. In 1903, Otto Schirmer modified
wetted by a lowering of the tears’ surface tension. examination is the interrupted or absent tear Koster’s method by reducing the width; of the
A. Conditions characterized by hypofunction '■* meniscus at the lower lid margin. Tenacious strips to 0.5 cm and their length to 3.5 cnii.
of the Lacrimal Glands: | yellowish mucous strands are sometimes seen in The accessory lacrimal glands of V/olftijng
Sj ogren’s syndrome. r the lower conjunctival fornix. The bulbar and Kraus secrete enough tears to keep thc’ccr^ea
Riley-Day syndrome (familial dysautonomia). conjunctiva loses its normal luster and may be adequately wetted under most circumstances, gnd
Congenital alacrima. | thickened, edematous, and hyperemic. the major lacrimal glands secrete enoughTo help
Trachoma. cover stressful ,circumstances such as drying from
• • t
Surgical removal of the lacrimal gland. exposure to hot air, irritation from corneal injiliy,
Mumps. V: foreign bodies, emotional upsets, and ^arijeius
Neurogenic lesions. e noxious stimuli. Schirmer tests performedyrithdut
Systemic diseases with lacrimal gland r, topical anesthesia measure the function, ofjtjie
involvement (sarcoidosis, lymphoma, i: lacrimal gland, whose secretory activity is
leukemia, etc). * stimulated by the irritating nature of the fjller
Drug effects (eg, atropine, diuretics). ? paper. Schirmer tests performed after* top^al
Old age. $ anesthesia (instillation of 1 % tetracaine) measure
■j
the function ofthe accessory lacrimal glajnds |(jhe
B. Excessive Evaporation of the Tears, 4 basic secretors). ■
Caused by the Following: The Schirmer test is a good screening test-
Exposure keratitis; neuroparalytic keratitis. and definitely the simplest test-for the assessment,
Living in dry climates, eg, desert areas. of tear production. However, falsc-posi(ive| and
Deficiency of the supefeial lipid layer ofthe : false-negative results occur in 15% ofeyes tested;
FIG 3-1. The 3 layers of the tear film tear film (lid surgery, distichiasis). j FIG 3-2. Corneal filaments in a 56-ycar-old a positive result (decreased wetting of the filter
covering the superficial epithelial cell layer patient with keratoconjunctivitis sicca. paper strip) should be confirmed and a fiegative
ofthecornea. C. Mucin Deficiency, Caused by the ‘ result should by no means rule out dryness ojf the
Following: eyes, particularly if it is secondary to mucin
Mucin is elaborated by the goblet cells of Avitaminosis A. The damaged corneal and conjunctival deficiency. I
the conjunctiva, and recent studies have shown Stevens-Johnson syndrome. epithelial cells stain with 1% rose bengal , and A Schinner test showing less than 15 mtii of
that the lacrimal gland contributes to its Pemphigoid. . defects in the corneal epithelium stain with wetting in 5 minutes is considered abnorinal^ ;
production. Trachoma. fluorescein. The corneal epithelium shows
Periodic resurfacing of the tear film is Chemical bums. varying degrees of fine punctate stippling in the C. Tear Film Break-Up Time: At present
important to prevent dry spots and is Chronic bacterial or viral conjunctivitis. interpalpebral fissure. there is no practical method of measuring 'the
accomplished by blinking. In the normal eye, In the late stages of KCS, filaments may be mucin content of the tear fluid, but measurement
blinking maintains a continuous tear film over the D. Other Disorders: Ocular dryness may also seen - one end of each filament attached to the of the tear film break-up time may sometimes be
ocular surface. be due to less than normal mucin adsorption 33
32
 very use ill. Deficiency in mucin may not affect tension of the tears, assisting in the spreading?!,
the Schinner test But may lead to instability of the
tear film This caiises the film’s rapid break-up.
of the tears, and wetting the epithelium. If tbe^j
mucus is tenacious, as in Sjogren’s syndrome,
4
“Dry holes” are formed in the tear film, and a
I ba'ring or the corneal or conjunctival epithelium
mucolytic agents (eg, acetylcysteine, 20%) may £
give some relief. -
CONJUNCTIVA
! follows. This ‘ process ultimately damages the :’S-

; epithelia cells, which can then be stained with

L . --------------.....
rose benp al. Damaged epithelial cells may be shed OTHER DISORDERS OF THE LACRIMAL &
■
from the! cornea,^ leaving areas susceptible to SYSTEM %
punctate staining, when the corneal surface is
flooded with fluorescein. Lacrimal Hypersecretion
The tear film break-up time can be The causes of excessive tearing are varied J
measured by applying a slightly moistened but are due to stimulation ofthe lacrimal gland. ANATOMY & PHYSIOLOGY DISEASES OFTHE CONJUNCTIVA
flqorescain strip to the bulbar conjunctiva and • Psychic lacrimation is normally associated
asking th'e patient,to blink. The tear film is then with pain or emotional upsets. The fact that tliis f
scanned with the' aid of a slitlamp while the type of lacrimation appears after the first few « The conjunctiva is a thin, transparent CONJUNCTIVITIS
patierit jpfrains from blinking. A cobalt blue months of life explains why newborns do not mucous membrane which Lines the anterior
, filter and a broad? light beam are used for this produce tears when they cry. Conjunctivitis is the most common of
sclera up to the limbus and the 1 0 terior surface
puqjope.lThe time that elapses before the first Neurogenic lacrimation is brought about by •< all eye diseases in the Western hemisphere.The •
of the lb s, There are 2 divisions of the
hole (dry spot) appears in the corneal reflex stimulation. Eyestrain, corneal injury, a $ majority are exogenous, but conjunctivitis may
conjunctiva: palpebral and bulbar. The palpebral
I fluorescein layer is the tear film break-up time. blast of hot air, dry wind, or foreign body in the :•* rarely be endogenous. Bacterial conjunctivitis,
portion lines the lids and the bulbar portion lines
Normally, the break-up time is over 15 seconds, comea or conjunctiva may cause reflex trigeminal 4 the most frequent type, is self-limited, lasting
the eyeball. At the fornices the conjunctiva is
but it can be reduced appreciably by the use of irritation that excites lacrimation. Strong light J about 10-14 days untreated. Treatment with one
local anesthetics,-by manipulating the eye, or causes reflex visual irritation and copious (j reflected from the lids over the eye. Anteriorly
the conjunctiva is continuous with the corneal of the many available antibacterial agents
by; holding the lids open. The break-up time is lacrimation. Irritation of the facial nerve, yawning, -j
epithelium. Immediately beneath the bulbar usually clears the condition in 1-3 days.
i shorter in eyes with aqueous tear deficiency vomiting, and laughing are also associated with
conjunctiva is the anterior portion of Tenon’s
Trachoma and inclusion conjunctivitis can be
■ and is-always shorter than normal in eyes with reflex lacrimation.
\ mucin! deficiency. Epiphora may follow obstruction of the capsule. Tenon’s capsule and the conjunctiva treated successfully with antibiotics.
j j li
lacrimal drainage system. This can be caused by >. arc fused around the limbus in a zone about 3
Viral conjunctivitis is also relatively
) /Complications
“•. J.., ~
t punctal eversion or occlusion or by canalicular or | mm wide. In the region of thE inner canthus the
common. The discharge is usually watery rather
Early in the cdurse of KCS, vision is slightly nasolacrimal duct obstruction. Most cases of. £ conjunctiva is slightly thickened to form the
than purulent. Viral conjunctivitis is selflimited,
impaired and a few patients develop comeal nasolacrimal duct obstruction can be corrected ! % lasting 1-3 weeks regardless of treatment.
plica semilunaris. The caruncle, a small fleshlike
juiceration, comeal thinning, and perforation. surgically. 5 Topical antibiotics are generally used to prevent
epidermoid structure, is attached superficially
Secondary’ bacterial infection occasionally occurs, secondary infection.
■and corneal scarring and vascularization may result
to the inner portion of the plica. Several small
Paradoxic Lacrimation (“Crocodile Tears”) '* Allergic conjunctivitis causes tearing,
in markedI reduction, in vision. Appropriate early accessory lacrimal glands aTe present in the
This is an acquired unilateral (very rarely 1 itching, and injection with a slight stringy
treatment jmay prevent these complications. bilater-al) condition characterized by excessive . I superior fornix as well as the openings of the
discharge. It is usually chronic and bilateral.
tearing while eating. It occurs as a sequel to Bell’s I ducts from the lacrimal gland.
palsy (facial nerve palsy) and is the result of I Histologically, the conjunctiva consists of Corticosteroids instilled locally are effective.
TYeatmcrt
2 layers: the epithelium, composed of cylindric Fungal and parasitic conjunctivitides are
Tre itment depends upon the cause. In most aberrant regeneration of the facial nerve fibers. ;
jearly cases, the corneal and conjunctival epithelial i cells, and the substantia propria, which is usually unilateral and often present as a
changes are reversible. Bloody Tears divided into adenoidal and fibrous layers. The localized inflammatory granuloma in the
| Aqueous deficiency can be treated by This is a rare clinical entity attributed to a * conjunctiva. An example of this is the
substantia propria contains goblet cells. These
replacement of the aqueous with various types variety of causes. It has been associated with ! are essentially unicellular mucous glands; the granuloma seen in Leptotrichia conjunctivitis.
of artificial tears.. Mucin deficiency can be menstruation (“vicarious menses”). Blood- Fungal and parasitic conjunctivitides are rare
■pa^ti^Jly c —r
mucinous substance produced assists the tears
compensated for by the use of tinged tears may be secondary to conjunctival ’ in most parts of the world and are given only
in keeping the conjunctiva and cornea moist.
ophthalmic vehicles of high molecular weight­ hemorrhage due to any cause (trauma, blood » brief discussions in the following pages.
The conjunctival blood vessels are derived
leg,; water-soluble polymers-or by the use of the dyscrasia, etc) or to tumors of the lacrimal sac. *.
patient's own serum as local eye drops. Serum from the anterior ciliary and palpebral arteries.
They have also recently been reported in a >
Physiology of Symptoms
used for this purpose must be kept refrigerated hypertensive patient suffering from epistaxis j The nerves are from the ophthalmic division of
the fifth cranial nerve. The conjunctiva is rich Because of its exposed position, the
at all tinges. It acts by lowering the surface with extension through the nasolacrimal duct. I
conjunctiva comes in contact with more
1 in lymphatics.
34
35
 Atopic keratoconjunctivitis reaction consisting of fibrin and edema plus I
microorganisms than any mucous membrane. Etiologic Classification
Phlyctenular keratoconjunctivitis polymorphonuclear leukocytes, lymphocytes} apd
Organisms commonly found in the urethra seem
Drug reactions plasma cells. The number and type of cells depend
to thrive on conjunctival tissue, whereas those A- Bacterial:
1. Pyogenic- upon the stage of the inflammation. Certain
that grow abundantly on the nasal mucosa do »:
F. Traumatic: organisms tend to provoke, specifi c tissue
less well in the conjunctival sac. For example, the
conjunctiva is relatively immune to the virus of
Diplococcus pneumoniae*
Haemophilus injtuenzae
I Foreign bodies
•
responses, such as follicle and papilla fcmigtion,
!

Chemicals membranes, ulcers, and j loinas.

granulomas.
Staphylococcus aureus*
the common cold, whereas the gonococcus and Other irritants Histologically, follicles are nests of lyimhcif yLes
Neisseria gonorrhoeae *
.. the agent of inclusion conjunctivitis, neither of surrounded by a vascular network and having; a
Streptococcus viridans
which will grow in the nose, multiply rapidly in Haemophilus aegyptius (Koch-We bacillus) V G. Fungal: germinal center containing lymphoblasts Papillae
the urethra and in the conjunctival sac despite Neisseria meningitidis Candida albicans (ocular candidiasis, are nodular elevations at the -limbus cr tarsus,
!- thrush of eye) where the conjunctiva is adhere n" to the
the fact that the tears are constantly washing Proteus vulgaris
- organisms down the nasolacrimal duct into the Hemolytic streptococci Sporotrichum schenkii underlying tissue; they are caused- By
Pseudomonas aeruginosa subconjunctival exudation and cellular infiltration,
nose. Many organisms pathogenic for the
, genitourinary tract are also pathogenic for the Escherichia coli H. Parasitic: forcing the epithelium into polygonal elevations.
2.Nonpyogenic Onchocerca caecutiens A conjunctival membrane consists of a superficial
conjunctiva.
Corynebacterium diphtherias Loa loa fibrinous layer connected to; subconjunctival
The conjunctiva is rich in lymphoid tissue
Mycobacterium tuberculosis Wuchereria bancrofti granulation tissue; when this membrane Hs
and blood vessels and contains mucus-producing
Pasteurel la tularensis Thelazia califcmiensis removed, a raw bleeding surface is exposed:' j
cells but few pain fibers.
Moraxella lacunala (Morax-Axenfeld baciblus) J Thelazia callipaeda
When the lids are closed, as in sleeping, the Treponema pallidum 4 Nematodes (roundworms)
temperature of the conjunctival sac is elevated. Actinomyces israelfi j. Trematodes (flukes) (Schistosoma Clinical Findin gs
The incubating effect of this increase in haematobium) i'. ;
temperature promotes the growth of invading I■ !
B. Viral: 4 Cestodes (tapeworms) A. Symptoms and Signs: The or|ganisms
bacteria, and this in turn promotes the production Viruses of— | Arthropods (Phthirus pubis, ocular myiasis) listed account for most cases of b£acUrial
v- of pus cells. This explains the “sticking together Phaiyngoconjunctiva] fever (adenoviruses j conjunctivitis. They produce bilateral rotation
of the lids in the morning” in bacterial 3 and 7) J. Unknown Etiology: and injection, a purulent idischar g' *e ’with <
>■•5 conjunctivitis. Epidemic keratoconjunctivitis Conjunctival folliculosis consequent sticking together I of the lic_ds itipon
Conjunctivitis causes an increase in tear (adenovirus 8) Ocular rosacea awakening, and lid edema. The infection usually
Enteroviruses (epidemic hemorrhagic ■ Benign mucous membrane pemphigoid starts in one eye and is spread to the other eye by
production. The antibacterial function of the tears
conjunctivitis, coxsackievirus) Erythema multiforme (Stevens-Johnson the hands. It may be spread from one jjerdon to
is primarily mechanical, washing conjunctival
Variola syndrome) another through towels, doorknobs, and other
- debris and bacteria into the nasal passages for
Herpes simplex Dermatitis herpetiformis commonly used articles. Pus production in
excretion. The tears also contain lysozyme, which Newcastle disease Epidermolysis bullosa gonococcal conjunctivitis is unusually profuse,
inhibits the growth of certain bacteria. Tear Molluscum contagiosum Erythema nodosum Pneumococcal conjunctivitis, is often associated
production, the abundance of lymphoid elements, Varicella Psoriasis with subconjunctival hemorrhages, Conjunctival
. and the absence of a closed space (when the lids Vaccinia Superior limbic keratoconjunctivitis membranes may form in the lower palpebral area
are open) have their role in the self-limiting nature in streptococcal conjunctivitis.’ ’ ’
6 of conjunctivitis. C- Chlamydial: J. Associated With Systemic Disease: Eg, Pinkeye is pneumococcal conjunctivitis in
When the eyes become inflamed, the patient Chlamydia trachomatis
hyperthyroidism. temperate climates and Haemophilus aegyplius
" ■ usually seeks treatment early. A diagnosis of Chlamydia oculogenitalis U: |
conjunctivitis in warm climates.
Chlamydia lymphogranulomatis i: I
conjunctivitis is suggested by a history of red The Morax-Axenfeld bacillus Causes; —na
Chlamydia psittaci
eye of short duration, without pain or 1. BACTERIAL CONJUNCTIVITIS localized angular conjunctivitis associdted with
photophobia, and by the inflamed appearance of fissuring and dermatitis of the external canthi
D. Rickettsial.
the conjunctiva and the presence of a conjunctival Bacterial conjunctivitis is an extremely (fairly common) and a scanty conjunctival
exudate or sticky lids in the morning. The diagnosis common disorder. It is usually acute but may discharge.
E. Allergic:
can be confirmed and the pathogens identified by become chronic. Pathologically, it is characterized B. Laboratory Findings; In most cases of
Iatrogenic
by conjunctival hyperemia and a subepithelial bacterial conjunctivitis the Organisms cb,n be
microscopic examination ofstained material from Atopic conjunctivitis
the conjunctival surface. Cultures and antibiotic Hay fever ■' >
sensitivity tests are highly recommended before Verna!
initiating therapy. Keratitis, iritis, and acute
glaucoma must be ruled out ^Causes ophtthalmia neonatnrum
36 37
 |j Bacterial Fungal and * »■ (sometimes for life). Family contacts should be AU of the signs of trachoma are much more
.. il j Viral Purulent Nonpurulent Parasitic Allergic Chlamydial .=•. investigated and treated. severe in the upper than the tower conjunctiva.
Discharge ^Minimal Profutc Minimal Minima) Minimal Profuse ?s". The agent lives in the conjunctival and Pannus appears early in the disease, but may be
Tearing . ’Profuse Moderate Moderate Minimal Moderate Moderate
itahlnjti Minimal Minimal None None
corneal epithelium and produces a soluble antigen visible in the initial stages only upon
Marked Minimal :■£
Injection ^Generalized Generalized Localized Localized Generalized Generalized .< which provokes certain cell-mediated immune magnification with the loupe or slitlamp.
Localized [None None Frequent Frequent None None mechanisms which eventuate in necrosis and In the active phase of the disease, superficial
■conjunctival I?
1 cicatrization of the palpebral conjunctiva, keratitis is often present with or without limbal
... »£•
Prpauricular 'Common Uncommon Common Common None Common in indu- Li: particularly over the upper tarsi. During the active follicles- The limbal follicles tend to regress,
nodes' si on conjunctivitis f; stage, there is follicular and papillary hypertrophy leaving small depressed areas (Herbert’s pits)
i
i only of the superficial conjunctiva. The same visible on slitlamp examination. These are
Staincdjrinear Monocytes Bacteria, Bacteria. Usually Eosinophils PMNs, plasma cells, =?
ii I’MNs PMNs inclusion bodies 4
pathologic process involves the cornea to varying pathognomonic of trachoma.
negative
Associated sore Occasionally Seldom None None Nano None degrees, The upper limbus is always involved, Smears of conjunctival epithelial scrapings
throat and and scarring of the entire cornea occurs in severe stained with Giemsa’s stain Show inclusion bodies
fcverl l
untreated cases. in the epithelial cells in a small percentage ofmild
TAJJLE 4-1. Differential diagnosis of common causes of conjunctivitis. chronic cases and in a high percentage of cases
of active trachoma. There is an increased
identified I by a stained smear of conjunctival Course & Prognosis Clinical Findings production of polymorphonuclear and plasma
scrapings. .‘Culture^ are necessary, and antibiotic cells in the early stages. Leber cells (large
sensitivity!{studies are desirable. Mild itching and irritation are the principal macrophages containing necrotic material) are
Conjunctivitis caused by purulent organisms f
is almost always self limited. Untreated, it lasts | symptoms. If the process continues, blurring of diagnostic but not always present They are found
vision and increasing discomfort will occur. Some in material expressed from tlm conjunctival
about 10-14 days; if properly treated, it lasts 1-3 it
Complications & Sequelae days. The exceptions are streptococcal i people harbor the disease for a lifetime without follicles.
h i severe distress as long as secondary bacterial
•if conjunctivitis, which may enter a chronic phase, *
Chronic marginal blepharitis may occur if infection does not occur.
and gonococcal conjunctivitis, which, when j
staphylococcal conjunctivitis is not cured. untreated, can cause comeal perforation and '
The progress of the disease has been divided
Compal ulceration may result from infection into 4 stages by MacCallan: In stage I (incipient
endophthalmitis.
with Afefs.stertn gonprrJweae or the Koch-Weeks 3 trachoma) immature follicles are present,
bacilhis. Tpxic iritis may result from the presence particularly in the upper tarsal conjunctiva, and
there is incipient pannus at 12 o’clock. Stage II
in the-anterior chamber of the soluble toxin 2. CONJUNCTIVITIS DUE TO
produced By TV gonorrhoeae. (established trachoma) is divided into type (a), in
GHLAMYD1AE •
which follicular hypertrophy is predominant, and
type (b), in which papillary hypertrophy is
predominant ("acute trachoma”). Stage III is
Treatment i TRACHOMA |
characterized by early conjunctival scarring,
ji |
Specific therapy of bacterial conjunctivitis visible clinically as fine white lines, in the
Trachoma is the most common human r
depends u^on bactbriologic identification of the subepithelial areas of the conjunctiva, and
disease of any kind; approximately 500,000,000 ]
etiologic ajjent While awaiting laboratory results, increase in size of the corneal pannus In. stage IV FIG 4-2. Advanced trachoma.
people suffer from it. It affects all races, but is \
topical therapy with a sulfonamide or tetracycline there is smooth scarring of the tarsal conjunctiva
rare in American blacks, Eskimos, and Seminole •;
can be stained. Antibiotic sensitivity testing of the and the vascular pannus becomes inactive.
Indians. It is rampant in poor economic areas *
causative organism may be necessary in some where bathing facilities are usually inadequate and $
cases. Topfcal antibiotic treatment is sufficient to is consequently extremely common in China and ;*
eliminate? the infection in most cases. India. It is endemic in the countries bordering the y
Occasionally, systemic administration of Mediterranean, particularly Egypt. Trachoma is J
antibiotiesjs indicated. Saline or water irrigations rare in the USA except among the Southwestern 1
of the conjunctivaljsac may be used as indicated. American Indian population, in whom it is still i
Instruction of the'patient concerning personal prevalent. Trachoma is always bilateral.
hygiene to prevent spread of the disease is Trachoma has a special affinity for the eye ?
important!. and may also affect the genitourinary tract ;
epithelium. Spread is by direct contact. Parents ;
commonly transfer the disease to their children, <
who may have the disease for many yeais ‘ FIG 4-3. Ptosis with an “SJ’-shaped curve of
FIG 4-1. TVachoma, stage II b. lids associated with chronic trachoma.
38
39
 The agent of trachoma .(.Chlamydia Course & Prognosis by tetracycline, 1-1.5 g daily orally for 2-3 weeks. optical instruments, and swimming pools.'!
trachomatis ) can be easily grown in the yolk sac w Recurrences are rare. There are no complications, Treatment is nonspecific. Topical prophylaxis
with sulfonamides or antibiotics is! recommended
of the developing chick embryo. The results of Without treatment, some degree of.V and with treatment the disease can be cured in 1
large-scale trials of vaccine in several countries permanent comeal and conjunctival scarring isrt week. If not treated, inclusion conjunctivitis to prevent secondary' bacterial nfection. The
l| .
have been equivocal. inevitable. The prognosis is excellent whenM persists 3 months to 1 year. There is never any disease is self-limiting, and recover y occurs within
treatment is given early. About 20,000,000 people visual loss. In 15% of patients, otitis media 2-3 weeks.
in the world are blind as a result of trachoma. develops on the affected side.
Differential Diagnosis M
yvr 3. VIRAL CONJUNCTIVITIS ADENOVIRUS INFECTION i
Inclusion conjunctivitis is differentiated by
; ■- the presence of preauricular Lymph nodes and the INCLUSION CONJUNCTIVITIS
: i -
absence of macropannus, Herbert’s pits, and (Inclusion Blennorrhea)
scarring. The inflammatory reaction is greater in Viral conjunctivitis is common. Many Pharyngoconjunctival Fever j |
the lower rather than in the upper palpebral
S’ different viruses can cause conjunctivitis; some
« jl -:
>•--» conjunctiva. Inclusion conjunctivitis is an uncommon c types are severe and debilitating, whereas others Pharyngoconjunctival fever is characterized
Vernal conjunctivitis produces cobblestone infection characterized clinically by bilateral £ are relatively minor and self-limited. by fever of 38.5-40 °C (101-104 DF), sore throat,
’ papillae and eosinophils on the smear. Neither of conjunctival redness and a profuse exudate. Since .< and a follicular conjunctivitis in orie or both; eyes.
these signs is present in trachoma. tit ere is a reservoir ofthe virus in the genitourinary Bilateral or (less commonly) unilateral
tract (male urethra and female cervix), the disease -j conjunctival discharge is minimal and the cbmegs
is most common in newborns. Transmission in jj EPIDEMIC HEMORRHAGIC are clear. A slight transient superficial keratitis
_ ’ Complications & Sequelae adults is also now established as being from the ’? CONJUNCTIVITIS may occur. Preauricular lymphaderiopathy rlrfay be
genitourinary tract to the eye, so in this sense it is j* present Any of the symptoms may occur sjngty.
Secondary bacterial conjunctivitis increases a venereal disease. The causative agent is now h' An outbreak of a new type of viral Pharyngoconjunctival feverlis caused by
the hazard of corneal ulceration. If this occurs, called Chlamydia oculogenitalis. Eye-to-eye conjunctivitis was first observed in Ghana in type 3 or, less commonly, type 7 of tire adenovirus
corneal scarring is more severe. Without comeal transmission is rare. In adults there are numerous % 1969, The time of the epidemic coincided with group. The virus can be recognized on Itissuc
ulceration, blindness does not usually occur in papillae and follicles in the tarsal conjunctiva, '?• the Apollo XI trip to the moon, arid the disease is culture of conjunctival scrapings by its cyLotropic
trachoma. more pronounced in the lower. Since the newborn % often referred to as Apollo XI conjunctivitis. effect on HeLa cells. The diagnosis can also be
Entropion of the upper and lower lids due to conjunctiva does not form follicles, the fi Similar outbreaks were reported in other parts of made serologically by demonstrating arising titer
the scarred tarsi, with consequent trichiasis, appearance of the reddened conjunctiva in the world. of neutralizing antibody to the virus during tlife
causes constant corneal irritation unless the newborns is non specific. There are no bacteria a Preliminary virologic studies indicate that the disease. However, clinical diagnosis is|mote
entropion is corrected. Xerosis may occur as a in the smears, but numerous basophilic inclusion J etiologic agent is a new member of the practical, and laboratory studies arc seldom
result of scarring of the lacrimal gland and ducts. bodies are found in the conjunctival epithelial 3 picoma vims group. necessary. ji
Ptosis, nasolacrimal duct obstruction, and cells. Pathologically, there are many papillae and The disease is characterized by an acute This condition is more common in children
■ « dacryocystitis are common complications. a dense cellular infiltration in the subepithelial J onset of pain, photophobia, redness, and foreign than adults. Transmission is ofteh traceable to
tissues. ( body sensation after a short incubation period (8- contaminated waler in swimming pools. || q
Inclusion conjunctivitis is at times mistaken ? 48 hours). The clinical findings consist ofswelling No specific treatment is available.] The
Treatment for trachoma. Like trachoma, it is characterized | of the eyelids, subconjunctival hemorrhages conjunctivitis is self-limited, usually lasting^bout
by many conjunctival follicles and papillae, and \ (punctate or diffuse), profuse tearing, and 10 days.
Treatment should be started on the basis 011 stained conjunctival smears the inclusion . sometimes conjunctival chemosis. Conjunctival
follicles are observed in 60% of cases, and more >1
i , ofclinical findings without waiting for Laboratory bodies are indistinguishable. However, the comeal P .j
confirmation. Medical treatment consists of oral changes are much different. In most cases ofadult f frequently in the lower tarsal conjunctiva. Epidemic Keratoconjunctivitis |j
Superficial keratitis has been reported. Comeal
.tetracycline given in full doses for 3-5 weeks. Local
treatment is not necessary. Caution: Do not give
inclusion conjunctivitis, only epithelial keratitis r
develops. Subepithelial opacities may occur intraepithelial infiltrates and punctate staining of i
The vtrus of epidemic keratoconjunctivitis
i' ij
. tetracyclines during pregnancy or to young which are similar to those seen in epidemic ;j the epithelium are frequent, but subepithelial has been identified as type 8 of tjie adenovirus
children, and do not use old preparations. keratoconjunctivitis. In long-standing cases I infiltrates are usually absent. The disease is group. The disease is characterized clinically by
Hygienic measures are of great importance (weeks to months) there may be a micropannus bilateral in over 90% of cases. Anterior uveitis unilateral injection, moderate pain, and tearing!
both in prevention and treatment. but never any comeal scarring. In trachoma, the i has been observed. Preauricular lymph node There is no purulent discharge, but conjunctival
Corneal scarring may require corneal upper tarsal conjunctiva is more involved than j enlargement is often present. Fever, malaise, and inflammation is pronounced and a large
transplantation. Entropion and trichiasis require tli e lower. - • J generalized myalgia are observed in 25% of cases. preauricular lymph node is always present. A
plastic surgery to evert the lids. Dacryo­ In babies, J.% tetracycline drops in oil instilled j The disease is transmitted by the use of conjunctival membrane sometimes forms. Corneal
cystorhinostomy may be required tn nasolacrimal 5-6 times daily for 2-3 weeks is very effective. In non-running water for washing the face, finger- infiltrates, which distinguish the disease, from ntlvv
■»« 4V VUOVOi

41) 41
 ■j ; | * j NEWCASTLE DISEASE CONJUNCTIVITIS® VERNAL CONJUNCTIVITIS
They sire round, centrally located and subepitheliaL, with subsequent scarring. A diffuse epithelial
and usually appear 6-10 days after the onset of keratitis occasionally occurs. A central oval
the conjunctival inflammation and may persist for Newcastle disease conjunctivitis is a ratf-| Vernal conjunctivitis is an uncommon comeal ulcer sometimes occurs which responds
months; Corneal sensitivity is not affected. There disorder characterized by pain, injection, tearing. '■ bilateral allergic disease which usually begins in poorly to treatment.
are tip bacteria in the conjunctival smear, but and blurring ofvision. It is usually unilateral and?! the prepubertal years and lasts 5-10 years. It is
mononuclear cells are numerous. The virus can characteristically occurs following the handling^ much more common in. boys than in girls. The
be i dentified by serologic methods from material of live Newcastle virus used in inoculating^ specific al lergen is not known. People with vernal Treatment
taken; 'from the conjunctival surface. The virus chickens, or after handling infected birds. Thefl conjunctivitis often become allergic to other
tends; fo stick to fingers and instruments and will superior and inferior palpebral conjunctiva shows':-, things, particularly grass pollens. This type of Instill corticosteroid solution, 2 drops every'
grow in many eye solutions. The physician should a marked follicular reaction, and there may be?’ conjunctivitis is less common in temperate than 2 hours during the day in the acute phase, and
wash-his'hands between examinations and take associated superficial corneal opacities and lidu in warm climales and is almost nonexistent in cold apply cold water compresses for 20 minutes 4 times
precautions to prevent the spread of the virus by edema. Conjunctival discharge is minimal. Fever^ climates. daily. The patient-must be observed closely for
cofit aLmiijati on of instruments. The tonometer and headache are usually present also. There areX the complications of the treatment even more than
footplate ’should be cleaned with a sterile solution numerous mononuclear cells but no bacteria id’j of the disease since treatment may have to be
after cvbry use; after use on a red eye, tonometers the conjunctival smear. The virus of Newcastle?1- Clinical Findings continued for weeks or months. Herpes simplex
shpiild be sterilized in flame or in a special hot air disease can be isolated, but this is not necessary# keratitis, cataract, and glaucoma are the most likely
tonbmeter sterilizer. (Epidemic keratoconjunctivitis if the diagnosis can be made on clinical grounds.-* Symptoms are always more severe in spring complications, fungal overgrowth a less common
is thppnly known serious eye disease transmitted There is no specific treatment. and summer. The patient complains of extreme complication of local corticosteroid therapy.
during the course of tonometry.) itching. There are many giant papillae in the Topical application of sodium cromoglycate
X- palpebral conjunctiva, particularly the upper (Fig
ifl Epidemic keratoconjunctivitis in adults is eyedrops has been recently shown to give prompt
strictly 'uilocal disease. In children there may be 4-4), which give a typical cobblestone appearance. symptomatic relief in patients with vernal conjunc­
systemic; symptoms of viral infection, eg, fever MOLLUSCUM CONTAGIOSUM K A stringy conjunctival discharge and a milky' tivitis. However, the topical ophthalmologic prepa­
and'sore throat. I conjunctival pseudomembrane are frequently ration is not yet available in the USA.
; i No specific treatment is available. In conjunctivitis due to molluscum?; present. If there is papillary hypertrophy in the Desensitization to grass pollens helps in a
contagiosum there is a recurrent follicular;; region of the corneoscleral junction, the condition small percentage of cases.
unilateral conjunctival inflammation secondary tofl is known as limbal vernal conjunctivitis. Many A cool climate is beneficial, or sleeping in a
one or more molluscum nodules on either the?’ eosinophils and tree eosinophilic granules aTe cool air-conditioned room.
SIMPLEX CONJUNCTIVITIS upper or lower lid, or both. No bacteria are present^ found in a smear of the conjunctival exudate
in the conjunctival smear. Diagnosis is made by';f stained with Giemsa’s stain. Between attacks, mast
-|j Conjunctivitis due to infection with herpes excisional biopsy. Excision or simple incision off cells are found on the stained conjunctival smear Course & Prognosis
siriijilexf virus is an uncommon infection the nodules results in cure. J but no eosinophils.
characterized by unilateral injection, irritation, ■r Recurrences are common, particularly in the
andknild photophobia. It occurs only in primary spring. The episodes are more easily combated
(first attack) herpes simplex, usually in association 4, ALLERGIC CONJUNCTIVITIS Complications & Sequelae since the advent of corticosteroid therapy. The
widn'herpes simplex keratitis. Conjunctival papillae eventually disappear completely, leaving
follicular hypertrophy is observed, the upper and Slight to total pannus is an unusual no scars.
lower conjunctiva is hyperemic, and an enlarged CONJUNCTIVITIS ASSOCIATED WITH | complication. Superficial comeal ulcers may form
prettuncUlar node is present with or without HAY FEVER
vesicular lesions on the lids. Polymorphonuclear
leukocytes are present in the conjunctival smear, A mild nonspecific conjunctival inflammation-: PHLYCTENULAR
along with some monocytes. No bacteria are is commonly associated with hay fever (allergic^ KERATOCONJUNCTIVITIS
foiihd. The vinis can be isolated by scratching a Thinitis). Clinical findings include itching, mildj
rabbit’s cornea with an instrument contaminated photophobia, and mild injection of the palpebral^
with.cdnjunctival exudate or by tissue culture on and bulbar conjunctiva, plus a scanty ropy£ Phlyctenular keratoconjunctivitis is associated
ail! Pgg [membrane. The pathologic signs of discharge. Occasional eosinophils are found in|» with malnutrition, tuberculosis, or both. It is
inflammation are not characteristic. smears of the conjunctival scrapings. There are's extremely common among Eskimo children in
il i ; iTjreatment is with idoxuridine ointment 4 no conjunctival papillae or follicles. Ji Alaska. The essential lesion, the phlyctenule, is a
times:daily and sulfonamide locally as necessary Treatment consists of instillation of localj concentration of lymphocytes attheiimbus which
tdpftjVent secondary bacterial infection. The use vaso-constrictors during the acute phase. Cold?, is considered a delayed hypersensitivity reaction
of steroids is contraindicated since they have the compresses may be applied to relieve symptoms.'j to the tubercle bacillus or, in rare cases, to other
effect of aggravating herpes simplex infections. The response to treatment is good, but] FIG. 4-4. Vernal conjunctivitis. *’ Cobblestone “ organisms causing granulomatous infections (eg,
Uncomplicated cases clear in 7-10 days. recurrences are common. | papillae in superior tarsal conjunctiva. Coccidioides immitis).
42 43
 Photophobia is intense. The phlyctenules Conjunctivitis occurs in exacerbations
Acids denature the proteins in the tissues 7. CONJUNCTIVITIS DUE TO ANIMAL
occur typically in the limbal areas of both eyes, remissions similar to those of the skin.
and so produce their effect immediately; but PARASITES i j ?| {
but may be seen, in the cornea itself. They are red, Treatment of the keratoconjunctivitis in­
proteins are not precipitated by alkalies, which I
elevated, and wedge-shaped, with the apex toward patients with atopic dermatitis is usually
tend to linger in the conjunctival tissues and
the central cornea, and vary widely in number and discouraging. A short-term course of topical
continue to inflict damage over a period of hours NEMATODES
size. steroids may be given during the exacerbations;^
to days, depending on the molar concentration (Roundwoms)
Slight to complete corneal scarring may In cases of secondary bacterial infection, specific#
and the amount introduced.
develop (Fig 4-5). Salzmann’s nodular dystrophy antibiotic therapy should be started. In advanced
Pain, injection, photophobia, and
is a frequent complication. cases with corneal involvement, corneal transplant
blepharospasm are the main symptoms. A history Onchocerca Volvulus
Instill a corticosteroid suspension, 2 drops may improve tlie visual acuity.
of exposure can usually be elicited. Adhesion Onchocerciasis ranks behind trachomaiand
in each eye every 2 hours, during the acute phase. between the bulbar and palpebral conjunctiva vitamin A deficiency as the third leading C£|uvse of
Desensitization procedures are indicated with (symblepharon) and comeal leukoma are more blindness in the world. The disease is a ;ch^phic
tuberculin if the patient is tuberculin-positive. likely to occur if the offending agent is an alkali.
5. CHEMICAL CONJUNCTIVITIS infection with the parasite Onchocerca
Treat pulmonary tuberculosis if present A well- Immediate irrigation of the conjunctival sac Il is endemic in the greater part of tropical Africa
balanced diet is highly important. Secondary with water or saline solution is most important and Central and South America. The infectidri'is
bacterial infection must be controlled. If the Do not use chemical antidotes. General
Chemical conjunctivitis is a fairly common:! transmitted by bites of infested black 11 ies'qf the
corneal scarring becomes marked, corneal symptomatic measures include cold compresses
ocular condition, particularly as an occupational genus Simulium, which breed only in {rapidly
trans-plant is indicated. for 20 minutes every hour; atropine, 1%, 2 drops
hazard among men. Any irritating substance which-; moving rivers-thus the term "river blindness.’1 mfre
Recurrences are common if the basic causes twice daily; and systemic analgesics as indicated.
enters the conjunctival sac may caused adult worms encapsulate in the skip :hHd
are not eliminated. Corneal transplant may be required for corneal
conjunctivitis. Some common irritants are soapj subcutaneous tissue. The adult female discharges
scarring, and a conjunctival plastic operation for large numbers of microfilariae which lnaylrcaLch
fertilizers, deodorants, hair sprays, tobacco, make-,1
symblepharon. the eye and cause a variety of ocular lesions.
up products (eg, mascara), acids and alkalies, andl
If the proper treatment is started immediately, microfilariae may be seen in the anterior chamber.
irritating drugs intentionally or unintentionally^
the -prognosis is good. If treatment is delayed, The eye lesions include conjunctivitis, keratitis,
used by physicians. Silver nitrate instilled io?
permanent conjunctival and corneal scarring may chronic uveitis, cataract, chorioretinitis, apd Optic
infants’ eyes at birth as an anriinfective agent is al result.
common cause of chemical conjunctivitis inf neuritis. The ocular changes are caused by local
newborns. In certain sections of the world, smog- invasion of the ^microfilariae and by die tissue
has become the commonest cause of mild? reaction following their death. i ’ J -J l
6JFU N GAL CONJUNCTIVIUS Treatment with diethylcarbamazine hdlpsiin
chemical conjunctivitis.
a small percentage of cases. | ‘ H PJ

CANDIDIASIS
(Ocular Candidiasis, Thrush of Eye) Loa Loa (Filaria Loa, “Eye Wo rm”) ;
Infestation of the conjunctiva and lids Svith
FIG 4-5. Postphlyctenulosis. Vascularized scar in
this parasite is occasionally seen in the native!; pf
temporal portion of left cornea. Candida albicans is a rare cause of tropical West Africa and in missionaries returning
conjunctivitis. The conjunctivitis is always to the USA. The anterior chamber and vilreoiis
secondary to infection pf other mucous may be invaded, but Loa loa rarely migrated .to
ATOPIC KERATOCONJUNCTIVITIS membranes of the body. Clinically, one sees a thin the retina. Systemic chemotherapy jw-ith
gray membrane involving the upper and lower diethylcarbamazine (Hetrazan) is the treatment of
The cornea and conjunctiva may be involved palpebral conjunctiva. Pathologically, there is
in patients with atopic dermatitis (eczema). The nonspecific inflammatory reaction in the
symptoms are a burning sensation, mucoid subepithelial tissues and necrosis of the
discharge, redness, and photophobia. The clinical conjunctival epithelium. The ocular condition Wiichereria Bancroft!
picture consists of erythema of the lid margins clears with the disappearance of the candidal
FIG 4-6. Severe symblepharon following an alkali i This parasite may enter tlie conjunctiva1 and
■with conjunctival hyperemia. CorneaL involvement infection elsewhere in the body. Corneal ulcer
bum of conjunctiva and cornea. lids, producing extreme edema similar I to the
usually occurs late and follows repeated may occur. elephantiasis seen elsewhere in the body; If thp
exacerbations of the conjunctivitis. Superficial I The antifungal drug of choice is nystatin
peripheral keratitis occurs with vascularization. In The specific irritant in smog has not bees}
(Mycostatin), 2 drops in each eye every 1-2 hours
advanced cases the entire cornea becomes hazy positively identified, and the-treatment is|
night and day depending on the severity of the
and vascularized, resulting in decreased, visual nonspecific. There are no permanent ocular!
ulcer.
acuity. The disease has a protracted course. effects. ’
45
 ^CONJUNCTIVITIS OF UNKNOWN differentiated from ocular rosacea by the presence
The!azia\Californietisis *£. Thelazia Callipaeda retina, choroid, or vitreous to produce ocul-^J
ETIOLOGY ofextreme photophobia, the absence offacial ro­
cysticercosis. Infection is relatively common
i natural habitat of this roundworm is in
' j,:The sacea, and tire favorable response to cortisone. If
herbivorous animals, but accidental infection of Mexico and South America. Mechanical removal
of the parasite from the eye is sometimes possible;.*! marginal corneal ulceration occurs, vascular infil­
the human conjunctival sac and lacrimal apparatus chronic FOLLICULAR CONJUNCTIVITIS
•i. j trate may follow and persist. Secondary staphy­
may occur. Three cases have been reported in the (Orphan’s Conjunctivitis) lococcal conjunctivitis is a frequent complication.
U^A.’j Thelazia californiensis causes a ■5
Treatment of ocular rosacea consists of elimi­
nohpurufent conjunctivitis, but its counterpart,
ARTHROPODS nation of alcohol and foods which cause dilata­
Thdldzia callipaeda (Oriental eye worm), This is a transmissible, mild bilateral disease tion of the facial vessels and treatment of the sec­
penetrates and causes blindness. of childhood characterized by numerous follicles ondary staphylococcal infection, if present. A
.’J
in the upper and lower palpebral conjunctiva. short course of oral tetracycline is sometimes help­
Phthirus Pubis (Pedicuius Pubis, Pubic Louse) 3
There is a scanty conjunctival discharge and mini- ful.
Trichinclla Spiralis These parasites and their eggs form nitsgf
mal infl animation. There are no complications. The disease is chronic, recurrences are com­
J This parasite occasionally localizes in the which attach themselves to the lashes or to theV
eyebrows and produce an irritating conjunctivitis;^ Treatment is ineffective. The average duration of mon and the response to treatment is generally
extrao’cuiar muscles and produces conjunctival the disease is 2-3 years.
anil! lid edema. The worm does not enter the One percent gamma benzene bexachloride 'i poor. If the cornea is not involved, the visual prog­
A variation of chronic follicular conjunctivitis nosis is good The corneal lesions tend to recur
eyeball- Pie associated eosinophilia, fever, and (Kwell Shampoo) is recommended for treatment ■
is druginduced follicular conjunctivitis due to and progress, am vision grows steadily worse
muscular pain are helpful in the diagnosis. of the pubic area. Mechanical removal of the,:
prolonged administration of miotics (especially over a period of years if the comeii is affected.
ii| i I parasites and their nits from the eyelashes isj|
usually necessary, and 0.5% physostigmine^
physostigmine and isoflurophate) in glaucoma
Askaris J^umbricoides (Butcher’s Conjunctivitis) therapy.
1 Ascjiris larvas cause a rare type of violent ointment is applied to the lid margin twice daily?*
toxic conjunctivitis. The worms are spread by Family contacts should be examined and treated.-t
BENIGN MUCOUS MEMBRANE
accidental contamination to butchers and persons
Ocular Myiasis PEMPHIGOID
performing postmortem examinations. It is CONJUNCTIVAL FOLLICULOSIS
postulated that the poison exuded by the larvas Conjunctival infestation with fly larvas?] (Ocular Pemphigus)
lias *a'direct action on the conjunctival vessels. occurs frequently in the tropics but is rare in the *
THq reaction, varies in intensity, buL it typically USA. Several species of flies have been,
Conjunctival folliculosis is a fairiy common This is a rare form of bilateral conjunctivitis
produces extreme chemosis and lid edema. incriminated. Larvas invade the conjunctiva] sac’
benign, bilateral, noninflammatory disorder char­ which may occur in a purely ophthalmic form but
Treatment is by mechanical removal of the and produce a nonspecific inflammatory reaction.!
acterized by follicular hypertrophy of the palpe­ is usually associated with pemphigoid lesions of
organism. If they spread throughout the eye and orbit, thej
bral conjunctiva It is more common in children. the nose, gums, throat, and skin. The chronic form
inflammatory reaction and eventual necrosis;
Symptoms are minimal. The follicles are more nu­ is much mort common than the acute.
become severe. Destruction of the orbital contents;
merous in the lower palpebral conjunctiva. The Pathologically there is cicatrization of the
TREMATODES and bony walls of the orbit with invasion of the:
complete lack of inflammation and the inferior in­ conjunctiva, particularly in the lower fornix, and
(Flukes) meninges may occur.
volvement differentiate it from chronic follicular an irregular pannus. Thepatient complains ofpain,
• i« : Extreme itching and irritation are the cardinal*]
•• ? < conjunctivitis. There are no complications. irritation, and blurring of vision. There is a thick,
symptoms. The conjunctiva is red and excoriated, ?
No treatment is available. The disease runs it
Scliistospma Haematobium Numerous elongated white larvas are seen, I
course in about 2-3 years.
| !This parasitic disease is endemic in Egypt especially in the fomices. )
arid; produces granulomatous conjunctival Treatment consists ofmechanical removal of i
lesions,; The diagnosis is by biopsy and the larvas after first instilling cocaine (10%), which'
microscopic examination. Treatment consists of has a paralyzing effect upon them. If the larvas I
OCULAR ROSACEA
excision- of the conjunctival granulomas. can be removed when they are few in number, the;
course of the disease is automatically terminated.!
If they are allowed to multiply, the prognosis is!
Ocular rosacea is an uncommon complica­
cxtremeLy poor inasmuch as they invade the*
tion o facial rosacea, which occurs in persons with
jjjl ; CESTODES tissues out of reach of any form of treatment In j
light skin. It usually manifests itself as a
(Tapeworm) such cases, destruction of the bony orbital wall!
blepharoconjunctivitis. Small gray nodules may
•Hi i and its contents frequently occurs.
be present on the bulbar conjunctiva. Pathologi­
i;H I
Taenia
cally, these consist of lymphocyte and epithelioid
• :; i Solium cells. The patient complains ofmild injection and
j’T'his parasite occasionally causes irritation. Marginal corneal ulceration is sometimes
conjunctivitis but mote frequently invades the associated. Phlyctenular keratoconjunctivitis is FIG 4-7. Benign mucous membrane pemphigoid.
46 47
 ropy conjunctival exudate and shrinkage of the conjunctivitis with subsequent shrinkage of
SUPERIOR LIMBIC erroneously referred to by patients as cataracts.
fomices, particularly the lower fornix. Vesicles and conjunctiva. Dermatitis herpetiformis does not4
KERATOCONJUNCTIVITIS Pterygium is thought to be an irritative
bullae are rare. The secretory ducts from the ordinarily affect the comea, but epidermolysis
lacrimal and accessory lacrimal glands are bullosa sometimes produces severe corneal? phenomenon, as it is common in farmers and otiibrs
This is an unusual bilateral papillary who spend a large part of their lives out of ^dors
eventually obliterated, and the lack of tears ulceration with subsequent scarring. Dermatitis-’
keratoconjunctivitis involving the upper tarsus. in sunny, dusty, or sandy windblown areas) Tile
aggravates any corneal pathology. herpetiformis may produce conjunctiva^
It is characterized by hyperemia, punctate keratitis, pathologic findings in the conjunctiva are the softie
In the early stages ocular pemphigus is dif­ cicatrization, closing the ducts from the lacrimal-
filaments, a micropannus, and thickening of the as those of pinguecula. In the comea there; is
ferentiated from trachoma by the presence of a gland and resulting in xerosis. 2 epithelium. When scrapings are examined replacement of the epithelium and Bo>vmaft?ls
low-grade conjunctival eosinophilia, which is No specific treatment is available for either:;
microscopically, keratinization of the epithelia! membrane by the hyaline and elastic tissue,^; i <
never seen in trachoma. Trachoma involves the condition. t cells is the only finding. If the pterygium is enlarging, or prqduccs-a
upper comea and palpebral conjunctiva, whereas The disease has been associated with cosmetic blemish or visual disturbance, it should
the reverse is true of pemphigus. hyperthyroidism. be removed surgically, taking care to rdmbve ia
No specific treatment is available.
Treatingthe area occasionally with 1 % silver small portion of superficial clear cornea jbeyond
The course is prolonged, and the prognosis ERYTHEMA NODOSUM nitrate helps relieve symptoms in some patients, the area of corneal encroachment.
is poor. Blindness due to complete corneal scarring but in general the treatment is unsatisfactory.
is the usual end result Bulbar conjunctival nodules are occasionally;)
seen in association with erythema nodosum. The'l
conjunctival nodules disappear simultaneously^
with the cutaneous lesions. 41 DEGENERATIVE DISEASES
ll
OF THE CONJUNCTIVA
ERYTHEMA MULTIFORME BULLOSUM
(Stevens-Johnson Syndrome)
PSORIASIS PINGUECULA
Bilateral conjunctivitis is a common
complication of erythema multiforme. It may This skin disease may be complicated by a; Pinguecula is extremely common in adults.
simulate bacterial conjunctivitis or produce bacterial type of conjunctivitis which may produce.,' It is manifested by a yellow nodule on either side
extensive conjunctival membranes which conjunctival membranes plus numerous! of the cornea (more commonly on the nasal side)
ultimately invade the corneas and canaliculi. subepithelial corneal infiltrates associated willy I 1 i I
in the area of the lid fissure. FIG 4-9.Bilateral pterygiums,advanced. ’
Erythema multiforme, along with epidemic ! | i!'
marginal corneal ulceration. The conjunctival andj
keratoconjunctivitis, is one of the 2 most common corneal lesions vary with the skin lesions and ara
. causes of membranous conjunctivitis. Pain and not affected by specific treatment. There are nil
photophobia are common symptoms. The typical
serious permanent ocular effects. $
. nail, skin, and mucous membrane lesions V:
differentiate it from pemphigus. Secondary THEGONJUN CTIVA
bacterial infection is rare.
li
Both local and systemic corticosteroids are
REITER’S DISEASE LYMPHANGIECTASIS
* - helpful in shortening the course of the disease ■ •;'i
and in preventing permanent scarring of the
A mild, nonspecific, bilateral conjunctivitis!
i jl i
conjunctiva, the corneas, and the canaliculi. Lymphangiectasis is characterized by small,
is present as apart of the syndrome of urethritis^ clear, tortuous, localized dilatations- ir jtjre
The course is prolonged. Recovery may be
- . complete, or blindness may occur as a result of polyarthritis, conjunctivitis, and fever. Reiter’s} conjunctiva. They are merely dilated; lymph
disease occurs only in young men. There is no­ FIG 4-8. Pinguecula. vessels, and no treatment is indicated unless’t ^ey
corneal scarring. Recurrences are rare.
relation to gonorrhea. No specific organisms? are irritating or cosmetically objectionable in
have definitely been established as causes, but The nodules rarely grow, but inflammation Is which case they can be cauterized or excised.! «
mycoplasmas and chlamydiae have been common. Microscopically, they consist of hyaline
and yellow elastic tissue. No treatment is indicated. I i i:
DERMATITIS HERPETIFORMIS & isolated Id a few cases. The conjunctivitis is • | rii
EPIDERMOLYSIS BULLOSA sometimes complicated by iritis (anterior CONGENITAL CONJUNCTIVAL jjS;
uveitis), is not affected by treatment, and clears LYMPHEDEMA j I
in 2-3 weeks, usually before the general PTERYGIUM • t •■
These 2 uncommon skin conditions are This is a rare entity characterized byjpinl^i^i
occasionally complicated by membranous symptoms disappear.
Pterygium, is a fleshy, bilateral, triangular fleshy edema of the bulbar conjunctiva,-usually
encroachment of a Pinguecula onto the cornea. It observed as an isolated entity at birihJ.Tdie
always, occurs nasally. Pterygiums are often condition may be unilateral or bilateral.l Tfie
48
49

i
 T'"

lymphedema is thought to be due to a congenital solution (1%) into the conjunctival sac of each-1?
d^fcpi. to the lymphatic drainage of the eye immediately after birth. Inadvertent-’ 5
conjuhcfiya. It Has been observed in chronic instillation of more concentrated silver nitrate-’;
hereditary lymphedema of the lower extremities
(Mifroy’fc disease). Lymphedema of the
solutions will cause significant permanent-
corneal scarring, and even the 1% solution
CORNEA & SCLERA
conjUnct'iya in Milroy’s disease is considered an frequently causes chemical conjunctivitis; forX
ocular-manifestation of the disease rather than an this reason many ophthalmologists recommend-;
associated anomaly. that penicillin may be employed instead.
< :*• ' However, penicillin prophylaxis may favor the :•>
• »i Yi Hi .
= Ji ; • emergence of penicillin-resistant strains of- j
i SUBCONJUNCTIVAL HEMORRHAGE staphylococci in the nursery. Silver nitrate ■
< ': [8 i i prophylaxis is legally required in many states
| ’Tljigj common disorder .may occur and is the only method of prophylaxis approved ? LCORNEA The blood supply to the cornea is derived
spontaneously, usually in only one eye, in any by the FDA. from the limbal vessels. The comeahas arich nerve “
age group. Becabse of the sudden onset and supply from the fifth cranial nerve. The superficial
brig lit'red appearance, the patient is usually Anatomy & Functions comeal layers contain about 70 sensory nerve
alarmed. .The hemorrhage is caused by the rupture OCULOGLANDULAR DISEASES fibers, which accounts for the severe pain that
o fa sm’alj conjunctival vessel, sometimes preceded (Parinaud’s Syndrome) The cornea is a transparent and avascular results from, seemingly minor irritative
by a; bout of severe coughing or sneezing. tissue comparable in size and structure to the phenomena. Pj
; iTtiere is no treatment, and the hemorrhage crystal in a small wristwatch. At the scleral The transparency of the cornea is due to
This is a group of diseases characterized by
usually ti^sorbs in about 2-3 weeks. The best unilateral conjunctival granuloma and a • junction (limbus) there is a circumferential its uniform structure, avascularity, and
treatment is reassurance. preauricular lymphadenopathy. The disease is depression, the scleral sulcus. deturgescence. Deturgescence, or the state of
ini tare instances the hemorrhages are usually associated with fever. The causes include The cornea functions as a refracting and relative dehydration of the corneal tissue, is
bilateral; ;0r recurrent, in which case blood leptothricosis, tularemia, coccidioidomycosis, ■ protective “window” membrane through which maintained by the active Na+-K* cell “pump” of
dy.scragia^ should be ruled out. tuberculosis, syphilis, sarcoidosis, and infected light rays pass en route to the retina. It has a the endothelium and epithelium and by. their
chalazion. Only the most- common cause will be refractive power equivalent to a +43 diopter lens. anatomic integrity. The cell pumps have a
I d • ' I The average adult cornea is 1 mm thick and definite role in the dehydration of the cornea
1 ■i• j 1 discussed.
: J OPHTHALMIA NEONATORUM 11.5 mm in diameter. The 5 distinct layers of the and in preserving its transparency. The
j J ' J cornea are, from anterior to posterior: epithelium endothel ium is more important in the mechanism
^Ophthalmia neonatorum is any inflammation Leptothricosis (Parinaud’s Conj unctivitis) (continuous with the bulbar conjunctival of dehydration than the epithelium. Chemical or
or infection of the conjunctiva in the newborn. This is a fairly common, low-grade, nodular, | epithelium), Bowman’s membrane, stroma, physical damage to the endothelium of the cornea
Various types have already been discussed. The unilateral conjunctivitis due to infection with j Descemet’s membrane, and endothelium. The is far more serious than epithelial damage.
most-cbiiimon forms are chemical (silver nitrate) Lepiolrichia buccalis, which may be found in the j epithelium has 5-6 cell layers, the endothelium only Destruction of the endothelial cells may cause
conjunctivitis and‘staphylococcal, pneumococcal, mouths of humans and all cats. Approximately | one. The epithelium is a reliable barrier against marked swelling of the cornea and result in loss of
gonococcal, and inclusion conjunctivitis. 90% of cases with the oculoglandular syndrome i comeal infection arid usually must be traumatized its transparency? On the other hand, damage to .
' iTlic time of onset is an important factor in are due to leptothricosis. It is common in children before an infectious agent can gain a foothold in die epithelium causes slight and transient localized
diagnosis. Conjunctivitis appearing during the first and in those who come in close contact with cats. the corneal stroma. Bowman’s membrane is a clear, swelling of the corneal stroma which clears
24 b’cmrSj is probably due to silver nitrate. Symptoms and signs consist of slight structureless (ie, homogeneous, acellular) layer following rapid regeneration of the epithelial cells.
Cpnjitflctivitis which has its onset during the injection and irritation, a moderately large nodule which is a modified portion of the superficial Evaporation of water from the precorneal tear film
second do fifth days is probably due to on the upper or lower tarsal conjunctiva, an ; stroma. Descemet’s membrane is a clear elastic produces hypertoxicity of the tear film which may .
stiipbyld cocci, pneumococci, or gonococci. ipsilateral large preauricular lymph node, and low- ! membrane which can easily be detached from the be an important factor in drawing water from
Inclusfori .conjunctivitis appears 5-10 days after grade fever. The conjunctival ganuloma may I stroma; although on gross examination it appears the corneal stroma and helps in maintaining the
birth.i .I ulcerate. to be structureless, microscopic examination state of dehydration.
’ .'Diagnosis is confirmed by microscopic Pathologic examination is necessary to > shows Descemet’s membrane to contain many The penetration of drugs through the
examijiatibn of stained material from a smear of differentiate this condition from the previously ' fibers. The corneal stroma consists of lainellas and cornea seems to depend upon phase solubility.
the conjunctival scrapings and by conjunctival mentioned oculoglandular diseases. accounts for about 90% of the comeal thickness. The epithelium will allow the passage of fat­
crjltbriei -5t i Treatment consists of excision of the » Each lamellar fiber is transparent, 1 pm thick and soluble substances, and the stroma allows the
conjunctival granuloma (nodule). Close ! 15 pm wide, and as long as the diameter of the passage of water-soluble substances.
Prcvcrtttbh j contactwith cats must be avoided. In untreated : cornea. The stromal lamellas are parallel to the Therefore, in order for a drug to penetrate the
| bacterial conjunctivitis in newborn infants patients, the disease subsides within 3 months, j corneal surface but do intertwine. Each contains cornea, it must be both fat-soluble and water-
mayfbe^prevented by instilling silver nitrate and the prognosis is good. , a flattened cell nucleus. soluble.

