RESULT S OF INTRAOCULAR LENS IMPLANTATION IN

PAEDIATRIC APHAKIA

R. H. C. MARKHAM, P. A. BLOOM, A. CHANDNA, E. H. NEWCOMB

Bristol

SUMMARY

of residual image size difference between the two eyes

Intraocular lenses were implanted in 16 eyes of 13

even with contact lenses, possibly contributing to image

patients with congenital cataract, and visual progress was

plotted using a preferential-looking techniq ue. Initial

suppression in the aphakic eye between periods of patch

ing,14 and also the suspicion that repeated attention to the

surgery was by lens aspiration with preservation of the

contact lens makes patching more difficult to administer.

posterior capsule, and subsequent posterior capsulotomy

Epikeratophakia may offer another form of optical cor

rection to the older aphakic child,IS but at present is not

without

anterior

vitrectomy.

Poly-HEMA

posterior

chamber lenses were used, usually as a primary pro

widely available, and is expensive. In addition, the pro

cedure but in four cases as a secondary procedure after

cedure may not allow a clear visual axis early enough for

contact lens failure.

infants with congenital cataract, due to corneal haze which

No serious

complications

were

encountered. Most ey es achieved a very significant visual

improvement, and none were worse than preoperatively.
Residual refractive error was highly unpredictable, but
did not exceed

dioptres. The importance of rigorous

occlusion therapy is stressed. With close follow-up, this

procedure offers an effective and safe method for the cor
rection of unilateral paediatric aphakia, and, in selected
cases only, for bilateral aphakia.

:;

This is not

because cataract aspiration is difficult-modern instru

mentation has made this relatively straightforward-but
because of the problems of treatment of the accompanying
dense amblyopia. Where significant unilateral cataract is
present from birth, clarity of the media and correction of
the induced high hypermetropia must be established at a
very early age,6 and it is the latter problem that is so diffi
cult to overcome.
Aphakic spectacles might be adequate while the good
eye is being occluded, but are rarely satisfactory, because
of image size difference, when the patch is off, allowing
any improvement in amblyopia to slip back.7
Contact lenses have been the standard method of cor
rection, but while good results have been reported by
I
some,S I most have found the level of vision achieved to
be a poor reward for all the time, effort, expense. and
trauma of repeated contact lens fittings affecting patient,
parent, and clinic staff alike.

12.13

There is also the problem

From: Bristol Eye Hospital, Lower Maudlin Street, Bristol BS I 2LX.

Correspondence to: Mr R. H. C. Markham, Bristol Eye Hospital,
Lower Maudlin Street. Bristol BS I 2LX.

Eye

(1992) 6,493-498

Intraocular lens implantation avoids many of these dif

ficulties, while potentially adding some of its own.7.12.16-20
The change in refraction seen over the first two years or so
of a child's life-usually a loss of several dioptres of
hypermetropia-have suggested to some that an intra
ocular lens might need replacement at least once in a life
time to keep up with this change; full correction from the

Unilateral congenital cataract has long been regarded as a

condition with a poor visual prognosis. I

is often slow to clear.

start would lead to marked overcorrection later.8 Also,

although there is now forty years' experience with PMMA
as an intraocular material, this is perhaps only half a
child's expected lifetime, and PMMA itself is probably
not the most suitable lens material for an infant's eye,
because a rigid lens may be more difficult to introduce and
could damage corneal endothelial cells by direct contact.
There

is

even

shorter

experience

with

other

lens

materials.21
All methods of correction of aphakia have the disadvan
tage that whereas there may be some depth of focus, they
cannot compete with the large focusing range of the good
eye.
Despite these problems, we felt that the theoretical
advantages of intraocular lens implantation were very
great, and the theoretical disadvantages could be over
come. The traditionally poor visual prognosis in unilateral
congenital cataract calls for new approaches in this impor
tant cause of childhood ocular morbidity. If safety of
implantation can be shown in this group, the technique can
be extended to bilateral cataracts which traditionally have
a better visual prognosis.22
Experience with the later development of retinal

R. H. C. MARKHAMET AL.

494
detachment after adult intracapsular cataract extraction,

intraocular lens implantation in one eye and a secondary

extracapsular cataract extraction and capsulotomy in the

intraocular lens implant in the other eye after contact lens

absence of an intraocular lens, and after older methods of

failure (eyes i, j), and one, with Down's Syndrome, had a

congenital cataract removal,23-25 made us think that there

primary intraocular lens implant in one eye and elective

might be some merit in preserving the integrity of the pos

Use of a contact lens in the other (eye h).

