KANSKI Chapter 9 (Lens)

Subcapsular cataract
Anterior subcapsular cataract lies directly under the lens capsule and is associated with fibrous
metaplasia of the lens epithelium. Posterior subcapsular opacity lies just in front of the posterior capsule
and has a granular or plaque-like appearance on oblique slit lamp biomicroscopy (Fig. 9.1A), but
typically appears black and vacuolated (Fig. 9.1B) on retroillumination; the vacuoles are swollen
migratory lens epithelial cells (bladder or Wedl), similar to those commonly seen postoperatively in
posterior capsular opacification (see Fig. 9.21A). Due to its location at the nodal point of the eye, a
posterior subcapsular opacity often has a particularly profound effect on vision. Patients are
characteristically troubled by glare, for instance from the headlights of oncoming cars, and symptoms
are increased by miosis, such as occurs during near visual activity and in bright sunlight.
Nuclear sclerotic cataract
Nuclear cataract is an exaggeration of normal ageing change. It is often associated with myopia due to
an increase in the refractive index of the nucleus, resulting in some elderly patients being able to read
without spectacles again (second sight of the aged); in contrast, in the healthy ageing eye (and in
occasional cases of cortical and subcapsular cataract) there is mild hypermetropic shift. Nuclear
sclerotic cataract is characterized by a yellowish hue due to the deposition of urochrome pigment, and is
best assessed with an oblique slit lamp beam (Fig. 9.1C). When advanced, the nucleus appears brown
(Fig. 9.1D) or even black, the latter being typical of marked post-vitrectomy opacity.
Cortical cataract
Cortical cataract may involve the anterior, posterior or equatorial cortex. The opacities start as clefts and
vacuoles between lens fibres due to cortical hydration. Subsequent opacification results in typical
cuneiform (wedge-shaped) or radial spoke-like opacities (Figs 9.2A and B), often initially in the
inferonasal quadrant. As with posterior subcapsular opacity, glare is a common symptom.

Cataract maturity
Immaturecataractisoneinwhichthelensispartially opaque.
Maturecataractisoneinwhichthelensiscompletely opaque (Fig. 9.3A).
Hypermaturecataracthasashrunkenandwrinkled anterior capsule (Fig. 9.3B) due to leakage of water
out of the lens.
Morgagniancataractisahypermaturecataractinwhich liquefaction of the cortex has allowed the nucleus
to sink inferiorly (Fig. 9.3C).

Cataract in systemic disease

Diabetes mellitus
Hyperglycaemia is reflected in a high level of glucose in the aqueous humour, which diffuses into the
lens. Here glucose is metabolized into sorbitol, which accumulates within the lens, resulting in
secondary osmotic overhydration. In mild degree, this may affect the refractive index of the lens with
consequent fluctua- tion of refraction in line with the plasma glucose level, hypergly- caemia resulting in
myopia and vice versa. Cortical fluid vacuoles develop and later evolve into frank opacities. Classic
diabetic cata- ract, which is actually rare, consists of snowflake cortical opacities (Fig. 9.4A) occurring in
the young diabetic; it may mature within a few days or resolve spontaneously. Age-related cataract
occurs earlier in diabetes mellitus. Nuclear opacities are common and tend to progress rapidly.
Myotonic dystrophy
About 90% of patients with myotonic dystrophy (see Ch. 19) develop fine iridescent cortical opacities in
the third decade, some- times resembling Christmas tree cataract (polychromatic needle-like formations
in the deep cortex and nucleus); these evolve into visually disabling wedge-shaped cortical and
subcapsular opacities, often star-like in conformation (Fig. 9.4B) by the fifth decade. Later, the opacities
may become indistinguishable from typical cortical cataract.
Atopic dermatitis
About 10% of patients with severe atopic dermatitis develop cataracts in the second to fourth decades;
these are often bilateral and may mature quickly. Shield-like dense anterior subcapsular plaque that
wrinkles the anterior capsule (Fig. 9.4C) is characteristic. Posterior subcapsular opacities may also
occur.

