Brit. J. Ophthal. (1970) 54, 759

Treatment of congenital and juvenilecataract with intraocular lensimplants (pseudophakoi)

C. D. BINKHORST* AND M. H. GOBINt* Terneuzen, Netherlands, and tAntwerp, Belgium

Although surgical and optical techniques are continually improving, the treatment ofcataract in infancy and childhood is still complicated by such problems as amblyopia,squint, and monocularity. This holds true for traumatic cataract in early life and alsofor congenital and early acquired endogenous cataract.We have already reported the results of treating unilateral traumatic cataract in child-

hood with intraocular lens implants (pseudophakoi), mainly irido-capsular lenses of ourown design (Binkhorst, I967; Binkhorst and Gobin, I964, i967; Binkhorst, Gobin, andLeonard, I969). In the past 6 years we have also treated several children with congenitaland juvenile cataract, most of them by the same method of irido-capsular fixation.

Pseudophakos implantation is not indicated in every such case; the child's age and thefunctional status of the eye have to be carefully considered. The anatomy and the"surgical misbehaviour" of the very young infant eye may be contraindications for theprocedure, and eyes with total or subtotal cataracts that have to be operated upon at a veryearly age are not generally suitable. The youngest child to receive a lens implant was8 months old, and the eldest iI years old. The best anatomical results are obtained fromthe age of I year onwards.

Functional status is influenced by such criteria as the density of the cataract (complete,nearly complete, incomplete), the presence of associated anomalies (microphthalmos,nystagmus, abnormal electroretinographic activity), the presence of squint, and the visualacuity. In older children the visual acuity should be determined repeatedly with care andpatience under varying conditions of light and pupil size, with and without spectaclecorrection, after the child has been trained to cooperate in Snellen's E test. Very youngchildren may need to be admitted to hospital for observation so that the visual perfor-mance of each eye can be assessed accurately.The results in seventeen children are discussed below. The follow-up period in a few

is long enough to list these results as definitive, but in others our report is merely prelimin-ary. In all of them the pseudophakos is well tolerated, the pupil is clear, and there havebeen no complications.

Group I Unilateral cataract

Cases I to 9 were suffering from dense, total or subtotal cataract in one eye (Table I,overleaf).

Received for publication March 6, 1970Address for reprints: Axelsestraat 54, Terneuzen, The Netherlands

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Unilateral cataract is usually detected because of associated anomalies. All of thesenine patients had nystagmus, there was microphthalmos in five, and the affected eye wasusually squinting. In Case 5 there was intermittent deviation and the prism test waspositive. In every case, on occlusion of the normal eye, visual acuity proved to be nil;this was most probably due partly to cataract and partly to amblyopia.

Surgery of unilateral cataract is justified only when the child will benefit from it; forexample, visual acuity may be improved, but this can only be achieved and maintainedby energetic occlusion therapy. In all the children in our series the visual acuity improved,even if there was microphthalmos. Although we have not so far demonstrated visualacuity better than ooI to o i, all the children behaved as though they could see betterwhen the normal eye was occluded. Occlusion was readily accepted by those who didnot accept it before surgery, the common symptom of light sensitivity having disappeared.Nystagmoid movements were favourably influenced by the lens implantation, but therewas no improvement in squint. In two cases the pseudophakos was implanted for cosmeticreasons. The full history of a case of unilateral cataract is given below.

CASE 5

A girl aged 2 years had a dense nuclear and lamellar cataract in her microphthalmic right eye.There was a very slow searching and partially latent nystagmus. Artificial mydriasis did not affectfixation. An intermittent convergent squint was present. Extracapsular extraction and implant-ation of a pseudophakos were both performed in one operative session. The patient's cooperationin the occlusion of the left eye was excellent.Her present state (31 years after implantation) is shown in Fig. i. The pseudophakos is well

tolerated, the pupil is clear, and the visual acuity of the operated eye is o.o8. The visual acuity ofthe fellow eye is o-s. There is an intermittent convergent squint and latent nystagmus. Simultan-eous perception and fusion are present, the latter without amplitude.

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Group II Bilateral subtotal cataract

Cases IO to I are shown in Table II (overleaf). Implantation was performed in one eyein Cases I I 12, 13, 14, and I5, and in both eyes in Cases io, i6, and 17.The cataract was discovered at varying stages for various reasons; nystagmus and squint

led, in some cases, to its discovery at an early age. If no such signs were present thechildren were brought for examination because of apparently poor visual acuity at from3 to 8 years of age. Microphthalmos was not seen in this group.

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Pseudophakos in congenital andjuvenile cataract

After surgery, the visual acuity, either measured by Snellen's E-test or estimated by thechild's behaviour, improved markedly in every case. Nystagmus decreased and fixationimproved. Two children with mental retardation developed well after surgery (Casesi6 and I7).Three children had no squint before treatment (Cases IO, I I, and I2). In Case IO we

twice had to deal with breakdown of fusion, but finally succeeded in restoring full binocu-larity; this 8-year-old boy developed unequally dense cataracts in both eyes. After cat-aract extraction in the worse eye and the fitting of a contact lens, this eye developeda convergent squint which was cured by pseudophakos implantation. A few months later,however, decreasing visual acuity of the fellow eye induced a convergent squint of this eye.Again the squint was cured, this time by cataract extraction and implantation of a pseudo-phakos. Since then this bilaterally pseudophakic boy has had full binocular vision. CasesI I and 12 had the worse eye only operated on. Care was taken to establish favourableanatomical and optical conditions, viz. a clear pupil, and the implantation was carriedout in the shortest possible time. In both cases we succeeded in preventing breakdown offusion.

