Abnormal Vitreoretinal Interface Leading to Macular Hole-Associated Retinal Detachment in Straatsma Syndrome

Introduction: To report a case of macular hole (MH)-associated retinal detachment (RD) with Straatsma syndrome. Methods: A clinical case was retrospectively reviewed. Results: A 39-year-old woman with Straatsma syndrome was treated for an MH-associated RD. The MH was closed with vitrectomy and internal limiting membrane peeling, during which an unusually strong vitreoretinal adhesion was encountered in the area of myelinated nerve fibers. One day postoperatively, the patient developed serous choroidal effusions and a serous RD, which resolved by postoperative week 1. The patient's vision ultimately returned to baseline. Conclusions: The presence of an unusually strong vitreoretinal adhesion in the area of myelinated nerve fibers and the development of a postoperative serous RD suggest that myelination may contribute to vitreoretinal adhesion and the development of MHs in Straatsma syndrome.