50 51
 which frequently predisposes to the development--?, B. Viruses: among coal miners. It is parti cj.il arly hkclyjto]
ofherpes simplex keratitis. j Herpes simplex virus (Herpesvirus hominis) develop if the patient has a chronic pncumjococc'al j
Laboratory investigation of ulcerated comeal <1 Variola virus dacryocystitis. Purulent material from the infefctedi
lesions is of great importance since if specific-Jt Vaccinia virus tear sac pours into the conjunctival sac; if’ ihe;
therapy is not instituted immediately the cornea Herpes zoster virus corneal epithelium is not intact, the organism;
may be destroyed in 24 hours. Stained smears -4 enters the corneal substance and begins! Ito1
should be examined while the patient waits. | C. Fungi: multiply. It is curious that conjunctivitisjstldom!
Note: Diseases of the cornea are extremely : Candida (Monilia) albicans occurs in association with pneumqcobcal;
serious since improper management can result in £ Fusarium solani dacryocystitis. j H Hi •
permanent visual impairment ranging from slight K Nocardia Since the pneumococcus is sensitive to both i
blurring to total blindness. Most of the disabling Aspergillus sulfonamides and antibiotics, local therapyis.
complications of corneal diseases can be £ Penicillium usually effective. If untreated, the cornpafrnay.
prevented by prompt and accurate diagnosis and Ceplialosporium perforate and the eyemay be lost.Coqcurf£pt|
appropriate treatment. dacryocystitis, if present,should also be tteiiied. ’
D. Hypersensitivity Reactions:
To staphylococci (marginal ulcer)
To unknown allergens or toxins (ring ulcer) Streptococcal Ulcer
'
;
W
!
CORNEAL ULCERS ? To tuberculoprotein and, rarely, other
FIG 5-1. Transverse section ofcorneea. i bacterial proteins (phlyctenular This rare ulcer produced by Streptococcus
-,i keratoconj uncti vitis) haemolyticus has no creeping tendency bl|tiiis:
Scarring or perforation due to corneal 4 otherwise identical with the pneumococcal ulcpr:
Physiology of Symptoms ulceration is a maj or cause of blindness throughout •? E. Avitaminosis A (Xerophthalmia). in appearance, course, and response to trehtriieiit. fi
Since the cornea has many pain fibers, most the world. Most forms are amenable to therapy, i L'i
superficial corneal lesions such as a corneal but visual impairment can be avoided only if : E FifthNerve Lesions (Neurotrophic Ulcers).
. foreign body, corneal abrasion, or phlyctenule will
cause pain and photophobia. The pain is
. augmented considerably by the movement of the
appropriate treatment is instituted promptly; in
some cases this means within a matter of hours
after onset. Corneal ulceration has long been
’ G. Exposure.
Pseudomonas Ulcer

The corneal ulceration caused - by-

Hl!
ids over the cornea, particularly the upper lid. recognized as a distinct clinical entity, but the : H. Unknown Cause: (Mooren’s ulcer, or Pseudomonas aeruginosa characteristically starts =i
„ Jince the cornea serves as the window of the eye specific diagnosis and management of its various "chronic serpiginous ulcer.”) in a small area, usually in the center, and spreads ■
and refracts light rays, comeal lesions cause some types present problems which bear analysis. rapidly, frequently causing perforation’ pL0.ic:,
degree of blurred vision; blurring is greater if the cornea and loss of the eye within 48 hours.-It Often
esion is centrally located. Since there are no blood \
follows some minor comeal injury which has Jpqen |
/essels or mucus glands in the cornea, there is no Classification j L. BACTERIAL CORNEAL ULCERS examined with the aid of a pseudomonas-i
discharge except in the case of a purulent bacterial Corneal ulceration will be discussed here | contaminated fluorescein solution. The orgaqifcfn.1
ulcer. according to the type of ulcer produced by the ■ usually produces a bluish-green pigment Wpich •
The physician examines for corneal disease following agents: | Pneumococcal Ulcer (Acute Serpiginous Ulcer) is pathognomonic of Ps aeruginosa infedtfoftJ! ’j
. >y inspection of the cornea under adequate Three features of pseudomonas cbriieaD
illumination. Examination is often facilitated by A. Bacteria: (In order offrequency.) The pneumococcus (Streptococcus ulceration deserve emphasis'(IjPs aerii&iqpsa j
- first instilling a local anesthetic to relieve the pain Streptococcus pneumoniae (pneumococcus) pneumoniae) is the commonest bacterial cause of thrives in fluorescein solution and to a lessene^tent t
ind occasionally by instilling fluorescein to Pseudomonas aeruginosa (Bacillus ■. comeal ulcer. Typically it produces a gray, fairly in physostigmine solution. These solutions- c^n'
outline a comeal lesion which might not otherwise pyocyaneus) well circumscribed ulcer, with a marked tendency only be safely used if they arc-auto clayed al |
be easily visualized. The loupe and slitlamp are Moraxella liquefaciens (diplobacillus of to spread centrally if it begins near the limbus. frequent intervals (once a week),, since there ire*
useful but not essential aids in the examination of Petit) This creeping effect gave rise to the name "acute no reliable antibacterial preservatives available for :
he cornea; adequate illumination can be furnished Viridans streptococcus (alpha-hemolytic serpiginous ulcer”, a synonym for pneumococcal them. For this reason the use of sterile fluorescein J
. >y a hand flashlight. streptococcus) ulcer. Hypopyon, is a frequent development; papers moistened with the patient’s tears (cjnst&riJe >
It is important to elicit a history of trauma, Staphylococcus aureus nearly always sterile, it is a result of the passage individual dropper units) is strongly advocated I
■ rince foreign body and erosion are 2 of the most Streptococcus pyogenes (haemolyticus) ofa bacterial toxin through Descemet’s membrane as a substitute for fluorescein solution. (2) Spice .
common corneal disorders. The patient should be (beta-hemolytic streptococcus) into the anterior chamber. Fungal ulcers are an the organism flourishes better on the cornea iihin ’
• asked about previous corneal diseases to rule out Klebsiella pneumoniae (Friedlander’s exception. The hypopyon often contains fungi on any known culture medium, it can cause rapid
a recurrent corneal erosion. Local medications bacillus) which have penetrated the cornea. destruction of the cornea. Early; diagnosis?‘.’arid ■
;hould be investigated to rule out steroid therapy, Mycobacterium forluitum Pneumococcal ulceration usually follows vigorous treatment are therefore absplufqly:
corneal trauma and is consequently very common essential. (3) Polymyxin B is the most consistently j
52 53 ! ; k'! ’ !
active arilibiotic against Ps aeruginosa. 2. VIRAL CORNEAL U LCERS prolonged unduly. If perforation is imminent, of 3-4 more days. Resistant strains of herpes
Ps aeruginosa eye infections may occur immediate corneal transplantation or a thin simplex virus have appeared.
in premature infants; If these infections arc not conjunctival flap is indicated. Other modes of therapy include
properly treated, systemic dissemination and death Herpes Simplex Keratitis (Dendritic Keratitis) • The therapeutic objective is to denude the trifluorothymidine, cytarabine, and interferon
may ensjie. virus-containing corneal epithelium without inducers (eg, Poly I:C). Also under investigation
Polymyxin B sulfate is supplied in soluble 25 Corneal ulceration caused by herpes simplex disturbing Bowman’s membrane and the corneal is photodynamic inactivation with vital dyes
mg tablets for solution (1 mg = 10,000 units) and virus (Herpesvirus hominis) is fairly common, stroma. Infected epithelium is much more loosely (neutral red and proflavin ).
in vials containing ;50 ml (500,000 units) for almost always unilateral, and may affect any age ■' attached to Bowman’s membrane than healthy
injection1. Instill 2 drops of a solution containing group. The patient usually complains of mild - epithelium, arid is thus easily removed. The safest
10,000' uni ts/ml in saline every 15 minutes around irritation and photophobia, and, if the central J way to remove it is with a small, tightly wound
the clock; polymyxin-bacitracin (Polysporin) cornea is affected, of blurred vision. A history of ? sterile cotton applicator or a sterile spatula or
ointment isiinslilled every 2 hours after irrigation recent upper respiratory infection with “cold ’• chalazion curet. Chemical cauterization with
with saline solution. (The bacitracin is used for its sores” about the face can often be elicited. tincture of iodine or ether has no advantage over
effect agaidst gram-positive invaders.) A 1000 ml The commonest finding is of one or more 4. simple mechanical removal of the epithelium and
bottle of stqrile saline solution can be installed at dendritic ulcers on the comeal surface (Fig 5-3). introduces the risk of chemical keratitis.
the bedside on an intravenous standard, with an These are composed of clear vesicles in the / Homatropine, 5%, is instilled to relieve the
eye drdpper or dull needle attached to the end of corneal epithelium; when the vesicles rupture, the photophobia. A pressure bandage is applied to
the tubing.; A newer form of treatment involves area stains green with fluorescein. Although the ? hold the lid against the cornea. If sterile
the usb’of a: tube and cup device that fits over the dendritic figure is its most characteristic ; instruments have been used, there should be no
cornc^.’arid all owls a continuous flow of manifestation, herpes simplex keratitis may appear 4 need to instill an antibacterial agent However, the FIG 5-3. Dendritic figures seen in herpes simplex
antimicrobial solution over the ulcerated cornea. in a number of other forms. ;i patient should be observed on the following day keratitis.
Ip addition, inject gentamicin, 30 mg in 1 ml, Herpes simplex virus can be cultivated by --I to be certain that a secondary' bacterial infection
subcopjpnctivally daily. The pain associated with transferring direct scrapings from the corneal ulcer has not developed. The pressure bandage is
the subconjunctivaljinjections may be sufficient to the chorioallantoic membrane of a developing -J changed daily until the epithelium regenerates. Corticosteroid therapy is contraindicated at
to require strong analgesics or prior injection of a chick embryo. The dendritic figure is reproducible y Cryotherapy has been advocated for all stages of the disease. Corticosteroids increase
local anesthetic. on the rabbit cornea by scratch inoculation of its ? treatment of herpes simplex keratitis and is similar the activity of the virus, most likely by immuno­
Qplis-tin (Coly-Mycin) is identical to surface with scrapings from the corneal ulcer. The in action to denuding the diseased epithelium. suppression and by enhancing the action of col­
polymyxin; B in antipseudomonas effectiveness, virus also produces a typical cytopathic effect on ■ Idoxuridine (IDU; Dendrid, Herplex, Stoxil) is lagenase (produced by damaged corneal epithe­
and the tissue toxicity of the 2 drugs appears to HeLa cell cultures. V quite effective in the treatment of acute cases. It lial cells) on the corneal stroma. Before the corti­
be the; same. has no effect on chronic herpes simplex keratitis, costeroids came into common, use, herpes sim­
Hypopyon is an occasional complication of?: does not prevent recurrences, and does not plex virus infection never caused comeal perfora­
herpes simplex keratitis. It is usually sterile, a toxic /j protect against the harmful effects of tion.
Diplobacinus of Petit Ulcer reaction to secondary infection in about two-thirds 4 corticosteroids. It is instilled locally as 0.1 % Secondary fungal infection may be another
of cases but to herpes simplex virus itself in one- § solution or ointment every hour day and night for complication oflocal steroid therapy or ofunduly
typrdxeUa liquefaciens causes an indolent third. There is no specific treatment for the? 3 days and then gradually withdrawn over a period prolonged broad-spectrum antibiotic treatment of
ulcer,:‘Usually centrally located and very slowly hypopyon, but it may resolve spontaneously. * comeal ulcers of any type.
progressive. It is ordinarily superficial and unlikely When herpetic ulcer is diagnosed early and
to pericirate the cornea. Many of its. victims are Disciform keratitis is a fairly frequent? , treated.promptly, the prognosis is good. Recur­
alcoholics. 1 complication, particularly if iodine has been used ? rences are common, however, and there are sev­
’ibpical treatment with sodium sulfacetamide, to cauterize the initial lesion or if corticosteroids ' ■
eral “trigger mechanisms” which tend to induce
sulfisoxazole, or broad-spectrum antibiotics is have been used early. There is no specifics them. The most common of these are fever,
clTecjivc.j treatment, but the prognosis is usually good ifi| colds, exposure to sun, psychic stress, trauma,
there is no vessel encroachment from the limbus: and menstruation After the initial attack (pri­
iX i Vascularization inevitably produces a permanent??
mary' herpes), the patient develops circulating
Friedlander’s Bacillus jJlcer scar and permanent visual loss. Occasionally a--f
antibodies in the blood which remain at a con­
disciform lesion becomes necrotic centrally, and
stant level throughout life. The antibodies do
•irfielrare ulbcr caused by -Klebsiella this in turn leads to hypopyon formation,"J
not prevent recurrences but do modify the cu­
pneumoniae develops after trauma, is centrally perforation, or both. 0
taneous and conjunctival manifestations of the
located, progresses slowly, and rarely causes disease (unfortunately not the corneal manifes­
hypopyon. Treatment is with tetracyclines and Corneal perforation rarely occurs unless
tation), so that recurrences affecLing these tis­
corticosteroids have been used or antibiotics :] MG 5-2. Corneal scar caused by recurrent herpes
streptomycin. sues are much less severe than the primary at­
simplex keratitis. tack. If the primary attack is ocular, the con­
54 55
junctiva is affected as well as the cornea (pri­ The vast majority of fungaL ulcers arei? later. Although it is generally assumed that ring presence of a Bitot spot. This is a foamy,jw$dge-
mary herpetic keratoconjunctivitis); but in any preventable since they occur after corticosteroid ? ulcer is a hypersensitivity reaction, the underlying shaped area in the conjunctiva, usually oniithe
recurrence that affects the eye, whether it follows treatment or following herpes simplex keratitis or .(J cause can seldom be determined. temporal side, with the base of the wedge at the
an initial ocular or an initial cutaneous attack, the comeal trauma. Response to local corticosteroid therapy is limbus and the apex extending toward the lateral
conjunctiva is usually spared. There arc no reli­ Topical Pimaricin is presently thought to be canthus. Within the triangle the conjunctiva is
able methods of preventing recurrences. superior to amphotericin B in treating fungal ? furrowed concentrically with th'e limbus,'and dry
keratitis, especially when caused by Fusarium and ? flaky materia) can be seen falling from the area
Variola Virus Ulcer Cephalosporium species. ? Phlyctenular Keratoconjunctivitis into the inferior cul-de-sac. A stained conjunctiva)
Comeal ulcers due to variola virus still occur scraping from a Bitot spot will show diany
in areas of the world where smallpox is endemic. This is a disease of malnourished children saprophytic xerosis bacilli (Coryn ebapteriinn
Conjunctivitis is frequently present. with systemic tuberculosis and, less frequently, xerosis; small curved rods) and keratinized
4JCO RNEAL ULCERS DUE TO of adults who have had tuberculosis in childhood. epithelial cells. ! ;
Vaccinia Virus Ulcer HYi ERSENSITIV1TY REACTIONS For this reason the disease is common among Avitaminosis A comeal ulceration results
Corneal ulcers due to vaccinia virus are Eskimos and North American Indians. The eye from dietary lack of vitamin A or impaired
occasionally seen as an accidental complication lesions, usually bilateral, are in the great majority absorption from the gastrointestinal tijach-^nd
of smallpox vaccination. Prevention of secondary of cases the result of a sensitivity to impaired utilization by the body.jlt may develop in
bacterial infection is the only treatment. In Marginal Ulcers tuberculoprotein, which reaches the cornea via an infant who has a feeding problem; in an adult
accidental vaccinia of the lids, vaccinia immune the blood stream. In rare instances the agent of. who is on a restricted or generally inadequate diet;
gamma globulin given intramuscularly has been Marginal ulcers arc by far the commonest V the systemic disease may be the staphylococcus, or in any person with a biliary obstruction since
used prophylactically in an effort to prevent spread corneal ulcers. They are small, unilateral, single or ; Coccidioides immitis, the agent of bile in the gastrointestinal tract is necessary: for
to the cornea. multiple, superficial, round or rectangular ulcers ; lymphogranuloma venereum,- or some other the absorption of vitamin A. Lack of vitairinf A
which have a clear space between them and the pathogen to which the cornea has become causes a generalized keratinizaLion1 of the
limbus. About 50% are associated with hypersensitive. Bacterial conjunctivitis often epithelium throughout thebody.-The conjunctival
staphylococcal marginal blepharitis; scrapings serves as a trigger mechanism for an attack. and. corneal changes together are known as
X FUNGAL CORNEAL ULCERS and cultures taken directly from the ulcers contain • The phlyctenule is the characteristic lesion. xerophthalmia. Since the epithelium of ttjejiir
no bacteria, and so they are assumed to be the Jt is gray and wedge-shaped, and extends across passages is affected, many patients, if not; treated,
result of allergic reactions to staphylococcal the limbus onto the cornea. It may be single or will die of pneumonia. Avitaminosis A also causes
The incidence ofcomeal ulceration due to fungi infection. Many of the remaining 50% are due to multiple. Phlyctenules characteristically involve a generalized retardati on of osseous growth, phis
has been increasing, perhaps as a result of fungal food allergies. only the peripheral cornea, but may spread to cover is extremely important in infants; for example^ if
overgrowth from long-term antibiotic and Marginal and ring ulcers may also occur in the entire comeal surface. Microscopically one the skull bones do not groxv and the brain
■ corticosteroid therapy. The organisms find their way association with conjunctivitis caused by the sees many small round cells and dilated blood continues to grow, increased intracranial pressure
into the cornea following damage to the epithelium Koch-Weeks bacillus or Proteus vulgaris. vessels. and papilledema can result. ; I • r? y
by trauma or inflammation, eg, herpes simplex Topical corticosteroid drops are usually Response to local corticosteroids is dramatic. Between 10,000 and 15,000 IU ofvitamin A
• i
ulceration. Most fungal ulcers are gray, indolent, and curative within 2-3 days. Marginal ulcers tend to j Systemic steroid therapy is contraindicated since should be administered daily IM. (or orally if-the
■ slowly progressive. recur, Staphylococcal blepharitis, if present, | it could cause the dissemination of an existent absorption rate is known to be nortoa’i).
The so-called “satellite phenomenon,” a ring should be vigorously treated. pulmonary tuberculosis. Equally important in the Sulfonamide or antibiotic ointment can be Used
- of smaller opacities around a denser centraL ulcer, I
treatment is a well-balanced diet and management locally in the eye to prevent secondary bacterial
is an important diagnostic feature of fungal Ring Ulcers of the systemic tuberculosis or other underlying infection.The average daily requirement ofvitamin
’ comeal ulcer (Fig 5-4). i disease. A is 1500-5000 IU for children ^according to.age,
Ring ulcers are much less common but more j and 5000 IU for adults. *
destructive than marginal ulcers. The ring ulcer is ;
a unilateral, almost continuous circumferential i 5. CORNEAL ULCER
ulcer just inside the limbus. Smears and cultures | DUE TO VITAMIN A DEFICIENCY 6. NEUROTROPHIC CORNEAL ULCERS
from it are negative. In contrast to the marginal • 1 !
; •
ulcer, there is no relationship between ring ulcer
and conjunctivitis or blepharitis. I The typical corneal ulcer associated with If the trigeminal nerve, which supplies: (he
There may be an associated systemic i avitaminosis A is centrally located and bilateral, cornea, is interrupted by trauma, surgery, lunior,
disease, however. Two such related conditions j gray and indolent, with a definite lack of corneal inflammation, or in any other way, the cornea-loscs
are bacillary dysentery and polyarteritis nodosa. • luster in the surrounding area The cornea becomes its sensitivity and one of its best defenses ugiphst
In one case of bacillary dysentery; a ring ulcer ; soft and necrotic (hence the term, “keratomalacia"), degeneration, ulceration, and ^infection. Id 'the
occurred, with the initial attack and recurred and perforation is common. The epithelium of the early stages of a typical neurotrophic ujeer,
FIG 5-4. Corneal ulcer caused by Candida albicans. coincidentally with the second attack 5 months conjunctiva is keratinized, as evidenced by the fluorescein solution wiil produce punctate staiiiing
56
 Ifactffrro co/vug

of; ths'superficial epithelium. As this process pain is a characteristic feature. Typically it has □^GENERATIVE CORNEAL CONDITIONS disorders which usually appear in the second or
progresses, patchy areas of denudation appear. raised border at the advancing edge. No microbial third decades of life. Some aie hereditary. Other
Occasionally the epithelium may be absent from a agent has ever been isolated. The central corneal cases follow ocular inflammatory disease, and
large area of the cornea. is the last area to be destroyed. Mooren’s ulcer- 1. KERATOCONUS some are of unknown cause.
i The progress of the condition depends on must be differentiated from excavated marginal -
the treatment. Without treatment, the denuded ulceration associated with polyarteritis nodosa Fatty or Lipoid Degeneration
areas become infected. The integrity of the cornea Wegener’s granulomatosis, and ulcerative colitis. Keratoconus is an uncommon degenerative This disorder may begin in infancy or
-an Be maintained as long as the corneal surface Two types of Mooren’s ulcer are recognized: ’} bilateral disease which is inherited as an adulthood The cause is not known. There is a
is kept moist by wearing a Buller shield, * by (1) Mooren’s ulcer of unknown etiology is usually -5 autosomal recessive trait. Unilateral cases of generalized deposition of lipid material within the
iuturirigi the lids together, or by the use of a severe, bilateral, and occurs in the older age group Xi unknown cause occur rarely. Symptoms appear corneal stroma, replacement of Bowman’s
-ohjuhctival flap. Artificial tears may be ofbenefit. (above 50 years). (2) Mooren’s ulcer following ’ in the second decade of life. The disease affects membrane by macrophages, and thickening of the
Unddr. the best conditions, however, the trauma is moderate, unilateral, and occurs in the h all races. Keratoconus has been associated with a epithelium with some infiltration of lipid material.
□rogiiosis is poor. younger age group (under 50 years of age). number of diseases, including Down’s syndrome, Clinical findings include blurred vision and
■ i;.' atopic dermatitis, retinitis pigmentosa, aniridia, haziness and thickening ofthe cornea, particularly
■ ij i ii: i vernal catarrh, Marfan’s syndrome, Apert’s in the central zone.
• H 7. EXPOSURE KERATITIS syndrome, and Ehlers-Danlas syndrome. Symptoms and signs are slowly progressive
!•:; I Pathologically, there are generalized thinning and until useful vision is lost. Corneal transplant
I h; . i. . anterior protrusion ofthe central cornea, ruptures improves vision significantly in most cases.
i Exposure keratitis may develop in any inDcscemet’s membrane, and irregular, superficial
5itUaJa'pri|in which the cornea is not properly linear scars at the apex ofthe cone which is formed. Marginal Dystrophy
.noistened and covered by the eyelids. Examples Blurred vision is the only symptom. Signs This disorder begins in the later years. The
include exophthalmos from, any cause, ectropion, include cone-shaped cornea, indentation of the cause is not known. The corneal stroma is replaced
he absence of part of an eyelid as a result of lower Lid by the cornea when the patient looks by loose connective tissue; the cornea gradually
:raumu; find inability to close lire lids properly, as down, an irregular shadow on retinoscopy, and a becomes thinner in the limbal area, most often
in Bell’s palsy. The 2 factors at work are the drying distorted corneal reflection with Placido’s disk or superiorly, and Descemet’s membrane bulges for­
afjthls cornea ,and its ‘exposure to minor trauma, the keratoscope. The fundi cannot be clearly ward. There are usually no symptoms, but there
riic uncovered cornea is particularly subject to visualized. may be recurrent irritation and injection. The only
drying; during sleeping hours. If an ulcer develops FIG 5-5. Mooren’s ulcer. Corneal perforation may occur in advanced complication is traumatic rupture of the eyeball at
it usiiallyfollows minor trauma and occurs in the cases. When this happens, the eye should be the limbus, and this occurs only rarely.
inferibr third of the cornea. bandaged and the dressing changed daily until a Because foe course of progression is slow
; This type ofkeratitis will be sterile unless it is Histologic examination of specimens from corneal scar seals the wound. Corneal transplant and the central cornea is spared, the prognosis is
secondarily infected, and the therapeutic objective early cases shows loss of corneal epithelium with may be necessary. good.
is to proyide protection and moisture for die entire involvement of the anterior stromal layer. Contact lenses improve visual acuity in the
corneal surface. The [method depends upon the Bowman’s membrane is usually destroyed, and early stages. A comeal transplant is indicated Band Keratopathy
underlying condition^ a plastic procedure on the the corneal stroma is infiltrated with when the corrected visual acuity decreases to the This is a chronic disorder sometimes associ­
eyelids, ;a Buller shield, or surgical relief of polymorphonuclear cells and eosinophils. The point where it interferes with the patient’s normal ated with chronic uveitis due to Boeck’s sarcoid,
exophthalmos. base of the ulcer shows infiltration with activities. hypervitaminosis D, or Still’s disease in children.
lymphocytes and plasma cells. Keratoconus is slowly progressive until the Other associnted conditions include diabetes
i. ■ ■ ‘ ' Treatment of Mooren’s ulcer is usually ; patient becomes blind (between the ages of 20 meilitus, Down’s syndrome, congenital rubella,
: 8. MOOREN'S ULCER discouraging. Several modes of therapy have been ! and 60). If a corneal transplant is done before siderosis, and endothelial dystrophy. There is
described. Suggested treatment methods for this s extreme cornea thinning occurs, the prognosis is deposition of calcium sails in the superficial cor­
destructive process include the use of topical excellent; about 80% obtain.reading vision. nea and hyalinization of Bowman’s membrane.
; Mooren’s ulcer is a rare disorder of adults collagenase inhibitors (cysteine, EDTA), excision Symptoms include irritation, injection, and blur­
which is unilateral in 50-75% of cases. It starts as of foe conjunctiva adjacent to foe corneal lesion, ring ofvirion. A horizontally placed, whitish rect­
a rnarjjiha'l furrow in the temporal or nasal quadrant circumcorneal cryotherapy, and delimiting angular plaque 3-6 mm in diameter is seen extend­
near the limsbus and slowly progresses to destroy keratotomy of the corneal button. Penetrating 2. CORNEAL DYSTROPHY ing most of the way across the cornea. Ulceration
the entire! cornea and.eventually the eye. Severe keratoplasty is usually unsuccessful. OR DEGENERATION and infection may occur.
Band keratopathy is the only type of corneal
I ilk-ii . . ■
*l|hc>EJulipr shield is a water-tight cone ofexposed X-ray
dystrophy in which medical treatment is useful.
The corneal dystrophies are a rare group of Treatment consists of scraping the cornea with a
filpi secured to the surrounding skin with adhesive tape.
slowly progressive, bilateral, degenerative spatula and applying EDTA solution with a cotton

58 59
' ’ , applicator to dissolve the calcareous material. If 3. HEREDITARY CORNEAL DYSTROPHIES Z located in the pupillary area. Fingerprint or maplike uniform deposition of hyaline matcrinh Conical
this is ineffective, comeal transplant will improve fine opacities may be seen. The disease is more transplant is not needed except! in very seVerb’; j
. , .visual acuity significantly in most cases. common in females. Patients may develop and late cases. The inheritance is autosomal i ,
This is a group of rare hereditary disorders \- recunent erosion. Visual acuity is affected very dominant. ;
.Climatic Droplet Keratopathy of the cornea of unknown cause characterized by slightly or not at all. •• ’ i 1
(Pearl Diver’s Keratopathy, Bictti’s bilateral abnormal deposition of substances and . B. Macular Dystrophy: This type of stroma!T
. Keratopathy, Labrador Keratopathy, Spheroid associated with alteration in the normal comeal C. Fingerprint Dystrophy: This entity refers comeal dystrophy is manifested by a dense gray ;
Degeneration of the Cornea) architecture which may or may not interfere with ! to fine wavy concentric lines located anterior to central opacity which starts :in Bowman's.-;
This is a relatively new clinical entity-an ac­ vision. These corneal dystrophies usually Bowman’s membrane which can be seen best by membrane. The opacity tends to spread tow’ardj |
quired keratopathy affecting mainly male adults manifest themselves during the first or second > retroillumination with the slitlamp. These lines the periphery and later involves the deepe^j j
who spend most of their working hours out of decade but sometimes later. They may be ;■ have a map or fingerprint pattern. The condition stromal layers. Recurrent corneal erosion rtihy • |
foors. The corneal degeneration is thought to be stationary or slowly progressive throughout life. •: is asymptomatic, and the findings are discovered occur, and vision is severely impaired. Histologic': • i
.caused by exposure to ultraviolet light and is char­ Corneal transplant improves vision in most ? during routine examination. examination shows deposition of ac'id-|>- ■
acterized in the early stages by fine subepithelial patients with hereditary corneal dystrophy. J mucopolysaccharide in the1 stroma
. droplets in the peripheral cornea. As the disease Anatomically, comeal dystrophies may be degeneration of Bowman’s membrane. T
ldvances, the droplets become central with sub­ classified into 3 major groups: epithelial, stromal, •<. The inheritance is autosomal recessive. . 1
sequent comeal clouding which causes blurred and endothelial dystrophies. 1 .1’ i i
vision. Treatment is by comeal transplant C. Lattice Dystrophy: Lattice dystrophy ’ <
starts as fine, branching linear opacities- ' •
‘Jalzmann’s Nodular Dystrophy Epithelia] Corneal Dystrophies Bowman’s membrane in the central areu and-h ’
This disorder is always preceded by corneal spreads to the periphery. The dc’ep stroma may ' :
inflammation, particularly phlyctenular kerato- A. Meesman’s Dystrophy: This slowly $ become involved, but the process does not reach J t !
; conjunctivitis. There is vascularization and de- progressive disorder is characterized by * Descemet's membrane. Recurrent erosion niay;; .
» ■ * . jeneration of the superficial cornea which involves microcystic areas in the epithelium. The onset is $ occur. Histologic examination rbveals amyloidji I
\ he stroma, Bowman’s membrane, and epithelium. in early childhood (first 1-2 years of life). The main g deposits in the collagen fibers. -;l «£• i
? . Symptoms include redness, irritation, and blurring symptom is slight irritation, and vision is slightly |
a*’ ’
• ?fvision. There is a superficial vascularization, with affected. The inheritance is autosomal dominant. J
? !|! *‘‘|i 1
•vhitish nodules sometimes occurring in chains in- §• Endothelial Corneal Dystrophies, |
.. .erspersed among the blood vessels. B. Cogan’s Dystrophy: This condition is ?: i ■» ?»l/ !
. Cornea 1 transpl ant wil I s ignifi cantly improve characterized by discrete comma-shaped or.; A. Fuchs’s Dystrophy: This-disorder begins'j; j
i 'he visual acuity in most cases. rounded, gray-white intraepithelial opacities FIG 5-7. Band keratopathy. in the third or fourth decade 'and is slowly;- ;
progressive throughout life. Women arc mdre; j
commonly affected than men. There are central p «
D. Recurrent Corneal Erosion wartlike deposits on Descemet’s membrahepb: ■
thickening of Descemet’s membrane, and defects: J I
E. Others: Reis-Buckler dystrophy, vortex in the endothelium. Edema of thejcomeal stroma,;! I
dystrophy. and epithelium occurs, causing blurring ofvision; ; i
The cornea becomes progressively more opaqile>4 • i
Corneal dystrophy, glaucoma, or iris atrophy may ' j
Stroma] Corneal Dystrophies be associated with this disorder. Histologid:,’ I
examination of the cornea reveals wartlike:?i i
There are 3 types of stromal corneal excrescences over Descemet’s membrane whiieHd; i
dystrophies: are secreted by the endothelial cells. Thinning and ' j
pigmentation of the endothelium and thickenihg ' ■ >
A. Granular Dystrophy: This usually of Descemet’s membrane are observed. J •: <
asymptomatic, slowly progressive corneal
dystrophy most often begins in early childhood. B. Posterior Polymorphous Dystrophy:/;
The lesions consist of central, fine, whitish This is a common disorder with; onset in cariy: ',
“granular” lesions in the stroma of the cornea. childhood. Polymorphous plaqttes of calcium
The epithelium and Bowman’s membrane may be crystals are observed in the deep ’stromal layers. ■'
FIG 5-6. Left: Side view of normal cornea. Right: Keratoconus.
affected late in the disease. Visual acuity is Vesicular lesions may be seen in tlie endothelium^ s
slightly reduced. Histologically, the comen shows Edema occurs in the deep stroma - The conditiemi
60 61
is asymptomatic in most cases, but in severe cases MISCELLANEOUS CORNEAL DISORDERS? be easily seen with the slitlamp or loupe. Blood Staining
epithelial and stromal edema may occur. The Subepithelial opacities underlying the epithelial This disorder occurs occasionally as a
inheritance; is autosomal dominant. lesions (ghosts) are often observed in patients complication of traumatic hyphema and is due to
1.SCLEROKERATITIS who have been misdiagnosed as having herpes hemosiderin in the comeal stroma. The cornea is
(Sclerosing Keratitis) simplex keratitis and treated with topical IDU. golden brown, and vision is blurred. In most cases
No causative organism has been identified, the cornea gradually clears in 1-2 years.
Sclerokeratitis is an uncommon, unilateral ' but a virus is suspected. A varicella-zoster virus
localized inflammation of the sclera and coniea.L has been isolated from the corneal scrapings of Kayser-Fleischer Ring
The cause is not known, but tuberculosis hash one case. This is a pigmented ring of variable hue
been implicated. Pathologically, there are manyjr Mild irritation, slight blurring of vision, and (usually green or brown) 3-4 mm in diameter just
chronic inflammatory cells (small round cells) in.;- photophobia are the only symptoms. The inside the Limbus. It is a striking sign of
the involved portion of both structures. Fibrosis-- conjunctiva is not involved. hepatolenticular degeneration (Wilson’s disease),
occurs in the later stages. The patient complains-.: Epithelial keratitis secondary to and is therefore rare. The fine, dense pigment
of pain, photophobia, and irritation, but there ish staphylococcal blepharoconjunctivitis is granules collect between the endothelium and
no discharge. A moderately severe iritis (anterior -1 differentiated from superficial punctate keratitis Descemct’s membrane. Vision is not affected.
nongranulomatous uveitis) is usually associated? S by its involvement of the lower third of the ElectronmicroscQpic studies suggest that the
cornea. EpitheliaL keratitis in trachoma is ruled pigment is a copper compound. It has been shown
i i
out by its location in the upper third of the clinically that the intensity of the pigmentation
; FIG 5-8. Arcus senilis. cornea and the presence of pannus. Many other can be reduced markedly by the use of chelating
forms of keratitis involving the superficial agents such as penicillamine.
cornea are unilateral or are eliminated by their
histories. Stahli’s Line
J ' ; 4. ARCUS SENILIS Short-term instillation of corticosteroid Stahli’s line (Hudson’s brown line, Hudson-
; .< (Corrieal Annulus, Anterior Embryotoxon) drops will often cause disappearance of the Stahli line) is an uncommon phenomenon which
opacities and subjective improvement, but occurs only in elderly persons. It is seen with the
" I f Arcus senilis is an extremely common, recurrences are common. The ultimate slitlamp as a horizontal brown line in the inferior
bilateral benign peripheral corneal degeneration prognosis is good since there is no scarring or third of the cornea. It does not extend to the limbus
whithjfnayioccur at any age but is far more common vascularization of the cornea. Untreated, the on eitherside. The line probably represents tears
in elderly people as part of the aging process. disease runs a protracted course of 1-2 years. in Bowman's membrane. It causes no visual
Wh^riiarciis senilis is present prior to age 50, FIG 5-9. Sclerokeratitis. Note fibiovascular scai’ji Long-term treatment with topical corticosteroids disturbance.
hypcrpholesterolemia is usually; associated with in upper nasal quadrant of cornea. ■ may prolong the course of the disease for many
■Hi i years.
i ! Pathologically, Lipid droplets involve the No specific treatment is available. Tire pupil;
entirc’conibal thickness but are more concentrated should be kept dilated with atropine, 2%, 2 drops-.
in the superficial and deep layers, being relatively once daily. Warm compresses and local 3. CORNEAL PIGMENTATION IL SCLERA
sparse in the corneal stroma. corticosteroid drops are used to relieve the,
: There are no symptoms. Clinically, arcus discomfort. Although the process starts as a small ■
senilis appears as a peripheral, annular, hazy gray area of infiltration, it may progress to total corneal Pigmentation of the comcamay occur with The sclera is the fibrous outer protective
ring abauf 2 mm in width and with a clear space opacification. If it does subside, it usually takes r or without ocular or systemic disease. There are coating of the eye. Jt is dense and white and
betwefen itfand the limbus (Fig 5-8). No treatment months to years. several distinct varieties: continuous with the cornea anteriorly and with
is necessary, and there are no complications. Since the dural sheath of the optic nerve posteriorly. At
afeus-senilis cause no visual defect, it is not Krukenberg’s Spindle the insertion of the rectus muscles it is about 0.3
always classified with the comeal dystrophies. 2. THYGESON’S SUPERFICIAL In this common type, brown uveal pigment mm thick; elsewhere it is about 1 mm thick. A few
PUNCTATE KERATITIS ts deposited bilaterally upon the central strands of scleral tissue pass over the optic disk.
endothelial surface in a vertical spindle-shaped This sievelike structure is known as the lamina
Superficial punctate keratitis is an uncommon fashion. It occurs in a small percentage of people cribrosa. Around the optic nerve the sclera is
chronic and recurrent bilateral disorder of young ■ over age 20, usually in , myopic women. It can penetrated by the long and short posterior ciliary
people. Jt is characterized by discrete and elevated be seen grossly, but is best observed with the loupe arter-ies and the long and short ciliary nerves.
round epithelial opacities which show punctate or slitlamp. The visual acuity is only slightly Slightly posterior to the equator the 4 vortex veins
staining with fluorescein, mainly in the pupillary affected, and the progression is extremely slow. exit through the sclera, usually one in each
area. The opacities are not visible grossly but can i Pigmentary glaucoma should be ruled out. quadrant. About 4 mm posterior to the limbus the

62 63
 4 anterior ciliary arteries and veins penetrate the equatorial, at the anatomic equator; and posterior, HYALINE DEGENERATION
sclera. Each set penetrates slightly anterior to the between the equator and the optic nerve.
insertion of a rectus muscle. In most cases no treatment is available. Small Hyaline degeneration is a fairly frcqucnL " >
The outer surface ofthe sclera is covered by staphylomas are best left alone. In severe cases, finding in the scleras of persons over 601 It is l
a thin layer of fine elastic tissue, the episclera, i
the staphyloma gradually enlarges and manifested by small round, translucent, grayhrea^
containing numerous blood vessels which nourish enucleation is eventually necessary. Scleral They are usually about 2-3 ram in diameter, located '
the sclera. The brown pigment layer on the inner transplants have been tried with limited success. slightly anterior to the insertion of the fectuX j
surface is the lamina fusca, which is continuous muscles. They cause no symptoms Gif" ;
with the sclera and the choroid. On the inner complications. ■ ■;. i
surface at 180 degrees (in a horizontal plane SCLER1T1S • )
through 9 and 3 o’clock) there is a shallow groove
from the optic nerve to the ciliary body in which Scleritis is a rare chronic, nonspecific SCLEROMALACIA PERFQRANS;
are embedded the long posterior ciliary artery and inflammation of the sclera of unknown cause but i
the long ciliary nerve. The nerve supply to die sometimes associated with systemic collagen This is a rare scleral disease characterized by:
sclera is from the ciliary nerves. FIG 5-10. Nodular episcleritis, right eye. • disease. It may be diffuse, or localized andnodular. the appearance of one or more dehisccncesHn the .
Histologically the sclera consists of many If nodular, there is an elevated dark blue area in sclera in the absence of obvious inflammatory
dense bands of parallel and interlacing fibrous Less frequently, the episcleritis assumes aS the anterior portion of the sclera. In both types signs. The cause is not known, but severe
tissue bundles, each ofwhich is 10-16 mm thick nodular form (Fig 5-10), with exactly the same signs-?, there is considerable pain and photophobia, and rheumatoid arthritis is usually associate^. Thd
and 100-140 mm wide. The histologic structure and symptoms except for the presence of a small; y a severe anterior uveitis is often associated. If the uvea is visible, and may be bulging.
of the sclera is remarkably similar to that of the inflammatory nodule in the episcleral tissues which'A adjacent cornea becomes inflamed it is known as If the sclera becomes necrotic, a conjunctival
cornea, which raises the question why the is firmly attached to the sclera. Treatment with* a sclerokeratitis. flap must be performed in order to cover the
cornea is transparent and the sclera opaque. corticosteroids is less effective than in the usuals Scleritis is resistant to all types of treatment. diseased area with healthy conjunctiva. ! 111 [•
The apparent physiologic reason is the relative type of episcleritis, and the disease usually runs a-■ Scleromalaciaperforans persists indefihiteiy-
. deturgescence of the cornea and the less course of several weeks or months. and the prognosis is poor depending upon the ;
uniform structure of the sclera as compared with extent of the process. • •; j j
, the cornea. The cornea has the ability to absorb
There is no medical treatment and .scleral I
a great deal of water, whereupon it becomes STAPHYLOMA transplant is seldom successful. 1
, opaque. The sclera is almosL completely hydrated
in its normal state. Scleral staphylomas are localized, thin, dark •! !
blue, bulging areas lined on their inner surfaces •’
with uveal tissue. They may be congenital or may
occur following trauma or chronic inflammation j i
EPISCLERITIS which has weakened the scleral wall, and may be ,
single or multiple. )
Episcleritis is a common, localized, unilateral
disease which usually occurs temporally. The
i
cause is not known, although ah allergic back­
ground is often present A small percentage of I,
■
cases are associated with Tlieumatoid arthritis, i
L
keratoconjunctivitis sicca, or coccidioidomycosis. |

The patient complains of slight pain, photopho­

bia, tenderness, and redness. The inflammation
always spreads by contiguity to the sclera inter­
nally and Tenon’s capsule and the conjunctiva
i
externally. Conjunctivitis can.be ruled out by the
absence of a discharge and the localized nature of
epi-scleritis. A mild iritis (anterior FIG 5-1L Ciliary staphyloma.
nongranulomatous uveitis) is frequently associ­
ated. There are no complications, and the inflam­ Staphylomas are named according to their
mation can be resolved in 3 or 4 days by instilling anatomic location. The following types are
corticosteroid drops 4 times daily. Recurrences recognized: intercalary, between the ciliary body j;
are uncommon. and tne cornea; ciliarv, over the ciliary bodv;
64 I
65

I
 of the lens, which allows the lens to become more of the retina. The endothelium of these capillaries
6 convex or less convex for near (accommodation)
or far vision, respectively. The ciliary processes
is fenestrated and hence leaks intravenously
injected fluorescein. Most of the large vessels
UVEAL TRACT themselves are composed mainly ofcapillaries and
veins which drain through vortex veins. The
consist of veins. These coalesce and leave the
eye as the 4 vortex veins, one in each of the 4
capillaries are large and fenestrated and hence leak posterior quadrants. The vessel layers of the
intravenously injected fluorescein. choroid also contain some elastic fibers and
The pars plana consists of a thin layer of chromatophores.
ciliary muscle and vessels covered by ciliary The choroid is firmly attached to the margin
epithelium. The zonular fibers, which hold the lens of the optic nerve posteriorly and extends to the
in place, originate in the valleys between the ciliary ora serrata anteriorly, where it joins the ciliary
processes (see Fig 6-1). The blood vessels to the body.
|; Theiuveal tract is composed of 3 parls: the Because of the presence of a thick” ciliary body come from the major circle of the iris
iris, the ciliary body, and the choroid. Il is the collagenous adventitia, normal iris vessels have (Fig 1-8). The sensory nerve supply is through
middle vascular layer of the eye, protected the histologic appearance of being quite sclerotic, p the ciliary nerves. Functions of Uveal Structures
externally by the cornea and sclera. It contributes The iris capillaries have an unfenestrated'-i The function of the iris is to control the
to‘-th^blood supply of the retina. endothelium and hence do not normally leak 2 amount of light which enters the eye. This occurs
• ■ j. i intravenously injected fluorescein. The 2 posterior Anatomy of the Choroid by Teflex constriction of the pupil under the
layers of epithelium are heavily pigmented and The choroid (the posterior portion of the stimulus of light and dilatation of the pupil in
Anatomy of the Iris represent the anterior extension of the pigmented :■ uveal tract and the middle coat of the eye) lies darkness. The ciliary body forms the root of the
I, (The jris is the anterior extension of the ciliary epithelium of the retina plus the retina proper. Tbc between the retina and the sclera. It is largely iris and serves, through the zonular fibers, to
bo’dyklt presents a relatively llat surface with a round b lood supply is from the major circle of the iris. * composed of blood vessels. The choroidal vessels govern the size of the lens in accommodation.
aperture ip the middle called the pupil. It forms Che When the iris is cut, as in doing a small ; are bounded by Bruch’s membrane internally and Aqueous humor is secreted,by the ciliary
posterior wall ofthe anterior chamber and die anterior peripheral iridectomy for acute angle-closure ; the suprachoroid externally. The avascular processes into the posterior chamber. The choroid
wall of the posterior chamber. The pupil varies in glaucoma, it seldom bleeds, and the wound ■- suprachoroid is composed of lamellae of consists of abundant blood vessels; its function
size and has the same form and function as the remains permanently with no tendency to heal. • collagenous and elastic tissue. Bruch’s membrane is to nourish the outer portion of the underlying
aperture ofa camera lens. The iris is in contact with Pain fibers are present, as shown by the pain ! can be divided into 3 portions: an outer elastic retina.
the ifens and the aqueous posteriorly and the caused by traction on the iris during surgery. sheath, a middle collagenous sheath, and an inner
aqueous anteriorly. It has 2 zones on its anterior
.i1 cuticular sheath. (The latter is actually the
sprfape, the ciliary and pupillary zones. Ute sphincter basement membrane of the retinal pigment Physiology of Sympto ms
and dilator muscles, which seive to constrict and Anatomy of the Ciliary' Body epithelium.) Symptoms of uveal tract disorders depend
dihitethe pupil, are in the iris stroma. The ciliary body is a roughly triangular - ; The lumens of the blood vessels decrease upon the site ofthe disease process. For example,
structure which extends forward from the anterior 1 the deeper they are located in the choroid. There since there are pain fibers in the iris, the patient
termination of foe choroid to lhe root of the iris, a are 3 layers of blood vessels: large, medium, and with, iritis will complain of moderate pain and
distance of about 6 mm. Grossly it consists of 2 small. The innermost layer of small bloodvessels photophobia. Inflammation of foe iris as such does
zones: the corona ciliaris, the corrugated anterior ; is known as the choriocapillaris and consists of not cause blurring of vision unless the process is
2 mm; and the pars plana, the smoother and flatter ; large capillaries which nourish the outer portion severe or advanced enough to cause clouding of
posterior 4 mm. The surface ofthe corona ciliaris
consists of many elevations and depressions.
There are 2 layers of ciliary epithelium, the
external pigmented and foe internal nonpigmented,
both of which continue as pigmented layers over
the posterior surface of the iris. The pigment
epithelium represents the forward extension of the
pigment epithelium of the retina. Thenonpigmenl
epithelium, with the exception of the pigment
epithelium ofthe internal limiting membrane ofthe
retina, is continuous with the entire retina.
The ciliary' muscle consists of longitudinal,
radial, and circular portions. Its function is to
; FIG 6->l. Posterior view of cilia ry body, zonule, contract and relax the zonular fibers. This results
Iehs, and ora serrata. in decreased or increased tension on Lhe capsule Fig. 6-2. Cross section of choroid.
66 67
a
 Cornea
the aqueous, cornea, or lens. Choroidal disease granulomatous. Because pathogenic organisms? posterior uveitis is generally classified as
itself does not cause pain or blurred vision. have not been found in the nongranulomatous?; granulomatous.
Because of tlie close contact of the choroid with type and because it responds to corticosteroid?; The patient should be asked about previous
the retina, choroidal disease almost always affects therapy, it is thought to be a sensitivity^ episodes of arthritis and possible exposure to
the retina (eg, chorioretinitis). If the macular area phenomenon. Granulomatous uveitis usually toxoplasmoiJis, histoplasmosis, tuberculosis, and
of the retina is involved, central vision will be follows active microbial invasion of the tissues'? syphilis. The remote possibility of a focus of
impaired. " • by the causative organism (eg, Mycobacteriunti infection elsewhere in the body should also be
The vitreous may also become cloudy as a tuberculosis or Toxoplasma gondii). However? , investigated.
result of posterior uveitis. The impairment of these pathogens are rarely recovered, and a? In granulomatous uveitis (may cause anterior
vision is in proportion to the density of vitreous definite etiologic diagnosis is seldom possible® uveitis, posterior uveitis, or both), the onset is
opacity, and is reversible as the inflammation The etiologic possibilities can often be narrowed' • usually insidious. Vision gradually becomes
subsides and the vitreous haze clears. to a few probable causes by clinicaL and laboratory^! blurred and the affected eye becomes diffusely red
The physician examines for disease of the examination. ?? with circumcomeal flush. Pain is minimal, and
anterior uveal tract with the flashlight and loupe Nongranulomatous uveitis occurs mainly inq photophobia is less marked than in the
or slitlamp, and disease of the posterior uveal tract the anterior portion of the tract, ie, the iris and r nongranulomatous form. The pupil is often
with the ophthalmoscope. ciliary body. There is an inflammatory reaction, as'L . constricted and becomes irregular as posterior
evidenced by the cellular infiltration of: synechias form. Large gray “mutton fat” KPs on the
lymphocytes and plasma cells in significant- posterior surface of the cornea maybe seen with the
numbers and an occasional mononuclear cell. In? slitlamp. Flare and cells are seen in. the anterior
UVEITIS severe cases a large fibrin clot or a hypopyon;? chamber, andnodules consisting of clusters ofwhite
may form in the anterior chamber. . cells are seen on the iris (Koeppe nodules). These
Granulomatous uveitis may involve any nodules are the equivalent of the mutton fat KPs.
„Uveitis” is a general term forinflammatory dis­ portion of the uveal tract but has a predilection.' Fresh active lesions of the choroid and retina
orders of tlie uveal tract “Anterior uveitis” is the pre­ for the posterior area. Nodular collections ofii| appear as yellowish-white patches seen hazily
ferred general term for uveitis and iridocyclitis. “Poste­ epithelioid cells and giant cells surrounded by. *) with the ophthalmoscope through the cloudy
rior uveitis” is the preferred teim for choroiditis and lymphocytes are present in the affected areas, vitreous. Such posterior cases are generally
cliorioretinitis.The term <,liritis,”used above, means acute Inflammatory deposits on the posterior surface oft} classified as granulomatous disease. Because of
anterior nongranulomatous uveitis. the cornea are composed mainly of macrophages;’! the intimate relationship of the choroid and retina,
The clinical differentiation of the 2 types is and epithelioid cells. It is possible to make a?! the retina is nearly always involved also
not always clear. In some clinics'this classification specific etiologic diagnosis histologically in an 3 (chorioretinitis). As healing progresses, the
has been discarded in favor of an anatomic enucleated eye by identifying the pseudocyst of i vitreous haze lessens and pigmentation occurs
differentiation into 3 types: anterior, posterior, and toxoplasmosis, the acid-fast bacillus of>t gradually at the edges of the yellowish-white
panuveitis. tuberculosis, the spirochete of syphilis, the rrj spots. In the healed stage, there is usually FIG 6-4. Anterior synechias ( adhesions).
Inflammation of the uveal tract has many distinctive granulomatous appearance of ft considerable pigment deposition. If the macula The pheripheral iris adheres to the cornea. .
causes and may involve one or all 3 portions sarcoidosis and sympathetic ophthalmia, and a 3 has not been involved, recovery of central vision
simultaneously. The most frequent form ofuveitis few other rare specific causes. ? is complete. The patient is usually not aware of
is acute anterior uveitis (iritis), usually unilateral the scotoma in the peripheral field corresponding
and characterized by a history of pain, lo the scarred area.
photophobia, and blurring of vision; a red eye Clinical Findings
(circumcomeal flush) without purulent discharge; B. Laboratory Findings: Extensive
and a small pupil. It is important to make the A. Symptoms and Signs: In the 4 laboratory investigation is usually not indicated
diagnosis early and to dilate the pupil to prevent nongranulomatous form, the onset is in anterior uveitis, particularly if it is
die formation of permanent posterior synechias. characteristically acute, with pain, injection, nongranulomatous or is readily responsive to
Inflammatory disorders of the uveal tract, photophobia, and blurred vision. There is a j nonspecific treatment. In persistent
usually unilateral, are common principally in the circumcomeal flush caused by dilated limbalblood * nonresponsive anterior or posterior uveitis, an
young and middle age groups. In most cases the vessels. Fine white deposits (keraticprecipitates, t attempt should be made to arrive at an etiologic
cause is not known. In posterior uveitis the retina “KPs”) on the posterior surface of the cornea can ■: diagnosis. Skin tests for tuberculosis,
is almost always secondarily affected. This is be seen with the slitlamp or with a loupe. The ■ histoplasmosis, and toxoplasmosis may be h elpful,
known as chorioretinitis. pupil is small, and there may be a collection of ■: as well as complement fixation tests and methylene
Two major types of uveitis may be fibrin with cells in the anterior chamber. Ifposterior T.; blue dye tests (toxoplasmosis). On the basis of
distinguished upon clinical as well as pathologic synechias are present, the pupil will be irregular j these tests and the clinical appearance, it is FIG 6-5. Posterior synechias. .j
grounds: nongranulomatous (more common) and in shape and the light reflex will be absent, j possible to make an etiologic diagnosis. The iris adheres to the lens. -i‘»
68 69
 constant pupillary dilatation lessens the likelihood^ with all forms of primary glaucoma). Carbonic The uveitis may precede the arthritis by 3-10 years.
of both kinds of synechias. Interference with leijJB anhydrase inhibitors often effectively reduce The knee is the most common joint involved. The
metabolism may cause cataract. Retina/} intraocular tension by depressing aqueous cardinal signs of the disease are calcific band
detachment occasionally occurs as a result off: production. Epinephrine also lowers intraocular keratopathy, cells and flare in the anterior chamber,
traction on the retina by vitreous strands. Cystoitf /. pressure by reducing ciliary body secretion of small to medium-sized white keratic precipitates
macular edema and degeneration can result ■froihri aqueous. with or without flecks of fibrin on the endothelium,
long-standing anterior uveitis. Cataract frequently develops in chronic posterior synechias, often progressing to
uveitis. The eye tolerates removal of such seclusion of the pupil, complicated cataract,
cataracts very poorly; but ifvision is poor enough, variable secondary glaucoma, and macular edema.
Ndngran uk»m*l ow Granuloniat am • >>
cataract surgery may be essential. Retinal Corticosteroids and mydriatics are of value,
Onset Acute Jnrfdioiu
Pai ft Marked None or minimal
detachment is also very difficult to treat especially in acute exacerbations, but their long-
Photophobia Marked Slight successfully surgically when it occurs in range effect seems merely to delay the inevitable,
Blurred vision Marked Marked
Ctrcumcomeml Audi Marked Sight
association with uveitis. ie, severe visual impairment or phthisis bulbi. The
FIG; 6r<5. Posterior synechias ( anterior view ). Ko ratio precipitate* Fine white Large gray (“mutton- . prognosis is very poor because of the relentless
Theiiris is adherent to the lens in several places fat") and progressive character of the disease, which
as a jeis'ult of previous inflammation, causing an Pupfl Small and Irregular Small and irregular “
(variable) ‘W Course and Prognosis leads to serious complications. These patients
irregular fixed pupil. Posterior synechia* Sometimes Sometime* ~ tend to do poorly after surgery.
Anterior chamber Flare predominater Cells predominate 2-. With treatment, an attack of nongranulomatous Iridocyclitis occurring in association with
irj* nodule* Absent Sometimes -’,r
Vitreous haze Absent Sometimes -sX uveitis usually lasts a few days to weeks. adult peripheral rheumatoid arthritis is strictly
Differential Diagnosis Site Anterior tract Posterior tract (vari­ Recurrences are common. coincidental. The adult group is more likely to
able) -
Cou rec Acuta Chronic /; Granulomatous uveitis lasts months to years, develop scleritis and sclero-uveitis. It is
J’h; conjunctivitis, vision is not blurred, PlOgrUMU Good Fair io poor . sometimes with remissions and exacerbations, and unfortunate that the associated cells and flare in
pupillary responses are normal, a discharge is Recurrence Common SanuUmea
may cause permanent damage with marked visual the aqueous humor that accompany the scleritis
present, and there is no pain, photophobia, or loss despite the best available treatment. The have been misinterpreted in the past as
ciliary injection. TABLE 6-1. Differentiation orgranulomatous and; prognosis for a focal peripheral chorioretinal “kido-cyclitis.”
= In keratitis or keratoconjunctivitis, vision may nongranulomatous uveitis, xaC-Pi lesion is considerably better, often healing well About 10-60% of patients with Marie-
be blurred and pain and photophobia may be J with no significant visual loss. Strumpell ankylosing spondylitis develop an
pregebii Some causes of keratitis such as herpes anterior uveitis. There is a marked
simplex and herpes zoster may be associated with Treatment ’ preponderance in males. The uveitis presents
a trucfanierior uveitis. as a mild to fairly severe nongranulomatous
• ljhacute glaucoma the pupil is dilated, there A. Nongranulomatous Uveitis: Symptomatic \ type of iridocyclitis with moderate to severe
are no-posterior synechias, and the cornea is measures include warm compresses for 10 minutes ; P CIFIC TYPES OF UVEITIS ciliary injection, pain, blurred vision, and
steamy.; 3-4 times a day, systemic1 anaTge&tcs as necessary (Uveitis Syndromes) photophobia. It is usually recurrent and
"After repeated attacks, nongranulomatous for pain, and dark glasses for photophobia. The : eventually may lead to permanent damage if not
uveiti^ may acquire the characteristics of pupil must be kept dilated with atropine, 2%, 2 adequately treated.
granulomatous uveitis. In recent years there has drops at least twice daily. Local steroid drops are Ocular examination shows ciliary injection,
beep less emphasis on this differentiation usually quite effective for their antiinflammatory UVEITIS ASSOCIATED WITH JOINT moderate cells and flare in the anterior chamber,
} arid some authorities are disregarding it action, in severe and unresponsive cases, systemic DISEASE and fine white k cratic precipitates located
completely. ’Nevertheless, to the clinician the steroids are given also. mostly on the inferior cornea. Posterior
differentiation is still of value as a guide to synechias, peripheral anterior synechias,
treatment and prognosis. B. Granulomatous Uveitis: If Lhe process =’ About 20% of children with rheumatoid cataracts, and glaucoma are common
includes the anterior segment, pupillary' dilatation arthritis (Still’s disease) develop a chronic complications after hyperacute attacks of
with atropine, 2%, is indicated. Since it is often ■- bilateral nongranulomatous iridocyclitis. Females inflammation. Macular edema occurs in 1 % of
Complications & Sequelae possible to make a tentative or likely etiologic are far more commonly affected than males (12:1). cases with severe anterior iridocyclitis, which,
diagnosis, an attempt with specific therapy is The average age at which the uveitis is detected if persistent, leads to cystoid degeneration and
.. Anterior uveitis may produce peripheral indicated. is 51 /2 years. In most cases the onset is insidious, loss of central vision.
anteridr synechias, which impede aqueous the disease being discovered only when the child Confirmation of the diagnosis is by X-rays of
outflow at the anterior chamber angle and cause C. Treatment of Complications: Glaucoma is is noted to have heterochromia of the iris, a the lumbosacral spine. In about 50% of patients
glaucoma. Posterior synechias can cause a common complication. Treatment ofthe uveitis difference in the size or shape of the pupil, or the with ankylosing spondylitis, the clinical signs and
glaucoma by impeding the flow of aqueous from is of primary importance, particularly dilating the onset of strabismus. There is no correlation symptoms may all be absent so that the diagnosis
the 'posterior to the anterior chamber. Early and pupil with atropine (not constricting the pupil, as between the onset of the arthritis and of the uveitis. may be made only by the radiologist.

70 71
 TABLE 6-2. Treatment of granulomatous uveitis. removed safely despite the low-grade active LENS-INDUCED UVEITIS
uveitis. This disease is believed to be
Anti-infective Chemotherapy Use of Corticosteroids degenerative rather than inflammatory because There are no data at present to substantiate
Toxoplasmosis If central vision is Uireatcned, give pyrimetha­ If the response is not favorable after 3 there are no posterior synechias, corticosteroid the implication that lens material perse is toxic,:S0
mine (Darapririi), 75 mg Drally B3 a loading weeks, continue anti-infectivc therapy and therapy has no effect, and the eyes are remarkably that the term phacotoxic uveitis should no longer
dose for 2 day6 followed by 25 mg twice dally give systemic corticosteroids, eg. predniso­ tolerant to cataract surgery. . be considered as a type of lens-induced uveitis.
for 4 weeks, in combination with trisulfapy­ lone, 40—80 mg every other day for 4-6 The terms phacogenic or lens-induced uveitis’are
rimidines (sulfadiazine, sulfani era zinc, and weeks,* to protect the macula. Corticoste­
sulfamethazine, 0.167 g of each per tablet), 4 roids may activate the organisms of toxo­ more appropriate when referring to an autoimmune
g orally as loading dose followed by 0.5 g 4 plasmosis and tuberculosis, but are given as a disease secondary to lens antigen. The classic
times daily for 4 weeks. If a fall in the white calculated risk to control the in tlammatory CHRONIC CYCLITIS case of lens-induced uveitis occurs when the lens
or platelet count occurs during therapy, give response when it threatens vision. (Pa rs Planitis) develops a hypermature cataract. The lens capsille
folinic add (leucovorin), 1 ml IM twice week­
leaks and lens material passes into the anterior
ly.
Tuberculosis Isoniazid, 100 mg 3 times daily, and amino­ If a favorable response does not occur in 6 Chronic cyclitis refers to an entity that is chamber, causing an inflammatory reaction
salicylic acid (PAS), 4 g 3 times daily after weeks, continue antimyeobacterial therapy believed to begin in the ciliary body. The typical characterised by the accumulation of plasma cells-,
meals. If PAS is not tolerated, give strepto­ and give systemic corticosteroids, eg, pred­ patient is a young adult presenting with a chief mononuclear phagocytes, and a few
mycin, I g IM twice weekly. Continue treat­ nisolone, 40-80 mg every other day for 2 complaint of floating spots. Pain, redness, and polymorphonuclear cells. The eye becomes:red
ment for 4—6 months. months.*
Sarcoidosis Treat with local corticosteroids and mydriati.es and, during active stages, with systemic cortico­ photophobia do not occur. The patient is often and moderately painful; the pupil is small; and
steroids such as prednisolone, 40—80 mg every other day.* Give supplemental potassium chlo­ unaware of any problem, but the physician vision is markedly reduced (at times to light
ride, 2 g 3 times dally. The usual contraindications to systemic corticosteroid therapy apply. detects vitreous opacities * on routine perception only). Lens-induced uveitis may also,
sympathetic Treat with local corticosteroids and mydriatics and systemic corticosteroids in high doses, eg, examination. occur foil owing traumatic cataracts.
ophthalmia prednisolone, 40-120 mg every other day.* The usual contraindications to systemic corticoste­
roid therapy apply, and the drugs may be needed in higher doses and for a longer time. On biomicroscopy there are usually more Endophthalmitis phaco-anaphylactica,i the
Therefore, management of the side effects is often more difficult. In severe cases that fail to cells in the anterior vitreous than in the anterior term used for the more severe form of lens-induced
respond to corticosteroids, treatment with antimetabolites and alkylating agents has met with chamber. Other signs of anterior chamber uveitis, occurs following an extracapsular lens
some success. Caution: White blood counts and platelets must be monitored very carefully in inflammation such as flare, KPs, and posterior extraction when the same operation has already
these patients, and these drugs should not be used without careful consideration. synechias are minimal. been performed on the fellow eye and the patient
♦Administration every other day has been advocated to minimize the effects of stress and make drug withdrawal Indirect ophthalmoscopy often reveals has been sensitized to his own lens material. Many
■jasier and safer.
soft, round, white opacities (snowballs) over polymorphonuclear leukocytes and mononuclear
HETEROCHROMIC UVEITIS Cataract develops within a few years in the peripheral retina. These cellular exudates phagocytes appear in the anterior chamber,
(Fuchs’s Heterochromic Cyclitis) about 15% ofcases. Glaucoma occurs in 10-15% may be confluent, and they may overlie the pars eye becomes red and painful, and vision is blurted.
of cases. No treatment affects the course of this plana. Some of these patients have vasculitis Since most of the lens material has already lJepij
This disease of unknown cause accounts type of uveitis. It is not necessary to dilate the as evidenced by perivascular sheathing. removed, treatment is conservative, consistirigjojf
or about 3% of all cases of uveitis. It is pupil, as this is one type of uveitis in which In the majority of patients, the disease corticosteroids locally and systemically plus
essentially a quiet cyclitis associated with posterior synechias do not form. The disease remains stationary or improves slowly over a atropine drops to keep the pupil dilated. If this is
r-% , depigmentation of the iris in the affected eye. does not subside spontaneously, but the visual period of 5-10 years. However, a minority of ineffective, the cataract incision must be opened
'athologically, the iris and ciliary body show prognosis is good since the cataract can be patients can develop cystoid macular edema and the anterior chamber irrigated. ' ?J
...... v noderate atrophy, diffuse fibrosis, patchy and degeneration as welL as complicated Glaucoma is a common complication ?jof
depigmentation of the pigment layer, and cataracts. Some patients develop cyclitis lens-induced, uveitis. Treatment consists of lens
...... . diffuse infiltration of lymphocytes and plasma membranes and retinal detachments. extraction after intraocular tension has been
:ells. Involvement is typically unilateral but may The cause of this entity is not known. brought under control. If this is done, batlt the
iv * . »e bilateral, and the eyes are of different colors. Treatment is usually reserved only for those uveitis and the glaucoma are cured, and the visual
The onset is insidious in the third or fourth patients who have decreased vision secondary prognosis is good if the process has not beep
decade. The lighter-colored eye is usually to macular edema. Topical corticosteroids are present for more than 1 -2 weeks. } 'i *
iffected, and there is no redness, pain, or used initially. If they fail, posterior sub-Tenon’s
•; photophobia; the patient is often unaware of or retrobulbar injections of corticosteroids may
the disorder until cataract formation results in be used. It must be remembered, however, that
f blurred vision. even relatively soluble drugs such as SYMPATHETIC OPHTt IALMIA i »
With the slitlamp (or loupe) one sees fine triamcinolone may persist for several months (Sympathetic Uveitis)
.vhite deposits on the posterior corneal surface, when injected into orbital tissues. i j ?:
flare and cells in the anterior chamber, and a Complications include glaucoma and cataracts Sympathetic ophthalmia is a rare tiut
slightly atrophic iris. Anterior vitreous floaters clue to the long retention of corticosteroids. devastating granulomatous bilateral uvpilis
nay be evident with the ophthalmoscope or These patients do well after cataract surgery. which comes on 10 days to many years following
dillamp. I-
73
 perforating eye injury in the region of the TUBERCULOUS UVEITIS tuberculin skin test is usually negative or only the retinal tissues. The clinical picture is variable,
iliaryhody, or following retained foreign body, faintly positive. When the parotid glands arc but typically there is a chorimtUnal lesion or
'he Cijusql is not known, but the disease is Tuberculosis causes a granulomatous type involved, the disease is called uveoparotid fever lesions, usually in the macular area, v. hich io the
rob ably! related to hypersensitivity to uveal of uveitis. Tuberculous uveitis is diagnosed (Heerfordt’s disease); when the lacrimal glands early stages appear as elevated while masses
igmenUjJt very rarely occurs following clinically far more often than the disease can be ; are involved, it is called Mikulicz’s syndrome. partially obscured by a hazy vitreous. m the later
ncomplicated intraocular surgery for cataract proved by positive identification of tubercle bacilli ", ’ Thirty percent of cases are complicated by stages, a pnnehed-out pigment -d chorioretinal
r glaucdiria. in the tissues. Although the infection is said to be ’■ chronic bilateral anterior uveitis, whereas posterior lesion develops through which ill- jclera is clearly
The rnjured (exciting) eye becomes transmitted from a primary focus elsewhere in the < uveitis is far less common. Anterior uveitis is visible. The methylene blue dye test on the
iflarijpd 'fjist and the fellow (sympathizing) eye body, uveal tuberculosis is rare in patients with nodular, and in prolonged cases may lead to severe patient’s serum is positive, ie, antibodies in the
econd. tPathologically, there is a diffuse active pulmonary tuberculosis. 4 visual impairment due to cataract and secondary serum prevent the dye from staining toxoplasma
ranujoma’tous uveitis. The epithelioid cells, Tuberculous uveitis may be diffuse but is !.. glaucoma. Posterior uveitis is characterized by organisms.
>gct$er with giant cells and lymphocytes, form characteristically localized in the form ofa severe C multiple whitish-yellow retinal exudates and Although the pathology of the chorioretinitis
oncaseating tubercles. From the uveal tract the caseating granulomatous chorioretinitis. The - perivasculitis. is essentially the same, there are 2 distinct clinical
iflairima^ory process spreads to the optic nerve tubercle itself consists of giant cells and ? Corticosteroid therapy given early in the types of ocular toxoplasmosis: congenital and
nd th tlie' pia and arachnoid surrounding the epithelioid cells. Caseation necrosis commonly . disease may be effective, but recurrences are acquired. The congenita] type is due to
ptic’nery^. occurs. common and the long-term visual prognosis is intrauterine infection. As in the acquired (adult)
the 'patient complains of photophobia, The patient complains of blurred vision, aod . poor. type, the chorioretinitis is usually confined to the
sdnqss, and blurring of vision. If a history of the eye is moderately injected. If the anterior j macular area. The inflammatory process is more
raunria is. obtained, look for a scar representing segment is involved, iris nodules and “mutton fat’1 severe in congenital than in acquired
ae wbunWpf entry in the exciting eye; With the KPs are visible on slitlamp examination. If the T0XQPLASM1CUVE1TIS toxoplasmosis, and is frequently bilateral. Cerebral
litlamp orloupe one sees KPs and a flare in the choroid and retina are primarily affected, one can « involvement, with radiopaque calcification and
ntenor chamber of both eyes. Iris nodules may see a localized yellowish mass partially obscured ? Toxoplasmosis is primarily an inflammation mental deficiency, is present in 10% of cases of
•c present by a hazy vitreous. of the CNS caused by Toxoplasma gondii, a the congenital type.
Sympathetic ophthalmia may be The nodules and the localized nature of protozoan parasite. In congenital toxoplasmosis, Acquired ocular toxoplasmosis is usually
iffere ntjilted from other granulomatous Luberculous uveitis help to make a clinical ‘ chorioretinitis is almost invariably present. milder than congenital toxoplasmosis. It may
veitides by the history of trauma dr ocular differentiation from sympathetic ophthalmia, and ' Toxoplasmosis occurring in later life does not appear at any age, is often unilateral, and
urgcry and by the fact that it is bilateral, the caseation necrosis differentiates it usually involve the eye in the acute stage, but frequently occurs in the absence of CNS
.iffuse,hand acute Tather than unilateral, pathologically from sympathetic ophthalmia and chorioretinitis frequently occurs in the chronic involvement. The patient complains pf blurred
ocalizedjand chronic. Lens-induced uveitis is Boeck’s sarcoid. form. vision if the macula is involved.
dluteraljimd there is no history of injury. The pupil should be kept dilated with The disease is more common, in tropical The chorioretinitis progresses to a healed
The1 recommended treatment of a severely atropine, 2%, 2 drops 2-3 times daily. countries. Pathologically, there is a granulomatous stage, leaving a scarred retina and choroid. If the
njur^d eyb (eg, a penetrating injury through Antituberculosis drugs should be prescribed chorioretinitis. The retina is characteristically macula has been involved, loss of central vision
he sclera^ ciliary body, and lens, with loss of systemically if a reasonably certain clinical necrotic, and toxoplasma organisms are found in is permanent
’ilrepiusTis immediate enucleation to prevent diagnosis can be made.
ympathctic ophthalmia, and every effort must After a prolonged course of several months,
>e rnade-jto secure the patient’s reasoned the disease usually resolves, leaving permanently
lonsertt Jbbthe operation. If enucleation can be damaged tissue andblurred vision due to scarring
lerfpnnediwithin 1.0 days after injury, there is of the retina.
ilmo^t nefchance that sympathetic ophthalmia
vill develop. However, when the inflammation
n the sympathizing eye is advanced, it is wise SARCOIDOSIS
jot td enucleate the injured eye since it may (Boeck’s Sarcoid)
wenjfially prove to be the better of 2 very bad
jyesJV Sarcoidosis is a chronic granulomatous
If inflammation has appeared in the disease of unknown cause characterized by
lympiftl lining eye; begin treatment immediately multiple cutaneous and subcutaneous nodules
.vith local'corticosteroids and atropine. Systemic with similar invasions in the viscera and bones
iorlippsUroids may be required. and periodic exacerbations and remissions. The
$/ith&ut treatment, the disease progresses onset is usually in the third decade. The Ussue
slowly but relentlessly over a period of mouths reaction is much less severe than in tuberculous
3T ycarsito complete bilateral blindness. uveitis, and caseation does not occur. The

