Diseases of Lens

• Shape, transparency and location. The lens is a

transparent, biconvex, crystalline structure placed between iris
and the vitreous in a saucer shaped depression, the patellar fossa.
• Diameter is 9–10 mm.
• Thickness varies with age from 3.5 mm (at birth) to 5 mm (at
extreme of age).
• Surfaces. Anterior surface is less convex (radius of curvature 10
mm) than the posterior (radius of curvature 6 mm). These two
surfaces meet at the equator.
• Total dioptric power is about 18 D (range 16–20D).
• Accommodative power of lens varies with age, being 14–16 D
(at birth); 7–8 D (at 25 years of age) and 1–2 D (at 50 years of
age).
Lens consists of:
1. Lens capsule. It is a thin, transparent, hyaline membrane
surrounding the lens.
2. Anterior epithelium. It is a single layer of cuboidal cells
which lies deep to the anterior capsule. In the equatorial region
these cells become columnar, are actively dividing and elongating
to form new lens fibres throughout the life. There is no posterior
epithelium, as these cells are used up in filling the central cavity of
lens vesicle during development of the lens.
3. Lens fibres. The epithelial cells elongate to form lens fibres
which have a complicated structural form. As the lens fibres are
formed throughout the life, these are arranged compactly as
nucleus and cortex of the lens.
Lens structures
Nucleus. It is the central part containing the oldest fibres. It
consists of different zones, which are laid down successively as the
development proceeds. Depending upon the period of
development, the different zones of the lens nucleus include:
• Embryonic nucleus. It is the innermost part of nucleus which
corresponds to the lens upto first 3 months of gestation.
• Fetal nucleus. It lies around the embryonic nucleus and
corresponds to the lens from 3 months of gestation till birth.
• Infantile nucleus corresponds to the lens from birth to
puberty, and
• Adult nucleus corresponds to the lens fibres formed after
puberty to rest of the life.
Cortex. It is the peripheral part which comprises the youngest
lens fibres.
Suspensory ligaments of lens (Zonules of Zinn), also
called as ciliary zonules, consist essentially of a series of
fibres passing from ciliary body to the lens. These hold the
lens in position and enable the ciliary muscle to act on it.
Cataract refers to an opacification of sufficient
severity to impair the vision.
A. Etiological classification
I. Congenital and developmental cataract
II. Acquired cataract
1. Senile cataract
2. Traumatic cataract
3. Complicated cataract
4. Metabolic cataract
5. Electric cataract
6. Radiational cataract
7. Toxic cataract e.g., corticosteroid-induced cataract
8. Cataract associated with skin, osseous and miscellaneous diseases
B. Morphological classification
1. Capsular cataract.
2. Cortical cataract. It involves the major part of the cortex.
3. Nuclear cataract. It involves the nucleus of the crystalline lens.
4. Polar cataract. It involves the capsule and superficial part of the
cortex in the polar region.
Congenital cataracts occur due to some disturbance in the
normal growth of the lens. When the disturbance occurs before
birth, the child is born with a congenital cataract. Therefore, in
congenital cataract the opacity is limited to either embryonic or
foetal nucleus.
Etiology
I. Idiopathic. About 33% cases are sporadic and of unknown
etiology.
II. Heredity. About one-third of all congenital cataracts are
hereditary.
III. Maternal factors
1. Infections. Maternal infections like rubella are associated with
cataract in 50% of cases. Other maternal infections associated with
congenital cataract include toxoplasmosis and cytomegalic
inclusion disease.
2. Drugs ingestion. Congenital cataracts have also been reported
in the children of mothers who have taken certain drugs during
pregnancy (e.g., thalidomide, corticosteroids).
3. Radiation. Maternal exposure to radiation during pregnancy
may cause congenital cataracts.
Rubella Cataract
• Rubella cataract-typically, the child is born with a ‘pearly white’
nuclear cataract. It is a progressive type of cataract.
