SURGICAL TREATMENT OF A MACULAR HOLE IN X-LINKED RETINOSCHISIS

To report a case of full-thickness macular hole in a patient with X-linked retinoschisis and the outcome after surgical repair.

A 15-year-old boy with a history of X-linked retinoschisis presented to his ophthalmologist for routine follow-up and was found to have a Stage-3 macular hole in his left eye. His vision was 20/200. The patient underwent pars plana vitrectomy and internal limiting membrane peeling, and he received long-acting gas. Color fundus photography and spectral domain optical coherence tomography (Cirrus; Carl Zeiss Meditech Inc, Dublin, CA) recorded images at office visits, before and after surgical repair.

The initial spectral domain optical coherence tomography of the left eye showed a full-thickness macular hole of 1,370 μm in diameter as well as schisis cavities in the macula. After pars plana vitrectomy and repeat fluid-gas exchange, the hole was closed at the sixth-week follow-up visit. The patient noted a subjective decrease in the size of a central scotoma. Best-corrected visual acuity returned to the baseline of 20/80.

A large full-thickness macular hole can develop in X-linked retinoschisis, and pars plana vitrectomy with internal limiting membrane peeling may be helpful for successful surgical closure.