South African Family Practice

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The gradual loss of vision

N Du Toit (Consultant Ophthalmic Surgeon)

To cite this article: N Du Toit (Consultant Ophthalmic Surgeon) (2013) The gradual loss of vision,
South African Family Practice, 55:6, 493-500, DOI: 10.1080/20786204.2013.10874404

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 CPD Article: The gradual loss of vision

The gradual loss of vision

Du Toit N, MBChB, DipOphth(SA), FRCS(Ed), FCOphth(SA), MMed

Consultant Ophthalmic Surgeon, Division of Ophthalmology, Groote Schuur Hospital; Senior Lecturer, University of Cape Town
Correspondence to: Nagib du Toit, e-mail: [email protected]
Keywords: gradual, loss of vision, cataracts, refractive error, optic atrophy, glaucoma, age-related macular degeneration

Abstract
Gradual loss of vision is a clinical problem that is encountered fairly regularly in most primary healthcare settings. Patients
present with chronic, slowly progressive and generally painless visual loss. The reduction in vision is usually bilateral, though
frequently asymmetrical, and occurs over weeks to years. A goal-directed assessment of the patient presenting with gradual
loss of vision is required. A history of the type of visual loss, e.g. central or peripheral, and whether it is worse for near vision
or distance vision, is helpful. The examination should focus on visual acuity, confrontation visual field testing, pupil testing
for the presence of an afferent pupil defect and assessment of the red reflex and fundoscopy. The more common conditions
that cause gradual loss of vision can be divided into two groups based on the reversibility of the visual loss. Cataracts,
refractive error, corneal blindness and early diabetic macular oedema are generally reversible. Optic atrophy, glaucoma,
retinal degeneration and age-related macular degeneration usually cause permanent loss of vision. Most of these conditions
are briefly discussed in this article. This has been performed at a level that is suitable to primary care.

Peer reviewed. (Submitted: 2013-12-19 Accepted: 2013-04-10). © SAAFP S Afr Fam Pract 2013;55(6):493-500

Introduction more, although this symptom depends on the morphology

of the lens opacity. The examination should focus on visual
Patients with gradual loss of vision present with chronic,
acuity, visual field testing, pupil testing for the presence of
slowly progressive loss of vision which is generally painless.
an afferent pupil defect, and assessment of the red reflex
Visual loss is usually bilateral, but may occur asymmetrically,
and happens over weeks to years. Poor vision in one eye and fundoscopy. Many of the conditions discussed will
may only be noticed when the patient closes the other eye, initially cause reversible visual loss, but a few may become
and thus may be reported to be sudden in onset in some permanent if they are not appropriately managed.3
cases. At this stage, it is important to note that blindness is The more common conditions that cause gradual loss
defined as visual acuity worse that 3/60 in both eyes, or a
of vision can be divided into two groups, based on the
visual field restricted to less than 10 degrees around central
reversibility of the visual loss. The conditions in these two
fixation bilaterally. Visual impairment is divided into different
categories are listed in Table I. These will be discussed later.
categories, but generally refers to visual acuity worse than
6/18 in the better eye.1 The need for an update in this field
became apparent during a recent study on primary health Table I: The common causes of gradual loss of vision

eye care knowledge by general practitioners.2 This article Reversible causes

is the third in a series which attempts to address this issue. Cataract
Refractive error
Approach to the patient
Corneal blindness
A goal-directed assessment of the patient who presents
Diabetic macular oedema
with gradual loss of vision is required. A history of the
type of visual loss, e.g. central or peripheral, and whether Irreversible causes

it is worse nearby or at distance is helpful. Central loss is Optic atrophy

found with lesions of the macula, while peripheral loss is Glaucoma
found in glaucoma. Patients with myopia and certain retinal
Age-related macular degeneration
degeneration may experience poor vision with dim light, and
Retinitis pigmentosa
those with a cataract may find bright light to affect the vision

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 CPD Article: The gradual loss of vision

APD: afferent papillary defect, VA: visual acuity

Figure 1: Flow chart for the primary care assessment of patients with gradual loss of vision

The more common conditions will be discussed in more metabolic or idiopathic. Nuclear sclerotic, cortical and
detail than the less common ones. posterior subcapsular opacities are the most common
morphological types in age-related cases.
It is important to note that most of these conditions
cause bilateral visual loss, but they may be asymmetrical A cataract is a very common cause of blindness,4-6 but
and initially affect one eye more than the other. Using the patients may initially experience glare (with the cortical
reversibility of the visual loss and the presence of an afferent
pupil defect as the primary differentiating factors, with
associated symptoms and signs as secondary, the causes
can be narrowed down as indicated (Figure 1).