74 75
 parenchymal layers are displaced centrifugally;
7 3nd the internal limiting membrane is thin.
to be used later when the need arises. ; ; ,j.«
These chemical changes initiate a transient j
Throughout most of the retina, the axons of the excitation of the receptor which is propagated/:
RETINA receptor cells pass directly to the inner side of the
outer plexiform layer where they connect with
along its axon. The first step in analysis of these'
raw data is achieved by a complex system of-
processes of horizontal and bipolar cells which interconnections between the axon and th^.celhl
extend outward from the inner nuclear layer. In processes of the horizontal and bipolar cells; 7? he
the macula, however, the receptor cell axons follow bipolar cells transmit the refined information to ■
L* an oblique course, and this region is called the the inner plexiform layer,-where it is once iriprfc-
Henle fiber layer. The normally empty extracellular modified through connections between amacrine,,1
space of the retina is potentially greatest in the bipolar, and ganglion cells. The ganglion ccll$pass.;
macular area, and diseases that lead to this reanalyzed information to the brain. ’ /
The retina covers the inner aspect of the especially noticeable when young, heavily
accumulation of extracellular material cause
posterior two-thirds of the wall of the globe. A pigmented patients are examined with an indirect /
considerable thickening of this layer.
fundus photo of a normal retina is shown in Fig 7-1. ophthalmoscope. On examination with the direct v Outer member i
The bipolar cells establish connections with
The retina is a multilayered sheet of neural ophthalmoscope, the concave foveal surface 3
amacrine and ganglion cells in the densely woven
tissue closely applied to a single layer of produces a clearly visible inverted image of the ••
inner plexiform layer. The long axons of the Ellipsoid
pigmented epithelial cells (Fig 7-2). The anterior lamp. Absence of this foveal reflection may?/
ganglion cells course through the nerve fiber layer (fiber apparatus) ^/-Ellipsoid ! ’-J
‘ extremity of the retina is firmly bound to the indicate disease, but the reflection may be absent ■"
to the optic nerve. / J: ’ ;|
pigment epithelium, which in turn is attached to in blond or elderly patients even though the retina Z- Inner member
The retina receives its blood supply from 2 Inner mernbpr
Bruch’s membrane. Posteriorly, the optic nerve is normal. '{■ ■ij
sources. The choriocapiltaris is a single layer of Myoid
fixes the retina to the wall of the globe; elsewhere, The retina is composed ofhighly organized, External limiting__ I
closely spaced capillaries intimately attached to
the retina and die pigment epithelium are easily delicate tissue consisting of 9 histologic layers. 3 membrane
the outer surface of Bruch’s membrane. The Cono nucleus
separated. In adults, the ora serrata-the partly The fovea centralis, which lies about 3.5 mm lateral Rod fiber
i.
choriocapillaris supplies the outer third of the Cone fiber ./
serrated anterior end of the retina-is about 6.5 mm to the optic disk, is specialized for fine visual
retina including the outer plexiform and outer
behind Schwalbe’s line on the temporal side of discrimination. In the fovea, the receptors are all £ Rod nucleus-—-
nuclear layers, the photoreceptors, and the
the eye and 5.7 mm behind it nasally. cones; the outer nuclear layer is thinned; the other 3
pigment epithelium. The inner two-thirds of the Varicosity
The retina is 0.1 mm thick at the ora serrata
retina receive their nourishment from branches of
and 0.23 mm thick at the posterior pole. It is Bruch's membrane End-bulb Cone
the central retinal artery. The macula receives all
thinnest at the fovea centralis, the center of the •pigment epithelium
of its blood supply from, the choriocapillaris, FIG 7-3. Rod (left) and cone (right). .
macula. The retina is normally transparent, and
Rods and cones rendering it more susceptible to irreparable damage
some of the incident light is reflected at the
when the posterior retina is detached. The cones are used for detailed vision feg;i-[
vitreoretinal interface. The resulting sheen is External limiting
reading or distinguishing distant objects):bud-'
membrane
color perception. They predominate at the macula^ I
Outer nuclear layer PHYSIOLOGY the center of visual attention; and the fovea,"the}
site of best visual acuity. The rads predominate ift;
The ability of the retina to analyze all other parts of the retina and function best irt;
information is best understood in light of its origin reduced illumination. The principal roles of the,
Outer plexiform layer
as an outpouching of the brain. extramacular retina are night vision and Vishal
The optical system of the eye focuses a orientation. Far example, the peripheral retina1
miniature image of the field of vision upon the makes it possible to walk without trippingioyer.
Inner nuclear layer
outer segments of the rods and cones, where light objects that are discerned as hazards even thoiigh '
functions as the initiating event in a complex chain -they are not clearly seen. The orienting function,
Inner plexiform layer ofchemical reactions. The light-sensitive pigment makes it possible to fix one’s gaze on a mdving;
is composed ofa small molecule, retinal (vitamin object. f.J •
Ganglion cell layer
A aldehyde), bound to a large protein, opsin. The
pillory
retinal is the same in both rods and cones, but the Physiology of Symptoms J
rve fiber layer opsin differs. Light isomerizes the retinal from the The retina is designed to receive visual:
Internal limiting 11-cis to an all-trans shape, so that it no longer images and transfer them to the brain. It conteinSL
membrane remains adherentto die opsin. The liberated all-trans. no pain fibers. When tire retina is normal and:tbere:
FIG 7-1. Ophthalmoscopic view of a normal retina. FIG 7-2. Layers of the retina. retinal may be metabolized to the 11-cis form and be are no uncorrected refractive errors, the images
Note deep physiologic cup. reformed to opsin, or it may be stored as vitamin A received will be sharply in focus. Most disorder^
76
 ■f
of thefrctiha cause blurred vision. If the macular consensual light reaction remains normal. RETJNAL VEIN OCCLUSION CoiupUo.tfohS & Pi UgHOsis
arpa' is diseased^ the patient’s central visual If the patient is seen within 2 hours of the J Cystoid degep‘nation of the maciija,
will be affebted and he will have difficulty onset of symptoms, one should attempt tar* neovascularization,, vitreous hemorrhage, optic
reading and discerning objects in the distance restore the blood flow by massaging the globe, ? 1. CENTRAL VEIN OCCLUSION atrophy, and hemorrha, ic (i.eovascu’lariTlaiicoiTia
(bg.iStreet signs); If the peripheral portion of by anterior chamber paracentesis, and perhaps^ arc serious cornplicationsjrf,centra I retinal vein
thi? ^tina.is diseased, side vision is impaired with vasodilators. Papaverine and tolazoline This is an uncommon unilateral condition occlusipJEWbout hall of patients will have no
but tiic patient will continue to read well. In may be given with the patient at bed rest. It has? which occurs more frequently in older patients. useful vision (less than 20/200), and one-fifth
extreme cases of) contracted peripheral visual not been proved to be effective. The globe is ? The obstruction occurs at the lamina cribrosa as a develop hemorrhagic glaucoma.. Young patients
fields; the patient can read the finest print but massaged by pressing firmly through the closed? result of an anatomic nanowing of the central with central retinal vein occlusion have a better
will ibhrap into large objects such! as chairs and lids for several seconds and then abruptly? retinal vein just behind the lamina. Distal to it, the prognosis than older individuals.
desks when passing through a strange room. releasing the pressure. This maneuver is y- vessels become engorged, the retina becomes
; ‘ There is no pain with retinal disease, and repeated several times. edematous, and retinal capillaries rupture,
the; by p docs not become red or inflamed. Anterior chamber paracentesis is b resulting in retinal hemorrhages. The patient
: • ■ '.The clinical Work-up of patients with retinal performed. The rationale of this procedure is to? usually complains of sudden painless loss of
diseases should include the following: history, lower the intraocular pressure suddenly in the ? vision.
visual'acuity, bioraicroscopy, direct and indirect hope that the blood behind an embolus will'd
oplitiYalmpscopy, fests for color vision and visual dislodge it to a more peripheral branch of the ?
fields; and medical evaluation. Other helpful vessel. '
diagnostic tests may include fluorescein The measures outlined above may help if?
ahgipjjcopy and angiography, electroretinography they are instituted shortly after the onset of?
(BrtG); clcctrooculography; (EOG), dark adaptation, symptoms because the dual blood supply of?
and echo-ophthalmography. the retina allows it to survive much longer than ?
one might expect. There is experimental?
evidence that oxygen under high pressure may ?
allow the retina to survive still longer. However, f;
1 f complete loss of vision persi sts for more than
i
DISEASES OF THE RETINA 2 hours, the value of any type of therapy is
questionable. FIG 7-6. Thrombosis of the central retinal vein,
FIG 7-5. Microscopic section showing thrombus
in the central retinal vein. (drawing ).

i RETINAL ARTERY OCCLUSION Ophthalmoscopically, the retina appears Treatment

I edematous, the veins are dilated and tortuous, Anticoagulants and corticosteroids have
Blockage or
■; j ftsiocKage of me or one
the central retinal artery di and there are diffuse retinal hemorrhages with a been shown to be of no value in central retinal
of if^branches is an uncommon unilateral1 few cotton wool patches. Fluorescein vein occlusion. Photocoagulation of the retina
disorder of older patients. The obstruction may angiography reveals leaks offluorescein from the beneath the new blood vessels-may help in
bfedub to an embolus or may be caused by dilated capillaries. The area around the optic disk d ecre a sin g thejncidence ofyitreous-hBrnerfhage.
intirnia’1 atherosclerosis of the vessel wall. When appears as though it had been slapped with a red Hemorrhagic glaucomajs.difficult to control and
thb central retinal artery is affected, the result paint brush (Fig 7-6). Vision is usually decreased
is su’dden complete loss of vision in the but not lost, ■ alcohol or by eniicleatioii_^-
aiiefcted eye. Ophthalmoscopic examination Diabetic retinopathy and hypertensive It should be emphasized that patients with
witlifp 2 hour's after occlusion shows retinopathy can be easily distinguished from central retinal vein occlusion and branch vein
sbjgni^ntation of the blood column. Later, the central retinal vein occlusion. Both conditions are occlusion nmsL have a medical evaluation so that
vessels may appear normal, but emboli are often bilateral, whereas central retinal vein occlusion is underlying medical diseases can be identified ami
at the bifurcations. The posterior retina usually unilateral. treated.
is pale because of changes in the axons of the About one-third of patients with centraL
nerve; fiber layer (Fig 7-4). Since the inner retinal vein occlusion have open-angle glaucoma
Tci-iuejl lijyers are absent at the fovea, the FIG 7-4. Twenty-four hours after left central in both eyes. This is considered to be a 2. RETINAL BRANCH VEIN OCCLUSION
ret-ma,' remains transparent in this area and retinal artery* occlusion. Ischemic changes have predisposing factor. Central retinal vein occlusion
the tiboroid shows through as a cherry-red made the nerve fiber layer pale and opaque. may' be less frequently associated with systemic Branch vein occlusion is a more frequently
stypL Alj visiop may be lost. The direct Because the fovea lacks this layer, the choroid diseases such as diabetes mellitus, hypertension, encountered clinical entity than central retinal vein
piipiljary response is absent, although the can be seen as a cherry-red spot. polycythemia vera, dysproteinemias, and sickle occlusion. Tire obstruction occurs at the site of
cell disease. the arteriovenous crossing. The superior temporal
78
 i
retinal vein is most often involved. The vein and occur early in the disease process. Leakage from- ‘ Treatment
its branches distal to the site of obstruction show vessels may result in retinal edema and hard and A
evidence of engorgement and increased soft exuduresL~Edefna~or exudates in the macularf;- Careful control of bloodglucose in patients
tortuosity. Hemorrhages are observed along the area are responsible for impairmentof visual acuity.^ with diabetes does not delay the onset of
sector which drains in the affected vein. Vision is The arterioles reveal no abnormalities.' • retmopathyLand..U('£L.not affect or modify the
affected when the macular area is involved. Histologically, the microaneurysms occur on the X course of the disease.
Fluorescein, angiography demonstrates the site venous side of capillaries in die inner nuclear layer. v:
Recent reports have indicated that long­
of obstruction. Round hemorrhages occur usually in the outer ‘ term therapy with sodium salicylate may prevent
Three-fourths of patients with branch vein piexiform layer but may occur in any layer of the
proliferative diabetic retinoiTathyi
occlusion have high blood pressure. Sickle cell retina. J " Because of the highly variable course of
disease, polycythemia vera, lymphoma, leukemia, The course of nonproliferative retinopathy ?. diabetic retinopathy, it is difficult to evaluate
macroglobulinemia, and multiple myeloma are less is variable. Over 60% of patients with visual the results of any form of treatment. The
common causes ofTetinal branch vein occlusion. impairment from diabetes suffer from 5 retinopathy may be relatively stable, slowly
Neovascularization, and macular edema are 2 nonproliferative retinopathy. In general, the .■ progressive, or rapidly progressive.
serious complications of bianch vein occlusion. prognosis with nonproliferative retinopathy is T
Spontaneous regression may occur in some
Neovascularization may predispose to vitreous better than with the proliferative type. patients.
hemorrhage. Macular edema is encountered in
occlusion of the superior temporal branch. B. Proliferative Retinopathy: The most - A. Nonprofiferative Retinopathy: The FIG 7-8. Diabetic retinopathy^ New vessels at
One-fourth of patients with branch vein outstanding feature of proliferative retinopathy pathologic findings consist of the vitreoretinal interface. } ’ll.
occlusion have no useful vision (less than 20/200). is new vessel formation as new blood vessels .?■ microangiopathies with leakage of capillaries i L’
which are confined to the retinal layers. The : s. . • S »i
Treatment intemai-luniiiflg-membr-afier Neovascularization - \ mfftrr CumpTIcation in these patients is < ‘ 5 I J1 i
Photocoagulation of the obstructed vein (Figs 7-8 ) may be limited to the surface of the •; EDEMA OF THE RETINA ; > •! •;
involvement oi the macuiar area,. "Ths causes a
may help in preventing neovascularization but retina or may involve the vitreous body also. In.
decrease Tn central visual acuity and is known i f hl ;
may not prevent macular edema. tlie“dptic nerve head area, neovascularization • ‘ f- .
as diabetic maculopathy. Careful and close
A full medical work-up is strongly indicated proliferates into the vitreous, an J sue . patients ?,
observation of these patients is mandatory Between the retinal cells there is a potential .
in all patients with retinal branch vein occlusion. have a poor visual prognosis and are difficult to
since certain early ch.angg_g.in the macular area space which fills with fluid when thc: retinal:
treaCThe most serious complication is intraocular can be-reversible-iLtreatecLptoperiy? Capillary capillaries leak. The accumulation is greatest-in. ;
hemorrhage. This induces fibrovascular /
leakage sites which are causing exudations-and the outer part of the outer piexiform lay er, less'id
proliferation..mth^fiieous-axharacteristicfeature ■■
fidema in the macular area-can„bE. localized by the outer and innerTiuclcar layers, and negligible-
DIABETIC RETINOPATHY of advanced proliferative retinopathy-and
fluorescein angiography and sealed off by in the remaining tightly woven layers. Because of i
ultimately^resuits in vitreous contraction, retinal =
phoiocoagulation. This method of treatment is the oblique course of the receptor cell axons in
traction, and retinal detaclunent with loss Qfvision. •
contraindicated in asymptomatic patients dr in the Henle fiber layer, ibemacula swells more than :
Diabetic retinopathy may be classified into 2 patients with normal visual acuity. the rest of the retina and the fluid is often visibly >
main types: nonproliferative and proliferative. Photpcoagulation in hire papillomacular area loculated in varicose tunnels radiating frontythei |
The course and prognosis depend on which carries thertsk dChemorrhage with subsequent fovea, which itself becomes a single Cystjike.
category thepatient falls in. Although early fundus visuai-less. ~ cavity. | ‘i ’ J
changes are difficult to place in proper perspective,
Chronic edema is often accompanied* by’
this clinical differentiation remains of crucial B, Proliferative Diabetic Retinopathy: minute yellow deposits, mainly in the outer’
importance in the management of patients with There is npjjffective treatment formost stages piexiform layer or beneath the retina. In the Hettle;
diabetic retinopathy. Qf_advanced proliferative retinopathy^ and fiber layer, the aggregates have a striking stellatei
phptocoaguiation isjcuf ram 1 icated in the late distribution. Elsewhere, a nidus of edemh :isj'
stages of the disease. Several methods of surrounded by a circular belt of tliese deposits;, df
prophylactic therapy, however, have been the circinate figure is close to■ the macula, tit’
Clinical Findings & Course described. The aim oi therapy is to arrest the spreads along die spaces of the Henle fiber lay eq.
proliferative process by decreasing the change forming one arm ofa star figure. Beneath the retiiuqi
A. Nonproliferative Retinopathy: Patients of intraocular hemorrhage/fhis can be achieved' the fatty material is irregularly clumped fik-s. .the .
with nonproliferative retinopathy have by photocoagulation. Photocoagulation is aggreg:.-.cs sometimes seen when new vessels' ■
funduscopic changes which are confined to the employed in an attempt to seal off new vessels grow ci’.:-, through Bruch’s membrane. J T|
retina. These retinal changes are characterized by and tufts oficapillaries which have proliferated The causes ofretinal edema include diabetes,'!
dilatation of veins, microaneurysms, and retinal through the internal limiting membrane on the
FIG 7-7. Background diabetic retinopathy. retinal wH. obstruction, hypertension, retinal1
heniorrhagesJFig 7-7). The microangiopathies surface of the retina andTFuo the vitreous. angiomas telangiectases, traction by (tbej?
80
81
vitreb'u's?; or preretinal membranes, and in 1942 and caused total blindness in a larg&A RETINITIS PIGMENTOSA The electroretinogram is reduced or absent,
inflamlftaLions involving the vitreous or retina. number of patients until 1954, when the cause*! and the electrooculogram gives a flat curve.
Irvine-Gjass syndrome-sudden, usually temporary was found to be excessive oxygen given during*} Commonly associated eye findings are myopia,
loss ofvisual acuity in aphakic patients-is probably the first few weeks of life. The high oxygen-* Retinitis pigmentosa is a group of hereditary posterior polar cataract, and glaucoma.
a ^pedifi’c example of retinal edema due to concentration caused an abnormal response by J In rare instances where retinitis pigmentosa
dystrophies of the retinal receptors transmitted
inflammation. the blood vessels. New cases of this disease-f as autosomal recessive, autosomal dominant, or is due to abetalipoproteinemia (Bassen-Komzweig
. , Blurred vision occurs only when the macula are rare. *•# X-linked traits. The rods are slowly destroyed, syndrome: steatorrhea, ataxia, and retinitis
is involved. Visual acuity may be only slightly Vascular spasm and edema of the immature?? with secondary atrophy of the remainder of the pigmentosa), its progress may be arrested in the
affccted!p‘r may be as low as finger counting. The peripheral retina are followed by vascular^? early stages by massive doses of vitamin A. There
retina and the pigment epithelium. Cells filled with
elevation., and ground glass appearance of the diLatation and fibrovascular proliferation into i epithelial pigment aggregate along the retinal is no specific therapy for the other causes of
macular hrea can be quite striking, and one may the vitreous. Total retinal detachment occurs.§ retinitis pigmentosa. Genetic counseling should
vessels to give the typical bone corpuscular
disc£rpa.;cystoid accumulation of fluid. Away from later. A appearance (Fig 7-9). These changes begin in the be offered in an attempt to prevent propagation
the; macula, areas of edema present a marked . In advanced cases, one sees in both eyes?; mid periphery, sparing the macular and peripheral of the disease.
contrast;between normal and abnormal retina. a white retrolental membrane containing blood \ regions until later. The retinal problems are often
When fefijnal edema is widespread, with relative vessels. The anterior chamber is shallow, and j associated with deafness, mental retardation, and
sparing pf the macula-as is often the case in the pupillary light reflex is absent. The lens is'7 other systemic findings, making several distinct
diabetic patients-the ophthalmoscopic diagnosis PAVING STONE DEGENERATION
clear, and transillumination is normal. The; syndromes. The most common of these is
is difficult The only clues are retinal pallor and Laurence-Moon-Biedl syndrome, which combines OF THE RETINA
contracting retrolental tissue draws the ciliary '7
difficulty; in distinguishing details of the pigment
processes inward so that they can be seen withr- retinitis pigmentosa, obesity, mental retardation,
epithelium and choroid. polydactyly, and hypogenitalism.
the ophthalmoscope at the periphery' of the.*
: Ijitfpvenous fluorescein confirms the Night blindness - the first symptom ofretinitis Paving stone degeneration is a striking but
dilated pupil.
diagnqsis of retinal edema because the dye leaks pigmentosa-usually occurs in early youth. harmless condition of the peripheral retina in
Differentiation from persistent hyperplastic t
from thefiprmally impermeable vesselsand stains
vitreous usually is not difficult since the latter •; Thereafter, the visual fields gradually constrict which the pigment epithelium and outer retinal
the-extracellular fluid and because cystoid
condition is unilateral. Retinoblastoma has a-4 (“gun barrel vision”) to become disabling in the layers are damaged or absent. The intact inner
collpctipius at the macula which, cannot be
later onset, may not transilluminate, and the? fifth or sixth decade, at which time macular vision retina is adherent to the exposed Bruch’s
discerned with the ophthalmoscope will become may also be lost The fundi may appear normal at
anterior chamber is of normal depth. If bilateral, membrane and also to the pigment epithelium at
easily Visible. Later, if there is frank cystoid
degenera'iion, the cavity contents no longer stain. the tumor is usually more advanced in one eye. ? first. Later, most patients have the typical scattered the edge of the lesion. The vitreous and the outer
Glaucoma, uveitis, cataract, and phthisis black pigmentary disturbance. The retinal choroidal structures are normal.
Fiubrestjein does not stain the waxy yellow The sharply outlined, rounded lesions are
bulbi frequently occur months or years after the arterioles become attenuated, and the disk
dcp'osLts observed in chronic macular edema. pale yellow, often with a partial rim of increased
onset of retrolental fibroplasia. becomes pale and waxy. In rare instances, the
' Persistent macular edema may eventually disease may affect only one eye or just one sector pigmentation. They range in size up to 1 disk
become transformed into cystoid degeneration, Premature infants with poor pulmonary £
function should receive just enough oxygen to C of an eye. diameter and may occur singly or in clusters.
with irreversible loss of central vision. In some
cases, th.!si is compounded by development of an maintain an adequate blood oxygen tension.
atrophic hole at the fovea. Oxygen administration should be controlled by \
• If macular edema is due to inflammation, blood gas measurements-not by percentage ?
corticosteroids may help when given systemically flow rates-and the arterial oxygen concentration */.
■-1
and by ipjarabulbar injection. Most patients with should be maintained in the range of60-80 mm ?
Irvine-^Gass syndrome need only explanation and Hg (never exceeding 100 mm Hg). If blood gas ■?
encouragement since they will improve without measurements are not available, the oxygen 1
treatment^ Focal edema due to vasculopathies- concentration should be kept below 40% and J
including diabetic retinopathy-often responds to the infant transferred as soon as possible to a f
photocb^gulation. There is no treatment for facility where such measurements can be made. ?
edema'caused by traction on the retina. In the meantime, the infant should be examined J.’
daily with the indirect ophthalmoscope for 4,
spasm of the retinal vessels, a sensitive gauge ?
of oxygen poisoning. |
retrolental fibroplasia Retrolental fibroplasia usually has its onset •>.
in the first few days of life and progresses ;•
rapidly to blindness over a period of weeks, y
' Retrolental fibroplasia is a bilateral retinal Once blindness occurs, there is no hope for ; FIG 7-9. Retinitis pigmentosa. Left: Typical “bone spicule” arrangement of pigmentary changes.
disease of premature infants. It was first reported restoration of sight.
Right: Clumped, scattered pigment, attenuated arteries, and choroidal sclerosis.
82 83
Several lesions may coalesce to form a the abnormal retina in over 10% of lesions. These? treatment can be given if a clinically significant
circumferential band with a scalloped margin. sclerosed vessels form a white latticework which r detachment does occur or is impending. In
inspired the name. younger patients, such detachments spread
slowly, with multiple demarcation lines, and
surgery is required.
The more common mechanism by which
lattice degeneration leads to retinal detachment
derives from the fact that, owing to their posterior
location, about two-thirds of the lesions have
established a firm vitreo-retinal adhesion behind
the vitreous base. If the vitreous detaches
posteriorly, the stress at the ends and the posterior
border of the lattice may be enough to produce a
tear. The break is in the relatively normal retina
beneath the rim and not through the thinned
retina. Small lesions far back may be completely
avulsed. Much more often the tear is J-shaped
and located at one end of the lattice. As the
vitreous continues to tug on the retina, these tears
usually cause extensive retinal detachment.
FIG 7-10. Paving stone degeneration. FIG 7-11. Lattice degeneration. The threat of retinal detachment has given retinal tear is an operculum of avulsed libsue; ifhe
lattice degeneration notoriety in excess of what it
The process occurs mainly in the lower The above features are often not visible on deserves. Most people with this condition enjoy a size of the tear may vary tremendously.-j ‘ |
quadrants, though it is found in any part of the routine inspection, and areas of lattice might be j normal asymptomatic existence. They should, Retinal tears may be due to constant or
peripheral fundus. It is fairly common in young missed were it not for the edge-on view afforded ■ however, be checked periodically, and must be seen intermittent vitreous traction. i
adults, and its incidence and the frequency of by scleraL depression. With tills aid to examination, - immediately if they report symptoms suggestive of Tears due to constant vitreous?traction.
bilateral occurrence increase with age. It is quite the shaqj rim and roughened depressed surface vitreous detachment If it is obvious that retinal Proliferative diabetic retinopathy, pdhbtrtrting
innocuous and the patient is unaware of its of the trough are readily seen. detachment is likely-because of retinal detachment injuries, and a variety of other disorders, cause
presence. The individual lesions are about one-third in the fellow eye, a family history of retinal scarring ofthe vitreous. Contraction of the schrred
disk diameter in width and one-half disk diameter detachment, or local findings-then one should vitreous bands may tear tbe retina at tpbiripoints
to 1 quadrant long. They generally lie parallel to perform prophylactic cryothermy or of attachment. Only a small percentageipfretinal
the ora serrata, are confined to the equatorial and photocoagulation. tears occur by this mechanism, but it is, irripdrtant
LATTICE DEGENERATION oral zones, and are concentrated toward the to recognize and treat them promptly; for they
vertical meridian. There may be as many as 20 usually lead to extensive retinal detac^fneht. <
lesions in one eye forming 2 or more parallel rows. Tears due to intermittent vitreoti^traction.
Lattice degeneration of the retina is Both eyes are affected in about a third of cases. RETINALTEARS & HOLES When the vitreous is partly cqptractqd,
characterized by elongated excavated troughs in About 8% of the population have lattice acceleration or deceleration of the glot>ej-eilher as
die peripheral retina. The lesions are surrounded degeneration, and the incidence is constant in a result of normal eye movements! fyigtJr.qus
by a distinct narrow rim which often projects above each age group after 10. Surprisingly, teenagers The fovea and the peripheral retina are tbe activity, or indirect trauma-will causCjpyhiplike
the level of the normal adjacent retina. The may have heavily pigmented lesions with extreme sites of predilection for retinal holes. In both motions of the collapsed gel with corfesponding
vitreous is firmly adherent to tire rim and forms a thinning of tbe retina, whereas septuagenarians places, the internal limiting membrane and retinal stress which may be sufficient to tear th’e retina at
canopy which envelops a, pocket of fluid lying in wilL often have rather superficial nonpigmented parenchyma are thin. Extramacular tears and holes normal points of vitreoretinal attachment asw.ell
the trough. Close to their insertion, the vitreous lesions. Atrophic round holes are common in all are described here. Retinal tears have an as at abnormal vitreoretinal attachments/iThp latter
fibrils are condensed and may be visible as a white age groups. operculum, (lid) of retinal tissues, whereas retinal may be due to a number of anatomic jahoiqalies
trill. Lattice degeneration is an etiologic factor in holes do not. and pathologic processes which nftlyitiot be
Glistening white spots are common on the about one-third of all cases of rliegmatogenous recognizable before the tear appears. ■ •. !
retinal surface, giving a snail-track appearance to retinal detachment. One of 2 distinct mechanisms The greater the space inside the, eye, the
some lattice areas. Although the pigment is responsible for the detachment. In one group I. RETIN AL TEARS greater the force that can be exerted id tear, the
epithelium beneath the lesions may appear normal, of patients, the leakage is through an atrophic (HOLES WITH AN OPERCULUM) retina. This may explain why aphakia,,(the large
it is often disordered and may vary from subtle hole. This may result in tiny localized: areas of glob, munonly associated with myopii, forward
disarray to gross disorganization. The walls of detachment that rarely spread, and no treatment The retina is tom by mechanical force,usually dispi. .. jincnt of the lens by miotics, ancLlocalized
Lhc retinal vessels are thickened where they cross is required other than periodic observation so that vitreous traction. The distinctive feature of a scler:-: -jetasia predispose to retinal tears,■
84
 epithelium is often altered; and a ring of pigment / RETINAL DETACHMENT
As a rule, the larger vessels do not break, so
epithelial reaction may appear at the edge of the
that the retina either continues to tear beyond the ■
narrow halo of retinal detachment which often
vessel and a segment of the vessel is elevated off .,i
accompanies even innocuous holes. In the Because the retina is only loosely adherent
the retina or the vessel arrests the shearing force, k
absence of frank retinal detachment, these findings to the pigment epithelium, the 2 may separate,
In younger patients, the acquired posterior .
(ell the examiner that the potentially dangerous allowing fluid to accumulate between them. The
extension of die vitreous base is narrow, so that
lesion has been present for some time without fluid usually comes from the vitreous, having
tears may occur close to the ora serrata. Since the '
causing trouble and impose on him a conservative passed through a hole in the retina. Less often, it
mechanical reinforcement of the retinal vessels is
attitude toward prophylactic therapy. leaks from blood vessels, as happens with central
lacking, the rip can extend a considerable distance ’
Most retinal holes are asymptomatic. Some serous retinopathy, choroidal tumors, some
around the circumference of the globe. [
are accompanied by symptoms of vitreous inflammatory conditions, malignant hypertension,
detachment. or when vitreous traction creates a subretinal
space without perforating tire retina.
Causes of Retinal Tears
Treatment
Vitreous traction, either intermittent or
The greatest problem in treating retinal Mechanism of Rhegmatogenous Retinal
constant, accounts for the vast majority of retinal :
holes is deciding whether or not treatment is Detachment
tears. This usually happens spontaneous!}', but
necessary. Unfortunately, the decision must be Rhegmatogenous (tear-induced) retinal .
indirect trauma occasionally precipitates a Tctinal
FIG. 7-4^- Retinal I tear. Completely avulsed made with an inadequate knowledge of the natural detachment is dependent upon* 3 factors: (1) a
tear in someone with preexisting abnormal
operculiirri- | I history of most types of hale and almost total retinal hole, (2) liquid in the vitreous compartment
vitreoretinal attachments and vitreous syneresis.
ignorance of the qualitative and quantitative with free access to the hole, and (3) a force
Direct trauma deforms the globe transiently
Thpbpbrculum pfmost tears retains a partial parameters of vitreous traction. sufficient to break the bond between the retina
and can cause conventional tears, giant tears at
attachment at the anterior margin of the break, so The objective ofprophylactic treatment is to and the pigment epithelium and transfer fluid from
the posterior margin of the vitreous base, or tears
that thcivitfeoiis continues to tug on the retina. establish a firm chorioretinal bond around the in front of to behind the retina. The vitreous •
in the non-pigmented epithelium of the pars plana
Even sd; such J retin il tears should be watched retina] break. The scar should be broader on the supplies this force in essentially the same way as
at the anterior margin of the vitreous base.
and not-treated unless (1) the vitreous cavity is side nearest the ora serrala, since this is usually it causes retinal tears.
Anomalous zonular attachments to the retina
unddly 'Iarg^; (2) the holes are multiple, large, or the site of greatest vitreous pull. Indeed, it is often Until one or more ofthese factors is nullified,
are also believed to cause tears. Another rare
c. ?T
1ar Behpi'd the ora serrala; (3) there is a history of mechanism is for the detaching retina-due to any safest to treat forward to the vitreous base to
forestall future vitreous traction.
the detachment will continue to spread. Most of
the retina may become detached in a few hours, or
retinal detachment in the fellow eye or a family cause-to tear at the site of chorioretinal adhesions
history of retinal detachment; or (4) there are recent There are 3 ways to seal a retinal hole; it may lake years for this to happen.
resulting from previous therapy, paving stone
symptoms 'of jvitreous detachment. In these degeneration, or chorioretinitis. In these latter
freezing, burning by light, or burning by electricity.
situation^ even though treatment is not always The mechanism in each case is to cause an Clinical Findings
cases, the tom fragment remains adherent to the
successful,! the danger of retinal detachment inflammatory response of the retina and choroid A. Symptoms and Signs: The common
external structure-not in its usual location al or
outweighs! the rijsk; of complications of central to the detached retina. that subsequently binds them together. sequence is for the symptoms of vitreous
prophylactic therapy. A. Cryothermy: A supercooled metal detachment to be followed minutes to years later
Retinal tissue may be tom completely free if cryoprobe is placed on the conjunctiva in an area by a “shadow” or “curtain” spreading across the
(he area ;pf vitreoretinal adhesion is small. Such 2. RETINAL HOLES corresponding to the borders of the retinal hole. field ofvision. When the detachment spreads very
tears are! generally round or oval and rarely exceed The instrument must have an adequate heat sink slowly, the patient may be unaware ofany problem
(WITHOUT AN OPERCULUM)
the diameteij ofthe disk. They are much less likely to propagate a dome of ice through the wall of the until the macula is affected.
(o lead to'retinal detachment than if the operculum eye. The surgeon observes and controls the On ophthalmoscopic examination, the
In addition to the atrophic holes associated
is still adherent to the retina. freezing which selectively destroys cells but detached retina bulges inward and is gently
with lattice degeneration andretinoschisis, about
. •«; • i
? ,
I
i 4% of people have small round nonop emulated leaves the fibrous structures intact. rippled or thrown into folds. It is translucent,
Relationship of Blo^d Vessels to RetinaL Tears B. Photocoagulation: Abright light focused obscuring the details of .the pigment epithelium
holes of unknown cause in the peripheral retina.
At. die time the retina is torn, capillaries are on the pigment epithelium will coagulate it and and choroid, and trembles with each movement of
These are usually close to the ora serrata and rarely
ofteii raptured’and the patient reports a shower the overlying retina. One may use a xenon arc or the eye. A careful search will almost always reveal
cause significant retinal detachment, though a tiny
of black> spots ’whicn represent shadows on the argon laser as the light source. This form oftherapy one or more holes. If the detachment is of long
self limiting detachment is often present.
maciiia caused by red blood cells in the retrovitreal is excellent for posterior lesions but is awkward to standing, the retina will usually be more
Blunt trauma can produce a peripheral retinal
spade. If a larger vessel is broken, the subvitreal infarct which, on resorption, may leave a large administer anterior to the equator. transparent and a characteristic demarcation line
bleeding:niay:be sp severe that the retina is round hole without an operculum. These holes C. Diathermy: The energy of a high- of pigment epithelial disturbance separates
obscured.1 Overnight bed rest with bilateral eye are prone to cause retinal detachment. frequency current, when applied to the sclera, is detached from normal retina.
patches; And elevation of the head will generally With the passage of time, the lips of a retinaL transformed to heat, which coagulates choroid and The other eye must be examined since it often
allojv the blcjod to settle enough to permit hole become white and rounded because of gliosis; retina. has retinal holes or vitreoretinal adhesions which
visualization of the retina. opercula slowly shrink; the subjacent pigment
86 87
 . light lead to tears. These should;be mapped recover to a remarkable degree eyen after several \ THE MACULA months of the onset of the disease. Most, of these
I I i • li■
carefully and treated prophyMctically by months of detachment. patients recover good vision. SoriietimdS
° <lite
in; ­
! » ? s(
photocoagulation or cryothcrmy. . detachment persists longer, increasing! t|j.c
likelihood of secondary cystoid degeneration-dr
•ifferential Diagnosis CENTRAL SEROUS DETACHMENTOF the macula. Permanent damage to the retina:ijj also
One must consider senile retinoschisis, THE RETINA more likely if there arc repeated episodes of central
r choroidal detachment, and malignant melanoma serous detachment. 1 N-’ ?
« • f the choroid. The retinoschisis cavity has a thin, If the pigment epithelial leakage^ persists
nmobilc inner wall. The honeycomb appearance This disorder occurs at any age past the early longer than 3 months and if itis nt
lot____
directly bcrteath
— and large breaks of the outer wall are teens and affects males more often than females. the fovea, photocoagulation is indicated.•'This
pathognomonic but are not always present. There Although it is usually monocular, both eyes may seals the leakage site and the subretinal fluid is
- iay be other areas of retinoschisis on the temporal, be affected either concurrently or sequentially. resorbed, with improvement in vision. Ajtnpugh
de of the same or fe! 1 ow eye. One or more small defects appear in the pigment central serous retinopathy appears to be d distinct
A detached choroid is not limited by the ora epithelial layer of an otherwise normal-appearing entity, it must be stressed that any condition
.^errata but is prevented from spreading where the fundus, and fluid leaks ffom the choriocapillaris damaging Bruch’s membrane ind the pigment pigthent
> ?rtex. vessels enter the sclera. The pigment to the subretinal space. This transudate is either epithelium may cause leakage ofserous fluid berieath
jithelhim is still visible, but the choroidal FIG 7-14. Retinal detachment 3 days after onset clear or partly opaque. The resulting smooth retinal the central retina. The differential diagnosis .thus
.... structures are noL with crescent-shaped retinal tear. detaclunent may extend up to 6 disk diameters in includes senile degeneration of Bruch’s membtan&
In malignant melanoma of the choroid, the circumference. angioid streaks, tumors such as choroidal angioma-
w . ibretinal fluid shifts on changing the patient’s nevus, and malignantmelanoma, falnilial’drusen,* high
:,.3sition. The tumor usually docs not myopia, and trauma. I | i i*.; .
transilluminate. and its position disregards both Very rarely, a pit of the optic nervejmay be
serrata and vortex vessels. associated with detachment in flie posteriprjpqlair
retina. In these;cases, there is no demonsiftible
reatment leak in the pigment epithelium, and it is ftibiight
Surgical repair is mandatory in the that the fluid may be cerebrospinal. \ ! ■
‘’■eatment of rhegmatogenous retinal
stachment since spontaneous reattachment is
i| i<:
i-.H /
.jtreraely rare. The first step is ophthalmoscopic i

examination of the entire retina and DETACHMENT OF PIGMENT EPITHELIUM

• ■'instruction of a detailed map showing each
jle and each area of vitreorefinal traction. The ! ; I i
■’ ’ djal is to seal the holes and prevent further Focal detachment of the pigment epithetmnf
holes from developing. By reducing the volume FIG 7-15. Retinal detaehmentand retinal tear 6 appears to be a manifestation of damage to Brudh’s'
the vitreous compartment and distorting it days after onset. membiane. It may develop in a nonrlal-iohkfng:
i various ways, vitreous traction is relieved. FIG 7-H7. Central serous retina! detachment. fundus or may be associated will the Conditions;
: retina is thus placed in contact with described above as sometimes pausing ’cqiitnil!
choroid, and the tissues are stimulated by The main symptom is blurred vision, and-as serous retinal detachment. Indeed, detachment.of
> / ’’athermy, cryothermy, or photocoagulation with most macular problems-exposure to bright the pigment epithelium and jcentijal serous,
i establish a permanent chorioretinal bond light makes this worse. Visual acuity is reduced to retinopathy are often concurrent, with one ofthem
-round the hole. a variable degree, and there often is a slight shift dominating the clinical picture, j i ! *’- ‘i
toward hyperopia. Ophthalmoscopic and slitlamp When the lesion is away ffom the matiidaj.
v; ~ourse & Prognosis examinations reveal a shallow retinal detachment the patient is usually asymptomatic. Even when
With care, 90% of retina! detachments can with loss of the foveal reflection. Intravenous the lesion is beneath the fovea, vision rpay be
repaired by one operation. ^Subsequent fluorescein demonstrates the site of leakage. surprisingly good. As viewed with.,the:
procedures salvage another 6%. If the retina is Because the fresh fluorescein-stained fluid is ophthalmoscope, the lesion is a sharply outlined^
‘ ill in place after 6 months, it is unlikely to become Warm, it rises by convection in the cooler low elevation of the pigment epithelium* which
etached again. preexisting fluid. The new fluid eventually masks the choroidal details. The ^litlaipp ieadityS
■ / ; The fovea may suffer irreparable damage in a becomes mixed with the old, and detectable confirms the blisterlike elevation’^ land
brief period of separation from its only blood fluorescence persists for about 30 minutes. retroilh-- nation
- Teveals the clarity of its contents
ipply, the choriocapillaris. By contrast, on FIG 7-16. Appearance of retinal In most cases the leak closes spontaneously, logethc • ith the commonly present! in-egufar-
^attaching the retina, extrafoveal function may days after onset. and the subretinal fluid is resorbed within 3 opaque' j on its anterior wall. i :
88 89
 Fluorescein’ promptly stains the cavity SENILEDEGENERATIONOF
DISCIFORM DEGENERATION characteristic pigment of malignant melanoma
contents,- which, though the pigment epithelium BRUCH’S MEMBRANE
OF THE MACULA appears. Unfortunately, eyes have been
may be quite opaque, shine brightly in contrast (Kuhnt-Junius Disease) enucleated on the basis of a clinical diagnosis • . '*•
with jthei rest of the fundus. This is probably
Irregular thickening of Bruch’s membrane ;‘-l malignant melanoma of the macular area whit
explained by the fact that the layer of fluorescein-
and associated sclerosis of the choriocapillaris .,‘1 upon pathologic examination showed only <•
stained fluid is mlich thicker here than elsewhere. Disciform macular degeneration is so named
are frequent findings in the posterior polar area in ;•* disci form degeneration. . .
If there is an associated serous detachment of the because of its discoid ophthalmoscopic
elderly patients. The overlying pigment epithelium No treatment is available. In foe juvenile typ . -
retinae the fluorescein may show a discrete leak in appearance. It is common in older people but may
is disturbed, especially in the macula and at the the vision may rarely return to normal in a matt .
the pigmpnt epithelium. occut in persons under 40 (juvenile type).
disk margin. Drusen - tiny discrete deposits • of weeks or months. In older people, there is
Pigment epithelial detachments either regress Pathologically, there is degeneration of the elastic
beneath the basement membrane of the pigment ~ usually complete loss of central vision, bvL
sponj^ndously, spread, result in atrophy of the membrane of Bruch, which permits extravasation
epithelium - may be present as further evidence of -u peripheral vision is maintained throughout lifi
pigm'dnt 'epithelium, or lead to neovascularization of serum, plasma, or whole blood between
damage to Bruch’s membrane.(Fig 7-18). Both eyes are seldom affected simultaneously, bi. .
beneath the pigment epithelium. Bruch’s membrane and the pigment
Photocoagulation may be indicated for the fellow eye eventually follows the same course
epithelium. If whole blood is extravasated, in the majority of cases.
lesiohs at a distance from the fovea. organization occurs and, after several weeks, a
!I
solidfibrotic mass involving foe retina and choroid
results. If serum is extravasated, as commonly
happens in foe juvenile type, resolution may occur ANGIOID STREAKS
:• NEW VESSELS BENEATH THE without fibrosis or permanent retinal damage.
1; | PIGMENT EPITHELIUM Central vision is blurred. In foe early stages
■’ i
I ■ there is an elevated red, solid-appearing mass in Linear breaks in foe fibroelastic layer of
h ’ : the macular area in one or sometimes both eyes. Bruch’s membrane look somewhat like blood •
New blood’ vessels may grow into the This later becomes an elevated white mass which vessels, for which reason they are called angioii
potential space between the mesodermal portion is usually round and fairly well circumscribed. The streaks. They arebrown irregularbands concentric
of Bniclf.’s membrane and the basement membrane optic nerve and the remainder of the retina are with the disk. Associated findings are
of the pigment epithelium. This happens mainly usually normal. In the juvenile type foe retina in pseudoxanthoma elasticum (Groenblad'
at thq posterior pole, and there usually is some the macular area is only slightly elevated and -Sirandberg syndrome), sickle cell disease, anc
recognisable predisposing cause such as senile appears somewhat edematous. Paget’s disease (osteitis deformans). From a
or hereditary degeneration ofBruch’s membrane, Malignant melanoma involving foe macula practical point of view, foe lacquer breaks ofhigh
detachment of the pigment epithelium, angioid may be difficult to differentiate from disciform, myopia and traumatic ruptures of Bruch’.f"
strcalcs, .traumatic rupture of Bruch’s membrane, degeneration. It is sometimes necessary to wait
FIG 7-18. Drusen in the macula. meir-brane can be grouped with angioid streaks.
or high myopia. The new vessels arise from tlie weeks or months to see if growth occurs or if the The pigment epithelium overlying all of these-
choiioidi and pass through defects in Bruch’s
merrjbrane or around its edge at the disk. They These changes get worse with time. In some
can-be rendered visible by fluorescein perfusion. cases, areas of the pigment epithelium may atrophy
• These vessels may cause serous or hemorrhagic completely; in others, serous detachment of the
detachment of the pigment epithelium, and the pigment epithelium luay develop; and in still others
subsequent scar tissue may continue to bleed and new vessels may develop with subsequent
leakfserous fluid. Blood will often leak beneath the bleeding. In the early stages-and, indeed, for many
retnyi, ai id on rare occasions it may rupture tlirough years - foe symptoms are minimal, with only a small
•foe retina into the vitreous. Overlyingthese so-called drop in visual acuity. However, the later
disciform hemonitages and scars, the retina suffers complications cause severe loss of central vision.
irreparable damage. If the lesion is central, the Although complications such as serous
associated scoloiqa will be devastating to fine visual detachment of the pigment epithelium and
pcrGbp(ion. However, these patients arc not subpigment epithelial neovascularization can
incapacitated because peripheral function usually sometimes be treated, there is no treatment for the
remains’ intact. They lose the ability to read but can primary changes in the capillaries and Bruch’s
ambulate easily in familiar surroundings. membrane. Even so, with optical aids, many of
; ‘IL is possible to close the new vessels by these patients can be helped to continue reading.
moderately heavy photocoagulation. However, if a Some younger patients with a family history
lesion is very close to the fovea, such therapy is not of drusen may show similar changes in the macular PIG 7-19. Quiescent disciform macular
area. FIG 7-20. Retinal photograph showing angioid
safe,; degeneration (drawing). streaks in retina.
90
91
 lesions slowly degenerates, and new vessels HEREDITARY DISEASES OF THE Color is dependent upon hue, saturation, and cone monochromatism is about 1:1,000,000.
sometimes grow through the defect in Bruch’s
MACULA ■’ brightness. Objects appear to have a particular (3) Rod monochromats: Rod; monochromatism
hue primarily because they reflect, irradiate, or is a very rare disorder in which there is complete jack
n mbrane to cause a disciform lesion.
In the absence ofneovascularization, angioid transmit light ofcertain wavelengths. The addition of cone function. It is always ^associated with
streaks are usually asymptomatic. If new vessels The hereditary diseases of the macula - al! of '■ of black to a given hue produces the various photophobia, nystagmus, and poor visual acuity.
which are untreatable - can be loosely divided ' shades. (4) Anomalous trichomats: Protaris haye
a- -s present and are close to the macula,
p itocoagulation may help. into those that involve primarily the f': Saturation is an index ofthe purity of a hue. similar but milder defects than occur in true
choriocapillaris and those that initially affect the • For example, scarlet is more saturated than pink protanopia; deutans have similar but mifder
pigment epithelium. because pin): is made up of red and white mixed defects than occur in true deuleranopia; and triians
Of the former group, central areolar choroidal ’? together. have similar but milder defects than occurpn'frue
MACULAR HOLE sclerosis is an autosomal dominant or recessive :• Brightness is that aspect of perception most tritanopia. >
disease leading to slow loss of central vision ia * closely related to light intensity. The common types of color blindness1; are
middle life. Abnormalities ofthe choriocapillaris Color blindness is a misleading term since inherited as X-linked characteristics. Adquued
and pigment epithelium are apparent early. By the 1 most color-blind people have normal visual acuity. causes of colorblindness include retinal disease
As in the retinal periphery, the parenchyma
time symptoms occur, there may be complete i According to Marriott, 8% of men and 0.4% of and poisoning. In the acquired cases, the patient
* g 1 internal limiting membrane at the avascular
atrophy of the central pigment epithelium. women interpret colors differently than tbe rest of may be color-blind in only one atea of the>vi feual
fu vea arc thin and prone to hole formation. These
Stargardt-Behr disease is usually transmitted mankind. They are classified as follows: field. J | ,ii
: -*■ atrophic macular holes are fairly common and can
f caused by prolonged macular edema or traction in an autosomal recessive manner. Commencing (1) Dichromats: Persons having bivariant Because of the obvious relationship to chojce
at different ages, the pigment epithelium slowly . rather than trivariant colorvision. They are divided of occupation, automobile driving, and general
■. 1 a preretinal membrane. In older patients, they
degenerates. The intensity of the pigment into 3 groups: Protanopes, or red-blind subjects, understanding of the world, everyone should
Judy develop spontaneously (Fig 7-21).
epithelial changes and the degree of visual loss are insensitive to deep red light. Deuteranopes have a color vision test early in life (eg, age 8-12).
The loss of vision is proportionate to the
vary from family to family. confuse shades of red, green, and yellow. However, the physician must be wary of attributing
1 ;e of the hole and the associated findings. If the
si ina is fl at, it is often difficult to tell ifthe macula In Best’s vitelliform macular degeneration, ’ Tritanopes are blue-blind subjects who confuse too much importance to a hereditary anomaly
there is a diffuse abnormality of the pigment . blue-and green shades and, generally, orange and which in its more common milder forms is little
is really perforated. However, one can be
epithelium but the visible changes are confined r pink shades. more than an occasional social inconvenience
reasonably confident of the diagnosis if the view
to the macula. Initially in this autosomal dominant (2) Cone monochromats: The incidence of Treatment is ofno value. |
; the underlying pigment epithelium is sharp, the
le margin is rounded and more opaque than the disease - though the vision is good - there are i ri
contiguous retina, and the background choroidal deposits in the pigment epithelium, giving the i ;1
fluorescence shows through clearly. appearance of a poached egg "sunny side up.” i
There is no effective treatment for atrophic Later, when the yolk ofthe egg appears scrambled,
»les. They very rarely lead to retinal detachment macular vision is seriously reduced.
‘ and should merely be watched. Photocoagulation
is seldom warranted.
COLOR VISIONS COLORBLINDNESS i<

Light may be defined as that portion of the

electromagnetic spectrum (400-700 nm) that readily i !s
stimulates human retinal receptors. To other 1 ii
species,"light” may encompass a different set of
i
wavelengths. I fr
The cones mediate color vision. In order to .
be stimulated, they require a greater intensity of t ?.
light than the rods. Thus, one cannot delect color . I
in moonlight. Each cone has one of 3 distinct I 'I
spectral sensitivity patterns. These curves are i ‘H
i
overlapping, with maxima at red, green, or blue. A I H
given rglit elicits different degrees of response in ; i$
each type of cone. This generates data which the
FIG 7-21. Atrophic macular hole. neural computer interprets as a specific color.

92 93
 the oxidized and reduced forms. The clinical degree of cataract formation,
There are no pain fibers, blood vessels, or assuming that no other eye disease is present, is
nerves in the lens. judged primarily by the visual acuity. Generally
physiology of Symptoms speaking, the decrease in visual acuity is directly
The only disorders of the lens are proportionate to the density of the cataract.
opacification and dislocation. Consequently, die However, some individuals who have clinically - >
patient with an opacity or dislocation of the lens significant cataracts when examined with the i
will complain of blurred vision without pain. The ophthalmoscope or slitlamp see well enough to
physician examines for diseases of the lens by carry ou with their normal activities. Others have
testing the visual acuity and by viewing the lens a decrease in visual acuity out of proportion to
with an ophthalmoscope, a hand flashlight, or a the degree of lens opacification. This is due to
slitlamp or loupe, preferably through a dilated distortion of the image by the partially opaque
anatomy & FUNCTION choroid forward and releasing the tension on the pupil. lens.
zonules. The elastic lens capsule then molds the?/
Cataract formation is characterized chemically
“ }Tbe lens is a biconvex, avascular, . olorless lens into a more spherical body with-7
by a reduction in oxygen uptake and an initial ?
and almost completely transparent structure, about correspondingly greater refractive power. The ?
increase in water content followed by dehydration. •-
4 nun thick and 9 mm in diameter. It is suspended physiologic interplay of the ciliary body, zonule, -J> CATARACT Sodium and calcium content is increased;
behind the iris by the zonule, which connects it apd lens which results in focusing near objects
potassium, ascorbic acid, and protein content is
witl| me ciliary body. Anterior to the lens is the upon the retina is known as accommodation. As /
the lens ages, its accommodative power is<^ decreased. Glutathione is not present in !
aqiibdiis; posterior to it, the vitreous. The lens A cataract is a lens opacity. Cataracts vary cataractous lenses. Attempts to accelerate or •
capsule (see below) is a semipermeabie membrane gradually reduced. 3 markedly in degree of density and may be due to retard these chemical changes by medical
(slightly more permeable than a capillary wall) a variety ofcauses but are usually associated with treatment have not been successful, and their '
wh'i'bh-will admit water and electrolytes. aging. Some degree of cataract formation is to be causes and implications are not known. ;
■i-J A subcapsular epithelium is present expected in persons over age 70. Most are bilateral,
anteriorly (Fig 8-1). The lens nucleus is harder although the rate of progression in each eye is
thahithe cortex. With age, subepithelial lamellar seldom equal. Traumatic cataract, congenital SENil E CATARACT
filScrsiare continuously produced, so that the lens cataract, and other types are less common.
gradually becomes larger and less elastic Cataractous lenses are characterized by lens Senile cataract (Figs 8-2, 8-3) is by far the
throughout life. The nucleus and cortex are made edema, protein alteration, necrosis, and disruption most common ty u. Progressively blurred vision
up pfHong concentric lamellas. The suture lines of the normal continuity of the lens fibers. In is the only symptom. Paradoxically, although
formed by the end-to-end joining ofthese lamellar general, lens edema varies directly with the stage distant vision is blurred in the incipient cataract
fib^rs^rre Y-shaped when viewed with the slitlamp. ofcataract development The immature (incipient) stage, near vision may be somewhat improved.
TfieY is erect anteriorly and inverted posteriorly. cataract is only slightly opaque. A completely Consequently, these patients lead better witlirait
' i ilEach lamellar fiber contains a flattened opaque mature (moderately advanced) glasses ("second sight”). The artificial myopia is
nucleus. These nuclei are evident microscopically cataractous lens is somewhat edematous. If the due to the greater ‘convexity of Ike lens in the
iliKuje peripheral portion of the lens near the water content is maximal and the lens capsule is incipient stage ofcataract formation. Glaucoma and
equator and are continuous with the subcapsular stretched, the cataract is called intumescent lens-induced uveitis are uncormnon complications.
epiuielium. (swollen). In the hypermature (far-advanced) Then f in medic' treatment for cataract. Lens
■ : The lens is held in place by a suspensory cataract, water has escaped from the lens, leaving extraction is indicated when visual impairment
li gament known as the zonule (zonule of Zinn). a relatively dehydrated, very opaque lens and a interferes with the patient’s normal activities.
Tlhs is composed of numerous fibrils which arise wrinkled capsule. Senile cataract is usually slowly progressive
FIG 8-1. Magnified view of lens showing^
Irdiplthe surface of the ciliary body and insert into Most cataracts are not visible to the casual over a period of years, and the patient frequently
termination of subcapsular epithelium ( vertical'j
th6 Hens equator. observer until they become dense enough (mature dies before surgery becomes necessary. If surgery
- ■! The sole function of the lens is to focus light section). or hypermature) to cause blindness. However, a is indicated, cataract extraction and an artificial
rays;upon the retina. Inorder to focus light from a cataract in its earliest stages of development can lens implantation definitely improves the visual
distant object, tire ciliary muscle relaxes, tautening Composition & be observed through a well dilated pupil with an
The lens consists of about 65% water, about” acuity in-well over 90% of cases. The remainder
t|ie zonular fibers and reducing the anteroposterior ophthalmoscope, loupe, or slitlamp. either have preexisting retinal damage (as optic
diameter of the lens to its minimal dimension; in 35% protein (the highest protein content of The ocular fundus becomes increasingly
tissue ofthe body), and a trace ofminerals common? nerve atrophy or macular degeneration) or develop
thjsi position the refractive power of the lens is more difficult to visualize as the lens opacity serious postsurgical complications such as
minimized, and parallel rays are thus focused upon to other body tissues. Potassium is mortk becomes denser, until the fundus reflection is
concentrated in the lens than in most tissues^ glaucoma, retinal detachment, vitreous
tjid:retina. In order to focus light from a near completely absent. At this stage the cataract is hemorrhage, infection that prevents significant
object, the ciliary muscle contracts, pulling the Ascorbic acid and glutathione arc present in both?
usually mature and the pupil may be white. visual improvement’
95
 not require surgery until the child is 10-15 years The cataract should be removed after the
of age. inflammation subsides and it is certain that no
' The visual prognosis for congenital cataract, further absorption of lens material is taking plate:
patients requiring surgery is not so good as that It should be noted that in people Under 20 ycAfc? ?
for patients with senile cataract. The complications of age the lens material in a traumatic cataract \yi|I- --
of the operation, associated amblyopia, and often absorb almost completely over a periodlofi
occasional associated anomalies ofthe optic nerve months without surgery. A thin membrane nidy]-‘
or retina lower the degree of useful vision remain, in which case discission (needling) nuiy ,i
obtainable in this group of patients. Probably no be necessary to improve vision.
more than 70% of operations for congenital
cataract result in significantly and permanently
FIG 8-3. Mature senile cataract viewed through a • increased visual acuity in the operated eye.
dilatated pupil. Rubella cataract has the worst visual prognosis.

