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Brit. J. Ophthal. (1976) 6o, 227 Br J Ophthalmol: first published as 10.1136/bjo.60.3.227 on 1 March 1976. Downloaded from

Evaluation of eyes enucleated for scleritis

FREDERICK T. FRAUNFELDER* AND PETER G. WATSON From the Scleritis Clinic, Moorfields Eye Hospital, and the Department of Pathology, Institute of

Of all ocular few are more painful or POSTERIOR SCLERITIS serious than scleritis but, as it is a fairly rare The diagnosis of posterior scleritis has in the past , most ophthalmologists have relatively depended on the observation of an accompanying little experience in its diagnosis and management. anterior scleritis, although we know that posterior For the past io years Moorfields Eye Hospital scleritis can occur on its own. Cleary, Watson, has held a weekly scleritis clinic in the hope that McGill, and Hamilton (I975) have recently des- the data obtained might further the understanding cribed the characteristic fluorescein angiographic of this disease. A review of 207 patients with changes and fundus appearances in this condition, scleritis shows that 27 per cent of those with and it may be that the diagnosis will now be made necrotizing scleritis died from an associated more frequently without the need to observe the systemic disease within five years of the onset of the signs of anterior scleritis. ocular (Watson and Hayreh, 1976). In this study the referring surgeons did not use this clinical classification and patients were classified copyright. Method and material as having anterior or posterior scleritis according It was therefore important to discover why eyes with to the observed pathological features (Table I). scleritis had been removed, and, if treatment had been given, why it had failed. Diagnosis A io-year retrospective study of enucleated eyes in which a principal histologic diagnosis of scleritis had The clinical diagnosis of scleritis was not made in

been made was undertaken. Altogether 30 eyes which I2 patients, 40 per cent of the total, although they http://bjo.bmj.com/ fulfilled these criteria were sent from hospitals in all had severe scleral disease histologically (Tables I parts of Great Britain to the Department of Pathology, and II). The diagnosis of an accompanying pos- Institute of Ophthalmology, London, from January terior scleritis was not made in any patient, although 1965 to January 1975. Each patient's record was sent it was present in I3 (43 per cent). None of the to the authors for review, or a questionnaire was com- patients in this series was found to have posterior pleted by the surgeon who had removed the eye. The data giving details of treatment were incomplete in scleritis alone (Table I). five cases and these have been excluded from Table IV. Scleritis is easy to overlook and the condition on September 23, 2021 by guest. Protected A similar review was undertaken by Sevel (i967) should therefore be borne in mind in eyes which Io years ago. This present study is of the same geo- are inflamed and refractory to treatment, present graphical area but it does not include any of Sevel's with exudative detachments, or have pericorneal cases. ulceration. The main physical sign which allows the diagnosis of scleritis to be made is scleral Classification swelling. The severe pain and the accompanying secondary often obscure the inflamma- Clinically, several types of scleritis can be distin- tion of the which is the primary condition. guished: Clinically glaucoma is rare unless the scleritis

ANTERIOR SCLERITIS A diffuse, nodular, necrotizing scleritis with Table I Pathological diagnoses (30 patients) surrounding inflammation, or necrotizing without Necrogranulomatous scleritis Patients surrounding inflammation (scleromalacia perforans). (no.) *Visiting Professor, University of Arkansas School of Medicine Anterior only I7 (sabbatical year) Anterior and posterior 13 Address for reprints: Peter G. Watson, 22 Parkside, Cambridge, Posterior only o England 228 British Journal of Ophthalmology Br J Ophthalmol: first published as 10.1136/bjo.60.3.227 on 1 March 1976. Downloaded from