Secondary cataract
A secondary (complicated) cataract develops as a result of other primary ocular disease.
Chronic anterior uveitis
Chronic anterior uveitis is the most common cause of secondary cataract, the incidence being related to
the duration and intensity of inflammation. Topical and systemic steroids used in treatment are also
causative. The earliest finding is often a polychromatic lustre at the posterior pole of the lens (Fig. 9.5A).
If inflammation persists, posterior and anterior opacities (Fig. 9.5B) develop. Cata- ract appears to
progress more rapidly in the presence of posterior synechiae (Fig. 9.5C).
Acute congestive angle closure
Acute congestive angle closure may cause small anterior grey- white subcapsular or capsular opacities,
glaukomflecken (Fig. 9.5D), to form within the pupillary area. These represent focal infarcts of the lens
epithelium and are almost pathognomonic of prior acute angle-closure glaucoma.
High myopia
High (pathological) myopia can be associated with posterior sub- capsular lens opacities and early-
onset nuclear sclerosis, which ironically may increase the myopic refractive error.
Hereditary fundus dystrophies
Hereditary fundus dystrophies (see Ch. 15) such as retinitis pig- mentosa, Leber congenital amaurosis,
gyrate atrophy and Stickler syndrome, may be associated with posterior and, less commonly, anterior
subcapsular lens opacities (Fig. 9.5E). Cataract surgery may improve visual function even in the
presence of severe retinal changes.
Traumatic cataract
Trauma is the most common cause of unilateral cataract in young individuals. Penetrating trauma (Fig.
9.6A). Blunt trauma may cause a characteristic flower-shaped
opacity (Fig. 9.6B). Electric shock is a rare cause of cataract, patterns including
diffuse milky-white opacification and multiple snowflake- like opacities, sometimes in a stellate
subcapsular distribution (Fig. 9.6C).
Infrared radiation, if intense as in glassblowers, may rarely cause true exfoliation of the anterior lens
capsule (Fig. 9.6D).
Ionizing radiation exposure such as for ocular tumour treatment may cause posterior subcapsular
opacities (Fig. 9.6E); these may not manifest for months or years.

MANAGEMENT OF AGE-RELATED CATARACT

Preoperative considerations
Indications for surgery
Visualimprovementisbyfarthemostcommonindication for cataract surgery. Operation is indicated when
the opacity develops to a degree sufficient to cause difficulty in performing essential daily activities.
Clear lens exchange (replacement of the healthy lens with an artificial implant) is an option for the
management of refractive error.
Medicalindicationsarethoseinwhichacataractisadversely affecting the health of the eye, for example
phacolytic or phacomorphic glaucoma; clear lens exchange usually definitively addresses primary angle
closure, but less invasive options are generally preferred (see Ch. 10). Cataract surgery to improve the
clarity of the ocular media may also be required in the context of monitoring or treatment of fundus
pathology.

informed consent
Points for discussion with the patient may include:
Most cataract operations are straightforward, with the patient achieving good vision.
Most complications can be dealt with effectively and cause no long-term difficulties, but some rare
problems can be very serious.
In about 1 in 1000 cataract operations the eye will be left with little or no sight; in about 1 in 10 000 the
patient will lose the eye.
Some complications mean that a second operation will be necessary.
Relatively mild and usually easily treatable but common complications include: periocular ecchymosis,
allergy to eye drops, intraocular pressure (IOP) spike, iridocyclitis, posterior capsular opacification
(currently in decline) and wound leak.
Moderate to severe but less common complications: posterior capsular rupture/vitreous loss (1% or
less for experienced surgeons, higher for trainees dependent on experience), zonular dehiscence,
cystoid macular oedema (CMO), dropped nucleus (about 0.2%), corneal decompensation sufficient to
need corneal graft, intolerable refractive outcome (may need contact lens wear, lens implant exchange
or corneal surgery), retinal detachment (<1%), IOL dislocation, persistent ptosis and diplopia.
 Rare but invariably very serious complications: endophthalmitis (0.1%) and suprachoroidal
haemorrhage (0.04%).
The risks of anaesthesia should be conveyed by the person administering it. Local anaesthesia carries
only a low risk of problems, though some rare complications have the potential to be very serious
including loss of the eye and even death: allergy to the anaesthetic agent, retrobulbar haemorrhage
(see Ch. 21), perforation of the globe, and inadvertent infusion of anaesthetic agent into the
cerebrospinal fluid via the optic nerve sheath causing brainstem anaesthesia.
There is virtually no risk to the other eye; sympathetic ophthalmitis is vanishingly rare following
modern cataract surgery.

