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Evaluation of Eyes Enucleated for Scleritis 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 Ophthalmology Of all ocular diseases 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 disease, 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 inflammation (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 glaucoma often obscure the inflamma- Clinically, several types of scleritis can be distin- tion of the sclera 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 Endophthalmitis 2 0 Episcleritis I 38-49 50-59 60-69 70-79 80+ Mooren's ulcer I Aqe at time of enucleotion Lens-induced uveitis I FIGURE The average age of patients at the time of Extraocular extension of ciliary body enucleation malignant melanoma I Inflammatory granuloma and staphyloma I much later stage in the disease than it was io years ago. involves the whole circumference of the globe. Peripheral corneal guttering, or keratolysis Reasons for enucleation associated with rheumatoid arthritis, 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 cataract formation, steroids in fluctuating doses, Perforation have produced vasculitis 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.
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