NEONATAL CATARACT: AETIOLOGY, PATHOGENESIS

AND MANAGEMENT

I . C. LLOYD', M. GOSS-SAMPSON2, B. G. JEFFREY', A. KRISS',

I . RUSSELL-EGGITT' and D. TAYLOR'
London

SUMMARY the treatment of children with congenital cataracts. Better

We review the epidemiology, aetiology, pathogenetic understanding of amblyopia, more sophisticated surgical
mechanisms and clinical management of neonatal catar­ techniques, and advances in contact lens technology have
act. Visual development and the effects of visual depri­ all contributed to this. Early treatment is one of the most
vation in the infant with congenital cataract are discussed important factors in determining the eventual visual out­
and related to the timing of surgery. Surgical techniques come.6 Early detection is therefore vital and it is important
and the important operative and post-operative compli­ that paediatricians and general practitioners receive
cations are discussed. We review post-operative manage­ adequate training in this area.7 Correct surgical manage­
ment and compare the different techniques available for ment and the subsequent post-operative care are impor­
aphakic correction, and describe the VEP changes found tant. This includes accurate optical correction together
in patients with monocular cataract. with appropriate occlusion therapy. Parental motivation to
achieve the same goals as the ophthalmic team is essential.
Much time, patience and understanding is required by
INTRODUCTION AND EPIDEMIOLOGY
ophthalmologists and their co-workers involved in the
Congenital cataract remains an important and frequently management of these children.
difficult problem to manage and the care of affected chil­
dren is costly and time consuming. A number of studies AETIOLOGY
have shown that although this condition is potentially All children with congenital cataract should be carefully
treatable it continues to account for a significant propor­ assessed in an attempt to establish a cause. This should
tion of children with partial sight and blindness. It was include a history and physical examination, examination
found to be the commonest cause of blindness among chil­ of other family members and, where indicated, appro­
dren attending the Yamanashi school for the blind in Japan priate laboratory studies, neurophysiological assessment
( 16/67)' and similarly one of the three major causes in the and imaging techniques. It is useful to work closely with a
Royal Blind School in Edinburgh, Scotland ( 12/99).2 paediatrician and clinical geneticist as this helps to reduce
Stewart-Brown and Haslum's recent British study indi­ unnecessary investigations and thereby saves time and
cated that it was the most common cause of partial sight or expense. The Flow Chart in Fig. 5 provides a schematic
blindness in a nationally representative cohort of 15,000 approach to the diagnosis and assessment of the neonate
ten year old children.3 Congenital cataract accounted for presenting with congenital cataract. The ophthalmologist
nearly 19% of children in British Columbia who were is often able to identify hereditary cataracts and those that
legally blind in a study by Robinson et al. covering 29 are typically developmental, i.e. in association with per­
years4 but a more recent survey of childhood blindness in sistent hyperplastic primary vitreous or anterior segment
the Republic of Ireland produced a much lower proportion dysgenesis, in an otherwise well child. Approximately
(8/ 172).5 half of all congenital cataracts are idiopathic,8 and in par­
In recent years there have been many improvements in ticular, a cause for unilateral cataract is rarely found.9
Hereditary cataracts are usually inherited as an autosomal
'Department of Ophthalmology. The Hospitals for Sick Children,
Great Ormond Street. dominant trait with associated microphthalmos.'o These
2Division of Biochemistry and Metabolism, The Institute of Child cataracts are often lamellar in morphology. The parents
Health, London. and all siblings of affected children should be examined in
Correspondence to: D. Taylor FRCS FRCP FCOphth. Department of
Ophthalmology. The Hospitals for Sick Children, Great Ormond Street,
every case as there may be very variable expressivity of
London. the gene within the family.9 Thus an apparently normal

