Letters to the editor

LETTERS TOTHE EDITOR

Calcification-like echographic pattern inuveal melanomas treated withbrachytherapy

EDITOR,-In a consecutive series of 1300patients with uveal melanomas treated withbrachytherapy ('"Ru/'MRh plaques),' threepatients developed unusual echographic find-ings following radiation. Pretreatment echo-graphic evaluation showed homogeneoustumour echoes with low inner reflectivity.The patients were a 48-year-old woman, a

63-year-old man, and a 73-year-old woman.Before treatment the maximum tumourheights were 6-9 mm, 7-7 mm, and 6-2 mmrespectively. The first patient received twocourses of radiation with 1360 and 1000 Gyscleral contact dose within 15 months and anadditional laser coagulation 6 months later.The other patients were treated once withscleral contact doses of 700 and 1000 Gyrespectively. Highly reflective echoes with pos-terior shadowing were detected 5, 7, and 2years after radiation in regressive residualtumours with a height of 2-7 mm, 1-2 mm, and1-7 mm respectively (Figs 1, 2).An increase of reflectivity usually occurs in

melanomas following radiation therapy.23However, very high reflectivity and markedposterior shadowing are very characteristicsigns of calcification and have not beendescribed previously. Histological findings fol-lowing radiation therapy include tumour

Figure I Echographic A-scan ofregressiveuveal melanomna 2 years after brachytherapy. Thesensitivity is reduced to 35 dB (standard tissuesensitivity 61 dB). On the right the smaller firstspike indicates the anterior surface ofthe residualtumour. The second spike indicates very highreflectivity within the tumour, the echoes of thesclera and orbita posterior to this signal aremarkedly diminished.

Figure 2 Echographic B-scan ofthe same eye atthe same time as Figure 1. A highly reflective areacan be seen within the residual tumour. Posteriorto this area a marked shadowing is present.

necrosis, haemorrhages, and lymphocytic infil-tration but calcifications have not beenobserved.45Two hypotheses may explain the unusual

findings. Either the intraocular tumour in ourpatients was misdiagnosed or calcification mayoccur in regressive uveal melanomas. In allpatients the ophthalmoscopic diagnosis of auveal melanoma was consistent with thefluorescein angiography and echographic find-ings, which makes a misdiagnosis unlikely.6Other ocular tumours presenting with calcifica-tion are retinoblastomas and osteomas, whichcan be excluded based on the age of the patientsand clinical findings. Calcification typicallyoccurs in necrotic areas for example, in retino-blastomas calcification most probably starts inthe mitochondria of degenerating tumourcells.78 Although histological evidence cannotbe presented in the successfully treated'eyes ofour patients, it is most likely that calcificationhas occurred in their necrotic residualtumours.

ULRICH KELLNERMICHAEL H FOERSTER

Freie Universitdt Berlin,Klinikum Steglitz,

Augenklinik,Berlin,

GermanyNORBERT BORNFELD

Zentrummfr Augenheilkunde,Universit&tEssen,

Essen,Germany

I Foerster MH, Bormfeld N, Schulz U, Wessing A,Meyer-Schwickerath G. Complications of localbeta radiation of uveal melanomas. Graefes ArchClin Exp Ophthalmol 1986; 230: 336-40.

2 Guthoff R, von Domarus D, Steinhorst U, Haller-mann D. 10 Jahre Erfahrung mit derRuthenium-106/Rhodium-106-Behandlung desmalignen Melanoms der Aderhaut-Bericht uber264 bestrahlte Tumoren. Klin MonatsblAugenheilkd 1986; 188: 576-83.

3 Eichler C, Hertel A, Lommatzsch P, Fuhrmann P.Echographische Befunde vor und nachfi-Bestrahlung ('1RuI'/`Ru) von Aderhaut-melanomen. Klin Monatsbl Augenheilkd 1987;190: 17-20.