Biometry
Biometry facilitates calculation of the lens power likely to result in the desired postoperative refractive
outcome; in its basic form this involves the measurement of two ocular parameters, keratometry and
axial (anteroposterior) length.
IOL power calculation formulae. Numerous formulae have been developed that utilize keratometry
and axial length to calculate the IOL power required to achieve a given refractive outcome. Some
formulae incorporate additional parameters such as anterior chamber depth and lens thickness to try to
optimize accuracy. The SRK-T, Haigis, Hoffer Q and Holladay 1 and 2 are commonly used. Specific
formulae may be superior for very short (possibly the Hoffer
Q) or long eyes, but opinions vary and it is always wise to plan individually for an unusual eye,
consulting the latest research and recommendations. Short eyes in particular are prone to unexpected
mean spherical and astigmatic errors following surgery.

Intraocular lenses
Positioning
An IOL (Fig. 9.8A) consists of an optic and haptics. The optic is the central refracting element, and the
haptics the arms or loops that sit in contact with peripheral ocular structures to centralize
the optic. Modern cataract surgery, with preservation of the lens capsule, affords positioning of the IOL
in the ideal location in the bag (Fig. 9.8B). Complicated surgery, with rupture of the posterior capsule,
may necessitate alternative positioning in the posterior chamber with the haptics in the ciliary sulcus (a
three- piece IOL only, not one-piece including those with plate haptics, as these may not be stable), or
in the anterior chamber (AC) with the haptics supported in the angle AC positioning requires a specific
lens type. In some circumstances a supplementary IOL may be placed in the sulcus in addition to an
IOL in the capsular bag, for instance to address a residual refractive error following primary surgery
(secondary pseudopolyphakia), and thin-profile IOLs are available for this purpose. It is preferable to
avoid a sec- ondary sulcus IOL (primary pseudopolyphakia) in very short (e.g. nanophthalmic) eyes due
to the risk of angle closure; off-the-shelf IOLs of power up to 40 D are available, and custom IOLs can
be produced in even higher powers.

Manual cataract surgery

When posterior chamber IOLs began to be widely used in the 1980s most surgeons adopted
extracapsular cataract extraction (ECCE), abandoning the older intracapsular technique (ICCE). In
ICCE, a cryoprobe is used to remove the lens complete with its capsule (Fig. 9.10A). In ECCE, after a
large anterior capsulotomy is created, an extensive limbal incision (810 mm) is completed and the lens
nucleus is expressed following hydrodissection to free its cortical attachments (Fig. 9.10B). Cortical
matter is then aspi- rated, leaving behind a sufficiently intact capsular bag to support an IOL. Suturing of
the incision is required, sometimes inducing considerable corneal astigmatism. Manual small-incision
cataract surgery (MSICS) is a variant of ECCE used to address the require- ment for high-volume
surgical throughput of dense cataracts in less affluent geographical regions; it involves the creation of a
small self-sealing sclerocorneal tunnel (Fig. 9.10C), manual one- piece expression of the nucleus (Fig.
9.10D), manual aspiration of the cortex and IOL implantation. Visual rehabilitation is compa- rable to
phacoemulsification but MSICS is faster and avoids the need for expensive technology.
Phacoemulsification
Introduction
Phacoemulsification (phaco) is the standard method of cataract extraction in developed countries, and
in regional centres in most developing countries.
Phacodynamics
Choosing appropriate settings makes surgery safer and easier. Level of irrigating bottle above the
patients eye is set to
maintain anterior chamber stability with a reasonable IOP.
Infusion flow is proportional to the height of the bottle. Aspiration flow rate (AFR) refers to the volume
of fluid
removed from the eye in millilitres per minute. For a higher AFR the irrigating bottle must be elevated to
compensate for increased fluid loss. High AFR results in attraction of lens material towards the phaco
tip, with faster vacuum build-up and swifter removal of lens matter but with less effective power. A high
AFR should usually be avoided by trainee surgeons.
Vacuum, measured in mmHg, is generated during occlusion when the pump is attempting to aspirate
fluid. Vacuum level determines how tightly material is held by the phaco tip when occluded, providing
the ability to manipulate lens fragments. High vacuum can decrease the total power required to remove
the lens. As with AFR, a lower-vacuum setting slows down the speed of intraocular events, reduces
theintensityofsurge(seebelow)andmakesinadvertent aspiration of iris or lens capsule less likely.
Post-occlusionsurge.Whenocclusionofthephacotipby lens material is broken, pent-up energy results in
a sudden temporary increase in outflow surge. This may result in complications such as capsular
rupture, and as far as possible is suppressed by modern phaco machines.
Handpiece
The phaco handpiece (Fig. 9.11A) features a tip consisting of a
hollowtitaniumneedlewithanenclosingfluid-coolingsleeve(Fig. 9.11B) to protect the cornea from thermal
and mechanical damage. Its emulsifying action is mediated by very high-frequency (ultra- sonic)
vibration leading to jackhammer, cavitation and other effects; some machines offer variants such as a
torsional phaco action or water jet-mediated phacoemulsification. Phaco tips of differing shapes and
sizes are available, each having particular cutting and holding characteristics

