Eur J Pediatr (2012) 171:625–630
DOI 10.1007/s00431-012-1700-1
REVIEW
Educational paper
Congenital and infantile cataract: aetiology and management
Wai H Chan & Susmito Biswas & Jane L Ashworth &
I. Christopher Lloyd
Received: 18 January 2012 / Accepted: 9 February 2012 / Published online: 1 March 2012
# Springer-Verlag 2012
Abstract Congenital cataract is the commonest worldwide
cause of lifelong visual loss in children. Although congen-
ital cataracts have a diverse aetiology, in many children, a
cause is not identified; however, autosomal dominant inher-
itance is commonly seen. Early diagnosis either on the post-
natal ward or in the community is important because appro-
priate intervention can result in good levels of visual func-
tion. However, visual outcome is largely dependent on the
timing of surgery when dense cataracts are present. Good
outcomes have been reported in children undergoing sur-
gery before 6 weeks of age in children with unilateral
cataract and before 10 weeks of age in bilateral cases.
Placement of an artificial intraocular lens implant after removal
of the cataract has become established practice in children over
2 years of age. There remains debate over the safety and
predictability of intraocular lens implantation in infants.
Despite early surgery and aggressive optical rehabilitation,
children may still develop deprivation amblyopia, nystagmus,
strabismus, and glaucoma. The diagnosis and management of
congenital cataracts has improved substantially over the past
30 years with a concurrent improvement in outcomes for
affected children. Many aspects of the pre-, intra-, and postop-
erative management of these patients continue to be refined,
highlighting the need for good quality data and prospective
collaborative studies in this field.
Keywords Congenital cataract . Lensectomy . Aphakia .
Pseudophakia
W. H. Chan : S. Biswas : J. L. Ashworth : I. C. Lloyd (*)
Manchester Academic Health Science Centre,
Manchester Royal Eye Hospital,
Central Manchester Foundation Trust,
The University of Manchester,
Oxford Road,
Manchester, M13 9WH, UK
e-mail: lloydic@yahoo.co.uk
Introduction
Congenital and infantile cataracts are an important cause of
lifelong visual impairment. The incidence in the UK is
approximately 2.5 per 10,000 by the age of 1 year, increas-
ing to 3.5 per 10,000 by age 15 [48]. It is estimated that
200,000 children worldwide are blind from cataract [23] and
thus improving outcomes for affected children is a priority
for the global vision 2020 initiative [66]. Visual function in
children may be impaired as a result of a dense central
opacity causing visual deprivation amblyopia (Fig. 1) whilst
others may develop anisometropia due to high degrees of
astigmatism (Fig. 2). Improved understanding of the neuro-
physiology of the visual system and the concepts of latent,
critical, and sensitive periods of visual development [18]
along with improved surgical techniques [54] and optical
and occlusive rehabilitation in the post-operative period [34]
has improved outcomes in these children.
The areas of research and debate in this field include:
1. Aetiology
2. The timing of surgery and its relationship to outcomes
3. The effect of visual deprivation on fixation stability and
nystagmus
4. The use of intraocular lenses in infants
5. Surgical techniques
6. Complications such as glaucoma, posterior capsule opa-
cification, and retinal detachment.
Aetiology
Congenital and infantile cataracts have a diverse aetiology.
A careful structured approach to the assessment of affected
children enables diagnosis of the aetiology [35]. The most
common cause is inheritance as an autosomal dominant trait
[50]. Other genetic abnormalities such as chromosomal tri-
somies (13, 18, and 21) deletions (5p, 18p, 18q) and
626
Fig. 1 An autosomal dominant sutural/nuclear congenital cataract in a
2-year-old boy occluding the visual axis
autosomal recessive inheritance [4, 13] may be causa-
tive [42]. There may be metabolic associations such as
diabetes, galactosaemia, hypoglycaemia, and galactoki-
nase deficiency [52, 53]. Congenital cataracts may also
be present as part of a broader spectrum of syndromic
conditions primarily affecting other organs like the kid-
ney (Lowe syndrome, Alport syndrome, and Hallerman–
Streiff–Francois syndrome) [21, 27, 55], the skeletal
system (Smith–Lemli–Opitz and Stickler) [16], the
CNS (Marinesco–Sjogren, Zellweger), the musculoskel-
etal system (myotonic dystrophy) [22], or the dermis
(Cockayne, incontinentia pigmenti, and ichthyosis)
[45]. TORCH infections, trauma, and radiation exposure
may predispose to cataract formation [15, 43, 51]. De-
spite this, most unilateral and around half of all bilateral
cases are idiopathic. Whether a cataract is visually sig-
nificant depends on its laterality, morphology, size, po-
sition, and density. Accurate pre-operative classification of
lens morphology can also be challenging and often requires
intra-operative 3D video evaluation [65]. Ultimately, the de-
cision to operate and timing of surgery will often depend on
clinical judgment as to whether there is likely to be significant
visual deprivation (Table 1).
