British Journal of Ophthalmology, 1989, 73, 494-497

Functional lid surgery in Down's syndromeR J MORRIS AND J R 0 COLLIN

From Moorfields Eye Hospital, City Road, London ECI

SUMMARY Down's syndrome is associated with characteristic eyelid abnormalities. Ten patientspresented with lid abnormalities leading to corneal problems. Five patients had ectropion, four ofwhom required skin grafting to correct the abnormality. One patient had entropion and four hadchronic blepharoconjunctivitis leading to trichiasis, which was treated with double freeze thawcryotherapy.

Down's syndrome is associated with a wide variety ofanatomical and biochemical abnormalities includingcongenital mental retardation, congenital heartdefects, immune-system deficiencies, and ocularabnormalities-most commonly cataracts, kerato-conus, Brushfield spots, and strabismus. Theincidence of the disease is approximately 1 in 700 livebirths and becomes greater with increasing parentalage.' The characteristic facial features associatedwith Down's syndrome include macroglossia,hypotonia of the lower lip, receding chin, hypoplasiaof the nasal bridge, epicanthic folds, oblique lid axis,and dysplastic ears. In 1977 Hohler described a caseof a girl with Down's syndrome in whom he under-took facial plastic surgery purely for cosmesis,2 andsince then there have been other reports describingthe results of cosmetic facial plastic surgery forDown's syndrome.' In some cases however, thecharacteristic lid abnormalities may require correc-tion for functional reasons. In particular, ectropiondue to skin shortage may lead to corneal exposure,and trichiasis resulting from chronic blepharitis maysimilarly lead to corneal problems.We report on 10 patients who presented with lid

abnormalities leading to corneal problems, nine ofwhom underwent lid surgery.

Patients and methods

Fifty four patients with Down's syndrome who hadattended the hospital were studied; of these 10 hadeyelid problems directly related to their disease, andthe rest had other congenital ocular abnormalities.There were five male and five female patients. Theage range at presentation was 7 months to 55 years.

Correspondence to J R 0 Collin, Moorfields Eye Hospital, CityRoad, London EC1V 2PD.

ECTROPION GROUP (FIVE PATIENTS)Four patients had bilateral upper and lower lidectropion as a result of severe lid skin shortage (Fig.1), and two developed spontaneous eversion of thelids during sleep and on crying (Fig. 2). All had poorlid closure, with an inadequate blink response andsigns of bilateral corneal exposure in the interpal-pebral zone. Three patients, all of whom had hadprevious bilateral tarsorrhaphies which had brokendown and failed to provide adequate corneal protec-tion, developed permanent corneal scarring in theinterpalpebral zone (Fig. 3); one of these haddeveloped a corneal abscess. The ectropion wastreated in all cases with full thickness skin grafting tocorrect the skin shortage, postauricular skin beingused as the donor site (Fig. 4). In three patients allfour lids were grafted and in the other patientpostauricular Wolf grafts to the upper lids werecombined with recession of the levator palpebraesuperiors. Although there was skin shortage of thelower lids, it was not considered severe enough towarrant surgery. Four years later this patient requiredfurther surgery as result of corneal exposure. Conse-quently bilateral lower lid scleral grafts and skingrafts from the upper arm, together with bilateral

Fig. 1 Child with upper and lower lid ectropion.494

Functional lid surgery in Down's syndrome

Fig..2.Spontaneou. e in oft.lids.. n .........| ~~~~~~~~.Fig. 2 Spontaneous eversion1 of tds lides own crying.

lower lid shortening for excess lid laxity, wereperformed. In another patient lid skin graftingwas combined with division of tarsorrhaphies andhorizontal shortening of the lower lids (Fig. 5).The fifth patient with ectropion in this group was

a 55-year-old man. He had bilateral lower lidectropion, bilateral blepharitis, madarosis, andcorneal scarring in the interpalpebral area. However,the lid abnormalities were not considered severeenough to warrant surgery, and he was treated withocular lubricants alone.

ENTROPION GROUP (ONE PATIENT)An 11-year-old girl presented with epiphora. Onexamination she had bilateral upper and lower lidentropion more marked medially, with punctatestaining of both corneas. The entropion wascorrected surgically with an anterior lamellarrepositioning of the upper lids and plication of thelower lid retractors (Jones type procedure).

TRICHIASIS GROUP (FOUR PATIENTS)Four adult patients had trichiasis leading to surfacecorneal changes, but not associated with anymalposition of the lid. Their ages were 15, 22, 27, and54 years. In each case the trichiasis was associatedwith severe chronic blepharitis, and a variety oforganisms were identified from lid margin cultures,including Staphylococcus epidermidis (threepatients), Haemophilus influenza (one patient), andCorynebacterium xerosis and a haemolytic strepto-

Fig. 3 Patient with upper and lower lid ectropion, bilateraltarsorrhaphies, and corneal scarring.

coccus (one patient). One of this group had beenreferred for corneal grafting for keratoconus but hadbeen found to have trichiasis and lash ptosis whichneeded correction prior to corneal surgery. Thetrichiasis in these patients was treated with doublefreeze thaw cryotherapy to the lid margin. The lidwas frozen from the lid surface with a special probe toa temperature of -200C; the tissues were thenallowed to thaw spontaneously and the freeze thawcycle was repeated. The temperature was moniteredwith a thermocouple needle placed deep toorbicularis oculi and superficial to the tarsal plate inthe region of the lash follicles. One patient hadelectrolysis to two isolated lashes on the right upperlid in addition to cryotherapy of the other lids.