Technique
Preparation Topical anaesthetic is followed by povidone-iodine 5%
(Fig. 9.12A) or chlorhexidine instillation into the
conjunctival sac and cleaning of the eyelids (Fig. 9.12B), ensuring thorough eyelash application; the
antiseptic should be left to work for a minimum of 3 minutes.
Careful draping (Fig. 9.12C) is performed, excluding the lashes and lid margins from the surgical field,
and a speculum is inserted.
Incisions
A side port incision is made around 60 to the left (in
right-handed surgeons) of the main incision; some surgeons prefer two side ports approximately 180
apart.
Viscoelastic is injected into the anterior chamber.
Many surgeons locate the main corneal incision on the steepest corneal axis, others prefer consistent
siting. Temporal incisions can provide better access and less induced astigmatism but may be
associated with a slightly higher risk of endophthalmitis.
Continuous curvilinear capsulorhexis is performed with a cystotome, a bent hypodermic needle and/or
capsule forceps (Fig. 9.13B).
Hydro dissection is performed to separate thenucleus and cortex from the capsule so that the nucleus
can be manipulated. A blunt cannula is inserted just beneath the edge of the capsulorhexis and fluid
injected gently under the capsule (Fig. 9.13C). A hydrodissection wave should be seen, provided there
is an adequate red reflex.
Divide and conquer is a widely used, safe technique for removal of the nucleus in which two
perpendicular grooves are created (sculpting), the phaco tip and a second instrument engaged in
opposite walls of the grooves and the nucleus cracked into quadrants by applying force in opposite
directions (Fig. 9.13D). Each of the quadrants is then emulsified and aspirated in turn (Fig. 9.13E).
Phaco chop has the advantage of generally greater speed and a lower total phaco energy
requirement, but commonly takes longer to learn. In horizontal chopping a blunt-tipped chopper is
placed horizontally underneath the capsule and rotated vertically as the equator is reached. Vertical
chopping is performed with a pointed-tip
chopper that does not need to pass beyond the capsulorhexis. The nucleus is separated into several
pieces for emulsification.
Stop and chop is a combination technique. Removal of lens cortex. Cortical lens matter segments
are
carefully engaged by means of vacuum, peeled away centrally from the lens capsule and aspirated.
Automated coaxial, bimanual automated (Fig. 9.13F) and manual aspiration (e.g. Simcoe cannula)
methods are available.
IOL insertion. The capsular bag is filled with cohesive viscoelastic. A loaded injector cartridge is
introduced through the main section and the IOL slowly injected and unrolled inside the capsular bag; a
toric IOL (see Fig. 9.8C) should be rotated to the correct alignment. Viscoelastic may be aspirated prior
to or following toric IOL
rotation. Completion. Side port incisions and the main wound may
be sealed with corneal stromal saline injection (hydrosealing). Prophylactic measures at the end of
surgery may include intracameral (anterior chamber) antibiotic injection, subconjunctival injection of
antibiotic and steroid and/or topical antibiotic.