Fig. 2 A pyramidal congenital lens opacity. This subtype of congenital
cataract is associated with a high degree of astigmatism
Eur J Pediatr (2012) 171:625–630
The timing of surgery, abnormal visual development,
and amblyopia
Prolonged visual deprivation produces irreversible loss
of vision. However, in the early neonatal period, the
immature visual system is still reliant on sub-cortical
pathways [19]. During this latent period, visual distur-
bance does not appear to impact on final visual out-
come. This latent period is 6 weeks for unilateral visual
deprivation. Thus, a unilateral birth-related macular hae-
morrhage resolving within 6 weeks does not lead to
permanent loss of vision [63]. Birch et al. [11] demon-
strated that unilateral cataract surgery performed within
this period produced excellent outcomes, whereas sur-
gery carried out after this had a poorer visual prognosis.
Lambert and Drack [32] demonstrated that the equiva-
lent latent period for bilateral visual deprivation may be
as long as 10 weeks, and this correlates well with
previous published studies of outcomes in infants with
bilateral congenital cataracts [25, 37, 49]. Jain et al.
[28] reported that visual acuity after surgery for bilateral
congenital cataracts appears to decline exponentially
with duration of visual deprivation although Birch et
al. [10] described a bilinear model with increased dep-
rivation amblyopia in bilateral cases operated after
14 weeks. The latent period for other visual modalities
such as ocular alignment and fixation stability may be
as short as 3 weeks [1]. The sensitive period of ocular
growth is the time frame during which the developing
visual system retains plasticity and can thus be influ-
enced before full visual maturation. This influence may
be positive where occlusion or optical correction can
improve vision, or negative, where there is an amblyo-
genic effect due to form deprivation or mal-alignment.
The sensitive period in humans is approximately 7 to
8 years. An infant with a unilateral congenital cataract
will need long-term “aggressive” occlusion therapy (6 to
8 h daily) if useful visual function is to be achieved in
the affected eye [11]. Children born with dense bilateral
cataracts typically exhibit strabismus, even after optimal
surgical management [1] [24] and are thus at risk of
strabismic amblyopia. Recent studies indicate that more
than 60% of children with unilateral congenital cataract,
who undergo optimal intervention, can achieve a useful
level of vision in the affected eye (VA better than 6/60)
if compliant with occlusion and optical correction [30,
31]. The better eye of children born with dense bilateral
cataracts has been shown to achieve a better outcome
than this where surgery is performed before 10 weeks
of age. Lambert and Drack [32] found that 88% of
infants who had their operation before 10 weeks
achieved 20/80 or better compared to those operated
on after 10 weeks, scoring 20/100 or worse.
Eur J Pediatr (2012) 171:625–630
Table 1 Definition of technical
terms. Aphakia
Anisometropia
Anisoeikonia
Capsulorexhis
Cycloplegia
Emmetropia
Hyaloid face
Hypermetropic
Hyphema
Intracameral
Myopia
Pseudophakia
Strabismus
Trabeculectomy
Vitrectomy
Effect of visual deprivation on development of fixation
stability and nystagmus
The duration of visual deprivation and timing of surgery for
infantile cataract are important in the development of fixa-
tion stability and nystagmus [12]. Although fixation has a
large impact on visual function, most published studies of
cataract surgery outcomes have focussed mainly on visual
acuity [12], and as such, the prevalence of nystagmus in
these cases remains unknown. Nystagmus amplitude, fre-
quency, waveform, and beat direction are also unknown
entities due to a lack of readily available expensive eye
movement recording systems. The impact of the severity
and duration of early onset visual deprivation on eye align-
ment and ocular stability was reported by Abadi et al. [1].
The authors concluded that major form deprivation, even
after early surgery, leads to nystagmus. In approximately
75% of children, this is manifest latent nystagmus (MLN).
Minor form deprivation appeared to have less of an effect on
ocular stability, although it was noted that large amplitude
saccadic intrusions affected most of the cohort. They con-
cluded that the latent period for fixation stability may be as
short as 3 weeks and that preoperative congenital nystagmus
(CN) can convert to the more benign MLN after successful
surgery.