Table II Clinical diagnoses made in histologically 10 19Total 19 confirmed scleritis (30 patients) 8 O c JTotal II Diagnoses Patients (no.) Anterior scleritis 12 Scleromalacia perforans 6 Malignant or absolute 5 glaucoma 2 4nIL."IIK 2 0 I 38-49 50-59 60-69 70-79 80+ Mooren's ulcer I Aqe at time of enucleotion -induced I FIGURE The average age of patients at the time of Extraocular extension of enucleation malignant melanoma I Inflammatory and staphyloma I much later stage in the disease than it was io years ago. involves the whole circumference of the . Peripheral corneal guttering, or keratolysis Reasons for enucleation associated with , can occur Pain was the reason for enucleation in 8z per cent with or without scleritis and is often a further of the patients (Table III). In two instances a source of confusion. In five cases Mooren's ulcer diagnosis was made of endophthalmitis in func- was at one time diagnosed or suspected, although tionless eyes; another had a massive staphyloma in Mooren's ulcer the accompanying inflammation with impending perforation, and a further patient copyright. is confined to the limbal area and does not extend had a small staphyloma resembling a malignant into the sclera. melanoma. In one case the patient died during When we first analysed the results we thought surgery for repair of an ocular perforation and the there must have been some mistake and that the eye was removed at necropsy; the eyes of patients failure to diagnose scleritis in 40 per cent of patients who had Wegener's granulomatosis and periarteritis was because we did not have sufficient information. nodosa were also obtained at necropsy. that the was It is almost certain, however, scleritis In a series of 207 patients with scleritis seen at http://bjo.bmj.com/ was not recognized or considered to be of im- Moorfields Eye Hospital during the same period, portance because seven of the I2 cases were not only three (I-5 per cent) came to enucleation, given steroids of any kind. In only one of the seven so it is rare indeed to enucleate an eye with scleritis; was there any contraindication for the use of steroids. (Watson and Hayreh, 1976). Pain should not be a major problem since with adequate systemic or- Age at enucleation topical steroids it can be relieved. Possibly some eyes could have been salvaged if the patients In this series there were more women than mer themselves had returned for follow-up, or had on September 23, 2021 by guest. Protected (i 6: i)-almost the same as the ratio found in the large scleritis clinic series (Watson and Hayreh, 1976). The average age of patients at the time of enuclea- Table III Reason for enucleation (30 patients) tion was 67-8 years for women and 69'i years for men (Figure). Many of these patients had had Reason for enucleation Patients scleral disease for up to 30 years before losing the (no.) eye, which correlates with the that the finding Pain with loss of vision average age of onset of necrotizing scleritis in 23 Endophthalmitis 2 patients seen in the scleritis clinic was 35 years. Large staphyloma I Sevel's series of 43 cases completed at the Institute Malignant melanoma I of Ophthalmology from 1950 to I964 had an average age of 63 years and a I 75:I ratio of women Necropsy to men (Sevel, I967). Died during ocular surgery for It is not known why eyes with severe disease repair of perforation I were enucleated five years later in this series Wegener's granulomatosis I than in that of Sevel, although increased steroid Periarteritis nodosa I (Perforation of the eye was not given as a reason for usage might have delayed the final outcome. enucleation) Possibly enucleation is now being undertaken at a Evaluation of eyes enucleated for scleritis 229 Br J Ophthalmol: first published as 10.1136/bjo.60.3.227 on 1 March 1976. Downloaded from