TRAUMATIC CATARACT
CONGENITAL CATARACT
Traumatic cataract is most commonly due to
Congenital cataracts are common but may not
'Tess^frequen^causes include ; arrows, rocks,
cause significant visual loss. Most are, bilateral FIG 8-4. Congenital cataract
and are probably genetically, determined. .They contusions, overexposure to heat (“glass-blower’s
occasionally occur as a consequence of maternal cataract”), X-rays, and radioactive materials. Most
jubella during die first trimester ofpregnancy. Oiily"! traumatic cataracts are preventable. In industry',
those congenital cataracts that cause a marked : the best safety measure is a good pair of safety CATARACT SECONDARY TO j• | ‘
loss of vision are discussed here. goggles. INTRAOCULAR DISEASE
The mother notices that the child does not - The lens becomes white soon after the entry (“Complicated Cataract”)
see well during the first few months ot years of of the foreign body since the interruption of the
life. The pupil may' be white. The opacities vary tens capsule allows aqueous and sometimes Cataract may develop as a direct effect of ;:
greatly in density. vitreous to penetrate into the lens structure. The intraocular disease upon the physiology of the*
If thE cataracts are bilateral, and dense patient is often an industrial worker who gives a lens (eg, severe recurrent uveitis). The cataract
enough so that the retinas are not clearly visible, history of striking steel upon steel. A minute usually begins in the posterior subcapsular aijea/’-'
lens extraction by aspiration should be done in fragment of a steel hammer, for example, may pass and eventually involves the entire lens structure/ :: i
one eye by the age of 6 months to permit normal through the cornea and lens at a tremendous rate Intraocular diseases commonly associated witlv
development of vision and to prevent nystagmus. of speed and lodge in the vitreous where it can the development of cataracts are chron.icf!§iO
If surgery on the first eye has been successful, usually be seen with the ophthalmoscope. recurrent uveitis,, glaucoma, reti ni tis pigmentosg;) J
surgery on the second eye can be performed 2-3 The patient complains immediately of blurred and retinal detachment ]
months later. Otherwise, surgery on the second vision. The eye becomes red, the lens opaque, These cataracts are usually unilateral. T.he
eye should be delayed 1-2 years until the eye is and there may be an intraocular hemorrhage. If visual prognosis is not as good as in ordinary 2
larger and there is less risk of operative aqueous or vitreous escapes from the eye, the senile cataract. if
complications. eye becomes extremely soft. Complications
Months or years following surgery, vitreous include infection, uveitis, retinal detachment, and
strands may develop which upon contraction glaucoma.
produce retinal detachment. Retinal detachment The intraocular foreign body must be CATARACT ASSOCIATED WITH
may be successfully treated by surgery in more removed without delay. SYSTEMIC DISEASE
• FIG 8-2. Cataract types. (1) - Senile cataract, “coronary* ’ than half of such cases. Recent improved technics Antibiotics and corticosteroids should be
type: club-shaped peripheral opacities with clear central of aspiration have materially reduced the incidence given systemically and locally over a period of Bilateral cataracts may occur in association./
lens; slowly progressive. (2) - Senile cataract, “cuneiform” ofvitreous loss and therefore the late complication several days to minimize the chance of infection with the followiug systemic disorders/
type: peripheral spicules and central clear lens; slowly of retinal detachment. and uveitis. Atropine sulfate, 2%, 2 drops 3 times Hypopa. 'hyroidism, myotonic dystrophy, atopje' •
progressive. (3>-Scnile cataract, “morgagnian” type Most congenital cataracts are hot de daily, is recommended to keep the pupil dilated dermatii.A, galactosemia, and Lowe’s, Werner?^-
(hypermature lens'); tire entire lens is opaque, and the enough to blur the vision significantly and and to prevent the formation of posterior and Dov "'s syndromes.
• tens nucleus has fallen inferiorly. not progressive. Others progress slowly andr synechias.
96
97
 wVJ ?•

!
TOXIC CATARACT lens and the zonular fibers holding it in place caijS 9
be seen in the pupil. If the lens is completely??
i Toxic cataract is uncommon. Many cases
appeared in the 1930s as a result of ingestion of
dislocated into the vitreous it can be seen with-3’
the ophthalmoscope.
VITREOUS
L r
dimtrqph^nol, a drug taken to suppress appetite. A partially dislocated lens is often*
Otliief offenders are triparanol (MER/29) and complicated by cataract formation. If so, theL
corticosteroids administered over a long period cataract may have to be removed, but this should?.•
of It’has been suggested that echothiophate be delayed as long as possible because vitreous*?
iodide/ a strong miotic now being used in the loss, predisposing to subsequent retinal?-,
treatTn,?nl1of glaucoma, may cause cataracts. detachment, is prone to occur during surgery. If*
ffl
the lens is free in the vitreous it may lead in later' AN ATOMY & FU NOTION
life to the development of glaucoma of a type.?,
. j'1- which responds poorly to treatment. ' The vitreous is a clear, avascular, gelatinous
J' 1 f ul If dislocation is partial and the lens is clear, ? body which comprises two-thirds of the volume
[, Xfter-cataract is the term applied to the the visual prognosis is good. and weight of the eye. It fills the space bounded
poijiipn ofthe lens remaining after an extracapsular
by the lens, retina; and optic disk (Fig 9-1). Since
cauifapt extraction or a partially absorbed traumatic
raumatic Leris Dislocation.^. it is quite inelastic and impervious to cells and
cataract The opacity usually consists of capsular Partial or complete traumatic lens dislocation ? debris, it plays an important role in maintaining
and tqfeical material.
may occur following a contusion injury such as a; the transparency and form of the eye. If the
i i If vision is reduced, discission of the
blow to the eye with a fist. If the dislocation is-- vitreous were removed, the eye would collapse.
memBiarje is the treatment of choice. Since
discission is a simple, safe, and usually successfill partial, there may be no symptoms; but if the lens When the vitreous is replaced by saline, as in
is floating in the vitreous the patient has blurred certain forms of vitreous surgery, cellular matter
procedure, it may be performed when visual Joss
vision and usually a red eye. rridodonesis, a and particulate debris are free to migrate into the
is relatively minor (VA 20/50 to 20/60) if this loss
quivering of the iris when the patient moves his . optical pathway.
is a definite handicap to the patient.
eye. is a common sign of lens dislocation and is The outer surface of the vitreous-thc hyaloid FIG 9-1. Schematic cross-section of adult eye.
due to the lack of lens support. This is present -. membrane-is normally in contact with the
r ■' both in partially and completely dislocated lenses, . fallowing structures: the posterior lens capsule,
i. the vitreous with its- anterior portion attached to
f ;;
but is more marked in the latter. the zonular fibers, the pars plana epithelium, the
‘j •i j DISLOCATED LENS retina, and the optic nerve head. The base of the the posterior surface of the lens. This point of
(Ectopia Lentis) Iritis and glaucoma are common
vitreous maintains a firm attachment throughout attachment can be seen as a black dot (MittendorTs
complications of dislocated lens, particularly if
life to the pars plana epithelium and the retina dot) with the ophthalmoscope.
b dislocation is complete.
If there are no complications, dislocated immediately behind the ora serrata. Its attachment
_ ___________________________
« Partial _ _________
or complete lens dislocation may_ be
lenses are best left untreated. If uncontrollable to the lens capsule and the optic nerve head is
hefedjtari/ or may result from trauma. firm in early life but soon disappears. This is the COMPOSITION
glaucoma occurs, lens extraction must be done
principal reason that intracapsular cataract
Hereditary Lens Dislocation despite the poor results of this operation. The
extraction without vitreous prolapse or “loss” is The vitreous is about 99% water. The
. ^Hereditary lens dislocation is usually bilateral technic of choice is cryoextraction. In this
possible in adults and not in children. In addition, remaining 1% includes 2 components, collagen
an diniay be associated with coloboma of the lens, procedure the lens is touched by a supercooled
the vitreous is prone to vitreoretinal adhesions at and hyaluronic acid, which give it its specific
he rripeystinuria, Marfan’s syndrome, and metal probe. After a few seconds an ice ball forms physical chai ‘ ■ lur.
the site of lattice degeneration of the retina,
Mjarbliespnt’s syndrome. The vision is blurred, in the lens, firmly uniting the lens to the The vitreuus. owes its gel-like form and
i congenital retinal rosettes, meridional retinal folds,
particularly if the lens is dislocated out of the line instrument. The cataract is then removed by gentle consistency to a loose syncytium of long-chain
vitreoretinal scars, and new retinal blood vessels,
offyisionj. If dislocation is partial, the edge of the traction on the probe. collagen molecules each about 1.2 nm wide and
as in diabetes and central retinal vein occlusion.
The hyaloid canal (Cloquet’s canal), which in capable ofbinding about 200 times its own weight
the fetus contains the hyaloid artery, passes in water.
anteroposteriorly from the lens to the optic nerve The hyaluronic acid molecules are very large,
head. The hyaloid artery usually disappears soon loose skeins capable of binding about 60 times
after birth, but the hyaloid canal remains their weight in water. Alone in water, they create a
throughout life. It is not visible ophthalmoscopically. viscid structureless solution. Combined with the
A rudimentary portion of the hyaloid artery collagen element, they account for the physical
occasionally remains and can be seen floating in characteristics of normal vitreous.

99
98
 EXAMINATION OF THE VITREOUS process associated with breakdown of the gel and -• for hours, days, or even weeks. It is most readily Operculum
formation offluid-filled cavities, and a large variety f identified on moving the eye and when illumination
Normal vitreous is not visible by either- direct of collagen forms. It affects at least 65% of persons is dim or absent
.or indirect ophthalmoscopy. The numerous over 60 years of age. Myopes are especially • : Although this phenomenon is unilateral, a
ophthalmoscopically visible features are susceptible, even in childhood. "j. similar episode commonly occurs in the other visual
anomalies attributable either to structural changes The enlarging fluid-filled cavities coalesce V field. The 2 episodes may occur simultaneously but
such as lire “floaters” of syneresis and the r.ing- and may discharge abruptly into the potential .4 more commonly are separated by an interval ofdays
Hke form associated with posterior vitreous space anterior to the posterior retina. The patient •< to many years.
detachment or to invasive elements’ such as blood, may be aware of the collapse and detachment of The light represents a cerebral awareness of a
white cel! masses, or fibrovascular proliferations the vitreous as an acute condition. He most . new abnormal vi treous stimulation ofthe retina. It is
from adjacent tissues. Normal vitreous, in situ and commonly complains of“vitreous floaters,” often 4 most commonly associated with recent collapse and
- a variety of very important anomalies such as the with “flashing lights.” He may be eager to relate it detachment of the vitreous due to syneresis with
retraction, condensation, and shrinkage to trauma but should be assured that it is a focal vitreous traction on vitreoretinal lesions such
characteristic of diabetes, injury, etc can be viewed spontaneous occurrence. The mobility of the r as lattice degeneration, meridional folds, congenital
only with a slitlamp. Although slitlamp examination collapsed vitreous on eye movement and rosettes, and other visually subclinical vitreoretinal
FIG 9-3. Schematic representation of vitrebiis
- of the vitreous is quite easy to Learn and plays an consequent potential for stress on hitherto adhesions. A careful history will readily distinguish
collapse causing the retina to tearand dejtachi ‘
r important role in the management of vitreous subclinical vitreoretinal adhesions may also cause it from the scintillating scotoma ofmigraine, which is
characterized by a symmetric quivering scotoma in
i fli’
> <: i
disease, too few ophthalmologists arc at present vitreous hemorrhage, retinal tears, and retinal
*. - making optimal use of this instrument detachment. both eyes, predictable configuration and intermittent and require large eye motion orispecia
Slitlamps are microscopes with specialized progression, and variable nausea or headache. positions merely to see them. Unlike “flashing
: illuminating systems which make transparent and Flashing lights require no treatment The lights,” they are most readily seen against brigh
near-transparent ocular fluids and tissues visible. patient may be reassured that the symptom will lights or a uniform light background. They .are
The anterior central vitreous (the anterior pass. However, as the causative mechanism may extremely common in myopes and peopje With
vitreous) can be seen by slitlamp alone; the also induce retinal tears, retinal detachment (Fig 9- syneresis. j h--?
posterior vitreous can be seen only with the 3). or vitreous hemorrhage, every new case requires Red cells not uncommonly cause thi
' additional aid of a contact lens; and the peripheral a survey of the vitreoretinal relationship, especially symptoms as a result of small hemorrhagesfntc
: .... vitreous requires a specialized contact lens in the periphery. the vitreous due to retinal tears or hemorrhagic
equipped with mirrors for off axis illumination. diseases such as diabetic retinopathy
B-scan ultrasonography is an important hypertension, leukemia, old branch occlusion jo;’
diagnostic and prognostic tool in the management “VITREOUS FLOATERS” the retinal vein, Eales’ tlisease, Coats’ disease;:aht
of many posterior segment problems associated subacute bacterial endocarditis. | • 'j
; with gross vitreous opacification. Where x-ray A vitreous floater is a fine vitreous opacity White cell invasion of the vitreous get
technics arc useless and lightdependent which can stimulate the retina by casting a shadow associated with pars planitis may also cause “spots
ophthalmoscopes and slitlamps are of limited upon it The mind projects the corresponding dark before the eyes.” i ? fH
value, the skillful use of B-scan ultrasonography form onto the appropriate area of the visual field. Vitreous floaters due to pigment are usually a
can extract much pertinent information about the FIG 9-2. Schematic drawing of collapse and The term vitreous floaters embraces a common consequence of long-standing tear-inducyid
state of the vitreous and adjacent structures. For detachment ofthe vitreous. and potentially serious symptom (hat was formerly deLachment of the retina that has not yet reached
example, it is possible in this way to identify and called, muscae volitantes-Latin for flies that flit, the macula. • j isr |
lo.cate vitreous membranes, Witreoretinal flutter, or fly to and fro. Vitreous floaters should never be dismissed
‘ ' relationships and retinal detachments greater than The onset may be either insidious or acute as harmless or imaginary. A careful survey of-tlie
1 mm in depth, scleral ruptures, and intraocular “FLASHING LIGHTS” and unilateral or bilateral. The patient is aware of vitreous and retina is always indicated in brddr to
• foreign bodies-even nonlucent plastic and glass. one or more (or even many) fine, dark forms in his identify their nature and origin and to debide -Qi.
“Flashing lights” are a common symptom. field of vision. Their configuration is usually sb management Failure to make such an examination fio:
The patient is aware ofa localized “light,” “glow,” pronounced that he spontaneously classifies them infrequently leads to missed diagnosis. In the absence
AGING OF THE VTTREOUS “streak of light,” or “flashing” (as of a neon tube) as “spots,” “soot,” "particles,” “spiders,” of a serious causative pathologic process, flic patien:
“cobwebs,” “threads,” “worms,” “dark streaks,” may be reassured that his condition is harmless, fh .
in his field of vision for which he can find no
Like all types of gel, the vitrqous, with the reasonable explanation. He can usually point to “a ring,” etc. Various combinations are often
> passage of time, becomes increasingly prone to a the disturbance, and often describes an arc­ reported. The objects continue to migrate after the
degenerative process known as syneresis. shaped flicker in the periphery of one or 2 eye comes to rest-hence the name floaters. ASTEROID H YALOS1S !1 ' : •}

Syneresis usually starts in and most severely quadmnts. The light seldom persists for mere than Central,'relatively immobile floaters are ■ ■ f

visually annoying and may even be disabling. Asteroid • hyalosis is an uncominpil.

affects the anterior central area, sparing the base a fraction of a second. It frequently recurs al short
intervals for a few minutes and then disappears Peripheral ones are readily overlooked as they are condition which occurs in otherwise Healthy
’’ or anterior peripheral sector. It is a multifocal
100 101
eyes in elderly people. It is unilateral 3 times INJ URY TO THE VITREOUS Vitreous Loss VITREOUS HEMORRHAGE
mori^ often than pjilateral. Hundreds of small Vitreous loss is an iatrogenic complication.
ye(jpw spheres consisting of calcium soaps are Contusion The vitreous gel prolapses through a surgical Vitreous hemorrhage may vary from minimal
sceiii'in the ■vitreous. These move when the eyes Because the vitreous is inelastic compared t wound* usually at (but not limited to) the corneal bleeding recognizable to the patient merely as
move but always return to their original with the adjacent tissues, contusions which-, ’ limbus during the course of operating on the lens, '"vitreous floaters” to a dense mass of blood, loss u
positions because they are attached to abruptly though briefly alter the shape of the eye ■ - iris, or cornea. of the red reflection, and a sudden reduction of . .
interlacing fibers.! There are no related ocular or are apt to cause injuries where the vitreous Fibrous tissue invasion and contraction are vision to as low as "light perception only.” With
systdhiiqi disease?. The opacities have little or adherent. frequent sequels that are prone to cause traction moderate bleeding, the initial hemorrhage is often __
no; effect upon Vision and arc of no clinical Disinsertion of the vitreous base is not ?■ complications involving the retina. Comeal edema graphically described as one or more dense “black 4
significance. , uncommon. It is frequently associated with tearing -1 and iris displacement (eg, “updrawn pupil") may streaks” that subsequently "broke up into -
of the pars plana or retina, vitreous hemorrhage, also occur. An acute prolapse can be effectively
numerous minute black dots.”
or detachment of the retina-as long as 20 years •• excised. An old prolapse may require surgery for
synchVsis scintillans Fresh blood is readily identified by slitlamp •
later. -• release of vitreous traction.
Less commonly, “flashing lights,” ‘"vitreous T examination. Ophthalmoscopically, there is a
; '• t i?
Syiichysis scintillans is an uncommon, floaters,” and even vitreous hemorrhage or : variable loss of fundus detail and floating debris
usually bilateral Condition in which the examiner detachment of the retina may result from stress ■ VITREOUS INFLAMMATION which is often recognizably red. With time, there .
seCs numerous glistening white cholesterol crystals behind the vitreous base. The affected sites may is a marked tendency to syneresis, loss of color,
whicK tend to settle in the lowest part of very fluid be hitherto subclinical anomalous vitreoretinal : Vitreous inflammation includes a wide and clearing of the optical pathway. Blood staining
vitreous jyhen the byes are motionless. When tire adhesions (eg, lattice degeneration) or areas of spectrum of disorders ranging from a few scattered that persists for 6 or more months is apt to be
eyesore moved, the crystals spring up in great frank vitreoretinal disease such as diabetic i white cells to abscess formation. Most commonly, permanent and to cause retinal atrophy
showers and fly around the vitreous cavity until retinopathy. one or more focal inflammatory' lesions in the (hemosiderosis) and cataract formation.
movement of the eyes stops. choroid or retina-as in chorioretinitis or retinitis- Many conditions may cause the bleeding. .
I ‘Synchysis scintillans usually has its onset Rupture ofthe Globe are responsible for a secondary cellular invasion Traumatic breakdown of adjacent vascularized
before age 40. There is usually no predisposing Rupture ofthe globe is always aserious injury of the liquid vitreous or relatively resistant gel. tissues by contusion, concussion, penetration, ,
cause, but it may follow chronic uveitis. No which may result in early or late blindness or even There may be a mild localized blurring of the or rupture may occur. Systemic diseases dial
relationship has been established with elevated loss of the eyeball. Prolapse of the vitreous fundus landmarks and lesions which provoke little are apt to affect the retinal vessels-especially
blddi clfoleslerol levels or any other systemic through the wound is a severe complication often or no visual complaint except for a possible diabetes, but hypertension and leukemia also-
abnormality. associated with acute secondary tearing or “vitreous floater” effect. With greater infiltration, are common causes. Hemorrhage is often due ■ ■
, There is no blurring of vision, and die patient detachment of the retina. A seemingly vision is decreased and the fundus is invisible or to vitreous traction on the new vessels that
is unaware of his condition. The visual prognosis uncomplicated prolapse may be followed by late almost so. The condition may be so marked fhar appear at the edge of prior branch occlusions
is excellent, as the condition does not progress. retinal detachment with or without tears due to the red reflection is lost and the vitreous appears of the retinal vein. Acute collapse of tlie
fibrous ingrowth from the orbit and subsequent opaque and white. Since these conditions spare
I’ vitreous, which is extremely common, will ‘
contraction. The latter may be visible as the anterior segment, there is no pain and the
•’
sometimes tear the retina and cause a vitreous
membranes or bands in the vitreous. Various forms external eye appears normal. The prognosis and
MASSIVE VITREOUS RETRACTION of vitreous surgery are used to prevent or treat hemorrhage and even subsequent retinal
treatment depend upon the underlying condition.
l' i;: jl i
such complications. The vitreous usually clears when the primary detachment.
' ifylassivc vitreous retraction is a grave defect.is quiescent. Vitreous surgery is used to No patient with a vitreous hemorrhage should
vitre'ous disease of unknown cause. The central Penetration of the Globe remove gross residual opacities that show no sign be dismissed until a pertinent history has been
arcaibecomes a large fluid-filled cavity whose An almost endless variety of material may of clearing spontaneously. taken and a thorough examination performed-
walls' are condensed, shrunken, and rubbery. accidentally penetrate the globe. Common especially of the retina.
The [residual posterior vitreous is invaded at examples are needles, BB shot, and small particles Vitreous abscess (or endophthalmitis) is a rare, The outlook and management will depend
several points by scar-forming elements from of metal, stone, or plastic that fly into the eye at painful condition associated with photophobia as upon the primary condition. A persistent
the rptina. high velocity. well as redness and edema of the conjunctiva and substantial opacity may be removed surgically,
J kfhe’
: consciquent traction distorts, Prolapse of the vitreous may occur at the lids. It is a unilateral infection usually caused by often with dramatic improvement in vision.
detaches | and may tear the retina, causing gross site of entry or exit or both. The part traversed by Bacillus subtilis, a common barnyard contaminant.
visual inapairment.
_ Tear-induced detachments the foreign body is permanently damaged and is In most reported cases, the organism has been
complicated by this condition respond poorly often marked by visible condensation, shrinkage, introduced by a penetrating injury. The vitreous is yilREQUS^URGERY
to sfixgifcal repair since the tears are less or fibrous elements. an excellent culture medium for this organism, and
fayprhble for closure and the residual retinal Vitreous surgery is increasingly used to the abscess characteristically progresses rapidly to Vitreous disease was virtually incurable until
distortion and detachment compromise vision prevent or treat complications such as retinal destroy the eye despite injections of antibiotics into recent advances in surgical technic and equipment 1
poSlopcriitivcly. detachment with or without tears. the vitreous. In rare instances, vitreous abscess is made it possible to dissect and remove abnormal
. ’ 1 I!! i
lhe result of blood-bome infection. vitreous and to replace it with saline solution
102 103 f
ft).'
 distortion, of the macula or traction detachment of
without the gross disruption and degeneration
which formerly almost invariably followed such the retina due to “vitreous loss” at the time of ; :1O I
» t. attempts. Persons with poor vision due to vitreous anterior segment surgery, fibrous invasion ’/
opacities and vitreous traction have benefited most following rupture or penetration of the globe, and •
advanced vasoproliferative disease such as
ORBIT
. ; Defmitivevitreoussurgeiy is most commonly
performed with one ofa number of fine instruments
. . ..for engaging, cutting, and removing a train of
diabetes or Eales1 disease. Complicated
detachments associated with, retinal breaks may '
5j
also benefit. In addition, a variety of dense :
minute fragments of vitreous and debris. The
instrument can be inserted through the pars plana ptoliferative and vasoproliferative vitreous strands I
with relative impunity. and membranes may be divided readily, without •
The opacity may be blood-as in diabetes, bleeding, by electrodissection. This procedure is
branch vein occlusion, Eales* disease, trauma, or called electrovitreotomy. It is distinct from
muscles, and other nearby structures:;(ljljthe
..hyphema with extension into the vitreous shortly endodiathermy, which is also performed with an ANATOMY
superior and inferior orbital fissures (motor nerves
after lens removal-persistent inflammatory debris electrode inserted through the pars plana.
of the ocular muscles; ophthalmic nerve and vein);
. following uveitis, or an amyloid deposit Endo diathermy causes only coagulation as
(2) the supraorbital notch (supraorbital netyesurid
Other indications for vitreous surgery include opposed to cutting with coagulation. The bony orbits are the sockets containing vessels from the orbit); (3) the optic foramen (o^tje
the eyeballs and associated structures. The orbital
nerve); and (4) the infraorbital foramen (infihorpital
cavity is roughly similar in shape to a truncated artery and nerve). i j '
pyramid, being composed of 4 walls which
The orbits are related to the frontal sinus
converge on the apex posteriorly. The anterior
above, the maxillary sinus below- and the ethniqic!
opening is the base of the pyramid, the and sphenoid sinuses medially.! ? ijPi
circumference of which is the orbital margin or
The lacrimal gland is located laterallybeneath
rim. The greatest diameter of each orbit lies just
the overhang of the superior orbital margin. Thje
within the rim, the overhang serving to protect
supraorbital notch cuts across the tnargin
the eyeball from injury. The periosteum ofthe orbit
superiorly at the junction of its medial third-zLp'd
is called the periorbita. lateral two-thirds. This transmit^ the supraorbital
There are several openings within and
nerves and vessels from within the orbit, Asecond
adjacent to the orbits through which pass blood
or frontal notch is sometimes found medial toebe
vessels and nerves to supply the eyeball, its I » »r<
Supraorbital foramen

Orbital plate of Supraorbital notch

frontal bone
Lesser wing
Superior of sphenoid
fissure

Optic foramen
of sphenoid

Ethmoid bone
Orbital surface of
zygomatic bone

Lacrimal fossa
Inferior orbital
fissure i

Lacrimal bone
Infraorbital groove

Orbital surface
Infraorbital of maxilla

FIG 10—1. Anterior view of bonus of orbit.

104 105
supraorbital notch: This transmits the medial PHYSIOLOGY OF SYMPTOMS q. Unilateral Exophthalmos: Retraction of the upper lid gives an
froritdl branch of the nerve if it has divided within 1. Inflammatory-Cellulitis, pseudotumor oforbit, exaggerated impression of the degree of
the orbit. ' abscess, tenonitis, lacrimal gland exophthalmos in thyrotoxicosis. Malignant
> Tfie lateral orbital rim, or zygomatic process, Owing to the rigid bony structure ofthe orbit, inflammation, panophthalmitis, cavernous exophthalmos (also called exophthalmic
is the strongest part of the orbital rim. Suspensory with only an anterior opening for expansion, any sinus thrombosis (may become bilateral). ophthalmoplegia and hyperophthalmopathic
ligaments, the lateral palpebral tendon (ligament), increase in the orbital contents taking place to the 1 2. Vascular-Hemorrhage, traumatic or Graves’ disease) is a progressive and more severe
and check ligaments have their connective tissue side of or behind the eyeball will displace that spontaneous; varicosities, aneurysms. form which is often triggered by surgical or medical
attachments to the orbital tubercle in this area. organ. Pressure behind the eyeball will push it 3. Traumatic-Fracture, hemorrhage, rupture of treatment of thyrotoxicosis. Disorders of the
! The Vjolume of the orbit is about 30 ml in forward (proptosis). Pressure on one side will the extraocular muscles, emphysema from pituitary gland have been thought to cause the
adults. The average entrance dimensions arc displace the eyeball to the other side. sinuses, aneurysms. tissue changes which produce malignant
about35mpi high and 40 mm wide, although there With the change in position of the eyeball, 4. Tumors-Primary, from the eye or orbital exophthalmos. These changes consist of
are grphC?racial and. individual differences. especially if it takes place rapidly, there may be contents; spread from surrounding infiltration of the extraocular muscle tissue with
; Al decrease in growth of the orbit occurs if enough interference with the movement of the eye structures, metastatic. edema fluid and lymphocytes and similar changes
an ehuefeation is performed in childhood. This to cause dissociation of ocular movements and - 5. Cysts-Congenital dermoid, parasitic, in the rest of the orbital contents, forming a firm
maylcailse significant facial asymmetry. diplopia (double vision). Pain is absent unless mucocele from surrounding sinuses. mass which pushes,the eye forward.
■ • i
there is extreme swelling of the tissues or unless 6. Relaxation of retractors of eyeball, as with
Contents of the Orbit the eyelids are unable to protect the cornea paralysis of the extraocular muscles. Treatment
Tlie contents of the orbit are the eyeball, the adequately and there is irritation from exposure. 7. General disease-Leukemia lymphoma. Thyroid exophthalmos is often greatly
extracjqijiiaij muscles,i connective tissue fascia and improved as soon as the hyperthyroidism is
ligaipehjfsjfat, blood vessels, and nerves. The D. Bilateral Exophthalmos: brought under control. Malignant exophthalmos
eyeb'ajl’ticdupies only 20% of the orbital volume. 1. Endocrine - (Sometimes begins as unilateral presents a much more difficult therapeutic problem.
It is situated anteriorly in. the orbit just within the EXOPHTHALMOS exophthalmos.) Thyrotoxic (hyperthyroid, High doses of systemic corticosteroids are
rim and is surrounded by its extraocular muscles, (Proptosis) Graves’ disease), thyrotropic (malignant, frequently effective in reducing the amount of
fascial attachments, and Tenon’s capsule, with an ophthalmoplegic). proptosis. Retrobulbar and subconjunctival
extramdscular fat pad posteriorly. Anteriorly the 2. Pseudoexophthalmos-Congenital corticosteroids may be effective also. If
eyebjall lies just beneath the conjunctiva, with Etiology & Classification macrophthalmos, high myopia, lid retraction prominence of the eye becomes so great that the
which’ iffuses 3 mmlfrom. the limbus. Above the Exophthalmos may be due to any of the (Graves' disease).
eyeball^ jn the lacrimal fossa of the frontal bone following factors: (L) a space-occupying lesion in
superiorly and temporally, is the lacrimal gland. the rigid bony orbit, displacing the only moveable
The iblotjd vessels apd the nerves which supply tissue, the eyeball; (2) swelling of the retrobulbar
the e/eball, its muscles, and other nearby tissues through edema or hemorrhage, pushing
structures enter the; orbit through its posterior the eyeball forward; (3) relaxation ofthe retracting
openings and terminate in the structures within effect o f the extraocular muscles through paralysis Frontal
the drbfpjodtraverse the orbit to reach the surface or trauma; and (4) the apparent forward convolution
(supraorbital and infraorbital structures). displacement (pseudoexophthalmos) seen with lid
: A sheet of connective tissue fascia extending retraction (Graves’ disease) and large eye (myopia, Optic nerva
from tli^sorbital rim to the tissue of the lids in the macrophthalmos).
area; of-the tarsus forms an anterior limiting septum
membrane known as the orbital septum. Tenon’s A. Acute Exophthalmos:
capsule is;a connective tissue capsule which 1. Emphysema due to rupture ofthc medial orbital chamber
surrounds the eyeball. It is continuous with the wall, allowing air from the sinus to enter the
fascial Expansions of the muscle sheaths, which orbit Cornea Ophthalmic
likewise; expand peripherally to form check 2. Hemorrhage either traumatic or spontaneous. artery
ligaments for these muscles.
B. PulsatingExophthalmos:
1. Carotid-cavernous sinus fistula. Orbital
2. Vascular tumors or aneurysms. septum
3. Cerebral pulsations due to a defect in the
orbital roof.

FfG 10-2. Orbital anatomy (right lateral view).

106 107
lids can no longer protect the cornea, surgical ORBITAL PERIOSTITIS responsible, mostpatients can be saved with good
closure of the lids (tarsorrhaphy) must be visual recovery. Before the antibiotics became
performed to prevent exposure keratitis. Even this available, all patients died. i •J1 ;
is not possible in many instances, however, in Chronic infection of the periorbita may occur
which case decompression, of the orbit must be as a result of tuberculous or, less commonly,
performed using the transcranial, antral, or syphilitic invasion of the bones and tissues of
temporal approach. the orbit Both occur rarely since the antibiotics PSEUDOTUMPROfi-ORBIT: i;
Surgical exploration of the orbit is indicated came into use. Tuberculous periostitis tends to
in unilateral exophthalmos if there is a palpabLe involve the bones and tissues ofthe lateral orbital
mass which can be biopsicd; if there are X-ray rim; syphilitic periostitis, the superior orbital rim. Pseudotumor of the orbit |s an uncommon
changes of bone; or if progressive visual loss Symptoms consist of painless red swelling with inflammatory reaction, usually unilateral, which
occurs. If there is no palpable mass, no X-ray “cold” abscess in tuberculous periostitis; painful clinically resembles a neoplasmJExophthalmds is
changes, and no visual loss or comeal changes, swelling in syphilitic. a prominent clinical finding.'Many bacierja,
conservative management is indicated. Diplopia Treatment is with systemic antisyphilitic or viruses, parasites, and other possible causes ha-ve
alone is Dot a sufficient indication forexploration antituberculosis drugs. Tuberculous lesions been investigated, but the cause has ript bpep
of the orbit. onset is often sudden. More severe involvement should be drained. determined. i . I «!| j
may cause greater tenderness upon palpation and Inflammatory pseudotumor is characterized
more pain as well as headache on rotation of the by restriction of ocular movement, exophthalmos
eyeball. Occasional intraocular hemorrhage and with occasional lateral displacement, swelling pf
ENOPHTHALMOS inflammatory signs are probably due to CAVERNOUS SINJJS_THROMBpSIS the lids, and resistance to retrodisplacemenf pf
involvement of die vessels of the retina or choroid. ? the eye with finger pressure. Pain and diplopia are
Constitutional symptoms vary, according to the present in about one-half of cases. The onset Jis
Retraction of the eye into the orbit is a normal severity ofthe infection, from mild fever, malaise, Orbital signs and symptoms are usually usually gradual, and other signs iof inflammation
change in elderly people which is due to senile and leukocytosis to high fever and marked debility. ; associated with thrombosis of the cavernous such as are seen with the more acute prbitaj
atrophy of the orbital fat. Enophthalmos in early Infection may spread to the cavernous sinuses or ■ sinus within the skull. Exophthalmos with edema cellulitis aie not present. ; ! ' I |
life (before age 25) occurs rareLy as part ofHomer’s meninges. of the orbit and eyelids, diminished or absent The resemblance of pseudotuiftof to
syndrome or due to atrophy of the orbiLal fat Orbital cellulitis must be differentiated from ■ pupillary' reflexes, impaired visual acuity, and neoplasm may lead to exploratory orbital surged;
following a severe contusion of the eye. tenonitis, orbital periostitis, and cavernous sinus papilledema are usually present. Since the third, biopsy of the tissue reveals only signs of chronic
thrombosis In children, rhabdomyosarcoma must : fourth, and sixth cranial nerves and the ophthalmic inflammation. \ ' .
be ruled out. division of the fifth cranial nerve traverse the Treatment is often difficult. Anti-infective
Almost all cases respond well to large doses" cavernous sinus, involvement of these nerves chemotherapeutic and antibiotic drugs and X-ray
ofpenicillin. Hot compresses are useful to localize leads to paresis of the respective muscles and radiation have been tried without,effect Systemic
the inflammatory reaction. Unless the condition*, limitations of ocular movement. Fever is of the steroids in high doses have proved to be effective
INFLAMMATORY ORBITAL is growing steadily worse, surgical drainage should/ septic type. in many cases. Therapy may have to be continued
DISEASES be delayed until absolutely necessary. Thrombosis of the cavernous sinus is usual ly for weeks or months to prevent serious relapi^ If
If penicillin or other antibiotics fail to bring due to infection spreading along the venous the swollen, chronically inflamed tissues impinge
the condition under control after a vigorous trial channels which drain the orbit, central face, throat, upon the optic nerve, there may be permapfept
of 2-3 days, surgical drainage must be employed. and nasal cavities. damage to the optic nerve. Corticosteroid therapy
ORBITAL CELLULITIS The safest method is to make an incision into the Differentiation from orbital, cellulitis is decreases the chances of optic atrophy. ; :
area of greatest fluctuation-avoiding, if possible/ sometimes necessary. Cavernous sinus
the areas of the trochlea of the superior oblique thrombosis may be bilateral, whereas orbital
Orb ita 1 9eI hdirix-»g usually caused by muscle and the lacrimal gland recess. Antibiotics cellulitis is almost always unilateral. In cellulitis
should be continued until the infection clears. the pupillary reflexes remain normal, there is no
same organisms that cause acute sinusitis. They The response to penicillin is generally good papilledema, and pain and tenderness are more
If not, the infection may localize anteriorly and severe.
enter the orbit by direct extension or through the
rupture or require drainage; or may extend Massive doses of systemic antibiotics are
vascular channels between the orbital contents
and infected ethmoidal, sphenoidal, maxillary, or posteriorly, causing cavernous sinus thrombosis, necessary. Prophylactic chemotherapy and
meningitis, or brain abscess. Optic neuritis with avoidance of manipulation of pyogenic infections
frontal sinuses.
secondary atrophy may follow severe which may drain into the cavernous sinus are of
Swelling ancLxedness nf. Jhp
inflammatory reactions. The visual prognosis B the greatest importance.
chemosis. exophthalmos of varying degrees, and
dull pain are usually present in mild cases. The excellent in the absence of complications. Since the pyogenic bacteria are usually

109
acute
SinusU is
 • '• ■ '
a
Sensory impulses are received by the rods the bipolar cells.: The bipolar cells synapse with
and cones of the retina, which may be considered
■ OPHTHALMOLOGY / the special end organ. The nuclei of the rods and
the ganglion cells of the retina whose axons form
the nerve fiber layer, which collects to form the
cones are in the outer nucleaT layer. The dendrites optic nerve.
synapse with the second neuron of this pathway,

Normal blind

i -
: ' \ ? I

The eyes are intimately related to the brain the eye. The orbital portion of the nerve is 25-30/
and*frequently give important diagnostic clues to mm long and travels, within the muscle cone to’- (I) Lemon in left superior tem­
(2) Total blindness, rl&ht eye.
GbTSkliaorders. Indeed, the optic nerve is a part of enter the bony optic foramen to gain access to/ poral retina causes a corre­
Complete lesion of right
sponding field defect in the Left Right
the CNS. Intracranial disease frequently causes the cranial cavity. The intraosseous portion/ visual visual optic nerve.
left inferior nasal visual
Visual ‘disturbances because of destruction of or measures 4-9 mm. After a 10 mm intracranial course
pressure upon some portion of the optic the nerve joins the opposite optic nerve to form*
pathways. Cranial nerves III, IV, and VI, which the optic chiasm and continues posteriorly to the/
control ocular movements, may be involved, and lateral geniculate bodies. Here synapse occurs on
nerv^$ V and VII are also intimately associated neurons whose axons tenninate in the primary
y^ilh ocular function. visual cortex ofthe occipital lobes. The nerve libers
become myelinated upon leaving the eye and arc
'• * i supported by neuroglia; this increases the Left nasal
diameter from 1.5 mm (within the sclera) to 3 mm: rat na
atomy of the Gptic Nerve (Fig 11-1)
(3) Chiasmal Hon causes bi­
; ■ The optic nerve is a trunk consisting of about (within the orbit). Eighty percent of the nerve is temporal hemianopsia.
(4) Left nasal hemianopsia due
to a kaion Involving the
;j irqillidn axons arising from the ganglion cells of made up of visual fibers en route to synapse in outer aspect of one tide of
die retina. These axons comprise the nerve fiber the lateral geniculate body. Twenty percent are temporal the chiasm (pressure on the
layer of the retina and converge to form the optic pupillary; these bypass the lateral geniculate body Left optic nerve retina optic nerve from aneurysm
of left internal canoiitl ar­
•nefycJ The nerve emerges from the back of the en route to the pretectal area. Since the ganglion tery).
Right optic
globe through a short, circular opening (0.7 mm cells of the retina and their axons which make up
•long, l.$5 mm in diameter) in the sclera situated I the optic nerve are actually extensions of the CNS, Lateral geniculate
Genicul Hcarine
mm below and 3 mm nasal to the posterior pole of they have no capacity to regenerate if severed. tract
■. « • i 5 '
■ i i 8

space
Central retinal vein

00
Subarachnoid

Dura
(5) Rijht homonymous hemi­
(6) Right homonymuat inferior
anopsia duo to a lesion of

M
L quadrantanopsia due to In­
the left optic tract (least
volvement of optic radia­
Arachnoid common site of hemianop­
sia). tions (upper left optic radia­
Central retinal tion in this case).
artery

(7) Right homonymous html-

Nerve bundles anopxui due to^a lesion- of
divided by septa the left occipital Iobt. The
pupillary light reflex is not
unpaired.
FIG 11-1. Cross section of the optic nerve.
110 FIG 11-2. The visual system; topographic diagnosis.
 demyelinization of the optic nene. A wide variety neutrophils, in the affected area. The nerve jitters. ,
of diseases can cause optic neuritis (see below). are swollen and fragmented! Fat-bearing;11
Retina
Loss of vision is the cardinal symptom and serves macrophages soon appear, carrying ay/ay<
to differentiate optic neuritis from papilledema, degenerated myelin material. As the process
which it may simulate ophthalmoscopically. becomes more chronic, lymphocytes and p.lasipa:
The term “optic neuritis” also includes ceils predominate. In mild attacks Of optic neuritis, «.
Optic foramen retrobulbar neuritis, a form in which the nerve is the nerve fibers may be preserved with a minimum: •;
affected posteriorly sd that there are no visible amount of scar tissue formatioii. When jnerve:,
ophthalmoscopic changes of the nerve head tissue is permanently destroyed,,fibrous gliosis'i’
unless optic atrophy appears. replaces the nerve elements. • . '•’ ‘ ’ •

Etiologic Classification Clinical Findings ?. r ’’ • C

There is usually a temporary?but severe loss ■;
A. Demyelinating Diseases: of vision. There may be pain in tile region of tltel ,
I . Multiple sclerosis. eye, especially upon movement of the globe J •!
2. Post-infectious encephalomyelitis. Vision characteristically improves dramatically' I
plexus
3. Other rare demyelinating syndromes, within 2-3 weeks. i ’ ‘ ;
neuromyelitis optica (Devic's disease), Central scotomas are the inost coinhjon;
diffuse periaxial encephalitis (Schilder's visual field defect. They ace usually circular, |
Central artery disease). varying widely in size and density. Almost-any:
unilateral field change is possible. The pupil reacts If’
FIG 11-3. Schematic drawing showing optic nerve and central retinal artery. B. Systemic Infections: sluggishly to light. : . . .
1. Viral-Poliomyelitis, influenza, mumps, measles. Ophthalmoscopically, hyperemia of the optic:.
The dura consists of tough, fibrous, relatively 2. Bacterial-Pneumonia and, rarely, other disk and distention of large veins 5a re early signs.’
•heaths of the Optic Nerve bacterial infections.
avascular tissue fined by endothelium on the inner Blurred disk margins and filling ofjthc physiologic' I
The fibrous wrappings which ensheath the surface. cup are common. The process may advance to;
optic nerve are continuous with the meninges.
The subdural space is between the dura and C. Nutritional and Metabolic: marked edema of the nerve head - but elevations . (|
, ‘'he pia mater cs loosely attached about the nerve 1. Diabetes mellitus.
arachnoid; the subarachnoid space is between the of more than 3 diopters (1 min) are unUstiaL ,i
jear the chiasm and only fora short distance within 2. Pernicious anemia. Extensive surrounding retinal edema mny’.bci1!
pia and arachnoid. Both are more potential than
the cranium, but it is closely attached around most 3. Malignant disease.
actual spaces under normal conditions, but are present. Hemorrhages may occur in the nerve fiber’ !|
> nf the intracranial and all of the intraorbital 4. Hyperthyroidism.
direct continuations of their corresponding layer near the optic disk, and arc usually flariie-!
s portions. The pia consists of some fibrous tissue
intracranial spaces. Subarachnoid or subdural, shaped. • 1 G ■ !?
_ yith numerous small blood vessels. It divides the fluid under sufficient pressure will fill these D. Leber’s Disease: Hereditary atrophy of the
. nerve fibers into bundles by sending numerous optic nerve.
potential spaces about the optic nerve. The
: nepta into the nerve substance. The pia continues
meningeal layers are adherent to each other and
o the sclera, with a few fibers running into the E. Local Extension ofInflammatory Disease:
to the optic nerve and the surrounding bone within
.. thoroid and lamina cribrosa. 1. Sinusitis.
the optic foramen, making the optic nerve resistant
The arachnoid comes in contact with the 2. Meningitis (purulent, tuberculous, syphilitic).
to traction from either end.
7ptic nerve at the intracranial end of the optic 3. Orbital inflammation.
oranien and accompanies the nerve to the globe,
4. Intraocular (chorioretinitis).
. where it ends in the sclera and overlying dura. 5. Syphilis.
This membrane is a diaphanous connective tissue
membrane with many septate connections with E Toxic Amblyopias: Tobacco, methanol, quinine,
DISEASES OF THE OPTIC
he pia mater, which it closely resembles. It i s more arsenic, salicylates, lead,
NERVE
4 v intimately associated with pia than with dura.
The dura mater lining the inner surface of G.Othcr Causes: Optic neuritis may also be a I
• - 'he cranial vault comes in contact with the optic feature of blood dyscrasias, trauma, and ischemia. FIG 11-4. Optic neuritis.
iervc as it leaves the optic foramen. As the nerve
OPITIC NEURITIS
inters the orbit from the optic foramen, the dura Pathology’ Differci-,: ml Diagnosis J * > ? ■ i-
splits, one layer (the periorbita) lining the orbital In the early stages of optic neuritis there is an Pt"ledema is the most common diffeienjialj
• cavity and the other forming the outer dural outpouring of white blood cells, predominantly diagnose problem. In papilledema there i^dften-
Optic neuritis (papillitis) is a broad term
covering of the optic nene. The; dura becomes
denoting in P umma lion, degeneration, or
continuous with the outer two-thirds of the sclera. 113
112
 •LteJ elctation of the optic nerve head» nearlY neuritis is multiple sclerosis. A diagnosis of Diplopia is a frequent early symptom of
multiple sclerosis is eventually made in 25-50% of
PAPILLEDEMA & OPTIC ATROPHY
^'C trit visual acuitX» norma* pupillary response extraocular muscle involvement, due most
ll°p'W' Bssjociated infereased intracranial pressure, patients between 20 and 45 years of age who have : frequently to intemuclear ophthalmoplegia. This
^nd ho visual field defect except an enlarged blind an attack of retrobulbar neuritis. Other causes are '.if condition, caused by a lesion of the medial
spo^' unless the visual pathway has been late neurosyphilis, toxic amblyopias, other <•. longitudinal fasciculus, is characterized by paresis
demyelinating diseases, Leber’s optic atrophy, £ PAPILLEDEMA
interrupted intracranially. Despite these obvious of one medial rectus muscle on conjugate lateral
diabetes meUitus, and vitamin deficiency. If the ? (Choked Disk)
differences’, differential diagnosis continues to be gaze to the opposite side while medial rectus
a problem because of the similarity of the process is sufficiently destructive, a retrograde function is normal for convergence. Weakness of
ophthalmoscopic findings. optic atrophy results. The disk loses its norma! /■ one medial rectus muscle orptosis may also occur;
pink color and becomes pale. In extreme cases a Papilledema is a noninflammatory
less commonly, weakness of the lateral rectus or congestion of the optic disk associated with
chalky- white disk with sharp outlines in a blind
Treatment other muscles, singly or together, also occurs. increased intracranial pressure. Papilledema will
Ideally, treatment is directed toward the eye results.
occur in any condition causing persistent
underlying'cause. Ifthe cause cannot be effectively
increased intracranial pressure; the most
ireafed or is not known, treatment is often common causes are cerebral tumors, abscesses,
unsatisfactory. Systemic corticosteroids have NEUROMYEI, ITIS OPTICA subdural hematoma, hydrocephalus, and
been reported to be help till in persistent optic (Devic’s Disease)
DEMYELINATING DISEASES malignant hypertension.An important factor in
neuritis due to any cause, but many.patients fail
the mechanism of papilledema is.obstructionto
to respond! Since the tendency is in the direction
the venous flow caused by pressure on the
of i improvement, many drugs have been reported TTi is rare demyelinating disease of the CNS central retinal vein where it leaves the optic
to be “successful” in 'the treatment of this disorder. (considered by many to be a form of multiple nerve and passes through the subarachnoid and
MULTIPLE SCLEROSIS sclerosis) is characterized by bilateral optic neuritis subdural spaces.
Course & (Prognosis and paraplegia. The causers not known. There is Edema of the optic nerve is the principal
Lpss of vision occurs within the first few usually a sudden onset of blindness in one eye, pathologic finding. Edema may be present in the
hours alter onset arid is maximal within several Multiple sclerosis is a chronic, relapsing Z followed soon by blindness in the other eye and adjacent nerve fiber layer of the retina. Subhyaloid
days! Visual acuity usually begins to improve 2-3 disorder of the CNS with a tendency to involve :?.) paraplegia. There is only a moderate tendency to hemorrhages and hemorrhages in the nerve fiber
weeks?after onset,’ and sometimes returns to the optic nerves and chiasm, brain stem, cerebellar i? recovery of both elements of the disease. The layers are common. Inflammatory signs are minimal,
normal in a few days. Improvement may continue peduncles, and spinal cord, although no part of i < mortality rate is 50% in some scries. There is no and leukocytes are seen histologically only in the
slowly over a period of several months. The the CNS is invulnerable. Pathologically, multiple treatment later stages. Degeneration of nerve fibers may
appearance of optic atrophy indicates some areas of irregular patches of demyelinization are - *
eventually occur.
permanent destruction of nerve fibers with present in the white matter. Early, there is ;
permanent loss of function. Optic neuritis degeneration of the myelin sheaths and a relative
Clinical Findings
assqbihtcd with systemic or local inflammatory' sparing of the axons. Glial tissue overgrowth and DIFFUSE PERIAXIAL ENCEPHALITIS The blind spot is enlarged. Visual acuity and
disease or of unknown cause does not usually complete nerve fiber destruction, with some round ? (Schilder’s Disease) visual fields are otherwise normal. Early
recur. Optic neuritis in demyelinating disease has cell infiltration are seen later. The disease has a .
ophthalmoscopic findings include hyperemia of
a fdvqrable prognosis without treatment for an predilection for the optic nerve and chiasm. ?
the disk, blurring of the disk margins, and
individual attack, £but over a period of years Nystagmus is a common early sign and, unlike?, This rare disease of young children is distention of retinal veins. It is difficult to be sure
significant visual loss is the rule since permanent most manifestations of the disease (which tend: • ■ characterized pathologically by widespread about early papilledema. One helpful sign is the
damage results from recurrent attacks. toward remission), it is often permanent (70%). T demyelinization of the white matter in the brain. absence of pulsation of the central retinal vein or
Optic neuritis (especially retrobulbar? Clinically there is an acute onset of rapidly failure to produce a pulsation with light digital
neuritis), characterized by acute unilateral loss of Z developing cortical blindness, mental deterioration, pressure on the globe. Frank (measurable) swelling
vision with a tendency toward recovery, is a? convulsions, bilateral spastic weakness, and of the disk, peripapillary retinal edema, and
: j RETROBULBAR NEURITIS frequent initial symptom of multiple sclerosis. The Z paralysis progressing to coma and death. hemorrhages radially about the disk in the nerve
other eye is often involved eventually. With each'? Cortical blindness is due to bilateral fiber layers appear later. Papilledema may elevate
attack there may be some residua] permanentfr widespread destruction of the visual cortex. A the disk to 6 or 10 diopters, and hemorrhages in
• Retrobulbar rieuritis is optic neuritis which damage (eg, loss of visual acuity or defective color *’ striking feature of cortical blindness may be the
severe cases are subhyaloid, occasionally
ocqinjs far enough-behind the optic disk so that vision). Abnormal electroretinographic findings? patient’s subjective unawareness of his disability breaking into the vitreous and markedly affecting
no parly [changes [of the optic disk arc visible often parallel the optic nerve damage. ri (Anton’s syndrome). Despite being totally blind, vision.
opfithalmoscopicajly. Visual acuity is markedly Because of the tendency toward selective? the patient believes that he can see.
reduced.! (“The patient sees nothing and the involvement of the papillomacular bundle within
doctor sdcs nothirig.”) • the optic nerve, central scotoma is by far the most-?
The? most frequent cause of retrobulbar common visual field defect during the acute stage.

114 115
 OPTIC NERVE ATROPHY H. Traumatic: Direct injury to a nerve (ie, severing, GENETICALLY DETERM INED OPTIC
avulsion, or contusion). ATROPHY i
< 1
Etiologic Classification I, Glaucomatous. j ;i
Leber’s Disease <
A. Vascular: Occlusion of the central retinal vein This rare disease, characterized by bilateral,
or artery; arteriosclerotic changes within die optic Clinical Findings rapidly progressive optic atrophy, occurs in young
nerve itself, disturbing its normal nutrition; or Loss ofvision is the only symptom. Pallor of men age 20-30 (very rarely in women), it ‘has
posthemorrhagic, due to sudden massive blood die optic disk and loss of pupillary reaction are classically been considered to be due to an>X-
loss (eg, bleeding peptic ulcer, traumatic usually proportionate to visual loss. linked recessive gene, but its real moder of
amputation). inheritance must still be considered to be in doubt.
Vision is not totally lost. There is no known
B. Degenerative: Consecutive atrophy secondaiy Treatment, Course, & Prognosis treatment.
to retinal disease, with destruction of ganglion It is rarely possible to treat the underlying i
cellsfeg, retinitis); or as part of a systemic cause effectively. Changes in visual function ; I ■
. . . . ! I ’*
degenerative disease (eg, cerebromacular occur very slowly over weeks or months. It is Congenital or Infantile Hereditary Optic Atrophy
FIG. 11-5. PapDled ema. Moderate disk elevation degeneration). difficult to assess prognosis on the basis of This occurs in. a severe autosomal recessive
without hemorrhages. >. ’ ophthalmoscopic findings alone. Atrophic form and a milder autosomal dominant ijorm. The
C. Secondary to Papilledema cupping, attenuation and reduced number of recessive form is present at birth or witlup 2 yelirs,
Complications vessels on the disk, and pallor with papilledema and is accompanied by nystagmus. The more
Papilledema may persist for a long time D. Secondary to Optic Neuritis (Including are unfavorable prognostic signs. Optic atrophy common dominant form has an insidious onset in
without permanently affecting vision, or Retrobulbar Neuritis) secondary to vascular, traumatic, degenerative, childhood, with little progress ion thereafter. There
secondary optic atrophy may occur as a and some toxic causes usually has a very bad is characteristically a centrocecal scotoma' wjtli
complication. Following reduction of increased E. Pressure Against the Optic Nerve: Aneurysm prognosis. Visual loss due to optic atrophy variable loss of central visual acuity7. i ■
intracranial pressure, papilledema improves of the anterior circle of Willis, bony pressure at secondary to pressure against the optic nerve may
rapidly. Hemorrhages, exudates, audretinal edema the optic foramen (eg, osteitis deformans), be restored, particularly if the cause is relieved
usually clear promptly. If optic atrophy does follow, intTaorbital or intracranial tumors, adhesive early. Behr’s Hereditary Optic Atrophy
slight to total permanent loss of vision results. constricting basal arachnoiditis. This rare autosomal recessive disease: is
characterized by (1) bilateral optic atrophy, rfcr&ly
Treatment F. Toxic: End result of toxic amblyopia complete; and (2) associated neurologic:findings
Treatment depends upon the underlying such as mild ataxia, a positive Babinski jsi^n,
cause. Papilledema associated with hypertensive G. Metabolic Diseases: Eg, diabetes mellitus, clubfoot, mental deficiency, nystagmus, and ‘otiier
retinopathy is an indication for vigorous treatment ganglioside disease. findings, at times with generalized slpw
with potent hypotensive drugs. progression to a static condition >byi lile
Caution: Although it is at times undertaken adolescence. There is no known treatment.• $
as a calculated risk with proper precautions, lumbar
puncture is usually contraindicated in patients
with papilledema because of the danger of
herniation of the brain into the tentorial incisure
or into the foramen magnum. Such herniation DISEASES OF
causes pressure particularly on the medulla and THE OPTIC CHIASM
can cause sudden death. I
Course & Prognosis
In general, the more rapid the onset, the FIG 11-7. Myelinated nerve fibers adjacent to Anatomy (Fig 11-8)
greater the danger of permanent visual loss. optic nerve head. Depending upon the size and The optic chiasm is variably situated in ear
Papilledema of more than 5 diopters, extensive distribution, they may be mistaken for retinal
the top of the diaphragm of the sella turcica (jnpst
retinal hemorrhages and exudates, and macular exudates, papilledema, or optic neuritis. often posteriorly, projecting io ver thejdorstim
stars imply a poor visual prognosis. Early pallor sellacj and usually separated from it b^ several
of the disk and attenuated arterioles, once the mm ->f subarachnoid space. Above, the lamina
edema of the nerve head clears, indicates that FIG 11-6. Optic atrophy.Note avascular white disk
term^alis forms the anterior wall of the third
some optic atrophy will follow. and avascu r network in surrounding retina.
116 117
ventricle'’ |The internal carotid arteries lie just Progression of such a lesion will then cause loss W
laterally, adjacent to the cavernous sinuses. The of the inferior temporal field, inferior nasal field f LEFT EYE RIGHT EYE
chiasm is jna<lc up of the junction of the 2 optic and finally the superior nasal field.
nerves arid provides for crossing of the nasal 30
fiber£ to the opposite optic iract and the passage Lesions Anterior & Inferior to the Chiasm
offcijipoi&I fibers to the ipsilateral optic tract. The (Common)
inactilar fibers arc arranged similarly to the rest of Pituitary tumors, meningiomas, meningitis, V: (1) Central visual fields of typi­
cal lesion interior and infe­
the. fibers except that their decussation is farther and arachnoiditis are most commonly responsible. ’•\ rior to chiasm with com­
posteriorly. In general, lesions of the chiasm cause Superior bitemporal quadrants are affected with pression of right optic
bitemporal hemianopsic defects. on e or both optic nerves involved, causin g central : nerve.
scotoma due to involvement of the papillomacular
bundle (the most susceptible fibers).

LESIONS AFFECTING THE OPTIC VA = 20/20 VA « 20/400

■: • CHIASM Lesions Posterior to the Chiasm (Uncommon)
. i i
These are due to suprasellar tumors, Rathke
" 'j \
pouch tumors, and tumors of the third ventricle.
Lesions Posterior, & Inferior to the Chiasm Increased intracranial pressure may be present,
intrasellar tuinors such as pituitary tumors and X-rays often reveal suprasellar calcification • (2) Central visual fields of a
lesion Hnterior and superior
(s£e'belohv) are; the most common causes. and destruction ofthe sella. The visual fields show ■ to the chiasm (affecting
Papilledema is hare, and destruction and bitemporal central scotomas due to compression right side predominantly).
enlargement of the sella turcica is seen by X-ray. of the posteriorly placed crossing macular fibers.
Visual fields reveal superior bitemporal field Since the nasal field (uncrossed) macular fibers
involvement with or without macular loss. are preserved, good visual acuity remains.

VA «= 20/20 VA-20/400
Olfactory tract
30

ji Frontal lobe lobe

i!
*! 1 (3) Central visual fields of pro­
gressive loaion inferior to
i Anterior perforated chiasm.
- substance

“I
;’ optic

V A = 20/20 VA «= 20/400

cinereum

(4) Central visual fields of a le­

bjA’am miliary sion posterior to the chiasm
body causing bitemporal hemian-
perforated substance opsic scotomas,
Fodr-th

Third nerve VA = 20/20

FIG 11-9. Typical examples of visual field loss.