Eye (1992)6, 184-196

NEONATAL CATA RA CTS 185

parent or sibling may have a visually insignificant sutural some patients the cataracts may disappear when the diet is
cataract whilst the more severely affected child has a altered (Fig. 4).17 Reducing substances are present in the
dense nuclear cataract. X-linked inheritance may also urine of patients with galactokinase deficiency and galac­
occur and present similarly with a markedly affected male tosaemia one to two hours after a galactose containing
child of a mildly affected or asymptomatic mother (Figs. 1 meal (milk). If this test is positive, further enzymatic
and 2).11 Autosomal recessive inheritance is uncommonl2 assays can be used to distinguish the two disorders. Chil­
but should be considered particularly if there is consan­ dren who fail to thrive and have cataracts should have their
guinity or multiple affected offspring with unaffected urine screened for amino-acids. Lowe's syndrome is an
parents. X-linked recessive condition manifested in infancy by
Any history of maternal febrile illness or skin rashes dysmorphic facies, mental and psychomotor retardation,
during pregnancy should be sought. First trimester rubella failure to thrive, aminoaciduria, vitamin-D resistant rick­
infection is a cause of severe multiple congenital anoma­ ets and cataracts.18 Virtually all affected males with
lies and cataracts (unilateral or bilateral) may be present at Lowe's syndrome have cataracts at birth and glaucoma
birth or develop in the first year of life.8 The cataract is may also be associated. The lens has a reduced ante­
related to direct virus invasion of the lens and virus may be roposterior diameter and may show developmental anom­
cultured from the lens matter removed at surgery up to alies such as posterior lentiglobus or a discoid shape.19 The
four years of age and is probably responsible for the vigor­ carriers of this syndrome commonly have fine peripheral
ous post-operative uveitis that often occurs in these chil­ dot lens opacities and so female relatives should be exam­
dren.13 Congenital infection with the varicella zoster virus, ined for this clinical sign?O
herpes simplex virus, toxoplasmosis and cytomegalovirus Hypocalcaemia in the neonate may be secondary to
have also been associated with cataracts. 14 Metabolic dis­ hypoparathyroidism or pseudohypoparathyroidism.8 Sub­
orders associated with cataract formation result in failure sequent cataract formation is related to increased lens cap­
to thrive and systemic symptoms in all except the mild dis­ sule permeability and the morphology of the lens opacity
order of galactokinase deficiency. Children with galacto­ is usually lamellar 'with punctate cortical dots. Fits are
kinase deficiency are usually. well and cataracts are the often the initial symptom associated with failure to thrive.
only clinical manifestation.15 It is inherited in an auto­ Serum calcium and phosphate levels should be checked in
somal recessive manner and homozygotes develop bilat­ such children and further management of their general
eral lamellar cataracts in fetal life or early infancy. problems undertaken by a paediatrician. Hypoglycaemia
'
Galactokinase deficiency is common, however, therefore in the perinatal period is usually characterised by fits and
a careful examination of even asymptomatic parents is neurological problems. Cataracts associated with this are
essential to exclude dominantly inherited cataracts in a often transient but may persist as lamellar opacities if the
child who is incidentally galactokinase deficient. Galacto­ hypoglycaemia is recurrent.1 0
saemia is also an autosomal recessive trait and is charac­ Low birth weight premature infants were noted in one
terised by a deficiency of galactose-I-phosphate uridyl study to have an incidence of transient cataracts of 2.7%.21
transferase. Tissue accumulation of galactose-I-phos­ These were characterised by fluid vacuoles bilaterally just
phate leads to failure to thrive, lethargy and bilateral anterior to the posterior lens capsule and usually under­
lamellar or 'oil-drop' cataracts (Fig. 3).16 Splenomegaly, went complete resolution over a one week to four month
hepatomegaly and jaundice and subsequent mental retar­ period. Pike et al. however feel that prematurity itself is an
dation ensue unless galactose is withheld from the diet. In unlikely cause of cataract. They propose that perinatal dif-

Fig. 1 Fig. 2

Figs. 1 and 2. X-linked sutural cataracts. Figure 1 shows the right 'lazy' eye of the mother of a boy with congenital cataracts. A
significant sutural and nuclear lens opacity (of which she was unaware) is present. Figure 2 shows the left eye which has a visually
insignificant sutural lens opacity.
186 I. C. LLOYD ET AL.

Fig. 4

Figs. 3 and 4. Galactosaemia, Figure 3 shows a typical 'oil­

drop' lens opacity in an infant with Galactosaemia, Figure 4 is
from the same eye after the infant has had dietary restriction of
galactose and shows complete regression of the lens opacity,

Fig. 3

ficulties are often related to preceding maternal or fetal the short arm of chromosome 5)27 are all associated with
abnormality and that this abnormality is more likely to congenital cataract. A number of studies involving genetic
predispose to cataract.22 mapping of families with congenital cataracts have been
Certain ocular disorders are associated with congenital performed in recent years.2S,29,3 0 It is estimated that any of
cataract and they are listed in Table I(V). Infants with Per­ twelve different genes may cause dominantly inherited
sistent hyperplastic primary vitreous need careful assess­ congenital cataracts but as yet only two such genes have
ment to exclude posterior vitreous and retinal involvement been assigned to specific chromosomal loci.3l Linkage
as in these cases the prognosis is very pOOr.23 Retinoblas­ analysis studies have found that the gene for autosomal
toma should be borne in mind in any child with a unilateral dominant pulverulent cataracts lies on chromosome 1 due
cataract in whom it is difficult to clearly visualise the fun­ to its close linkage to the Duffy blood group and similarly
dus.24,25 Ultrasound and/or CT scanning is necessary in an autosomal dominant posterior polar cataract lies in a
these cases. It is however very rare for these cases to pres­ region close to the haptoglobin gene on chromosome 16.29
ent in the neonatal period, Retinopathy of prematurity,24 Breaks in two non-homologous chromosomes follwed by
aniridia24 and the anterior chamber cleavage disorders26 aberrant reunion is known as reciprocal translocation.
are also associated with cataract in infancy, Autosomal dominant anterior polar cataracts associated
A large number of inherited disorders include cataract with a balanced reciprocal translocation between chro­
in the neonatal period as one of their manifestations mosomes 2 and 14 have been reported,zs Reese et al.
(Table I(VII)). A number of chromosomal syndromes fall showed reciprocal translocation between the short arms of
within this group and of these the commonest is trisomy chromosomes 3 and 4 [t(3;4)(p26.2;p15)] in a father and
21 (Down's syndrome). The general features of Down's son with autosomal dominant congenital cataracts.29 The
syndrome are well known.S Several types of lenticular locus for X-linked congenital cataracts and microcornea
opacities may occur in this condition including lamellar (Nance-Horan syndrome) has been shown to lie around
cataract, sutural cataract, posterior polar cataract and com­ Xp22.2-p22.3.30 All of these areas would be good sites for
plete mature cataract. The incidence of cataracts in young further investigations into the genetic basis of these types
children with Down's syndrome is of the order of 60% and of cataract.
this increases in adolescence. Trisomy 13,s Trisomy 18,s Many other inherited disorders are associated with
Turner syndromes and Cri-du-chat syndrome (deletion of cataract and it is essential to seek a genetic and paediatric
NEONATAL CATARA CTS 187