• Cataractous nucleus may harbor the virus up to two years of age.
Therefore, removal of such a cataract is usually followed by a
severe inflammatory reaction (uveitis or even endophthalmitis)
probably due to liberation of retained viruses.
Rubella Syndrome. Congenital rubella cataract may occur alone
or as part of the classical rubella syndrome which consists of:
1. Ocular defects. Congenital cataract, salt and pepper
chorioretinopathy, microphthalmos, cloudy cornea and poorly
dilating pupil.
2. Ear defects. Deafness due to destruction of organ of Corti
3. Heart defects. Patent ductus arteriosus, pulmonary stenosis
and ventricular septal defects.
Management of congenital cataract
1. Ocular examination should be carried out with special reference to:
• Density and morphology of cataract.
• Assessment of visual function.
• Associated ocular defects which need to be noted include
microphthalmos, glaucoma, PHPV, foveal hypoplasia, optic nerve
hypoplasia, and rubella retinopathy etc.
2. Laboratory investigations should be carried out to detect following
systemic associations in nonhereditary cataracts:
■Intrauterine infections viz. toxoplasmosis, rubella, cytomegalovirus
and herpes virus by TORCH test.
■Galactosemia by urine test for reducing substances, red blood cell
transferase and galacto kinase levels.
■Hyperglycemia by blood sugar level.
■Hypocalcemia by serum calcium and phosphate levels and X-ray
skull.
Surgical procedures. Childhood cataracts (congenital,
developmental as well as acquired) can be dealt with extra
capsular cataract extraction technique. Intraocular lens (IOL)
implantation is the method of choice for correcting aphakia.
‘Age-related cataract’ also called as senile cataract is the
commonest type of acquired cataract affecting equally
persons of either sex usually above the age of 50 years. The
condition is usually bilateral, but almost always one eye is
affected earlier than the other.
Etiology
1. Age. As mentioned above it usually occurs after the age of 50
years. When it occur before 45 years of age, the term pre-senile
cataract is used. By the age of 70 years, over 90% of the individuals
develop senile cataract.
2. Ultraviolet irradiations. More exposure to UV irradiation
from sunlight have been implicated for early onset and maturation
of senile cataract in many epidemiological studies.
3. Dehydrational crisis. An association with prior episode of
severe dehydrational crisis (due to diarrhoea, cholera, etc.) and age
of onset and maturation of cataract is also suggested.
4. Smoking has also been reported to have some effect on the age
of onset of senile cataract.
5. Diabetes mellitus. Age-related cataract occurs earlier in
diabetics.
Mechanism of loss of transparency. In this the
usual degenerative changes are intensification of the
age-related nuclear sclerosis associated with
dehydration and compaction of the nucleus resulting
in a hard cataract. It is accompanied by a significant
increase in water insoluble proteins. However, the
total protein content and distribution of cations
remain normal. There may or may not be associated
deposition of pigment urochrome and/or melanin
derived from amino acids in the lens.
Maturation of the cortical type of senile cataract
1. Stage of lamellar separation. The earliest senile change is
demarcation of cortical fibres owing to their separation by fluid. These
changes are reversible.
2. Stage of incipient cataract. In this stage, early detectable opacities
with clear areas between them are seen. Two distinct types of senile cortical
cataracts can be recognised at this stage:
a. Cuneiform senile cortical cataract. It is characterised by wedge-
shaped opacities with clear areas in between. These extend from equator
towards centre and in early stages can only be demonstrated after
dilatation of the pupil. Since the cuneiform cataract starts at periphery and
extends centrally, the visual disturbances are noted at a comparatively late
stage.
b. Cupuliform senile cortical cataract. Here a saucer-shaped opacity
develops just below the capsule usually in the central part of posterior
cortex, which gradually extends outwards. Cupuliform cataract lies right in
the pathway of the axial rays and thus causes an early loss of visual acuity.