Thus, in total, there were sixteen intraocular lens

terior lens capsule and anterior vitreous. This view is rein

forced by a reported incidence of aphakic cystoid macular

implants in thirteen patients (Table I).

oedema after lensectomy and anterior vitrectomy for con

Before intraocular lens implantation in any eye, the

genital cataract.26 A posterior capsulotomy should perhaps

possible treatment options and the investigational nature

only be carried out if, at the end of the operation for lens

of the procedure with its advantages and disadvantages

aspiration and implantation, central posterior capsular

were fully discussed with the parents.

Apart from cataract, convergent squint was present in

opacities could not be polished away, or if the capsule

one child preoperatively (eye k). There was no other

thickened up postoperatively.
The utilisation of forced choice preferential looking
(FCPL) tehniques using Teller Acuity Cards in our unit
has proved a powerful and accurate tool both in the pre
operative assessment of visual acuity in infants with con
genital cataract and in monitoring their subsequent
progress.27,28
We therefore report the results of a method of intra
ocular lens implantation in a group of children with con
genital cataract, unilateral in all but four subjects, and their
subsequent progress closely followed by FCPL visual
assessment. We used lens aspiration without primary cap
sulotomy in most cases, and poly-BEMA intraocular
lenses in all but one case. All are being kept under long
term surveillance.

ocular abnormality detected in any case. Aetiology was

generally unknown, except that one patient with bilateral
cataracts had a family history of dominantly inherited cat
aract (eyes f, g), one child suffered from Alagille's Syn
drome (eye k), and one from Down's Syndrome (eye h)
(Table I).
Preoperatively, an attempt was made to measure visual
acuity formally in all cases. According to age, methods
used were Forced Choice Preferential Looking (FCPL)
using Teller Acuity Cards, Kay Pictures, Sheridan-Gar
diner, or Snellen.
Age at original surgery varied from three to 57 months
for primary intraocular lens implants (eyes a, b, c, e, f, g, h,
i), and from three to 88 months for secondary lens
implants (eyes d, j, 1, m). One child listed as a secondary

MATERIALS AND METHODS

Six patients with unilateral congenital cataract underwent
lens aspiration and primary intraocular lens implantation
(eyes a, b, c, e, k, n). Two additional patients with uni
lateral congenital cataract originally undergoing lens
aspiration alone, had secondary intraocular lens implants

implant had spontaneous reabsorption of dense cataract

and therefore needed no primary operation (eye d)
(Table I).
Surgery consisted of lens aspiration using a vitreous
suction cutter through a limbal 20 gauge entry site and a
small anterior lens capsular opening. A second entry site,

after contact lens failure (eyes 1, m). In one other patient,

1800 opposite, was occupied by a 20 gauge cannula infu

spontaneous reabsorption of a unilateral cataract occurred

sing Balanced Salt Solution (Alcon). The suction cutter

and was followed by a secondary intraocular lens implant

and infusion could be swapped round at will to gain easy

in this eye (eye d).

access to all parts of the capsular bag. Most of the work

Four further patients had bilateral congenital cataracts.

was carried out using aspiration alone.

Of these, two had primary intraocular lens implantation in

At the end of this stage, the anterior capsular opening

both eyes from the start (eyes f, g, 0, p), one had primary

was enlarged using the cutting facility of the suction cutter

Table I.

Details of aphakic children treated by IOL implantation, (Note that subject indexes f &g, i &j and 0 &p refer to two eyes of the same patient)
IOL

Cataract
Subject Age (months)
at aspiration
index
a
b
c
d
e
f
g
h
j
k

I
m
n
0

51
19
0
88

40
7
7

Unilat.

