Vitrectomy for Bloch-Sulzberger syndrome progressing to macular tractional retinal detachment

A 8-year-old girl was referred to us for failing vision in her right eye. She had been diagnosed with Bloch-Sulzberger syndrome during infancy. Her corrected visual acuity was 0.2 right and 1.2 left. No gross abnormalities were present regarding refraction, intraocular pressure and anterior ocular segment. The right eye showed exudative and tractional retinal detachment involving the macula. The whole peripheral retina had become avascular in both eyes. The retinal detachment progressed in spite of transscleral cryocoagulation, necessitating vitreous surgery. Following lensectomy, the proliferative membrane in the posterior fundus was delaminated and removed. Artificial detachment of posterior vitreous was created towards the periphery in spite of marked vitreoretinal adhesion in the peripheral avascular retinal area. The retina became reattached and the visual acuity improved from 0.05 to 0.2. Presence of marked vitreoretinal adhesion in the periphery appeared to be a characteristic feature of this syndrome similar to retinopathy of prematurity and familial exudative vitreoretinopathy.