3. Immature senile cataract (ISC). In this stage, opacification
progresses further. The cuneiform or cupuliform patterns can be
recognised till the advanced stage of ISC when opacification becomes
more diffuse and irregular. The lens appears greyish white but clear
cortex is still present and so iris shadow is visible.
4. Mature senile cataract (MSC). In this stage, opacification
becomes complete, i.e., whole of the cortex is involved. Lens becomes
pearly white in colour. Such a cataract is also labelled as ‘ripe cataract’.
5. Hypermature senile cataract (HMSC). When the mature
cataract is left in situ, the stage of hypermaturity sets in. In some
patients, after maturity the whole cortex liquefies and the lens is
converted into a bag of milky fluid. The small brownish nucleus settles
at the bottom, altering its position with change in the position of the
head. Such a cataract is called Morgagnian cataract. Sometimes in this
stage, calcium deposits may also be seen on the lens capsule.
Maturation of nuclear senile cataract. Progressive
nuclear sclerotic process renders the lens inelastic and
hard, decreases its ability to accommodate and obstructs
the light rays. These changes begin centrally and spread
slowly peripherally almost up to the capsule when it
becomes mature; however, a very thin layer of clear cortex
may remain unaffected. The nucleus may become diffusely
cloudy (greyish) or tinted (yellow to black) due to
deposition of pigments. In practice, the commonly
observed pigmented nuclear cataracts are either amber,
brown (cataracta brunescens) or black (cataracta nigra)
and rarely reddish (cataracta rubra)in colour.
Symptoms.
1. Uniocular polyopia (i.e., doubling or trebling of objects). It is also one
of the early symptoms.
2. Coloured halos. These may be perceived by some patients owing to
breaking of white light into coloured spectrum due to presence of water
droplets in the lens.
3. Black spots in front of eyes.
4. Image blur, distortion of images and misty vision may occur in early
stages of cataract.
5. Deterioration of vision. It is painless and gradually progressive in
nature.
• Patients with central opacities (e.g., cupuliform cataract) have early loss
of vision. These patients see better when pupil is dilated due to dim light in
the evening (day blindness).
• In patients with peripheral opacities (e.g., cuneiform cataract) visual loss
is delayed and the vision improves in bright light when pupil is contracted.
• In patients with nuclear sclerosis, distant vision deteriorates due to
progressive index myopia.
Signs.
1. Visual acuity testing. Depending upon the location and
maturation of cataract, the visual acuity may range from 6/9 to just PL
+.
2. Oblique illumination examination. It reveals colour of the lens
in papillary area which varies in different types of cataracts.
3. Test for iris shadow. When an oblique beam of light is thrown on
the pupil, a crescentric shadow of pupillary margin of the iris will be
formed on the greyish opacity of the lens, as long as clear cortex is
present between the opacity and the pupillary margin.
4. Distant direct ophthalmoscopic examination. A reddish
yellow fundal glow is observed in the absence of any opacity in the
media.
5. Slit-lamp examination should be performed with a fully-dilated
pupil. The examination reveals complete morphology of opacity (site,
size, shape, colour pattern and hardness of the nucleus).
Complications
1. Phacoanaphylactic uveitis. Lens proteins may leak into the
anterior chamber in hypermature cataract. These proteins may act as
antigen and induce antigen-antibody reaction leading to
phacoanaphylactic uveitis.
2. Lens-induced glaucoma. It may occur by different mechanisms:
i. Phacomorphic glaucoma is caused by intumescent (swollen and
cataractous) lens. It is a type of secondary angle closure glaucoma.
ii. Phacolytic glaucoma. Lens proteins are leaked into the anterior
chamber in cases with Morgagnian type hypermature cataract. These
proteins are engulfed by the macrophages. The swollen macrophages
clog the trabecular meshwork leading to increase in IOP.
iii.Phacotopic glaucoma. Hypermature cataractous lens may
subluxate/dislocate and cause glaucoma by blocking the pupil or angle
of anterior chamber.