Primary

*
*

3
3

Secondary

*
*

Primary
*

42
41
16
4

57
52

Bilat.

Post. capsulotomy

*
*

* *

*
*
*

Complications/notes

Secondary

* *
*

*
*

Uveitis
PMMA lens
Spontaneous reabsorption of cataract
Dominant inheritance
Down's syndrome (CL only in RE, IOL in LE)
Minor IOL decentration
Alagille's syndrome, convergent squint
Uveitis
Secondary capsulotomy after aspiration, before IOL

PAEDIATRIC PSEUDOPHAKIA

495

instrument. The posterior lens capsule and anterior vit

rapidly to conventional treatment in two eyes (eyes a, m).

reous were preserved, save in three cases where a primary

Minor decentration occurred in one lens but is not con

capsulotomy was carried out, without anterior vitrectomy,

sidered visually significant (eye j) (Table I).

after lens implantation. In two children this was per

Thickening of the posterior lens capsule occurred

formed via a separate pars plana sclerotomy (eyes a, I),

between one month and two years in nine of the thirteen

and in the other child, through the cornea and behind the

eyes that had not had a primary posterior capsulotomy. In

only PMMA lens that was used in the study (eye b).

infants, this was often indicated by a drop in acuity

nylon

measured by FCPL of more than half an octave, that is,

suture, leaving the infusion in situ, and a shelving 7 mm

more than one Teller Acuity Card (approximately equiva

One corneal entry site was then sealed with a 1%

superior corneal limbal incision made to introduce the

lent to more than one line of Snellen acuity). However,

intraocular lens. We used Alcon Iogel poly-HEMA pos

four eyes have not developed posterior capsular thick

terior chamber intraocular lenses in all except one case,

ening to date (eyes c, d, j, 0). Two eyes required one repeat

mostly 1 1.3 mm diameter but one 10.3 mm diameter (eye

posterior capsulotomy (eyes e, k); all had been secondary

n). The one exception had a 14 mm diameter Sinskey pat

capsulotomies the first time round, and possibly inade

tern PMMA posterior chamber lens with polypropylene

quate in size from the beginning (Table I).

A variety of methods was used to assess visual acuity

loops made by Allergan Medical Optics (eye b).

preoperatively according to age, as follows:

At the end of the operation, subconjunctival gentamicin

.

(0.4 mg/kg body weight) and a depot preparation of

methylprednisolone (Depomedrone, Upjohn, 0.4 mg/kg
body weight) was given. Postoperatively, parents were

FCPL

Eyes b, c, f, g, i, j, n, 0, p

Kay Pictures

Eyes i, j, 0

Sheridan-Gardiner

Eye e

Snellen

Eye d

instructed to give prednisolone acetate 1 % (Pred Forte,

Allergen), and fucidic acid 1 % slow release eye drops

In five children no preoperative acuity could be

(Fucithalmic, Leo) twice a day, for approximately three

recorded, three of these associated with total cataract (eyes

weeks until the eye settled. The pupils were not kept

a, k, I, m, n). Finally, no attempt was made to formally test

dilated.

the acuity of one premature infant with Down's Syndrome

Follow-up was carried out at weekly, then monthly, and

and bilateral dense cataract (eye h). In these six children,

subsequently greater intervals depending on progress and

the denseness of the cataracts made it reasonable to

age of child, and an attempt was made to measure visual

assume that the eyes were effectively blind, and that in the

acuity assessed on each occasion by the most appropriate

five eyes where testing produced no result, this was

method for the age of the child (FCPL, Kay Pictures, Sher

because of blindness rather than technical problems.

idan-Gardiner, Snellen), but always including FCPL.