Table. Aetiology of Cataract in the Neonate

Idiopathic approximately 50% (8)

II Intrauterine Infection Rubella (14)
CMV (14)
(14)
Varicella Zoster
Herpes Simplex (14)
Toxoplasma (14)

III Metabolic Disorders Galactosaemia (McKusick 230400) (16)

Galactokinase deficiency (230350) (15)
Marginal Maternal Galactokinase deficiency (34)
Hypocalcaemia (8, 10)
Hypoglycaemia (10)
Alpha-mannosidosis (248500) (34)
Sorbitol dehydrogenase deficiency (182500) (34)
Hyperglycinuria (34)
Sialidosis (256550) (34)

IV Prematurity (21)
V Ocular Disease PHPV (35)
Aniridia (10620) (24)
Aniridia Plus (36)
Retinopathy of prematurity (24)
Anterior chamber cleavage syndromes (26)
Intra-ocular tumour (24, 25)

VI Inherited Without Systemic Abnormality Autosomal Dominant (116700) (10)

Autosomal Recessive (212700) (11)
X-linked Recessive (302200) (12)

VII Inherited With Systemic Problems

1. Chromosomal: Trisomy 21 (8)

Trisomy 13-15 (8)
Trisomy 16-18 (8)
Turner syndrome-45 XO (8)
Cri-du-chat syndrome-Deletion on chromosome 5 (27)
2. Skeletal disease: Conradi-Hunermann syndrome (118650) (37)
Rhizomelic Chondrodysplasia Punctata (215100/302950) (37)
Stickler syndrome (108300) (38)
CAMFAK syndrome (212540) (39)

3. Syndactyly, Polydactyly, or other digital anomalies: Rubenstein-Taybi syndrome (268600) (40)

Ellis van Creveld syndrome (225500) (41)
4. Central Nervous System abnormalities: Marinesco-Sjogren syndrome (248800) (42)
Norrie's disease (310600) (43)
Martsolf syndrome (212720) (44)
Cerebro-oculo-facial-skeletal syndrome (214150) (45)
Smith-Lemli-Opitz syndrome (270400) (8)
5. Muscle Disease: Myotonic dystrophy (160900) (8)
Cataract, lactic acidosis and cardiomyopathy (212350) (46)
6. Mandibulo-facial syndromes: Hallermann-Streiff syndrome (234100) (32)
Nance-Horan cataract-dental syndrome (302350) (30)
7. Renal Disease: Lowe's syndrome (309000) (18, 19)
8. Dermatological Disease: Goltz syndrome (305600)
Incontinentia pigmentii (308300) (47, 8)
Congenital ichthyosis (212400) (8)
Marshall's ectodermal dysplasia (8)
Schafer syndrome (8)
Siemens syndrome (8)
Cataracts, Alopecia and Sclerodactyly (212360) (48)

OpInIOn before starting treatment. Haller­ ditions associated with cataracts in case reports and the
mann-Streiff-Fran<;ois syndrome may present with dense use of a dysmorphology database (e.g. the London Dys­
white cataracts in association with short stature, skull morphology Database)33 may be useful in diagnosis.
abnormalities, hypotrichosis, thin curved nose and a
prominently veined atrophic skin.32 The clinical geneticist PATHOGENESIS OF CONGENITAL
or dysmorphologist is most important in identifying such CATARACT
children and in providing accurate genetic counselling for The pathogenic mechanisms of cataract formation in
the parents. A more complete list of causes of cataract in infancy and childhood are not yet fully understood, how­
the neonate is listed in Table I. There are many other con- ever, there has been some research on cataract associated
188 I. C. LLOYD ET AL.

CONGENITAL CATARACT
to be the only manifestation of this conditionl5. In these

I I
cases the aldose reductase pathway converts accumulated
f---.. GENETIC REFERRAL galactose to galactitol. The accumulation of this then leads
y to lens fibre swelling and opacities. Early galactose res­
triction in these patients (by six weeks) can prevent the

1? DYSMORPHIC ""'I --- ... CHROMOSOME ANALYSIS

progression of the cataracts and in some cases r!!gression
is observed (Fig. 4)17. In vivo studies using experimental
I Y GENETIC REFERRAL
N models of cataracts due to abnormal sugar metabolism
have shown that they can be alleviated or prevented by

r;1
? PREGNANCY RASH/ILLNESS ? INTRAUTERINE INFECTIO
?+VE TORCH Ig SCREEN
aldose reductase inhibitors49.
PAEDIATRIC REFERRAL
In infants with disorders of glycoprotein degradation

l N

? +VE URINE RED.SUBST METABOUC ASSESSMENT

such as a-mannosidosis there is an accumulation of
abnormal mannose-enriched oligosaccharides causing
I opacities of the posterior lens capsule which can be
? FAILURE TO THRIVE PAEDIATRIC REFERRAL
Y observed within the first few months of life50. In addition
? SYSTEMIC PROBLEMS
to these inherited metabolic diseases there are many others
l N as discussed above in which cataract is associated34.51 but
the underlying cataractogenic process is unknown.
I
SPORADIC/DEVELOPMENTAL
(PARTICULARLY UNILATERAL)