3. Subluxation or dislocation of lens. It may occur due to
degeneration of zonules in hypermature stage.
Diabetic cataract. Diabetes is associated with two types of cataracts:
1. Senile cataract in diabetics appears at an early age and progresses
rapidly.
2. True diabetic cataract. The classical diabetic cataract is also called
‘snowflake cataract’ or ‘snowstorm cataract’. It is a rare condition, usually
occurring in young adults due to osmotic over hydration of the lens. Such
opacities may resolve spontaneously or mature within a few days.
Cataract due to error of copper metabolism. Inborn error of
copper metabolism results in Wilson’s disease (hepatolenticular
degeneration).
■‘Sunflower cataract’ characterised by yellowish brown dots may be
observed rarely in such patients due to the deposition of cuprous oxide in
the anterior lens capsule and subcapsular cortex in a stellate pattern.
■‘Kayser-Fleischer ring’ (KF ring) i.e., a golden ring due to
deposition of copper in the peripheral part of Descemet’s membrane in
the cornea, is a more commonly observed ocular feature of Wilson’s
disease.
Sunflower cataract and Kayser-Fleischer ring
Complicated cataract refers to opacification of the lens
secondary to some other intraocular disease. Some authors use the
term secondary cataract for the complicated cataract. On the other
hand, many authors use the term secondary cataract to denote
after cataract.
Etiology. The lens depends for its nutrition on intraocular fluids.
Therefore, any condition in which the ocular circulation is disturbed
or in which inflammatory toxins are formed, will disturb nutrition of
the crystalline lens, resulting in development of complicated
cataract.
1. Inflammatory conditions. These include iridocyclitis,
choroiditis, hypopyon corneal ulcer and endophthalmitis. Anterior
uveitis is the most common cause of complicated cataract.
2. Degenerative conditions such as retinitis pigmentosa and
other pigmentary retinal dystrophies and myopic chorioretinal
degeneration.
3. Retinal detachment. Complicated cataract may occur in long-
standing cases.
4. Glaucoma may sometimes result in complicated cataract. The
underlying cause here is probably the embarrassment to the
intraocular circulation, consequent to the raised pressure.
Clinical features. Typically, the complicated cataract
starts as posterior subcapsular cortical cataract (PSC). The
opacity is irregular in outline and variable in density. In the
beam of slit-lamp the opacities have:
• ‘Breadcrumb’ appearance.
• ‘Polychromatic luster’ i.e., appearance of iridescent
coloured particles of reds, greens and blue is a very
characteristic sign (Rainbow cataract).
• Diffuse yellow-haze is seen in the adjoining cortex.
• Slowly the opacity spreads in the rest of the cortex, and
finally the entire lens becomes opaque, giving dirty white
or chalky white appearance.
• Deposition of calcium is common in the later stages.
Corticosteroid-induced cataract. Posterior subcapsular opacities
are associated with the use of topical as well as systemic steroids.
Prolonged use of steroids in high doses may result in cataract
formation. Further, intermittent regimes should be preferred over
regular therapy and whenever possible steroids should be substituted
by NSAIDs.
Management of cataract in adults. Treatment of
cataract essentially consists of its surgical removal.
However, certain nonsurgical measures may be of help, in
peculiar circumstances, till surgery is taken up.
1. Treatment of the causative disease, many a time,
may stop progression and sometimes in early stages may
cause even regression of cataractous changes and thus
defer the surgical treatment. Some common examples
include:
• Adequate control of diabetes mellitus, when discovered.
• Removal of cataractogenic drugs.
• Removal of irradiation (infrared or X-rays).
• Early and adequate treatment of ocular diseases like
uveitis may prevent occurrence of complicated cataract.
Preoperative evaluation and workup.
I. General medical examination of the patient to exclude the
presence of systemic diseases.