Posterior capsular opacification

Visually significant posterior lens capsular opacification (PCO), also known as after cataract, is the
most common late complica- tion of uncomplicated cataract surgery, historically occurring eventually in
up to 50% of patients. It is caused by the proliferation of lens epithelial cells that have remained within
the capsular bag following cataract extraction. The incidence of PCO is reduced when the capsulorhexis
opening is in complete contact with the anterior surface of the IOL. PMMA (and probably to a lesser
extent hydrogel) IOLs are particularly prone to PCO, but other- wise implant design is more important
than material; a square
optic edge appears to inhibit PCO (though may have a higher rate of dysphotopsia see below).
Diagnosis
Symptomsincludepersistentslowlyworseningblurring, glare and sometimes monocular diplopia.
VAisvariablyreduced,thoughdysfunctionmaybemore marked on contrast sensitivity testing.
Signstypicallyincludemorethanonepatternofopacification. Vacuolated (pearl-type) PCO (Fig. 9.21A)
consists of
proliferating swollen lens epithelial cells, similar to the bladder (Wedl) cells seen in posterior
subcapsular cataract (see Fig. 9.1B). They are commonly termed Elschnig pearls, particularly when
grouped into clusters at the edge of a capsulotomy (Fig. 9.21B), though strictly HirschbergElschnig
pearls refers to globular or grape- like collections of swollen cells seen following traumatic or surgical
anterior capsular rupture.
Fibrosis-type PCO (Fig. 9.21C) is thought to be due to fibroblastic metaplasia of epithelial cells, which
develop contractile qualities.
A Soemmering ring is a whitish annular or doughnut- shaped proliferation of residual cells that
classically formed almost in the periphery of the capsular bag following older methods of cataract
surgery, but is clinically uncommon now. It may form at the edge of a capsulorhexis or capsulotomy.
Treatment
Treatment involves the creation of an opening in the posterior capsule, termed a capsulotomy (see Figs
9.21B and D), with the Nd:YAG laser. Indications.Thepresenceofsignificantvisualsymptomsis
the main indication; less commonly, capsulotomy is performed to improve an inadequate fundus view
impairing assessment and treatment of posterior segment pathology.
Technique.Safeandsuccessfullasercapsulotomyinvolves accurate focusing and use of the minimum
energy required. Laser power is initially set at 1 mJ/pulse, and may be increased if necessary. A series
of punctures is applied in a cruciate pattern using single-pulse shots, the first puncture aimed at the
visual axis. The opening should equate approximately to the size of the physiologically dilated pupil
under scotopic conditions this averages around 45 mm in the pseudophakic eye. A larger
capsulotomy may be necessary if glare persists, or for retinal examination or treatment, but the
capsulotomy should not extend beyond the edge of the optic in case vitreous prolapses around its edge.
It may be prudent to adopt a higher threshold for treatment, and minimizing its extent, in eyes at risk of
retinal detachment (e.g. high myopia), CMO (e.g. history of uveitis) or lens displacement (e.g.
pseudoexfoliation). Some research suggests that the total energy applied should be less than 80 mJ in
order to reduce the risk of a significant IOP spike or increase in macular thickness.