The use of intraocular lenses (IOL) in infants
Controversy remains over the benefits of pseudophakia
(IOL implantation) (Fig. 3) in children when compared to
the use of aphakic contact lenses [9]. Aphakia following
lensectomy results in a high refractive error requiring con-
tact lens or thick spectacle lens correction. In unilateral
cases, this produces a high degree of both anisometropia
and anisoeikonia, which are strong amblyogenic stimuli
627
Without a lens (natural or artificial)
Difference in the refractive power of the right and left eye
Difference in magnification of the image produced by either eye
A circular opening created in the anterior capsule of the lens
Pharmacological relaxation of the ciliary muscle
Perfect focus—the focal point falls on the retina
Anterior aspect of the vitreous jelly, located just behind the lens
Long-sighted, the focal point falls in front of the retina
A collection of blood in the anterior chamber of the eye
Within the eye (usually describes an injection)
Short sightedness, focal point falls behind the retina
Natural lens replaced with an artificial implant
Misalignment of the two eyes
A controlled drainage fistula to allow fluid out of the eye
Surgical removal of vitreous jelly from the eye
without correction. Implantation of an IOL mimics the nat-
ural crystalline lens although an initial hypermetropic over-
correction (to allow for subsequent ocular growth) may
require contact lens wear for up to a year. The glasses
required for the correction of any eventual pseudophakic
residual refractive error are usually of a much lower dioptric
power, more cosmetically acceptable, and less cumbersome
than aphakic spectacles. In unilateral cataracts, visual results
now appear to be better following primary IOL implantation
compared with contact lens corrected aphakia [9], although
children undergoing primary IOL implantation seem to need
more secondary procedures than those left aphakic [7].
Recently, the infant aphakia treatment study published
results identifying lensectomy surgery in infants with pos-
terior involvement of the vitreous (persistent fetal vascula-
ture or persistent hyperplastic primary vitreous (PHPV))
treated with contact lens correction to be a risk factor for
the development of adverse events after surgery which
required further surgery, although visual acuity remained
similar at 1 year [41].
Fig. 3 The same eye as Fig. 1, 3 weeks after successful lensectomy,
posterior capsulotomy and intraocular lens implantation
628
Surgical techniques and IOL implantation
Our preference is for a superior 3.4 mm clear corneal inci-
sion (for lens insertion and creation of the capsulorexhis)
combined with temporal and nasal 20 G corneal incisions at
90° [56]. A continuous curvilinear capsulorexhis is per-
formed under viscoelastic with forceps although a push–pull
technique described by Nischal may be helpful in less
experienced hands [44]. The lens is aspirated using a
bimanual technique with a vitrectomy cutter and infusion/
manipulator. A foldable hydrophobic acrylic IOL is inserted
into the capsular bag. If the child is less than 4 years old, a
primary posterior capsulotomy and anterior vitrectomy is
performed. Intracameral heparin in the infusion or as a bolus
injection at the end of surgery may help to limit fibrinous
uveitis. An IOL may not be implantable for example in the
presence of PHPV, anterior segment dysgenesis, or glaucoma
when complications are more common and refractive
outcome unpredictable. High-powered IOLs required to
correct the microphthalmic eye (axial length less than
16 mm) are not readily available from most hospital
IOL banks and implantation into eyes with corneal
diameters of less than 10 mm are more likely to develop pupil
block glaucoma.
Biometry
Accurate biometry (axial length and keratometery measure-
ments) is essential to achieve the desired refractive outcome.
In older children, this can be carried out in the clinic pre-
operatively. In infants and younger children or those with
physical or intellectual impairment, it must be performed
under general anaesthesia. Axial length is measured using
A-scan ultrasound and corneal curvature by hand-held ker-
atometery. We use an SRK-T formula for IOL power calcu-
lation, but other formulas (SRK-II, Hoffer-Q, and Holladay)
have also given acceptable results in children [2]. However,
IOL power calculation formulas are less accurate in infants
less than 36 months old and in those with axial lengths less
than 20 mm [2] [57].
Rate of refractive growth in normal children versus
pseudophakia; the effect of pseudophakia on axial
elongation/ocular emmetropisation
Ocular growth in infancy is characterised by axial elonga-
tion complemented by corneal and lenticular flattening.
