Hypotonic maculopathy secondary to scleral defect in atypical retinochoroidal coloboma

Purpose To describe a patient with unilateral hypotonic maculopathy and optic disc edema after spontaneous bulbar perforation of a full-thickness scleral defect. Methods An 11-year-old girl underwent scleral buckling surgery. Preoperative and postoperative evaluation included optical coherence tomography and high-resolution 3T magnetic resonance imaging. Results The scleral defect was covered with Tenon capsule sheaths and a meridional silicone buckle. One year postoperatively, visual acuity and macular and optic disc morphology were completely restored. Conclusions Ocular hypotony related to a defect of the sclera forming the roof of a retinochoroidal coloboma is a rare event. Hypotony was a consequence of a defect at the level of an atypical retinochoroidal coloboma where the scleral wall was found to be absent.