Oxidative damage
, Y

ASSESS FOR:
In recent years oxidative damage of the lens and associ­
MICROPHTHALMOS ated structures by oxygen derived free radicals has been
PHPV implicated in the aetiology of cataract formation. Oxygen­
ANTERIOR/POSTERIOR
derived free radicals are highly reactive species of com­
SEGMENT PROBLEMS
pounds which if unchecked can oxidise lipids, proteins
Fig. S. Flow chart for diagnosis in congenital cataract.
and other cellular components causing changes in mem­
brane structure and function and cell damages2 • Such dam­
with inherited metabolic disorders and, recently, oxidative age in the lens and other tissues is normally prevented by
damage to the lens and associated structures by oxygen cellular antioxidant defences such as superoxide dis­
derived free radicals has been implicated. mutase, glutathione peroxidase, vitamin E and ascor­
bates3 • The potential for generating damaging
Metabolic disease
concentrations of free radicals in and around the lens is
Cataracts associated with inherited metabolic disorders considerable. The aqueous humour surrounding the lens
usually appear in infancy and early childhood since the contains high concentrations of ascorbate but this can
underlying defect often causes damage to the susceptible easily undergo auto-oxidation or in the presence of high
developing lens. It is now generally accepted that defects concentrations of transition metals (ie. Cu2+, Fe2+) gener­
in the metabolism of hexose sugars such as in diabetes and ate free radicals54,55. Furthermore, the lens is constantly
galactosaemia lead to osmotically induced cataracts. In exposed to light and absorbs light in the near UV region
untreated or poorly controlled diabetics, glucose accumu­ (300-400nm) which has been shown to cause oxidative
lates in the aqueous humour and lens and is reduced to the damage directly and via photosensitisers55,56.
impermeant polyol sorbitol via the aldose reductase path­
way. Subsequent metabolism of sorbitol is very slow and
results in its lenticular accumulation followed by hydra­
tion of the lens fibres with associated membrane permeab­
ility changes. This leads to electrolyte imbalance (which
inhibits protein synthesis), decreased levels of reduced
glutathione, ATP and amino acids. These biochemical
changes are accompanied by the disintegration and vacu­
olation of swollen fibres which eventually leads to the for­
mation of cortical opacities.49 This, however, is rarely seen
in infant diabetics but usually becomes apparent later in ALTERED LENS FIBRE
life. INTEGRITY

Osmotically induced cataracts can be observed within

the first week of life in patients with galactosaemia due to
galactose-I-phosphate uridyl transferase deficiency
(Fig. 3)16. In the milder variant of galactosaemia in which
there is an absence of the enzyme galactokinase, cataracts Fig. 6. Proposed sequence of events of cataract formation due
can be observed in the first few months of life and appear to oxidative damage.
NEONATAL CATARACTS 189

Patients in whom oxidative damage may be an impor­ membrane structure and permeability. In the presence of
tant factor in the aetiology of their cataract formation weakened membrane 'defences' and further lipid perox­
include those with Down's syndrome. There is now evi­ idation, oxidative cross-linking and aggregation of the
dence that patients with Down's syndrome have increased crystallins occurs resulting in lens opacity--or cataract.
indices of free radical activity and lipid peroxidation57. It The exact aetiological mechanism(s) in most types of con­
has been suggested that this is due to the increased levels genital cataract has yet to be delineated and much further
of CuZn- superoxide dismutase (carried on chromosome research is needed.
21) generating increased hydrogen peroxide concentra­
THE MANAGEMENT OF CONGENITAL
tions. In the presence of superoxide this produces highly
CATARACT
reactive hydroxyl radicals. Infants with compromised
antioxidant status and those undergoing radiation or ste­ The Sensitive Period, Visual Development and
roid therapy may also be more susceptible to oxidative Prognosis
damage. The rapid evolution of some infantile cataracts Both unilateral and bilateral congenital cataract cause sig­
may reflect more severe and readily detectable bio­ nificant visual deprivation in infancy and therefore the
chemical abnormalities such as increased free radical timing of treatment is crucial to the visual development
activity than those in age related cataracts. and successful rehabilitation of these children. Evidence
Evidence for a role of oxidative damage in cataract for­ for this has come from studies in both animals and
mation has come from a number of in vivo and in vitro humans. Hubel and WieseCo,71 and von Noorden and col­
studies. Cataracts have been induced experimentally in leagues72 conducted animal experiments in cats and mon­
animals by methods such as ionising radiation58, systemic keys respectively, and have shown that visual deprivation
methyl prednisolone59, streptozotocin diabetes60 and from birth to three months of age results in predictable
hyperbaric oxygen61, all of which have been shown to developmental changes in the animals' visual system
increase indices of free radical activity and lipid perox­ including a reduction in cortical neurons connected to the
idation. Treatment with antioxidants such as vitamin E58 amblyopic eye as well as a reduction in cortical neurons
ascorbate62 butylated hydroxytoluene63 and carnosine64 receiving binocular input. The lateral geniculate nucleus
have been shown to be effective in preventing or limiting shows a concurrent decrease in cell size in the layers
cataract formation. In a canine model of senile cataract, innervated by the amblyopic eye. These changes result in
where increased lipid peroxidation was demonstrated, reduced vision in the deprived eye. The age of onset and
antioxidant therapy caused partial restoration of lens duration of visual deprivation determines the extent to
transparency65. It has also been shown that the posterior which these changes occur. Correction of the visual depri­
subcapsular cataracts which develop in the RCS (Royal vation during the sensitive period can modify these
College of Surgeons) rat appear to be initiated by oxida­ changes.
tive products of unsaturated fatty acids in the retina which The visual systems of the different animal models are
leak into the vitreous and attack the posterior surface of sensitive to visual deprivation for different periods, and
the lens66• the ages at which any resultant changes are reversible also
Studies using lens cultures in the presence of free rad­ varies. The period of reversibility or critical period, and
ical producing systems have shown cataract formation in the timing of surgery in relation to this are important fac­
vitro with a concomitant increase in indices of lipid perox­ tors in the prognosis for infants undergoing cataract sur­
idation and changes in K+,NA+ ATPase activity. This then gery6. The response to clinical treatment of unilateral
leads to an ionic imbalance within the lens fibres similar to cataract has helped to establish the duration of the critical
those seen in osmotic sugar cataracts67. Changes in mono­ period in humans although it is not yet fully determined. It
and divalent cation concentrations, ATP levels and water appears to be within the first four months of life73• Drum­
content have all been reported in lenses which contain cat­ mond et al.74 have shown that surgery for unilateral con­
aracts. How lipid peroxidation initiates these changes is genital cataract and subsequent optical correction must be
not yet fully understood, however it is likely that they are performed by 17 weeks of age in order to obtain a visual
either due to direct oxidation of membrane proteins or oxi­ acuity of 20/100 or better. Robb et aC5 produced similar
dation of the membrane lipids which may affect mem­ results with the poorest outcome in infants operated on
brane fluidity and protein function. Primary, secondary after 4 months. In Gelbart and Hoyt's study of infants with
and end products of lipid peroxidation have all been bilateral congenital cataract all of those operated on after
shown to accumulate in senile cataracts68,69. Accumulation 13 weeks exhibited nystagmus and had a poor visual
of these compounds in the lenticular epithelial membranes resule6. Only one patient out of the seven operated on after
is a possible cause of damage preceding cataract forma­ 8 weeks achieved a visual acuity better than 6/60. These
tion. It has therefore been suggested that oxidation of findings were confirmed by work from Rogers et al.77•
membrane components is a pre-cataract state. Thus studies in both' humans and animals have shown
From the evidence the following sequence after an oxi­ that a critical period exists in which the effects of visual
dative insult can be hypothesised (Fig. 6). Oxidation deprivation upon the developing visual system are most
initiated at the membrane leads to peroxidation of mem­ marked and beyond which are largely irreversible. They
brane lipids and proteins with subsequent changes in also show that there is a longer sensitive period during
190 1. C. LLOYD ET AL.

