British7ournal ofOphthalmology, 1991,75,298-300

Capillary haemangiomas: an approach to theirmanagement

M J Boyd, J R 0 Collin

AbstractTwenty-five children with eyelid haeman-giomas were reviewed. Fifteen patients withenlarging lesions thought to be at risk ofcausing amblyopia were treated with intra-lesional steroids as soon after presentation aspossible. This appeared to reduce significantlythe incidence of amblyopia. Surgery wasreserved for older children in whom no furtherinvolution ofthe lesion was expected.

Capillary haemangiomas of the lid are a distinctclinical entity in children. They threaten visionby causing anisometropia, sensory deprivation,amblyopia, and strabismus.' Their managementand its effectiveness have remained controversialowing to the high complication rate and uncer-tain success of any treatment. The tumoursfollow a characteristic clinical course, appearingshortly after birth, growing quickly in the firstyear of life, and then beginning a slow, involu-tional phase; 40% resolve by age 4 and 70% byage 7.2 Despite this the risk of amblyopia hasencouraged a variety of treatments to be used.Radiotherapy is associated with cataracts andcutaneous scarring, and sclerosing agents withscarring and unpredictability. Radon seeds andcryotherapy both cause cutaneous atrophy.Eventually intralesional steroids were tried, firstas an addendum to a trial of systemic steroids byZarem and Edgerton,' then more extensively byMazzola4 and Kushner.'For 10 years we have been using intralesional

steroids during the active phase of lesionalgrowth and with adjunctive treatment (occlusionand glasses). Surgical debulking is done if neces-sary at a later inactive phase. We present ourresults on 25 patients over a 10-year-period.

Moorfields Eye Hospital,City Road, LondonEC1V 2PDM J BoydR J 0 CollinCorrespondence to:Mr J R 0 Collin, FRCS.Accepted for publication13 November 1990

Subjects and methodsAll the patients had capillary haemangiomasdiagnosed by virtue of the history of onset andprogression of disease and the appearance of thelesions. Seventeen of the 25 patients were female(68%). Sixteen (64%) were symptomatic withintwo weeks of birth and 23 (92%) before threemonths. The patients presented to our clinicbetween the ages of 1 month and 7 years. Twentyof 25 patients' lesions (80%) were on the upper

lid. The largest and smallest axes of the lesionswere measured, but computerised tomographywas not routinely performed, and without this itwas impossible to assess the variable bulk andvolume of the lesions.

Treatment was thought to be warranted if thelesion threatened the visual axis or if there were

signs suggesting amblyopia (strabismus or

refractive error). In accordance with their

severity some lesions were followed up expec-tantly; the children were refracted and treatedwith glasses and occlusion therapy as necessary.Other children, in whom amblyopia was agreater risk, received steroid injections. Theroutine injection consisted of 40 mg of methyl-prednisolone and 4 mg of dexamethasonesodium phosphate. The first was injecteddirectly into the lesion, and the second, moresoluble, was injected paralesionally. The directintralesional injection was given deeply toprevent long-acting depotsteroid deposits frombeing visible under the skin. The paralesionalinjection was given subcutaneously round theperiphery of the haemangioma. The injectionswere repeated three times at approximately six-week intervals if there was some response but nogross decrease in the size of the lesion. Twopatients had a total of four injections each.

Surgery consisted of lid debulking or adjust-ing lid height or contour and was done only afterthe tumour had passed into an inactive growthphase, usually after the age of six. Twenty twopatients presented to us without any previoustreatment, but three had initially undergonetreatment elsewhere: one had a course of cryo-therapy followed by one course of steroid injec-tion; one had had two courses of cryotherapy;and one arrived with ptosis after surgicalexcision.