Most corneal flattening occurs in the first 3 months of life
while the majority of axial elongation occurs in the first
18 months resulting in a logarithmic rate of refractive
growth (RRG) [17, 60]. Although the rate of axial growth
Eur J Pediatr (2012) 171:625–630
is slower in pseudophakic compared to aphakic eyes, myo-
pic shift in the pseudophakic eye is greater due to the change
in the relative position of the IOL as the eye grows [38]. In
aphakic eyes, the mean quantity of myopic shift from age
3 months to 20 years has been shown to be 9.7D [39]. In our
personal series of 25 infants (33 eyes), the mean myopic
shift at 12 months was 4.83D and this increased to 5.3D in
infants implanted before 10 weeks of age [5]. In infants
under the age of 10 weeks at the time of surgery, our desired
refractive outcome is 8–9D of hypermetropia. This is
reduced to 4D at 12 months, 2D at 24 months, 1D at
36 months, and emmetropia or low myopia after the cessa-
tion of ocular growth. This is determined on a case-by-case
basis, depending on the refractive status of the fellow eye.
The ideal outcome in adulthood is emmetropia or low
myopia. Children with Down's syndrome may exhibit
abnormal ocular growth and go on to develop significantly
more myopia [5].
Complications
Although management of amblyopia (and residual refractive
error) is the key to a good outcome, complications may still
occur [54]. Early and late glaucoma following paediatric
cataract surgery is well documented and varies from
20.2% to 59% depending on series [14] [46] [64] and is
more common in children operated on before 12 months
[46] [64]. The treatment of glaucoma following surgery is
initially medical but surgery is often required. Trabeculec-
tomy with anti-metabolite agents like Mitomycin C has a
low success rate in this group of patients [36]. Cyclodes-
tructive treatment using a diode laser may also be an option
[6]. Greater long-term success in intraocular pressure con-
trol has been achieved with the implantation of drainage
tube devices such as the Ahmed Valve or Baerveldt implant
[59].
In children undergoing lens implantation, the develop-
ment of posterior capsule opacification (PCO) (Fig. 3) is the
Fig. 4 A unilateral congenital cataract associated with persistent fetal
vasculature (PHPV). Note the prominent iris vasculature and vascular-
ised retrolental plaque
Eur J Pediatr (2012) 171:625–630
most common reason for further surgical intervention [29].
Children who have an IOL implanted without a primary
capsulotomy have significantly more PCO than in those
with a capsulotomy and anterior vitrectomy [58, 61, 62].
In infants whose posterior capsule had been retained, a laser
Nd:YAG capsulotomy is associated with high rates of
recurrence of PCO across the anterior hyaloid face [26].
Inflammation following lensectomy is heightened in infant
eyes and can be further exacerbated by iris manipulation or
prolonged surgery. This can result in posterior synechiae
formation risking seclusio pupillae, iris bombe, and subse-
quent secondary angle closure glaucoma. Prevention of this
is achieved with intensive topical steroids tapered over
several weeks along with regular cycloplegic drops. Intra-
cameral, peri-orbital depot and systemic steroids [33] and
heparin may be used [8]. Despite these measures, some
infants still develop a fibrinous uveitis unresponsive to
intensive steroids. Tissue plasminogen activator has been
advocated when fibrinous membranes are a significant prob-
lem if given within 3 weeks of their formation although if
administered too early (within 3 days) may provoke a
hyphaema [40]. Retinal detachment is a rare sight threaten-
ing complication. Eckstein et al. [20] noted an incidence of
1.8% (2 eyes) in a cohort of 65 children undergoing lensec-
tomy surgery. Retinal detachment following lensectomy
without implantation occurred in 3.2% of cases in a study
of 1,017 eyes of 579 patients and was associated with a less
hypermetropic refractive error [47]. The incidence may be
increased in patients with PHPV (Fig. 4), where traction on
the retina may be exacerbated by surgical disturbance of an
abnormally thick anterior vitreous face [3].
Conclusions
Future improvements in the management of children and
infants with cataract are likely to continue to be driven by
clinical research. Carefully planned multi-centre prospective
studies will lead to further clarification of the latent periods for
different visual modalities and an improved understanding of
the neurophysiological effects of visual deprivation. Capsule
management following IOL implantation remains a major
issue and may change in the near future. The prevention of
PCO and cellular proliferation may be achieved in the future
by the use of devices to specifically target lens epithelial cells
at the time of surgery. In summary, childhood cataract remains
an important but treatable cause of potentially lifelong visual
handicap. Timely referral to a specialist centre with a paediat-
ric ophthalmic team sub-specialising in this field is required to
prevent visual deprivation amblyopia.
Conflicts of interest The authors have no financial interests to declare.
629
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