been able to tolerate adequate steroid treatment. a proper reluctance to give adequate suppressive It is well known that patients with severe chronic dosages of steroids in the past, so that the disease disease do not readily take their medication or has been allowed to progress. The dangers of attend for follow-up (Maddock, I967); scleromalacia allowing the disease to progress have to be weighed perforans occurs without inflammation and the against the difficulties of long-term steroid treat- patient does not attend until it is too late. ment. Apart from the well known complications of cushingoid changes, hypertension, glaucoma, and formation, steroids in fluctuating doses, Perforation have produced in patients with rheuma- In i i of 30 cases (37 per cent) the eyes perforated toid arthritis (Hazleman, personal communication) before they were enucleated. and inhibited collagen formation in healing wounds Sevel (I967) pointed out that the clinical diagnosis (Ashton and Cook, I951); they have also been of perforation cannot always be confirmed histo- shown to produce corneal perforation in non-in- logically, since a thin residual layer of sclera may fectious corneal disease (Brown, Weller, and become transparent by dehydration, thus simulating Vidric, I970). However, these drugs have suppres- perforation. An incidence of histologically con- sed the initiating stimulus and reversed the collagen firmed perforation in this series of i i, or 37 per destruction in scleritis associated with connective cent, is comparable to his series. Yet, on the tissue disease (Ashton and Hobbs, 1952). Because pathology request slip or in the clinical record of this, steroids have become standard treatment only once was a massive perforation with a staphy- in the severest cases of scleritis and need to be loma given as the reason for enucleation. In our used at near maximum dosages to control exacer- experience staphylomas rarely perforate, except bations. Once the disease is controlled, the dosage in cases with raised intraocular pressure with or can be gradually reduced to a low maintenance without trauma, and if they should they can be level. This regimen may need to be repeated many managed surgically. times over a 30 to 5o-year span in many unfortunate patients. copyright. Effect of treatment Other changes found in the enucleated eyes was initiated to see This study partly if sub- Eyes enucleated for scleritis showed a high incidence conjunctival steroid injections predisposed to of other ocular In the six who not disease (Table II). Uveitis, primarily scleral perforation. patients did anterior, was the most common complication and receive any steroids, none perforated, while i I occurred in 68 cent of of i9 (nine of I4 topical and/or two of per cases. Glaucoma, which systemic, occurred in http://bjo.bmj.com/ five subconjunctival) who were on some form of 46 per cent, was often severe and steroid treatment did These frequently associated with peripheral anterior perforate (Table IV). synechiae. Three of these patients also had a data are, however, insufficient to differentiate which retinal vein or form of steroid delivery was more often associated artery occlusion. Retinal disease was with scleral perforation. Investigators have sug- found in 46 per cent and included non-rhegmato- gested that subconjunctival atropine (Tooker, genous retinal detachments (21 per cent), chorio- 1931; Swan, I951) or steroids (Sevel and Abram- , macular oedema, and macular degenera- son, 1972; Watson, 1974) may cause a necro- tion. Six patients had intravitreal or suprachoroidal on September 23, 2021 by guest. Protected granulomatous scleritis. Although the evidence is haemorrhages. Rubeosis, corneal ulcers, and des circumstantial, Sevel found the incidence of corneal cemetoceles were seen in each of three patients. perforation to be the same in those cases in which This high complication rate of scleritis was eyes had been enucleated before the general intro- not unexpected, although uveitis and glaucoma duction of steroids, as afterwards. There has been were seen more frequently in this series than in the scleritis clinic (Watson and Hayreh, 1976). In this series 68 per cent had uveitis as opposed to Table IV Method of steroid delivery and 37 per cent in the clinic, and 46 per cent of the incidence of ocular perforation (25 patients) enucleated eyes showed glaucoma as opposed to 28 per cent in the clinic. This suggested that scleritis Ocular with uveitis and glaucoma was an ominous sign. Steroids Cases perforation Per- The fact that 21 per cent of cases had intraocular (no.) (no.) centage haemorrhage and three eyes had vascular occlusion associated with None 6 o o scleritis was unexpected, since these Topical or systemic 14 9 57 complications are rarely seen clinically and might Subconjunctival injection 5 2 40 possibly have been related to the glaucoma. How many of the haemorrhages occurred during enu- 230 British Journal of Ophthalmology Br J Ophthalmol: first published as 10.1136/bjo.60.3.227 on 1 March 1976. Downloaded from

cleation is not known. The incidence of retinal cent, pain was the reason for enucleation, which detachment and cataract was less than that found by suggests that some patients were on inadequate Sevel (I967). levels of steroid treatment or were unable to tolerate them. In the series, 37 per cent of the eyes per- forated. Perforation occurred in those patients Summary who were having steroids but not in those who were A series of 30 enucleated eyes, all of which had a not, but the data are inconclusive as to which primary histological diagnosis of scleritis, was method of steroid administration was most likely analysed. The average age of patients at enucleation to cause ocular perforation. was 68 years; many of these patients had had the disease for more than 30 years. In 40 per cent the The authors wish to acknowledge the help of Professor diagnosis of scleritis was unsuspected and was Norman Ashton for his advice and encouragement often masked by multiple complications. Scleritis in the preparation of this paper, and also to thank with uveitis and glaucoma was the most common Mrs Jane Field for obtaining records of patients combination to come to enucleation. In 82 per throughout Great Britain.

References ASHTON, N., and COOK, C. (I95i) Brit. 5. Ophthal., 35, 708 , and HOBBS, H. E. (1952) Ibid., 36, 373 BROWN, S. J., WELLER, C. A., and VIDRIC, A. (1970) Amer. Y. Ophthal., 70, 744 CLEARY, P. E., WATSON, P. G., MCGILL, J. I., and HAMILTON, A. M. (I975) Trans. ophthal. Soc. U.K., 95, 297 MADDOCK, R. K. (I967) Y. Amer. med. Ass., I99, I69 SEVEL, D. (I967) Amer. Y. Ophthal., 64, 1125 and ABRAMSON, A. (I972) Brit. Y. Ophthal., 56, 79I copyright. SWAN, K. C. (195I) Arch. Ophthal., 45, 630 TOOKER, C. W. (193I) Amer. 5. Ophthal., 3,9II WATSON, P. G. (I974) Trans. ophthal. Soc. U.K., 94, 76 , and HAYREH, S. S. (1976) Brit. Y. Ophthal., 6o, I63 http://bjo.bmj.com/ on September 23, 2021 by guest. Protected