CONGENITAL CATARACT
Aetiology
Congenital cataract occurs in about 3 in 10000 live births. Two- thirds are bilateral and a cause can be
identified in about half of these. Autosomal dominant (AD) inheritance is the most common aetiological
factor; others include chromosomal abnormalities, metabolic disorders and intrauterine infections.
Isolated inherited congenital cataracts carry a better visual prognosis than those with coexisting ocular
and systemic abnormality. Unilateral cataracts are usually sporadic, without a family history or systemic
disease, and affected infants are usually otherwise healthy.
Associated metabolic disorders
Galactosaemia
Galactosaemia is an autosomal recessive (AR) condition charac- terized by impairment of galactose
utilization caused by absence of the enzyme galactose-1-phosphate uridyl transferase (GPUT). Unless
galactose (milk and milk products) is withheld from the diet, severe systemic complications culminate in
early death. Oil droplet lens opacity (see Fig. 9.25D) develops within the first few days or weeks of life
in a large percentage of patients. Exclusion of galactose may reverse early lens changes.
Lowe syndrome
Lowe (oculocerebrorenal) syndrome is an X-linked recessive (gene: OCRL1) inborn error of amino acid
metabolism with neu- romuscular, renal and other manifestations. Cataract is universal, and
microphakia may also be present. Congenital glaucoma is present in about half of patients. Female
carriers may have visually insignificant cortical lens opacities.
Fabry disease
See Chapter 6.
Mannosidosis
Mannosidosis is an AR disorder with deficiency of -mannosidase. Infantile and juvenile-adult forms are
seen, both of which feature progressive mental deterioration, musculoskeletal and other abnormalities.
Punctate lens opacities arranged in a spoke-like pattern in the posterior lens cortex are frequent;
corneal clouding can also occur but is less common.
Other metabolic disorders
Potential causes include hypo- and pseudohypoparathyroidism, and hypo- and hyperglycaemia.
Associated intrauterine infections
Rubella
Congenital rubella results from transplacental transmission of virus from an infected mother, and may
lead to severe fetal mal- formations. Pearly nuclear or more diffuse unilateral or bilateral cataract occurs
in around 15%. (See also Ch. 11.)
Toxoplasmosis
Ophthalmic features of congenital toxoplasmosis include cataract, chorioretinitis, microphthalmos and
optic atrophy. (See also Ch. 11.)
Cytomegalovirus infection
Systemic features of congenital cytomegalovirus (CMV) infection include jaundice, hepatosplenomegaly,
microcephaly and intra- cranial calcification. Ocular features apart from cataract include chorioretinitis,
microphthalmos, keratitis and optic atrophy.
Varicella
Systemic features include mental handicap, cortical cerebral atrophy, cutaneous scarring and limb
deformities; death in early infancy is common. Ocular features may include cataract, micr- ophthalmos,
chorioretinitis, optic disc hypoplasia and optic atrophy.
Others
Measles, syphilis, herpes simplex and human immunodeficiency virus (HIV). (See also Ch. 11.)
Other systemic associations
Down syndrome (trisomy 21)
Systemicfeaturesincludelearningdifficulties,stunted growth, distinctive facial and peripheral features,
thyroid dysfunction, cardiorespiratory disease and reduced life span.
Ocularfeatures.Cataractofvariedmorphology(75%);the opacities are usually symmetrical and often
develop in late childhood. Other features include iris Brushfield spots (see Fig. 12.14B) and hypoplasia,
chronic blepharitis, myopia, strabismus and keratoconus.
Edwards syndrome (trisomy 18)
Systemic features. Characteristic facial and peripheral features, deafness, cardiac anomalies, mental
handicap and early death.
Ocular features apart from cataract include ptosis, microphthalmos, corneal opacity, uveal and disc
coloboma and vitreoretinal dysplasia.
Miscellaneous
HallermannStreiff syndrome features impaired growth and other features, with cataract in 90%;
NanceHoran syndrome is an X-linked condition comprising distinctive dental and facial anom- alies
together with congenital cataract and microcornea. Female carriers may show Y suture opacities (see
Fig. 9.25F).
Management
Ocular assessment
Determination in the neonate of the visual significance of lens opacity is based principally on the
appearance of the red reflex and the quality of the fundus view. A very dense cataract occluding the
pupil; the decision to
operate is straightforward. Alessdensebutstillvisuallysignificantcataract(e.g.central
or posterior opacities over 3 mm in diameter) will permit visualization of the retinal vasculature with the
indirect but not with the direct ophthalmoscope.
Avisuallyinsignificantopacitywillallowclearvisualization of the retinal vasculature with both the indirect
and direct ophthalmoscope.
Otherindicatorsofseverevisualimpairmentinclude absence of central fixation, nystagmus and
strabismus.
Morphology Blue dot opacities (Fig. 9.25A) are common and
innocuous. Nuclear opacities (Fig. 9.25B) are confined to the
embryonic or fetal nucleus. Lamellar opacities affect a particular lamella of the lens
both anteriorly and posteriorly and may be associated with radial extensions (riders Fig. 9.25C).
Lamellar opacities may be autosomal dominant or occur in isolation as well as in association with
