Scleral Melting in a Patient with Conjunctival Br J Ophthalmol: First Published As 10.1136/Bjo.74.10.635 on 1 October 1990
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BritishJournal ofOphthalmology, 1990,74,635-637 635 Scleral melting in a patient with conjunctival Br J Ophthalmol: first published as 10.1136/bjo.74.10.635 on 1 October 1990. Downloaded from rhinosporidiosis R M L De Doncker, R J W de Keizer, J A Oosterhuis, A Maes Abstract A remarkable case ofa scleral melting reaction in association with conjunctival rhinosporidio- sis is presented and its surgical treatment with a scleral graft described. Rhinosporidiosis is a granulomatous disease of the mucous membranes, caused by Rhinospori- dium seeberi, an organism ofuncertain taxonomic position, most probably a fungus. It usually infects the mucous membranes of the nose and nasopharynx but may infect all other mucous membranes and even the skin.' Very rare cases of systemic infections of the liver and bones have been described. Figure I Scleral lesion (white arrow) and subepithelial The disease is worldwide but endemic in conjunctival bodies (black arrow). Africa and Asia, and most frequent in India and Sri Lanka. It occurs sporadically in other revealed no retinal abnormalities. Diascleral B countries."A Inoculations of the eye and related scan ultrasonography provided an acoustic-free structures are grouped as oculosporidiosis.2 scleral image at that site without signs of chorio- Most frequently the lacrimal sac is affected by retinal lesions. On fluorescein angiography no extension from the nasal mucosa. Next most abnormal conjunctival or scleral vessels were frequently the conjunctiva is subject to a primary seen; the scleral lesion was delineated by hypo- infection, which may spread to the orbital tissue, fluorescence (Fig 3). The results of these investi- the sclera, and the eyelids. A scleral defect gations pointed to a scleral defect. associated with conjunctival rhinosporidiosis is Because ofimpending perforation of the globe http://bjo.bmj.com/ rare, only two cases having been described. A it was decided to perform a homograft of pre- third case is presented here. served human sclera over the scleral staphyloma. First an argon-laser coagulation of the retina round the lesion was performed. To obtain Case report maximal reduction of the intraocular pressure A healthy 23-year-old white male agriculturist during surgery mannitol was administered was referred to the ophthalmic outpatient intravenously, and paracentesis and a large on September 27, 2021 by guest. Protected copyright. department by his optician because he had canthotomy were performed. The abnormal part noticed a bluish tumour on the temporal sclera of ofthe conjunctiva was resected; Tenon's capsule the right eye when fitting contact lenses. Accord- was kept intact. A 10 mm diameter scleral ing to the patient this tumour had developed over homograft was inserted over the 8 mm diameter one year and did not cause any trouble. There defect with interrupted 6-0 silk sutures (Fig 4). was no history ofocular disorder or trauma. The In addition a running suture of 10-0 silk was corrected visual acuity was 1 2 with +3 00 D. placed over the margin of the graft (Fig 5). Biomicroscopic examination ofthe cornea and During a two-year follow-up the conjunctival the anterior chamber showed no abnormalities. Department of In the inferior temporal part of the eye was a Ophthalmology, bluish circular University Hospital, swelling of the sclera about 4 mm Leiden, The Netherlands in diameter. The overlying conjunctiva was R M L De Doncker movable over the scleral lesion. More temporal R J W de Keizer and under the margin of the scleral lesion were J A Oosterhuis dilated conjunctival capillaries and scattered Department of grey-white subepithelial corpuscular bodies of Pathology, University of less than 1 mm diameter (Fig 1). The scleral Leiden, Leiden, The Netherlands lesion showed dim transillumination. It was A Maes decided to keep the lesion under observation. Correspondence to: Several months later the patient was examined R M L De Doncker, because of an adenovirus Department of keratoconjunctivitis. Ophthalmology, University The scleral lesion had increased in size, and Hospital, Rijnsburgerweg 10, a strongly protruding scleral 2333 AA Leiden, The ectasia had Netherlands. developed, with considerable transillumination Accepted for publication (Fig 2). Fundus examination at the site of the 26 April 1990 scleral lesion with the Goldmann contact lens Figure 2 Scleral ectasia. 