(2001) 239:649655
C L I N I C A L I N V E S T I G AT I O N
DOI 10.1007/s004170100315
Petra Meier
Ina Sterker
Peter Wiedemann
Introduction
A cataract affecting vision in babies and infants is always associated with development of amblyopia, so that
if possible a lens operation must be performed without
delay in these eyes. However, implantation of an intraocular lens (IOL) in babies and infants is controversial owing to the development-related changes in refraction of
the childs eye. Implantation of an IOL in children less
than 1 year of age is possible [13], but many surgeons
are reluctant to place an IOL in an eye of a child with
monocular cataract who is younger than 1 year [3].
Many surgeons implant an IOL only after the end of the
second year of life [9, 19, 38]. Now a multicentre clinical trial, the Infant Aphakia Treatment Study, is being or-
habilitate vision. All patients received intensive orthoptic and pleoptic treatment. Results: This surgical
technique provided in all eyes a clear
visual axis. During follow-up of 3
months to 4.5 years, secondary cataract developed in five eyes. Retinal
detachment, glaucoma and endophthalmitis did not occur. One patient
developed contact lens intolerance
and a secondary intraocular lens
was placed in the ciliary sulcus.
Discussion: Lentectomy via a pars
plana or pars plicata approach is a
suitable and safe method for treating
cataract in children. Our chosen
method of lentectomy is an alternative to early implantation of an intraocular lens. It is possible to perform
uncomplicated secondary implantation of an intraocular lens in the ciliary sulcus.
ganised in the United States to critically compare treatment with IOL versus contact lens correction for infantile aphakia [26].
In principle, in the paediatric population in whom
placement of an intraocular lens at the time of cataract
removal is not a consideration, an approach close to the
limbus or a pars plana or pars plicata approach can be
chosen for the operation. If the lens capsule is not removed in children, however, there is an extremely high
probability that secondary cataract will develop. This
can be prevented by complete lentectomy, but the secondary implantation then gives rise to problems. In recent years, surgical techniques have therefore been developed which enable lentectomy that is as complete as
possible, leaving behind a peripheral capsular ring [4,
650
Fig. 2 After aspiration of the lens, anterior and posterior capsulotomy, the irrigation cannula of the bimanual aspiration and irrigation system and the cutter are visible at the level of the pupil
Fig. 1 Histological section of a a mature neonate and b a 12-monthold child. The pars plana of a newborn baby is very short (distance
between arrows) and develops quickly within the first year of life (hematoxylin-eosin; original magnification 32)
Distance
<3 months
36 months
>6 months
1.5 mm
2.0 mm
2.5 mm
scleral incision, the lens capsule was opened via the equator and
the lancet was advanced until the tip was visible at the level of the
pupil. A cannula connected to an infusion via a handpiece as well
as a cutter was then pushed forward into the lens nucleus. The last
eight eyes were operated on using the irrigation cannula of the bimanual aspiration and irrigation system (Storz). After removing
the nucleus of the lens, all the cortical parts were aspirated. Finally, the posterior and anterior capsule was cut out with a cutter
(Fig. 2). The capsulotomy had a diameter of about 3.54 mm. Vitrectomy of the anterior one third of the vitreous was then performed. After careful removal of prolapsed vitreous from the region of the scleral incisions, the sclerotomies were closed with
Vicryl 7/0 and the conjunctiva with Vicryl 8/0; the knots were
countersunk.
Immediately after the operation, a contact lens was inserted in
accordance with the corneal radii determined preoperatively with
the hand keratometer. Blue light was used to check whether the
contact lens was properly located. All children were provided with
contact lenses (WUK-Vision). In preverbal children, visual acuity
was assessed by teller acuity cards. Once the child was capable,
visual acuity was assessed by tumbling Es or Snellen letters.
The contact lenses were adapted to close vision and checked every
6 weeks. In children operated on unilaterally, the partner eye was
occluded for a time which comprised, on average, half of the waking period. After bilateral lentectomy, there was no occlusion
treatment when there was fixation on both sides. If one eye took
the lead, these children also received occlusion treatment. Progressive spectacles were fitted from the 3rd year of life.
651
20
13:7
30
Age at operation
Mean
Range
Median
Nystagmus
Strabismus
13 months
6 weeks to 36 months
21 months
3
8
Table 3 Aetiology
Aetiology
Number of patients
Idiopathic
Hereditary
12
2
1
1
Time at onset
Therapy
after lentectomy
9 weeks
3 months
8 months
8 months
19 months
Contact lens intolerance 3 years
(one eye)
Discission
Discission
Discission
Discission
Discission
Implantation of
posterior chamber lens
in ciliary sulcus
Results
The patients clinical data are summarised in Tables 2
and 3. For the eyes included in this study, a follow-up of
3 months to 4.5 years (mean 31 months) was available.
In all eyes the surgical technique provided a clear visual
axis postoperatively.
At times between 3 months and 4.5 years, secondary
cataract was found to have developed in five eyes; in
each case it was successfully managed by reoperation
(Table 4). Retinal detachment, glaucoma and endophthalmitis did not occur. A 3-year-old child developed
contact lens intolerance and a posterior chamber lens
was placed in the ciliary sulcus (Fig. 3). Strabismus was
diagnosed in eight patients (six children with unilateral
cataract; two with bilateral cataract). Nystagmus was ob-
Fig. 4 Last corrected visual acuity in correlation to age at unilateral cataract operation (Fix = fixation; nFix = no fixation, o = mentally retarded child)
served in three patients with bilateral cataract. The bilateral lens operation was performed at the age of 8 weeks,
6 months and 3 years. Whereas the nystagmus did not regress at a postoperative visual acuity of 0.16 and 0.2
(preoperative visual acuity of R=L 0.1) in the 3-year-old
child, there was a marked decrease of the nystagmus in
the two babies.