which deprivation continues to cause changes in the Surgical Techniques

developing visual system. Early surgery and detailed Two primary techniques are currently utilised for congeni­
management of the optical correction are therefore essen­ tal cataract surgery:
tial and have improved the long term prognosis particu­ (A) Aspiration is a method originally popularised by
larly in the bilaterally aphakic child76-78• However, visual Scheie85 and subsequently developed by other surgeons86•
outcome may vary markedly even in those patients whose The basis of this technique is that the posterior capsule is
treatment appears ideal and, in particular, in the infant left intact at the end of the initial operation. Subsequent
4
with unilateral catarace4 7. 5,78-8 . surgical or Yag capsulotomy is performed only if the cap­
The situation in infants affected by partial cataracts, and sule becomes opaque enough to interfere with vision. It is
in particular lamellar cataracts, is often more flexible and
a safe, simple procedure with a low rate of complications.
in many cases these children can be managed conser­
However, opacification of the posterior capsule is vir­
vatively by close follow-up until a more complete as�ess­
tually invariable in the infant and may be extremely rapid
ment of visual function and visual behaviour can be
in onset. This opacification often occurs during the critical
obtained. Early surgery in some of these children can
period for visual development and thus is a powerful cause
result in strabismus, amblyopia and loss of binocularity
of amblyopia due both to the occlusive effect of the opac­
thereby counteracting the benefit of improved visual
ity and its prevention of accurate retinoscopy. Posterior
acuity. It should be noted that anterior polar cataracts do
capsule opacification is less rapid and vigorous in the
not usually result in form deprivation amblyopia but may
older child and so this is the technique of choice only in
be associated with anisometropia, whilst posteriorly
patients after the age of 18 months. It is not indicated ill the
located cataracts are generally more amblyogenic25•
presence of other ocular abnormalities such as PHPV, iris
A dense unilateral cataract in general carries a very poor
anomalies, lens plaques, posterior lenticonus or calcified
visual prognosis unless surgery is performed by at least 17
material in the lens.
weeks of life and is followed by accurate optical correc­
tion and aggressive occlusion to the contralateral The use of a horizontally mounted YAG laser avoids the
eye74,.7578-84. Subsequent careful momtormg
' . 0f the VISIon
.. . m
necessity for surgical capsulotomy and its associated com­
both eyes by electrophysiological or psychophysical plications but does not avoid the need for a further general
methods is helpful in assessing the degree of occlusion anaesthetic in the young child. A YAG capsulotomy may
therapy required75,81,82. Difficulty in cooperation with be made electively after the wound has been closed at the
occlusion therapy is a major factor accounting for rela­ end of the primary procedure in some cases, particularly if
tively poor visual results in otherwise uncomplicated eyes. the previously operated eye showed a brisk posterior cap­
Surgical intervention may not be indicated if the parents sule opacification. The YAG laser may of course be used
are not committed to the visual rehabilitation of the eye as an outpatient procedure in the older child87•
and some unilateral partial cataracts may be managed by (B) Lensectomy-vitrectomy using a closed eye vitrec­
occlusion therapy of the contralateral eye alone. tomy system is the method of choice in children under 18
It is often difficult to determine the onset of a cataract months of age. The whole lens is removed including both
and occasionally an eye expected to be densely amblyopic the anterior and posterior capsule and a shallow anterior
will develop good vision after cataract extraction. This is vitrectomy is performed. This provides a clear visual axis
due to it being a progressive infantile cataract rather than for the crucial first 1 8 months thereby enabling easier ret­
one present from birth. inoscopy and reducing the risk of amblyopia secondary to
posterior capsule opacities. Secondary cataract formation
SURGERY
following lensectomy and anterior vitrectomy can occut8
Pre-surgical Assessment due to vigorous regrowth of residual peripheral cortex and
Surgery should only be performed after careful assess­ this is more common in microphthalmic eyes89• Some sur­
ment of the whole globe and in particular optic nerve and geons perform lensectomy through a pars plana/pars pli­
retinal function in all children with significant cataracts. cata approach rather than the limbal approach we favour.
Clinical tests such as fixation behaviour are helpful It is claimed that this results in easier removal of periph­
together with pupillary reflexes. An afferent pupil defect eral cortex, less iris manipulation and less damage to the
suggests a poorer long term prognosis due to optic nerve or corneal endothelium89• This approach does carry with it
retinal pathology. The posterior pole should be assessed the risk of entry site complications, particularly in small
by ophthalmoscopy and ultrasound performed if the catar­ eyes in which the pars plana is not fully developed90• There
act prevents an adequate view. Electroretinography, visual is a danger of disinsertion of the oral retina at the time of
evoked responses and forced choice preferential looking instrument insertion and as any pars plana entry site is
are also extremely helpful when deciding whether surgical equivalent to a penetrating injury of the posterior segment
intervention is warranted. there is inevitable incarceration of basal vitreous into the
Infants with visually significant bilateral cataracts wound. Thus there is a danger of post-operative tear for­
should undergo surgery at the earliest opportunity without mation at the posterior border of the vitreous base. The
jeopardising the child's general health and our policy is to effect of this may not become apparent for many years
patch the first operated eye until the second eye has had since there may be a substantial latent period before retinal
surgery, to prevent amblyopia in the second operated eye9• detachment occurs in aphakic children.
NEONATA L CATARA CTS 19 1

