Sex linked juvenile retinoschisis with optic disc and peripheral retinal neovascularisation.

Abstract

We wish to report a case of sex linked juvenile retinoschisis with optic disc and peripheral retinal neovascularisation. Both forms of neovascularisation resolved following panretinal photocoagulation. Optic disc neovascularisation has not been previously reported with this condition, though peripheral retinal vascular anomalies are well recognised. Case reportThe patient, a 24-year-old white male, first attended Moorfields Eye Hospital at the age of 7 years when referred with poor visual acuity at school eye testing. At that time his best corrected visual acuities were Snellen 6/36 in the right and 6/24 in the left eye. Slitlamp biomicroscopic examination revealed bilateral foveal retinoschisis in a 'wheel-like' configuration ( Fig. 1). Peripheral retinoschisis was absent. A general examination gave normal results. Low visual aids were dispensed when, at the age of 10 years, binocular distance visual acuity had deteriorated to 6/60 unaided.There was a family history of sex linked retinoschisis, the patient's maternal grandfather having the condition. The mother and the twin sister of the patient were not, however, affected.At the age of 20 the patient was re-examined after developing a symptomatic floater in the visual field of the right eye. His best corrected visual acuities at this time were 1/60 right and 6/36 left. Vitreous haemorrhage precluded examination of the fundus of the right eye. The right visual acuity improved to 6/36 as the vitreous haemorrhage cleared, but no source for the haemorrhage could be detected clinically at this stage. A further three vitreous haemorrhages occurred in the right eye during the next seven months, after which time fine, forward new vessels were detected at the right optic disc (Fig. 2) together with tortuosity of the peripheral retinal vessels. Flat new vessels in the retinal periphery were observed in the superonasal quadrant of the left eye.Correspondence to Mr R Pearson, FRCS. 311Fluorescein angiography showed leakage of the dye from new vessels originating on the right optic disc (Fig. 3) and in the retinal periphery, together with areas of non-perfusion of the retinal capillary circulation in the periphery. There was leakage from peripheral retinal new vessels in the left eye, but no disc neovascularisation was present.Panretinal photocoagulation of the right eye with argon blue-green laser was administered in three sessions over a five-month period, with regression of the neovascularisation within four months of the final treatment session (Fig. 4). A horizontal traction fold of the internal limiting membrane was noted to have formed accross the right macula (Fig. 4). Laser treatment was not administered to the left eye, and the peripheral new vessels underwent sheathing followed by spontaneous resolution.Three years after panretinal photocoagulation the best attainable visual acuities were 6/36 right, and 6/60 left. Ophthalmoscopy showed bilateral cystoid macular oedema, peripheral retinoschisis, patchy sheathing of peripheral retinal vessels, many of which were ...

Sickle cell retinopathy. A focused review

“…A wide spectrum of diseases that result in either macular ischemia, or peripheral retinal neovascularization, should be considered in the differential diagnosis [40][41][42][43][44][45] (Table 2). On the other hand, sickle retinopathy should be considered as a differential diagnosis in retinal vascular disease.…”

X linked retinoschisis.

“…In contrast with senile retinoschisis, outer leaf breaks are rare in XLRS. Vascular changes are a prominent feature of the peripheral retina in XLRS: perivascular sheathing23 (Fig 5), dendritiform patterns,30 microvascular anomalies, and even neo-vascularisation3l 32 have been reported. A superficial silvery reflex arising from the vitreoretinal interface (Fig 6) may also be seenl026 and a tapetal reflex in association with the Mizuo phenomenon has been described.…”

Unusual peripapillary new vessels in eye with central serous chorioretinopathy

“…Apart from retinal detachment, inferior retinoschisis in juvenile X‐linked retinoschisis has also been noted to cause retinal and optic disc neovascularisation. This retinoschisis leads to splitting of retinal layers and the mechanism of optic disc neovascularisation in these eyes has been thought to be the ischaemia of the peripheral retina, secondary to obliteration of retinal capillaries in the split layers of the peripheral retina . Our patient had inferior retinal detachment and eventually developed arborising peripapillary vessels.…”