II. Ocular examination.
1. Visual status assessment should include:
■Visual acuity.
■Perception of light (PL) must be noted. Absence of PL
indicates nil visual prognosis.
■Projection of light rays (PR). It is tested in a semi-dark room
with the opposite eye covered. A thin beam of light is thrown in the
patient’s eye from four directions (up, down, medial and lateral)
and the patient is asked to look straight ahead and point out the
direction from which the light seems to come.
2. Pupils should be examined to check for: light reactions and
ability of the pupils to dilate adequately before surgery.
3. Anterior segment evaluation by slit-lamp biomicroscopy.
■Cornea should be examined to note any scarring, endothelial
status.
■Keratic precipitates (KPs) noted at the back of cornea suggest
management of subtle uveitis before the cataract surgery.
■Cataractous lens should be evaluated for morphology and
maturity of cataract and for grade of nuclear sclerosis.
■Other signs to be particularly looked for include posterior
synechiae, pseudoexfoliation, pigments over the anterior lens
capsule, and anterior chamber depth.
4. Examination of lids, conjunctiva and lacrimal apparatus.
Search for local source of infection should be made by ruling out
conjunctival infections, meibomitis, blepharitis and lacrimal sac
infection.
5. Intraocular pressure (IOP) should be measured in each case,
preferably by applanation tonometry.
6. Fundus examination, wherever possible, should be carried out
with special attention on macula, to rule out other causes of
decreased vision.
7. Macular function test: colour perception indicates that some
macular function is present and optic nerve is relatively normal.
8. Objective test for evaluating retina are required if some retinal
pathology is suspected. These tests includes ultrasonic evaluation
of posterior segment of the eye; electrophysiological studies such
as ERG (electroretinogram), and VER (visually evoked response);
9. Keratometry and biometry to calculate power of intraocular
lens (IOL).
Extracapsular cataract extraction by phacoemulsification along
with foldable posterior chamber intraocular lens implantation in
the bag is the procedure of choice for cataract surgery.
Displacements of the lens from its normal position (in patellar
fossa) results from partial or complete rupture of the lens zonules.
Clinico-etiological types
I. Congenital displacements. These may occur in the following
conditions:
1. Marfan’s syndrome. It is an autosomal dominant mesodermal
dysplasia. In this condition, lens is displaced upwards and temporally
(bilaterally symmetrical). Systemic anomalies include arachnodactyly
(spider fingers), long extremities, hyperextensibility of joints, high
arched palate and dissecting aortic aneurysm.
2. Weill-Marchesani syndrome. It is condition of autosomal recessive
mesodermal dysplasia. Ocular features are spherophakia, and forward
subluxation of lens which may cause pupil block glaucoma. Systemic
features are short stature, stubby fingers and mental retardation.
II. Traumatic displacement of the lens is usually associated with
concussion injuries.
III. Consecutive or spontaneous displacement results from
intraocular diseases.
Subluxation is partial displacement in which lens is
moved sideways, but remains behind the pupil. It results
from partial rupture or unequal stretching of the zonules.
Clinical features are as follows:
• Defective vision
• Uniocular diplopia may result from partial aphakia.
• Anterior chamber becomes deep and irregular.
• Iridodonesis is usually present.
• Edge of the subluxated lens is seen as dark crescent line on
distant direct ophthalmoscopy.
• Phacodonesis, i.e., trimulousness of lens may be seen.
• Retinoscopy reveals hypermetropia in aphakic area and myopia
(index) in phakic area.
Management. Spectacles or contact lens correction for phakic or
aphakic area (whichever is better) is helpful in many cases.
Surgery is controversial and usually associated with high risk of
retinal detachment. Lensectomy with anterior vitrectomy may be
performed in desperate cases.
Coloboma of lens. It is seen as a notch in the lower quadrant
of the equator. It is usually unilateral and often hereditary.