Clinical conditions

Cataract
A cataract is probably the most common cause of gradual
visual loss.1 It refers to “clouding” or loss of clarity of
the crystalline lens, with a resultant decrease in acuity. A
cataract may occur congenitally, but senile cataracts are
much more common, and almost everyone over 70 years
of age has some degree of lens opacity.3 The other causes
Source: The Division of Ophthalmology, University of Cape Town
of cataracts include diabetes, trauma, steroid therapy Figure 2: Mature cataract (with absent red reflex) visible through the
and chronic uveitis. Congenital cataracts may be familial, dilated pupil

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 CPD Article: The gradual loss of vision

type), monocular diplopia or a change in refraction, usually • Small-incision ECCE: A smaller (usually scleral) (Figure 4)
a “myopic shift” because of nuclear sclerosis. Cataracts incision is used.
are recognised by a decrease in the red reflex (Figure 2), • Phacoemulsification: An ultrasound probe (Figure 5) is
with resultant poor visualisation of the retina on attempted used to emulsify the cataract and aspirate it through a
fundoscopy. It is important to exclude other co-existing small scleral or corneal wound which needs no sutures.
ocular pathologies which may limit the chances of visual This surgical technique requires a shorter recovery time.3
recovery after surgery. A cataract is also the most common More recently, femtosecond laser has been introduced to
cause of leukocoria (white pupil).3 Cataract blindness is perform some of the steps of the procedure.
reversible by microsurgery which aims to improve visual
Patients should be referred for surgery when the visual
function and is safe, quick and cost-effective.
disturbance interferes appreciably with the patient’s
Cataract extraction can be performed in two basic ways, lifestyle.1
viz. intracapsular cataract extraction (ICCE), whereby the
lens is removed together with its capsule, and extracapsular Refractive error
cataract extraction (ECCE), whereby the lens is removed This is also a common cause of blindness,4,6 and is
leaving the capsule behind, which is the preferred technique. correctable with spectacles. Images, caused by refraction
This can be further subdivided into: at the cornea and lens, are formed either in front of, or
• Traditional ECCE: A large incision (usually limbal) (Figure behind, the retina. Light (parallel rays) from a distant object
3) is made. A needle is used to make a hole in the is focused on the retina in emmetropia (normal sight).
lens capsule, and the cataract is expressed by putting Accommodation occurs when diverging rays from a near
pressure on the globe. object are focused on the retina by thickening of the lens,
which occurs when the ciliary muscle contracts and the lens
zonules relax, causing the lens to become more spherical
in shape.

With hypermetropia (long-sightedness), parallel rays from

distant objects are focused behind the retina. This is
corrected with convex lenses. Myopia (short-sightedness)
causes parallel rays to be focused in front of the retina, and
is corrected with concave lenses. Degenerative myopia
occurs when patients with high degrees of myopia have
“stretchy eyeballs”, which may become progressively longer
with time. They develop “cracks” in the choroid, and lose
vision because of haemorrhage from neovascularisation
around these cracks.3 Retinal degeneration may also be a
Source: The Division of Ophthalmology, University of Cape Town cause of loss of vision in high myopes, in addition to the
Figure 3: Large limbal wound of traditional extracapsular cataract earlier onset of cataracts and glaucoma.7
extraction

Source: The Division of Ophthalmology, University of Cape Town

Figure 4: Smaller scleral wound of small-incision extracapsular Source: The Division of Ophthalmology, University of Cape Town
cataract extraction Figure 5: Ultrasound probe used for phacoemulsification

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 CPD Article: The gradual loss of vision

Source: The Division of Ophthalmology, University of Cape Town Source: The Division of Ophthalmology, University of Cape Town
Figure 6: Conically shaped cornea apparent in a downward gaze Figure 7: Uneven corneal surface in climatic droplet keratopathy