FIG 11-8. Relationship of optic chiasm from inferior aspect
118 119
 Lesions Anterior & Superior to the Chiasm in many instances. Visual loss or endocrine^ evacuation of the cystic contents and removal of from foe left field to the right cerebrum.1 J
(Rare) dysfunction is an indication for treatment. Visual?*! evst wall are often all that can be done. Each optic tract sweeps arbund the
Aneurysms of the circle ofWillis, particularly acuity and visual fields may improve dramatically £’ hypothalamus and cerebral peduncle; io end’in
of the anterior cerebral artery, cause a descending after pressure has been removed from the chiasm '■ the lateral geniculate body, with a smaller portion
optic atrophy. Angiography is often diagnostic X-ray evidence of erosion of the sella is frequently < carrying pupillary impulses continuing tb the
in these cases. Inferior temporal quadrant visual seen as an incidental finding on skull films in ■ - SUPRASELLAR MENINGIOMAS pretectal area and superior colliculi. (Thepupillary
field defects with a central scotoma on the side of patients over 50 with benign asymptomatic pathway is diagrammed in Fig ‘11-1 l.)[^he visual
the optic atrophy are usually found. tumors. No treatment is indicated in such cases. i fibers synapse in the lateral geniculate)body.
Suprasellar meningiomas arise from foe dura cell bodies of this structure give rilse to foe
Lesions Posterior & Superior to the Chiasm Chromophobe Adenoma (Neutrophilic) around the chiasm and sella. The tumor is usually geniculocalcarinc tract, the final neuron o.f foe
• (Rare) This is by far the most common pituitary anterior and superior to foe chiasm, and the visual visual pathway. The geniculocalcarine tract phases
These are caused by internal hydrocephalus tumor. The onset is gradual, and the incidence field changes are characteristic. The optic nerves through the posterior limb of foe inteniiil capsule
resulting from tumors of the wall of the third increases with age; these tumors are most; are often involved early (but asymmetrically) in and then fans into the optic radiation, which
ventricle, such as hypothalamic tumor. Signs of commonly seen in the 50s and 60s. Headache and the slowly progressive damage to the visual traverses parts of the temporal, and parietal lobes
. increased intracranial pressure are present, and visual loss are the most common presenting ' pathway. Skull X-rays may reveal hyperostosis or en route to the occipital cortex.
ventriculograms reveal the hydrocephalus. The complaints. Later, bitemporal field defecls and stippled calcification within foe tumor, but foe sella
visual fields will show inferior bitemporal loss. eventually optic atrophy appear along with turcica is normal. Carotid arteriograms usually Detection of Lesions of the Optic Pathways; . •
(Same as the second diagram in Fig 14-10, symptoms of hypopituitarism. show displacement offoe normal vessels and often The primary method of localizing I’esion&of
illustrating anterior and superior lesions, except filling ofabnormal tumor vessels as well. Treatment the optic pathways is by central and peripheral
that good central vision is usually retained longer.) Chromophile Adenoma (Eosinophilic) consists of surgical removal. visual field examination. Fig 11 jl 1 shows; the tyjtas
Eosinophilic adenoma causes marked of field defects caused by lesions in various
Lesions Lateral to the Chiasm endocrine disturbance, resulting in gigantism locations of foe pathway. Lesions anterior to foe
Aneurysm of the internal carotid artery is the before foe epiphyses of long bones are closed chiasm (of the retina or optic nerve) cause ^unilateral
most common cause, but tumors such as and acromegaly thereafter. The visual field GLIOMA OF THE-QPTIC CHIASM field defects; lesions anywhere in |lje visual
meningiomas or intrasellar or extrasellar tumors changes appear later, after foe tumor has eroded pathway posterior to the chiasmcausc contralateral
occasionally cause lateral pressure upon the optic through the sella or pushed against foe chiasm. homonymous defects. Chiasmal lesiqns cause
chiasm. The visual field change is a unilateral nasal Acromegaly may progress slowly for years. Optic chiasm glioma is a rare disorder, usually bitemporal defects and are discussed separately.
hemianopsia. Binasal hemianopsia occurs if there of children, that sometimes occurs as part of the Multiple isopters (field tests with several
is lateral pressure on both sides of foe chiasm. clinical picture of neurofibromatosis. Onset may objects of different sizes) should be used in order
Lesions causing lateral pressure on foe chiasm be sudden, with rapid loss ofvision. Optic atrophy to evaluate the defects thoroughly. A field defect
•' include meningioma of foe lesser wing of the CRANIOPHARYNGIOMA occurs, and visual field defects reveal a chiasmal shows evidence of actively spreading disease
• sphenoid, arachnoiditis, aneurysm ofthe internal (Rathke Pouch Tumor) syndrome. Orbital X-rays may reveal enlargement when there are areas of “relative scotoma” (ie, a
' carotid artery, and arteriosclerosis of the carotid of foe optic foramen; air contrast studies may larger field defect for a smaller test object). Such
.• ’ artery. reveal displacement of the third ventricle. visual field defects are said to be “sloping.” This
Craniopharyngiomas are a group of tumors Treatment is by irradiation, since surgical removal is in contrast to vascular lesions; with steep borders
! {'7 arising from epithelial remnants of Rathke’s pouch usually results in blindness. (ie, the defect is the same size no mattcr-what size
(80% of the population normally have such test object is used). ’ } j
INTRASELLAR PITUITARY TUMORS remnants) and characteristically are first seen Another important generalization' is that the
between foe ages of 10 and 25 years. They are more congruous the homonyfoous field defects
usually suprasellar, occasionally intrasellar. The THE OPTIC PATHWAY (ie, the more similar the 2 fields), the farther
The anterior lobe of the pituitary gland is the signs and symptoms vary tremendously with foe posterior the lesion is in foe visual pathway,: A
site ofcrigin of pituitary tumors. Three types of age of the patient and foe exact location of foe lesion in the occipital region causes’identical
cells are normally present (basophils, eosinophils, tumor as well as its rate of growth. When a Anatomy defects in each field, whereas loplic triibt lesions
and neutrophils), and any one type can suprasellar tumor occurs, chiasmal syndrome field The second cranial nerve is the pathway for cause incongruous (dissimilar) bomony'mous field
predominate in a tumor. Symptoms and signs defects are prominent. Pituitary deficiency may the special sense of vision. It is made up of visual defects. Also, the more posterior the lesion,-the
include loss of vision and field changes (90%), X- result; and posterior pressure may cause fibers (80%) and afferent pupillary fibers (20%). more likely that there will be macular sparing and,
ray evidence of bony erosion of foe- sella (80%), hypothalamic disturbances such as diabetes At the chiasm, more than half of the fibers (those therefore, maintenance of good visual jacuifyJ.j
pituitary dysfunction (60%), extraocular nerve insipidus. With foe passage ofyears, calcification from the nasal retinas) decussate and join foe
palsies ( 10%), and papilledema (rarely). and ossification of parts of the tumor give a uncrossed temporal fibers of foe opposite side to The Normal Pupil u , .
SurgicaL removal is foe usual method of characteristic radiographic appearance. Treatment form the optic tracts. All of the fibers receiving The size ofthe normal pupil varies at different
treatment. Irradiation has been equally effective consists of surgical removal, if possible, but impulses from the right visual field are thus ages and from.person to person. The normal
projected to the left cerebral hemisphere; those pupillary diameter is usually about 13-4jn^m,
120 121 i- ?'
tending to be larger in childhood and progressively THE PUPILLARY PATHWAYS plcuroanatomy of the Pupillary Pathways to light) or unilateral. Some degree of Argyll
smaller w th advancing age. Many normal persons Robertson pupil is present in over 50% ofpatients
have aj slight difference in pupil size (physiologic A. Light Reflex: The pathway for the light with CNS syphilis. A wide variety of other CNS
anisocona). Occasionally there is a marked reflex is entirely subcortical. The afferent pupillary diseases infrequently cause incomplete Argyll
difference in pupil size in otherwise normal eyes. The evaluation of the pupillary reactions is •:> fibers are included within the optic nerve and Robertson pupil. These include diabetes, chronic
Mydtlhti^ and cycloplegic drugs work more important in localizing lesions involving the optic •' pathway until they leave the optic tract just before alcoholism, encephalitis, multiple sclerosis, CNS
effectively on blue eyes than on brown eyes. The pathways. A knowledge of the neuroanatomy of v the visual fibers synapse in the lateral geniculate degenerative disease, and tumors of the midbrain.
function of the pupil is to control the amount of the pathway for reaction of the pupil to light and body. They go to the pretectal area of the midbrain The site or sites of the CNS lesion are not definitely
light entering the eye to give best visual function the miosis associated with accommodation is very and synapse. Impulses are then relayed by known, but Fig 11-11 shows the most likely
undef.lthe various degrees of light intensity important. crossed fibers through the posterior commissure locations.
normally encountered. The pathways controlling to the opposite Edin ger-Westphal nucleus. Some
this purely reflex function are described below and fibers also go directly ventral to the- ipsilateral
are diagrammed in Fig 11-10. Edinger-Westphal nucleus. The efferent pathway
is via the third nerve to the ciliary ganglion within TONIC PUPIL
the retrobulbar extraocular muscle cone. The
postganglionic fiber goes via the short ciliary
nerves to innervate the sphincter muscle of the This not uncommon entity is characterized
ins. by a delayed or diminished direct and consensual
B. Th e Near Reflex: When the eyes look at a reaction to light (80% unilateral) in a pupil larger
near object, 3 reactions occur: accommodation, than normal. It may be associated with loss of
convergence, and constriction of the pupil, tendon reflexes (Adie’s syndrome). It results from
bringing a sharp image into focus on damage to the ciliary ganglion and aberrant
corresponding retinal points. There is convincing regeneration of nerve flow. The cause is obscure
evidence that the final common pathway is but definitely is not syphilis, and it is important
mediated through the oculomotor nerve with a that tonic pupil be differentiated from Argyll
synapse in the ciliary ganglion. The afferent Robertson pupil.,It is most frequently seen in
pathway has not been worked out, but there is young adult women. It may come on abruptly and
evidence that it enters the midbrain ventral to the be noticeable to the patient because of increased
Edinger-Westphal nucleus and sends fibers to sensitivity to ligfet. A weak (2.5%) solution of
both sides of the cortex. Although the 3 methacliDline instilled into the conjunctival sac
components arc closely associated, it cannot be causes a tonic pupil to constrict; normal pupils
considered a pure reflex as each component can are not affected. The tonic pupil dilates slowly in
be neutralized while leaving the other 2 intact-ie, the dark and reacts promptly to mydriatics.
by prism (neutralizing convergence), by lenses
(neutralizing accommodation), and by weak
mydriatic drugs (neutralizing miosis).
HORNER'S SYNDROME

ARGYLL ROBERTSON PUPIL Homer’s syndrome is caused by a lesion of

the sympathetic pathway in the brain stem, upper
spinal cord, or peripheral sympathetic chain.
A typical Argyll Robertson pupil is very Unilateral miosis, ptosis, and absence ofsweating
suggestive of CNS syphilis associated with tabes on the ipsilateral face and neck make up the
dorsalis or general paresis. The pupil is less than complete syndrome. Causes of Homer’s syndrome
3 mm in diameter (miotic) and does not respond to include cervical vertebral fractures, tabes dorsalis,
light stimulation. The pupil does constrict with syringomyelia, cervical cord tumor, apical
accommodation. The finding is nearly always tuberculosis, goiter, enlarged cervical lymph
bilateral. The pupils are commonly irregular and glands, apical bronchogenic carcinoma,
eccentric and dilate poorly with mydriatics. Less mediastinal tumor, and aneurysm of the carotid or
FIG i‘l-10. The optic patliway.The dotted lines represent nerve fibers from the retina to the commonly, the sign is incomplete (slow response subclavian artery.
cortex that carry visual and pupillary afferent impulses from the left half of thevisual field.
'* ‘ 122 123
 EXTRAOCULAR MOVEMENTS to the appropriate oculomotor nuclei to effect
vertical gaze. The vestibular nuclei undou btedly
affect this pathway also.
i' .
This section deals with the neural apparatus C. Convergence: It is probable thiat (the
that controls the movements of the eyes and supranuclear impulses for convergence travel
causes them to move simultaneously in tandem much the same pathway as do those for cori jugate
up or down and side to side as well as in horizontal and vertical gaze, arriving at a mii’d brain
convergence or divergence. J-"
synapse near or in the oculomotor nucleusl From
this synapse, stimulating impulses go th each
medial rectus, and inhibitory impulses go ip each
lateral rectus via the medial longitudinal
Ciliary SUPRANUCLEAR PATHWAYS fasciculus. ; jri ’
ganglion
D. Divergence: Electromyography Has
Accessory
flanglion The supranuclear neural pathways of the established divergence as an active process (hot
extraocular muscles innervate conjugate lateral a relaxation of convergence, as was once thought).
Mesencephalic The supranuclear pathway is probably TiorB'pr
root of fifth nerve
and vertical gaze as well as the disjunctive
movements ofconvergence and divergence. They less the same as for convergence, arriving ,al a
consist of CNS connections to the nuclei of cranial midbrain center near the sixth nerve nuclei.
nerves BI, IV, and VI located in the midbrain. The Stimulating impulses go to each lateral rectus;and
highest centers for these functions are located in inhibitor impulses to each medial rectus viaHhe
the frontal Jobe (voluntary or command medial longitudinal fasciculus as the eyes undergo
movement) and the occipital lobe (involuntary or divergence. | P'
fixation movement).
Oculomotor * U!:
nucleus Anatomy of Pursuit Horizontal & Vertical
Anatomy ofVoluntary Conjugate Movements Conjugate Movements | *’ i
I
Edinger- The cortical center is a large ill-definled-aren
Westphel A. Horizontal: The cortical center that
nucleus controls horizontal conjugate movements of the of the occipital lobe. The descending pathway is
eyes is situated posteriorly in the second not definitely known, but in general it fc/lloSys a
convolution of the frontal lobe. The pathway route similar to the voluntary pathway intcVjthe
into pie
descends with the pyramidal tract in the internal internal capsule, midbrain, vestibular nuclei] and
capsule through the basal ganglia and into the medial longitudinal fasciculus. This plathfyay
brain stem. The fibers leave the pyramidal tract in carries the impulses of the “following mev ienj^htM
colliculus Pretectal nucleus
the midbrain, cross, and probably go to the as demonstrated by optokinetic nystagmlus.jijThc
vestibular nucleus. Impulses then go to a eyes will “follow” a slowly moving object 'even
(1) Lailon al optic nerve. Losj of direct and oppoxlte ceiuensuil
hypothetical nucleus for conjugate lateral gaze when the voluntary pathway is nonfunc foiling.
(4) Leskm between decussation and Edinger-Westphal nucleus.
reaction. Retention of near reflex. Loss of Ipsilateral direct and consensual reaction. Near reflex
which may be identical with the abducens nucleus. When both pathways are intact, the vc luntpry
(2) Lesion at optic tract Contralateral hemianopslc loss of Intact (unilateral Argyll Robertson pupD). The ipsilateral lateral rectus and the contralateral influence dominates the pursuit influencej* !|
reaction (Wernicke’*), (5) Lesion of all fibers from pretectal nucleus to Edinger-
(3) Lerion at optic tract beyond the exit of the pupillary fibers.
medial rectus muscles are stimulated to produce
Westphal nucleus. Lois of all light response. Retention of
No pupUlaiy reaction low (rijjit homonymous hcraHnopih. near reflex (complete Argyll Robertson pupil).
conjugate movement via the medial longitudinal
present). (6) Lesion or third cranial nerve. Ipsilateral pupillary paralysis. fasciculus. 1. LESIONS OF SUPRANUCLEAR >.
(7) Leiion of ciliary ganglion. Ipsilateral lota of Bghl reflex irith PATHWAYS
retention of near reflex (unCaterel Argyll Robertson pupfl).
B. Vertical: The centers and pathways are
probably the same as for horizontal movement
except that the subcortical pathway leaves the Frontal Lobe i
: -j
pyramidal tract somewhat higher and synapses in
the pretectal area. The impulses then go to the Irritative lesions of the frontal lobe involving
FIG 11-11. Pupillary pathways for light reflex and miosis of accommodation. Solid lines= efferent
medial longitudinal fasciculus and are distributed the supranuclear ocular pathways may clause
pathway. Dotted lines = afferent pathway.
124
125
ipyoluntary turning of the eyes to the side frequently encountered (in order of frequency^,1 If hysteria is the cause, atropine, 1 %, 2 drops NUCLEAR & INFRANUCLEAR
opposite! the lesion during the acute phase. are vascular accidents, multiple sclerosis, tumors-. - jnCach eye twice daily, or minus (concave) lenses CONNECTIONS
Destructive lesions cause deviation to the same and encephalitis. may give temporary relief. Psychiatric consultation
side/'and the eyes cannot be turned to the is indicted for treatment of an underlying mental
opposite ’Side. This is termed a gaze palsy. The cause. Peripheral & Intermediate Connections of the
“following reflex” is retained since the involuntary 4. OCULOGYRIC CRISIS Nuclei of Cranial Nerves in, IV, & VI
pathway is unaffected. There is no diplopia. An Convergence Paralysis
exaggerated end point nystagmus is often the first Convergence paralysis is characterized by a A. Oculomotor (III): The motor fibers arise
sign of a gaze palsy or may occur as a residuum of sudden onset of diplopia for near vision, with from group of nuclei in the central gray matter
Oculogyric crisis is a tonic, conjugate/-
a-cledring gaze palsy. absence of any individual extraocular muscle palsy. ventral to the cerebral aqueduct al the level of the
spastic upward movement of the eyes; it is -
His caused by hysteria or destructive lesions of superior colliculus. Mainly uncrossed fibers
: . 5 * frequently associated with a backward head tilt • ■
i • • i the supranuclear pathway for convergence. course through the red nucleus and the inner side
It occurs most commonly in parkinsonism ’
Occipital’Lobe Multiple sclerosis, encephalitis, tabes dorsalis, of the substantia nigra to merge on the medial
epidemic encephalitis, and as a consequence .’
tumors, aneurysms, minor cerebrovascular side of the cerebral peduncles. The nerve runs
of phenathiazine toxicity. Tabes dorsalis,./
The|“following reflex” may be lost with accidents, and Parkinson’s disease are the most alongside the sella turcica, in the outer wall of the
multiple sclerosis, cerebral tumors, and hysteria-<
lesions of the posterior hemispheres. The patient common organic causes. cavernous sinus, and through the superior orbital
are rarer causes. Generalized painful sensations
is Unable Ito follow a slowly moving object in the fissure to supply the internal, superior, and inferior
opened mouth, and protruding tongue are other
direction of the gaze palsy: The command rectus muscles and the inferior oblique and levator
characteristic signs of an attack which may last-
movement is not lost. palpebrae muscles. .
from a few seconds to several hours. Attacks
6. SUPRANUCLEAR LESIONS & The parasympathetics arise from the Edinger-
occur sporadically or frequently, at times even STRABISMUS Westphal nucleus just Tostrad to the motor
daily, and are often, followed by sleep. The .
nucleus of the third nerve and pass via the.
specific cause is not known, but this disorder<
nasociliary branch of the third nerve to the ciliary
is generally considered to be due to malfunction Strabismus is dealt with in Chapter 12, but it ganglion. From there the short ciliary nerves are
of the extrapyramidal system. should be mentioned here that idiopathic distributed to the sphincter muscle of the iris and
Oculogyric crisis is a poor prognostic sign esotropias and exotropias with onset early in life to the ciliary muscle, which controls the shape of
• Thid syndrome is characterized by loss of
in parkinsonism, often just preceding invalidism may result from abnormalities of the supranuclear the lens during accommodation.
voluntary upward gaze and (frequently) loss of
or death. Recent neurosurgical procedures have pathways regulating divergence and convergence.* 1 2 3 4 5
the pupillary light response with retention miosis
relieved or reduced oculogyric crisis. Mild
response in the near reflex. Nystagmus
cycloplegics such as hyoscine given topically may
retractoriu.s, which consists of retraction
shorten or abort an individual attack.
movements of the globe on attempted upward
gaze or convergence, is also frequently present,
lihjcre may also be accommodative spasm, a loss
of conjugate voluntary downward gaze associated
with Joss of convergence and accommodation, TABLE 14-1. Differentiation of cortical and brain stem lesions causing gaze palsies.
5- SUPRANUCLEAR SYNDROMES
ptosis or lid retraction, papilledema, or third nerve
p’aisy. Surrounding structures may also be INVOLVING DISJUNCTIVE OCULAR
involved;depending upon the size and location of MOVEMENTS ___________ Lesion Above Brain Stem_____________ _____ Lesion in Brain Stem___________
the lesion!. Conjugate horizontal ocular movements (1) Conjugate deviation is usually present early, is usu­ (1) Conjugate deviation is rarely present and is of small
ally of large magnitude, and is usually toward the magnitude.
arc usually not affected. The syndrome results from side of the lesion.
tcctalprpretectal lesions affecting die periaqueductal Spasm of the Near Reflex
(2) Gaze palsy is usually of short duration. (2) Gaze palsy is away from the side of the lesion and
area. Pinealomas, infiltrating gliomas, vascular is present as long as the Lesion remains.
lesions, and trauma may produce this picture. The near reflex consists of 3 components: (3) Lesion is frequently irritative. (3) Lesion is rarely irritative.
;; ; i convergence, accommodation, and constriction (4) Head is turned in direction of deviation. (4) Head is straight or turned in direction opposite the
of the pupil. Spasm of the near reflex is usually ocular deviation.
> (5) Individual Intranuclear muscle palsy is not present; (5) Infranuclcar muscle palsy is often present; diplopia
i i 1 caused by hysteria, although encephalitis, tabes diplopia is never present. is common.
I 3. LESIONS OF THE BRAIN STEM dorsalis, and meningitis may cause spasm by
1■ !: irritation of the supranuclear pathway. It is
characterized by convergent strabismus \
i• ■■ ■ i
| ‘ Lesions of the brain stem-are the most diplopia, miotic pupils, and spasm
dommonjeauses of gaze palsies.' The lesions most accommodation.

126 127
I
 B. Trochlear (IV): Motor (entirely crossed) SUMMARYOFJDISORDERS OF CRANlAf ' and, quite often, ptosis, with normal pupillary!
Symptoras & Signs of Extraocular NIusclc
fibers arise from the trochlear nucleus just caudad NERVES III, IV & VI •: ’ reactions and accommodation. It may begin atjany I
palsies
to the third nerve at the level of the inferior Diplopia occurs when the visual axes are not age and progresses over a period of 5-15 years tb:
colliculus, run posteriorly, decussate in the aligned. This is especially true when the onset of complete external ophthalmoplegia. Generalized;
anterior medullary velum, and wind around the Oculomotor Paralysis (Cranial Nerve III) muscular dystrophy occasionally ensues. MoStj
strabismus is after age 6 (suppression and
cerebral peduncles. From here the fourth nerve authorities believe that this disease is a variant of;
abnormal retinal correspondence does not
follows the third nerve along the cavernous sinus A. Complete Oculomotor Paralysis: The • develop)- Dizziness is often associated. Head tilt a type of hereditary' muscular dystrophy primarily ?
to the orbit, where it supplies the superior oblique lesion involves the third nerve anywhere from thfc'A affecting extraocular muscles. There is no effective j
occurs, especially in paresis of the superior
muscle. nucleus (midbrain) to the peripheral branches uj; ’: oblique or superior rectus muscle, when the head treatment, although cosmetic surgery fo'ri
the orbit. It causes divergent strabismus since thfe- tilt is to the opposite side to avoid diplopia by strabismus or ptosis is often necessary,.*
, . C. Abducens (VI): Motor (entirely uncrossed) eye is turned out by the intact lateral rectus muscle?/ moving the eye out of the field of action of the Myasthenia gravis is tire major differential problem.,
fibers arise from the nucleus in the floor of the and slightly depressed by the intact superior? paralyzed muscle.
. fourth ventricle in the lower portion of the pons oblique muscle. There is a dilated fixed pupil-/ B. Bcnedikt’s Syndrome: Ipsilateral1
Ptosis is caused by weakness or paralysis of
near the internal genu of the facial nerve. Piercing absent accommodation, and ptosis of the upper/ oculomotor paralysis, which may be partial, and a {
the levator muscle.
the pons, the fibers emerge anteriorly, .the nerve lid, often severe enough to cover the pupil. The. contralateral hyperkinesis from involvement of the
running a long course over the tip of the petrous eye may only be moved laterally. Trauma, carotid red nucleus are caused by a lesion of the:red >
•_... portion of the temporal bone to the outer wall of aneurysm, and diabetes are the most common - nucleus in the midbrain which involves*
the cavernous sinus. It enters the orbit with the causes. In diabetes, the pupillary responses ate: SYNDROMES AFFECTING CRANIAL oculomotor fibers (cranial nerve III) passing
third and fourth nerves to supply the lateral rectus usually intact; in carotid aneurysm, they almost NERVES 111. IV. &VI through it. A cerebrovascular accident is the most1
muscle. never are spared. frequent cause. i » i
B. Complete Internal Ophthalmoplegia: This; ! : • I
consists of a dilated and fixed pupil and paralysis Peripheralinvolvement ofCranialNerves ID, IV, & VI C. Weber’s Syndrome: Crossed hemiplegia
Central Reflex Connections of the Nuclei of of accommodation. The lesion is nearly always (including the lower face) and ipsijateral’
Cranial Nerves JU, IV, & VI peripheral in the ciliary ganglion and often results A. Gradenigo’s Syndrome: This is oculomotor paresis are caused by a lesion in. the i
in tonic pupil. f upper portion of the midbrain (see below)
characterized by pain in the face (from irritation of
The central reflex connections ofthese nuclei involving the peduncle and cranial neiwe 111.
the trigeminaL nerve) and abducens palsy. The
- originate in 5 areas: (I) From the pretectal region
syndrome is produced by meningitis at the tip of Cerebrovascular accidents and tumors hre.tbc
via the posterior commissure to the Edinger- Trochlear Paralysis (Crania! Nerve IV)
the petrous bone and most often occurs as a rare most frequent causes. 1 J
Westphal nuclei for mediation of ipsilateral and There may be esotropia (convergent complication of otitis media.
consensual light reflexes. Interruption of this strabismus) in. the primary position, with diplopia D. Foville’s Syndrome: Ipsilateral paralysis
pathway may cause an Argyll Robertson pupil. relieved by tilting the head to the side opposite B. Cavernous Sinus Syndrome ofthe muscles of facial expression, with abducens«
(2) From the superior colliculi via the tectobulbar the affected superior oblique muscle-a valuable palsy and loss of conjugate lateral gazt| to’tbe.l
tract to the nuclei of cranial nerves III, IV, and VI diagnostic clue. There is difficulty in turning the C. Orbital Fissure Syndrome: All extraocular same side, is caused by a lesion in the pons
for the mediation of miosis associated with eye downward, which may cause difficulty ia peripheral nerves pass through the orbital fissure involving the abducens nucleus and facial fibers
accommodation. (3) From the inferior colliculi via reading or in descending stairs. This is the least and can be involved by a traumatic bone fracture which arch around it before making their;central
■the tectobulbar tract to the eye muscle nuclei for common oculomotor palsy and most often occurs in this area or by tumor encroaching on the fissure exit. It may also include Homer’s syndrome, loss
reflexes correlated with hearing. (4) From the as a result of trauma. from the orbital or cranial side. of taste, deafness, and facial analgesia on the same ‘
vestibular nuclei via thE medial longitudinal
side. |
fasciculus forreflex gaze movement correlated with
D. Orbital Apex Syndrome: This is similar to
equilibrium. (5) From the cortex through the Abducens Paralysis (Cranial Nerve VI) the orbital fissure syndrome with the addition of E. Millard-Gubler Syndrome: This is.
corticobulbar tract for mediation of voluntary and This is the most common single muscle palsy, optic nerve signs. It is caused by an orbital tumor essentially the same as Foville’s syndromq except
involuntary conjugate movements of the eyes. since one nerve with a long intracranial course or trauma which damages the optic and extraocular that the lesion is lower in the pons and misses the i
supplies one extraocular muscle. Abduction of the nerves. supranuclear pathway for conjugate lateral gttee. j
eye is absent; esotropia is present in the primary The pyramidal tract is affected along with fibers (
position and increases upon gaze to the affected of cranial nerves VI and VII as they leave the pons.
side. Movement of the eye to the opposite side is Brain Stem Syndromes There is therefore a crossed hemiplegia and
normal. Cerebrovascular accidents are a common
cause: basilar artery disease, increased intracranial *”Extc;’Uii ophthalmoplegia" is a general term that
A. Chronic Progressive External
pressure, tumors at the base of the skull Ophthalmoplegia:* This rather rare disease
denotes inability ia move the eyes normally as. a result
of any n«c or infranucicar involvement oi' cranial
meningitis, diabetes, and trauma are other frequen1 involves all 3 cxtraocular nerves. It is characterized nerves til, IV, or VI; ike pupillary reaction anil
causes.
by a slowly progressive inability to move the eyes accommodation arc normal. i ;■

128
129
ipsilateral facial and abducens palsy without a smaller the amplitude and vice versa. ?;£ Nystagmus Elicited by Stimulation of
B, Pathologic Nystagmus:
horizontal gaze palsy. Known factors relating to ocular movements: - Semicircular Canals
1 .Congenital -
iivte! Wernicke’s Syndrome: This syndrome malfunction of which can cause nystagmus, arc a. Sensory defect type. A. Bdrany Rotating Chair: The horizontal
as follows. The labyrinth exerts influence on eye­ b. Motor defect type. canals are horizontal with the floor when the chair
consists pf bilateral external ophthalmoplegia, movements by 2 mechanisms: (1) the otolith-’ is depressed 30 degrees on the chest. Rotation of
c. Latent.
nystagrqus, ataxia" and Korsakoff’s psychosis. apparatus influences torsional eye movements in the subject causes a jerky nystagmus in the
2. Spasmus nutans.
Ptosis! is present! in 50% of cases; internal response to head position; (2) the semicircular; 3. Nystagmus due to neurologic disturbances direction of the turning. The slow component is
ophthalmoplegia is less frequently present. Other canals influence eye movements in response t0 a. Acquired pendular or jerky in the opposite direction, the same as the flow of
cranial .nerves are commonly affected, and acceleration and deceleration. The gaze nystagmus. endolymph in the semicircular canals.
polyneuritis may’occur elsewhere. Although mechanism influences the supraconnections of IJ, Caloric Stimulation: With the subject
b. Ocular flutter.
scvci-e! chronic alcoholism is the most frequent extraocular muscle function. These complicated supine and the head flexed on the chest, cold water
c. See-saw nystagmus.
cliriical jsetting in] which this disorder occurs, pathways are known in a general way, but many ear irrigation produces nystagmus with the slow
d. Nystagmus retractorius.
hyperemesis gravidarum and other malnutritional details are far from established. The fixation 4. Vestibular nystagmus. component toward the side of irrigation while
statejj may be causative. The metabolic defect has mechanism also is not completely understood but 5. Gaze nystagmus. warm water produces nystagmus with the slow
bcqrf.clearly shown to be thiamine deficiency. undoubtedly involves the retina, the visual 6. Voluntary and hysterical nystagmus. component away from the side of irrigation.
Treiil^h^rit consists of immediate parenteral pathways, the brain stem, and the cerebellum.
thiartiiric and should also include alcohol
witilclrawhl and proper diet with other vitamin Physiology ofSymptoms
supplementation. This will effect a complete cure Reduced visual acuity is caused by inability PHYSIOLOGIC NYSTAGMUS PATHOLOGIC NYSTAGMUS
if tire |disease is nciL too far advanced. Without to maintain steady fixation. False projection is
trehtmerit the course is rapidly downhill to stupor, evident in vestibular nystagmus, where past­
delirium, and death-within several weeks. pointing is present. Head tilting is usually The following 3 types of nystagmus can be
involuntary, to decrease the nystagmus. The head elicited in the normal person. Alteration of normal 1. CONGENITAL NYSTAGMUS
is turned toward the fast components in jerky response may be helpful diagnostically.
nystagmus, or set so that the eyes are in aposition
' i.fi; j . .
which minimizes ocular movement in pendular End Point Nystagmus Sensory Defect Type
NYSTAGMUS nystagmus. The patient sometimes complains of A jerky type of nystagmus ofsmall amplitude Congenital impairment of vision in any part
illusory movements of objects (oscillopsia). This with the fast component in. the direction of gaze of the eye or optic nerve can result in pendular
is more apt to be present in nystagmus due to commonly occurs in extreme lateral gaze after a nystagmus. Causes include corneal opacity,
lesions of lower cenLers, such as the labyrinth, or latent period of not more than 30 seconds. The cataract, albinism, posterior polar chorioretinitis,
' Nystagmus is defined as involuntary, associated with the sudden arrest of nystagmus nystagmus appears earlier and is. of larger aniridia, and opticatrophy. Oscillations of the head
rhytlnijically repealed oscillations of one or both in an adult. The apparent movement of the amplitude in general fatigue states. which are synchronous with the nystagmus but
eyes lift any or all fields of ga2c. The movements environment occurs during the slow component in the opposite direction often accompany
arc>eitljer.pcndulan with undulatory movements and causes an extremely distressing vertigo, so Optokinetic Nystagmus sensory defect congenital nystagmus.
of eqiial: speed, amplitude, and duration in each that the patient is unable to stand. Head nodding This is ajerky type of nystagmus which may
direction; 'Orjerky, with slower movements in one is mast apt to accompany congenital nystagmus, be elicited in all normal individuals, most easily Motor Defect Nystagmus
direction (slow component) followed by a rapid spasmus nutans, and miner’s nystagmus. by means of a rotating drum with alternating black This is manifested as a jerky nystagmus on
retiim-tq the original position (fast component). Nystagmus is noticeable and cosmetically and white lines. The slow component follows the gaze to either side with the fast component in the
Thbiifulli mechanism remains unknown, and the disturbing except when excursions of the eye are object and the fast component moves rapidly direction of gaze. The eyes are otherwise normal
locaiibn of the defeat can usually not be specified. very small. counterwise to fixate each succeeding object. except when strabismus is associated (20%).
'Nystagmus is classified as grade I, present Unilateral or asymmetrical horizontal response There is always a position of relative rest. If this
on|y with the eyes directed toward the fast usually indicates a parietal lobe tumor. Anterior position is with the eyes deviated to one side, the
coiinqonent; grade 11, present also with the eyes Classification of Nystagmus cerebral (ie, frontal lobe) lesions may inhibit this head will be turned toward the opposite side to
in primary positionor gTade III, present even with response only temporarily, which suggests the obtain the best possible vision. This condition
lhe’ eyes directed toward the slow component. The A.Physiologic Nystagmus: presence of a compensatory mechanism which is shows some spontaneous improvement up to
inqyenicints may be horizontal, vertical, oblique, 1. End point. much greater than for lesions situated farther about age 10. The cause is not known, but a lesion
rotatory,’circular, or a combination of these. The 2. Optokinetic. posteriorly. Asymmetry ofresponse in the vertical of the brain stem is probably responsible. About
direction'- may change depending upon the 3 .Stimulation ofsemicircular canals. plane suggests a brain stem lesion. 20% of cases are inherited as an autosomal
diredidh of gaze. Amplitude refers to the extent recessive trait.
of the-movement; fate refers to the frequency of
oscillation. Generally, the faster the rate, the

130 131
 Latent Nystagmus brain stem and is associated with signs of 1^ an inhibitory influence ou nystagmus on its own CENTRAL NERVOUS SYSTEM
Latent nystagmus occurs upon occlusion of retraction, loss of upward gaze (Parinaud's side through the cerebellobulbar tract. Thus a TUMORS OF NEURO ' ' »
either eye. The nystagmus is conjugate and jerky, syndrome), and abnormal pupillary reactions. lesion on one side causes nystagmus toward the OPHTHALMOLOGIC IMPORTANCE
with the fast component toward the side of the side of the lesion.) i • I '
covered eye. The condition .has no known
neurologic significance and is only of
consequence when the affected individual loses 4. VESTIBULAR NYSTAGMUS
one eye. 5. GAZEJ1YSTAGMUS

Vestibular nystagmus is always of the jerky Frontal Lobe Tlimors

type. The slow component is considered to be a Gaze nystagmus is probably the most
2. SPASMUS NUTANS response to impulses originating in the common nystagmus and often represents the first Mental changes (depression, euphoria, or
semicircular canals; the fast component is a sign or the residuum of a gaze palsy. These patients mental deficiency) and contralateral hemiparesis
corrective movement Vestibular nystagmus is not have no nystagmus on forward gaze but develop arc the most frequent signs of frontal lobe tumors.
This uncommon condition of unknown cause dependent upon visual stimuli, ie, it is present nystagmus in one or more fields of gaze with the Pressure upon the olfactory tracts may? cause
occurs in infants 4-12 months of age. The with the lids closed as well as open and can be fast component in the direction of gaze. anosmia. Ophthalmologic signs includeflic
nystagmus is of the dissociated vertical or elicited in blind individuals also. Rotatory' The causes are variable and include drug following: r • |
asymmetric horizontal pendular type and is often movements are especially characteristic of toxicity (especially diphenylhydantoin, but A. Papilledema (50%); pue to increased
associated with nonsynchronous head nodding. vestibular nystagmus, but horizontal or vertical barbiturates also) and demyelinating, intracranial pressure. ; ■ ! •"j
The prognosis is good; recovery occurs within a vestibular nystagmus also occurs. degenerative, neoplastic, or vascular disease. Gaze B. Visual Field Changes (30%); These’are
few months to 2 years. Physiologic nystagmus elicited by j nystagmus is of no specific localizing value except not characteristic. Almost any defect is possible
stimulation ofthe semicircular canals by means of that it is suggestive of lesions of the posterior as a result of pressure upon the optic paihWay..(
the Barany chair or caloric stimulation depends fossa. C. Irregular Nystagmus (5-10%); This is
upon normal vestibular function. of no localizing value. | . i -I !*•»
3. NYSTAGMUS DUE TO NEUROLOGIC The following characteristics of vestibular D. Gaze Palsies: Irritativejlesions cause, file
DISTURBANCES nystagmus demonstrate its origin in labyrinthine eyes to deviate to the opposite‘side. Destructive
and vestibular nerve disease: (1) Vertigo, tinnitus, 6- VOLUNTARY & HYSTERICAL lesions cause deviation to the same side. ThCsp
and deafness are apt to be associated. (2) NYSTAGMUS gaze palsies are usually incomplete and last Only
Pendular or Jerky Nystagmus Nystagmus is maximal early in the disease, and a few weeks, or maybe manifested by on end point
Acquired pendular or jerky nystagmus is tends to improve or disappear in 2-3 weeks (unless nystagmus only. I I i .! ’ j
usually horizontal (occasionally vertical) and die vestibular nuclei are affected directly, in which Voluntary nystagmus is an uncommon r! •• i
. . causes oscillopsia. It is frequently seen in case nystagmus may be permanent). (3) The lesion “parlor trick.” The individual “wills” a rapid
demyelinating disease (occasionally in vascular is always destructive, and its direction (fast horizontal nystagmus of high frequency and low Temporal Lobe Tumors
disease) and is due to a lesion of the brain stem. component) is away from the side the lesion is on. amplitude which can be maintained only a few
seconds. Gliomas are the most .common; type.
. . Ocular Flutter Specific Lesions Causing Vestibular Nystagmus Hysterical nystagmus is similar to voluntary Meningiomas are next most commopBand
Ocular flutter is a sign of cerebellar disease. Vestibular nystagmus may be due to ' nystagmus. It may be even more rapid, and is angiomas are rare. :
It consists of a series of pendular horizontal labyrinthitis, Meniere’s disease, traumatic particularly common in anxiety neuroses. Temporal lobe tumors may -. c'iuse
movements while fixating an object. (including surgical) destruction of one labyrinth; psychomoLor convulsions arid uncinate «£its,
vascular, inflammatory, or neoplastic lesions of sometimes preceded by an aura of abnormal smeji.
See-Saw Nystagmus the vestibular nerves; lesions of the vestibular Visual hallucinations may occuri A generalized or
This rare type of nystagmus consists of nuclei (encephalitis, multiple sclerosis, localized convulsion (chewing, sucking, cir
regular reciprocating oscillations in which one eye syringobulbia, poliomyelitis, thrombosis of the smacking movements) follows. Auditory
• rises while the other falls. Tumors in the region of posteroinferior cerebellar artery); or cerebellar disturbances are not common. Ophthaltnol'qgic
the optic chiasm and diencephalon are the most tumors and abscesses (probably as a result of signs include papilledema (often) and incongnjdus
frequent cause. pressure on the vestibular pathways). (Note: There visual field changes (usually). The typicabyisuffl
is some dispute about whether a cerebellar lesion field change is an incongruous contrahftdr^l
Nystagmus Retractorius per se can cause nystagmus. Evidence suggests homonymous hemianopsia'- of the jupper
With convergence or on upward gaze, that nystagmus in such cases results from pressure quadrants. !| ,
nystagmus associated with retraction of the eyes on the vestibular structures. An alternative ■
is present. It is diagnostic of a lesion of die upper explanation is that the cerebellarhemispberes exert

132 133
Occipital Lobe Ttmiors especially in children. nerve sometimes occurs as part of branch can occur). These small plaques
i.. :{l'he
I occipital
{ <• '
lobe is only rarely involved by
Lesions involving the red nucleus produce Recklinghausen’s disease. More often, the tumor (Hollenhorst plaques) may be visible with the . .
Benedikt’s syndrome and Weber’s syndrome. occurs alone. A cerebellopontine angle tumor may ophthalmoscope as small glistening yellow spots
turnqrl Gliomas, meningiomas, and metastatic Cerebellar signs (ataxia, hypotonia, and also be a meningioma. Cerebellar signs include situated at bifurcations of tlie retinal arteries. A
tumors mqy occur. Epileptic attacks (grand or petit nystagmus) due to pressure on or direct hypotonia, ataxia, and unsteady gait Vestibular finding of reduced ophthalmic artery pressure as
irjal) as well as mild mental abnormalities may be involvement of the cerebellum may also be nystagmus is common as a result of pressure on determined by ophthalmodynamometry, bruits
present. Papilledema can be extreme (up to 6 or 8 present. the brain stem. Increased intracranial pressure over the internal carotid artery, and angiography
diopters). Contralateral homonymous hemianopsia causes papilledema. Cranial nerve palsies include helps to confirm the diagnosis. «
occurs in 85% of cases. It tends to be congruous facial paresis (seventh nerve), tinnitus, deafness, Removal of the plaque by carotid
with sparing of the macula. Loss of involuntary Thmors of the Pons vertigo (eighth nerve), loss of corneal sensation endarterectomy is frequently indicated, and may
conjugate :eyc movements is due to a lesion of the and sneeze reflex (fifth nerve), and dysphagia prevent a major stroke or a central retinal artery '
supranuclear pathway. Visual agnosia occurs as a Gliomas are the most common pontine (tenth nerve). Motor involvement produces occlusion.
rcsiiTt ‘of involvement of the visual association tumors. Multiple nuclear palsies (particularly in contralateral hemiplegia (pyramidal).
' ‘. I
areas. childhood) involve principally the fifth, sixth, Complete or partial excision of the tumor is
J'■ !i I seventh, and eighth cranial nerves in combination usually possible. Thrombosis of the Middle Cerebral Artery
with motor and sensory long tract signs in the
limbs. Increased intracranial pressure develops This disorder may produce severe
‘1 i I CEREBELLAR TUMORS late. Foville’s syndrome is sometimes present. The Pineal Tumor contralateral hemiplegia, hemianesthesia, and
course is usually chronic and progressive. homonymous hemianopsia. The lower quadrants
i
i This rare tumor (2% of all gliomas) may occur ofthe visual fields (upper radiations) are most apt
Cerebellar tumors arc the most common brain at any age. Papilledema occurs early as a result of to be involved. Aphasia may be present
tumors before age 15. Gliomas are common, and Tfiimors of the Medulla blockage of the cerebral aqueduct. Parinaud’s
about oneifourth of these are the very malignant syndrome develops later. Pressure on adjacent
medulloblastomas of the roof of the fourth Early manifestations include vertigo, cardiac structures commonly occurs, causing cerebellar Vascular Insufficiency of the Vertebral-Basilar
ventricle!. Astrocytomas of the cerebellar irregularities, swallowing difficulties, and signs, extraocular muscle palsies, deafness, and Arterial System
hem^sphdre also occur, mainly in children. hoarseness. Paralysis of the tongue and pupillary signs.
Papilledema due to increased intracranial pressure papilledema develop later. Eye signs are not Surgical removal, sometimes followed by Brief episodes of transient bilateral blurring
and' tib'dudens palsy due to pressure on the sixth prominent. Death usually occurs early. irradiation, continues to be the only treatment. of vision commonly precede a basilar artery
nervq atthb base of the brain are common findings. The mortality and morbidity rate continues to be stroke. An attack seldom leaves any residual visual
Vestibular nystagmus also occurs. Systemic very high despite advances in neurosurgical and impairment, arid the episode may be so minimal
cci$t>efiar| signs include ataxia, hypotonia, and radiation technics. that the patient or doctor does not heed the
tremor. OTHER INTRACRANIAL TUMORS warning. The blurring is described as a graying of
OF NEU RO-OPHTHALMOLOGIC vision just as if the house lights were being turned
IMPORTANCE down at a theater. The change seldom lasts more
than 5 minutes (often only a few seconds) and
I BRAIN STEM TUMORS CEREBROVASCULAR LESIONS may be associated with other transient symptoms
Meningioma of Sphenoidal Ridge OF OPHTHALMOLOGIC of vertebral-basilar insufficiency. Such episodes
I
i IMPORTANCE of visual loss are termed amaurosis fiigax.
Tuinprs of the Midbrain The tumoT arises from the small wing of the
t ; sphenoid bone, ipsilateral slight (occasionally
Lesions ofUte upperpart of the midbrain (near severe) progressive exophthalmos is present in Thrombosis of the Basilar Art cry
the quadrigeminal bodies) cause Parinaud’s about 40% of cases and is often the presenting Vascular Insufficiency & Thrombosis of the
syndrome^ sometimes associated with retraction sign. Visual field changes are at first unilateral and Internal Carotid Artery Complete or extensive thrombosis of the
of file lids-or ptosis. Lesions of die lower midbrain later bilateral, and are not characteristic. Headache basilar artery nearly always causes death. V/iih
produce paralysis of downward gaze, paralysis of is common. All changes occur gradually over partial occlusion of basilar “insufficiency” due to
Episodes of amaurosis fugax frequently occur
convergence, loss of pupillary reaction to light or many years, since the tumor grov/s quite slowly. as a result of atherosclerotic lesions of the arteriosclerosis, a wide variety of brain stem and
accbnitnodation (or both), and unequal pupil size. ipsilateral internal carotid artery. Cerebral and cerebellar signs may be present. These include
Increased intracranial pressure is often present retinal disturbances occur as a result of small nystagmus, supranuclear oculomotor signs, and
as atT-esulliOf obstruction of the cerebral aqueduct. Tumors of the Cerebellopontine Angle emboli breaking loose from the sclerotic plaque involvement ofcranial nerves 111, IV, VI, and VII.
Papilledema may be an early or a late sign. Gliomas and lodging in cerebral or retinal arterioles Prolonged anticoagulant therapy has
and plneiilomas are the most common tumors, Neurofibroma (or neurinoma) of the acoustic (occlusion of the central retinal artery or a major become the accepted treatment of partial basilar
artery occlusion or “insufficiency.”
134 135
 Thrombosis of the Posterior Cerebral Artery evacuation of blood. Unequal, miotic, or mydriatic causes oculomotor palsies (abduccns palsy;isitlie
pupils can occur, or there may be no pupillary C. most common). Ptosis and pupillary’chdnges,
Occlusion of the posterior cerebral artery signs. Other signs, including vestibular’ including sluggish light reaction and anispeoria,
seldom causes death. Occlusion of the cortical nystagmus and cranial nerve palsies, also occur. : are also frequent. Papilledema indicative of
branches (most common) causes homonymous Many of these signs result from herniation and increased intracranial pressure may be prominent.
hemianopsia, usually superior quadrantic (the compression of the brain stem, and hence often . Locally the disease may be complicated by
artery supplies primarily the visual cortex). appear late with stupor and coma. chemosis of the conjunctiva and edema of the
Lesions on the left in right-handed persons can Skull films may show a shift of a calcified •: lids, with photophobia a common symptom.
"v. cause aphasia and dyslexia: Occlusion of the pineal gland. Carotid arteriography frequently ; Metastatic uveitis with resulting endophthalmitis
proximal branches may produce the thalamic confirms the diagnosis. and loss of all function occurs at times, especially
syndrome (thalamic pain, hemiparesis, Treatment consists of surgical evacuation of ■ in children. Treatment consists of imassiye
hemianesthesia, choreoathetoid movements) and the blood. Without treatment, the course is systemic administration of sulfonamides and
■ cerebellar ataxia. Weber’s, Benedikt's, or progressively downhill to coma and death. With antibiotics (and intrathecal anti-irifecji’Vcs
Parinaud’s syndrome may. result. early and adequate treatment, the prognosis is “ administered by lumbar puncture). Culttire Of the
good except for infants, in whom repeated subdural spinal fluid nearly always establishes thentiqlogic
taps may be only temporarily lifesaving or may diagnosis. I j : :

result in residual damage (convulsions, low t i ’•

mentality, motor and sensory defects). :• H''
HEAD TRAUMA FIG 11-12. Subhyaloid hemorrhage around optic Tuberculous Meningitis < j t ;■
disk associated with subarachnoid hemorrhage. i * i
(Drawing.) Tuberculous meningitis is a more chjrortic
SUBARACHNOIDHEMORRHAGE form of bacterial meningitis. It occurs rarely, and
SUBDURAL HEMORRHAGE Arteriography following injection of usually in children. The general symptomatology
radiopaque substances may help to demonstrate is the same as outlined above for acute bac'tqrial
Subarachnoid hemorrhage most commonly and localize the aneurysms. Blood is present in meningitis. When miliary lesions can'be |S0bn
Subdural hemorrhage results from tearing or results from ruptured congenital berry aneurysms theCSF. ophthalmoscopically as small oval yellowish
shearing of the veins bridging the subdural space of the circle of Willis in the subarachnoid space. It Ligation of aneurysmal vessels or of parent lesions in the choroid, the prognosis for life.is
from the pia mater to the dural sinus. It leads to an may also result from trauma, birth injuries, arterial trunks may be advisable. Supportive very bad. In the past, tuberculous meningitis Was
encapsulated accumulation of blood in the intracranial hemorrhage, hemorrhage associated treatment, including control of blood pressure, is uniformly fatal. Long-term systemic administration
subdural space, usually over one cerebral with tumors, arteriovenous malformations, or at times all that can be done. of isoniazid, rifampin, ethambutol, and
hemisphere. It is nearly always caused by trauma systemic bleeding disorders. streptomycin has reduced, the mortality! ijatc
to the head. The trauma may be minimal and may The most prominent symptom of significantly.
precede the onset of neurologic signs by weeks subarachnoid hemorrhage is sudden, severe
or even months. headache, usually occipital and often associated
In infants, subdural hemorrhage produces with signs of meningeal irritation (eg, stiff neck).
INTRACRANIAL INFECTIONS OF Syphilitic Meningitis
progressive enlargement of the head with bulging
Drowsiness, loss of consciousness, coma, and OPHTHALMOLOGIC IMPORTANCE
’ fontanels. The diagnosis is established by finding Acute syphilitic meningitis may be quite
death may occur rapidly. Ocular symptoms arc not
bloody spinal fluid on tapping the subdural space similar to acute infectious meningitis. Thb cranial
always present. Extraocular muscle palsies are the
and by enlarged head measurements. Ocular signs nervesparticulnrly the oculomotor nerves 4 are
most common single ocular sign. An oculomotor
include strabismus, pupillary changes, MENINGITIS often involved, causing diplopia. Papilledema and
palsy with associated numbness and pain in the visual field changes, indicating chiasmal
papilledema, and retinal hemorrhages.
In adults the symptoms of chronic subdural distribution of the ipsilateral trigeminal nerve is arachnoiditis (bitemporal hemianopsia), are aUo
hematoma are severe headache, drowsiness, and pathognomonic of a supraclinoid, internal carotid, Acute Bacterial Meningitis common. AU manifestations of meningitis tfpkr
mental confusion, usually appearing hours to or posterior communicating artery aneurysm. well following antisyphilitic therapy. |
weeks (or even months) after trauma. Papilledema usually appears late when it does Acute meningitis may be due to Neisseria I
Symptomatology is similar to that of cerebral occur. Various types of retinal hemorrhage occur meningitidis, Diplococcus pneumoniae, ii i •’. '•.
tumors. Papilledema is present in 30-50% of cases. infrequently (preretinal hemorrhages are the most Haemophilus influenzae, Staphylococcus aureus. Encephalornyelitides Associated With Infectious
Retinal hemorrhages occur in association with common). Exophthalmos may occur as a result of Streptococcus viridans, or other bacterial agents. Diseases I | IV
papilledema. Ipsilateral dilatation of the pupil is extravasation of blood into orbital tissues. There are many ocular manifestations of general
the most common and most serious pupillary sign Pressure of an aneurysm on the optic nerve may meningeal irritation, dependingupon the main area Measles, mumps, vaccinia, chickenpox,
and is an urgent indication for immediate surgical cause blindness in. one eye. of associated inflammation. Basal meningitis herpes simp lex, and smallpox are the most dommon

136 137
viral diseases with neurologic complications. sheaths. Other developmental anomalies, exudation, retinal detachment, and absolute
Complications may be due to activation of a particularly of the bones, may be associated. • glaucoma occurs later, and usually destroys the Tuberous Sclerosis (Bournevine’s Disease)
neurotropic virus} a toxin, or an allergic Inheritance is autosomal dominant with incomplete - eye within 5-15 years after onset. The disease is
phenomenon. There (is no treatment. penetrance. Thus the disease can be quite mild in unilateral in 65% of cases. In 25% of cases the This is a generalized disease whose
Neurologic si'gns result from cerebral, one generation and appear as a full-blown retinal angiomatosis is associated with a similar manifestations include adenoma sebaceum (85%),
cerebellar, or cord involvement as well as from debilitating disease in the next >: generalized process, most often affecting the CNS tumors, retinal tumors (50%), renal tumors
opt^c‘neuritis. Most patients recover completely, Neurofibromas are made up of randomly ; cerebellum and less commonly the pancreas, (50%), and multiple lung cysts. The signs may be
but.pcr’nanent sequelae and death do occur, oriented or paLisaded cells of either fibroplastic or kidney, adrenal gland, and other organs. The present at birth or may develop within the first
especially following smallpox, vaccinia, and herpes Schwann cell origin. Nerve fibers often course . evidence at present suggests that this is all one few years of life. Onset is with convulsive seizures
simplex.1. through or over the tumor. The tumors are discrete genetically determined disease showing and mental retardation. The large papular skin
i ’ $ and benign but may undergo sarcomatous autosomal dominant inheritance with variable lesions have the appearance of overgrown
1 ‘ r’ degeneration. •>. expression. Several reports of abnormal “blackheads,” and are often the earliest sign of
Tumors may occur anywhere in the body • chromosomal patterns have been reported. the disease. The retinal tumors appear as oval or
: : if BRAIN ABSCESS including the eye. Caffc au lait spots (small' Early treatment of retinal lesions with circular white areas tn the peripheral fundus, and
• ? 'll pigmented areas of skin) tend to enlarge and photocoagulation has been effective in some characteristically have a mulberrylike appearance.
darken with age. Tumors of the lids are often cases. Cerebral and cerebellar tumors have beeD Histologically, the Tetinal tumors are composed of
i Abscesses form in the brain, by direct present. Tumors of the orbital portion of the optic ? successfully removed, but if the CNS is involved hyaline material with areas of calcification. Large
extension of infection from the nasal sinuses nerve are particularly common, causing ; the prognosis for life is poor. A downhill course drusen of the optic nerve head are also frequently
(fTontaljlobc) and middle ear (temporal lobe or papilledema and retrobulbar neuritis early and ? and death, usually by middle age, is the rule. observed.
cerebellum). Penetrating head wounds and optic atrophy later in the disease. There may be The disease is inherited as an autosomal
septicemia may also produce brain abscesses. iris nodules and corneal nerve changes. dominant with high penetrance. No treatment is
Symptoms include persistent headache, fever, and Neurofibromatosis of the lid is associated - Sturge-Weber Syndrome available. The prognosis is very poor, with a
vomiting. Increased intracranial pressure may rarely with unilateral infantile glaucoma. progressive downhill course and death in
dexMop-rapidly. Papilledema is present Localizing Intracranial gliomas may be associated with This uncommon disease is recognizable at adolescence the rule.
cefebntfjsigns of cerebral abscess are the same as neurofibromatosis. Spinal cord neurofibromas ; birth by a .characteristic nevus flammeus (port wine
fat cerebral tumors'. Diagnosis is more difficult frequently occur. The acoustic nerve is the cranial stain type of angioma) on one side of the face
today lifecause of the masking effect of inadequate nerve most commonly involved and produces the following the distribution of one or more branches
antibiotic therapy. Treatment consists ofsystemic syndrome of the cerebellopontine angle. of the fifth cranial nerve. Unilateral infantile CEREBROMACULAR DEGENERATION
antibiotics and surgical drainage of the abscess. Bone development is affected when the tumor glaucoma on the affected side frequently develops
involves periosteum. Pulsating exophthalmos | if there is extensive involvement of the eye with
i occasionally occurs when an osseous defect of hemangioma of the choroid. Lid and conjunctival Genetically determined (autosomal recessive)
the posterior orbit is present involvement nearly always implies ultimate neuronal lipid storage diseases of the brain may
* * * Orbital or intracranial surgery may be needed intraocular involvement and glaucoma. Extensive affect the neural elements of the retina as well.
to remove tumors for functional or cosmetic venous aneurysms in the meningeal sheaths The clinical forms are classified mainly by the age
reasons. When lesions are confined to the skin, extending into the brain substance account for at onset. The pathologic changes are present
the prognosis is good. Intracranial and intraspinal the high incidence of CNS disturbances, ofwhich prenataUy, with clinical manifestations occurring
=i; THE PHAKOMATOSES lesions are usually multiple and have a bad jacksonian convulsive seizures are the most as a critical level, of intraneuronal lipidosis is
V prognosis. The disease tends to be fairly common. These cranial lesions are on the same reached. A definite diagnosis cun be established
stationary, with only slow progression over long side as the skin lesions and usualLy become readily by rectal biopsy or appendectomy showing
periods of time. manifest with in the first decade. Radiographically, ganglioside accumulation even before clinical
J !
i TJjicsc 4 disease entities are logically grouped calcification in the cerebral cortex is usually signs are present Five forms of cerebromacular
together because they arc characterized by a present. The disease follows an autosomal degeneration (ganglioside lipidosis) arc
co'ipbijjation ofskin1 and CNS lesions with frequent Angiomatosis Retinae dominant hereditary pattern with variable recognized: congenital, infantile (Tay-Sachs), late
oculariinvolvemenL All are genetically determined (Von Hippel-Lindau Disease) expression. There is at least one study reporting a infantile, juvenile (SpielmeyerVogt), and adult.
asjabtqsomal dominants. 22 trisomy on cytogenic study. Severe mental and physical deterioration
This rare disease occurs most commonly in There is no effective treatment of Sturge- occurs, usually causing death within a few years.
men in the third decade but can appear at any age Weber syndrome, although the glaucoma can be
! ! 'i: ;
Neurofibromatosis (Recklinghausen’s Disease) up to 60. The earliest signs are dilatation and controlled in rare cases by cyclodiathermy. Other
The later the onset, the milder the disease. The
liver and spleen show increased gangliosides. The
I 5 =■ tortuosity of the retinal vessels, which glaucoma operations have been unsuccessful. striking ocular finding of a cherry-red spot in the
j • (Neurofibromatosis is a generalized develop into an angiomatous formation The prognosis for life as well as for sight is macula is seen in congenital and infantile cases.
hereditary disease characterized by multiple tumors hemorrhages and exudates. A stage of ma poor, with death before age 30 the rule. Optic atrophy and retinal pigmentary changes aie
of thdtskin, CNS, peripheral nerves, and nerve
138 139
frequently present in the juvenile and adult forms. and, very rarely, through the skin. the diet is deficient in vitamin B complex, Inlay
Extraocular muscle dysfunction is a less frequent There is a marked destruction of the ganglion develop centrocecal scotomas that are usually* of
30
finding in all forms.
cells ofthe retina as well as degeneration ofnerve constant density. When density of the scotoma
The exact enzyme abnormality is still not fibers in the optic nerve, occasionally extending varies, the most dense portion usually;lics
known, but a deficiency of serum fructose-] - well past the optic chiasm in severe cases. between fixation and the blind spot.
phosphate aldolase is present in carriers as well Heavy drinking with pr withoutjhdavy
as affected persons. smoking is most often associated with the poor
Clinical Findings nutritional state. Occasionally, there is aihistory
of heavy smoking without drihking. j '
A. Symptoms and Signs: Acute symptoms Bilateral loss of central vision is present in
NIEMANN-PICK DISEASE appear within 18 hours of ingestion. Weakness, over 50% ofpatients, reducingivisual acuity,below
(Sphingomyelin-Sterol Lipidosis) anorexia, nausea, vomiting, headache, dizziness, 20/200. Most of the others have severe Central
Kussmaul respiration, and pain in the back, loss in one eye with some deficit, often ab.Sut 20/
extremities, and abdomen may occur in succession 50 visual acuity, in the better eye. Centrajjyisual
This entity is quite similar to the ganglioside or almost simultaneously. Extensive exposure will fields reveal scotomas which nearly always include
lipidosis. There is a deposition of glycolipid in lead to delirium, convulsions, coma, and death. both fixation and the blind spot (centrocecal
the ganglion cells of the brain and retina. The Visual disturbances range from “spots before scotoma). Pallor of the optic disks may be present.
spleen, liver, and other reticuloendothelial organs the eyes” to complete blindness. The field defects Loss of the ganglion cells of the macula arid
are massively infiltrated with glycolipid. are quite extensive and nearly always include the destruction of myelinated fibers of the optic nerve,
Inheritance is autosomal recessive, and 2 clinical centrocecal area (Fig 11-13). and sometimes of the chiasm as well arethcl main
forms are recognized. The infantile form is the most Hyperemia of the disk is the fiist FIG 11-13. Methyl alcohol amblyopia showing histologic changes. ’ \
common and most severe, with death usually ophthalmoscopic finding. Within the first 2 days very large centrocecal scotoma.VA=hand Chiasmal lesions can cause similar visual field
occurring in 2 or 3 years. A cherry-red spot in the a whitish, striated edema of the disk margins and movements only. changes, but the scotomas generally slop’kt-llie
macula may be present. The juvenile or adult form nearby retina appears. Papilledema can last up to midline, allowing differentiation. Rarely, multiple
is much more benign and usually without eye 2 months, and is followed by optic atrophy of sclerosis, pernicious anemia, methanol poisdning,
findings. mild to severe degree. retrobulbar neuritis, or maculaf. degeneration may
Decreased pupillary response to light occurs Course & Prognosis cause diagnostic confusion. ; ft ’
in proportion to the amount of visual loss. In
MISCELLANEOUS DISEASES OF severe cases the pupils become dilated and fixed. Patients with dilated, fixed pupils during the
NEURO-OPHTHALMOLOGIC Extraocular muscle palsies and ptosis may also acute attack usually die. If they survive, they have
IMPORTANCE occur. severe visual loss. Patients with retinal edema
usually have moderate to marked permanent visual
B. Laboratory Findings: The most important loss. The initial loss of vision shows an early
laboratory determination is the blood CO2 improvement that may be only transitory. Visual
AMBLYOPIA DUE TO METHANOL combining power. A reduced CO., combining improvement occurs only during the first week; if
power (below 25 mEq/liter) indicates acidosis, little or no immediate improvement occurs,
(METHYL ALCOHOL) POISONING
which requires alkali therapy; a level below 10 mEq/ eventual optic atrophy with very low visual acuity
liter is critical. is the rule. Only in mild cases is normal visual
function regained.
Methyl alcohol (wood alcohol) has long been
used as an intoxicating drink, either by mistake or
because ethyl alcohol was not available. It may Treatment
be mixed either accidentally or purposely with
ethyl alcohol. Its breakdown product, If ethanol and methanol are ingested or NUTRITIONAL AMBLYOPIA
. formaldehyde, can cause severe poisoning marked inhaled simultaneously, the effects of methanol (Tobacco-Alcohol Amblyopia)
will not become evident until most of the ethanol FIG 11-14. Nutritional amblyopia showing
by gastroenteritis, pulmonary edema, cerebral
is excreLed. If the patient is seen soon after centrocecal scotoma. VA=20/200. $ i
edema, and extensive retinal damage. There are
great individual variations in tolerance; small exposure, gastric lavage should be performed. Nutritional amblyopia is the preferred term
Acidosis should be controlled with large and for the entity sometimes referred to as tobacco-
amounts (1 oz) may cause profound effects in some
repeated doses of bicarbonate. Ethanol inhibits alcohol amblyopia, tobacco amblyopia, or alcohol Adequate diet plus- vitamin’ B
persons while much larger amounts cause no
the metabolic oxidation of methanol; therefore, a amblyopia since they are all the same entity. supplementation is nearly always effective iii
poisonous effects in others. Significant systemic
blood ethanol concentration of 100 mg/100 ml Persons with poor dietary habits, particularly if completely curing the disease; Withdrawal of*
absorption has been reported from inhaled fumes
blood should be maintained until all methanol has
140 141
 opacity may persist and markedly reduce vision. may be necessary. In well-established cases,’
tdbaccp'pnd alcoliol is advisable and may hasten, 30
Severe, persistent neuralgia may be a late suturing the lids together temporarily may be the
the cure; blit innumerable cases are known in
manifestation- best way to control keratitis.
vMicll adequate nutrition alone effected the cure
Compiete internal and external
despite continued excessive intake'of alcohol or
tobacco 'Or both.; Improvement usually begins ophthalmoplegia occurs rarely. Incomplete palsy
\yithiri i-2 months, although occasionally of the third nerve is die most common type of
significant improvement maynot occur for a year. involvement. Optic neuritis is rare but is quite MIGRAINE ’
serious if it does occur since recovery offunction
In ribarly all cases, visual function returns to
ndniial; of nearly normal sooner or later; permanent is minimal. Gaze palsies and encephalitis seldom
occur. Migraine is a common episodic illness of
optic atrophy occurs in a low percentage of cases
with long-standing nutritional deficiency. There is no specific therapy for shingles. Eye unknown etiology and varied symptomatology
treatment is directed toward protecting the characterized by severe unilateral headache, visual
exposed cornea and combating secondary disturbances, nausea, and vomiting. It is
infection. Antibiotic ointments are used locally. associated with dilatation of the external carotid
■i AMBLYOPIA DUE TO QUININE & Dilatation of the pupil with homatropine, 2-5%, artery and its branches. Autosomal dominant
. /. RELATED COMPOUNDS puts the eye at rest and is particularly useful if inheritance is nearly always present. The disease
iritis is present. Systemic corticosteroids do not usually becomes manifest between ages 15 and
help, and there are several reports of death 30. It is equally common in the 2 sexes but is often . *
Quinine and ’quinacrine, used primarily in the following theft administration. Postherpetic more severe in women, perhaps because of
FIG 11-15. Quinine amblyopia showing only a
treatment and.prevention ofmalaria, occasionally neuralgia has been treated with a variety ofagents, endocrine factors. Prodromal symptoms are t ; •*
central cone olfield remaining. VA*=20/25.
cause visual disturbances on an idiosyncratic recovery is rare. including the major tranquilizers, with variable common and include drowsiness, paresthesias,
reports of success. “scintillating” scotomas, blurred vision, and other . - • *
basis^ Tpc onset is acute, most often following a
singlejqose. Other symptoms are a feeling of The prognosis is good, and recurrences are symptoms. In’ some patients, homonymous
fullhbs^i in the ftead, ringing in the cars, and almost unknown. hemianopsia can be accurately recorded on the • •*
deafness. There is constriction of the visual field HERPES ZOSTER tangent screen during attacks. The visual
(Fig 1:1-15) and, rarely, total blindness. The (Shingles) symptoms usually last no longer than 15-30
tendency is toward partial recovery, with minutes. Many factors, particularly emotional
permanent peripheral field defects the rule. The N EUROPARALYTIC KERATITIS ones, may predispose or contribute to attacks.
ganglioji cells of the retina are affected first, Infection with herpes zoster virus is Ergotamine tartrate, when given early in an
presumably as a result of marked vasoconstriction characterized by the appearance of vesicles upon attack, is often effective. Once the attack is well
of the retinal arterioles, easily visible with an the skin along the course of a nerve. The virus Loss of function of tbe sensory nerve to the under way, treatment is of little value. The
ophthalmoscope. Varying degrees ofretinal edema has a predilection for the gasserian ganglion and cornea (nasociliary branch of the ophthalmic headaches last from several hours to several days.
early, iifid optic atrophy later, occur bilaterally. the first 2 divisions of the fifth cranial nerve. division of the fifth cranial nerve) can lead to Bed rest is often helpful if not essential.
p}lie most ‘important treatment is drug Severe pain over one side of the face may precede trophic changes in the cornea. Punctate epithelial
Withdrawal, after which there may be some by several days the eruption of vesicles on the lesions appear first, usually as small vesicles
improvement, no change, or gradual continued forehead and eyelids on one side. The vesicles which lead to irritation, photophobia, and ciliary
deterioration ofvisual function. Vasodilators such contain clear fluid which rapidly becomes injection. If the process continues, epithelium is CRANIOSYNOSTOSIS
as amyl .nitrate, acetylcholine, and sodium nitrite purulent. These rupture, leaving ulcers which lost and a corneal ulcer may develop with (Oxycephaly)
maj'favorably influence some cases in the acute usually become secondarily infected and form secondary infection. Iritis, hypopyon, and, at
crusts. Some permanent scarring always results. times, an overwhelming endophthalmitis with loss
phase- 3
The eyelids become red and edematous, and of the eye can occur. Under this general heading are grouped a
there is tearing with scanty discharge. Tbe Neuroparalytic keratitis usually occurs as a number of rare dysostoses causing distortion of
: i• I
j Amblyopia due to salicylates conjunctiva is red and the cornea shows complication of section of the sensory fifth nerve the face and head and having recessive or irregu­
discrete white subepitheliat opacities root in the treatment of tic douloureux, and it also lar dominant inheritance. In oxycephaly the su­
(involvement of the nasociliary branch of the occurs following herpes zoster keratitis, posterior tures of the bones of the face and head close be­
first division of the fifth cranial nerve). Fine fossa tumor extirpations, and other rarer fore the brain growth is complete. Continued
• j ,• ’Salicylic ac!id derivatives in very large doses
dendritic lesions may occur. Corneal sensitivity conditions. growth offrontal bone allows for brain expansion,
cause'rpclinical picture of toxicity which is quite
sinnlariio that caused by quinine. Constriction of is markedly decreased, favoring exposure and Protection of the cornea and treatment of leading to tower skull or other abnormal shapes
the visual field like that seen in quinine amblyopia secondary infection. The keratopathy lasts secondary infection are the only available of the skull. At times the sphenoid bone does not
(Fig- m 15), dilated pupils, tinnitus, and deafness several months and may clear with minimal measures. Antibiotic ointments should be applied develop properly, leaving an inadequate optic
scarring. Complications of keratitis include locally, particularly at night. Protective eye shields canal and constricting the optic nerve and caus-
may aljibe present. Upon withdrawal of the drug,
Visual function usually improves, but complete secondary glaucoma, iritis, and scleritis. Comeal
142 143
ing optic atrophy. Increased intracranial pressure adults age 20-40, though it may occur at any age. steady, and remissions are frequent. During a sudden onset of homonymous hemianopsia,
may result from the inadequate cranial vault vol­ The onset may follow an upper respiratory severe exacerbation, the patient may die from hemiplegia, convulsions, and other signk -and
ume. One variant of particular note is gargoylism infection or an injury, and has been noted as a paralysis of respiration. symptoms has occurred in persons; in good health
(Hurler’s disease), a form of dwarfism with ox­ transitory condition in newborn infants of The prognosis depends to a great extent receiving no other medication. Warnings against
ycephaly and hypertelorism (widely spaced eyes). myasthenic mothers. The disease has been upon the patient’s response to medication and the use'of such drugs in persons jwith a histdijy
Gargoylism is an autosomal recessive disease due associated with hyperthyroidism, collagen his ability to regulate his medication. An of thrombophlebitis or other vascular problems
to overproduction of mucopolysaccharides.Acid, disease, and diffuse metastatic carcinoma. intelligent patient, well oriented to his disease, were issued by the drug industry prior jto
mucopolysaccharides and glycoproteins are de­ Accumulating evidence suggests that an can live a normal life span. issuing oral contraceptives. Migraine has alsb
posited in the cornea, abdominal organs, and CNS, autoimmune mechanism is a significant factor.
been noted to develop in persons taking oral
causing death before age 20. The differential diagnosis includes contraceptives, and migraine sufferers should
progressive nuclear ophthalmoplegia, brain stem be advised against using such drugs. The Glj{S
lesions, epidemic encephalitis, bulbar and CENTRAL NERVOUS SYSTEM effects have cleared in some cases and.haVe
pseudobulbar palsy, and postdiphtheritic
GQfiO.J.CAT10NSQEUSEOEPRAL resulted in permanent neurologic deficits'in
MANDIBULOFACIAL DYSOSTOSIS paralysis. CONTRACEPTIVES others. While the percentage of Complications
(Franceschetti’s Syndrome) Although substantial neurophysiologic reported so far is very low, there is no longer any
evidence indicates that the site of the disorder is question about the relationship. is important
the neuromuscular junction, convincing evidence Since 1964, a number of reports have for physicians to be aware of the possibility;of
Patients with this rare disorder have a of morphologic changes has not been presented. appeared of cerebrovascular accidents in younger the complications that can occur with such a xi/idely
characteristic antimongoloid facies with a It is known that there is insufficient utilization or women taking birth control pills of all types. The used group of drugs.
temporally placed noLch in the lower lids, acetylcholine at the motor end plate. Thymomas
hypoplasia of some facial bones, high palate, and have been reported in 25% ofmyasthenic persons r. - i ’

other less striking facial changes. The disease is over age 35. About half ofpatients with thymomas ir

genetically determined as an irregular dominant. have myasthenia gravis. The 2 diseases must be . I n
; t J
related, but the mechanism remains obscure. •. i H
i l
Cholinesterase destroys acetylcholine at the
myoneural junction, and cholinesterase-inhibiting
WAARDENBURG’S SYNDROME drugs (neostigmine) markedly improve the
condition. The edrophonium chloride (Tensilon)
test is used in addition to the neostigmine-atropine
This rare entity is distinguished by wide diagnostic test Edrophonium, 2 mg (0.2 ml), is
separation of the inner canthi, broad nasal root, given IV over 15 seconds. Relief of ptosis
heavy eyebrows, heterochromia of the iris, white constitutes a positive response and confirms the
forelock, and congenital deafness. It is inherited diagnosis of myasthenia gravis. If no response
as an irregular dominant. occurs in 30 seconds, an additional 8 mg (0.8 ml)
are given. The test is most critical when marked
ptosis is present. It is best performed with the
patient looking upward, as this most effectively
MYASTHENIA GRAVIS demonstrates improvement in levator action (30-
60 seconds after injection).
Neostigmine bromide (Prostigmin) remains
This disease, characterized by ease of the drug of choice in most cases. A typical dose
fatigability of the striated muscles, often is first is 15 mg 4 times daily. Pyridostigmine (Mcstinon)
manifested by weakness of extraocular muscles. is also widely used. Topical anticholinesterase
Unilateral ptosis is a frequent first sign, with drops, especially demecarium bromide (Humorsol), i

subsequent bilateral involvement of extraocular sometimes help with ocular signs, which are often
muscles so that diplopia is often an early symptom. particularly resistant to systemic therapy. Ptosis
Generalized weakness of the arms and legs, usually does respond to treatment, but extraocular
difficulty in swallowing, weakness of jaw muscles, muscle weakness often does not respond.
and difficulty in breathing may follow rapidly in Thymectomy appears to be of benefit for patients
untreated cases. There are no sensory changes. with generalized muscle weakness.
The disease is not rare. It usually affects young The course of this chronic disease is not
h
144 145
 12 follows: medial rectus, 5 mm; inferior rectus, 6
nini; lateral rectus, 7 mm; and superior rectus, 8
light, if the corneal reflection is centered on the
pupil, die visual axis and the central pupillary
nim (Fig 12-1). line coincide and the angle kappa is zero.
STRABISMUS B. Oblique Muscles: The 2 oblique muscles Ordinarily, the light reflex is 2-4 degrees nasal to
control primarily torsional movement and, to a the pupillary center, giving the appearance of
; P, lesser extent, upward and downward movement. slight exotropia (positive angle kappa). A
The inferior oblique muscle arises from the nasal negative angle kappa gives the false impression
d >d 'i orbital wall several mm behind the orbital rim; it of esotropia.
•5 -G n
passes under the inferior rectus and curves
did
j; U< i around the eyeball, making a large arc of scleral Ductions: Monocular rotations (other eye
|| s contact, and inserts in the posterior lateral covered).
jt |j‘ Unjdcr normal conditions the image of the ANATOMY quadrant of the eye just under the lateral rectus Adduction: InwaTd rotation.
object-of regard falls on the fovea of each eye. muscle. The main muscle body of the superior Abduction: Outward rotation.
When me eyes are positioned so that the image oblique muscle originates from the annulus ofZinn Supraduction: Upward movement.
fblislunon the fovea of one eye but not the other, Muscles just above the origin of the superior rectus muscle Infraduction: Downward movement.
jtjji. Second eye is deviating (squinting) and and passes to the cartilaginous pulley (trochlea)
|str^i>idmus is present. The deviation may be attached to the nasal side of the superior orbital Fusion: The cortical integration-of the images
Six extraocular muscles control the movement
|in\yardL| outward, up, or down. The amount of rim. At the pulley it is reflected downward, outward, received simultaneously by the 2 eyes.
of each eye: 4 rectus and 2 obi ique muscles.
Ideyiptiph is a measurement of the angle formed and posteriorly, passing under the tendon of the
A. Rectus Muscles: The 4 rectus muscles ’
sby-tlic visual axes of the 2 eyes. superior rectus muscle to insert into the sclera. In Heterophoria (orphoria): A deviation of the eyes
originate at a common ring tendon (annulus of
| p I 5” Strabismus is present in about 3 % of chi Idren. the primary position, the muscle plane of the corrected by the fusion mechanism.
Zinn) surrounding the optic nerve at the posterior-
iTreh'tnidnt should begin as soon as the diagnosis superior and inferior oblique muscles forms a 51 Esophoria: Tendency for one eye to turn
apex of the orbit. They are named according to ■
ds; definite in order to ensure the development of degree angle with the optical axis. inward.
their insertion into the sclera on the medial, lateral; •
jtiidhesj possible visual acuity and a good cosmetic inferior, and superior surfaces of the eye. The" Exophoria: Tendency for one eye to turn
result apid increase the chance for normal binocular muscles are about 40 mm long, becoming
outward.
iViSiual 'function.- The idea that “the child may Innervation Hyperphoria: Tendency for one eye to deviate
tendinous 4-6 mm from insertion, and are about 10
quigroiw his crossed eyes” should be mm wide at the point of insertion. The approximate upward.
'discouraged. distances of insertion from the comeal limbus are.
The abducens nerve innervates the lateral Hypophoria: Tendency for one eye to deviate
rectus muscle; the trochlear nerve innervates the downward.
Superior superior oblique muscle; and the oculomotor
rectus nerve innervates the other 3 rectus muscles and Heterotropia (ortropla): Strabismus, or “squint”;
the inferior oblique muscle. deviation of the eyes not corrected by the
fusion mechanism.
Esotropia: “Crossed eyes”; convergent
fascia strabismus.
Exotropia: “Wall eyes”; divergent strabismus.
The rectus and oblique muscles are Hypertropia. Deviation of one eye upward.
Lateral ensheathed by fascia. Near the points of insertion Hypotropia: Deviation of one eye downward.
Medial
rectus rectus of these muscles the fascia is continuous with Bi' common usage one usually refers to a
Tenon’s capsule, which is between the sclera and vertical deviation in terms of hypertropia
conjunctiva. Fascia! condensations to adjacent rather than hypotropia.
orbital bony structures serve as check ligaments
RIGHT EYE for the extraocular muscles and limit ocular Orthophoria: The absence of any tendency of
rotation. either eye to deviate when fusion is suspended.
This state is rarely seen clinically. A small degree
of phoria is “normal.”
Inferior
rectus
DEFINITIONS Primary deviation: The deviation measured with
the normal eye fixing and the eye with the
paretic muscle deviating.
; jRftS 12-1. Approximate distances of the rectus muscles from the limbus, and the approximate ■' .Angle kappa: The angle between the visual axis
’ -lejiglits of tendons. and the central pupillary line. When fixing a Prism diopter (D): A unit to measure deviations.
146 147
 One D is that strength of prism that will deflect TABLE 12-1. Functions of the ocular muscles process regardless of the alignment of (fie eyes,/
~ movers are termed yoke muscles. In any conjugate
a ray of light 1 cm at a distance of 1 meter. The and therefore is classified into 3 grades Dr'clegridql.
. movement, the yoke muscles receive equal
deflection is toward the base of the prism. Muscle Primary Action Secondary Acthj
innervation (Hering’s law), 'lable 12-2 lists the yoke
Another commonly used, unit, a degree (° ), L&tcril rectus Abduction None ■
equals about 2D. Media! rectus Adduction None '
muscle combinations.
Sensory Changes in Strabismus