Limbal vitreo-Iensectomy cannot be assumed to be an with bilateral congenital cataracts were esotropic while
inherently safe technique from this point of view, as the the unilateral cases showed an equal distribution between
use of high suction forces and/or an inefficient vitreous esotropia and exotropia, This contrasts with children with
cutter may cause excessive vitreous base traction. It does acquired cataract in whom 6 1% developed strabismus, the
however avoid vitreous incarceration as long as the sur­ majority being exotropic. Lambert et al.9 reported that
geon is scrupulous in removing vitreous tracking to the approximately 2/3 of patients with bilateral congenital
corneoscleral wounds. The use of air injected into the cataracts developed strabismus but that in most cases the
anterior chamber keeps the vitreous away from the entry angle was small and squint surgery was not necessary.
sites and acetylcholine facilitates identification of a Surgical intervention may be necessary before contact
peaked pupil. lens fitting or glasses correction is successful as a marked
The limbal approach has other advantages in that the abnormality of eye position can lead to constant decentra­
surgeon can deal with congenital anomalies such as miotic tion of soft contact lenses and considerable prismatic and
pupils, iris anomalies and anterior hyperplastic primary spherical aberration in aphakic spectacle wear.
vitreous under direct vision91. A disadvantage of the len­ Retinal detachment is a well recognised and extremely
sectomy technique in general is the possible occurrence of serious post-operative complication. It may occur within
cystoid macular oedema92. Relatively recent studies how­ months of the initial surgery, presumably related to vit­
ever have shown that the incidence of this complication reous traction during the procedure, but most occur 20 to
appears to be low89,93. 30 years after surgery90. The incidence of this complica­
Instruments such as intraocular scissors, intraocular tion is difficult to determine because of this delay. Retinal
diathermy and intraocular forceps may be required to deal detachments in patients who have had infantile cataract
with congenital anomalies such as thick retrolenticular surgery are often difficult to repair due to difficulty visua­
membranes. Iridectomy in eyes with extensive posterior Iising the peripheral retina through miotic pupils, capsular
synechiae can be performed with the vitreous cutting remnants, decompensated corneas and the presence of
instrument. nystagmus. In a series from Moorfields Eye Hospital96 the
affected eyes were generally axially myopic and there was
OPERATIVE AND POST-OPERATIVE a very high frequency of bilateral retinal detachment in
COMPLICATIONS patients who had undergone bilateral cataract surgery. In
More modem microsurgical techniques have reduced two thirds of eyes vitrectomy techniques were required for
markedly the incidence of operative and post-operative adequate surgical management of the detachment.
complications in infantile cataract surgery. Bacterial endophthalmitis is a rare but potentially dev­
Vitreous wicks to the corneoscleral wound are a not astating complication. Good et al. reported three cases
uncommon problem after lensectomy91 and can be from two centres and each showed infection by gram posi­
avoided by carefully examining the pupil for peaking tive organisms97• All three had rapid onset of signs and
intraoperatively and the use of air and miochol. A vitreous symptoms of endophthalmitis. They point out the need for
wick places the eye at risk of infection and epithelial prompt aggressive attempts at diagnosis, and subsequent
downgrowth. treatment. A careful pre-operative evaluation for the signs
Aphakic glaucoma is less common with current sur­ and symptoms of upper respiratory tract infection is sug­
gical techniques but is still seen in approximately 6% of gested and that nasolacrimal obstruction is dealt with prior
patients94. The incidence is much increased in children to cataract surgery. Pre-operative antibiotic eye drops and
with congenital rubella, chronic uveitis, PHPV and micro­ subconjunctival antibiotics at the end of surgery, provid­
phthalmos94,23 but can occur in other children following ing gram positive and gram negative cover are recom­
congenital cataract surgery and is often related to inade­ mended. Simultaneous surgery for bilateral congenital
quate vitrectomy at the time of lensectomy. This then leads cataracts has been advocated98 in order to avoid a second
to vitreous prolapse into the anterior chamber, pupil block, general anaesthetic and allow earlier optical correction.
synechiae formation and iris bombe. The rare but real occurrence of endophthalmitis argues
Physical signs suggestive of glaucoma including photo­ against synchronous bilateral surgery as it would place the
phobia, corneal haziness, enlarged corneal diameters, child at risk, however slight, of bilateral intraocular infec­
shallowing of the anterior chamber and optic disc cupping tion and potential blindness.
should be sought. Any evidence of these warrants Chronic inflammation and corneal oedema may follow
measurement of the intraocular pressure, if necessary, surgery on children with congenital rubella syndrome99,
under anaesthetic. The subequent management of aphakic Topical steroids and mydriatics are useful for the inflam­
glaucoma is often difficult and may involve a further vit­ mation while the corneal oedema usually gradually clears
rectomy initially. Drainage surgery may subsequently be spontaneously.
required.
Strabismus is a problem for the majority of children POST-OPERATIVE MANAGEMENT
after surgery for congenital cataract: France and Frank's The infant aphakic eye must be accurately optically cor­
series95 showed that 86% of such children had developed rected and in our unit we fit most infants with contact
manifest strabismus following surgery. All their children lenses which we have found to be well tolerated and
192 I . C. LLOYD ET AL.