Presbyopia occurs with ageing in the mid forties, because

of loss of the ability of the lens to accommodate. Patients
need convex lens correction for reading or near work.
Astigmatism usually results from different curvatures in the
two refracting planes of the cornea, causing an image from
each plane to be formed at two different points in front of
the retina, behind the retina, or both in front of and behind
the retina. Keratoconus, which leads to progressive myopic
astigmatism, is defined as a conically-shaped cornea (Figure
6). It has numerous associations. Atopy, eye rubbing and
Down’s syndrome are common associations.3 Keratoconus
is treated initially with hard contact lenses, and later corneal
cross-linking or corneal transplantation.
Source: The Division of Ophthalmology, University of Cape Town
Any patient complaining of poor vision should have visual Figure 8: Iris details obscured by corneal scarring
acuity measured with a 1-mm pinhole. Only parallel rays of
light pass through the pinhole centrally, so that the eye does
not have to refract them to obtain a clear retinal image. If
the patient has a refractive error, vision will improve with the
pinhole and the patient should be referred to an optometrist
for spectacles. If the patient already has glasses, the pinhole
may be used over them, and if there is further improvement,
the patient will need to change his or her spectacles.1
Contact lenses are useful for greater refractive errors, but
meticulous lens care is needed to avoid complications.
Excimer laser correction of refractive errors should be
reserved for those who are intolerant of glasses and contact
lenses.3

Corneal blindness Source: The Division of Ophthalmology, University of Cape Town

Figure 9: Laser scars visible in the macular area in a patient with
Conditions which lead to opacification of the cornea will diabetic maculopathy
cause a reduction in visual acuity. These include trachoma
in equatorial countries, which leads to entropion, trichiasis
and scarring of the cornea; interstitial keratitis following opacification is usually easily diagnosed by the presence of
infections, such as congenital syphilis, tuberculosis and a reduction in the red reflex, with underlying iris details not
measles; and climatic droplet keratopathy (Figure 7), which being clear in the area of opacification (Figure 8). Generally,
is caused by prolonged exposure to the elements, and is corneal grafting is needed to remove the opacified, scarred
common in the elderly from the Eastern Cape.3 Corneal corneal tissue and to restore vision.

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 CPD Article: The gradual loss of vision

Source: The Division of Ophthalmology, University of Cape Town Source: The Division of Ophthalmology, University of Cape Town
Figure 10: Cupping of the optic disc in glaucoma Figure 11: Blurred disc margins with pallor, indicating atrophy
Diabetic maculopathy secondary to swelling

Macular oedema may occur in diabetes, leading to insidious

loss of vision, which can be prevented with appropriate
laser therapy (Figure 9). This topic will be covered in another
paper on systemic disease and the eye.

Optic atrophy
Pallor of the optic disc results from damage to the nerve
fibres at any point from the retina to the lateral geniculate
body. Patients have poor vision with an afferent pupil defect,
decreased colour vision and usually a central visual field CDR: cup-to-disc ratio

defect. Causes of optic atrophy include compression of the Figure 12: Diagrammatic presentation of cup-to-disc ratio as
assessed on fundoscopy
optic nerve, e.g. tumour; damage to the optic nerve head
in chronic glaucoma; central retinal artery occlusion; and
metabolic, infective and genetic diseases of the retina, e.g.
retinitis pigmentosa, syphilis, tobacco-alcohol amblyopia,
ethambutol toxicity and chronic papilloedema. Headaches,
focal neurological signs or endocrinological signs should
alert the clinician to possible intracranial mass lesions.1
Clinically, disc pallor on fundoscopy may be divided into
three groups:
• Glaucomatous cupping (Figure 10).
• Diffuse pallor of the disc in non-glaucomatous optic
atrophy.
• A raised, pale disc (Figure 11) in optic atrophy, following
disc swelling.3
Source: The Division of Ophthalmology, University of Cape Town
Figure 13: Disc pallor due to end-stage cupping
Glaucoma
Glaucoma is defined as an “optic neuropathy” with cupping the gonioscopic findings during assessment of the anterior
of the optic disc, a characteristic pattern of visual field chamber angle. The most common type is chronic primary
loss, and in most cases, raised intraocular pressure (IOP).3 open-angle glaucoma. It is an asymptomatic disease with
Measured by tonometry, normal IOP is 10-21 mmHg. Visual insidious onset, and is a result of trabecular resistance
acuity and IOP may be normal in a patient with glaucoma.8 to aqueous outflow.8 Patients with raised IOP, but no
Glaucoma is classified as acute or chronic, depending on optic neuropathy or field loss, are referred to as ocular
the rate of onset, and primary or secondary, depending on hypertensives. Underlying ocular causes of secondary
whether or not there is an underlying ocular cause. This can glaucomas may be cataracts, trauma, inflammation
further be divided into open or closed angle, depending on or neoplasias. Risk factors for primary open-angle