By contrast, a postoperative FCPL acuity was recorded

Secondary posterior capsulotomy, where required, was

on every child at some stage (this may partly have

carried out via a pars plana incision using a vitreous suc

reflected our increasing skills with this technique). Where

tion cutter, and with an anterior chamber infusion of

possible without loss of accuracy, all acuity measurements

Balanced Salt Solution via the cornea, as in the primary

were converted to Snellen equivalents for ease of compari

capsulotomies. No attempt was made to carry out an

son. It has to be appreciated that these can only be approxi

anterior vitrectomy.

mate conversions. In cases where both FCPL and Snellen

RESULTS

acuities had been recorded at the same time, there was

usually remarkable correspondence between the two.

There were no serious intraoperative or postoperative

Preoperative, best postoperative, and latest recorded

complications. Postoperative fibrinous uveitis responded

Table II.

Visual acuity before and after TOL implantation for paediatric aphakia
Snellen equivalent visual acuity (6/...)

Subject
index
a
b
c
d
e
f
g
h

k
I
m
n
o
p

Unilateral
cataract
*
*

*
*
*

Age (months)
at aspiration

Pre-op

Best post-op

Follow-up
(months)

180
28
138
36
24
38
75
75
60

15
31
3
6
20
7
7
6
21

113

209

22
18

180
120
563
18
36

38
36
3
5
10

180
28
138
36
18
38
75
75
28

51
19
10
88
40
7
7
3
42
41

Not recordable
419
563
90
60
209
419
Not recordable
120

16
4
3
3
57
52

No red reflex
No red reflex
No red reflex
Not recordable
60
60

56
28
563
18
36

120

Latest post-op

R. H. C. MARKHAM ET AL.

496
Table

III.

Additional

procedures

after

IOL

implantation

for

paediatric aphakia

implantation for paediatric aphakia

Secondary

EUN

Subject
index

post.

Removal Freeing'
of
of

refraction capsulotomy
*

a
b
d

sutures synechiae

k
m

23.5

+6.00

24.0

-4.00

25.0

+6.00

25.0

+0.50
+1.00

22,5

I'

23.0

Plano

23.0

+0.75

* *

refraction

I
*

Post-op

power

IOL

index

g
h

Subject

GAs

op,

* *

Total no.
Squint additional

Residual mean spherical refractive error following IOL

Table IV.

24.0

Plano ('!)

23.5

+1.25

23.5

+0.75

26.0

-3,00

24,0

-4.00

26.0

+4.00

+3.00

24,0

no

-5.50

22.5

-2.50

visual acuities were then compared. No postoperative best

or latest acuity was worse than preoperatively, and most
were very significantly improved (Table II; Figs. I & 2).
Comparisons between preoperative and best post
operative acuity estimates and, for further comparison,
preoperative and latest visit acuity estimates were treated
as paired data. To determine whether surgical intervention
and postoperative treatment had made any significant
impact on improvement in acuity, the difference between
the two sets of paired data for the affected eyes were calcu
lated in octave units. (An octave is a doubling or halving of
the spatial frequency; for example, the difference between

15 and 30 cycles/degree or, in approximate Snellen

equivalent terms, the interval between 6/12 and 6/6.) A
Wilcoxon signed ranks test on the differences revealed a
highly significant improvement in acuity from the pre

Additional general anaesthetics after lens implantation

were required in some children for examination or further
procedures such as secondary posterior capsulotomy, free
ing of anterior synechiae, removal of sutures, or squint
correction (Table III). These procedures were often com
bined during one anaesthetic. No child had more than two
additional anaesthetics, and four children have needed no
additional anaesthetics to date (eyes d, j, m,

0,

Pl.

Residual postoperative refractive error and the power of

the intraocular lens are shown in Table IV. It can be seen
that even when lens aspiration and implantation was
carried out at three months of age, the residual en'or could
easily be corrected by glasses, or a temporary low power
contact lens in one case (eye n), We found the maximum
overcorrection to be less than we had anticipated before
the study started, and also to be surprisingly unpredictable

operative visual status for both the best achieved acuity (p

in value,

0.00 I, median improvement 2.27 octaves 1approx. 4

Snellen lines], 95% CI 11.69, 2.89]), and the latest,visit
acuity (p
0.00 I, median improvement 1.83 octaves
r approx. 3 Snellen lines], 95% CI 11.32, 2.28 D. The mar

If intraocular lens implantation for the correction of apha

DISCUSSION

ginal decrease in median improvement for the latest visit

acuity is probably due to a premature relaxation in the
occlusion regime-a drop which we would hope to
reverse.
Unilateral ClI'taraC1

Preop VA (8 I

kia after aspiration of congenital cataracts can be shown to

be as effective and safe a method as it is in adult aphakia,
the management of congenital cataract will become very
much simpler, allowing our efforts to be directed to the
relief of the other major problem-amblyopia.