4 MacFaul PA, Morgan G. Histopathologicalchanges in malignant melanomas of the choroidafter cobalt plaque therapy. Br J Ophthalmol1977; 61: 221-8.

5 Messmer E, Bormfeld N, Foerster MH, SchillingH, Wessing A. Histopathologic findings in eyestreated with a ruthenium plaque for uvealmelanoma. Graefes Arch Clin Exp Ophthalmol1992; 230: 391-6.

6 The Collaborative Ocular Melanoma Study Group.Accuracy of diagnosis of choroidal melanomas inthe Collaborative Ocular Melanoma Study,COMS report No 1. Arch Ophthalmol 1990; 108:1268-73.

7 SpencerWH, ed. Ophthalmic pathology: an atlasandtextbook. 3rd ed. Philadelphia: Saunders, 1985:14-5.

8 Lin CCL, Tso MOM. An electron microscopicstudy of calcification of retinoblastoma. Am JOphthalmol 1983; 96: 765-74.

Herpetic corneal ulcers in Malawi

EDrroR,-There have been reports of largenumbers of herpetic corneal ulcers inTanzania,"2particularly in association withmeasles or a history ofmalaria. As with many ofthese reports, we also must rely on clinicalappearance and response to therapy for diag-nosis ofherpetic ulcers and we also find that themajority of these are geographic or stromal.However, our experience in Malawi is thatherpetic ulcers are relatively uncommon.Malawi has a good system of ophthalticmedical assistants, active in every district.They successfully treat many bacterial cornealulcers and refer non-healing ulcers or thosethey suspect of being herpetic to the central

hospitals. Thus, we would expect to see adisproportionately large number of herpeticulcers compared to bacterial ulcers in thecentral hospitals. In Malawi, febrile illnessesare common (malaria is holoendemic andmeasles epidemics still occur) and the sero-prevalence of HIV is one of the highest inAfrica. All these factors would be expected tocontribute to a large number ofherpetic ulcers,if herpes simplex were common in the popula-tion. None the less, at the Queen ElizabethCentral Hospital, which serves the populationof five million in the southern region, fewerthan 10% of the corneal ulcers are presumed tobe herpetic. Among the 250 children admittedto the measles ward from March 1992 toJanuary 1993 there were no patients withcorneal ulceration or herpetic mouth ulcers.Similarly, among 350 children with cerebralmalaria admitted to the Malaria ResearchProject over the past 6 years none has hadcorneal ulceration or herpetic mouth ulcers.(TE Taylor, ME Molyneux, personal com-munication.) Although these data are notpopulation based, they suggest that herpessimplex is not common in this population.There is good documentation of the high

prevalence of herpetic corneal ulcers inTanzania and Nigeria.' However, the epidem-iology of herpes simplex in Africa may still betoo unclear to justify the assumption that it is acommon cause ofcorneal blindness throughoutthe continent.

SUSAN LEWALLENM C CHIRAMBO

Queen Elizabeth Central Hospital,PO Box 2273,

Blantyre,Malazwi

1 Foster A, Sommer A. Corneal ulceration, measles,and childhood blindness in Tanzania. Br JOphthalmol 1987; 71: 331-43.

2 Yorston D, Foster A. Herpetic keratitis inTanzania: association with malaria. Br J Oph-thalmol 1992; 76: 582-5.

3 Sandford-Smith JH, Whittle HC. Corneal ulcera-tion following measles in Nigerian children. BrJ7Ophthalmol 1979; 63: 720-4.

Glaucoma screening

EDITOR,-We were very pleased to see MrHitching's editorial on glaucoma screening inthe June edition of the journal. Readers mightlike to know that the RNIB figures quoted wereobtained from the RNIB Survey into Blind andPartially Sighted Adults in Britain, 1991(HMSO) by Ian Bruce, Aubrey McKennelland Errol Walker. A second volume is alsoavailable on the blind and partially sightedchildren in Britain.