636 De Doncker, de Keizer, Oosterhuis, Maes Br J Ophthalmol: first published as 10.1136/bjo.74.10.635 on 1 October 1990. Downloaded from FigureS A running suture of10-0 silk is placed over the margin ofthe homograft. Figure 3 Anterior segment fluorescein anviography ofthe scleral lesion. male adults who are affected. In all studies6 "' " a male-female ratio of 4 3/1 is found. Epidemio- logical studies showed that conjunctival rhino- infection showed no relapse, nor were there signs sporidiosis has a predilection for agricultural ofgraft failure. workers,'" so that contact with contaminated soil may be a causative factor. Light and electron- microscopic studies'214 have provided most HISTOPATHOLOGY information on the organism. A young tropho- Light and electron microscopic examination of cyte undergoes nuclear division until it contains the conjunctival lesions showed a typical appear- over 4000 nuclei. The inner layer of the tropho- ance. Below a normal epithelium many double cyte membrane differentiates, and a mature walled cysts were observed in the conjunctival sporangium containing thousands of endospores stroma, with an inner layer staining strongly is formed. The free spores can be isolated in the positive with periodic acid Schiff and an outer tears ofan affected person. Alcian blue positive layer. The cysts contained Scleral melting in association with conjunc- many vacuoles and eosinophilic granular tival rhinosporidiosis is very rare, having until material (Figs 6, 7, 8). These cysts were typical now been described only twice.6' In contrast to of rhinosporidium spores in different stages of the normal clinical picture the granulomatous maturity. reaction ofthe conjunctiva in our patient was less obvious; a scleral defect with scattered grey- http://bjo.bmj.com/ white subepithelial granules on the margin was Discussion the main symptom. Conjunctival rhinosporidiosis is a well known The clinical diagnosis of this type of sclero- ocular infection. Clinically it causes vascular- malacia is based on the concomitant conjunctival ised, dark red, polypoid masses.' 6 The surface of findings, but light and electronmicroscopic the polyps is covered with distinct grey-white examinations were required for the confirmation spherules (sporangia). The granulomatous of the diagnosis. A differential diagnosis from on September 27, 2021 by guest. Protected copyright. masses can grow to resemble mucosal tumours.7 other non-inflammatory scleral melting syn- The ratio of nose to eye infections is 4-6/1.8 In dromes must be made (Table 1). Scleromalacia contrast to conjunctival sporotrichosis and perforans is more frequent than rhinosporidiosis coccidioidomycosis Parinaud's oculoglandular and affects the eyes of persons with seropositive syndrome never occurs. The mode ofinfection is rheumatoid arthritis. Spontaneous intercalary unknown; experimental inoculation in animals perforation is unrelated to rheumatoid arthritis has been unsuccessful, and cultures of the and affects persons ofa younger age. organism have failed to grow.9 The only effective treatment is radical surgical Transmission from animals to humans has excision followed by thermal and chemical never been seen. It is mainly children and young coagulation. Nevertheless, relapse is the rule rather than the exception. dse~~~~nj~~~i...Sllllll.||*l~~~~~~~~~~~~l.:'~.......... Figure 6 Light microscopic section ofpreserved conjunctival Figure 4 Preserved sclera is placed over the scleral ectasia by lesion. In the lamina propria numerous cysts are seen. interrupted sutures of6-0 silk. (Haematoxylin-eosin, x 30.) Scleral melting in a patient with conjunctival rhinosporidiosis 637 Figure 7 One cyst in detail Br J Ophthalmol: first published as 10.1136/bjo.74.10.635 on 1 October 1990. Downloaded from contains granular eosinophilic material and lipid vacuoles. (Haematoxylin-eosin, * 40 x53.) 0;.. .. .....I,, ads~~ \..~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~..... w _ ... ....., ..i.4|.... *>: Figure 8 Electron micrograph ofa young trophocyte (sporoblast) showing the homogeneous electron-dense DNA material and the lipid bodies. At the top ofthe image the bilaminar wall ofa trophocyte in a later stage of 3 endosporulation is seen. Notice the inner homogeneous electron-dense layer (open asterix) and the outer granular layer (arrow). (x2625.) .......;' the scleral defect suggests that an immunological or enzymatic process could be the cause. Further investigation has so far been impossible because attempts to culture the spore have been without success. Table I A differential diagnosis between the various conditions with scleral melting* 1 Desai SC. Ein Uberblick uber die Rhinosporidiose. Mykosen 1961; 4: 64-8. Spontaneous 2 Franqois J, Rysselaere M. Oculomycoses. Springfield: Thomas, Scleromalacia intercalary Hyaline Rhinosporidiosis scleral meltingg, 1971: 361-9. perforans perforation plaques 3 cases 3 Neumayr TG. Bilateral rhinosporidiosis of the conjunctiva. Arch Ophthalmol 1964; 71: 379-81. Age 50-75 25-50 60-75 <25 4 Jimenez JF, Young DE, Hough AJ. Rhinosporidiosis. A Sex Females Either Either Males report of two cases from Arkansas. AmJ Clin Pathol 1984;