Bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis

X-linked juvenile retinoschisis is a rare hereditary retinal disease characterized by a tangential splitting of the neurosensory retina which may cause early-onset visual impairment. Existence of the retinal neurosensory layer splitting on cross-sectional images of optical coherance tomography (OCT) and the absence of leakage on fluorescein angiography (FA) help confirming the diagnosis. Such diagnostic tests are also helpful in determining the management of the disease. However, most of the retinoschisis cavities remain stable and rarely extend to the posterior pole, many authors suggest laser prophylaxis to avoid the potential risk of retinal detachment due to holes in the outer retinal layer. Herein, we report a case with bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis who was evaluated with detailed ophthalmologic examination. Visual acuity, fundoscopy, OCT, and FA remained stable in the second year of follow-up after prophylactic argon laser treatment.     

Autosomal juvenile retinoschisis without foveal retinoschisis.

We describe hereditary retinoschisis without foveal retinoschisis in a girl (the product of a consanguineous marriage) and her father. The father had peripheral retinoschisis with dendritic changes of the retina and good visual acuity. The daughter had peripheral retinoschisis complicated by vitreous haemorrhage and retinal detachment. Neither had foveal retinoschisis. The pattern in a scotopic single-flash electroretinogram differentiated this condition from other forms of retinoschisis. We believe that these cases represent the autosomal recessive form of juvenile retinoschisis without foveal retinoschisis.     

Results of peripheral retinoschisis treatment in sex-linked congenital retinoschisis

Overall study of 20 years of curative and prophylactic peripheral retinoschisis treatment in 60 cases of sex linked congenital retinoschisis. Peripheral retinoschisis was present in 36 out of 120 eyes. Twelve eyes of children under 10 years severely affected, have undergone surgery (8 improvements and 4 aggravations) and 21 cases were photocoagulated (18 stabilizations, 3 aggravations which were subsequently treated by surgery). Six cases were not treated. Aggravations after surgery or photocoagulation have always been observed in early postoperative follow-up period. The above findings bring to light the problems of treatment. An epidemiological study of these 60 patients demonstrates that peripheral retinoschisis into bullous phase is only present under 5 years of age with tendency to regress with age (none reported after age of 50). This study corroborates ad absurdum the results of some authors who think that this type of retinoschisis has a tendency to improve spontaneously, and that indications for prophylactic and curative treatment must be thought of very carefully.     

Postchoroidal retinoschisis]

In the past literature only 8 cases of postchoroidal retinoschisis have been described. Their characteristic finding is a cystic protrusion of the inner retinal layers within an area of post-choroiditic scarring. The lesion is unilateral, and usually located in the temporal or upper fundus periphery; no retinal defects are present, the vitreous is intact, and there is no tendency toward progression. The etiology appears to be different according to age: in patients under age 40, alterations of the glia may contribute to the cyst formation, in older patients, however, the combined damage of the choroidal and retinal vascular bed seem to lead to a retinoschisis.     

X-linked congenital retinoschisis

The natural history and electrophysiological findings of 52 patients with X-linked congenital retinoschisis with a follow-up of up to 26 years are described. The mean visual acuity was reduced to 0.24±0.2 and remained unchanged in most patients during this time. If visual loss occurred, it usually happened in the first decennium. The complications were retinal detachments in 11% and vitreous hemorrhages in 4% of the eyes. In general, the vitreous hemorrhages resolved spontaneously. Retinal detachments were treated successfully with conventional buckling procedures. Redetachments occurred in about 40%. Prophylactic laser coagulation was of no use because it was complicated by detachment in 43% of our series. The electro-oculogram was usually normal. In addition to the known electroretinographic findings of normal a-wave and reduced b-wave amplitudes, we found prolonged b-wave latencies and implicit times, as well as a reduced 30 Hz flicker response.     

Surgical management of retinoschisis

Most cases of X-linked juvenile retinoschisis and degenerative retinoschisis never require any type of surgical intervention. However, on rare occasions, associated problems such as retinal detachment come about and threaten the visual prognosis. Both conditions have in common the adherence of cortical vitreous to the inner layer of peripheral schisis cavities, which can make the secondary complications technically difficult to manage. Although good surgical results can often be obtained, it is not unusual to require relatively advanced vitreoretinal techniques to achieve long-term surgical success.