SUE GRINDEYRNIB Health Services Unit,

224 Great Portland Street,London WIN 6AA

Gillespie syndrome reported as bilateralcongenital mydriasis

EDITOR,-Richardson and Schulenberg'reported a 21/2-year-old girl with bilateral con-genital mydriasis, developmental delay, and anatrophic vermis of the cerebellum. The familyhave recently consulted me and I would like tosuggest that this child has Gillespie syndrome,which is characterised by the triad of cerebellarataxia, partial aniridia, and developmentaldelay. Gillespie syndrome is distinct fromreports of autosomal dominant congenitalmydriasis without other complications.2'The first report of this phenotype is usually

attributed to Gillespie' who described a brotherand sister sib pair and suggested autosomal

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Letters to the editor828

recessive inheritance. The condition has beenassigned the McKusick catalogue number206700.5

Several authors have added single casereports to the literature.67 Sibling pairs havealso been reported9 10; in neither case wasconsanguinity noted. Crawfurd et al" reporteda family with three affected members; a brotherand sister had Gillespie syndrome; the sisterlater married an unrelated healthy male andhad an affected son. The authors suggested thatthe sister had married a heterozygote carrierand that provisionally the disorder should stillbe regarded as autosomal recessive. An alterna-tive explanation of autosomal dominant plusreduced penetrance, with most affected indi-viduals not reproducing, cannot be totallyexcluded. '

In conclusion, parents of an affected childshould still be advised of a one in four recur-rence risk. Further cases should continue to bedescribed in the literature.

0 QUARRELLNorth Trent Clinical Genetics Service,

Centrefor Human Genetics,1)7 Manchester Road,

Sheffield S10SDN

I Richardson P, Schulenberg WE. Bilateral con-genital mydriasis. Br i Ophthalmol 1992; 76:632-3.

2 White VW, Fulton MN. A rare pupillary defectinherited by identical twins. J Hered 1937; 28:177-80.

3 Caccamise WC, Townes PL. Bilateral congenitalmydriasis. Am i Ophthalmol 1976; 81: 515-7.

4 Gillespie FD. Aniridia, cerebellar ataxia and oligo-phrenia in siblings. Arch Ophthalmol 1965; 73:388-91.

5 McKusick VA. Mendelian inheritance in man.Baltimore: Johns Hopkins Press, 1991.

6 Sarsfield JK. The syndrome of congenital cere-bellar ataxia, aniridia and mental retardation.Develop Med Child Neurol 1971; 13: 508-11.

7 Lechtenberg R, Ferretti C. Ataxia with aniridia ofGillespie: a case report. Neurology 1981; 31:95-7.

8 Nevin NC, Lin JHK. Syndrome of partialaniridia, cerebellar ataxia and mental retarda-tion in Gillespie syndrome. Am J Med Genet1990; 35: 468-9.

9 Francois J, Lentine F, De Ronk F. Gillespiesyndrome. Ophthalmol Paediatr Genet 1984; 1:29-32.

10 Wittig EO, Moreira CA, Friere-Moia N, Vianna-Morgante A. Partial aniridia, cerebellar ataxiaand mental deficiency (Gillespie syndrome) intwo brothers. AmJ7 Med Genet 1988; 30: 703-8.

11 Crawfurd M d'A, Harcourt RB, Shaw PA. Non-progressive cerebellar ataxia, aplasia or pupil-lary zone of iris, and mental subnormality(Gillespie's syndrome) affecting 3 members of anonconsanguineous family in 2 generations.J MedGenet 1979; 16: 373-8.

Reply

EDITOR,-We are grateful to Dr Quarrell forsuggesting a more specific diagnosis of Gil-lespie's syndrome in the case we reported. Shepresented with developmental delay, partialaniridia, and was late in achieving her motormilestones. Cerebellar ataxia was not con-firmed on clinical examination and it was feltshe did not fulfil the characteristic triad ofGillespie's syndrome. An atrophic vermis anddilated fourth ventricle was reported by Nevinand Lin' in a case of Gillespie's syndrome.These changes were present in our case, sup-porting Dr Quarrell's argument.The syndrome is rare but should be con-

sidered in the differential diagnosis of casespresenting with congenital aniridia to allowgenetic counselling.