metabolic disorders and intrauterine infections.
Coronary (supranuclear) cataract lies in the deep cortex, surrounding the nucleus like a crown. It is
usually sporadic but occasionally hereditary.
Central oil droplet opacities (Fig. 9.25D) are characteristic of galactosaemia.
Posterior polar cataract (Fig. 9.25E) may be associated with posterior lenticonus or fetal vascular
remnants including a Mittendorf dot. This form of opacity is often closely integrated with the lens capsule
and/or a pre- existing defect, with a very high risk of dehiscence during surgery.
Sutural, in which the opacity follows the anterior or posterior Y suture (Fig. 9.25F); may be seen in
female NanceHoran carriers.
Anterior polar cataract may be flat or project into the AC. Occasional associations include persistent
pupillary membrane, aniridia, Peters anomaly and anterior lenticonus.
Associated ocular pathology may involve the anterior (e.g. corneal clouding, microphthalmos,
glaucoma, persistent fetal vasculature) or posterior segments (e.g. chorioretinitis, Leber amaurosis,
rubella retinopathy, foveal or optic nerve
hypoplasia). Its presence may give an additional indication of visual prognosis. The differential diagnosis
of leukocoria may apply (see Ch. 12).
Assessmentoffamilymembersforsubclinicalfamilial cataract is prudent.
Ultrasonographyshouldbeperformedifthefundusisnot visible, and may reveal a definitive cause such as
persistent fetal vasculature.
Specialtestssuchasforced-choicepreferentiallookingand visual evoked potentials may provide useful
supporting information.
Systemic investigations
Investigation of familial cataract is unnecessary, but otherwise the following should be considered.
Assessment beyond a search for infection and possibly urinary reducing substance is probably
unnecessary in unilateral cases.
Screeningforintrauterineinfectionsshouldusuallybe performed in all unilateral and bilateral cases.
Urine.Urinalysisforreducingsubstanceafterdrinkingmilk (galactosaemia) and chromatography for amino
acids (Lowe syndrome).
Otherinvestigationsmayincludefastingbloodglucose, serum calcium and phosphorus, red blood cell
GPUT and galactokinase levels. Children who have calcium and phosphorus anomalies severe enough
to cause cataract are likely to be unwell.
Referraltoapaediatricianmaybewarrantedfor dysmorphic features or suspicion of other systemic
diseases. Chromosome analysis may be useful in this context.
Treatment
The requirement for urgent surgery is balanced by the fact that the earlier this takes place, particularly
before 4 weeks of age, the higher the chance of glaucoma developing during the juvenile years.
Bilateral dense cataracts require surgery between 410 weeks
of age to prevent the development of stimulus deprivation amblyopia. If severity is asymmetrical, the eye
with the more dense opacity should be addressed first.
Bilateral partial cataracts may not require surgery until later, or indeed at any stage. In cases of doubt
it may be prudent to defer surgery in favour of careful monitoring.
Unilateral dense cataract merits more urgent surgery; there is no consensus regarding timing except
that 6 weeks is the latest point at which elective surgery should be performed. Many authorities would
advocate surgery between 4 and 6 weeks, followed by aggressive anti-amblyopia therapy, despite
which results are often disappointing. If the cataract is detected after 16 weeks of age then the visual
prognosis is particularly poor.
Partial unilateral cataract can usually be observed or treated non-surgically with pupillary dilatation
and possibly part-time contralateral occlusion.
Surgery involves anterior capsulorhexis, aspiration of lens matter, capsulorhexis of the posterior
capsule, limited anterior vitrectomy and IOL implantation, if appropriate. It is important to correct
associated refractive errors.
Postoperative complications
Surgery carries a higher incidence of complications than in adults.
Posteriorcapsularopacificationisnearlyuniversalifthe
posterior capsule is retained, and can have a substantial amblyogenic effect. Posterior capsulorhexis
with vitrectomy is generally performed during the primary lens extraction.
Secondarymembranesmayformacrossthepupil, particularly if postoperative uveitis is not treated
aggressively.
Proliferationoflensepitheliumisuniversal,oftenforming a Soemmering ring; it is usually visually
inconsequential.
Glaucoma Secondary open-angle glaucoma may develop in up to
two-thirds of eyes by 10 years after surgery. Angle-closure may occur in the immediate postoperative
period secondary to pupillary block, especially in
microphthalmic eyes. Retinaldetachmentisanuncommonandusuallylate
complication.
Visual rehabilitation
The visual results of cataract surgery in infants are hampered by amblyopia, which should be treated
aggressively (see Ch. 18). Spectaclesareusefulforolderchildrenwithbilateral
aphakia. Contactlensesprovideasuperioropticalsolutionfor
unilateral or bilateral aphakia. After the age of about 2 years compliance may worsen as the child
becomes more independent.
IOLimplantationisincreasinglybeingperformedin younger children and appears to be effective and safe
in selected cases. Hypermetropia (correctable with spectacles) is initially targeted, and as the child ages
decay towards emmetropia should occur; however, the final refractive outcome is variable.