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 CPD Article: The gradual loss of vision

Source: The Division of Ophthalmology, University of Cape Town Source: The Division of Ophthalmology, University of Cape Town
Figure 14: Print out from automated visual field analyser, showing Figure 16: Fundus photograph illustrating the triad of signs in retinitis
advanced loss pigmentosa

Tunnel vision is found in advanced cases and eventually

ends in blindness.
Management includes referral to an ophthalmologist for
treatment and regular follow-up. Patients are monitored
for progression of cupping and field loss, which can be
prevented by lowering the IOP.8 This can be achieved
medically with topical agents (drops), e.g. β blockers
and prostaglandin analogues, or surgically with drainage
procedures, e.g. trabeculectomy (Figure 15). Patients with
a family history of glaucoma in a first-degree relative should
be referred for screening.1

Retinitis pigmentosa
Source: The Division of Ophthalmology, University of Cape Town
Figure 15: Conjunctival bleb after trabeculectomy This is the most common inherited retinal condition, and
occurs because of dystrophy of the photoreceptors.
glaucoma include genetic [trabecular meshwork-inducible Patients complain of night blindness, tunnel vision and
glucocorticoid response (TIGR) myocillin gene], being of eventual blindness. Features include a “waxy” pallor of the
Black African ancestry, having myopia, being over 45 years disc, bone-spicule pigmentary retinopathy and attenuated
of age, and having diabetes and uncontrolled hypertension
retinal blood vessels (Figure 16). Retinitis pigmentosa has
(possibly).3
many systemic associations, including deafness, heart
Symptoms vary, but generally do not include pain. Visual block and cerebellar disease. Many different genetic
acuity may be normal early on (asymptomatic) as central mutations are responsible. Most types are not treatable, but
vision is preserved until late in the disease, or the patient those associated with metabolic disorders may be arrested
may be blind on presentation. Glaucoma is diagnosed by by treatment of the metabolic abnormality, and patients
looking at the disc, as well as assessing the fields and IOP. need to be referred for diagnosis, genetic counselling and
The optic cup is a pale depression in the centre of the disc. social services.1
The disc is made up of neural tissue. A cup-to-disc ratio of
> 0.6 is suggestive of glaucomatous cupping, in which the Papilloedema
loss of nerve fibres leads to pathological enlargement of the
This refers to disc swelling that is usually bilateral and due
cup (Figure 12).
to raised intracranial pressure. Acute papilloedema does
The following are also signs of glaucomatous cupping: not cause loss of vision, but may cause visual obscurations
baring, bayoneting and nasalisation of the disc vessels; disc that last a few seconds and which occur several times a
haemorrhages; exposed lamina cribrosa (laminar dot sign); day. Chronic papilloedema can cause slow loss of vision
notching of the cup and disc pallor (Figure 13).3 because of optic atrophy. If the disc is swollen and the
Loss of visual field in glaucoma follows the pattern of the patient complains of sudden loss of vision, papillitis (optic
retinal nerve fibre layer, and these defects are detected neuritis) should be suspected, and the patient referred
by automated perimetry using a field analyser (Figure 14). to an ophthalmologist urgently. It is important to look for

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 CPD Article: The gradual loss of vision

Source: The Division of Ophthalmology, University of Cape Town Source: The Division of Ophthalmology, University of Cape Town
Figure 17: Drusen around the fovea (centre of the macula) Figure 18: Exudates and haemorrhage in “wet” age-related macular
degeneration
papilloedema in patients with symptoms of nausea, early
morning headaches and personality changes, as well as
those with sixth cranial nerve palsies.9 The patient should
be referred to the neurologist for imaging. Investigation is
indicated in these cases.