61lateralCall!ri!lCt

r-------

600

'00

'00

'00

'00

Preop VA (6,

300

200

'00

'00

200
lates1

300
Poslop VA (6: I

.00

500

r-------

600

Fig. 1. Graph comparing preoperative Snellen equil'alellf

\'isual acuity (VA) with hest recorded postoperative VAjiJllowing
10L implantation for paediatric aphakia.

'00

Fig. 2.

Brlateral Cataract

) 300

200

100

Unilateral Cidaract

200

300
Best PostoD VA (6/ )

400

'00

600

Graph comparing preoperatil'e Snellen equil'aleflt

visual acuity

(\A)

with latest recorded postoperati\'e VA

jiJllowing 10L implalltatioll jiJl' paediatric aphakia.

497

PAEDIATRIC PSEUDOPHAKJA
For the most part. our study concerned the management

infants is that hypermetropia decreases markedly over the

of unilateral congenital cataract, and it seems reasonable

first two years of life in most children, and therefore the

at this stage to limit intraocular lens implantation to uni

required full correction of aphakia at three months of age

lateral cases, which have a poor visual prognosis, until

is likely to be a marked overcorrection at two years.32 We

longer follow-up has definitely established the safety of

aimed to manage this problem by implanting lenses of a

this tehnique and favourable long-term visual results. The

power that we estimated would subsequently give a refrac

technique can then be extended to the category of bilateral

tion of approximately -2.00 dioptres in later life. In

cataract which has a better visual prognosis.4 However ,

where it is believed that neither glasses nor contact lenses
will prove to be a satisfactory method for correction of
bilateral aphakia. bilateral intraocular lens implantation
should in our opinion be considered now.
We identified and addressed potential problems in six
areas in this study.

(1) Suitahle intraocular lens material

Poly-HEMA as a lens material has the advantage that
inflammatory cells are not readily deposited on the
anterior or posterior surface.29 This is important since
anterior uveitis is the most commonly reported complica
tion in our and other reported studies,7.'6'o11 particularly if
there has been any manipulation of the iris.
So far. there has been no reason to doubt the biocom
patibility of this material in the intraocular situation.

infants, this would leave residual hypermetropia that

could be corrected with glasses or a low power contact
lens which is easy to manage compared with a full aphakic
contact lens. It was never our intention to change the intra
ocular lens at any stage, and, as can be seen from our
results, residual refractive error did not seem to be a major
problem (Table IV). Toward the end of the study we began
to carry out biometry and keratometry in order to gauge
suitable intraocular lens power with more accuracy
immediately preoperatively; in practice, this did not seem
to decrease the postoperative residual error, but merits
more detailed study in future cases.

(4) Treatment of amblyopia

All unilateral and some bilateral congenital cataracts will
have dense amblyopia requiring occlusion or penalisation
therapy. We aimed to carry out total occlusion of the good

(2) Lens design and metho d (!f intraocular .fixation

eye for eight hours daily starting within one week of lens

We used 10gel l l03 lenses except in two eyes. One had the

aspiration, and lasting for a period depending on response

smaller diameter logel 1003 pattern. There appeared to be

to treatment. We anticipated that this treatment might be

no difference between the two logel lenses. One other

more readily accepted by child and parents if they did not

child had a PMMA lens (Allergan Medical Optics) which

have to manage an aphakic contact lens at the same time.

was quite satisfactory but these lenses might be difficult to

We cannot say whether this is true at the present stage.

manoeuvre in an infant eye.