Secondary deviation: The deviation measured

Superior rectus
Inferior rectus
Superior oblique
Elevation
Depression
Depression
Adduction, intonii,//1..
Adduction, exto^
intorsion, abducti^
IT
Up to the age of 6 or 7, the sensory pattern of
TABLE 12-2. Yoke muscle combinations. - - . L . t • , L? i
with tlie paretic eye fixing and the normal eye Inferior oblkjue Elevation Extrusion, abduedkv'.
the eyes is not entirely fixed and the eye is sapablq
deviating. ofadjusting to new mechanical alignments. Ifonq
eye deviates, the image of an object obsfc veftbvi
Torsions: Wheel-like motion of the eye on its Field of Action Csdind Direc-
Yukfl Maudes
the nondeviating eye falls on an extrafoveal retinal
anteroposterior axis. rionof Gsze
area ofthe deviating eye. If the sensory cquditipiis
Right superior rectus and left inferior oblique
Intorsion (incycloduction): Torsion of The field ofaction of a muscle is that direction By« up, right are normal, diplopia will result tThc fove!a ciftbiq
Right literal rectus wid k ft medial rectus
superior limbus toward the nose. in which its primary action is greatest. Every; Eyes light
deviating eye will also be directed toward nnoihei-
Eyes down, right Right Inferior rectus and left superior oblique
Extorsion (excycloduction): Torsion of movement of the eye involves the cooperation of object in space, and this second object |Wili be
£ye-i down, left Right superior oblique and left inferior rectus
superior limbus away from the nose. all the muscles (each contracting or relaxing as its Byes left Right medial rectus and kft lateral rectus perceived as if it were superimposed upon the
antagonist relaxes or contracts), but in each of Eyes up, left Right infedor oblique and kft tuperioT rectus object seen by the nondeviating eye. This causes!
Vergences (disjunctive movements): Movement the 6 cardinal directions of gaze there is always= canfusion of images. Under these conctitiohs of
of the 2 eyes in opposite directions. one muscle of each eye whose pull predominates; diplopia and visual confusion, suppressiori rapidly
Convergence: The eyes turn inward. occurs. Suppression consists of the devellpptnen
The Evolution of Binocular Movement
Divergence: The eyes turn outward.
of a scotoma which involves the macula as wel I as
Synergistic & Antagonistic Muscles • The movements of the eyes at birth are the point on which the image of regard hills (th)
Versions: Binocular voluntary movement of the (S herrington ’ s Law)
iTTegular and uncoordinated. By 4 weeks of age, image of the object being fixed by the dominap
eyes in conjugate gaze.
the conjugate fixation reflexes are sufficiently eye). Suppression exists only under bi jopulai
Dextroversion (levoversion): Movement of the Two or 3 muscles of the same eye work .- developed so that the infant’s eyes follow a slowly conditions and is a method of obtaining relieffrorr
eyes to right (or left). together to produce a certain movement. In;f •moving light By 3 months of age -the eyes will the diplopia and confusion caused iby. the
Supravertfon (infraversion): Movement ofthe elevation, for example, the superior rectus and deviating eye. With the nondeviat'.ijig ejje
eyes up (or down). follow any moving object, but occasional
inferior oblique muscles are synergistic. deviations or “wandering” eye movements are covered, there is no discemiblejloss of yasipri o|
Dex/rocycZoversmn: Torsional movement of Synergistic muscles for one function may be', demonstrable scotoma. Under binoctfthr
seen until age 6 months. If a deviation is still
both eyes to the right (clockwise). antagonists for another, eg, the superior rectus /; present after 6 months, the child has strabismus conditions using suitable testing apparatus,.
Levocycloversion: Torsional movement of both and inferior oblique are synergists for elevation: “suppression scotoma” can be elicited, = I; H i I
and should be under the care of an
eyes to the left (counterclockwise). but antagonists for torsional movement since the If monocular strabismus persists untrealec^
ophthalmologist.
superior rectus causes intorsion and the inferior suppression will usually deepen into nmjjlyppia
oblique extorsion. When a muscle is stimulated, / of the deviating eye. In this case; the visilal acuiQ
its antagonist is simultaneously and equally may be reduced to finger cduntingl qnl/y dr
PHYSJQLOGYlMOTQRASPECTS inhibited (Sherrington’s law of reciprocal PHYSIOLOGY: SENSORY ASPECTS of hand mnxr»mnn(o
perception rxPUanzl movements. i• I-|: -i !
innervation). Thus, in dextroversion (eyes turning <
i.ii 1
to the right), the right medial and left lateral rectus I • :: t
Individual Muscle Functions muscles receive inhibitory impulses that cause Binocular Vision Abnormal (Anomalous) RctinalCorrcspondcnce
them to relax. i| -J
The lateral rectus muscle has the lone An extrafoveal area of the deviating eye may
In normal binocular vision, the image of the
function of abducting the eye; the medial rectus I'ahdad-Y
adapt to give a new scuse of “straight [aheacfY
object of regard falls on the 2 foveas. The
muscle, that of adducting the eye; the other Yoke Muscles (Hering’s Law) The fovea of the fixing eye and the exttafpyeal
impulses travel along the optic pathways to the
muscles have both primary and secondary actions area of the deviating eye will then experience i
occipital cortex, where a single image is perceived.
which vary according to the position of the eye. In coordinated eye movements, a muscle of common visual direction. This isjcalled ap&maioui
is pal led anirnalouh
This is known as fusion. With normal use of the
The elevation-depression actions of the one eye is paired with a muscle of the opposite retinal correspondence
retinal and represents
correspondence and represents ji a cni'di
crude
eye, the fovea looking at an object in space has a
superior and inferior rectus muscles increase as eye to produce movement in the 6 cardinal attempt at binocular vision i «the presence wf
visual direction of “straight ahead.” The foveas
the eye is abducted; the elevation-depression directions of gaze. (Eyes straight up and eyes down have a common visual direction and are the strabismus. In alternating strabismus theiselisory
actions of the superior and inferior oblique arc not considered primary directions of gaze since patteni changes, depending ...ion which eye is
principal corresponding points. An extrafoveal
muscles increase as the eye is adducted. no single pair of yoke muscles is primarily fixing, so that when the right is being jpsod for
point (or area) of one eye having the same visual
responsible for this action.) These paired primary’ direction as an extrafoveal point of the other eye fixation the left eye is suppress-. L (and vibe vc(s:-|).
is called a corresponding point. Fusion is a relative
148 149
Eccentric Fixation OBJECTIVES & PRINCIPLES OF therapy to obtain 2 straight eyes with good vision CLA.SSIF1CATIQN OF STRABISMUS
;i = > !
THERAPY OF SJ RABISMl JS but without good fusion. (Absence of fusion is
In eyes with amblyopia, an extrafoveal area not a serious handicap, affecting depth perception
is usually employed for fixation even when the and estimation of distance to a limited degree.) A. Esotropia:
doininant eye is covered (monocular conditions). There are 3 main objectives in the treatment A' 1. Nonparalytic (comitant)-Thc angle of
This is termed cccdntric fixation. Itmay be a retinal of strabismus: Occlusive therapy (“patching”) may be deviation is constant in all directions of gaze.
arca-hear the fovea and detectable only with (1) Good visual acuity in each eye, achieved ■ • required for variable periods depending upon the a. Nonaccommodative
special pieoptic instruments, or itmay be an area by occluding the good eye to force the child to 0ge at which strabismus is discovered and the b. Accommodative
disjtaifi from the fovea depending upon such use the deviating eye or, in some cases, by means ' presence of amblyopia. It is essential to secure c. Combined accommodative and
variables as angle of deviation, duration of of pieoptics. : (and maintain, if necessary by frequent urging) nonaccommodative
strabismus, age, and other poorly understood (2) A good cosmetic appearance. The eyes the complete cooperation, of the child and his 2. Paretic (noncomitant)-The angle of
factors. Gross eccentric fixation can be readily can be “’straightened” by surgery or spectacles, • parents; it must be carefully explained to them deviation varies in different directions of
identified clinically by occluding the dominant eye or by a combination of both. that this simple procedure willprevent the loss of gaze.
and directing the patient’s attention to a light (3) Binocular vis ion (fusion, stpreopsis). This . vision in one eye. Patching is effective up to age
sourde held directly in front of him. An eye with also depends upon surgery, orthoptics, and ' 7, In older children, pieoptics may be effective. B. Exotropia;
gross eccentric fixation will not point toward the refractive lenses, and often is an unobtainable •? Surgical correction of muscle imbalance 1.Intermittent
ligfit;source but Will appear to be looking in a goal. should not be undertaken until maximal visual 2. Constant
different directioni Poor visual acuity and cosmetic defects are \ acuity has been restored by means ofpatching or
easier to correct if the child is seen early; the ideal' • ? pieoptics. (It is important to emphasize that C. Hyperfropia:
i age at which to begin therapy is 6 months. Normal JZ strabismus surgery does not correct the visual 1. Paralytic
fusion is difficult to obtain unless the child has / disturbance nor the underlying cause, of ocular 2. Nonparalytic
already developed his powers of binocularvision deviation, but merely the cosmetic deformity.) T\vo
before the onset of strabismus; it is the ideal--, or more operations may be required before the
objective of therapy but is attained less than half '■ eyes are straightened, since there is no
the time. It is a satisfactory result in strabismus > mathematically precise relationship'between the
number of mm the surgeon will recess or resect ESOTROPIA
and the number of degrees of deviation corrected. (Convergent Strabismus, “Crossed Eyes”)
However, in each case the surgeon can usually
make a good estimate of how many millimeters he
should recess or resect; these clinical estimates
are based on the degree of deviation and the size Esotropia is by far the most common type of
of the muscle as observed at surgery. strabismus, comprising about 75% of cases. It is
In most cases it does not greatly matter divided intD paralytic (due to paresis or paralysis
whether surgery is performed on the straight eye of one or more extraocular muscles) and
or the deviating eye, but the deviating eye is nonparalytic (comitant). Comitant esotropia is the
usually chosen since this is easier to explain to most common type in infants and children; itmay
parents. be accommodative, nonaccommodative, or a
In general, strabismus surgery can be combination of both. Paralytic strabismus
regarded as a type of calculated “trial and error” comprises nearly all cases of adult and a minority
surgery which creates an artificial defect in one or of childhood strabismus.
more extraocular muscles in an effort to compensate
Primary deviation Secondary deviation (right eye fixing; for an existing defect of unknown cause (probably,
(loft eye fixing) "inshoot“ of sound left eye) . in most cases, neural).

: l 5 i
FIG 12-2. Paresis of horizontal muscle (right lateral rectus). Secondary deviation is greater than
primary deviation because of Hering’s law. With the left eye fixing, the right eye is deviated inward
because of the paretic right lateral rectus. For the right eye to fix, the paretic right lateral rectus muscle
must receive excessive stimulation. The yoke muscle, the left medial rectus, also receives the same
excessive stimulation (Hering’s law), which causes “inshoot” shown above.

150 151
 NONPARALYTIC ESOTROPIA frequently more marked in one eye . expectations. It is more widely used in Europe than and his parents should be warned that 2 or mpr.e
(anisometropia). Adduction is commonly :; in the USA. A wide variety of technics have been operations may be necessary before a good result
increased and abduction decreased in one or both ■ utilized, all designed to disrupt eccentric fixation is obtained. One of the best opportunities to
eyes. Convergence may be unaffected. Visual and establish foveal fixation. One frequently used improve fusion occurs just before and just after
1. NON ACCOMMODATIVE ESOTROPIA acuity is not affected in alternating esotropia, but ■ technic is based on stimulating the dormant fovea strabismus surgery since the sensory status is
it usually becomes reduced in the deviating eye Awhile discouraging the eccentric fixation point by made temporarily more flexible by strabismus
in monocular esotropia. The deviation usually ‘ dazzling and blocking out appropriate retinal areas. surgery. j !»
More than half of all cases of esotropia fall varies only slightly when the eyes are focused on ■ ? : The entire parafoveal area out to 30 degrees is
into this group. In most cases the cause is obscure. near and distant objects, and correction of the dazzled with an ophthalmoscope light source . i
Characteristically, the convergent deviation is refractive error does not appreciably affect the- ’; which has a central dark shield to protect the
manifest early in life, usually by the first year and deviation. ::V jnaculaffom the dazzle. After the light is removed, 2. ACCOMMODATIVE ESOTROPIA
often at birth. By definition, the deviation is If the strabismus has been present for several • the macula stands out as a positive after-image. i . i ’;
coroitant, ie, the angle of deviation is months, alternating esotropia will usually result Shortly thereafter, a negative after-image becomes
approximately the same in all directions of gaze in alternate suppression and often abnormal retinal ' apparent to the patient The patient is then taught About a third of cases of esotropia falliintp
and is not affected very much by accommodation. correspondence (but not amblyopia). Monocular that the afterimage is in the straight-ahead this group; another 15-20% have sonic
The cause, therefore, is not related to the refractive strabismus is most likely to cause amblyopia. position, and in this way foveal vision can be accommodative factor. TheSe patients'arc
error nor dependent upon a paretic extraocular gradually reoriented to the straight-ahead hyperopic, usually 2 diopters or more. For tliis
muscle. It is probable that some cases are due to position. Many concentrated hours of pieoptic reason they must accommodate for clear distance
anomalous insertions of the horizontally acting Treatment work are required to attain proper macular and vision. Accommodation is associated within
muscles, abnormal check ligaments, or various foveal orientation so that improved visual acuity certain amount of convergence; the amount df
other fascial abnormalities. It is also quite likely A. Occlusive Therapy: The objective of ‘ can occur. Some patients respond fairly quickly, convergence is too great to be overcome by the
that many cases are due to faulty innervational occlusive therapy is to equalize the visual acuity but others require months or years of treatment available fusional amplitude, convergent
development, especially of the supranuclear in both eyes. Early diagnosis followed by patching ■ For ideal results, hospitalization is necessary-an strabismus results. Accommodative esotrbpra
pathways for controlling convergence and the good eye is the best means of preventing . almost prohibitive financial factor in the USA. The sometimes takes the form of esophoria for distance
divergence. amblyopia. However, even with patching and : . average visual improvement is from 20/200 to 20/ and esotropia for near. In these cases there is, an
There is also good evidence that “idiopathic” successful surgical correction, many patients 70, but some exceptional cases have improved from overresponse of convergence in association with
strabismus may occur on a genetically determined (especially those who have strabismus at birth) hand movement visual acuity to 20/25. Even many accommodation. ji
basis. Esophoria and esotropia are frequently do not develop normal fusion. In general, the of these apparently successfully treated patients : 11
passed on as an autosomaL dominant trait. Siblings earlier the deviation is discovered and the fixing have reverted to previous visual levels shortly
, often have similar ocular deviations. At other times, eye patched, the sooner will the deviating eye after cessation of treatment. Clinical Findings
when there is no apparent family history, the develop useful visual acuity. Even a few days of The best age group for treatment is from age
parents of an esotropic child may be found to patching may be sufficient prior to age 1, whereas 7-14, but a few remarkable results have been The onset of this type of esotropia is
have significant esophoria, and have passed on several months may be necessary by age 5. After . reported on amblyopic eyes of elderly patients characteristically between 18 months and 4 ycafcs
the defect in a more severe form. An age 7, only pieoptics can help develop or restore who have lost the vision of the dominant eye from of age (because the faculty of accommodation iis
accommodative element is often superimposed visual function, and this is a very time-consuming some organic cause. not well developed until then). The deviation !is
upon comitant esotropia, ie, a correction of the procedure in which there is no assurance of most often monocular but may be alternating. The
hyperopic refractive error corrects some but not success. C. Surgical Treatment: There are basically 2 hyperopic refractive error is usuajly from +2 fo +5
all of the deviation. Occlusion has a favorable effect upon surgical approaches to the correction of diopters. Sensory findings reveafthat suppression
abnormal sensory patterns but rarely on the strabismus: strengthening a muscle or weakening develops rapidly in the deviating eye, but notrnnl
degree of deviation. When nothing further can be a muscle. In the case of the horizontal muscles, retinal correspondence is usually retained. I*
Clinical Findings accomplished by occlusion, surgical realignment the strengthening operation is usually resection i i;v
of the eyes is indicated. and the weakening operation is usually recession. j.
Aside from the cosmetic defect and the Occlusive therapy may be combined with Other rarely used procedures include tucking and Treatment j •
” <. personality problems it may create in older orthoptics, utilizing the arablyoscope in an effort advancement for strengthening; and various types 5: •'
children, there are usually no symptoms due to to develop better binocular function. The sensory of tenotomy for weakening. Pure accommodative esotropia responds
- r ■ strabismus. abnormalities of suppression and abnormal retinal satisfactorily to correction of'hyperopia with
The deviation may be monocular (ie, the same correspondence are also broken down, if possible, eyeglasses if treatment is instituted within a fairly
eye always deviates) or alternating (either eye may by orthoptic means. Prognosis short time, usually not more than 6 months ajftjir
deviate). The refractive error in alternating onset. If treatment is delayed beyond this time,
< esotropia is approximately the same in each eye; B, Pieoptics: The value of pieoptics as a Strabismus surgery is empirical, and the abnormal sensory' pattern’ is apt to liayc
in monocular esotropia the refractive error is practical therapeutic tooL has not lived upto early ■ consistent results cannot be predicted. The patient become so well established thir. glasses along,will

152 153
hq^correct the strabismus. In this event, treatment comitant esotropia. These cases result from birth
isj'•’generally as outlined above for paralysis of the lateral rectus muscle, EXOTROPIA
injuries or congenital anomalies of a lateral rectus strengthening by resection of the muscle will not (Divergent Strabismus)
n'Oiiaccqmmodative esotropia. If amblyopia has muscle, its nerve supply, or its fascial attaclimeuts * - attain the desired result. The Hummelsheim
developed in one eye, occlusion is indicated, and
operation is preferred, ie, half of the superior
this may so reverse the abnormal sensory pattern
rectus and half of the inferior rectus tendons are Exotropia is less common than esotropia,
tjhatlnonsurgical cure is possible. More frequently, Clinical Findings
transplanted to the insertion of the lateral rectus particularly in infancy and childhood. Its incidence
hbwpvev, surgical treatment will be necessary in
muscle to aid in abduction of the eye. This is increases gradually with age. Not infrequently, a
long-standing cases. In cases which have a partial Ifthe lateral rectus muscle is totally paralyzed, combined with recession of the medial rectus tendency to divergent strabismus beginning as.
dc.c immodative factor, a prescription for the eye will not move temporally beyond the muscle. The surgical treatment of noncomitant exophoria progresses to intermittent exotropia and
hyperopic eyeglasses will reduce the amount of midline. Paralysis of a right lateral rectus muscle esotropia is seldom a total success, but some finally to constant exotropia if no treatment is
dq'v ption and the residual deviation must be causes a right esotropia which becomes more improvement can usually be obtained. Knapp has given. Other cases begin as a constant or
q'oiTfcte# surgically. Bifocals may be prescribed if marked as the eyes attempt to fix an object moving advocated transplanting the entire superior and intermittent exotropia and remain, stationary. As
the. deviation is much greater for near than for to the right ofthe midline. The deviation is absent inferior rectus muscles to the insertion of the in esotropia, there is a strong hereditary element.
chsfapccj. upon conjugate movement of the eyes to the left. paretic lateral rectus. This not only greatly Exophoria and exotropia (considered as a single
j |Long-acting miotics in .weak strengths In children age 6 or younger, suppression improves abduction, but does not significantly entity of divergent deviation) are frequently
(^cljothi'ophatc [Phospholine] iodide, 0.06 or will develop under conditions of deviation, but affect upward or downward movement ofthe eye. passed on as autosomal dominant traits, so that
0U^% solution; or isoflurophate [Floropryl] entirely normal binocular relationships may remain one or both parents of an exotropic child may
ointment, 0.025%) applied once daily have been when the eyes are aligned in the field of gaze demonstrate exotropia or a high degree of
ujje'e . with success in treating accommodative opposite the side of the paretic lateral rectus exophoria.
c's'oC 'opia. In younger children (age 2-4), these muscle. There may even be a change of retinal
drugs can be used instead of glasses if the correspondence from normal to abnormal as one
hyperopia is less than 4 diopters and there is Little passes into the field of strabismus. In adults with
ast||npatism. Miotics are particularly useful in cases a sudden onset of paralytic esotropia, the patient
where hyperopic eyeglasses align the eyes for experiences diplopia whenever an eye deviates,
distance:vision but esotropia remains at near since the sensory pattern is fixed and the object
visiph. (These drugs act by altering the of regard falls on noncorresponding retinal areas.
aqqpqimddative convergence relationship in a
faycirable way so that fusion, is maintained despite
accommodation. In addition, the miotic effect Treatment Primary position: right esotropia
allolVs clear vision with less accommodation both
for near and distance. The treatment of persistent paralytic
esotropia is exclusively surgical. In adults who
have a sudden onset of strabismus, a period of at
iiji least 6 months is allowed to pass before surgery
■PARETIC
■ .... __ _ ESOTROPIA
!' (NONCOMITANT)
(Abducens Palsy)
since the condition may correct itself. During this
period, occlusion ofthe eye with the paretic muscle
is necessary to relieve diplopia.
ft I In children, or in adults who have had no
;> I ,In noncomitant strabismus -there are always
oh'c'of more paretic extraocuiar muscles. In the
improvement after 6 months, surgical treatment is
indicated. In paresis of the lateral rectus muscle, I
cas^j'pf noncomitant esotropia, the paresis is an attempt may be made to strengthen this muscle I
alSya^s oil one of the lateral rectus muscles, usually by resection, sometimes combined with
as a result of palsy of die abducens nerve. These
castes arc most often seen in adults who have had
advancement of the insertion. In this operation
the resection is carried out as described for
« 2:
c^broviascular accidents or diabetes, but nonparalytic esotropia. Placement of the sutures
abducens,palsy may occasionally be the first sign anterior to the original insertion effects an Left gaze: no deviation
of 4.^jmqr or of inflammatory disease involving advancement. In addition, the medial rectus
theCjNS. llead traumacan also produce abducens muscle of the same eye may also be weakened by
pM i recession to enhance the effect of the surgery.
• Noncomitant esotropia is also seen in infants The type and amount of surgery will depend on
andjipliildren, but much less commonly than the extent of the deviation. In the case of total FIG. 12-3. Nonconcomitant strabismus (paralytic). Paralysis of the right lateral rectus
muscle,with left eye fixing.
154
155
 INTERMITTENT exotropia may be the result of anomalous insertions of (he . little as 10 diopters to 80 diopters or more. myasthenia gravis, multiple sclcir.dsis,
extraocular muscles, other defects of the muscles In children age 6 or under with alternating thyrotoxicosis, DTbital tumor, and b'raih istem
or faulty innervation. It has been postulated that exotropia, alternate suppression characteristically disease. Many of the specific enlitits lire
develops. If the squint is monocular, amblyopia considered in the chapter oil rJ
Intermittent exotropia comprises well over in infants and young children a tendency toward neuro-
half of cases of exotropia. The onset of the excessive convergence may be present which is ■ of the deviating eye results. Abnormal retinal ophthalmology. The discussion here will be ( jtnited
Hsi
deviation may not be noted prior to age 2 or 3. controlled by nuclear and intranuclear pathways correspondence is an infrequent sensory change. to “idiopadiic cases,” ie, those due to ppraiy sis o f
'rhe history reveals that the condition has become and held in check by supranuclear influences Eccentric fixation of the deviated eye almost never unknown cause involving one or more M .(he
progressively worse, A characteristic sign is which maintain the proper alignment of the eyes. occurs. Exotropia very seldom has its onset in vertically acting muscles. 1
closing one eye in bright sunlight, as this avoids There probably also is a divergence center with adulthood unless there is significant loss of visual
diplopia. There is usually a manifest exotropia for similar supranuclear influences, and both acuity in the deviating eye, and diplopia is
i
distance. The patient can fuse for near vision, esotropia and exotropia may well result from therefore not usually a troublesome symptom. Clinical Findings
overcoming a moderate to large angle exophoria. abnormalities of the supranuclear influences. Such Surgery is the only treatment available. A
Convergence is frequently excellent. There is no common terms as “convergence excess and practical goal is a good cosmetic appearance with Hypertropia with onset after age 6 jeauses
correlation with a specific refractive error. convergence insufficiency’* and “divergence maintenance of good vision in each eye. In diplopia if both eyes have good visual a’cuity.
Since the child fuses at least part of the time, excess and divergence insufficiency” are merely general, slight overcorrection is attempted since There may be an associated head tilt or-abil inhal
there is usually no gross sensory abnormality. For descriptive. For example, “divergence excess” the resultant small angle esotropia is less likely to xposture
. of
_ the head.
---------------- , I}:
distance, with one eye deviated, there is means an exodeviation, either phoria or tropia, revert to exotropia. If surgery leaves a residual The deviation may vary from slight (a few
suppression but normal retinal correspondence which is greater for distance than for near; small angle exotropia, the eye is apt to deviate prism diopters) to marked. At onset and for a
t and no amblyopia. “convergence excess” means an esodeviation outward again. variable period thereafter, the deviation SyilUbe
Treatment is principally surgical, sometimes which is greater for near than for distance. It is Even in children with good vision in each greater in one position or direction of gt-ze than in
enhanced by preoperative orthoptic exercises or true that the tendency toward divergence eye, a good fiisional result is difficult to obtain. others. Subsequently, heterotropia teiVds/tq
", optical devices such as base-in prisms or becomes greater as age increases. If there is no In monocular exotropia it is usually necessary ——-
become —:*—«. since
comitant, -------------
secondary1---------- -upji;of
contract
excessive minus lenses. Fusion exercises may fusion, as in the case of one blind eye, the blind to operate upon at least the 2 horizontal muscles the direct antagonist and the yoke muscle q.cciirs.
“increase the qualify of fusion” and set the stage eye usually deviates inward in a person up to age of the deviating eye, resecting the medial rectus It is important to determine which muse or
for a better functional result following surgery. 6 and usually deviates outward if the visual loss and recessing the lateral rectus. In large muscles are at fault. This becomes more diflji hill if
The choice of surgical procedure is based occurs after age 6. Many cases of exotropia deviations, 3 or all 4 horizontal muscles may have the disorder is of long duration. Many teS S are
on a number of considerations, the most important develop as a result of loss of vision in one eye, to be altered to straighten lire eyes. available which are of aid in specific muscle
; of which is the amount of deviation and a with subsequent progressive exotropia. diagnosis. In any one case all tests may n'ptI’be
comparison of near and distance measurements. The most important consideration is the consistent, but the bulk of evidence jviliipojnt
'■ . When the deviation is greater for distance than psychic factor resulting from the cosmetic toward paresis of one or more specific miiscl^s.
for near, recession of each lateral rectus muscle appearance. The minimal loss of depth perception Treatment is based upon these dctenniiiati.ous;
gives the best results. If the near deviation is which occurs is seldom of practical importance, HYPERTROPIA The superior oblique is the most freqt tcn.tly
approximately equal to the distance, many even to an athlete. involved, with die superior rectus the next iinpsi
. surgeons prefer a recession of one lateral rectus frequent. Isolated palsies of the inferior recti s■ and
and a resection of the medial rectus of the same inferior oblique are uncommon. j ■;
- . eye. As with other types of strabismus, one Vertical deviations are designated according i -■
operation does not always result in the desired to the eye that is deviated upward. “Right A. Measurement of the Deviation; -,nic ,The
result and a second operation may be required. hypertropia” means that the right eye deviates deviation must be measured in the 6 cardinal
i • upward when the left eye is fixating. The same directions of gaze botii for near and for disknee,
condition could be called a left hypotropia when and with each eye fixing. Secondary de bp .is
the right eye is fixating, but this terminology is larger than primary deviation; therefore,be
CONSTANT EXOTROPIA seldom used. Hypertropia is much less common deviation measured while the eye containing] the
than, horizontal deviation and usually has its onset paretic muscle is fixing will be larger that); that
later in life. measured while die eye with the nonparetic liidscle
Constant exotropia is much less common FIG 12-4. Right exotropia. There are many causes of hypertropia. is fixing. In general, a deviation which jis greater
than intermittent exotropia. It may be present at Trauma may cause paralysis of a vertically acting for near than for distauefe implicates an oblique
birth or may occur when intermittent exotropia Constant exotropia is most often monocular muscle. Abnormal insertions, abnormal fascial muscle rather than a rectus muscle and Viice»yejsa,
progresses to constant exotropia. Some cases have (the same eye always deviating), but may be attachments, and other congenital anomalies may since in adduction (position of the eyes' for- i£ar)
their onset later in life, particularly following loss alternating.-Abduction is frequently increased and also cause hypertropia. the eyes depend more upon the oblique mpsejes
adduction decreased in both eyes, with little or .Complications of systemic disease also than the rectus muscles Jr elevalioii|;.atid
of vision in one eye.
As with esotropia, the cause of exotropia is no convergence. Hypertropia of varying degTee account for a great number of cases, eg. depression. With die eyes i;: the straight-atoMd
usually not known. Congenital constant exotropia is often associated. The deviations vary from as ................ ■
156 157
position (as for distance), the rectus muscles are paretic oblique muscles. The patient fixes a tight This frequently consists of patching one eye. HETEROPHORIA
more prominent as elevators and depressors than at 20 feet (6 meters). The eye to belested may be When hypertropia is due to a complication of a
are the oblique muscles. placed under cover (still visible to the examiner), systemic disease, treatment of the underlying
The deviation in the cardinal directions is but this is not necessary. The head is tilted first disease will materially affect the hypertropia (eg,
measured with prisms. In general, if deviation is toward one shoulder and then the other. An myasthenia gravis). Heterophoria is a deviation of the eyes
greater in one ofthe cardinal directions, this points increased hypertropia is diagnostic of oblique which is held in check by the fusion mechanisir
toward a specific yertical muscle paralysis. For paralysis. B. Surgical Treatment: Many approaches Almost all individuals have some degree c .
example, in right tjypertropia, if the deviation is Example: The right eye is covered (may be are used in the surgical treatment of hypertropia. heterophoria, so that small amounts ai~-
greater with the eyes up and to the right, this left uncovered) and the head is tilted 45 degrees The recent tendency has been to strengthen the considered normal. Larger degrees of
indicates underaction of the left inferior oblique upon Che right shoulder. The eye moves upward. weakened muscle. At times it may be necessary heterophoria are a common cause of “eyestrain** ■■
muscle. • This indicates paralysis of the right superior to weaken the direct antagonist or the yoke (asthenopia), since a strain is placed upon th
oblique muscle. This occurs upon tilting the head muscle, or both. The vertically acting rectus extraocular muscles to overcome the latent
because the 2 incycloductors (right superior rectus muscles can be recessed or resected in the same deviation. From an etiologic standpoint,
and right superior oblique) receive impulses to manner as the horizontally acting rectus muscles, heterophoria and heterotropia differ only i: '•
contract (as above). In addition to their synergistic although within somewhat smaller limits. The degree. The same etiologic concepts that hav<
function of incycloduction, these muscles are superior oblique muscle can be strengthened by been discussed above apply to the 3 types Oi
antagonistic in the function of elevation and tucking the tendon. This effectively shortens the heterophoria. As with heterotropia, many of the
depression; the normal superior rectus (elevation) superior oblique tendon by as much as 10 mm and causal factors are unknown. Heterophoria r
will not be balanced by the paretic superior oblique therefore strengthens the muscle. The superior clinically significant only if it causes symptoms ?
(depression), and the eye will elevate. oblique muscle can also be weakened by intrasheath Symptoms will not always correlate well with
tenotomy in which the sheath ofthe superior oblique the degree ofheterophoria, since the personality
■t •
C. Sensory Changes in Hypertropia: As in tendon is opened and a full tenotomy performed. and occupation of the individual are importan
12-5. Right hypertropia. other forms of strabismus, a sensory adaptation The sheath is then resutured. This gives a fairly in determining the symptomatology.
will take place if the strabismus has its onset before predictable amount of correction, usually about 15 Asthenopia (“eyestrain”) as a symptom ol
B. Head Tilt Qlielschowsky’s) Test: (See Fig age 6. The sensory pattern may vary' with the diopters. The inferior oblique muscle can be heterophoria takes a wide variety of forms.
12-5.) -A person with a paretic oblique muscle may position of the eyes, so that suppression or even effectively weakened by a recession of 8 — 10 mm, or There may be a feeling of tiredness or discom­
tilt bistliead toward one shoulder. This is often abnormal retinal correspondence may be present by myotomy. A number ofother operations that both fort of the eyes varying from a dull ache to deep
•useful#ift diagnosing paralysis of an oblique in the direction of gaze of strabismus and normal strengthen and weaken the vertical muscle are pain located in or behind the eyes. Headaches
muscle. .Normally, ,wben tilting the head toward retinal correspondence without suppression in available but are used less commonly. of all types occur. Easy fatigability, blurring of
the right shoulder, the incycloductors of the right directions of gaze where strabismus is absent If
eye (superior oblique and superior rectus) and the onset is after age 6, there is no sensory
the exeycloductoTs pfthe left eye (inferior oblique adaptation and diplopia is constant.
and inferior rectus) tend to cycloduct each eye to
neutralize the tilled head position. The oblique
muscle predominates in this torsion movement. Treatment
For example, if the fight superior oblique is paretic,
the patient will have a right hypertropia and cany A. Conservative Treatment: In smaller
his head tilLed to the left. This is because the deviations, usually under 10 diopters with
• intorsidnal movement of the right eye is in this relatively comitant strabismus, prisms may
instance produced;by the right superior rectus; neutralize the hypertropia. In the case of a right
its elevating action is unopposed, since its hypertropia of 8 diopters, a 4 diopter prism base
antagonist in vertical movement is the right down, right eye, and a 4 diopter prism base up,
superior oblique. With the head tilted to the left, left eye, may be incorporated into the eyeglasses
the cxpycloductiorj required of the right eye is and fusion reestablished. With larger deviations
accomiplished by the right inferior oblique and or in cases where the strabismus is noncomitant
right inferior rectus. The vertical component is even though not of great magnitude, surgery is
balanced, and no hypertropia is present. Thus, in usually indicated. If there are medical
order to avoid vertical diplopia, the patient will contraindications, such as systemic disease, or if
usually tarry his head lilted to die left. there is a good possibility that the strabismus may FIG 12-6. Head tilt test (Bielschowsky). Left: Paresis ofright superior oblique muscle. Head is carried to ?
like head till test of Bielschowsky utilizes clear or improve subsequently, temporizing shoulder on sound side (left), where fusion is obtained. This is a postural attitude called a “head tilt.”
the above principle to aid in the diagnosis of measures are necessary to relieve the diplopia. Right: When the head is tilted to the shoulder on the paretic side, the right hypertropia becomes
exaggerated. This is a positive forced head tilt test of Bielschowsky. .
158 159
 13
GLAUCOMA
—vpiers for near or
__ asthenopic symptoms.
. oignificant Esophoria for Near and Dis­
tance: If the patient has uncorrected hyperopia, the
,ruen dealing addition of plus sphere to the correction, or the pre­
scription of eyeglasses if not previously worn, may doubtful cases should be
Glaucoma includes a complex of disease en­
^vztisory pattern as determined by the relieve the symptoms. Less accommodation is thus ophthalmologist fot conf,
tities which have in common an increase in in-
inblyoscope is nearly always normal, includ- required both for near and for distance, and so less management.
tra*y?j*£ presenre <n>ffirirnl.ta.cause degenera­
ng normal retinal correspondence and absence convergence is induced. If full hyperopic correction ■ tion of the optic disk and defects in thejyisual
-f suppression or amblyopia. still leaves a significant esophoria, base-out prism fidd.”fhe incidence of glaucomaJn unselected
The prism vergence test determines equally divided in the 2 spectacle lenses may be Classification 3*4^
p'ersons over age 40 isJiciwcc.tdLail(L2% •
vhether an individual has the reserve fusional prescribed. One-half to one-third of the residual de­
The chief threat of chronic (open-angle)
nechanism required to overcome any hetero- viation is usually prescribed, although more than a k generally accepted class
glaucoma is insidious visual impairment. The
phoria that is present. The test is carried out by total of 4 prism diopters is usually poorly tolerated.
degree of interference wiTF^visibnrvancs glaucoma is as follows: ’ f •
asking the patient to fixate on a small light at 20 If symptoms persist and optical methods are unsat­
slight blurring tocompleteblin^SS. The disease \ • ‘
'eet (6 meters) with both eyes uncovered. Prisms isfactory, surgery is indicated. It is usually neces­
is bifateraTand HlUSt be genetically determined, A. Primary Glaucoma:
>f gradually increasing strength or the rotary sary to operate on only one muscle, most often a
recession of a medial rectus muscle. probably by multifactorial or polygenic l.Open-angle glaucoma-Al so cal.
prism of Risley in gradually increasing strengths
are placed before one eye until diplopia occurs inheritance. Infantile glaucoma usually has an glaucoma, wide-angle glaucom;
'“break point”). This is done with both base-in B. Significant Esophoria for Hear and Not autosomal reces&ve mode of inheritance, whereas simple giauebma. TheippMj^pjgj^
aid base-out prism measurements for both near for Distance: In this instance the complaint is some specific glaucoma syndromes are 2Angle-dosurn glaucoma^lso callei
«nd distance. This measures the fusional con­ “tired eyes’ or other symptoms upon reading or transmitted as autosomal dominant diseases. angle glaucoma, closedVhngle gfi
vergence and divergence, respectively. Prisms using the eyes for close work. Treatment by opti­ Acute glaucoma T^gJecTosure glaucoma) acule~CQnggsiive glaucoma.
re used base-up or base-down to measure the cal methods is usually satisfactory. In the case of comprises less"thah 5% of primary glaucoma a. Acute. '
elativc vertical divergence. (Positive vertical a hyperope who has been wearing full correction, cases. b. Subacute or chronic.
divergence if the right eye is directed upward; the addition of more plus sphere for reading (up In most cases, blindness can be prevented if
negative vertical divergence if the left eye is to 2 or 2.5 diopters) will often be helpful. This treatment is instituted early. The objective of B. Congenital Glaucoma:
lirected upward.) In calculating the numerical reduces necessary accommodation and accom­ therapy is to facilitate the excretion of aqueous 1. Primary congenital or infantile glauco>
esults of these tests, one must use the reading panying convergence, so that it is possible to re­ through existing outflow channels by the use of A Iso call ed buphthalmos orhydfophthah
for distance heterophoria as a starting point. duce or nullify the esophoria. In the case of a miotics and in some cases to inhibit the secretion 2. Glaucoma associated with congem
The normal values for prism vergence at 20 feet myopic individual the same principle can be ap­ of aqueous by the ciliary processes, using anomalies-lncludes\types formerly classif.
re as follows: prism divergence, 7 diopters; plied, giving less minus correction. If significant systemically and topically administered drugs. as juvenileglaucoma.
>rism convergence, 20 diopters; vertical diver­ esophoria persists, base-out prism may be added The most commonly used miotic is pilocarpine. a. Pigmentary glaucoma.
gence, 5 diopters. At 13 inches (33 cm), the val­ to the prescription and adjusted until the symp­ Operative treatment is sometimes indicated inthe b. Aniridia. i
ues are as follows: prism divergence, 20 diopt­ toms disappear. In rare instances, if optical de­ later stages when medical management is no c. Axenfel d’s syndrome.
ers; prism convergence, 20 diopters; vertical vices fail, a recession of one medial rectus muscle longer sufficient to control the intraocular d. Sturge-Weber syndrome.
jjvergence, 5 diopters. may be performed. pressure. e. Infantile glaucoma developing late.
The management of glaucoma is best left in f. Marfan *s. syndrome. 1
the hands of the ophthalmologist, but all g. Neurofibromatosis. j
Exophoria physicians should participate in the diagnosis by h. Lowe’s syndrome.
making tonometry and ophthalmoscopy a part of LMicrocomea.
Larger amounts of exophoria are tolerated the routine physical examination of all patients
than of esophoria, particularly for near vision old enough to cooperate. The procedure is simple C. Secondary Glaucoma:
where it is not unusual to have a measurement of and the equipment is not expensive. Tonometry 1- Due to changes of the lens V
7-10 diopters without symptoms. Exophoria above and ophthalmoscopy should be doriFTbutifiely” a. Dislocation.
3 diopters for distance is considered abnormal. by the internist and general practitioner, and b. Intumescence.
160
161

I
 PHYSIOLOGY OF GLAUCOMA ^Tonometry 3
i; ■ c. Phacotoxic or phacoanaphylactic. B. Formation and Flow of Aqueous: A great
’fl. Ex foliative62,syndrome (nseudoexfoliation of deal is known about the dynamics of aqueous
■ ' - lens capsule, glaucoma capsulare). humor, but the exactmechanism ofproduction and This is the most important test in establishing
; e. Spherophakia. Aqueous Humor elimination of aqueous is not completely the diagnosis ofglaucoma since it measures the
■2. Due to changes of the uveal tract- understood. Water, electrolytes, and intraocular pressure. A single normal reading either
■ ■ a. Iridocyclitis. The intraocular pressure is determined bythe jionelectrolytes enter and leave the eye at varying with the Sohiotz or applanation tonometer' does"
; b.Tumor, rate of aqueous production by the ciliary body rates. Water enters both by diffusion from the not rule out glaucoma, however, as the intraocular
> c.Es^ntlaJjrifi_atrophy. epithelium and the resistance to outflow of ciliary body and by secretion from the epithelium pressures may vary within wide limits. A single
;3i Due to trauma- aqueous from the eye. Some knowledge of the of the ciliary processes. From the posterior “hi-rh normal” readin? (24-32 m g) suggests
, a. Massive hemorrhage into the anterior physiology of aqueous is necessary to an chamber the fluid passes through the pupil into glaucoma and requires repeated testing before"
!< '■ chamber. understanding of glaucoma. theanteriorchamber. The flow in the anterior making a diagnosis.
b. Massive hemorrhage into the posterior chamber is peripheral, toward the filtering
■ :, chamber. A. Composition of Aqueous: The aqueous is trabecular meshwork and into Schlemm’s canal
: c. Corneal or limbal laceration with iris a clear liquid that fills the anterior and posterior Efferent channels from Schlemm’s canal (about 30 GoniDscopy
prolapse into tliewound. chambers of the eye. Its volume is about 125jil. collector channels and about 12 aqueous veins)
;d. Rctrodisplacement of iris root following The.QsmjoLic pressure of aqueous is slightly conduct the fluid into theyienous systernTThere This test differentiates angle-closure from
r contusion. higher than that of plasma. The total protein is also a constant exchange ofnonelectrolytes as open angle glaucoma, demonstrates the extent of
4-:Following surgical procedures- con tenths 0.02% fas compared with 7% in blood well as a major exchangeofwater in andjmtnfthft peripheral anterior synechias, and offers the only
>■ ,a.AJ^pitheliaUngrowth.into the anterior serum). The albumin-globulin ratio is the same as iris stroma. (The vessels ofthe iris are impermeable means of detecting an impending angle closure
1 ■ > chamber. that of blood serum (2:1). In general, thesame to ions.) before there is any rise in intraocular pressure. It ‘
jti. FailurgiQfprompt restoration ofthc anterior electrolytes and other components are found in is an essential part of any glaucoma evaluation.
ch amb erfol lowing. cataract extract ion. the aqueous as in plasma, although the
5.1 Associated with rubcosts (diabetes mellitus concentrations differ.
■aind'central retina] vein occlusion). Emptying of the anterior chamber, either by SPECIAL DIAGNOSTIC TECHNICS Provocative Tests
h.Associatcd with pulsating exophthalmos. surgery or trauma or during intraocular
7. 'Associated with topical corticosteroids. inflammatory conditions, causes the formation of A. Dark Room Test of Seidel: The patient
8. Other rare causes of secondary glaucoma. plasmoid aqueous, which closely resembles blood A number of special diagnostic tests have
[B sits in-a dark room for 1 hour without going to
serum and has a mueb higher protein been developed to help detect, classify, and follow sleep. A rise in intraocular tension of more than 8
D. 'Absolute Glaucoma: The end result of any concentration than normal aqueous. the course of glaucoma. mm indicates that iris blockage is impeding outflow
uncbntrqlled glaucoma is a hard, sightless and
pfte'q pai.nfu 1 eye.
X
Cornea

AC -! anterior chamber I ® iris S = sclera TM “ trabecular weshwork

fC ’ =| cornea I*C = iris-corneal angle SC = Schlemm’s canal TI’B = trabecular pigment band
<CB «| ciliary body IP = iris processes SL - Schwalbe’s line
;CP =! ciliary process P « pupil SP c scleral spur

! FIG 13-1. Composite illustration showing anatomic (left) and gonioscopic (right) view of no rmal
< ■ anterior chamber angle. FIG 13-2. Anterior segment structures. Arrows indicate direction of flow of aqueous.
162 163
 * when the pupil is relatively dilated. Positive results indicated in Fig 13-3, Increased intraocular
'•£ are obtained in acute and subacute angle-closure pressure at the nerve head will gradually destroy
glaucoma. The test is usually negative in open- the function of a bundle of these fibers, and the
‘ angle glaucoma. A negative result docs not rule resulting visual field defect is spoken of as u
1 3
- out impending angle-closure glaucoma. “nerve fiber bundle defect.” As the nerve fiber
bundle defect enlarges, it takes an arcuate, shape
B. Mydriatic Test: The same principle is from tbe blind spot eneiTcIingthe fixation arep; it
r ■ utilized as in the dark room test except that a drug arches into either the superior or inferior fieldand
: is used to dilate the pupil. Phenylephrine (Neo- ends at the horizontal meridian (Bjcmim scqtotna).
: Synephrine), 2.5%, or a similar weak mydriatic A double arcuate scotoma (one in the superior
Baring of the Blind spot. The ear­ solution, is instilled into one eye at a time and and one in the inferior field) forms a full-ring
Incipient double nerve fiber bun­ Bjerrum scotoma isolated from
liest nerve fiber bundle defect. dle defeat (Bjerrum scotoma). tonometric readings are taken at intervals of 10 scotoma around the central fixation area. ■ 1
blind spot. . minutes for 1 hour. A rise of 5-10 mm Hg Loss of peripheral field occurs lalerj inllie
constitutes a positive test. The test is hazardous, course of the disease. The nasal and superior
and miotics must be used immediately if there is fields are usually lost first. The last remnant of{the
10 visual field is usually a temporal island. |
4 any evidence of acute glaucoma.
The field of vision may slowly contract in
C. Water Drinking Test: Breakfast is some cases down io 5 degrees from fixation,
withheld and an initial tonometric pressure is leaving the patient with good central vision but
recorded. The pati ent then drinks 1 quart ofwater, no peripheral vision. Central visual hetfity,
and intraocular pressures are recorded after 30, therefore, is not a reliable index of the progress of
End stages in glaucoma field loss. ■ 45, and 60 minutes. A rise of more than 8 mm the disease. There is no substitute for ‘careful
Remnant of central field still Fully developed nerve fiber bun­
indicates a poor outflow mechanism. The test is periodic study of the visual field combined with
shows nasal step. dle defect with nasal step (arcuate
scotoma). usually negative in acute angle-closure glaucoma ophthalmoscopic visualization of the optic disks.
but may be positive in subacute angle-closure The Goldmann perimeter has become increasingly
glaucoma. It is often positive in open-angle popular for following visual fields in glaucoma.
9 glaucoma (30%), although a negative test does
6
not rule ouL open-angle glaucoma.
The basic visual field loss in glau­
coma is lhe nerve fiber bundle de­
D. Prone Position Test: If a patient lies in the OPEN-ANGLE GLAUCOMA? <
fect with nasal step and peripheral 1 J b
prone position and the intraocular pressure rises . > •; L. „a e~
nasal depression. It is here shown
8-10 mm Hg, this may indicate angle-closure
<"4-i , l A TV
superimposed upon the nerve fiber /
layer of the retina and the retinal glaucoma.. As with tests A and_B_(above). At least 90% of cases of primary glaucoma
Peripheral depression with double
vascular tree. AH perimetric Double arcuate scotoma with pe­ gonioscopy is ftFCe^sarytq'confirm the angle are of the open-angle type. Open-angle glautbma
nerve fiber bundle defect. Isola­ changes in glaucoma are variations
tion of central field. ripheral breakthrough and nasal closure if the pressure rises. is bilateral, insidious in onset, and.slpwly
of these fundamental defects. step. progressive. There are no syjnptoms until visual *£
impairment occurs, often too late to salvage useful .
Visual Fields in Glaucoma vision. Jtfs th e re fore th e p hy s i cian s rcspon silifti Ey <
to diagnose glaucoma before irreversible Optic
The visual field test is most important in nerve damage has occurred. Early treatment
detecting open-angle glaucoma and in following prevents or delays visual deterioration. ;
the course of visual deterioration caused by the Significant advances in the understanding
disease. The tangent screen, using a 2 mm white of the course of open-angle glaucoma have been
object at 1 meter, gives important information. made in recent years, but unsolved problems
Nasal depression connected with Peripheral breakthrough of large Small extensions of the blind spot or early nerve remain and there are still differences of opmion
arcuate scotoma. Nasal step or Seidel scotoma. Islands of greater
Ronne.
nerve fiber bundle defect with visual loss within a nerve fiber fiber bundle defects not necessarily connected to among authorities cm some issues. Itnoyv Seems
well developed nasal step. bundle defect. the blind spot are noted early in the disease. certain that increased intraocular pressure is
Ideally, the diagnosis is made before visual field caused by interference with aqueous outflow due
loss occurs. Under these circumstances medical to degenerative changes in the tjrabeculpm,
FIG 13-3. Visual field changes in glaucoma. control can usually prevent visual loss entirely. Schlemm’s canal, and adjacent channels (see
The nerve fibers are arranged in the retina as belowJfTHere is also a rare type of primin’’ open-
r':i-
164
165

