Retinal dystrophies associated with peripheral retinal vasculopathy.

Abstract

SUMMARY A pair of identical twins with cone-rod dystrophy and 1 patient with dominant retinitis pigmentosa are reported who had a peculiar vascular retinopathy characterised by perivenous accumulation of fluorescein in the temporal periphery. Other retinal dystrophies associated with leaking retinal vessels are discussed.

Retinitis pigmentosa and inner retina. Functional study by means of oscillatory potentials of the electroretinogram

“…The aforementioned experimental studies seem to exclude any alteration of neural retina until the latest stages of the disease, when inner retina degenerates because of melanin and lipofuscin deposits. Clinical reports described Coats-like vascular abnormalities with subretinal exudation, generalized subretinal alterations, and cystoid macular edema in retinitis pigmentosa [24,25,26]. The fluorescein angiography better defined the general impairment of the vascular retinal layer: the arteries were described as narrow, containing dye in a concentration lower than usual, with abnormal delay in filling of the arterial and venous vessels [9,27].…”

Cases reported at the FAN club meeting, Bonn, November 1984

“…Retinal vascular changes in association with retinitis pigmentosa has been previously reported (1)(2)(3)(4)(5)(6)(7)(8)(9). Generalised permeability of retinal capillaries, cystoid macular oedema, and Coats'-like retinal vascular abnormalities with subretinal exudation, and retinal detachment have been described (2-6) seen in retinitis pigmentosa (4)(5)(6).…”

Immunological studies in retinitis pigmentosa associated with retinal vascular leakage.

“…Retinal vascular leakage in patients with retinitis pigmentosa (RP) is well recognised (Spalton et al, 1978;ffytche, 1972;Grizzerd et al, 1978), and we have recently studied a number of these patients (Spalton et al, 1978).…”