Results

Ectropion group. In the four patients who underwentfull thickness skin grafting the surgery corrected theectropion as well as enabling good lid closure andimproved corneal protection (Fig. 4). In all thesecases the signs of corneal exposure resolved, thoughin three patients there was residual corneal scarring(Fig. 5). The cosmetic results of skin grafting in theseyoung children were not good but improved withtime. One patient in this group with ectropion, a 55-year-old man, did not require surgery but respondedwell to treatment with ocular lubricants alone.

Fig. 5 Same patient as in Figs. 2 and 3 postoperatively.

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Fig. 4 Same patient as in Fig. I postoperatively.

R J Morris andJR 0 Collin

Entropion group. Surgery corrected both upperand lower lid entropion in this case.

Trichiasis group. Cryotherapy was effective incontrolling the trichiasis in all four patients with norecurrences. The patient with keratoconus subse-quently underwent successful bilateral penetratingkeratoplasty.

Discussion

Congenital ectropion is a well recognised and poten-tially sight threatening condition seen in childrenwith Down's syndrome but rarely other children.Many cases respond to conservative treatmentalone,7 but in children with Down's syndromesurgical correction is often necessary.89 In thisstudy four of our fifty-four patients had congenitalectropion leading to corneal exposure and requiringsurgery. Two cases were severe enough to lead tospontaneous eversion of the lids, which increased onforced lid closure. In three patients lagophthalmosand associated corneal exposure led to cornealulceration and permanent scarring; in one case it wassevere enough to lead to a corneal abscess andhypopyon. It is therefore imperative that cases withsignificant corneal exposure are treated promptlywith appropriate surgical techniques to prevent anypermanent corneal complications.

In these patients the mechanism for the ectropionwas skin shortage of the lids leading to verticalshortening of the anterior lid lamella, which pulls thelid margin away from the globe and results inectropion. Associated conjunctival inflammation andchemosis further aggravate the condition, and inlong-standing cases the problem may be com-pounded by the development of horizontal lid laxity.

Lateral tarsorrhaphies and horizontal lid shorten-ing procedures have been used in an attempt toimprove corneal cover but with variable results.89Three of our patients had had previous tarsorr-haphies which had broken down, resulting in poorcorneal protection, with one patient developing acorneal abscess and hypopyon as a result of cornealexposure. This technique should not be used in youngchildren in whom the primary abnormality is skinshortage but reserved for cases of paralytic ectropionrequiring temporary corneal protection.We believe that the correct management of the

congenital ectropion in these cases is correction ofthe underlying skin shortage with skin grafting. Inmost cases there is skin shortage of both upper andlower lids, and therefore transposition flaps cannotbe used. Full thickness skin grafts are preferred andshould be extended beyond the horizontal limb of thecanthi to compensate for subsequent contraction ofthe graft. The preferred donor site is postauricular

skin, and even in young babies adequate skin isusually present. Other possible donor sites includeskin from the supraclavicular fossa or upper innerarm. Our results show that this is an effective way ofcorrecting the ectropion and poor lid closure, therebypreventing corneal exposure.

In long-standing and severe cases additionalsurgical procedures may be necessary fully to correctthe ectropion. If excess lid laxity is present, a fullthickness horizontal wedge resection can be per-formed at the same time as skin grafting, or may benecessary if the ectropion recurs. In other cases skingrafting with or without a horizontal lid resectionmay be combined with a lid lengthening procedure ifcorrection of the ectropion alone does not result inadequate lid cover. The upper lid may be lowered,provided the skin shortage is also corrected, bytarsotomy, recession of levator palpebrae superioris,or scleral grafting, depending on the degree of lidlengthening required. The lower lid may be raised byinterposing a scleral graft between the anterior lidretractors and the lower border of the tarsus.The patient with ectropion who did not require

surgery was an adult with involutional ectropion,which was not therefore associated with skinshortage.

In the older patient with Down's syndrome chronicblepharoconjunctivitis may lead to the developmentof cicatricial entropion and trichiasis. Four of ourpatients were adults with long-standing blepharitis,and all showed signs of corneal disturbance as a resultof the trichiasis. If neglected, such corneal changesmay progress and corneal suppuration develop.

Electrolysis may be used for single or aberrantlashes, but for large numbers it is time consuming,poorly effective, and may produce scarring of thetarsal plate. These cases are better treated withdouble freeze thaw cryotherapy to the lid margin.10More severe cases of trichiasis and those withcicatricial entropion may be treated surgically asappropriate for the degree of scarring.'I 12

This study highlights the specific malpositions seenin patients with Down's syndrome and outlines anapproach to the management of these problems.

References

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6 Olbrisch RR. Plastic surgical management of children withDown's syndrome: indications and results. Br J Plast Surg 1982;35: 195-200.

7 Stillerman ML, Emanuel B, Padorr MP. Eversion of the eyelidsin the newborn without an apparent cause. J Pediatr 1966; 69:656-8.

8 Gilbert HD, Smith RE, Barlow MH, Mohr D. Congenital upperlid eversion and Down's syndrome. Am J Ophthalmol 1973; 75:469-72.

9 Johnson CC, McGowan BL. Persistent congenital ectropion of

all four eyelids with megaloblepharon. Am J Ophthalmol 1969;67: 252-6.

10 Johnson RLC, Collin JRO. Treatment of trichiasis with a lidcryoprobe. BrJ Ophthalmol 1985; 65: 267-70.

11 Kemp EG, Collin JRO. Surgical management of upper lidentropion. Br J Ophthalmol 1986; 70: 575-9.

12 Collin JRO. A manual of systematic eyelid surgery. Edinburgh:Churchill Livingstone, 1983: 12-27.

Acceptedfor publication 3 November 1988.

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