ACTA O P H T H A L M O LOG I CA 72 (1994) 533-536

Diffuse chronic retinal pigment epitheliopathy and exudative retinal detachment

Leila Laatikainen

Department of Ophthalmology, University of Oulu, Oulu, Finland

Abstract. Two patients with bilateral chronic retinal pigment epitheliopathy are presented. Both patients had large areas of pigment epithelial decompensation and small pigment epithelial detachments at the posterior pole. In the macula subretinal fluid was scanty, but the first patient developed an extensive bullous retinal de- tachment with shifting of subretinal fluid with changes in posture in both eyes, the second patient had similar de- tachment in one eye. The etiology of the pigment epithe- lial disorder remained unknown. No inflammatory cells were found in the vitreous specimen or subretinal fluid in the first patient. Treatment with peroral corticoste- roids alone or in combination with azathioprine and cy- closporin A, as well as surgery for retinal detachment in one eye, proved unsuccessful. Argon laser coagulation of the decompensated areas in the macula resulted in re- sorption of subretinal fluid and reattachment of the exu- dative detachment.

Key words: exudative retinal detachment - pigment epi- theliopathy - central serous chorioretinopathy.

In 1973, Gass reported bullous exudative retinal detachment in 5 patients with atypical central ser- ous choroidopathy. All patients had one or more localized serous detachments of the retinal pig- ment epithelium (RPE) at the posterior pole. A severe form of central serous chorioretinopathy (CSC) was later named as diffuse retinal pigment epitheliopathy by Zweng et al. (1977). The charac- teristic features in diffise retinal pigment epithe- liopathy are chronic course, multiple recurrences, often bilateral occurrence, extensive changes in the pigment epithelium but only little subretinal fluid and worse visual prognosis than in the clas- sical form of central serous chorioretinopathy

(Zweng et al. 1977; Benson et al. 1980; von Winning et al. 1982; Cohen et al. 1983; de Bustros et al. 1984; Yannuzzi et al. 1984; Roseman & Olk 1988; Bran- cato & Bandello 1991). In some of these cases bul- lous exudative retinal detachment has been no- ticed. The treatment of this potentially blinding condition is not well established. In this paper I re- port our experiences in two such cases.

Patients Case 1 A 45-year-old maq had had intermittent visual symptoms in both eyes since 1987, and in 1991 the visual acuity in the left eye decreased to 0.1 (20/200) due to pigment clumping in the fovea. Biomicroscopic examination revealed no Mam- matory signs in the anterior chamber or vitreous in either eye. By ophthalmoscopy large areas of whit- ish thickening at the level of the retinal pigment epithelium (RPE) (Fig. 1A) as well as small pigment epithelial detachments and pigment epithelial atrophy were noticed at the posterior pole in both eyes. On some of these lesions the neuroretina was slightly elevated and retinal capillaries were di- lated. Fluorescein angiography revealed several small pigment epithelial detachments and large areas of fluorescein staining indicating marked de- compensation of the RPE at the posterior pole (Fig. 2). In repeated angiograms definite progres- sion of the lesions over months could be seen.

In 1992 the visual acuity of the right eye de- creased to 0.01 due to extensive fresh lesions in the macula. At the same time bullous exudative retinal

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A

B

C

Figs. I A - C. Case 1. Extensive subretinal yellowish infiltrates at the posterior pole of the right eye in February 1993 (A). Pig- mented scar in the fovea surround by active subretinal le- sions and bullous retinal detachment in the inferior quadrants in the left eye in April 1993 (B). In September 1993, the left fundus showed laser scars and subretinal fi- brosis but the retina had reattached (C).

detachment with shifting subretinal fluid with changes in posture developed in both eyes (Fig. 1B).

This patient had had arterial hypertension and

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signs of coronary disease since 1987, and in 1987 he had also had an operation for an aneurysms in the anterior communicating artery. Because of these disorders he had an ACE inhibitor, nitroglycerin, aspirin and dipyridamol medication. Extensive la- boratory workup disclosed an increased level of rheumatoid factor (293 W/l, normal range <25 IU/l) and antinuclear antibodies were weak posi- tive (1/40). Serum ACE level and lysozyme were normal, cardiolipin was negative and no borrelia and HIV antibodies were detected. Medical work- up did not discover signs of sarcoidosis or rheuma- toid diseases.

In spite of negative results in medical and labor- atory workup, an inflammatory etiology was sus- pected. Therefore treatment with peroral predni- solone starting from 60 mg/day and later with a combination of prednisolone, cyclosporin A and azathioprine was tried, but the treatment was not successful. On the contrary, during cortison treat- ment total retinal detachment developed in the right eye. Vitrectomy, subretinal drainage, periph- eral cryocoagulation and intraocular silicone oil filling were performed, but the eye became blind. No inflammatory or malignant cells were found in the vitreous and the subretinal fluid specimens.

