Foveal Schisis as a Cause of Retinal Detachment Secondary to Macular Hole in Juvenile X-Linked Retinoschisis

Diagnostic and Therapeutic Challenges

“…Macular hole development is extremely rare in eyes with XLRS 3-5 and has not, to my knowledge, been reported previously in eyes with isolated foveal retinoschisis. 4 However, in the case described here and in two other reported cases of macular hole complicating XLRS, 4,5 the pathogenic mechanisms responsible for macular hole formation are not obvious. 13,14 Foveal destabilization by traction-induced foveal damage preceding vitreofoveal separation is likely also to explain the occasional development of idiopathic macular hole in eyes with preexisting PVD.…”

“…[3][4][5] Because of the rarity of macular hole in this setting, we have no other published experience to guide us in treatment. [3][4][5] Because of the rarity of macular hole in this setting, we have no other published experience to guide us in treatment.…”

A Female Case of X-Linked Retinoschisis with Macular Hole Bilaterally

“…Our case had also RS1 mutation. Most of the cases reported in the literature have male gender [1][2][3][4][5][6]. One of the conditions that make our case special is her gender.…”

Bilateral macular holes in X-linked retinoschisis: Now the spectrum is wider

“…Although cortical vitreous is strongly adherent to the internal limiting membrane in some cases of XLR, and exerts a tangential traction on the macula, dragging it nasally, particularly when vitreous veils create temporal redundancy,[6] no such observations can be made in our case. In another case report Shanmugam[7] has described unilateral macular hole in an XLR patient associated with retinal detachment. OCT images provided by the author showed prominent vitreoretinal traction.…”