ResultsOf the 25 patients 15 needed occlusion treat-ment, nine required glasses, and 15 had intra-lesional steroids. Of those not treated withsteroids six had presented prior to its usage, twowere children with lesions not warranting injec-tions (too small or thought not to be at risk ofcausing amblyopia) and two presented after theactive growth phase of the tumour had ended.Twelve of the 15 (80%) required only one or

two injections, but one case needed three andanother two needed four. The injections ofteninitially caused some extravasation of blood. Thepatients were reviewed after four weeks whenany haematoma had absorbed. A subjectiveassessment was made by asking the parents ifthey thought the lesion had regressed, and anobjective assessment was made by measuring themajor and minor axes of the lesion. The injec-tions had a significant reducing effect in nine of15 (60%) cases (Figs 1A, B), questionable effectin four, and little or no effect in two (Figs 2). Inthose cases that responded there was a consider-able reduction in subcutaneous vascularity aswell as a reduction in bulk, but this was not easyto quantify. If subjectively and objectively therewas a dramatic response, the injection was notrepeated, but if there was only a partial improve-

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Capillary haemangiomas: an approach to their management

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Figure lA Four-month-old child with actively enlargingupper lid haemangioma.

ment the injection was repeated up to four times.If there was no response or the lesion actuallyincreased in size despite the injection, it was notrepeated. It was not possible to identify thefactors which made it more likely that somelesions would respond while others would not.No systemic side effects from the steroids werenoted. Follow-up for all patients was a mean of 1year 11 months, being more than twice as longfor the non-injected group - 1 year 4 months(range 3 months to 3 years) versus 2 years 10months (range 1l/2to 5 years).The main reason for treating the patients,

particularly with steroids, was the risk ofamblyopia. Amblyopia is described as aunilateral or bilateral decrease in vision for whichno obvious cause can be detected.6 It proved tobe a major complication with these tumours,occurring in 64% ofour cases (16/25). It occurredin eight out of 10 cases (80%) treated withoutsteroids and in eight of 15 (53%) receiving steroidinjections. It was much more frequent with theupper lid lesions, being seen in 16 of 20 cases(80%) compared with 1 of 5 (20%) patients withlower lid lesions. This single case of lower lidhaemangioma was associated with long-termdense amblyopia and was left with countingfinger vision in the affected eye, but the lesionwas very large (Fig 3). Although it was notpossible to measure the lesions accurately, it wasnoted that, if the lesion was near the eyelidmargin and the visual axis was threatened oroccluded, amblyopia was common. We did notfield that earlier injections gave better visualresults than the later ones. The most amblyopiccases in the injected groups included both a childwho had been injected at the earliest age (2months) with an end vision of hand movementsand the child who had been injected at the latest

Figure 2A Three-month-old child with actively enlargingupper lid haemangioma.

OK~~~~~~~.Figure IB Same child three months after steroid injectionshowing rapid resolution.

age (2/2 years) with an end vision of countingfingers.

Six children had a total of eight operations at a

mean age of 7½yearss to correct the residual bulkof the lesion, entropion, ectropion, or ptosis.Five operations were debunking procedures viaan anterior skin approach (with one including ananterior lid repositioning); one was a full-thick-ness lid resection (Fig 4), another was a ptosisrepair (anterior levator resection), and theremaining operation was a skin crease reforma-tion procedure. Postoperatively the results weresatisfactory, though in two cases the skin crease

was not exactly symmetrical and the ptosis was

slightly overcorrected in one case and under-corrected in another.

DiscussionThere have been several large studies reviewingeither the complications or treatment of capillaryhaemangiomas of the lid. Stigmar et a17 reviewed51 cases of eyelid haemangiomas and found 27with ophthalmic complications: 44% developedamblyopia and 34% strabismus. They advocateda close follow-up with surgery, steroids, or x rays

if vision was threatened. Cosmetic repair was

delayed until age 8.Haik et al' analysed their results of treating

101 eyelid haemangiomas. Clinical features in-cluded early recognition by the family (95% by 6months of age) and female predominance (61%)and superior lid involvement (75%). Forty eightcases had long-term follow-up, with 60% show-ing amblyopia, usually secondary to sensory

deprivation or anisometropia. Various types oftreatment were used, but the type of treatmentdid not noticeably alter the cosmetic or func-tional outcome. Kushner' was an early advocate

Figure 2B Same child oneyear after steroid injectionsshowing progression oflesion despite treatment.