Figure 4 shows the best corrected visual acuity in
eyes that underwent combined aspiration of the lens, anterior and posterior capsulotomy and anterior vitrectomy
for treatment of unilateral congenital cataract, and figure 5
shows the best corrected postoperative visual acuity after
operation of bilateral congenital cataract. One child with
unilateral cataract and two of the children with bilateral
652
Discussion
Pseudophakia in children is predicted to result in a large
amount of myopic shift, particularly in very young children [29]. Children normally have a small amount of
myopic shift as they grow, despite the large increase in
axial length (from 16.8 mm at birth to 23.6 mm in adult
life). This is because of the correspondingly large decrease in power of natural lens during this time, i.e. the
natural lens power declines from +34.4 diopters to +18.8
diopters [16]. Therefore, because of the constant power
of an IOL, a child with pseudophakia might be expected
to experience a large myopic shift as the eye grows.
Children who underwent surgery in the first 2 years of
life had a significantly greater myopic shift than older
children and a large variance in this shift [29]. Eye
growth and refractive change are most rapid during the
first 2 years of life. It would therefore follow that patients who undergo surgery at a very young age have the
largest changes in axial length and refraction [13, 28,
34]. The difficulty in predicting refraction is the main
disadvantage of IOL implantation. If an IOL calculated
for emmetropia is implanted in the first few years of life,
myopia of the eye will result after only a few months.
The use of a hyperopic target refraction for an IOL is associated with the risks of amblyopia immediately post-
653
months of age. In summary, the 23 monocularly pseudophakic infants with 2 years and more follow-up whose
cases had been reported in the literature had a mean visual acuity of 20/60, i.e. 0.3 (minimum 20/200 to maximum 20/30). These data show a better visual outcome
for children with IOL correction than for contact lens
correction. Now the Infant Aphakia Treatment Study is
being organised to critically compare a treatment with
IOL versus contact lens correction for infantile aphakia
[26]. The results of this study will help us to evolve
guidelines for the use of contact lenses or IOL in children for correction of aphakia.
If there is bilateral cataract of the same severity with a
good view into the fundus, one can wait before removing
the lens, since there is no acute danger that severe deprivation amblyopia will develop. If the lens opacities are
unequal, the eye with the more pronounced cataract
should be operated on first, since according to Crawford
[10] the eye with the greater lens opacity achieves better
vision postoperatively than the partner eye.
If bilateral cataract surgery is indicated, an interval of
27 days between the operations is recommended [8,
35]. Operating on both eyes in a single session has the
advantage of an optimal rehabilitation of visual acuity
with less stress from the anaesthesia and lower costs.
However, it is rejected by most surgeons because of the
danger of bilateral endophthalmitis. Some authors recommend bilateral occlusion to avoid deprivation between the operations. This procedure is not followed
when the operation is carried out within a few days [35],
and we also did not perform bilateral occlusion since the
operation on the second eye always took place within
35 days.
If the cataract operation is carried out when deprivation
amblyopia with nystagmus is already present, the prognosis with regard to visual acuity is relatively poor. However, an increase in visual acuity to 0.4 and better was occasionally observed after cataract operations in manifest
nystagmus [8]. Three children in our patient series had already developed nystagmus. The bilateral lens operation
was performed at the age of 8 weeks, 6 months and 3
years respectively to deal with a dense cataract in both
eyes. Whereas the nystagmus did not regress at a postoperative visual acuity of 0.16 and 0.2 (preoperative visual
acuity of R=L 0.1) in the 3-year-old child, there was a
marked decrease in the nystagmus in the two babies.
On principle an approach close to the limbus or a pars
plana or pars plicata approach can be chosen for the cataract operation in children. In our experience, after making a tunnel incision near to the limbus and after setting
up paracenteses it is often necessary to apply a suture to
these incisions owing to the relatively high content of
elastic fibres in childrens eyes, so that a contact lens
cannot be fitted immediately after the operation. It also
appears to us that removal of the anterior one third of the
vitreous via a pars plana or pars plicata approach is safer
654
References
1. Aiello AL, Tran VT, Rao NA (1992)
Postnatal development of the ciliary
body and pars plana. A morphometric
study in childhood. Arch Ophthalmol
110:802805
2. Asrani SG, Wilensky JT (1995) Glaucoma after congenital cataract surgery.
Ophthalmology 102:863867
3. Biglan AW, Cheng KP, Davis JS,
Gerontis CC (1996) Results following
secondary intraocular lens implantation
in children. Trans Am Ophthalmol Soc
94:353379
4. Birch EE, Stager DR (1988) Prevalence of good visual acuity following
surgery for congenital unilateral cataract. Arch Ophthalmol 106:4042
5. Birch EE, Stager DR (1996) The critical period for surgical treatment of
dense congenital unilateral cataract.
Invest Ophthalmol Vis Sci 37:1532
1538
6. Birch EE, Stager D, Leffler J, Weakley
D (1998) Early treatment of congenital
unilateral cataract minimizes uneqal
competition. Invest Ophthalmol Vis Sci
39:15601566
7. Bonomo PP (1989) Pars plana and ora
serrata anatomotopographic study of
fetal eyes. Acta Ophthal 67:145150
8. Bradfort G, Keech R, Scott W (1994)
Factors affecting visual outcome after
surgery for bilateral congenital cataracts. Am J Ophthalmol 117:5864
9. Brady K, Atkinson CS, Kilty LA, Hiles
DA (1995) Cataract surgery and intraocular lens implantation in children.
Am J Ophthalmol 120:1964
10. Crawford JS (1977) Conservative management of cataracts. Int Ophthalmol
Clin 17:3135
655
Reproduced with permission of the copyright owner. Further reproduction prohibited without permission.