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BritishJournal ofOphthalmology, 1990,74,635-637
Scleral melting in a patient with conjunctivalrhinosporidiosis
RM L De Doncker, R JW de Keizer, J A Oosterhuis, A Maes
AbstractA remarkable case of a scleral melting reactionin association with conjunctival rhinosporidio-sis is presented and its surgical treatment witha scleral graft described.
Rhinosporidiosis is a granulomatous disease ofthe mucous membranes, caused by Rhinospori-dium seeberi, an organism of uncertain taxonomicposition, most probably a fungus. It usuallyinfects the mucous membranes of the nose andnasopharynx but may infect all other mucousmembranes and even the skin. ' Very rare cases ofsystemic infections of the liver and bones havebeen described.The disease is worldwide but endemic in
Africa and Asia, and most frequent in India andSri Lanka. It occurs sporadically in othercountries."A Inoculations of the eye and relatedstructures are grouped as oculosporidiosis.2Most frequently the lacrimal sac is affected byextension from the nasal mucosa. Next mostfrequently the conjunctiva is subject to a primaryinfection, which may spread to the orbital tissue,the sclera, and the eyelids. A scleral defectassociated with conjunctival rhinosporidiosis israre, only two cases having been described. Athird case is presented here.
Department ofOphthalmology,University Hospital,Leiden, The NetherlandsRM L De DonckerR JW de KeizerJ A Oosterhuis
Department ofPathology, University ofLeiden, Leiden, TheNetherlandsA MaesCorrespondence to:R M L De Doncker,Department ofOphthalmology, UniversityHospital, Rijnsburgerweg 10,2333 AA Leiden, TheNetherlands.Accepted for publication26 April 1990
Case reportA healthy 23-year-old white male agriculturistwas referred to the ophthalmic outpatientdepartment by his optician because he hadnoticed a bluish tumour on the temporal sclera ofthe right eye when fitting contact lenses. Accord-ing to the patient this tumour had developed overone year and did not cause any trouble. Therewas no history of ocular disorder or trauma. Thecorrected visual acuity was 1 2 with +3 00 D.
Biomicroscopic examination of the cornea andthe anterior chamber showed no abnormalities.In the inferior temporal part of the eye was abluish circular swelling of the sclera about 4 mmin diameter. The overlying conjunctiva wasmovable over the scleral lesion. More temporaland under the margin of the scleral lesion weredilated conjunctival capillaries and scatteredgrey-white subepithelial corpuscular bodies ofless than 1 mm diameter (Fig 1). The sclerallesion showed dim transillumination. It wasdecided to keep the lesion under observation.
Several months later the patient was examinedbecause of an adenovirus keratoconjunctivitis.The scleral lesion had increased in size, anda strongly protruding scleral ectasia haddeveloped, with considerable transillumination(Fig 2). Fundus examination at the site of thescleral lesion with the Goldmann contact lens
Figure I Scleral lesion (white arrow) and subepithelialconjunctival bodies (black arrow).
revealed no retinal abnormalities. Diascleral Bscan ultrasonography provided an acoustic-freescleral image at that site without signs of chorio-retinal lesions. On fluorescein angiography noabnormal conjunctival or scleral vessels wereseen; the scleral lesion was delineated by hypo-fluorescence (Fig 3). The results of these investi-gations pointed to a scleral defect.
Because ofimpending perforation of the globeit was decided to perform a homograft of pre-served human sclera over the scleral staphyloma.First an argon-laser coagulation of the retinaround the lesion was performed. To obtainmaximal reduction of the intraocular pressureduring surgery mannitol was administeredintravenously, and paracentesis and a largecanthotomy were performed. The abnormal partof the conjunctiva was resected; Tenon's capsulewas kept intact. A 10 mm diameter scleralhomograft was inserted over the 8 mm diameterdefect with interrupted 6-0 silk sutures (Fig 4).In addition a running suture of 10-0 silk wasplaced over the margin of the graft (Fig 5).During a two-year follow-up the conjunctival
Figure 2 Scleral ectasia.
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FigureS A running suture of 10-0 silk is placed over themargin ofthe homograft.
Figure 3 Anterior segment fluorescein anviography ofthescleral lesion.
infection showed no relapse, nor were there signsof graft failure.
HISTOPATHOLOGYLight and electron microscopic examination ofthe conjunctival lesions showed a typical appear-ance. Below a normal epithelium many doublewalled cysts were observed in the conjunctivalstroma, with an inner layer staining stronglypositive with periodic acid Schiff and an outerAlcian blue positive layer. The cysts containedmany vacuoles and eosinophilic granularmaterial (Figs 6, 7, 8). These cysts were typicalof rhinosporidium spores in different stages ofmaturity.
DiscussionConjunctival rhinosporidiosis is a well knownocular infection. Clinically it causes vascular-ised, dark red, polypoid masses.' 6 The surface ofthe polyps is covered with distinct grey-whitespherules (sporangia). The granulomatousmasses can grow to resemble mucosal tumours.7The ratio of nose to eye infections is 4-6/1.8 In
contrast to conjunctival sporotrichosis andcoccidioidomycosis Parinaud's oculoglandularsyndrome never occurs. The mode ofinfection isunknown; experimental inoculation in animalshas been unsuccessful, and cultures of theorganism have failed to grow.9
Transmission from animals to humans hasnever been seen. It is mainly children and young
dse~~~~nj~~~i...Sllllll.||*l~~~~~~~~~~~~l.:'~..........