00
safe1 . Contact lenses are fitted within 1 week of surgery. normal life-span. As mentioned above, some children
In bilateral cases the eyes are operated on within 2 days of develop contact lens intolerance particularly in the second
each other, and the first eye is occluded until the second year of life. If the patient is bilaterally aphakic, spectacles
eye has been operated on to prevent any early visual may be well tolerated despite their poorer cosmetic
advantage being gained. In unilateral cases no occlusion is appearance and their inherent optical aberrations. Uni­
used until the contact lens has been fitted. Daily wear soft laterally aphakic children rarely tolerate spectacle correc­
contact lenses are used; initially high water content and tion because of image size disparity between the two eyes.
later in childhood more durable low water content contact Epikeratophakia is a technique in which a tissue lens
lenses. The power of the lens is decided by retinoscopy shaped from donor cornea is sewn into a trephined bed in
and an overcorrection of two to three dioptres is pre­ the aphakic child's cornea. This has been shown to correct
scribed. Contact lenses with powers between twenty and 73% of children undergoing such surgery to within three
0
thirty five dioptres are typically required during infancy. dioptres of emmetropia1 6 and appears to be a safe and
The parents are carefully instructed in lens insertion, reliable procedure. Its main indication is in the child with
removal and cleaning. The lenses require replacement unilateral cataract who is contact lens intolerant, but it has
regularly due to changes in refractive error and lens loss. been combined with cataract surgery in the older child
0
Parents are given spare lenses to avoid interruptions in with acquired or progressive catarad 7• Elsas reports
their use following a loss. They are instructed to remove however that in his series epikeratophakia did not facili­
the contact lenses and contact the department if their tate occlusion therapy for amblyopia as expected and that
child's eyes become red, photophobic or develop a visual results remain largely related to occlusion regimes
0
discharge. and cooperation 1 8
In a recent series from this hospital only twelve of OCCLUSION THERAPY AND AMBLYOPIA
eighty-three patients discontinued use of their contact MANAGEMENT
0
lenses in a three year period1 1. In two of the twelve there
Good results in children with bilateral cataracts have been
was hypoxic ulceration of the cornea whilst the remaining
possible for many years. Studies have shown that if early
ten had either an excessively high lens turnover, diffi­
surgery is performed and the acuity of the two eyes moni­
culties in handling the lenses or just preferred glasses. In
tored, then careful occlusion can help to overcome any
total forty three eyes had complications due to the use of
inter-ocular acuity difference as found by visual evoked
contact lenses including bacterial conjunctivitis, lens 0
potentials or preferential looking assessmene6,1 9 Occlu­
intolerance, corneal vasularisation, corneal oedema,
sion does not usually need to be very aggressive in the
hypoxic ulceration and punctate keratitis. All of these
bilateral aphake although there may be superimposed stra­
problems responded to treatment and only the two with
bismic amblyopia.
hypoxic ulceration had to cease contact lens wear. Gas
The situation is different in the unilateral aphake in
permeable contact lenses often provide a better optical
whom there is a great tendency for deprivational amblyo­
correction and are less frequently associated with micro­
pia in the aphakic eye. Several studies7s,78,81,82,84 have
bial keratitis, although they are more easily lost and more
shown that good visual results are now possible but that
difficult to fit.
aggressive occlusion of the phakic eye is necessary. Robb
Extended wear soft contact lenses may be worn for long
et aes found that if occlusion was performed for less than
periods of time which is helpful in preventing amblyopia
four hours of the waking day then there was greater than
in children at high risk but they are expensive and their use
one octave difference in visual acuity between the two
carries a higher incidence of microbial keratitis.
eyes as measured by preferential looking acuity cards.
Intra-ocular lenses are advocated by some surgeons
One octave difference in spatial frequency between the
particularly in the correction of children with unilateral
02 two eyes is a strong indication of amblyopia. Lewis et
congenital cataracts1 . Good visual acuities have been al.llo in reporting 17 cases of unilateral cataract found that
reported in some children. Problems such as severe secon­
only two were compliant with their regime of occlusion of
dary glaucoma and corneal decompensation occuring 6 to
0 the phakic eye which consisted of 50% of waking hours.
7 years after implantation have been reported1 3, and other
In the two who were compliant the aphakic eye acuity was
short term complications such as vigorous intraocular
better than 6/15 whilst in the others who were not com­
inflammation and hyphaema are not uncommon. There is
pliant the acuity varied from 6/60 to perception of light.
a high risk that secondary operations will be required. A
It would appear that full time occlusion of the phakic
case of bilateral blinding uveitis following a secondary
eye for the first week post-operatively followed by 50% of
lens implant for unilateral cataract has been seen at this
waking hours until age 6 to 7 is generally accepted as
hospital. Sympathetic ophthalmia may have played a role necessary for unilateral aphakes. This may be modified
0
in the pathogenesis of this uveitis1 4. ,
according to clinical findings or VEp76,81,111,112 or PL7S,82 111
The eye changes in its refractive indices markedly in the estimates ot acuity.
first year of life and therefore an intraocular lens could not
accurately correct an infant's refractive error long termlOS• VEP CHANGES IN UNILATERAL
We feel that intra-ocular lens implantation in infancy CATARACT
carries an unacceptable risk of long-term problems over a Occlusion therapy has been reported as significantly
NE ONATAL CATARACTS 193