, (ri i j2-CT ■ x-; X <'\-r-------- *----- —

 I
oQhz.-
-Tr

•i ' I i - ~a • 5®
angjc glaucoma capsed by hypersecretiou of a late finding. Although the disease is nearly Si mg 4 times daily arc used if drops do not Precipitating Factors
aqupous; nuinor (confirmed by tonography). always bilateral, one eye is frequently involveddC-' -•adequately control intraocular tension.
Increased pressure,| whether caused by faulty A sudden increase in the volume of the
earlier and more severely than the other. •?:
excretion yr increased production of aqueous, B. Surgical treatment: Filtering operations posterior chamber may push the iris forward
Optic disk changes are important early'•
affects primarily tliei retina and optic nerve, the findings. The temporal disk margin thins (Fig • may be performed if the intraocular pressure is against the trabeculum. This results from
functional elements of the eye. 4) and the cup gradually becomes wider and deeper; .;; ’'?« ,nol maintained within normal limits by medical congestion or edema of the iris, ciliary body, or
.Sojrncj authorities feel that in open-angle The large vessels become nasally displaced, and '? . therapy and there is progressive visual loss. Also choroid or from a sudden increase in aqueous
glaucoma th'pre is also a primary degenerative the affected area of the disk becomes atrophic-’4: • microsurgery directly on Schlemm’s canal and production. The causes of these changes are not
disqrdclr oT the optic nerve,due to vascular known.
(light gray or white rather than pink). y trabecular meshwork is performecL
insufficiency This I view is supported by the The intraocular-pressure is increased. The/, Therapeutic or physiologic mydriasis
observation! that loss of function sometimes anterior chamber angle may be normal on < s .s**
occasionally bunches the iris into the chamber
continues tja {progress even after the intraocular gonioscopy. The loss of visual function from angle sufficiently to precipitate an acute attack of
pressure has‘been normalized by miotic dierapy glaucoma can best be determined by repeated ANGLE-CLOSURE GLAUCOMA angle-closure glaucoma. Caution: Dilation of the
or surgipyj , i pupil should be avoided ifthe anterior chamber is
studies of the visual field, preferably using a. ( Acute Glaucoma)
Thtercsafe few detectable histologic changes Goldmann type perimeter. shallow. This is easily determined by oblique
in the earlyistages of open-angle glaucoma. Tn the illumination of the anterior segment of the eye.
later stages nonspecific changes occur which are Angle-closure glaucoma occurs when there An eye which is predisposed to acute angle­
common to'all forms qf primary glaucoma. Studies is a sudden increase in the intraocular tension closure glaucoma has a shallow anterior chamber.
upon eyes with early open-angle glaucoma have due to a block of the anterior chamber angle by Otherwise the eye is normal, with complete
revealed primary degenerafion in the trabecular the root of the iris which cuts off all aqueous permeability ofthe trabeculum, Schlemm’s canal,
mcshwqrk, with degeneration of the collagen and. outflow, causing severe pain and sudden visual and the aqueous veins. A rapid increase in
elasticjfrbers of the trabeculum as well as loss. intraocular pressure occurs when the peripheral
cndpthjelial! proliferation and edema. The An acute attack of angle-closure glaucoma iris is forced against the trabeculum, occluding
trabecular {spaces tqnd to be obliterated. The can develop only in an eye in which the anterior the outflow channels.
collector? channels jilso undergo degenerative chamber angle is anatomically narrow. Such eyes
chtnjge$ij ; | often have shallow anterior chambers and tend to
Tf |ihe pressure remains elevated, gross have a short axiaMength (hyperopic eyes). The Pathology
duniagtlq the eye' occurs. The optic nerve factors listed below may cause further
undergoes cldgenerat|On, often assuming atypical •; ■ encroachment upon the anterior chamber angle, The pathologic changes in acute glaucoma
bcan-p{pt |cupping appearance. There is setting the stage for angle-closure glaucoma. include peripheral anterior synechias and edema
degeneration'of ganglion cells and nerve fibers in (1) Physiologic iris bomb6: In eyes which and congestion ofthe ciLiary processes andiris.
the retina. The irisj and ciliary body become FIG 13-4. Typical glaucomatous cupping. Note tend to have the above-listed anatomic These are secondary to the vascular strangulation
atrophic,.and the ciliary processes show hyaline the nasal displacement of the vessels and configurations, the iris has a relatively large arc of which results from the high pressure. Late changes
degqnei'atiqnl Chronic edema ofthe cornea results hollowed-out appearance of the optic disk except contact with the anterior surface of the lens. This are the result of interference with circulation and
in loosening of the corneal epithelium and for a thin border. may obstruct the free passage of aqueous from the continued high pressure. The iris and ciliary'
formation! of epithelial bullae (bullous the posterior to the antenor chamber. As^ressure body become atrophic, and the ciliary processes
keratonatj-iy). Eventually the lens shows builds up in the posterior chamber, the peripheral show hyaline degeneration. Chronic edema ofthe
catafacfous changes. Treatment iris is pushed forward. Ifthe iris is pushed forward cornea results in loosening of the corneal
' Genetic aspeetk. Open-angle glaucoma is a epithelium and the formation of epithelial bullae
far enough so that it lies against the trabeculum,
familial', genetically determined disorder, probably A. Medical treatment: Miotics facilitate an attack of acute angle-closure glaucoma occurs. ' bullousJceratopathyh-The only important
by multifactorial pr polygenic inheritance. aqueous outflow by increasing the efficiency of (2) Increased size of the lens: Normally, the pathologic change is damage to the nerve elements:
Regardless o;fthe mdde of inheritance, the family the outflow channels. The drug is pilocarpine, 1- lens continues to enlarge slightly with age. During degeneration of nerve, fibers and a loss of
history ahd routine systematic testing of relatives 4%, 2 drops in each eye up to 5 times daily. the act of accommodation there may be a further substance of the optic cup associated with a
of glaucofng patients are most important in Pilocarpine frequently causes a temporary increase in the forward displacement of the lens, backward bowing of the cribriform plate. The
glaticoiph detection.! dimness of vision for 1-2 hours after instillation pushing the iris against the trabeculum. ganglion cell layer and the nerve fiber layer of the
and sometimes ocular congestion. Epinephrine (3) Increased thickness of the iris: This is retina undergo degeneration. Eventually the lens
■ i i hydrochloride or borate instilled locally greatly increased during pupillary dilatation and may show cataractous change. In the later stages
Clinical Findings supplements the action of miotics by decreasing may be a contributing factor in blocking an these eyes are similar to eyes in .the late stages of
aqueous production. Beta-blockers are also used anatomically predisposed angle. open-angle glaucoma.
jOjbenhtingle glaucoma causes no early in instillations 2 times daily. Carbonic anhydrase
symptoms.! Subjective visual loss is nearly always inhibitors such as acatazolamide (Diamox) 125 - -
' c
166 J, A'-LV -
I
—< /n zn-/.
 6
£
Clinical Findings C. Atrophy of the Retina and Optic Nerve: - .'. anterior segment of the eye), as well as edema and fSECONDARY GLAUCOMA 1 !
The nerve elements of the eye withstand-T opacity of the corneal stroma. The iris inserts
anteriorly onto the trabeculum instead of into the
I IJ J: '
Angle-closure (acute) glaucoma is increased intraocular pressure poorly:?' * ’ • i• • 1
characterized by a sudden onset of blurred vision Glaucomatous cupping of the optic disk and retinal ciliary body. Increased imraocular tension occurring as
followed by excruciating.painNausea and atrophy, particularly of the ganglion cell layer,: one manifestation of intraocular disease is.callbd
vomiting are_3lrn-pFesent The pain is usually occur. Neurovascular changes in the posterior secondary glaucoma. i
i
localizecTm and around the eye. Other findings w
segment _______________
of the eye may contribute ___
to this process. 1
! i
include markedly increased'mtraocular pressure, •*- c I iI
n shallow
a chan™, an terior chambei an Pf
an^rJr.r io™tAllc -Aniwa,
edematous Absolute Glaucoma: The end result Of - I : 5

decreased visual acuity (at times limited to light

■ uncontrolled angle-closure glaucoma is the same GLAUCOMASECOHQARY
perception, only), a fixed, moderately dilated pupil,
/ as that of any other uncontrolled glaucoma, ie, TO CHANGES IN THE LENS
and ciliary injection. ~ /'
absolute glaucoma. The eye is stony hard, I n
>
|
" -f- / sightless, and often quite painful, in which case
Differential Diagnosi^g^ri J I enucleation or retrobulbar
, alcohol injection is Lens Dislocation (Traumatic)] j
Differential Diagnosi^'>, \ necessary. The lens may dislocate interiorly,-pressing
' -? ~ . -I A. . -a ’l the iris against the posterior cornea and blbbkihg
Acute iritis and conjunctivitis must be \ aqueous outflow, or it may diilocate posteriorly.
considered with acute angle-closure glaucoma in \ Secondary glaucoma is a free uent cojnpFcatipn
the differential diagnosis of any acutely inflamed PRIMARY CONGENITAL OR FIG.13-5. Infantile glaucoma (buphthalmos). of posterior dislocation of thei ens and.i$ no easy
i
eye. (1) Acute iritis causes more photophobia and : INFANTILE GLAUCOMA to explain. Often it may be due to angle recession
less pain than acute glaucoma. Intraocular or trabecular damage which o jeurred at the time
pressureTlTnormai, ihe'pupil is constricted, and Differential Diagnosis ofthe trauma. In other cases pupillary block-1 cciirs
the cornea is not edematous. Marked flare and Infantile galucoma can be defined as that when a wedge of vitreous curls ar outs 1 the
cells are present in the anterior chamber, and there Term of increased intraocular pressure (bilateral) Megalocomea, secondary glaucoma, and dislocated lens and plugs the jupillary opt ning.
is deep ciliary injection. (2) In acute conjunctivitis ■which has its onset at birth or in the first 3 years traumatic corneal haze should be ruled out. Whatever the mechanism, surgery ’ nifty be
there is little or no pain and no visual loss. There of life. It is usually an autosomal recessive trait. Measurement of intraocular tension is the key to rzH'C'iirrir'hrin'Al tvra
necessary if tire intraocular pressurerehririol be
is discharge from the eye and an intensely inflamed (The filtration angle forms by a cleavage between differential diagnosis. controlled medically. • |i Ii :’ !i I
conjunctiva, but no ciliary injection. The pupillary the corneal elements and the less rapidly growing Goniotomy is the treatment of choice. If !> i, a
responses are normal, the cornea is clear, and iris elements in the embryo. Incomplete cleavage, repeated goniotomies fail, a filtering operation is Intumescence offlie Lens j ! •I{
intraocularpressure is normal, (3) Iridocyclitis with preventing normal angle development, prevents used. The long-term visual prognosis is then much The lens may take up donsidctable|fluid
secondary gl aucoma occasionall y .presen ts a normal outflow of aqueous and causes infantile less favorable. during cataractous change, increasing its sibie
difficult problem of differentiation. Gonioscopy glaucoma. markedly. It may then encroach upon the anterior
to define the type of angle is most helpful. If , The insertion of the iris onto die trabeculum chamber, produce a pupillary block, or causdjangle
corneal or anterior chamber haze prevents good is abnormally high. The scleral spur is poorly Course & Prognosis occlusion, resulting in angle-closure [glaucoma.
visibility, gonioscopy of the other eye will usually developed and is more posterior than normal. Treatment consists of lens extraction. ■ i '
l J , f !
. confirm the diagnosis. Because aqueous is unable to reach it, Schlemm’s In untreated cases, blindness occurs early.
canal is usually collapsed. The eye undergoes marked stretching and may Exfoliative Syndrome (Pseudocxfoliatiori Of the
even rupture with minor trauma. Typical Lens Capsule, Glaucoma Capjsiilarc) I ; i ‘ •
Complications & Sequelae glaucomatous cupping occurs relatively soon, In exfoliative syndrome, deposits dfjuriknoWn
Clinical Findings emphasizing the necessity of early effective origin and composition are’seen on thd lens
A. Formation of Peripheral Anterior treatment. surface, ciliary processes, lens zonules, pqbjterior
Synechias: The earliest and most constant symptom is The earlier the disease becomes manifest, iris surface, loose in the anterior charnbpffand'jn
The peripheral iris adheres to the posterior epiphora. Photophobia may be present. Increased lhe less favorable the prognosis, since the early the trabecular meshwork. Glaucoma and. sometimes
corneal surface in the trabeculai area and blocks intraocular pressure is the cardinal sign. appearance of symptoms implies a more severe cataract eventually develop, liens extraction has
the outflow of aqueous. Glaucomatous cupping of the optic disk is a defect, of aqueous drainage. no effect on the glaucoma. Miotics are inpderateiy
ft
i relatively early and most important change. Later effective, but a filtering operation, njay be
B. Cataract Formation: The lens sometimes . findings include increased corneal diameter necessary.
swells, and a cataract may develop. The enlarged ■ (above 11.5 mm is considered significant),
lens pushes the iris even farther anteriorly; this? epithelial edema, tears ofDescemet’s membrane.
increases the pupillary block, which in turn’ t
and increased depth of the anterior chamber
increases the degree of angle block. ? i
(associated with general enlargement of the .
S' ’
169
 • GLAUCOMA SECONDARY TO massive hemorrhage into the anterior chamber the iris and into the anterior chamber angle, those that do, withdrawal of the medication
; CHANGES IN THE UVEAL TRACT The intraocular tension is immediately elevated : interfering with normal aqueous outflow. Miotics eliminates the glaucoma; but permanent damage
and blood breakdown products or organized clots '
ii
Uveitis J'
. ■ lodge in the outflow mechanism. One serious
are of little value. Cyclocryosurgery is the best
■Oierapeutic technic available, but results are poor.
can occur if the condition goes unrecognized too
long. If topical steroid therapy is absolutely
complication is blood staining of the cornea. Once necessary, miotics or other open-angle glaucoma
Often the intraocular pressure is below well established the staining may require several therapy usually will control the glaucoma. It is
normal c-arly in uveitis. This is because the inflamed years to absorb. If the intraocular pressure cannot imperative thatpalients receiving long-term topical
Ciliary ijody is functioning poorly and does not be controlled with systemic hypotensive drugs, GLAUCOMA SECONDARY TO THE steroid therapy should have periodic tonometry.
secrete tire elements which produce the difference the hyphema should be evacuated through a USE OF TOPICAL CORTICOSTEROIDS It is equally important to beware of "topical
in osmo(i'c pressure between aqueous and plasma. limbal incision. administration of corticosteroids in the eyes of
There is’ edema of the trabeculum as well as the patients known to have glaucoma ora family history
ciliary body and; iris, and this may result in a Corneal or Limbal Laceration With Prolapse of Much interest has been aroused by the of glaucoma. Less commonly, glaucoma can occur
decreased facility of aqueous outflow. As long as Iris Into the Wound observation that topically administered in patients on long-term systemic corticosteroids.
tjiere is no osmotic difference between blood and Lacerations ofthe anterior eye or contusions corticosteroids may produce a type of glaucoma Injections of corticosteroids under the conjunctiva
aqueous! there will be no rise in tension, but when causing anterior rupture of the eye are sealed that simulates open-angle glaucoma. Most and under Tenon’s capsule may cause elevated
t|ie;ciliary body begins secreting there will be an spontaneously by prolapse of uveal tissue into persons do not develop significant intraocular intraocular pressure for several months. Surgical
abrupt r^sc of pressure unless there has been a the wound. This ordinarily causes loss of the pressure elevations while on such treatment. In excision of the residual steroid deposit may allow
simultaneous improvement in the patency of the anterior chamber and the rapid closure of the the pressure to return to normal.
outflow ‘channel's. Long-standing or repeated chamber angle by the adherence of the iris to the
attacks of iridocyclitis cause permanent damage cornea. The primary objective of treatment is the
to die traibeculum or extensive permanent anterior re-formation of the anterior chamber to prevent
synechias. In these cases, after the inflammatory permanent anterior peripheral synechias. Excision
reaction'has subsided, miotics or even filtering of prolapsed uvea, tight closure of the wound,
procedures may be needed to control the and injection of saline into the anterior chamber
intraocular pressure. are of paramount importance.
;i
Thninr I ’ Contusion Causing Retrodisplacemcnt of the Iris
; Rapidly growing melanomas originating in Root andDeepening of Anterior Chamber Angle
the uveal tract can cause increased intraocular (Angle Recession Glaucoma)
pressure, by volume replacement, encroachment A number of clinicians have called attention
dp ftje filtration angle, or by blocking of a vortex to this type of trauma-induced unilateral secondary
veirii Enucleation is indicated. glaucoma. Following a contusion injury, the
anterior chamber is observed to be significantly
EssentialAtrophyofthelris deeper than in the uninjured eye. Gonioscopically
Slowly progressive atrophy of iris tissue is a one sees a recession of the angle with exposed
rare disorder of unknown etiology which is almost ciliary’ body. Glaucoma occurs if there is sufficient
always ’.associated with glaucoma. Anterior associated damage to the trabecular meshwork to
synechias form and the degenerated iris elements interfere with aqueous outflow. The condition
block tile trabecular meshwork, creating a often responds to standard open angle glaucoma
glauconia that is; very difficult to control either therapy, although occasionally aflltcring.operation
medically or surgically. The condition is nearly is necessary.
ail ways unilateral:

li ;
f GLAUCOMA SECONDARY TO TRAUMA 1RIDIS

t !
Massive-Hemorrhage Into the Anterior Chamber Rubeosis often follows central vein occlusion
. i Contusion or penetrating injuries of the globe and occurs frequently in advanced diabetes
can cause tears in the iris or ciliary body and thus mellitus. Small vessels grow on the anterior surface

170 171
 ]YfDl!uscumContagiosum(Fig 14-1) Hemangioma (Fig 14-3) .■ .f
14 The typical lesion of this unusual disorder is
a small, flat, symmetric, centrally umbilicated
Two main types ofcongenital vascular Luihors.
occur in the lids: cavernous hemangiomas and
TUMORS •growth along the lid margin. It is caused by a large
virus and may produce toxic conjunctivitis if the
capillary hemangiomas. Cavernous hemangiomas,
are composed of large venous Channels lying ir>=
lesion sheds into the conjunctival space. the subcutaneous tissue; they are bluish in color-
Cure can usually be obtained by incising the and change in size according to their distentiofl:
lesion and allowing blood to permeate the central with blood. Capillary (strawberry) heniarigiofpaii:
portion, by cautery, or by excision. are bright-red spots composed of] pilated:
capillaries. They are painless unless spontaneous'
hemorrhage causes marked swelling. They may­
show rapid growth in the newborn period anti-
Both benign and malignant tumors are obstruct vision or produce secondary changes in the frequently undergo involution later.
encountered in the eye and its related structures. eye such as retinal detachment, a rise in intraocular . Treatment is usually not indicated in jinfaucyi
Most can be diagnosed early since they are visible, pressure, or anterior uveitis. or early childhood unless the defect is extensive
interfere with vision, or displace the eyeball. Care A history ofrecent change in size or appearance
enough to cause occlusion amblyopia.' yaripus.
must be taken not to overlook the possibility of of an external ocular growth should cal! for careful
types of treatment have been ’used, including’
malignancy. Fluorescein angiography is helpful in observation, including photographs. If there is any surgical excision ofsmallerlesions or freezing witil
the detection of intraocular tumors but may fail to suspicion ofmalignancy, biopsy or total removal is
COr Radiation is not recommended because iOf
differentiate benign from malignant lesions. Delay indicated for microscopic examination. the extensive scarring it causes. t
in diagnosis makes curative surgery technically more
difficult and may result in loss Df all useful vision. FIG 14-1. Molluscum contagiosum.
As far as possible, biopsies should be taken of all
accessible suspicious lesions, excising completely the
smaller lesions, since apositivediagnosis ofmalignancy' LID TUMORS Xanthelasma (Fig 14-2)
can only be made by histologic examination. Accurate Xanthelasma is a common disorder that
diagnosis of intraocular and retrobulbar tumors is occurs on the anterior surface ofthe eyelid, usually
difficult but vital, since curative therapy can only be bilaterally near the inner angle of the eye. The
given early; later, only palliation is possible. BENIGN TUMORS OF THE LIDS lesions appear as yellow, wrinkled patches on the
Secondary (metastatic) ocular malignancies do (Nevus, Verruca, MoIluscumContagiosiim, skin, and occur more often in elderly people. FIG 14-3. Cavernous hemangioma ofleft uppcrlidj
occur but are quite rare. The most frequent site of Xanthelasma, Hemangioma) Xanthelasma represents lipid deposits in
metastasis is the choroid. The ophthalmoscopic histiocytes in the dermis of the lid. Clinical l I
appearance maybe difficult to differentiate from that evaluation ofserum cholesterol levels is indicated,
of primary malignancies ofthe choroid, and finding Nevus but only rarely is a direct relationship found.
the primary' tumor elsewhere is of the greatest Nevi of the eyelids are. common congenital Treatment is indicated for cosmetic reasons. PRIMARY MALIGNANT |
diagnostic importance. The prognosis for cure of benign pigmented tumors having the same Surgical removal is simple. Cauterization of the TUMORS OF THE LIPS 1i
metastatic cancer by' enucleation is of course pathologic structure as nevi found elsewhere. smaller lesions is sometimes effective. Recurrence (Carcinoma, Xeroderma Pigmentosum,
hopeless. X-ray or other treatment may relieve the They are usually congenital but may be relatively following removal is not unusual. Sarcoma, Malignant Melanoma) • ■
ocular condition. unpigmenled at birth, enlarging and darkening I !i
during adolescence. Nevi rarely become malignant Il ' ' .
Physiology' of Symptoms Nevi may be removed by shave excision if Carcinoma (Figs 14-4 and 14-5) |i .j ‘
Smail tumors ofthe lids are asymptomatic except desired for cosmetic reasons.
in the case of vcrrucae and molluscum contagiosuro, Carcinoma of the lids has the highest
which occasionally shed a toxic substance into the eye Vcrrucae incidence of any malignant ocular tumor (42%)’.It'
to cause chemical conjunctivitis. There may be no Warts commonly appear along the margins of the is most frequent in men over 50 years ofiage.' •
complaints until the late stages, when the lesion is large lids as fleshy, multilobulated, flat-based to pedunculated The commonest site of the tumor isuearihe^
enough to cause pressure or displacement Tumors of lesions. They are thought to be caused by viruses. margin of the lower lid near thp inner canthus
the conjunctiva are painless unless there is lid irritation. If treatment is indicated for cosmetic reasons, Ninety five percent of lid carcinomas are of the
A central corneal lesion causes decrease in vision. An vcrrucae may be removed by excision with
basal cell type. The remaining 5% consist of?
iobaocular lesion involving the macula causesblurring cauterization at the base of the lesion. Care must
squamous cell carcinomas and sebaceous
ofvision as a presenting symptom. Extram-cular tuinois be exercised to avoid producing a marginal notch
> (meibomian; ! -nd) carcinomas. Khratoacanthoihns
are not manifested until they become large enough to in the eyelid. FIG 14-2. Xanthelasma. and inverted lbliiculur keratoses are benighpesibhs •
172 173
 I
*
■wljtcfi resemble squamous cell carcinoma In the eroding and fissuringunti! an.ulcer develops. ThgS ? ^pderg0 carcinomatous degeneration. The eyelids CONJUNCTIVALTUMORS
past this was not recognized, and the incidence base of the ulcer is indurated and hyperemic niff?* ^ire frequently affected and may be the first area
of sqhamous cell carcinomas was thought to be Lhe edges hard. Unless the tumor is excised eaily^ t To show degenerative changes, causing atrophy
higher than it actually is. Basal cell carcinoma is it grows through the skin, connective tissu&Fv< ? ‘•and ectropion with secondary inflammatory
rauclj more common in the lower lid; squamous cartilage, and bone until large areas are destroyed^ ‘ Ranges of the conjunctiva, symblepharon, PRIMARYBENIGN TUMORS
celi|C,arcinoma in the upper lid. Diagnosis is based in a fungating crater which may eventually reack. • I • corneal ulceration, and carcinoma of the lids. OF THE CONJUNCTIVA
upon clinical appearance and biopsy. the cranial cavity. Pain then becomes severe and ? -! <The malignant change may be of either the (Nevus, Papilloma, Granuloma, Dermoid,
'j. jSquamous cell carcinoma tends to spread via constant When sensory nerves are involved, thi-4 ■ squamous cell or basal cell type. This condition Dermolipoma, Lymphoma, Fibroma, Angioma)
thcjlymphatic system. On the upper lid it drains
thcdymphatic pain may be excruciating. The patient may die ofbn J js inherited as an autosomal recessive trait.
intpjthe preauricular nodes; on the lower lid, into hemorrhage, meningitis, or general debility. Carriers can often be identified by excessive
hubmaxi Hary node. Basal cell tumors grow very
IhcbubmaxiHary
the BasaL cell, carcinoma begins in a similar^? ; ^freckling. Nevus (Fig 14-6)
slowly,
E ■ are locally invasive,
‘ . and do not spread to manner, eventually forming the typical rodentulcer^' The disease appears early in life and in most
1het|r^gional
fricfegional lymph nodes. Systemic metastases with a raised nodular border and indurated base^r < ■cases is fatal by adolescence as a result of Conjunctival nevi are common congenital
:r. basal
are extremely rare in been! cell carcinoma
ccccir.crr.c but do
de IL eventually erodes the surrounding tissue ig •’* ■ ■metastasis. "Life may be prolonged by carefully pigmented tumors, although the pigment may not
occpi in squamous cell carcinomas. somewhat the same way as squamous ceipy Tprotectingthe skin from actinic rays and treating become visible until puberty or later. They usually
■V ‘ carcinoma, but much more slowly. Biopsy of the carcinomatous tumors as rapidly as they appear. appear as brownish or black, flat lesions which
■ sr
!:cjrgu. : tumor itself is a simple office procedure, and is the..:- move with the conjunctiva and are most frequently
only sure method of diagnosis. ..•' f; located in the caruncle or on the bulbar
R-' Basal cell tumors of the lower lid near the -' Sarcoma conjunctiva near the limbus. Bleblike or cystic
inner canthus tend to invade the structures of the."” elevated forms which have a gelatinous
inner canthus and the orbit. Complete eradication' ’ Sarcoma of the lids is rare and usually appearance (cystic nevus) occur less commonly
of these tumors is important j represents an anterior extension of an orbital but are an important diagnostic point.
Any suspicious warty' growth on the Iids.\X;- sarcoma. Rhabdomyosarcomas involving the Histologically, the nevi are composed of nests
should be removed and submitted for pathologic - orbit and lids represent the most common or sheets of typical pigmented nevus ceLIs which
examination. The objective of treatment is thc-‘^>-- malignant tumor in these tissues in the first theoretically are derived from the neural crests.
complete destruction of the tumor. The squamous '/ decade of life. Other sarcomas (usually named Malignant changes are rare.
cell type is resistant to X-ray radiation, and the - after the predominant type of cell) also occur. Conjunctival nevi should be observed for rare
only effective means of treatment is complete\ Most are radiosensitive, but a combination of malignant change, but excision is not required
surgical excision. If the diagnosis is made while#*- surgery and radiation is often required. They unless they become cosmetically disfiguring. If
the growth is still a local nodule in the eyelid, a-J maybe associated with similar lesions elsewhere removal is indicated, excision should be well
Flp •] 4-4. Squamous cell carcinoma of upper lid. large “V” resection may be performed well beyond ' ' in the body. outside the possible area of extension of
the limits of the tumor. Plastic repair of theP unpigmented cells.
deformity should be done concurrently. (■
Basal cell carcinomas are more radiosensitive, MfalignantMelanoma
but. each case should be evaluated individually. >
Simple excision and electrodesiccation ofthe small Contrary to general belief, nevi rarely
lesion is usually quite effective and avoids the undergo malignant change. Malignancy should
complication ofradiation bums. Good resultshave be suspected when a lesion enlarges or becomes
been reported with a combination of surgery and darker.
irradiation. :■ Treatment must be radical, and. is of value
only if performed early. If the lesion is small and
seems to be circumscribed, widespread local
Carcinoma Associated With Xeroderma excision may be used. If there is any evidence
Pigmentosum of spread, total exenteration of the orbit with
sacrifice of the lids is advocated by many
Basal cell carcinoma of left lower lid. This rare, congenitally determined (usually authorities. The prognosis with any form of FIG 14-6. Conjunctival nevus.
autosomal recessive) disease is characterized by therapy is poor.
cell carcinoma grows slowly and the appearance of a large number of freckles in
and may be present for many months the areas pf the skin exposed to the sun. These
is noted. It usually begins as a small warty are followed by telangiectases, atrophic patches,
with a keratotic covering, gradually and eventually a warty growth which may

174
■
Papilloma frequently with hairs protruding. A dermoid tumor “:- ! *• f
Angioma the cornea and is considered to be carcinoma :jh
may remain quiescent throughout life, although it situ, although atypical infiltrating squamous-iiell
Conjunctiva] papillomas are not rare, often increases in size during puberty. Removal is Conjunctival angiomas may take 2 forms: tumor may develop from it. It is resistant to Xnray
occurring most frequently near the limbus, on the indicated only if cosmetic deformity is severe or if
hemangioma or lymphangioma. The latter are rare radiation, and surgical excision is requi-r^d.
caruncle, or at the lid margins. Those on the Vision is impaired or threatened. Recurrences are not infrequent. Histologic steely
and usually congenital. Conjunctival
caruncle and lid margin are usually soft and
hemangiomas may appear as diffuse of excised tissue is indicated.
pedunculated, with irregular surfaces. They
telangiectases or capillary nevi, or as encapsulated j
frequently recur after removal. Papillomas at the Dermolipoma cavernous hemangiomas (more common). The
limbus arise from abroad base and tend to remain MalignantMelanoma . j. \
latter consist oflarge communicating, fairly well
flat while spreading onto the conjunctiva and Dermolipoma is a common congenital tumor encapsulated vascular spaces which have a
cornea. These tumors are composed of a which usually appears as a smoothly rounded tendency to enlarge. Treatment is by excision or Malignant melanoma of the conjunction ps
connective tissue core covered by proliferating growth in the upper temporal quadrant of the electrocoagulation. rare. It may arise from a preexisting nevus, fro^ah
epithelium. Malignant degeneration does bulbar conjunctiva near the lateral canthus. area of acquired melanosis, or de novo from riotmdl
occasionally occur. Treatment is usually not indicated, but at least conjunctiva. Its pigmentation may vary greatly,
partial removal should be done if the growth is and its clinical course is often unpredicttfljie.
enlarging or is cosmetically disfiguring. Posterior PRIMARYMAUGNANT TUMORS QF THE Eventually, most conjunctival melanomas enhijge
Granuloma dissection must be undertaken with extreme care BULBAR CONJUNCTIVA but do not usually invade the interior of the jei^c.
(if at all) since this lesion is frequently continuous (Epithelioma, Malignant Melanoma, Histologically, these tumors have the same
Granulomas are seen as vascular fungating with orbital fat; orbital derangement may cause Lymphosarcom a) structure as other malignant melanomas. ’ ■ t i
masses protruding from areas in which the scarring and complications far more serious than Simple excision is often impossible but ipay
palpebral conjunctiva (usually at the lid margins) the original lesion. be successful if the lesion is small enough. jfeay
has been broken or incised, as in draining Epithelioma (Carcinoma) radiation is ofno benefit. Exenteration of the orbit
chalazions, open conjunctival wounds, or is advocated, but the prognosis is poor.
conjunctival foreign bodies. Occasionally, specific Lymphoma & Lymphoid Pseudotumors
Epithelioma is an uncommon tumor which
etiologic agents such as the tubercle bacillus or arises most commonly from the limbal transition
cysts containing Coccidioides immitis are These are uncommon conjunctival lesions zone, less frequently from the mucocutaneous Lymphosarcoma
identified histologically. They can attain large size which may appear in adults without evidence of juncture at the lid margin and caruncle. It appears
rapidly, and may outgrow their blood supply and systemic disease or as part of the clinical picture initially at the limbus as a small opaque elevation Conjunctiva! lymphosarcoma is
strangulate, with spontaneous recovery. of lymphosarcoma, lymphocytic leukemia, firmly fixed to the underlying tissue. In many Histology, course, and management are the
Treatment is by surgical excision and cleansing Hodgkin’s disease, or other related conditions. instances growth is rapid. Extension can occur as for lymphosarcomas of the lids.
of the base. The wound may have to be closed Lymphomas appear most often at the fomices on through the perivascular and perineural lymphatics
with sutures. the bulbar conjunctiva as firm, reddish, slowly into the suprachoroidal space and Schlemm’s
encircling lesions. canal. Eventually the entire eyeball may be
Histologically, lymphomatous tissue is
destroyed by tumor tissue. Death may ultimately
Dermoid 'Dimor (Fig 14-7) composed of dense masses of lymphocytes occur as a result of distant metastases. CORNEAL TUMORS
without germinal centers and with a sparse
Histologically, most conjunctival epitheliomas
This rare congenital tumor appears as a connective tissue framework and abundant blood
behave as Squamous cell carcinomas.
smooth, rounded, yellow elevated mass, vessels. The differential diagnosis between Small epitheliomas may be safely excised. If
lymphoma and lymphosarcoma may be difficult
the tumor’is large ot if there is any possibility of PRIMARY MALIGNANT TUMORS
Treatment is by X-ray radiation ifmalignancy
extension, enucleation is indicated. If extension is OF THE CORNEA
is proved by biopsy, and is remarkably effective. widespread, exenteration may be required.
I
(Epithelioma, Melanoma) !
Irradiation is sometimes used as a routine measure ••I :

following surgical excision.

i: i
Fibroma Intraepithelial epithelioma (carcinoma in situ) Only a few’ cases of epithelioma add
is a more common type of epibulbar tumor which melanoma ofthe cornea are on record. They aj pear
Fibromas are rare small, smooth,
may begin at the limbus and involve the cornea to as irregular masses of gray epithelium over'the
pedunculated, transparent growths which may a greater extent than the conjunctiva. It is typically surface of the cornea. M;
appear anywhere in the conjunctival tissues but a smooth, flat opaque growth over the surface of i
are most often seen in the lower fornix.
Histologically, they consist Df fibrous
overgrowths covered by epithelium. Treatment is
FIG 14-7. Dermoid tumor at the inferior limbus. by excision.
176
177
 ' INTRAbCU LAR TUMORS Retinal Angio m a PRIMARY MALIGNANT TUMORS
OF THE INTRAOCULAR STRUCTURES
Angioma of the retina is a rare congenital (Malignant Melanoma, Retinoblastoma,
disorder. Blurring ofvision may result if bleeding V Diktyoma)
; Primary benign intraocular occurs or if the retina is secondarily detached. •
Stumors Occasionally, angioma is associated with angioma
(J?evus, Angioma,Tuberous Sclerosis, in the cerebral cortex (Lindau’s disease). The tumor Malignant Melanoma
Hem angioma of Choroid) occurs in the posterior fundus, often in the lower
temporal quadrant. It is globular in outline and It has been estimated that intraocular
may be located near one of the pairs of enlarged malignant melanoma occurs in 0.02-0.06% ofthe
Nevus =• retinal vessels. Angiomas may enlarge. total eye patient population in the USA. It is seen
Photocoagulation therapy (xenon or argon laser) only in the uvea! tract, and is the most common
Nevi may occur on any of the 3 portions of and cryotherapy are currently utilized to eradicate intraocular malignant tumor in the white
the uvea: the iris, ciliary body, or choroid. They these lesions. population. The average age of patients with this
are usually flat pigmented lesions lying in the FIG 14-10. Tbberous sclerosis. disorder is 50 years. It is almost always unilateral.
strbm.tt> of the tissue but not interfering with the Eighty-five percent appear iD the choroid, 9% in
function of the tissue itself. On the anterior surface Tuberous Sclerosis (Bourneville’s Disease) the ciliary body, and 6% in the. iris. Most of the
ofithe’iris they may be noted as iris “freckles.” Hemangioma ofthe Choroid choroidal tumors are in the posterior portion of
Posteriorly in the choroid one may see flat The rare intraocular tumor associated with the eye, especially on the temporal side. In the
pigmented areas. Large choroidal nevi arc difficult tuberous sclerosis varies in size and color but is Choroidal hemangioma occurs in most cases iris, the lower half is most often affected.
to differentiate from malignant melanomas. Their often described as a yellow or white nodular of Sturge-Weber syndrome associated with Intraocular malignant melanoma is rare in blacks,
unchanging slate-gray color and flat appearance swelling, frequently mulberry in appearance, unilateral infantile glaucoma. Cases occurring although uveal nevi are common.
and tlie lack of extension are important in the located in any portion of the posterior fundus but with no other signs of Sturge-Weber syndrome This tuinor may be seen in its early stages
differential diagnosis from malignant melanoma. with a predilection for the area near the optic nerve. are frequently mistaken for malignant melanoma only accidentally during routine ophthalmoscopic
‘ Because of the difficulties in differentiation Other manifestations of tuberous sclerosis include of the choroid (see beLow), and the mistaken examination or because of blurring due to macular
from rhialignant melanomas, fundus photographs skin changes (adenoma sebaceum), intracranial diagnosis has often led to unnecessary removal invasion. Blood-borne metastases may occur at
(including fluorescein angiography) or careful line changes causing epilepsy and mental retardation, of the affected eye. The tumor involves the any time, and deathmay occur before local spread
drawings should be made of all suspicious lesions. and other neurologic symptoms. posterior pole, usually near the optic disk and occurs or ocular symptoms appear. Glaucoma may
Observations should be made periodically for There is no treatment. sometimes extending out to the equator, most occur as a late manifestation
changes. often on the temporal side. Solid elevation of
the retina is present, with tortuous retinal
vessels. The borders are irregular, and the tumor
is never pigmented. Hemangiomas can produce
arcuate field defects or localized scotomas.
Histologically, the tumor consists of
endotheliumlined spaces engorged with blood
separated by spare connective tissue. Retinal
degeneration, over the tumor is common.
Secondary glaucoma, usually severe and
refractory to any treatment, is associated with
larger choroidal hemangiomas.
The differentiation from malignant
melanoma is most important and frequently
difficult .
There is no treatment, although eyes
tolerate smaller hemangiomas very well.
Enucleation may be necessary for intractable,
painful secondary trauma. FIG 14-11. Malignant melanoma of the choroid,
macular area, left eye (drawing).
FIG 14-8. Nevus of the iris. FIG 14-9. Nevus of the choroid.

178 179
 Histologically, these tumors present a been reported at almost every age. It is bilateral in Malignant epithelioma of the ciliary
variable cellular picture of spindle cells, epithelioid about 30% of cases. The tumor results from epithelium is a rare tumor seen'in adults. It |ilso is
cells, and reticulum. When properly catalogued, mutation of an autosomal dominant gene, which treated by enucleation. Cycil ectomy may be
these features have prognostic significance. is passed on with fairly high penetrance. Children SlIC.CftRRflll

Intraocular malignant melanomas may spread of parents who have produced one child with ,
directly through the sclera, by local invasion of retinoblastoma have about a 4-7% chance of
intraocular structures, or by metastasis. having retinoblastoma. Persons who have been
Clinical manifestations are usually absent cured of retinoblastoma should be told that any
unless the macula is involved. In the later stages, children produced will have nearly a 50% chance
growth ofthe tumormay lead to retinal detachment of being affected by retinoblastoma, with a greater FIG 14-12. Retinoblastoma visible through pupil.
with loss of a large amount ofvisual field. A tumor chance of binocular involvement.
located in the iris may be large enough to change Retinoblastomas usually arise from the
the color ofthe iris or deform the pupil. Pain does posterior retina. Growth tends to be nodular, with
not occur in the absence of glaucoma. numerous satellite or seeding nodules which may
The first step in diagnosis is to suspect the produce multiple secondary tumors. They gradually
lesion. Most intraocular malignant melanomas can fill the eye and extend through the optic nerve to the
be seen ophthalmoscopically. Transillumination brain and along the emissary vessels and nerves in
is of some value in differentiation from serous the sclera to the orbital tissues. Microscopically, most
retinal detachment. retinoblastomas are composed of small, closely
A high incidence of intraocular tumors has packed, round or polygonal cells with large, daikly FIG 14-14. Retinoblastoma with multiple seedings
been found in the study ofblind, painful, phthisic staining nuclei and scanty cytoplasm. A radial and optic nerve invasion.
(atrophic) eyes, one writer reporting that 10% of “pseudorosette” arrangement around bloodvessels
‘ such eyes contained previously unsuspected is common. Degenerative changes are frequent, with
malignant melanomas. necrosis and calcification. A few spontaneous cures
Enucleation is indicated if the tumor is have been reported.
contained within the eyeball. If there is any Retinoblastoma usually remains unnoticed ORBITAL TUMORS
evidence of extraocular exLension, exenteration of until it has advanced far enough to produce a
the orbit is usually indicated. Ifdistant metastases white pupil-unless strabismus has occurred, FIG 14-13. Retinoblastoma after X-ray radiation.
have occurred, enucleation will not help unless leading to earlier diagnosis. Otherwise, the tumor
the eye becomes painful or unsightly. Small is usually seen in the early stages only when Cryosurgery has been tried in various types Gradually enlarging orbital tumors may altpin
melanomas of the iris which have not invaded the sought for, as in children having a hereditary of intraocular tumors. The results have been a diameter of 1 cm before any displacementof the
root can be successfully removed by iridectomy. background or where the other eye has been disappointing except in the case of some eyeball is noted. The direction of displacement
In recent years, lesions which invade the iris root affected. In the early stages small, yellowish-white retinoblastomas which are sensitive to offers a clue to the location of the tunior-eg,
have been excised by iridocyciectomy, an extensive nodular masses may be seen protruding into the temperatures of-100 °C. posterior tumors tend to displace the eyeball
but effective operation. vitreous from the retina. Infants and children with anteriorly, whereas tumors between the -eyeball
The prognosis of malignant melanoma is only esotropia should be examined to rule out and one of the walls of the OTbit will cause lateral
fair. Removal of the eye may appear to have retinoblastoma, since blind eyes of children will Epithelial Tumor of the Ciliary Body (Diktyoma) displacement. These observations indicate tfie site
eradicated the primary lesion, but distant metastases often turn inward. of exploration when biopsy is indicated.
have been reported 20 yeaTs after removal of (he Retrolental fibroplasia and persistence of the Diktyoma is an extremely rare epithelial tumor With displacement of the eyeball, diplopia is
primary tumor. The histologic examination is very primary vitreous may simulate retinoblastoma. growing from the ciliary body and having the a common symptom. Pressure resulting from
■helpful in prognosis. Tumors of the spindle cell In general, the earlier the discovery and histologic characteristics of undifferentiated marked exophthalmos may interfere witli blood
variety have a much better outlook than tumors treatment of the tumor, the better the chance to embryonic retinal tissue. It occurs only in young supply to the optic nerve and retina, causing
composed ofepithelioid ormixed cell types. Smaller prevent spread through the optic nerve and orbital children. Diktyomas may be visualized as they blurred vision. Exposure of the eye due to inability
tumors (less than I sq cm) have asignificantly better tissues. to close the lids causes cornehl epithelial,dam age
grow over the posterior surface of the iris between
prognosis than larger ones. Immediate enucleation is the treatment of with resultant pain and irritation.
the iris and the lens, and may be present al the
choice in all unilateral cases. The mortality rale is pupillary border as a white, irregular nodular sheet. The common or otherwise important tumors
still about 20%. In bilateral cases, Reese has Growth may be very slow, but it is inevitable, and from the following classification will be discussed
Retinoblastoma suggested enucleating the more involved eye and the tumor is said to be locally malignant without below. References are given to discussions
massively irradiating the other, using radioactive metastasizing. Eventually it may fill the entire globe elsewhere in the text. The uncommon--orbital
Retinoblastoma is a rare but life-endangering cobalt. and follow-up treatment with tumors are briefly described.or only listed.
and perforate the sclera.
tumor of childhood. Two-thirds of cases appear triethylene melamine (TEM). Enucleation is the only treatment.
before the end of the third year; rare cases have

130 181

1
 nation B. Neurofibroma: There is.doubtthat this lacrimal fossa: (1) Epithelial tumors of the lacrimal
PRIMARY ORBITALTUMORS
tumor ever occurs as an isolated orbital tumor gland (50%), (2) inflammatory pseudotumors
without other evidence of neurofibromatosis (30%), and (3) lymphoid hyperplasias (20%). The ..
latter 2 are classified below under inflammatory
A:.Primary in Orbit: Choristomas
■ - j: I
tumors (pseudotumors) of the orbit.
• 1. £horistomas-Dermoid cyst, epidermal Mesenchymal Tumors Lacrimal gland tumors are divided into benign
A choristoma is defined as a tumor composed
pyst, teratoma, and malignant mixed tumors and carcinomas
of tissue elements not normally present in the area.
A. Lipoma: Rare. Usually minor or no unrelated to mixed tumors.
- 2.IIamartomas-Hemangioma, For the orbit this includes dermoid cyst, epidermal
J ii]: neurofibroma. clinical signs. Most mixed tumors are benign but are locally.,
cyst, and teratoma.
’ 3. Mesenchymal- invasive into adjacent periosteum and soft orbital
Dermoids are congenital tumors that occur
ii. Adipose-Lipoma, liposarcoma.
B. Liposarcoma: Very rare malignant orbital tissue. Surgical removal is indicated, but -
only rarely in the orbit, where they are found as
tumor. recurrence frequently occurs as a consequence
•i b. Fibrous-Fibroma, fibrosarcoma. solid tumors or cysts. They aTe more common in
c. Myomatous-Rhabdomyosarcoma. the eyelids and conjunctiva. In the orbit they are ofincomplete removal.
C. Fibroma: Simple fibromas are rare, but Carcinoma not associated with mixed tumor
^.Cartilaginous-Chondroma, most frequently found in the superior temporal
more common in the upper and inner portions of is haTd to differentiate clinically from other tumors
, chondrosarcoma. portion, usually anterior to the lacrimal gland. It is
i the orbit than elsewhere. They are usually evident of the lacrimal fossa. Frozen sections at the time
4. Neural-Neurofibroma, not always possible to differentiate tumors of the
% neurilemmoma, other rare tumors. lacrimal gland and dermoids on clinical grounds.
by the third decade. Exophthalmos, diplopia, and of surgery are important because this tumor is-
5. ^Epithelial-Lacrimal gland tumors. displacement of the eyeball are the first symptoms. highly invasive and requires radical surgical
Hair, cartilage, teeth, bone, and other tissues may
6. Pseudotumors-lnflammalory, Fibromatous tumors are usually management, usually including exenteration.
be found in varying degrees. Dermoids seldom
Jymphoid, and other rare causes. encapsulated, firm, and have a meager blood
metastasize or show malignant changes and have
supply. Simple excision is feasible in many cases
little tendency to grow.
if necessary to relieve symptoms. Pseudotumors
iB. Secondary in O rbitlfrom Adj acent
D. Rhabdomyosarcoma: This is the most A. Inflammatory Pseudotumors: One of the
Sfiiictujres: Hamartomas
I • ' ’J i'j %
common malignant tumor of mesenchymal origin most frequent causes of unilateral inflammatory- •
in the orbit. These tumors occur most frequently proptosis has been labeled pseudotumoT of the
1. IntraocuIar-MalignanL melanoma, A. Hemangiomas: These are the most
in Caucasians prior to age 10, and there is a slight orbit. The cause is not known, and the clinical
retinoblastoma. common orbital tumors, usually appearing by early
male preponderance. They usually cause proptosis course is quite varied. There are frequently
2. .Cornea and conjunctiva-Malignant adulthood. They vary greatly in size, the larger
downward and temporally and grow rapidly. external ocular signs of inflammation such asu
melanoma, epidermoid carcinoma. ones causing marked proptosis. The tumor mass
3. Eyelids and face-Basal cell carcinoma, Metastases to the brain and lungs are common. chemosis of the conjunctiva, episcleritis, or
varies from small capillary-sized endothelium-lined
Although the prognosis is very poor, occasional scleritis. Proptosis may be marked. The
other rare malignant tumors. channels (called capillary hemangiomas) to large
4.1 Upper respiratory tract-Carcinoma cures have been effected by early exenteration. diagnosis is mainly one of exclusion in the
vascular channels called cavernous hemangiomas.
pf the upper respiratory epithelium, There is usually a mixture with additional absence of diagnostic stigmas of orbital tumor
Sarcoma, mucocele.
E. Cartilaginous: Chondromas and as demonstrated by X-ray or ultrasound with
inflammation or scarring. Malignant
5. Cranial-Meningioma, other
chondrosarcomas are very rare in the orbit. high doses of a systemic corticosteroid (eg, 100
angiosarcomas are very rare. Treatment is usually
intracranial tumors. unnecessary unless significant proptosis exists, Chondrosarcoma is associated with osteosarcoma mg prednisone daily). Treatment is usually
I in which case surgical excision (partial or total) is
following radiation therapy for retinoblastoma. promptly effective and the response to
I indicated. treatment often serves as a means of
F. Osseous: Osteoma and osteosarcoma differentiating pseudotumor from other causes'
(p. Metastatic From Distant Sites:
1 ‘ Carcinoma, sarcoma, neuroblastoma. are very rare. of proptosis.

i J i
■1 2 3:
DpOtlidr Types:
Neural Tumors
B. Lymphoid Pseudotumors: Malignant
lymphomas and lymphoid hyperplasia occur in"
the orbit infrequently and may cause some
i! * ’ 1. Reticuloendothelioses-Juvenile Neurofibroma (see above) and other rare proptosis, but they are most frequently noted'
tumors. only after intrusion into the upper or lower cul-
Xanthogranuloma, eosinophilic
granuloma, other rare entities. de-sac has occurred. Diagnosis and treatment
2. Metabolic disordcrs-Thyroid involve the same difficult problems as do these
Epithelial (Lacrimal Gland) Thmors tumors elsewhere in the body. Local irradiation
Exophthalmos.
3. Phakomatoses-Neurofibromatosis is very effective in shrinking lymphoid
(Recklinghausen’s disease). FIG 14-15. Orbital tumor displacing left eye. There are 3 main types of tumors of the hyperplasia.

182 183
 SECONDARY ORBITALTUMORS &
metastatic TUMORS FROM
usually call for a radical surgical approach,
including exenteration, unless the prognosis 15
DISTANT SITES is already hopeless because of malignant
tumor spread.
Metastatic orbital tumor masses are rarely
TRAUMA
These are listed in the orbital tumor removed surgically. Radiation is occasionally
classification (see above). used in the orbit as a palliative measure. The
All tumors from adjacent structures orbit is a relatively uncommon site for blood­
invade the orbit by direct extension and borne metastases.

In spite of the protection afforded by the

( L
recovery from the injury, and a tefrfictioii
j i
bony orbit, the cushioning effect of the retrobulbar performed if vision is below normal. This r.ccord
fat, and the lids and lashes-aud in spite of the may have legal significance.
great strides made in recent years in the In severe injuries it is important ibrjthd
development of protective devices, especially the nonspecialist to bear in mind the possibility of
use of safety goggles-the incidence of eye injuries causing further damage by unnecessary
remains high. Childhood eye injuries continue to manipulation. ; f J I
occur as a result of air rifle, bow and arrow, catapult Caution: Topical anesthetics, dyes, arid oihej
(slingshot), and throwing accidents. medications placed in an injured eye be
Pain or photophobia caused by the injury sterile. • j |
may produce blepharospasm severe enough to Both tetracaine and fluorescein can* be
prevent examination of the eye. If this happens, autoclaved repeatedly without impairment 0;f their
instill a sterile topical anesthetic. With the aid of a pharmacologic properties. Most ophthalmic*
loupe and wellfocused light, the anterior surface solutions are now available in indixiidiiaj
of the cornea is examined for foreign materials or disposable sterile units. | •’ - i
wounds, regularity, and luster. The conjunctiva is
inspected for hemorrhage, foreign material, or
tears. The depth and clarity of the anterior chamber
is noted. The size, shape, and light reaction of the NONPENETRATING INJURIES... .. !
pupil should be compared with those of the pupil OFTHE EYEBALL I ' , j
of the uninjured eye. If the eyeball is intact, the (Abrasions, Contusions, Rupture,1 i
lids are carefully inspected to the fomices, everting Superficial Foreign Bodies, Burns)) «| j
the upper lid. The lens, vitreous, and retina are
I i i
examined with an ophthalmoscope for evidence
of intraocular damage such as hemorrhage or
i H.! I
Abrasions 1 ;
retinal detachment.
If the patient complains of a foreign body Abrasions of the lids, cornea, or cjonjvmctjva
sensation but none can be seen with oblique do not require surgical treatment. 1’he kydund
illumination, instill sterile fluorescein. This may should be cleansed of imbedded foreign material I
demonstrate an irregularity of the corneal surface In order to facilitate the examination, tile paid
due to a minute abrasion, laceration, or foreign, associated with abrasions of the cprnba'and
body. conjunctiva can be relieved by instillation =df a
A small child may be difficult to examine topical anesthetic such as 0.5% tetracaine
; adequately. If a rapture or laceration of the eyeball solution, but routine instillation of a topical
i is suspected, it is best not to struggle but to anesthetic by the patient wmsZ not be pernutled
; examine with the aid of a short-acting general since it delays normaL healing of the cpitheliunu
f anesthetic. If a sever injury is not suspected, the Ophthalmic antibiotic ointment instilled intojlhe
lids may be manually separated under topical eye lessens the. chance of infection. An:eye
j anesthesia with the use of lid retracting forceps, bandage applied with firm but gentle pressure
j - It is important to determine and record visual lessens discomfort and promotes healing; by
i acuity. Visual acuity should be tested again upon preventing movement ofthe lids over tlie involved

1B4 185 • ■ ;
area. The‘dressing should be changed daily and Acetazolamide, mannitol, or other systemically
chamber can be reformed (if necessary) under “flash bums” are caused by careless exposure in ’
Lhp Wound inspected for evidence of infection or administered agents to lower intraocular pressure
sterile conditions. Many types of instruments are the mistaken belief that foe eyes can be burned in
ulcer foilnation. may be necessary.
used for removing superficial corneal foreign this way only when looking directly at the arc. A
■ Corneal abrasions cause severe pain and may
bodies; including special “hockey stick” or “golf short circuit in a high-voltage line may cause the
lead: to recurrent corneal erosion, but they rarely
club” spuds, scalpel blades, and the points of same type of injury.
bccbmciiqfected. Rupture of the Eyeball
hypodermic needles. A dental drill ofthe burr type In severe cases of “flash bum/’ instillation
is often useful for removing an imbedded nist ring. of a sterile topical anesthetic may be necessary
Rupture of the eyeball may occur due to
for examination. A mydriatic (eg, homatropine
Contusions penetrating trauma. It may also occur as a result
hydrobromide, 2-5%) should be used. Systemic
of contusion which causes a sudden increase in
sedation or narcotics are preferable to topical
■: Contusions of the eyeball and its surrounding intraocular pressure, causing the wall of the
anesthetics, which interfere with corneal healing.
tissues are commonly produced by traumatic eyeball to tear at one of the weaker points. The
Patching and cold compresses are indicated to
cdntact jwjth a blunt object. The results of such most common site of rupture is along the limbus;
relieve discomfort
injury- are variable pnd are often not obvious upon occasionally, rupture occurs around the optic
Infrared exposure rarely produces an ocular
supcrfibifll examination. Careful study and nerve. Anterior ruptures can be repaired surgically
reaction. (“Glassblower’s cataract” is rare today
adccpjatp follow-up are indicated. The possible by interrupted sutures unless intraocular contents
but once was common among workers who were
reJaj’US 6C contusion injury are hemorrhage and are so deranged that useful fimetion of the eye is
required to watch the color changes in molten glass
swelling ofthe eyelids (ecchymosis, “black eye”), not possible, in which case enucleation should
in furnaces withoutproper filters.) Radiant energy .. x i;-
subfoonjupctival hemorrhages, edema or rupture be done. If either of these procedures is required,
from viewing the. sun or an eclipse of the sun
of the ccjrnea, hemorrhage into the anterior implantation of a plastic sphere is useful as a
without an adequate filter, however, may produce
chamber (hyphema), rupture of the root of the iris space-filler and to aid in movement of a prosthesis
a serious bum. of the macula resulting in permanent
(iridodihlysis), traumatic paralysis of the pupil (artificial eye), which can be fitted much like a large
contact lens 2 or 3 weeks later. impairment of vision. Persons using ,
(mydriasis), rupture of the iris sphincter, paralysis
FIG 15-1. Metallic corneal foreign body. hallucinogenic drugs such as LSD have been
or sphsm’j of the muscles of accommodatiou,
particularly prone to solar macular burns.
arite'riodchamber angle recession with subsequent
Following removal, ofthe foreign body, an Excessive exposure to radiation (X-ray)
seciondjafy glaucoma, traumatic cataract, Corneal & Conjunctival Foreign Bodies
antibiotic ointment such as polymyxin B-bacitracin produces cataractous changes which may not .
di.Slocatio'n of the lens (subluxation and luxation),
or gentamicin should be instilled 3 times a day appear for many months after the exposure. The
vi'treous hemorrhage, retinal hemorrhage and Foreign bodies (fig 15-1) are the most
into the conjunctival sac to prevent infection. If same risk is inherent in exposure to nuclear
=5 retinal edema (most common in the macular area, frequent cause of eye injury. Small metallic or
the wound is extensive, an eye bandage can be devices.
called commotio retinae, or Berlin’s traumatic nonmetallic foreign bodies are frequently blown
used to minimize movement of the lid over the
edema)! detachment of the retina, rupture of the into the eye and may become lodged under the
injured area. The wound should be inspected daily
chcirbid, and optic-nerve injury. upper lid or be embedded in comeal epithelium. In
for evidence of infection until it is completely
: Mady of these injuries cannot be seen on removing corneal foreign bodies, a sterile topical
healed. PENET 'TING INJURIES TO THE EYE
external observation. Some, such as cataract, may anesthetic is essential. Minute corneal foreign
(Lacerations, Intraocular Foreign Bodies)
no(= develop for njany days or weeks following bodies which are not readily visualized with the
ihejnjufy- naked eye or loupe may be outlined with sterile
Burns
■ ■_ Except for injuries involving rupture of the fluorescein. If a foreign body containing iron has
Note: Tetanus prophylaxis is indicated
eyeball jtScIf (sec below), most of the immediate remained in the tissue for any length of time, rust
Thermal burns of the eye structures are whenever penetrating eye injury occurs.
effects |of contusion of the eye do not require penetrates the comeal tissue and must be removed
treated as bums of skin structures elsewhere, as
iitnncdiafe definitive treatment. However, any to prevent further irritation.
j the tissues of the lids are most commonly
injury‘severe enough to cause intraocular Although foreign bodies may often be
i involved- If the damage has been deep enough to Lacerations
htmorrhpge involves the danger of delayed removed satisfactorily using a light and.
; cause sloughing of the corneal tissue, the eye is
secondary hemorrhage from a damaged uveal magnifying loupe, the most satisfactory method
? almost certainly lost by extensive scarring or Lacerations are usually caused by sharp
vessel, which may'cause intractable glaucoma and is under direct observation with the aid of the
I perforation. objects (knives, scissors, a projecting portion of .
permanent damage to the eyeball. Patients who greater magnification and illumination of the
i Ultraviolet irradiation, even, in moderate the dashboard of an automobile, etc). Such
slfow cyipence ofintraocular hemorrhage should slitlamp. Although the cornea is ver}' tough, it is
j doses, often produces a superficial keratitis which injuries are treated in different ways depending
bC put atjabsolute bed rest for 4 or 5 days with also thin ( 1 mm ). Care must be taken not to
? is quite painful, although recovery occurs within upon wheter or not there is prolapse of tissue.
both c^ies bandaged to minimize the chance of penetrate the cornea in the process of removing a
i 12-36 hours without complications. Pain often
farther!bleeding. Secondary hemorrhage rarely deeply imbedded foreign body. When in doubt,
i comes on 6-12 hours afteT exposure. This type of A. Lacerations without Prolapse ofTissue:
occurs after 72 hours. A short-acting cycloplegic such deeply placed foreign bodies should be
• injury occurs following exposure to an electric If the eyeball lias been penetrated anteriorly * i
such, as! 5% homatropine may be used. removed in the operating room where the anterior
• welding arc without the protection of a filter. Many without evidence of prolapse of intraocular

186
1B7
 contents, and if the wound is clean and apparently of ocular tissues by degenerative changes ■ “halving lhe lid” and produces a smooth repair.
. free from contamination, it can usually be repaired (siderosis from iron and chalcosis from coppervV? Rarely, extreme edema of the tissues prev ent s
by direct interrupted sutures of fine silk or catgut. Some of the newer alloys are more inert and may ' apposition of the wound for primary closure, and
Blood clots can be gently removed from the be tolerated. Other kinds ofparticles, such as glass ’ the repair must be delayed (secondary repair) until
anterior chamber by irrigation and the chamber or porcelain, may be tolerated indefinitely and arc this has subsided. Local debridement and
re-formed after corneal repair by injection of usually better left alone. irrigation, with use of antibiotics, should be
normal saline solution or air. A mydriatic should A complaint of discomfort in the eye with" carried out until it is possible to approximate the
be used and an antibiotic solution instilled in the blurred vision and a history of striking steel upon; edges of the wound.
conjunctival sac, and bilateral eye bandages steel should arouse a strong suspicion of an Lacerations of the lids near the inner,canthus
.. applied. The patient should be placed at bed rest intraocular foreign body. The anterior portion of frequently involve the canaliculi. If these are not
for a few days and systemic antibiotics given to the eye, including the cornea, iris, lens, and sclera^ repaired, permanent stricture with epiphora will
_ . minimize the chance of intraocular infection. should be inspected with a loupe or slitlamp in an result. In repair of a canaliculus, a small
attempt to localize the wound of entry. Direct polyethylene tube or large piece of heavy stiff
B. Lacerations With Prolapse: If only asmall ophthalmoscopic visualization of an intraocular suture material is introduced through both
portion of the iris prolapses through the wound, foreign body may be possible. Ail orbital soft portions of the severed canaliculus and :
.. this should be grasped with a forceps and excised tissue X-ray must be taken to verify the presence maintained in place while the tissues are;
at the level of the wound lip. Small amounts of of a Tadiopaque foreign body as well as for reapproximated. This procedure will often maintain '
-• uveal tissue can be removed in a similar way. The I
medicolegal reasons. INJURIES TO THE LIDS a patent duct while the tissues are healing. The i
wound should then be closed in the same manner Localizing X-rays can be obtained by several Viers canaliculus rod is an effec-tive carialiculus j
- . as a wound without prolapse, and the same follow­ methods, usually by the method of Comberg, splint for most cases. The pigtail probe is helpful j
up care given. If uveal tissue has been injured, using a contact lens; or the method of Sweet, with Many lacerations of the lids do not involve in identifying the cut end of the canaliculus and .
the possibility ofsympathetic ophthalmia is always a geometric calculation following accurate lhe margins and may be sutured in the same way serves as a guide for threading polyethylene'
present positioning ofa guide post By one ofthese special as other lacerations of the skin. If the margin of tubing. These should be left in place for several:
If the wound has been extensive and loss of means the radiologist is able to plot the the eyelid is involved, however, precautions must weeks, and subsequent dilatations performed if j
intraocular contents has been great enough that approximate position of the foreign body within be taken in an attempt to prevent marginal strictures tend to form.
’ the prognosis for useful function is hopeless, the eye or orbit. notching. The most effective technic is to split Canaliculus repair should be performed j
evisceration or enucleation is indicated as the If the foreign body is anterior to the lens the lid margin on both sides ofthe laceration in a immediately inasmuch as subsequent attempts al I
primary surgical procedure. zonules, it should be removed through an • i
frontal plane (anterior, posterior) through the gray repair are much more difficult. 1
incision into the anterior chamber at the limbus. line, forming 2 sheets of tissue. A small ,CV” of
If it is located behind the lens and anterior to skin can then be removed from the anterior surface
the equator, it should be removed through the of one side of the laceration and a small “V” of
area of the pars plana which is nearest to the tarsus removed from the other side, allowing the 2
foreign body because less retinal damage is flaps to overlap in 2 suture lines. This is called INJURIES INVOLVING THE;
caused in that manner. If the foreign body is I
posterior to the equator, it should be removed
directly through that point on the wall of the
eyeball which is nearest to it, unless that area is Bony Injury
at the macula.
If the foreign body has magnetic properties, Fractures of the walls of the orbit may be':
the sterilized tip of a hand magnel (or giant caused by direct blows or by extension of a’
FIG 15-2. Cornea! laceration with sutures in magnet) near the area of exit can be used to fracture line from adjacent bones. The oilier table
place. Note also traumatic cataract. facilitate its removal. If it is nonmagnetic and of the frontal bone above the orbit may receive
removal is essential, small forceps have been crushing injuries without damaging the orbital,
devised for introduction into the posterior portion contents. Similarly, fractures and displacement of
Intraocular Foreign Bodies of the eye with minimal displacement and trauma the zygomatic bone, nasal bone and accessory
A special instrument has been devised to grasp a sinuses, and the medial wall of the orbit can be
Foreign bodies which have become lodged spherical air rifle or shotgun pellet. involved in depressed injuries of the face in
within the eye should be identified and localized Any damaged area of the retina must be automobile accidents. If a fracture involves llid
FIG 15-4. Complete laceration of upper lid and
as soon as possible. Particles of iron or copper treated with diathermy or photo coagulation to paranasal sinuses-most frequently the. ethmoid
i upper and lower cnnaficuli. Large sutures used
must be removed to prevent later disorganization prevent retinal detachment. bone-emphysema may be noted by crepitation oil
! in repair of severed cnnaliculi and medial canthal
■ tendon. palpation. Such an involvement frequently is

188 1B9
 I1!-!
jfcl'lowed by the development of chronic
‘Osteomyelitis.
<•
the orbital floor defect is bridged with a graft of
bone, cartilage, or plastic material. 16
B owb'ut Fracture Penetrating Injury
OPTICS & REFRACTION
I.
b, •■Jisolated orbital floor or “blowout” fracture, Penetrating injuries of the orbital tissue may
w lijqpt concurrent orbital rim fracture, usually be produced by flying missiles or sharp
To Hows blunt injury to the eye. Orbital contents instruments. Radiopaque foreign bodies can be
!lk nijijte into the maxillary sinus, and the inferior localized by X-ray methods similar to those used
■rectus; or inferior oblique muscle may become in Locating foreign bodies within the eye. Most
tin operated at the fracture site. orbital foreign bodies are best left alone.
The sense of sight depends upon an external phenomenon, tempered by an inherited tendency
TSigns and symptoms are pain and nausea
stimulus which is received by the eye in the form toward nearsightedness, and explains why
altjfejtime
1 of injury and diplopia on looking up
of light rays that pass to the retina through the “exercises” or visual training have no influence
jbi down. Diplopia may occur immediately or Contusions
cornea, aqueous, lens, and vitreous. The varying on a condition due primarily to the size of an organ.
,wjtljijii a few days. Enophthalmos (backward
optical densities (or refractive indices) of these (Rarer causes of myopia and hyperopia due to
’displacement
ipl^Cement of the eyeball) may not be present Contusion injuries to the orbital contents
Untiliiie orbital reaction clears. The fracture site structures cause the light rays to change direction abnormal refractive changes in the optical media
may result in hemorrhage or subsequent atrophy
•r as they pass through. If the image does not focus are not considered here.)
jis bfjst demonstrated by antral roof deformation of the tissue, with enophthalmos. Traumatic
on the fovea, the object will appear to be blurred.
;oq Waters' view X-rays or laminagrams. There paresis of the extraocular muscles occasionally
The science of optics forms the basis of the
Jislliputed movement of the eye even with forced occurs in this way but is usually transient.
diagnosis and correction of refractive, errors of
duCtiqns.
the eyes.
!* I the fracture is large or muscle imbalance
Isigiiiifipant, prompt surgical reduction is Pulsating Exophthalmos
jimpSirative. If the vertical imbalance is small,
'iSurgeijy can be delayed a few days or weeks as Pulsating exophthalmos occasionally follows
PHYSIOLOGIC ASPECTS OF OPTICS
•long ais steady improvement is noted. a penetrating or contusion injury to the orbital
•’ j ’ Ttwo effective means of surgical treatment contents which has caused a shunt between the
lajje. available. A Caldwell-Luc anfrostomy can arterial and venous channels so that the pulse is
Emmetropia, Hyperopia, Myopia
be usdd for antral packing after direct reduction transmitted into die orbital tissues. (This condition
Tfprp.^elow. Packing is generally left in place 2 may develop spontaneously, but is more frequently
The emmetropic (normal) eye is one in which
jwBfejkk. Traction on the extraocular muscles by traumatic in origin.) A common site of involvement
parallel rays of light are brought to a focus on the
iftyceps or sling sutures will verify reduction. is a fracture through the cavernous sinus.
fovea without the use of accommodation. A
■The fracture site may be approached through Pulsating, exophthalmos occasionally
■fhje-lower lid along the orbital floor. In this hyperopic (farsighted) eye is smaller than normal
requires ligation of the carotid artery on the side
and fails to converge the rays of light enough to
H'n'stance, the prolapsed tissue is reduced and of the aneurysm.
focus upon the foves without the use of
accommodation. The act ofaccommodation or the
placing of a convex (“plus”) lens in front of the
eye helps converge the light to a focus on the
fovea. A myopic (nearsighted) eye is larger than
normal and tends to focus the light in front of the
fovea. A concave (“minus”) lens in front of the
eye helps to diverge the light rays to focus on the
fovea.
Since the size of the eyeball governs the
focus to such a large extent, and the eyeball
continues to grow until about age 25, it is easy to
see how a farsighted eye of a I0-year-old child
• becomes less farsighted or even nearsighted by
; age 20, and a normal or nearsighted eye becomes
more nearsighted. This is a normal growth FIG 16-1. Emmetropia, hyperopia, and myopia.