W E SCHULENBURGThe Western Ophthalmic Hospital,

Marylebone Road,London NWI SYE

I Nevin NC, Lin JHK. Syndrome of partial aniridia,cerebellar ataxia and mental retardation in Gil-lespie syndrome. Am J Med Genet 1990; 35:468-9.

An intralenticular foreign body and a clearlens

EDITOR,-We would like to draw your atten-tion to the following case which we feel is ofinterest.A 21-year-old mechanic presented with a

red, uncomfortable eye and blurred vision, 3days after noticing an object hit his left eyewhile working in a slate pit. There was a sealed,laceration of the central cornea, and a puncturewound ofthe overlying anterior lens capsule. Alarge intralenticular slate fragment wasobserved towards the lens equator (Fig 1). The

Figure I Slit image photograph demonstratingthe intralenticular particle ofslate at I year.

posterior capsule and the retina wereunaffected. The fragment was left in situ, sinceslate is chemically inert.' At 1 year, his visualacuity was 6/6. The anterior capsule wound hadhealed (Fig 2), but the lens has not opacified(Fig 3).

In 5% of cases of perforating ocular injurieswith retained intraocular foreign bodies, theforeign body lodges in the lens,2 which usuallybecomes opaque and requires cataract extrac-tion for visual rehabilitation." Documented

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Figure 2 Scheimpflug photographdemonstrating the reformation of the anterior lenscapsule with the intralenticularforeign body inthe anterior cortex at 3 months.

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Figure 3 Retroillumination photographshowing the slate particle against the red reflex at1 year. The thin arrow shows the corneal scar andthe thick arrow shows the anterior capsule scar,but the red reflex is otherwise clear.

cases of retained intralenticular foreign bodiesare either associated with mature cataracts, orlocalised lens opacities.'4 Unprogressive,localised lenticular opacities, such as capsularscars, opacities along the track of the injury, orposterior subcapsular opacities have also beendescribed following penetrating injuries withsmall sharp objects such as needles.' Thehealing capacity of the anterior lens capsule, incontrast to the posterior capsule, is well docu-mented and is thought to be due to the presenceof the subcapsular epithelium."' Epithelialproliferation creates a plug which seals thewound. The plug reduces as new capsule isformed, and reconstituted lens fibres fill in thetrack. We believe that our patient did notdevelop a significant opacity because the pos-terior capsule was intact, and the entry site wassmall and linear, allowing the breached capsuleto seal itself rapidly. We believe that this case isof interest since it is extremely rare to findreports of intralenticular foreign bodies withminimal opacification,6 and it is normal prac-tice to remove such lenses. By electing to waitand observe the outcome, we have avoidedsurgery with the subsequent refractive prob-lems that can occur in young patients.

We thank Mr Awdry and Mr Cheng for allowing us toreport this case.

A J E FOSSJ E FORBESJ MORGAN

Oxford Eye Hospital,Walton Street,

OxfordOX2 6HE1Duke-Elder S, MacFaul PA. Injuries. Part 1:

Mechanical. System of Ophthalmology. Vol 14.London: Kimpton, 1972: 352-502.

2 Coleman DJ, Lucas BC, Rondeau MJ, Chang S.Management of intraocular foreign bodies.Ophthalmology 1987; 94: 1647-53.

3 Fisher RF, Wakely J. Changes in lens fibres afterdamage to the lens capsule. Trans Ophthalmol SocUK 1976; 96: 278-84.

4 Blatt N. The tolerance of the crystalline lens tometallic foreign bodies. Am

J Ophthalmol 1930;13: 132-8.

5 Uga S. Wound healing in the mouse lens. Exp EyeRes 1981; 32: 175-86.

6 Keeney A. Intralenticular foreign bodies. ArchOphthalmol 1971; 86: 499-501.

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