Age-related macular degeneration

Age-related macular degeneration is a disease of the elderly
and is common in Caucasians. Symptoms and signs which
patients may experience include slow loss of vision (“dry”
type), sudden loss of vision from bleeding (“wet” type) and
metamorphopsia (distorted images).3 Age-related macular
degeneration is characterised by drusen, which are pale
deposits in the macular area (Figure 17), and which are very
common in the elderly. Initially, drusen do not interfere with Source: The Division of Ophthalmology, University of Cape Town

vision. The atrophy of the retinal pigment epithelium, with Figure 19: Calcific corneal deposits in band keratopathy
resultant central pigmentary changes, occurs in dry age-
related macular degeneration. Haemorrhage and exudation results in calcification of the cornea, e.g. with chronic uveitis.
from subretinal neovascular membranes occurs in wet
Juvenile idiopathic arthritis is associated with uveitis and
age-related macular degeneration (Figure 18). Age-related
causes painless, slow loss of vision, without causing a red
macular degeneration is a very common cause of bilateral
visual loss in the developed world,10 especially in patients eye. These children should have regular eye examinations.
over the age of 65 years. Central vision is affected, but Children with pauci-articular rheumatoid factor-negative,
peripheral vision spared, thus allowing continued patient anti-nuclear antibody-positive arthritis, are at highest risk.3
mobility.
Patients with age-related macular degeneration who
Conclusion
develop loss of central vision due to leakage and bleeding Gradual loss of vision is a common problem at primary care
from subretinal neovascular membranes, may benefit from level, especially in older patients. Cataracts are the most
laser therapy or intravitreal injection of antivascular growth
cause of loss of vision. Diagnosis is easily made in most
factor agents.3 Visual loss is usually bilateral, but may be
cases. Appropriate referral and management generally
asymmetrical. Patients with established visual loss require
“low vision aids”, e.g. magnifiers, and should be referred result in good outcomes, except in those with irreversible
to low vision services, as well as considered for blind visual loss from optic atrophy with its various causes,
registration and other support.10 and possibly also in those with macular degeneration.
Newer treatments of age-related macular degeneration
Chronic ocular inflammation have improved prognosis in many cases, and this field is
Band keratopathy (opaque calcific corneal deposits) (Figure expanding. There is hope for improved treatment of this
19) may be a sign of chronic intraocular inflammation which condition in the future.11

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 CPD Article: The gradual loss of vision

Acknowledgements 3. Du Toit N, Cook C. Gradual loss of vision. Cape Town: Juta; 2009.
4. Salomao SR, Cinoto RW, Berezovsky A, et al. Prevalence and causes of vision
The Division of Ophthalmology of the University of Cape impairment and blindness in older adults in Brazil: the Sao Paulo Eye Study.
Town is acknowledged for providing permission to use its Ophthalmic Epidemiol. 2008;15(3):167-175.

clinical photographs. 5. Murthy GV, Vashist P, John N, et al. Prevalence and causes of visual impairment
and blindness in older adults in an area of India with a high cataract surgical rate.
Ophthalmic Epidemiol. 2010;17(4):185-195.
Conflict of interest 6. Pascolini D, Mariotti SP. Global estimates of visual impairment: 2010. Br J
There is no conflict of interest to declare. Ophthalmol. 2012;96(5):614-618.
7. Morgan IG, Ohno-Matsui K, Saw SM. Myopia. Lancet. 2012;379(9827):1739-1748.

References 8. Quigley HA. Glaucoma. Lancet. 2011;377(9774):1367-1377.

9. Selhorst JB, Chen Y. The optic nerve. Semin Neurol. 2009;29(1):29-35.
1. Elkington AR, Khaw PT. Gradual visual loss, partial sight and blindness. BMJ. 10. Siemsen DW, Brown WL. Vision rehabilitation of persons with age related
1988;297(6647):548-549.
macular degeneration. Semin Ophthalmol. 2011;26(3):65-68.
2. Van Zyl LM, Fernandes N, Rogers G, Du Toit N. Primary health eye knowledge
among general practitioner’s in the Cape Town metropole. S Afr Fam Pract. 11. Kaufman SR. Developments in age-related macular degeneration: diagnosis and
2011;53(1):52-55. treatment. Geriatrics. 2009;64(3):16-19.

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