Comparison of Normal Eyes of Unilateral The 18 patients were divided into the following groups for
Cataract Patients with Controls comparison:

Group 1: (n = 8) Cataract removed before 13 weeks of age

A. Pattern VEP Amplitude
Age at test: mean = 164 weeks, SD = 90 weeks

I
14 Group 2: (n = 6) Cataract removed after 9 months of age

!! 1 !
12
! Age at test: mean = 26 5 weeks, SD = 76 weeks
10
P100
Group 3: (n = 4) Cataract not removed because of late
Amplitude 8
(uV) presentation
6

4 !I Age at test: mean = 193 weeks, SD = 17 1 weeks

2 x 25' checks Figure 7 shows the mean pattern YEP amplitudes and
050' checks
0 latencies (25 and 50 minute checks) of the 'normal' eye of
Controls Group 1 Group 2 Group 3 the three groups. Results from a group of 12 normal con­
trols similar in age range to group 1 patients (mean 17 1
=

weeks, SD = 82 weeks) are also shown.

B. Pattern VEP Latency
Figure 7 shows that patients who had surgery before 13
weeks old (group 1) had significantly smaller YEP ampli­
125 tudes (p<0.05) from the phakic eye when compared with
normal controls. The mean amplitudes of the other two

tI
120
groups while smaller than the control group are not signifi­
115

i I
P100 cantly different.
Latency 110 Pattern YEP latencies were also measured from the
i
I
(mS) t I phakic eye but no significant differences were noted
105
between any of the groups and the controls.
100
x 25' checks The reduced VEPs in the phakic eye of group 1 patients
95 were not associated with any visual acuity changes.
0 50' checks
90 This preliminary study examined the clinical records of
Controls Group 1 Group 2 Group 3
patients retrospectively and it was not possible to establish
Fig. 7. Comparison of VEPs from normal eyes of unilateral the presence/absence of latent nystagmus in the non­
cataract patients with controls. Pattern YEP amplitude and affected eye of many of the patients at the time of testing.
latency (25' and 50' checks) recordedfrom the non-affected eye Pattern YEP amplitude for small check sizes are reduced
of children with congenital unilateral cataract andfrom a group by the presence of nystagmusl15. While a large refractive
of age matched controls. Error bars indicate 95% confidence I
error may also reduce VEPS 16, none of our patients had
interval of standard error of the mean.
significant refractive error at the time of YEP recording.
These results are from a preliminary study on a rela­
increasing YEP latency in the patched eye of unilateral
tively small number of congenital unilateral cataract
amblyopes with no associated change in visual acuityll3.
patients but raise some interesting questions. The average
Whilst these YEP changes were reported to have occurred
age of the children at YEP testing was over three years old
as a result of patching, Leguire et al.114 reported that the
and all patients who had surgery had undergone occlusion
contrast sensitivity function was abnormal in both eyes of
therapy. Patients who had undergone early surgery (group
children with unilateral amblyopia before occlusion ther­
1) had the smallest VEPs. These patients had undergone
apy had started. They suggested that the amblyopic eye
occlusion therapy for the longest duration and also during
may negatively affect the non-amblyopic eye at a cortical
the critical period of visual development. Does occlusion
level.
account for these YEP changes alternatively, are reduced
As part of a preliminary study in our department we
VEPs from the phakic eye associated with abnormal corti­
examined the YEP records of 18 patients born with a uni­
cal interaction as suggested by Leguire? Further record­
lateral cataract. One aspect of this study was to determine
ings from young children prior to any occlusion therapy
if YEP changes were evident in the unaffected eye of these
should provide more insight into the effects of monocular
children. Pattern VEPs provide information about the
III deprivation and occlusion.
quality of spatial vision and we routinely record pattern
VEPs for the clinical assessment of infants and children
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