The poly-HEMA lenses are easy to insert through a
shelving corneal incision without loss of the anterior
chamber depth even without the use of a viscoelastic sub
stance. No attempt was made to insert the lens into the
capsular bag for three reasons. First, it is very difficult to
insert any lens into the capsular bag in children because
the two leaves are usually pressed tightly together requir
ing excessive manipulation that in children often leads to a
marked fibrinous uveitis.7 Secondly, allowing the anterior
and posterior leaves of the lens capsule to adhere behind
the intraocular lens means that after a subsequent posterior
capsulotomy the size of the gap is limited, very much
reducing the possibility of posterior dislocation of the lens
into the vitreous cavity-otherwise a potential problem
with these lenses.29 Finally. capsular fixation can result in
marked decentration of a soft intraocular lens from
unequal contraction of the capsular bag. This is particu
larly so if one flange of the lens is in the bag and one in the
sulcus.29
The apparent marked bicompatibility of the poly
HEMA material means that no tissue reaction develops to
hold the lens firmly in place as in PMMA lenses. This
probably contributed to the slight decentration observed in
two of our lenses. If any lens had to be removed sub
sequently, this would clearly become an advantage.

In retrospect, we feel that our occlusion regime was not

adequate in practice. probably resulting in poorer visual
results than could otherwise have been obtained. We hope
to remedy this in future cases, believing that parents need
to know exactly what regime and targets are planned, so
that they can in tum give their full co-operation.

(5) Measurement of visual acuity

In order to follow progress in amblyopia treatment, and
recognise problems such as posterior capsular thickening
in the youngest patients, we needed a reliable system of
visual acuity measurement in this age group. The util
isation of the Forced Choice Preferential Looking method
of visual acuity assessment in this unit has fulfilled this
role where previously no accurate method existed for this
age group.28 Not all children were assessed by FCPL pre
operatively because our system was not fully developed at
that stage. Preoperative visual acuity in some children was
too poor to allow a response to any method of testing.
All children were assessed postoperatively by FCPL
however , and some by other standard methods such as
Kay Pictures, Sheridan-Gardiner, and Snellen, in order to
cross-check

reliability.

W here

comparisons

between

FCPL and the other standard methods were possible, there

was remarkable consistency between the two.

(3) Intraocular lens power

(6) Method of posterior capsulotomy

One of the criticisms of intraocular lens implantation in

We felt that in order to minimise the possibility of

R. H. C. MARKHAM ET AL.

498
development of retinal detachment in later years, there
should be the minimum of disturbance of the anterior vit
reous body. Thickening of the posterior lens capsule is
very frequent after lens aspiration in children. 16 Posterior
capsulotomy through the cornea before or at the time of
lens implantation in children usually results in the forma
tion of vitreous strands to the corneal entry site unless an
anterior vitrectomy is carried OUt.7,12
We therefore elected to carry out secondary posterior
capsulotomies only when the capsule developed thick
ening (shown in some infants by a drop in FCPL acuity),
or primary capsulotomies if posterior capsular opacities
could not be polished away at the time of lens aspiration.
We used a vitreous suction cutter introduced via the pars
plana and with an anterior chamber infusion of Balanced
Salt Solution through the peripheral cornea. Even then,
only posterior lens capsule was removed and no attempt
made to carry out an anterior vitrectomy. It is likely that a
suitably mounted Neodymium Y AG laser would make
posterior capsulotomy an even easier and safer procedure.
One of our children had a prmary capsulotomy after
implantation of the only PMMA lens. This was carried out
through the cornea and is easier with this pattern of rigid
lens but still runs risks of vitreous incarceration into the
corneal entry site.
We believe that recent work,12,21 including our own,
shows that we are already a considerable way along the
path of safe intraocular lens implantation in children.
Close and continued follow-up of implanted eyes will be
essential, but we feel confident that this will prove to be an
ideal method of unilateral aphakic correction, extendable
to bilateral cases as experience develops.
Key words: Amblyopia, Congenital cataract, Intraocular lenses, Poly
HEMA lenses, Preferential looking, Residual refraction.

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