Stickler Syndrome is the most common inherited cause of rhegmatogenous retinal detachments (RD) [1]. It is a collagen disorder identified by the ocular manifestations of abnormal vitreous development and unusually high myopia, with systemic findings of craniofacial abnormalities, hearing loss, and arthropathy [2]. Approximately 40–60% of patients with Stickler Syndrome will develop a rhegmatogenous RD in their lifetime [3]. Several factors contribute to the complexity of RD repair in patients with Stickler Syndrome, including the presence of multiple retinal tears peripherally and posteriorly, giant retinal tears (GRT), proliferative vitreoretinopathy (PVR), young age at presentation, and an abnormal vitreous with an anomalous vitreoretinal interface. These considerations present a therapeutic challenge to the vitreoretinal surgeon for prophylaxis strategies and for long-term sustained retinal reattachment.
Retrospective studies have demonstrated that we are able to achieve high levels of anatomic success in Stickler Syndrome-associated RD, though this often requires multiple surgeries due to recurrent RD [4]. In this study, we aimed to assess factors leading to the development of recurrent RD and characterize features of the eyes at recurrence. Furthermore, because patients often present early in life, we wanted to explore the development of late recurrent RD.