In four children in this group (Cases 14 to I7), squint was present before treatment and,except in Case 14, it was accompanied by nystagmoid movements of the squinting eye.In none of these children was lens implantation, either unilateral or bilateral, followed bya cure of the squint and the establishment of binocular vision, either spontaneously orafter muscle surgery. It must be stated, however, that the conditions were unfavourable.In Case 14 more than 4 years elapsed between the first appearance of the squint and theimplantation. In Case I5 nystagmus, and in Cases I6 and I7 nystagmus and mentalretardation, complicated the squint. In Case I3 there was nystagmus without squint.The superiority of pseudophakic treatment is clearly shown in this group. In Case IO

implantation of a pseudophakos cured a convergent squint that had been started off byfitting a contact lens, and in Cases i I and I 2 implantation prevented breakdown of fusion.For the pseudophakic correction to be successful, however, aniseikonia and prismaticimbalance must be absent.

Extracapsular cataract extraction and implantation of the pseudophakos at the sameoperative session ensure favourable conditions for fusion. Great attention should be paidto signs pointing to a breakdown of fusion and thus to a squint, either of the operated eye(if visual acuity is not re-established quickly) or of the fellow eye (if visual acuity is toofar below that of the operated eye).

Long-term postoperative pleoptic treatment is much more acceptable to a child wearinga lens implant than to one who has to cope with a high-powered spectacle correction orwith a contact lens.Another advantage is that the use of a lens implant greatly reduces feelings of inferiority.

If a squint develops as a result of cataract, prompt pseudophakic treatment of this eyeoffers a fair chance of relieving it.

Such cases may be compared with those in which unilateral traumatic cataract causesa squint to develop (Binkhorst and Gobin, I967; Binkhorst, Gobin, and Leonard, I969).An important feature of pseudophakic treatment, clearly demonstrated in Cases I I and

I2, is that the implantation of a pseudophakos into the eye with the poorer visual acuitygives not only better visual acuity, but also binocular vision. In this way the surgicalrisk is limited to one eye only, whereas to achieve fusion by correcting the aphakia with acontact lens or with spectacles, would require bilateral surgery with the further risk ofbreakdown of fusion through prismatic imbalance.

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C. D. Binkhorst and M. H. Gobin

The following case history is a typical example of bilateral congenital cataract treatedwith a pseudophakos implantation in one eye.

CASE I2

A boy aged 5 years had reduced visual acuity due to bilateral cataracts of the lamellar and anteriorpolar types. In favourable lighting the visual acuity was 0-3 in the right eye and 0-5 in the left.The eyes were straight. Extracapsular cataract extraction and implantation of a pseudophakosinto the right eye were performed at one operative session. Occlusion of the left eye was then carriedout each afteinoon. The visual acuity of the right eye is now gradually improving and there areno oculomotor problems.

His present state (2 years after implantation) is shown in Fig. 2. The pseudophakos is welltolerated, and he is able to attend school. The visual acuity of the operated eye is 0-4, and oB8with -2 D. cyl., axis 850. That of the left eye is 0-4, and 0-5 with -2 D. sph. The eyes are straight.Fusional amplitude ranges from -8° to -t30°. The synoptophore stereo test and fly stereo test arepositive. The Wirt stereo test is 0-3.

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Summary and conclusions

The implantation of an irido-capsular pseudophakos is a useful procedure wherebychildren with congenital, infantile, or juvenile cataract can be provided with a clear pupiland approximately normal refraction. Seventeen children underwent pseudophakosimplantation, fourteen in one eye and three in both, for unilateral or bilateral cataract.Their ages ranged from 8 months to I I years. No complications were observed in thefollow-up period which ranged from i 2 to 7 2 years. The lower age limit for pseudophakicimplantation is 6 months to I year. The authors' experience leads them to make thefollowing recommendations:

(a) Pseudophakic implantation may be considered for optical reasons (nystagmus, oligo-phrenia), for social reasons (eye test), or for psycho-cosmetic reasons.

(b) Pseudophakic implantation is indicated in cases of imminent or recently developedsquint as well as in bilateral and unilateral cataract. It is the best way of providing thesquinting eye with better visual acuity and a good chance of fusion. It facilitates visualtraining and makes occlusion more acceptable to both child and parents. Surgeryshould usually be performed at a single session.

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Pseudophakos in congenital andjuvenile cataract 765

(3) Pseudophakic implantation is strongly indicated in cases of bilateral cataract withbinocular vision but insufficient acuity. Unilateral implantation into the worse eyeprovides the child with the required visual acuity and helps to prevent breakdown offusion. Surgery should be performed in a single session. Implantation into the othereye is not indicated unless a squint develops.

References

BINKHORST, C. D. (I967) Brit. J. Ophthal., 51, 767and GOBIN, M. H. (I964) Ophthalmologica (Basel), 148, I69

(I967) Ibid., x54, 8Iand LEONARD, P. A. M. (I969) Brit. J. Ophthal., 53, 5I8

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