Figure 4 Preserved sclera is placed over the scleral ectasia byinterrupted sutures of6-0 silk.
male adults who are affected. In all studies6 "' " amale-female ratio of 4 3/1 is found. Epidemio-logical studies showed that conjunctival rhino-sporidiosis has a predilection for agriculturalworkers,'" so that contact with contaminated soilmay be a causative factor. Light and electron-microscopic studies'2 14 have provided mostinformation on the organism. A young tropho-cyte undergoes nuclear division until it containsover 4000 nuclei. The inner layer of the tropho-cyte membrane differentiates, and a maturesporangium containing thousands of endosporesis formed. The free spores can be isolated in thetears of an affected person.
Scleral melting in association with conjunc-tival rhinosporidiosis is very rare, having untilnow been described only twice.6' In contrast tothe normal clinical picture the granulomatousreaction ofthe conjunctiva in our patient was lessobvious; a scleral defect with scattered grey-white subepithelial granules on the margin wasthe main symptom.The clinical diagnosis of this type of sclero-
malacia is based on the concomitant conjunctivalfindings, but light and electronmicroscopicexaminations were required for the confirmationof the diagnosis. A differential diagnosis fromother non-inflammatory scleral melting syn-dromes must be made (Table 1). Scleromalaciaperforans is more frequent than rhinosporidiosisand affects the eyes of persons with seropositiverheumatoid arthritis. Spontaneous intercalaryperforation is unrelated to rheumatoid arthritisand affects persons of a younger age.The only effective treatment is radical surgical
excision followed by thermal and chemicalcoagulation. Nevertheless, relapse is the rulerather than the exception.
Figure 6 Light microscopic section ofpreserved conjunctivallesion. In the lamina propria numerous cysts are seen.(Haematoxylin-eosin, x 30.)
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Scleral melting in a patient with conjunctival rhinosporidiosis
Figure 7 One cyst in detailcontainsgranulareosinophilic material andlipid vacuoles.(Haematoxylin-eosin, * 40x53.)
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3.......;'
Table I A differential diagnosis between the various conditions with scleral melting*
SpontaneousScleromalacia intercalary Hyaline Rhinosporidiosis scleral meltingperforans perforation plaques 3 cases
Age 50-75 25-50 60-75 <25Sex Females Either Either MalesRheumatoid arthritis Constant Unrelated Unrelated UnrelatedBinocularity Bilateral Either Bilateral EitherDistribution Multiple Single Symmetrical SingleInflammation Nil Nil Nil NilPathology Necrosis Unknown Degeneration Unknown
(atrophy?) (immunological or enzymaticPain Nil Nil Nil NilEvolution Progressive Stationary Stationary ProgressivePrognosis Bad Good Good Bad
*Partly reprinted from Duke-Elder S. System ofophthalmology. London: Kimpton, 1965; 8: 1055.
No medical treatment has proved to be effetive in the systemic and mutilating conditionGriseofulvin and amphotericin have been uswithout success. In cases of scleral ectasiahomograft of preserved human sclera gives goresults.The pathological basis of the scleral meltij
reaction in the immediate environs of the cojunctival infection is obscure. The absencedirect contact between conjunctival lesions a]
\..~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.....
Figure 8 Electron micrograph ofa young trophocyte(sporoblast) showing the homogeneous electron-dense DNAmaterial and the lipid bodies. At the top ofthe image thebilaminar wall ofa trophocyte in a later stage ofendosporulation is seen. Notice the inner homogeneouselectron-dense layer (open asterix) and the outer granular layer(arrow). (x2625.)
the scleral defect suggests that an immunologicalor enzymatic process could be the cause. Furtherinvestigation has so far been impossible becauseattempts to culture the spore have been withoutsuccess.
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2 Franqois J, Rysselaere M. Oculomycoses. Springfield: Thomas,g, 1971: 361-9.
3 Neumayr TG. Bilateral rhinosporidiosis of the conjunctiva.Arch Ophthalmol 1964; 71: 379-81.
4 Jimenez JF, Young DE, Hough AJ. Rhinosporidiosis. Areport of two cases from Arkansas. AmJ Clin Pathol 1984;82:611-5.
5 Satyendran OM, Jagannadha Row, Phatak BK, Sarma SR.Rhinosporidiosis of the eye and adnexa. Orient ArchOphthalmol 1965; 3: 332-5.
6 Kuriakose ET. Oculosporidiosis. Rhinosporidiosis of the eye.BrJ Ophthalmol 1963; 47: 346-9.
7 Sharma KD, Shrivastav JB, Agarwal S. Ocular rhinosporidio-sis simulating a tumour. BrJ Ophthalmol 1958; 42: 572-4.
8 Karunaratne WAE. Rhinosporidiosis in man. London: AthlonePress, University of London, 1964.
9 Grover S. Rhinosporidium seeberi: a preliminary study of themorphology and life cycle. Sabouraudia 1970; 7: 249-51.
10 Owor R, Wamukota WM. Rhinosporidiosis in Uganda. A11 review of 51 cases. EastAfrMedJ7 1978; 55: 582-6.11 Kannan Kutty M, Sreedharan T, Mathew KT. Some observa-
Is. tions on rhinosporidiosis. AmJ7Med Sci 1963; 246: 695-701.ed 12 Defrenne P, Dorzee J, Appelmans M, Jansen E. Conjunctiviteprolifrrante par 'Rhinosporidium seeberi'. Bull Soc Belgea Ophthalmol 1953; 104: 247-52.
od 13 Sood NN, Narayana S. Rhinosporidium granuloma of theconjunctiva. J Pediatr Ophthalmol Strabismus 1969; 6:141-4.
ng 14 Savino DF, Margo CE. Conjunctival rhinosporidiosis. Lightand electron microscopic study. Ophthalmology 1983; 90:
Mn- 1482-9.of 15 Lamba PA, Shukla KN, Ganapathy M. Rhinosporidium
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