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DOI 10.1007/s00431-012-1700-1
REVIEW
Educational paper
Congenital and infantile cataract: aetiology and management
Received: 18 January 2012 / Accepted: 9 February 2012 / Published online: 1 March 2012
# Springer-Verlag 2012
Effect of visual deprivation on development of fixation without correction. Implantation of an IOL mimics the nat-
stability and nystagmus ural crystalline lens although an initial hypermetropic over-
correction (to allow for subsequent ocular growth) may
The duration of visual deprivation and timing of surgery for require contact lens wear for up to a year. The glasses
infantile cataract are important in the development of fixa- required for the correction of any eventual pseudophakic
tion stability and nystagmus [12]. Although fixation has a residual refractive error are usually of a much lower dioptric
large impact on visual function, most published studies of power, more cosmetically acceptable, and less cumbersome
cataract surgery outcomes have focussed mainly on visual than aphakic spectacles. In unilateral cataracts, visual results
acuity [12], and as such, the prevalence of nystagmus in now appear to be better following primary IOL implantation
these cases remains unknown. Nystagmus amplitude, fre- compared with contact lens corrected aphakia [9], although
quency, waveform, and beat direction are also unknown children undergoing primary IOL implantation seem to need
entities due to a lack of readily available expensive eye more secondary procedures than those left aphakic [7].
movement recording systems. The impact of the severity Recently, the infant aphakia treatment study published
and duration of early onset visual deprivation on eye align- results identifying lensectomy surgery in infants with pos-
ment and ocular stability was reported by Abadi et al. [1]. terior involvement of the vitreous (persistent fetal vascula-
The authors concluded that major form deprivation, even ture or persistent hyperplastic primary vitreous (PHPV))
after early surgery, leads to nystagmus. In approximately treated with contact lens correction to be a risk factor for
75% of children, this is manifest latent nystagmus (MLN). the development of adverse events after surgery which
Minor form deprivation appeared to have less of an effect on required further surgery, although visual acuity remained
ocular stability, although it was noted that large amplitude similar at 1 year [41].
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.
Surgical techniques and IOL implantation is slower in pseudophakic compared to aphakic eyes, myo-
pic shift in the pseudophakic eye is greater due to the change
Our preference is for a superior 3.4 mm clear corneal inci- in the relative position of the IOL as the eye grows [38]. In
sion (for lens insertion and creation of the capsulorexhis) aphakic eyes, the mean quantity of myopic shift from age
combined with temporal and nasal 20 G corneal incisions at 3 months to 20 years has been shown to be 9.7D [39]. In our
90° [56]. A continuous curvilinear capsulorexhis is per- personal series of 25 infants (33 eyes), the mean myopic
formed under viscoelastic with forceps although a push–pull shift at 12 months was 4.83D and this increased to 5.3D in
technique described by Nischal may be helpful in less infants implanted before 10 weeks of age [5]. In infants
experienced hands [44]. The lens is aspirated using a under the age of 10 weeks at the time of surgery, our desired
bimanual technique with a vitrectomy cutter and infusion/ refractive outcome is 8–9D of hypermetropia. This is
manipulator. A foldable hydrophobic acrylic IOL is inserted reduced to 4D at 12 months, 2D at 24 months, 1D at
into the capsular bag. If the child is less than 4 years old, a 36 months, and emmetropia or low myopia after the cessa-
primary posterior capsulotomy and anterior vitrectomy is tion of ocular growth. This is determined on a case-by-case
performed. Intracameral heparin in the infusion or as a bolus basis, depending on the refractive status of the fellow eye.
injection at the end of surgery may help to limit fibrinous The ideal outcome in adulthood is emmetropia or low
uveitis. An IOL may not be implantable for example in the myopia. Children with Down's syndrome may exhibit
presence of PHPV, anterior segment dysgenesis, or glaucoma abnormal ocular growth and go on to develop significantly
when complications are more common and refractive more myopia [5].
outcome unpredictable. High-powered IOLs required to
correct the microphthalmic eye (axial length less than Complications
16 mm) are not readily available from most hospital
IOL banks and implantation into eyes with corneal Although management of amblyopia (and residual refractive
diameters of less than 10 mm are more likely to develop pupil error) is the key to a good outcome, complications may still
block glaucoma. 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
Biometry more common in children operated on before 12 months
[46] [64]. The treatment of glaucoma following surgery is
Accurate biometry (axial length and keratometery measure- initially medical but surgery is often required. Trabeculec-
ments) is essential to achieve the desired refractive outcome. tomy with anti-metabolite agents like Mitomycin C has a
In older children, this can be carried out in the clinic pre- low success rate in this group of patients [36]. Cyclodes-
operatively. In infants and younger children or those with tructive treatment using a diode laser may also be an option
physical or intellectual impairment, it must be performed [6]. Greater long-term success in intraocular pressure con-
under general anaesthesia. Axial length is measured using trol has been achieved with the implantation of drainage
A-scan ultrasound and corneal curvature by hand-held ker- tube devices such as the Ahmed Valve or Baerveldt implant
atometery. We use an SRK-T formula for IOL power calcu- [59].
lation, but other formulas (SRK-II, Hoffer-Q, and Holladay) In children undergoing lens implantation, the develop-
have also given acceptable results in children [2]. However, ment of posterior capsule opacification (PCO) (Fig. 3) is the
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].
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