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Figure 3 Two-year-old child with large lower lidhaemangioma causing amblyopia.

of intralesional steroids and has reported hisresults of injecting 25 eyelid haemangiomas.Total resolution occurred in 16; seven showedless than 50% decrease in the size of the lesion,but enough resolution to prevent amblyopia.Only three cases failed to respond to treatment.Our study confirms many of these findings.

They include the high female predominance,predilection for the upper lid, and early clinicalpresentation. Amblyopia remains the foremostcomplication, occurring in 64% of the cases inour study. Its frequency emphasises the need forearly treatment and early follow-up. There was areduction in the frequency ofamblyopia with theuse of injectable steroids (80% down to 53%),and this should become more pronounced as thefollow-up becomes longer.We elected to use the same dosage ofintra- and

perilesional steroids for all haemangiomas asdescribed by Kushner.' I Bonavolonta et a19adjusted the injectable steroid dose to the size ofthe haemangioma as determined by CT, but theycould not prove that this method was moresuccessful, and at least one case developed Cush-ing's syndrome. Our arbitrary approach with acommon steroid dose irrespective of size orlocation of the tumour showed a successfulmethod of managing this lesion without majorcomplications.

Surgery in this study was limited to theinactive growth phase of the tumour. It was usedin cosmetic debulking procedures and wasroutinely successful. It was not associated withexcessing haemorrhage or rebound growth.Technically the skin crease and lid height weremost difficult to predict postoperatively.

In conclusion, eyelid haemangioma remains adistinct clinical entity associated with a high riskof amblyopia and complications from treatment.A method of management has been describedwhich includes an intralesional steroid injectionin sight threatening lesions, with elective

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Figure 4A Seven-year-old child with involuted upper lidhaemangioma.

Figure 4B Same child after debunking andfull-thickness lidresection ofthe upper lid haemangioma.

surgical debulking at a later age. This treatmentis successful in diminishing tumour size and therisk of amblyopia and is associated with minimalcomplications.

This paper was presented as part of the Dermot Pierse lecture atthe Royal Society of Medicine on 12 September 1990.

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2 Margileth A, Museles M. Cutaneous Hemangioma in children:diagnosis and conservative management. J Am Med Assoc1%5; 194: 523-6.

3 Zarem HA, Edgerton MT, Induced resolution of cavernousHemangiomas following prednisolone treatment. PlastReconstrSurg 1%7; 39: 76-83.

4 Mazzola RF. Treatment of haemangiomas in children byintralesional injection of steroids. Chirurg Plast (Berlin) 1978;4:161-71.

5 Kushner BJ. Intralesional cortical steroid injection for infantileadnexal Hemangioma. AmJ Ophthalmol 1982; 93: 496-506.

6 Von Noorden GK. Binocular vision in ocular motility. St Louis:Mosby, 1980: 219.

7 Stigmar G, Crawford JS, Ward, CM, Thompson HG. Oph-thalmic sequelae of infantile hemangiomas of the eyelids andorbit. AmJ Ophthalmol 1978; 85: 806-13.

8 Kushner BJ. Capillary haemangiomas of the adnexa in infants.Oculoplastic orbital and reconstructive surgery 1: Eyelids. Horn-blass A, eds. In: Baltimore: Williams and Wilkins, 1988:243-50.

9 Bonavolonta G, Vassallo P, Uccello G, Tranfa F. Ourexperience with intralesional cortical steroid injectiontherapy for infantile adnexal hemangioma. Orbit 1985; 4:177-81.

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