In the left eye, argon laster treatment on the de- compensated areas in the W E performed in sev- eral sessions resulted in gradual resorption of sub- retinal fluid and reattachment of the retina (Figs. 1C and 2).

Case 2 A 38-year-old woman had complained of periods of blurred vision since 1992, first in her left eye. Several areas of whitish subretinal thickening at the posterior pole were observed. These resulted in pigmentary changes and deterioration of vision in both eyes. Both ophthalmoscopic and fluores- cein angiographic findings were less marked than in the previous case. No inflammatory signs were observed in the anterior chamber or vitreous. This patient had no general diseases or systemic medi- cation, and medical and laboratory investigation did not reveal any etiologic cause for ocular changes.

Administration of peroral prednisolone seemed to have no effect. During treatment bullous exuda- tive retinal detachment developed in the inferior quadrants in the left eye. At the same time subreti- nal neovascular membrane appeared nasal to the

A

C

B

D

Figs. 2 A - D. Fluorescein angiogram of the left eye taken after the first laser treatment showed dilated retinal capillaries (A) and marked staining (B) in the area of the inferior temporal vascular arcade as well as accumulation of fluorescein in localized pigment epithelial detachments in the papillomacular area and above the fovea (B, arrows). Two months later, after the second laser treatment session leakage had decreased remarkably and Subretinal fluid had almost reap- sorbed (C, D).

optic disc. Treatment of the neovascular mem- brane and the decompensated area in the macula with argon laser resulted in resorption of the sub- retinal fluid and in at least temporary improve- ment in the patient’s condition.

Discussion

The pathogenesis of central serous chorioretino- pathy is poorly understood. Most authors believe that the cause of serous retinal detachment in the macula is a disturbance in the RPE, which, on the other hand, may be caused by ischaemic, degener- ative, inflammatory or other unknown factors. In the classical CSC the long-term visual prognosis is usually good in spite of recurrences and remaining pigment epithelial changes (von Dickhoff et al.

1989). Also, small multiple pigment epithelial de- tachments in young people usually dry without serious consequences (Lewis 1978; Laatikainen & Hoffren 1991). In a few cases of CSC, particularly in those with multiple PE detachments, bullous reti- nal detachment has, however, been reported (Gass 1973).

Zweng et al. (1977) described ‘diffuse retinal pig- ment epitheliopathy’ as a variant of CSC. Both con- ditions occur most commonly in men aged 30 to 50 years. No etiological factors for pigment epithelial changes in diffuse retinal pigment epitheliopathy and exudative retinal detachment can usually be found (Tsukahara & Uyama 1978; Benson et al. 1980). In one of the three cases reported by de Bu- stros et al. (1984) an infectious cause by a low-vi- rulence organism was suspected because of the pa- tient’s history of frequent infections. Cytologic

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examination of the subretinal fluid specimen in another case did not show any causative organisms and only a few macrophages and polymorphonu- clear leucocytes were discovered (de Bustros et al. 1984). We took vitreous and subretinal fluid speci- mens in one eye in connection with detachment surgery, but no inflammatory or malignant cells were found.

In our cases exudative retinal detachment de- veloped or progressed while the patients were on peroral corticosteroid medication. Deterioration of exudative retinal detachment and reduction of vision in eyes with diffuse pigment epitheliopathy during peroral corticosteroid treatment was also observed by Mazzuca & Benson (1986), and several authors report no response to systemic steroids (Gass 1973; Benson et al. 1980; Tsukahara & Uyama 1978; de Bustros e t al. 1984).

In our cases the only efficient treatment proved to be laser treatment of the decompensated RPE. The advantageous effect of photocoagulation on the exudative retinal detachment related to diffuse retinal pigment epitheliopathy has been demon- strated by several investigators (Gass 1973; Benson e t al. 1980; Tsukahara & Uyama 1978; de Bustros et al. 1984; Yannuzzi e t al. 1984; Mazzuca & Benson 1986; Roseman & Olk 1988).

Acknowledgments Ophthalmologists at the Eye Departments in Seinajoki Hospital and Vaasa Central Hospital are thanked for sending these patients. This paper was presented at the seventeenth annual meting of the Macular Society, Fe- bruary 1994, Rancho Mirage, California.

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Received on March 29th, 1994.

Corresponding author:

Prof. L. Laatikainen Department of Ophthalmology Oulu University Hospital SF-90220 Oulu, Finland.

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