190 191
 Accommodation .The vitreous has no optical function other important reason for this is the number ofvariables
fraction, but the majority in common use compare
than light transmission. It is a clear gelatinous which influence refraction such as corneal
the patient’s acuity with the average normal for
Accommodation is the mechanism by which medium between the lens and the retina through curvature, depth of anterior chamber, length of
that distance. “20/20” indicates the line read by
, the focusing apparatus of the eye adjusts to which light rays pass unrefracted. the eye, and shape of the lens.
average nonnai people at 20 feet. 6/6 (tbe metric
objects at different distances. Contraction of the equivalent) is more commonly used in England
, ciliary muscle, which is supplied by the and the Commonwealth countries.
parasympathetic third nerve, results in increased Ennnetropia
Snellen tests for near vision are constructed
■_ curvature and gives the eye greater dioptric power, HYPEROPIA
on the same principle as those for distance. A
so that divergent rays of Light coining from within In the absence of neural and retinal disorders (Hypermetropia, Farsightedness)
common near card is the Jaeger test type.
. infinity are now focused on the retina. The and opacities of the cornea, lens, and vitreous,
Visual acuity test results vary greatly with
and assuming that the eyeball is of normal size,
mechanism is largely reflex and so well adjusted the illumination of the test cards; some increase
the unaccommodated eye will receive and focus In hyperopia, parallel rays of lightare brought
. _ that normal individuals are not aware of the in acuity is noted with increase in illumination up
parallel rays of light from a distant light source to a focus behind the retina when accommodative
process. to a certain point of brightness. It has been
into a sharp image upon the fovea. This condition powers arc relaxed. The result is indistinct vision
Just how contraction of the ciliary muscle suggested that a diffuse illumination of about 80-
is called emmetropia (“sight in proper measure”). at all distances. Accommodative efforts bring
■* increases the curvature of the lens is still disputed. 100 footcandles on the chart is best.
Emmetropia is best thought of as an ideal rather objects into focus up to the limits of the powers
Young and Helmholtz assumed that the lens was
than a normal condition since almost all adults of accommodation.
; - elastic and that contraction of the ciliary muscle,
have some degree ofrefractive error. Hyperopia may be caused by shortness of
• - particularly the circular fibers of the ciliary muscle
. (fibers of Muller), drew the ciliary processes the eyeball or weakness of the refractive power of
... together so that the zonule was relaxed. The zonule the cornea or lens. “Physiologic” hyperopia is
REFRACTIVE ERRORS Ametropia
*.4 is a sheet of tissue made up of numerous fibers present at birth in about 80% of children. This is
■ - that support the lens in its capsule. due to the shortness of the eye, partially
Variations from the emmetropic state not due compensated for by the fact that the infant lens is
The lens itself is actually not elastic, but the to opacities or disease are collectively referred to as
— lens capsule is. When the lens is taken out of the more convex than the adult lens. About 5% of
Optical System of the Eye ametropia (“sight not in proper measure”). The
.. eye in its capsule, it, is more circular than when it children are born myopic, and about 15%
principal forms of ametropia are hyperopia
- is in the eye during the nonaccommodated state. emmetropic. During the years from about age 2 to
A ray of light entering the eye passes (hypermetropia, “far sight”), myopia (“near sight”),
When the zonule relaxes, the elastic lens capsule through the cornea, the aqueous, the anterior and astigmatism (“not at a point”), and presbyopia (“old
about age 25, there is a slight gradual decrease in U j
forces the lens to assume a more spherical form hyperopia. Most persons remain slightly
posterior surfaces of the lens, and the vitreous to sight”). Less importantvariations are anisometropia ! , i
and hence its anterior surface becomes more hyperopic during adulthood.
focus upon the fovea. Because of its greater (variation in refractive errors in the 2 eyes) and
■ convex. curvature, the refractive power of the cornea is aniseikonia (difference in image size in the 2 eyes).
Contraction of the ciliary muscle causes a greater than that ofthe lens. However, the cornea '|j1 ■
V1 ;
1
- - change in refractive power of the crystalline lens Clinical Findings
has no powers of accommodation; its optical
from the earliest age at which accommodation can ■; 1 ■
function is to refract light and to be clear and Nonvisual Symptoms & Signs
be measured up to about age 45 or 50. Except in severe degrees ofhyperopia, vision
uniformly curved. Because the aqueous has the I • :■ I
is normal beyond a range of 6 meters. Ifthe effort
same refractive index as the cornea (1.33), these 2 In addition to the visual disturbances caused j •’
required for accommodation is not too great, near
optical media are considered as one. ; by refractive errors, a great many associated
Visual Acuity vision may likewise be unimpaired (latent
The variable refractive medium of the eye is j symptoms may occur. These are especially important :> * V
; in the diagnosis of refractive errors in children, hyperopia). If greater accommodative effort is
the crystalline lens (in recent literature called
Many subjective tests have been proposed j Common signs and symptoms include blinking, required, the patient complains of “eyestrain,”
simply the lens). The lens is an elastic structure
for the determination of visual acuity using | frowning, rubbing the eyes,head tilting, closing one sometimes accompanied by eye pain, headache,
situated in the anterior portion of the eye behind
pictures ordifferent objects, circles and dots, and ; eye, clumsiness, photophobia, injection, and tearing, and nausea. In severe degrees of hyperopia (“high
tiie iris. In shape it resembles a convex glass lens
letters and numerals. Snellen was the first to i Adults may complain of “eyestrain” caused by the hyperopia"), distant vision can be maintained only
(“magnifying glass”). In young people the lens is
construct a system of test objects so arranged | effort of accotnmodati on. Headache, dizziness, and by accommodative effort and near vision is blurred
capable of precise instantaneous adjustments in
that acuity could be expressed by a number. In his ’ occasionally nausea may have their origin in even with maximum accommodative effort.
its shape in order to bring into focus objects at
test chart, tbe characters consist of letters or j refractive errors. Hyperopia may be confused with myopia when a
varying distances from the eye within a range of
numerals each stroke* nr qnnrr* nf which nrrnmcc ---- 4-—\ Tut. :..i________________ I child holds a book close to his eyes in order to
\sec uijuvcj. i^ignt rays entering me eye rroni 1
character occupying 5 minutes of arc at the stated • Inheritance of Refractive Errors In children such nonvisual symptoms as '
objects farther away than 20 feet are considered
distance. headache and disinterest in reading are <
to be parallel, and so accommodation is not
Many expressions of relative acuity have ! Refractive errors of all kinds tend to be occasionally associated with,hyperopia of [
necessary. In this case the eye is said to be
been devised to attempt to record the value as a . inherited but in no definitely predictable way. An moderate to severe degree. ‘ B-tf j ;;
unaccommodated.
■
192 193
‘I ! ■ - >
i! r ;
} ( [-Convergent strabismus in children is Heredity plays a large role in myopia. Myopia be worn all day and should be removed during . Clinical Findings
[frequently Associated with hyperopia. usually increases during the teen ages and levels sleep. They are not recommended for patients
j’ .* ‘The lens required in the ophthalmoscope to off at about age 25 regardless of external factors with chronic ocular allergies orinfections. Lasik The manifestations ofdegenerative myopia are
fo’cus on the retina is a rough index of the degree such as amount of close work, lighting, rest, surgery would be performed in myopia. the same as those ofsimple myopia except th at with
of hyperopia (or myopia). The space between the vitamins, endocrine balance, exercise, etc. severe degenerative changes the visual acuity may
jetirjal vessels is smaller than normal, the optic not be corrected to normal with any lenses. The
(disklis smaller than normal, and the retinal veins degenerative changes may have no direct
rnay'jbe tortuous. Precise diagnosis depends upon Clinical Findings PSEUDOMYOPIA relationship to the degree ofmyopia. Thus a myopia
•the trial lens examination. Because children have of mild degree may show marked degenerative
'strorig accommodative powers, a cycloplegic drug The most common symptom is the inability changes, while a “high” myopia of 6-20 diopters may
is required.; Because the accommodative powers to distinguish objects clearly in the distance, such Pseudomyopia is a rare disorder that is show no degenerative changes. The diagnosis,
decrease iri adults, this is not necessary after as the blackboard in school or road signs. Many usually due to a spasm of the ciliary muscles therefore, depends upon ophthalmoscopic
about age 40. myopic children, however, never having (spasm of accommodation), which causes examination. The main characteristics are as follows:
•! •• • | experienced sharp distance vision, are not aware parallel rays of light to converge to a focus in
ofwhat is missing and are usually only discovered front of the retina, as in true myopia. This may A. Changes at the Optic Disk: A temporal
ilYeatnicnt to be myopic by routine visual screening tests in occur with excessive accommodative effort in crescent is seen ophthalmobcopically where the
school. uncorrected hyperopia, or it may be a hysterical choroid pulls away from the edge of the disk,
p b .Hyperopia may be of incidence of the light baring the white sclera. Outside-of this area there
Frowning (squinting) in an effort to see is phenomenon.
ray’s entering the cornea and lens. Correction ofa common, as the acuity is sharpened by making a The diagnosis of pseudomyopia is may be a second choroidal crescent of disturbed
moderate degree of hyperopia in children without tiny lid aperture similar to the “pinhole camera"; confirmed upon relaxation of the spasm (and pigmentation. In nasal supertraction of .the
symptoms dr strabismus is rarely necessary. this achieves a lenslike focus by eliminating the correction of the myopia) by the instillation vessels on the disk, the retinal tissue seems to
peripheral rays of light entering the eye, allowing of a cycloplegic drug. Refraction without the have been pulled over the nasal edge of the disk,
only the more axial rays to reach the retina for a use of a cycloplegic drug and the prescription distorting the course of the vessels.
Course & Prognosis clearer image. (This increases the depth of focus of concave lenses only necessitate greater
j! . ■ ■■ i accommodative effort, which increases spasm B. Changes in the Choroid and Retina: The
and is similar to “stopping down” the aperture of
H fj -Most children with hyperopia do not need a camera.) This frowning sometimes results in and therefore the degree of pseudomyopia. choroid is stretched and thinned, with areas of
corrective lenses unless esotropia is present. As fatigue headaches and lid irritation and is often atrophy and pigment change. These changes may
actpiiitnodativc ability fails (late in the fourth interpreted as photophobia. result in bare white patches of sclera showing
Heqa’de), a -partial strength correction may be Myopic children may hold their reading through, or in pigment clumping resembling the
nepdtdfor reading. Bifocals (or 2 pairs of glasses) DEGENERATIVE MYOPIA changes seen in chorioretinitis. Rarely, there may
material quite close to the eyes if the degree of
ijreiusuhlly required by age 45. myopia is sufficient to bring the far point closer (Malignant Myopia, be a proliferation ofan unusual amount of.pigment
than the normal reading distance. Progressive Myopia) in the region of lhe macula to form the Forster-
Fuchs black spot.

I SIMPLE MYOPIA Treatment Since myopia is frequently progressive, the C. Changes in the Sclera: There may be a
(Nearsightedness) term “progressive myopia” is a pooT one for localized stretching of the sclera to form a
> this special condition, which maybe more aptly staphyloma. The most common site is the posterior
Concave (minus) lenses to diverge the rays
of light so that they will focus on the retina will called degenerative myopia, since the diagnosis pole of the eye. The staphyloma increases the
i’: In myopia, parallel rays of light are brought afford the myopic individual “normal vision” and is based upon the changes which are observed axial length of the eye and thus makes that area
io ia [focus in front of the retina. Thus the “far should not be looked upon as a crutch which will 1 in the retina and choroid upon ophthalmoscopic more myopic than the surrounding retina.
jjoint[*df thtj eye, wliich is at infinity in emmetropia be habit-forming or which should be limited in j examination.
ahfi hyperopia, is at a definite finite distance use. Corrective lenses neither “strengthen” nor s Degenerative myopia is much less common D. Changes in the Vitreous; Fibrillary
sbine where [short of 20 feet (6 meters), according “weaken” eyes but allow the patient to see more | than simple myopia. It has been reported to have degeneration and posterior vitreous detachment
io ijic degree of myopia. Willi myopia of 1 diopter, clearly or more comfortably. i a higher incidence in females and in certain races are common.
for example1, the far point of clear focus is 1 meter : and ethnic groups, eg, the Chinese, Arabs, and J£
Corrective lenses for mild degrees of myopia
fromithe eye; as the myopia increases, the far point (less than 1 or 1.5 diopters) in children in the first i Jews, and is undoubtedly genetically
decreases (inverse ratio). few grades of school are not indicated, as the i deteraiined-usually as a recessive. It is more Pathology
i h Myopia may be caused by largeness of the demands for sharp distance vision are not great | common in Southern Europe than in Northern
^ypbUll (axial myopia) or by an increase in the "during this time. I Europe and the USA. Two types have been Pathologic changes are degenerative in
sircrigth ofi the refractive power of the media In recent years, corneal contact lenses have j described: congenital and developmental. The nature, with a generalized thinning and atrophy
(refractive myopia). Most cases are axial in type. enjoyed a great increase in popularity'- They may I pathology is the same in either case. of the coats of the eye.
i
194 195
Treatment during the growth period, most moderate astigmatic
Cataract s Clinical Findings
errors are fairly constant throughout life. Irregular
As long as the visual acuity can be corrected With mild degrees ofastigmatism there may astigmatism due to kcTataconus usually progressed
In the incipient stage ofcataract development
with concave lenses* these should be used. be no symptoms, or merely asthenopia with to dn uncorrcclable degree; corneal transplants are
thE change in the substance of the lens may be
Surgical shortening or reinforcing of the posterior merely a fluid change, producing myopia without prolonged use of the eyes. The person with eventually necessary.
sclera is still largely experimental. Complications noticeably affecting the transparency of the lens. astigmatism tries to achieve a clearer image by
(eg, retinal detachment) have to be specifically rapidly changing focus (accommodation), with
dealt with. resultant fatigue. With greater degrees of
astigmatism, clear visual acuity may not be PRESBYOPIA
possible at any distance. Astigmatism may be (Old Sight)
ASTIGMATISM
Course & Prognosis discovered through routine screening tests in
school or through comparison of visual abilities
The degenerative changes may gravely with normal associates.
At birth the lens substance is soft and pliable
Astigmatism is distorted vision caused by
affect visual function. Thinning and atrophy of The child with astigmatism tends to hold and is easily altered in shape by the action of the
a variation in refractive power along different
the choroid is almost always accompanied by reading material close. There is also a tendency ciliary muscles (accommodation). Throughout life
meridians of the eye. Astigmatism may be
loss of retinal function in the involved area. to firown to obtain the “pinhole” effect, as in there is a gradual hardening of this substance?,
“regular” or “irregular.” “Regular” astigmatism
Choroidal hemorrhages may occur. Changes in myopia- Headaches due to the frowning and beginning with the nucleus, so that it becomes
may be “with the rule” or “against the rule."
the macula will markedly reduce central vision. accommodative effort are common. more resistant to changes in shape until by th.c
Most cases are due to irregularities in the shape
Retina] detachment is a frequent complication, Because ofthe difference in retractive powers mid 40s the average person with normal vision
of the cornea, but the lens may also cause
and vitreous degenerative opacities reduce astigmatism (eg, in old age, due to cataractous of the 2 principal meridians, the retinal blood has difficulty in focusing on near objects and
vision. There is also a higher incidence of vessels coursing in one direction may be out of reading fine print. This is presbyopia, and dods
change). Heredity plays a role in astigmatism
cataracts and secondary glaucoma among these focus upon ophthalmoscopic examination while not imply an increase in hyperopia but merely =a
as well as in myopia and hyperopia.
patients. those in the opposite meridian are clear, and a lessening of the effective powers of
In regular astigmatism the varying
refractive powers in different meridians can be different lens must be rotated into the accommodation.
reduced to 2 principal meridians at right angles ophthalmoscope to obtain a clear image. The disk
to each other. In astigmatism with the rule the is elongated. The retina itselfis normal. The precise
ACQUIRED MYOPIA vertical meridian has the greater curvature. This determination of the amount and axis of Clinical Findings
is usually the case with astigmatism in young astigmatism depends upon a careful refraction with
people, since most such cases are caused by trial lenses. The symptoms occur with close work. There
A few instances of myopia do not fall into the shape of the cornea. Later in life the cornea is blurring of near objects, or discomfort or fatigue
the above categories. If there is a history of tends to flatten, so that small amounts of with attempted near work. Persons with normal
fairly rapid blurring of distant vision and if a astigmatism with the rule may disappear, or | Treatment vision may complain ofa blurring of distant objects
myopic change in refractive error has been astigmatism against the rule (with the greater for a few moments after looking up, due to the lag
demonstrated, several conditions should be curvature in the horizontal meridian) may The refractive error is treated with a cylindric of relaxation of accommodation after excessive
investigated. They must be differentiated from I concave or convex lens oriented in the proper effort at near focusing. Usually the person has
appear. Irregular astigmatism is due to the
true refractive errors. ! meridian (axis) to restore a spherical effect, with a noticed that he must hold his reading material
uneven bulges ofkeratoconus, comeal scarring,
lens irregularities, and the pressure on the cornea I combination lens incorporating concave or convex farther from his eyes to see the print, andia
of small tumors in the lids. spheres for the resultant myopia or hyperopia. frequent statement is to the effect that his “arms
Diabetes Mellitus Astigmatism is identified as to the type of Small degrees of astigmatism are physiologic and are too short.”
cylindric lens needed to correct it: a concave need no correction.
Uncontrolled diabetes mellitus tends to cylinder would be used for myopic astigmatism, Mild degrees of irregular astigmatism due to
produce refractive error, either myopia or and a convex cylindric lens for hyperopic { keratoconus or corneal astigmatism which cannot Treatment
hyperopia, probably as a result of a change in astigmatism. i be corrected with simple lenses are often benefited
tissue fluids in the lens substance. Control of Astigmatism may be simple (no hyperopia j by corneal contact lenses, which tend to restore Following refraction, a lens is prescribed
the diabetes by appropriate medical means or myopia); combined with hyperopia : the spherical refracting surface over the cornea. which corrects the basic refractive error, together
usually reverses this state. The authors have (compound hyperopic astigmatism) or with with die proper convex reading addition (“add”)
examined several such cases where routine myopia (compound myopic astigmatism); or which brings the near point within suitable range
urine examination for sugar and even fasting mixed (myopic combined with hyperopic Course & Prognosis for the individual’s requirements. Since this lens
.blood glucose values were normal, but where astigmatism or hyperopic combined with myopic will blur the distance vision, these requirements
glucose tolerance tests confirmed the suspected astigmatism). Aside from the changes described above which may necessitate a bifocal or multifocal lens for
■diabetes. tend to alter small amounts of regular astigmatism convenience in use. Some persons are content

196 197
wftlv'the use of only the reading tens, whereas prescription is required about every 2 years. As
others prefer 2 pairs of glasses.
4 I
these powers fait in the following years, several
changes in strength of reading additions may be
17
Course & Prognosis
required until the early 50s, at which time the glasses
accomplish most ofthe change in focus required for
PREVENTIVE OPHTHALMOLOGY
close work. Little changemay be necessary thereafter
•; The average person requires hisfirst reading unless there is some alteration in the refractive state
glasses between the ages of 42 and 45. This first of the eye caused by developing lens opacities,
prescription merely: makes up for the deficiency glaucoma, degenerative myopia, or other pathologic
of die accommodative powers at that time. A new changes.

I
The most productive area of health care is visual field examination is normally done on the
that of prevention. Prevention of ocular disorders tangent screen. Confrontation field testing using
; includes both elimination of hazards to the eyes the examiner’s fingers as a target will pick up only
i (eg, accident prevention, sterility of ophthalmic gross visual field defects.
; solutions) and recognition of disease states at a In recent years many glaucoma surveys have
| time when improvement, containment, or cure can been carried out in various parts of the world.
i be achieved with proper management (eg, Their chief value is case-finding. Tonometry-
I glaucoma). should be a part of every physical examination in
The responsibility for effective prevention the age group over 20. This can be accomplished
: often rests with nonspecialists. If all practicing by teaching tuuometry to medical students,
•- physicians were alert to the special problems of general practitioners, internists, and other
• preventive ophthalmology, many cases of visual interested physicians. It would also be worthwhile
i impairment due to injury, glaucoma, strabismus; to establish permanent glaucoma detection
j infection, etc could be prevented. It is indeed a centers. These could be established as single units
; tragedy to find hitherto undetected advanced or as parts of multiphasic disease detection
ocular disease in a patient who has been receiving centers.
regular medical treatmentfor another illness. Every
physician should be aware of the disorders
potentially leading to blindness so that he may
take advantage of the opportunities for prevention AMBLYOPIA EX ANOPSIA
by detection, proper management, and education ’ (“Lazy Eye”)
of tile patient.

Amblyopia ex anopsia can be defined for the

purposes of this discussion as decreased visual
GLAUCOMA acuity in one eye in the absence of organic eye
disease. The function of vision develops from birth
to age 7. If vision has not developed by age 7,
Approximately 2% of all persons over 35 there is no chance that it will develop later.
have glaucoma. Patients do not seek treatment In the absence of eye disease, the 2 main
until late in the disease because there is no pain abnormalities that will prevent a child from learning
and the visual acuity (central vision) remains good to sec are strabismus and anisometropia.
as the peripheral field is constricting.
The best means of detecting glaucoma is Strabismus: If a young child develops
routine tonometric examination every 3 years on esotropia or exotropia, he will have double vision.
all persons over 20 (or every year if there is a He quickly leams to suppress the image in the
family history of glaucoma). The next most deviating eye and therefore uses only the straight
important diagnostic steps are the visual field eye, and leams to see normally with this eye.
examination and the ophthalmoscopic examination Unfortunately, vision does not develop in an
for pathologic cupping of the optic disk. The unused eye; unless the good eye is patched thus
198 199
 1•
forcing the child to use the deviating eye, sight pharmacists should exercise at least the same | Most states have laws requiring the use of silver of function or may cause significant atrophy <if
never will develop in that eye and the child will degree of care in compounding ophthalmic j nitrate. the tissue and produce a defect which is visible
grow up with one perfectly normal eye which is solutions for use in injured eyes as iu preparing ophthalmoscopically as a macular hole. In the latter
essentially blind since it has never developed a solutions for intravenous administration or for use case a permanent central scotoma will result.
I
functional connection with the visual centers in in a surgical field. Eclipse retinopathy is easily preventable if
the brain. This is more likely to occur with esotropia In this discussion, “intact eye” will be used adequate filters arc used in observing eclipses,
than with exotropia. to mean one in which the corneal epithelium has OCULAR INJURIES but the safest way io do so is on television.
not been damaged and “injured eye” one in which The damage which results from a focus of
Anisometropia: Young children are more the comeal epithelium has been damaged. The intense light upon the retina has been put to
concerned with perception of near objects than use of sterile eye drops is desirable in the care of practical use in the photocoagulation treatment
with distance vision. If one eye is nearsighted an intact eye, but in the case of an injured eye it is INTRAOCULAR FOREIGN BODIES ofretina] detachment and certain vascular diseases
(myopic) and the other farsighted (hyperopic), the an absolute necessity. Fortunately, the vast of the retina. Some of the complicntiorri of this
child will favor the nearsighted eye. Small children majority of liquid eye medications areusedathome type of therapy can be prevented by skillful
are concerned primarily with near objects, and it and in the physician’s office in eyes whose corneal Intraocular foreign bodies still occur as application of the beams.
takes no effort to focus the nearsighted eye epithelium is intact. If contaminated drops arc industrial accidents. They usually happen when
whereas it does take effort to focus with the instilled into an intact eye, a conjunctivitis may a worker is striking steel on steel and a piece of
“farsighted eye. Hence the farsighted eye will not result which can usually be brought under control; the hammer flies off at high speed and passes
be used even though it is straight. The result will if they are instilled into an injured eye, serious through the sclera or cornea into the eye. BLOWOUT FRACTURE OF THE ORBIT
be the same as in untreated strabismus, ie, infection can develop and the eye may even be Prevention consists of properly fitted safety
monocular blindness due to failure of visual lost. Therefore, 2 standards arc recommended for goggles.
'development in an unused eye. The incidence of the preparation and use of ophthalmic solutions: Early repair prevents enophthalmos and oilier
anisometropia is about 0.75-J %. (1) For the intact eye, sterile solutions which may complications.
The best way to prevent amblyopia is visual be packaged in multiple-dose containers. (2) For
acuity testing of all preschool children. By the the injured eye, small single disposable units RADIATION INJURY
time a child reaches school, it is usually too late containing sterile solutions for single patient use. * * *
for occlusion therapy. The test can be done by Two procedures that will ensure sterility of
i
the parents at home using the illiterate “E” chart. multiple-dose containers are autoclaving and • Ultraviolet irradiation may cause superficial
Pediatricians and othera responsible for the care of sterile filtration. ; epithelial keratitis manifested by pain, redness,
small children should test visual acuity at age 4. J and photophobia. The symptoms appear 6-12 . XEROPHTHALMIA
i hours after exposure. This type of injury occurs
i following exposure to ultraviolet light, eg, while
OPi iTHALMIA NEONATORUM | skiing or using an electric welding device. Vitamin A deficiency disease may be due to
CORNEAL OR INTRAOCULAR INFECTION I Prevention consists of avoidance of exposure or deficient diet resulting from poor economic
! the wearing of appropriate protective sunglasses circumstances, chronic alcoholism, deprivation
In many parts of the world it is customary' for ; or goggles. weight-reducing diets, dietary therapy of an
The invasion of the cornea by an antimicrobial agent io be placed in the eyes of allergic condition; or failure of absorption of
microorganisms is barred effectively by the intact newborn babies. This procedure was originally vitamin A in the gastrointestinal trac] (as in
Solar retinitis (eclipse retinopathy) is a
comeal epithelium. Once this barrier is broken, designed for the prevention and treatment of : specific type of radiation injury which usually obstruction of bile ducts or the common duct,
however, organisms may enter the cornea freely gonococcal conjunctivitis, which, if allowed to occurs following solar eclipses as a result of direct or in severe diarrhea). In prescribing {dietary
and cause infection. One very dangerous and progress, can cause corneal ulceration and observation of the sun without an adequate filter. therapy for any disorder the physician must be
opportunistic organism, which actually grows in blindness. For many years silver nitrate (1%) was Under normal circumstances sun-gazing is difficult careful to include vitamin A among the vitamin
the cornea better than in any other known medium, the only solution used, and this is still the only : because of the glare, but cases have been reported supplementation. The estimated: daily
is Pseudomonas aeruginosa. This organism may method of prophylaxis approved by the FDA. If ■ in young people who have suffered self inflicted requirement of vitamin A is 1500-5000 IU daily
be a contaminant of many commonly used eye inadvertently used in strong solution (5-10%), macular damage by deliberate sun-gazing, perhaps for infants and children and 5000 IU dpily for
solutions. silver nitrate can cause permanent corneal scarring while under the influence of drugs. The optical adults. Large parenteral doses are used for
Any ophthalmic solution placed in an injured and possibly blindness. system of the eye behaves as a strong magnifying treatment of the established process; Early
eye must be sterile. If a contaminated solution Penicillin and other antibiotics have been lens in focusing the light onto a small spot ou the recognition and treatment of vitamin A
has been instilled into the eye, a serious infection used in many hospitals. Penicillin is effective macula, usually in one eye only, producing a deficiency disease can prevent loss of sight due
will probably result and may destroy the cornea against Neisseria gonorrhoeae, docs not cause thermal bum. The localized edema of the retinal to comeal perforation or secondary infection.
and ultimately the eye. For this reason physicians, sensitization in newborns, and does not cause a tissue which results may clear with minimal loss
nurses, and manufacturing or prescription chemical conjunctivitis as silver nitrate often does.

200 201
 EXPOSURE KERATITIS chronic open-angle glaucoma and cataracts and
can both provoke and worsen attacks of herpes 18
simplex keratitis. Locally administered,
i Patients in coma who have lid retraction corticosteroids are much more potent in this
respect and have the added disadvantage of
SPECIAL SUBJECTS OF
(exophthalmos) or facial nerve paralysis may
develop exposure keratitis. This condition can be
presented by patching the eyes, suturing the lids
causing fungal overgrowth if the corneal
epithelium is not intact. For these reasons,
PEDIATRIC INTEREST
together (tarsorrhaphy), artificial teardrops corticosteroids in any form1 must be administered
instilled frequently into the eyes, or application with discretion.
of a soft contact lens.

Hazards of Chloroquine

VISUAL LOSS DUE TO DRUGS Chloroquine given over a prolonged period,

& OTHER IATROGENIC FACTORS as in the treatment jof discoid lupus The immediate examination of the newborn examination will demonstrate any comeal, lens, or
erythematosus, involves an approximately 10% I infant consists of a brief observation of color, vitreous opacities as well as abnormalities in the
risk of partial visual loss. The corneal opacities ? responses, extremities, and digits, and a quick fundus. Neonatal retinal hemorrhages have been
- i s inspection of body surfaces. The more complete
Different! litDiagnosis of “Red Eye” caused by chloroquine disappear when the drug reported in 15% of newborns,’ usually clearing
■j . i is discontinued, but macular edema and • examination is done in the nursery. completely within a lbw weeks and leaving no
?! ‘ ! . Because the development of the eye often
In the .local treatment of eye disease the pigmentation are often irreversible. Patients permanent visual dysfunction.
requiring chloroquine on. a chronic basis should ' reflects organ and tissue development ofthe body
patient with the “red eye” presents the most
common diagnostic challenge. The majority of be examined by an ophthalmologist every 4-6 as a whole, many congenital somatic defects are
casps of ocular inflammation are due to months. Between examinations the patient j mirrored in the eye. A careful eye examination soon
conjunctivitis, which is usually self limited; but if should make a rough test of Iris own visual ; after birth may suggest the need for further THE NORMAL EYE IN INFANTS
a diagnosis pf conjunctivitis is not clear-cut one acuity once a week. ; investigative procedures. Subjective response is & CHILDREN
« limited to uie following response to a moving light
must consider iritis, glaucoma, and comeal ulcer-
all of which are far more severe and a greater threat The only instruments required for the ocular
Hazards of Mydriatics . examination of the newborn are a good hand light, Eyeball
to vision than conjunctivitis. If the patient has
an ophthalmoscope, and a loupe if necessary for
iritis, the pupil must be dilated with a cycloplegic
Iu dilating the pupils *.o facilitate observation : magnification. In (lie newborn, the eye is relatively larger in
such as atropine or scopolamine. If the patient
has:'glauconia, cycloplegic agents may aggravate of the lens and retina, the physician should comparison with body size than in later life.
the condition. Corneal uLcer must be treated estimate the depth of the anterior chamber. If the However, the anteroposterior diameter, which
iris appears to be quite close to the cornea, it is Extern al Inspection determines the-focusing of the eye, is relatively
promptly with specific antibiotics in order to
prevent scarring. best to leave the decision to dilate in the hands of shorl (averaging about 17.3 mm). This would
the ophthalmologist. Acute angle-closure The eyelids are inspected for growths, produce a marked hyperopia if it were not for lhe
'! • i ’ glaucoma can be produced in less than 30 minutes, deformities, lidnotches, and symmetric movement greater curvature of the lens at this time.
and surgery is almost always required to relieve with opening and closing of the eyes. The
Hazards of Prolonged Corticosteroid Therapy
it. Surgeiy is usually but not always successful, absolute tn J relative size of the eyeballs is noted,
and permanent and complete blindness may be as well a*, rosition and alignment The size and Cornea
It has-been clearly demonstrated that long­
Lhe result. luster of the corneas are noted, and the anterior
term systemic corticosteroid therapy can cause
chambers are examined for-clarity and iris The cornea of the newborn is also relatively
configuration. The size, position, and light reaction large and reaches adult size by about 2 years of
of the pupils are also noted. age. ft is flatter than the adult cornea, however,
and the curvature is greater at the periphery than
in the center; whereas the opposite is true in the
Ophthalmoscopic Examination adult.

With undiluted pupils, some information can

be obtained by use of the ophthalmoscope in a Lens
dimly lighted rcoiu. Ideally, however, all newborns
should be examined with an ophthalmoscope At birth the lens is more globular than in
through dilated pupils. Ophthalmoscopic adulthood, and its greater refractive power
202 203
compensates for the shortness of the eye. The greater curvature of the lens is the rule jn | nasolacrimal System almost white at the periphery. In black infants there
lens grows throughout life as new fibers are added prematures, and leads to myopia (usually is more pigment in tire fundus and a gray-blue;
>. * •
to the periphery, and this causes it to flatten. The temporary). E. fetal development of the nasolacrimal sheen is seen throughout the periphery. In white
consistency of the lens material changes I - passages begins as cords of cells which usually infants a white periphery is normal and should
throughout life from a soft plasticlike material to -•-hollow out about the time of birth. Because there not be confused with retinoblastoma. During the,
the glassy consistency seen in old age. This Iris .'may normally be a few weeks’ delay in duct next several months pigment continues to be;
accounts for the gradual loss in power of . formation, failure of tear production in the first deposited in the retina, and usually at about 2
accommodation with advancing age. At birth there is little or no pigment on lhe few weeks does not necessarily indicate any years of age the adult color is evident.
anterior surface of ths iris. The posterior pigment ^difficulty; failure of the tear ducts to function by 3
layer shows through the translucent tissue months of age, however, demands attention.
usually giving the effect of a bluish or slate-gray
Pediatric Eye Examination Schedule color. As the pigment begins to appear on the CONGENITAL EYE DEFECTS
anterior surface, the iris assumes its definitive j Optic Nerve .
Hospital Nursery color. If considerable, pigment is deposited, the
External eye examination and eyes become brown. Less pigmentation results in L T‘ ie rnedullation of optic nerve fibers usually Most congenital ocular defects aT-e.
ophthalmoscopic examination through dilated, blue, gray, hazel, or green eyes. It may take 1-2 • takes place soon,after birth. ' genetically determined. Examples include
pupils as outlined in the text. Two drops of years for the pigmentary deposits to occur; in the congenital ptosis, refractive errors, aniridia,'
sterile 5% homatropine and 2.5% phenylephrine meantime it is impossible to ascertain the ultimate strabismus, retinitis pigmentosa, arid
in each eye are instilled I hour prior to color of the eyes. 1 The Normal Ocular Fundus oflnfants & Children arachnodactyly (Marfan’s syndrome). Absence
examination. Special emphasis should be of a positive family history is no proof that the:
placed on the optic disks and maculas; detailed f The ophthalmoscopic appearance of the defect is not in the germ plasm. ! i
examination of the peripheral retinas is not Pupil ?. normal fundus in an infant differs greatly from Other congenital defects may be caused by
necessary. j that of an adult Most of the differences are due interference with the development of the embryo]
In the newborn the pupil is situated slightly ■ to the distribution of pigments. such as the multiple defects associatcd.with rubella
Age 4 to the nasal side of and below the center of the f • .In premature infants, remnants of the tunica infection ofthe mother during die first 3 months of
Visual acuity test with illiterate “E” chart cornea. Because of tire refractive power of the ? vascutosa lentis are frequently visible with the pregnancy. In this instance the infant may suffer
to rule out amblyopia ex anopsia. Visual acuity cornea in the neonatal period, the pupil appears i ophthalmoscope, either in front of the lens, behind from any or all of the following: cataracts, heart
is normal (20/20 [6/6] to 20/30 [6/9] ) by 4-5 larger than it actually is. The apparent diameter j the lens, or in both positions. The remnants are disease, deafness, microcephaly, nricrophthalmosj
yeare of age. varies between 2.5 and 5.5 mm and averages about J usually absorbed by the time the infant has , and mental deficiency'. Eye defects are common in
4 mm. In infancy the pupil is smaller than at birth. ’• reached term, but rarely they remain permanently cerebral palsy. | i
Ages 5-16 The pupillary reflexes appear at about the fifth ‘ and appear as a complete or partial “cobweb” in i :
Test visual acuity at age 5. If normal, test fetal month and are active by the sixth month. At the pupil. At.other times remnants of the primitive * !
visual acuity with the Snellen chart every 2 about age 1 the pupil begins to widen, and it hyaloid system fail to absorb completely, leaving Anophthalmos
years until age 16. Color vision should be tested reaches its greatest diameter during adolescence. • a cone on the optic disk which projects into the
at age 8-12. No other routine eye examination It again becomes smaller in,old age. Myopes have vitreous and is called Bergmeister’s papilla. This is a rare condition in which one or both
(eg, ophthalmoscopy) is necessary if visual larger pupils than hyperopes. Physiologic cupping of the disk is usually eyeballs are absent or rudimentary. There may be
acuity is normal and the eyes appear_nonnal Normal pupils are round and regular and their ; not seen in premature infants and is rarely seen at cither a congenital absence of any ocular structure
upon inspection. diameters are constantly changing, Anisocuria, a term; if seen then, it is usually very slight. In such or an arrest of development to the point where
difference in the size of the 2 pupils, is often a cases the optic disk will appear gray, resembling only histologic evidence is present. The eyelids
normal finding; in the absence of neurologic optic nerve atrophy. This relative pallor, however, are usually present. They are often adherent ;at
abnormalities, it requires no further special gradually changes to the normal adult pink color the margins but can be separated. Anophthalmos
Refractive State diagnostic consideration; at about 2 years of age. is associated with a chromosomal variation. I |
The foveal light reflection is absent in infants.
About 80% of children are bom hyperopic, Instead, the macula has a bright “motherrof-pearl”
5% myopic, and 15% emmetropic. About 10% have Position appearance, with a suggestion of elevation. This Congenital Cystic Eye ’J
refractive errors which require correction before is more pronounced in black infants. At 3-4
age 7 or 8. Hyperopia remains relatively static or During the first 3 months of life, eye. months of age, the macula becomes slightly This is a developmental abnormality resulting
gradually diminishes until 19 or 20 years of age. movements may be poorly coordinated and there concave and the foveal light reflection appears. from complete orpartialfailyreof invagination of-
After age 7-8, myopia often increases until about may be some doubt about the straightness of the The peripheral fundus in the infant is gray, in the primary optic vesicle. The eye is variable in
age 25, with the greatest change at the time of eyes. By 6 months off ge* however, the binocular coni - jSt to the orange-red fundus of the adult. In size and is usually associated with some degree
puberty. There is usually little change in refractive reflexes are well developed; any deviation, noted wbirt infants the pigmentation is more pronounced of neurologic proliferation. The null formation
error during the third and fourth decades. Much after that time should be investigated. near the posterior pole and gradually fades to occurs at about the fourth week of embryonic life.
204 205
Cyclopin recessive trait. It must be differentiated from I Any lens opacity which is present at birth is Marfan’s Syndrome
infantile glaucoma. There are usually no ' a congenital cataract, regardless ofwhether or not
; ' fCyclopia, which is a rare midline fusion of associated defects. 5 jt interferes with visual acu i ty. Congeni tai cataracts This is a congenital’and familial disorder
developing eye structures together with i are often associated with.other conditions. of mesodermal origin which is nearly always
generalized anterior brain and skull defects, is I jrfa.temal rubella during the first trimester of transmitted as an autosomal dominant trait. The
usually not compatible with life since it is Iris & Pupillary Defects ! pregnancy is a common cause of congenital major features are (1) long, thin, spideriike
transmitted by a recessive lethal gene. ' cataract. Other congenital cataracts have a fingers and toes (arachnodactyly), (2)
Misplaced or ectopic pupils aie frequently !; hercditaiy background. generalized relaxation of ligaments, (3)
observed. The usual displacement is upward and j If the opacity is small enough sd that it does generalized muscular underdevelopment, (4)
Palpebral Colobomas laterally (temporally) from the center ofthe cornea. I not occlude the pupil, adequate visual acuity is bilateral dislocation of the lenses (ectopia
■ T | Such displacement is occasionally associated with i attained by focusing around the opacity. If the lentis), (5) abnormalities of the heart and,
; • J A unilateral cleft of one upper lid is the most ectopic lens, congenital glaucoma, or microcornea. | pupillary opening is entirely occluded, however, occasionally, aortic aneurysm, (7) high-arched
common type of palpebral coloboma. Bilateral Multiple pupils are known as polycoria. A true ■ normal sight does not develop, and the poor palate, and (8) other deformities of the sternum,
clefts or fissures can occur in lower as well as pupil must constrict on exposure to light, indicating i fixation may lead to nystagmus and amblyopia ex thorax, and joints.
upper lids and may be associated with other a sphincter muscle. Congenital miosis is due to a : anopsia. The lenses are usually dislocated and
Malformations of the face or globe. No specific poorly developed dilator muscle. Little change in i visual acuity suffers because the patient is not
embryonic maldcvelopment has been established pupillary size is noted after instillation of a seeing through the center of his lenses.
I
as being causative. mydriatic. Congenital mydriasis is characterized by j Choroid & Retina Dislocation is usually upward and often is
large and inactive pupils and underdeveloped '< incomplete. Cataracts frequently develop in the
; ’i •
sphincter muscles, and must be differentiated from ; Gross defects of the choroid and retina are subluxated lenses. Cataract surgery in these
Microphthalmos mydriasis due to juvenile paresis and pineal tumor. 1 visible with the ophthalmoscope. The choroidal cases carries a guarded prognosis.
• I
Coloboma of the iris indicates incomplete closure ; structures may show congenital colobomas,
j ’ Ho microphthalmos, one or both eyes are of the fetal ocular cleft and usually occurs below ’ usually in the lower nasal region, which may also
markedly smaller than normal. Many other ocular and nasally. It may be associated with coloboma • include the iris and all or part of the optic nerve. Osteogenesis Imperfecta
abpohnalitics may be present also, eg, cataract, of the lens, choroid, and optic nerve. Aniridia ; Central posterior (macular) scarring is a pigmentary
glddcoma, aniridia, and coloboma. Somatic (absence ofthe iris) is a rare abnormality, frequently ( disturbance often caused by intrauterine This rare affliction is characterized by
abnormalities are also often present, eg, associated with secondary glaucoma and due to i toxoplasmosis. Other congenital lesions of the increased fragility of the bones and laxity of
poiydactyly, syndactyly, clubfoot, polycystic an autosomal dominanthereditaiy pattern. Various choroid and retina include drusen, aneurysms, the ligaments, with frequent fractures and
kidneys, cystic liver, cleft palate, and abnormalities in the shape of the pupils have been optic nerve malformations, medullated nerve dislocations; dental defects, deafness, andblue
meningoencephalocele. Microphthalmos is neatly described but are not necessarily significant. ‘ fibers, and hereditary macular degeneration. scleras. The blue color is darker in the anterior
always genetically determined-most frequently as Persistent mesodermal remnants usually appear as parts of the scleras over the ciliary bodies. It is
a recessive but occasionally as a dominant trait threadlike bands running across the central thought to be due to abnormal thinness of the
pupillary space and attached to the lesser circle of sclera and remains unchanged throughout life.
the iris. They rarely have clinical significance or DEVELOPMENTAL BODY DEFECTS Cataracts, incgalocornea, and keratoconus may
Corneal Defects interfere with visual acuity. ASSOCIATED WITH OCULAR DEFECTS also be present. It nearly always occurs as an
The color of the iris is determined largely by autosomal dominant trait.
; There may be partial or complete opacity of heredity. Abnormalities in color include albinism,
the -corneas such as is found in congenital due to the absence ofnormal pigmentation of the Albinism
glaucoma, faulty development of the cornea until ocular structures and •frequently associated with
persistent comeal-lcns attachments, birth injuries, poor visual acuity and nystagmus; and Congenital deficiency ofpigment may involve Gargoylism or Hurler’s Syndrome
intrauterine inflammation, interstitial keratitis, and heterochromia, a difference in color in the 2 eyes the entire body (complete albinism)'or a part of
mucopolysaccharide depositions of Uie cornea as which may be a primaiy developmental defect with the body (incomplete albinism). When incomplete This is a rare condition due to autosomal
in Hurler’s syndrome. The most frequent cause of no functional loss or may be secondary to an albinism involves only the eye, the function may recessive inheritance in which there is infiltration
bphque corneas in infants and young children is inflammatory process. bE normal orimpaired. In complete ocular albinism, of mucopolysaccharides into the tissues,
congenital glaucoma. In most instances, the eye there is usually an abnormal development of the especially the liver, spleen, lymph nodes,
is ’ larger than normal (macrophthalmos,* macula, a severe refractive error, nystagmus, and pituitary gland, and corneas. Other oculaT
hytltophlhalmos, buphthalmos). Birth injuries may Lens Abnormalities severe photophobia. The eyebrows and signs include slight ptDsis, larger thickened
cause extensive comeal opacities with edema as a eyelashes are white, the conjunctiva is hyperemic, eyelids, and strabismus (esotropia). The
reSujt of rupture of Descemct’s membrane. These The lens abnormalities most frequently noted the irides are either gray orred, and the pupil appears corneas show a diffuse haziuess, which
Usually clear spontaneously. are cataracts, although there may be faulty : red. Treatment consists of relieving photophobia progresses to a milk-white opacity. Glaucoma
t * b Mcgalocomea is an enlarged cornea with development, forming colobomas, or subluxation, by means of tinted glasses or opaque contact lenses may eventually develop. There is no
normal function usually transmitted as an X-linked as seen in Marfan’s syndrome. in which only a small hole is left in the center. satisfactory treatment.
206
Oxycephaly Leu kocoria (White Pupil)
ofDown’s syndrome). Strabismus and nystagnius f blennorrhea has its onset between the fifth and
(Acrocephaly, Tower Skull, Steeple Head) are also present. ? ; tenth days. The presence of typical inclusion
Parents will occasionally see a white spot
■ bodies in the epithelial cells confirms this
This deformity is evident at birth but is through the infant’s pupil (leukocoria). Although
often attributed to normal distortion during i diagnosis.
Laurence-Moon-Biedl Syndrome i ■ Silver nitrate conjunctivitis, is usually self retinoblastoma must be ruled out, the opacity is
delivery and is seldom diagnosed at the time of more often due to cataract, retrolental fibroplasia, I;
i ‘ limited. Bacterial conjunctivitis requires instillation
delivery. It is characterized by a high, dome­ persistence of the tunica vasculosa lentis, or
This syndrome includes retinitis pigmentosa, \ of antibacterial agents such as sodium
shaped or pointed skull, high forehead, bulging polydactyly. obesity, hypogenitalism, and mental ! sulfacetamide, bacitracin, or tetracycline ointment comeal scarri ng.
temporal fossae, flattened cheekbones, shallow retardation. for several days. Treatment should be instituted
orbits, a high, narrow palatal arch, and
j without waiting for the results of the culture.
synostosis of the cranial sutures. Syndactyly Retinoblastoma
• Inclusion conjunctivitis is treated with
may also be present. The ocular signs include
i .'sulfonamide or tetracycline ointments,
exophthalmos (due to flatness of the orbits), This rare malignant tumor ofchildhood is fatal jl
POSTNATAL PROBLEMS j Silver nitrate solution (1 %) .should be used
wide separation of the eyes, and exotropia. . if untreated. Two-thirds of cases occur.before the .J
i j in sealed, single use, disposable containers. Some
Closure of the eyelids may be difficult or . end of the third year; rarely, cases have been 3
j institutions advocate the. use of antibiotic
impossible. Loss of vision may follow increased reported up to 7 and 10 years of age. In.about 30%
The commonest ocular disorders of children ’ ophthalmic preparations in place of silver nitrate.
intracranial pressure. Nystagmus is common. of cases, retinoblastoma is bilateral. The tumor ||
are external infections of the conjunctiva and Prenatal diagnosis and treatment of maternal
Various operative procedures have been results from mutation of an autosomal dominant
eyelids (bacterial conjunctivitis, sties,blepharitis), I gonorrhea will prevent many cases of-neonatal
devised for the relief of intracranial pressure. If strabismus, ocular foreign bodies, allergic . gene which is passed-with fairly high.penetrance.
I gonococcal conjunctivitis; however, prophylactic
vision is to be preserved, surgery must be ■. Children of survivors therefore have a nearly 50% ■J
reactions ofthe conjunctiva and eyelids, refractive j medication of(he newborn must not be neglected,
performed before optic atrophy has progressed. errors (particularly myopia), and, to a lesser extent, chance of having retinoblastoma. It is more upt to
j Pievention ofinclusion conjunctivitis is difficult since
The Syndrome is due to an autosomal dominant be bilateral in succeeding generations. Parents j!
the congenital eye disorders. Since it is more j it is carried in the mother’s genitourinary tract.
gene of weak penetrance. difficult to elicit an accurate history of causative who have produced one child with retinoblastoma ■=
’ Instillation ofsilver nitrate or an antibiotic (usually
factors and subjective complaints in children, it is run a 4-7% risk ofproducing the disease in each
I neniciilin) is required by law in most states.
subsequently born child. Retinoblastoma is J
not uncommon to overlook significant ocular
Acrobrachyccplialy disorders (especially in very young children). usually not discovered until it has advanced far jj
Aside from the altered frequency of occurrence enough to produce an opaque pupil. Infants and U
iRetrolental Fibroplasia
In this abnormality the head is wide, of the types of ocular disorders, the causes, children with presenting symptoms of strabismus ;■
whereas in oxycephaly it is narrow. manifestations, and treatment of eye disorders are
should be examined carefully to rule out-j
This condition, seen.as a white Tetrolental
AcrobrachycephaLy is caused by premature about the same for children as for adults. Certain retinoblastoma, since a deviating eye may be the ; j
membrane in the pupillary opening, was until quite
closure of the coronal sutures. Growth occurs special problems encountered more frequently in first sign of the tumor.
recently a frequent cause of blindness in infants
only laterally and vertically, and .the Enucleation is the treatment of choice in all b
infants and children are discussed below. weighing less than 2 kg. It occurred as a result of ■q
anteroposterior diameter is short The ocular unilateral cases.
high oxygen concentrations in incubators.
signs are similar to those of oxycephaly.
Other abnormalities involving the Ophthalmia Neonatorum
development of the skull are scaphocephaly (Conjunctivitis ofthe Newborn) Juvenile Xnnthogranuloma -i
Congenital Glaucoma
(increased anteroposterior diameter due to i| ■
premature closure Df the sagittal suture) and This uncommon entity is most ofteiij f
Conjunctivitis in tlie newborn may be of Congenital glaucoma (see Chapter 13) may
phigiocephaly (asymmetric flattening, usually due chemical, bacterial (including chlamydial), or viral diagnosed by spontaneous unilateral hyphema;.-
occur alone or in association with many other
to premature closure of a single coronal suture). The fellow eye is normal. Diffuse thickening arid5
origin. Differentiation is usually made according congenita! lesions. It is important to recognize
to age at onset and by appropriate smears and nodulation of the iris may be seen on slitlanip,
t this disorder early, as untreated.glaucoma
cultures. Chemi cal conjunctivitis caused by the examination. The iris lesion is occasionally
results in permanent blindness. It is often
Craniofacial Dysostosis (Crouzon’s Disease) silver nitrate drops instilled into the conjunctival localized enough to be excised. Associated skin
bilateral. Early signs are corneal haze or opacity,
sac at birth is the most common form. Inflammation lesions or nevoxanthoendotheliomas are frequent! ’
increased corneal diameter, a deep anterior
This rare hereditary deformity, due to an is greatest during the first or second day of life. The small, elevated yellowish nodules appear id
chamber, and ill-defined discomfort. Since the
autosomal dominant gene, is characterized by Bacterial conjunctivitis is usually of outer coats of the eyeball are not as rigid in the the skin of infants and characteristically TegresS i
exophthalmos, atrophy ofthe maxilla, enlargement staphylococcal, pneumococcal, pseudomonas, or spontaneously. The ocular and dermal lesions are:
child, the increased intraocular pressure
of the nasal bones, abnormal increase in the space gonococcal origin, the latter being the most serious expands the corneal and scleral tissues. Useful not neoplastic and microscopically arc composed
between the eyes (ocular hypertelorism), optic because of potential corneal damage. The onset vision may be preserved by early diagnosis and of multinuclcated giiint cells and eosinophils.
atrophy, and bony abnormalities of the region of of bacterial conjunctivitis is between the second Large, thin-walled capillaries frequently nint
medical and surgical treatment by an
the perilongitudinal sinus. The palpebral fissures and fifth days of life; the diagnosis is confirmed through the lesions. They are easily ruptured,-
ophthalmologist.
slant downward (in contrast to the upward slant by bacterioiogic smear and culture. Inclusion
20B 209
 causing hyphema and possible secondary
glaucoma^ important in preventing amblyopia ex anopsia.
’s' «
Jt ’
Strabismus * * * SELECTED REFERENCE BOOKS
Strabismus is present in about 2% ofchildren.
Its early recognition is often the responsibility of TESTS FORVISUAL ACUITY
the pediatrician or (he family physician. Systemic
factors.; ciusing strabismus such as physical 1. Buiuc S, Jolobceastai L. - Oftalmologie practica, vol 1. Ed. Junimea, Iasi 1979.
exhaustion, neuromuscular disorders, and 2. Buiuc S, Jolobceastai L. - Oftalmologie practica, vol 2. Ed. Junimea, Iasi, 1981.
hypoglycemia should be considered. Treatment In the early years, visual acuity should be
3: RatiuE -Tulburarile motilitatii oculare. Ed. Medicala, Bucuresti 1970.
of strabismus is best started nt the age of 6 months appraised as part of each general “well child”
to ensure development of the best possible visual examination. It is best not to waituntil the child is
4. Zolog IIcana —Cornea. Ed. Mirton, Timisoara, 1997.
acuityidndta good cosmetic and functional result old enough to respond to visual charts, since these 5. Offfet II. -Oeil et virus. Ed.Masson, Paris, 2000.
6. Cemea P. - Anomalii oculare congenitale. Ed. Medicala, Bucuresti,1988.
(bino'cula| vision). The idea that a child may may not furnish accurate information until school
7. Cemea P. -Tratat de Oftalmologie. Ed. Medicala, Bucuresti, 1997.
outgrow grossed eyes should be discouraged. age. Estimations of vision should be made in the 8. Yanoff M., Duker S J. - Ophthalmology. Mosby International Ltd., London, 1999.
Neglect in2 the treatment of strabismus may lead first few days by ascertaining the pupillary
9. Chiselita D. - Oftalmologie generals. Ed. Dosoftei, Iasi, 1997.
to undesirable cosmetic effects, psychic trauma, responses to light, which rules out complete 10. Cemea P.,Constantin Florica - GlaucomuL Ed. Medicala, Bucuresti, 1979.
and permanent impairment ofvision (see below) dysfunction, of the eyes. In later weeks, light 11. FadoT F., Dinulescu Arety — Morfopalologia ochiului si anexelor sale. Ed.Medicala, Bucuresti,
in thei^exuating eye. Strabismus is covered in fixation reflexes can be elicited-single and
dcpthlin Chapter 12. bilateral reflexes first, and then binocular 1980. F., Pop D. Popa Doina - Oftalmologie. Ed. Didactica si Pedagogica, Bucuresti, 1991.
12. Fodor
following and converging reflexes. A good 13. Blaustein H.B. - Ocular Manifestations ofNeurologic Diseases. Mosby-Year Book Inc., USA, 1996.
Hi J response consists of prompt fixation and 14. Perkin G.D., Clifford Rose F. - Optic Neuritis and its Differential Diagnosis. Oxford University
Amblyopia ex Anopsia following reflexes, equal in each eye, with the
i* < ’ light reflex centered in the pupil when the source Press, Ileana
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and his apparent sharpness of vision are Mirton, Timisoara, 1995, pg. 71-93.
common causes are uncorrected
_____ — —strabismus
— and
UO CAIIVJ 17. Zolog Ileana, Szabo ludit - Afectiuni genetice in oftalmologie. Ed. Eurobit,
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27. Buiuc S., Romanescu Carmen - Notiuni de propedeutic^ chirurgicala oftalmologica. Ed. Eurocart,
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40. Lofoco G., Bardocci A. - La Periferia del Fondo Oculare. Ed. SIF1, Catania, Italia, 1995. Bone(s)
Abrasions oforbit, 105,106,107
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Malattia Ipertensiva. Piccin Nuova Libraria, Padova, 1999. of eyeball, 185
Accommodation, 192 Bowman’s membrane, 18
..... 42. Risse J.F. - Exploration de la fonction visuelle. Ed. Masson, Paris, 1999.
Adenoma Bruch’s membrane, 67
43. Bee P., Ravault M., - La pSripherie du fond d’oeil. Ed. Masson, Arne J.L., Trepsat C. Paris, 1980.
chromophile, 120 Buphthalmos, 169
. 44. Royer J., Adcnis J.P., Bernard J. A., — L’appareil lacrymal. Ed. Masson, Paris, 1982. Metaireau J.P.,
chromophobe, 120 Bums of eyeball, 187
RenyA.
eosinophilic, 120 t I
45. Berard P.V., Quer6 M.A., Roth A. - Chirurgie des strabismes. Ed. Masson, Paris, Spielmann A.,
neutrophilic, 120 Canaliculi, 28
Woillez M. 1984. Canal ofSchlemm, 17,18
After-cataract, 98 <-
46. Saragoussi J.J., Arne J.L., - Chirurgie refractive. Ed. Masson, Paris, 2001 .Colin J., Montard M. 5
Alkali bum, 44 Canthus, 15
47. Spoor Th., Nesi F., - Management of Ocular, Orbital and Adnexial Trauma. Raven Press, New York,
Allergic . Carcinoma
1988. of conjunctiva, 177 f
conjunctivitis, 42 i
48. Haut J., Limon Sylvie, —La laser en ophtalmologje. Ed Masson, Paris, Massin M., Perdriel G. 1981. of lid, 173 with xeroderma pigmentosum, 174
rhinitis, 42 associated
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50. Schepens C.L., Neetens —The Vitreous and Vitreoretinal Interface. Springer Verlag, New York £nc,,
Amblyopia
due to methanol poisoning, 140 basalcell, 174 j;
ri B
. i;
1987. due to methyl alcohol poisoning, 140 squamous cell, 174 5
51. Vladutiu Cristina — Strabismul. ghid practic. Ed. Napoca Star, Cluj Napoca, 1999. due to quinine and related compounds, 142
Caruncle, 35
- 52. Stanila Adriana — Cristalinul, Sibiu, 2001. due to salicylates, 142 lacrimal, 16
53. Bosco F., Griguolo F.M., Nuzzi R. - Argomenti di Radio-Oftalmologia Diagnostica. Ed. Minerva nutritional, 141 Cataract, 94-98
Medica, Torino, 1991. Anatomy o feye, 15-21 Cavernous
■
54. Newell F.W. - Ophthalmology. Moby Company, USA, 1986 Anoph thalmos, 205 hemangiomas, 173
• 55. Burato L. -Extracapsular Cataract Microsurgery and Posterior Chamber Intraocular Lenses. Center Anterior sinus, 19
Ambrosiano Microchirurgia oculare, Milano, 1989, chamber, 17,19 thrombosis, 109
56. Spalton D.J., Hitchings R_A., - Atlas of Clinical Ophthalmology. Wolfe Hunter P.A„ Publishing, angle, 18,21 Cellulitis, orbital, 108
estimation ofdepth of, 167
Singapore, 1993. Central I
normal, 69 retinal
segment artery, 110
structures, 163 vein, 110
vascular supply to, 21 occlusion, 79
’ Aqueous humor, 162 thrombosis of, 80
| Arterial circle ofiris, 20,21
Chalazion, 24
j Astigmatism, 196,197 Chemical conjunctivitis, 45
Atrophy Choriocapillaris, 67,77
optic Chorioretinitis, 68
genetically determined
Choroid(al), 17
I Behr’s,]! 7
; congenital, 117 anatomy of, 67
cross section of, 67
; infantile, 117
hemangioma of, 179
Leber’s, 117 malignant melanoma of, 179
nerve, 116
Ciliary
Berlin’s traumatic edema, 186 artery, 17,18,21
body, 17,21
Binocular epithelium, 18
movement, 149
ganglion, 122,123
Blindness
cortical, 115
213
212
 Exotropia, 155 Hyperphoria, defined, 147 Laceratio n(s)
' r muscle, 18,66
Hypertropia, 157 ofcanaliculi, 189
. nerve, 17,18 Eye
anatomy, 15-21 Hyphema, 72 comeal, 188
•/ . process, 18,66
defects, congenital, 205 Hypophoria, defined, 147 of eye, J 87
*;• /■ vein,2J
Hypopyon, 54 of lid, 189
; Cpiobpina(s) normal, in infants and children, 203
Hypotropia, defined, 147 Lacrimal
■9: ofiris, 206 optical system of, 192
apparatus, 28-30
■ijj palpebral, 206 penetrating injuries to, 187
trauma to, 185 Idiopathic strabismus, 152 caruncle, 16
: Conjunctiva (1), 17,35-50
Inclusion drainage system, 28
j/Cantusion(s)
blennorrhea, 40 gland, 28
’’i ;• of eyeball, 186 . Farsightedness, 193
bodies, 39,40 lake, 16
* Hocular, and secondary glaucoma, 170 Foramen
infraorbital, 105 conjunctivitis, 40 punctum, 16,28
i of orbital contents, 189
Infection, comeal or intraocular, prevention of, 200 sac, 28
■ H ‘Convergent strabismus, 151 optic, 105
:‘.;jC0mea, 17.19,21.51-63 Injuries Lamina
Foreign bodies
to lids, 189 cribrosa, 17,63
/Cryotherapy, 55,87 comeal, 186
nonpenetrating, ofeyeball, 185 fusca, 64
'‘Gyclijis intraocular, 188
of orbit and its contents, 189 Lens(es), 17,18,19
'// ./ chronic, 73 Forster-Fuchs black spot, 195
penetrating, to eye, 187 Leukocoria,209
Fuchs’s heterochromic, 72 Fovea centralis, 76
Intraocular Limbus, 15,21
j-, b ;
j;j; ijacryoadenitis, 30 Geniculate body, lateral, 121,122 disease, cataract in, 97
Geniculocalcarine tract, 121,122 foreign bodies, 188 Macula(r), 17, B9-93
i’j Dacryocystitis, 29
tumors, 178-181 edema, 81
[/ Dendritic keratitis, 54 Gland(s)
Iridocyclitis, 71 hereditary diseases of, 92
■»;; pescemet’s membrane, 18,51 ofKrause,22,33
IridodQnesis-9 8 hole, 92
|.!i Diabetic retinopathy, 80 meibomian, 22
Iris, 15,17,21 Malignant
Diplopia, 28 of Moll, 22
anatomy of, 66 melanoma, 175,177,179
■'/ ’Divergent strabismus, 155 ofWolfring, 33
arterial circle of, 20,21 myopia, 195
Drusen, 90 ofZeis, 22
pigmentation, 18,204 Megalocomea, 206
Glaucoma, 161-175
and pupillary defects, 206 Meibomian gland(s), 16,22
H: ;-Eetfopion, 25 Globe
stroma of, 18 Moll glands, 22
/ /Emmclropia, 191,193 penetration of, 102
vessels, 66 Muscles
•’’•; /Endophthalmitis, 103 rupture of. 102
Iritis, 68 extraocular, 19,146
?/ /pnophthalmos, 108 Gonioscopy, 163
ocular, functions of, 148
■ ?r /.Entropion, 25 synergistic and antagonistic, 148
Erratic precipitates, 68
J/ /Epiphora, 34 Halving lid, 189
Keratitis Myopia, 191
•\ .Episcleritis, 64 Hemangioma
dendritic, 54
•’.Epithelioma of choroid, 179
disciform, 54 Nearsightedness, 194
; of conjunctiva, 177 of lid, 173
exposure, 58 Neuritis
J*:i j ofcornea, 177 oforbit, 182
herpes simplex, 54 optic, 112
'• ; Epithelium Hemorriiage
neuroparalytic, 143 retrobulbar, 114
corneal, 51 subarachnoid, 136
i Keratoconjunctivitis Neuro-ophthalmology, 110-145
lens, 94 subconjunctival, 50
atopic, 44 Neuroparalytic keratitis, 143
nonpigment, 66 subdural, 136
epidemic, 41 Nystagmus, 130-133
,-J i j pigment, 17,66,67 subhyaloid, 137
Herpes phlyctenular, 43
,ii- / Erythema
primary herpetic, 55 Occlusion, retinal
multifonne,48 simplex
sicca, 32 artery, 78
bullosum.,48 conjunctivitis, 42
keratitis, 54 Keratoconus, 59 branch vein, 79
l;. '. nodosum, 48
Keratopathy vein, 79
: Esophoria, 160 zoster, 142
band, 59 Oculomotor
defined, 147 Hyaloid
Bietti’s, 60 nerve, nuclear and infranuclear connections of, 127
Esotropia, 147,151-155 . canal, 99
climatic droplet, 60 paralysis, 128
. / Exophoria, 160 membrane, 99
Labrador, 60 Open-angle glaucoma, 165
defined, 147 Hypermetropia, 193
Hyperopia, 191,193
genetic aspects of, 166
■ Exophthalmos, 106

215
214
 ... Ophthalmia diabetic, 80
neonatorum, 50 eclipse, 201
sympathetic, 73 Retrobulbar neuritis, 114
Optic
.... , . atrophy Sarcoma of lid, 175
genetically determined, 117 Schlemm, canal of, 17,18
, hereditary, 117 Sclera(l), 15,17,18,21,63-65
chiasm, 117,121 Sinus cavemosus, 19
disk, 17 Strabismus, 146-160,199
nerve, 17,18,110-114 Sympathetic
. . neuritis, 112,113 ophthalmia, 73
tract, 121,122 uveitis, 73
Optics, 191-198 Synechias, 69,70
' Ora serrata, 17,66, 76
posterior view of, 66 Tests for visual acuity, 210
Orbit(al), 105-109 Thrombosis
Orthophoria, defined, 147 arterial, 135
cavernous sinus, 109
Palpebral Trauma
,} colobomas, 206 to eye, 185-190
tendons, 22 glaucoma secondary to, 169
J Papilla(e), 39 Traumatic
Papillitis, 112 cataract, 97
Pars lens dislocation, 98
plana, 18,66,67 Tumors, 172-184
planitis, 72
Penetrating injuries Uveal
to eye, 187 structures, functions of, 67
oforbital tissue, 189 tract, 66-75
‘ Pigment epithelium, 17,66,67 glaucoma secondary to changes in, 170
-a Posterior chamber, 17 tumor, glaucoma secondary to, 170
‘ Presbyopia, 197 Uveitis, 68-75
Progressive myopia, 195
Ptosis of lids, 27,39 Venous drainage system, 19,20
Pupil(s), 15,17 Visual
Pupillary acuity, 192
defects, iris, 206 field(s) in glaucoma, 165
pathways, 121 system, topographic diagnosis, 111
lesions in, 121 Vitreous, 17,19,99-104
neuroanatomy of, 121 Vortex vein, 17,18

"• 1 - Rectus musclc(s), 17 Water drinking test for glaucoma, 165

Refraction, 191-198 White pupil, 209
Refractive
errors, 192 Zeis, glands of, 22
, • - index of aqueous and cornea, 192 Zinn
state, changes in, 204 annulus of, 146
■ Retina(l), 17,76-93 zonule of, 94
Retinitis pigmentosa, 83 ZonuHr fibers, 18
i. . Retinoblastoma, 180 Zonule, 17,66
Retinopathy

216