Rhegmatogenous Retinal Detachment in Morning Glory Syndrome

Abstract

Morning Glory syndrome, or congenital optic disc dysplasia, is a rare autosomal dominantly inherited anomaly, with a prevalence of 2.6/100 000, described by Kindler in 1970. Findings include funnel-shaped excavation of the optic disc, markedly enlarged, central glial tissue, peripapillary chorioretinal pigmentation, and spoke-like vessels. The most serious complication is retinal detachment, which is seen in 1/3 of cases. Three mechanisms have been proposed: rhegmatogenous, tractional and exudative. In rhegmatogenous retinal detachment, retinal tears tend to be commonly located at the margin or in the retina within the optic disc; optical coherence tomography is very useful for their identification. Pars plana vitrectomy, peripapillary laser endophotocoagulation and the use of long-acting tamponades have been the most widely implemented technique but there is no standard treatment. We present the case of a 22-year-old male patient with bilateral Morning Glory syndrome, with a history of sudden loss of the inferior temporal visual field in the left eye with 7 days of evolution. Rhegmatogenous retinal detachment was diagnosed, with retinal tear at the optic disc level. Pars plana vitrectomy with hyaloidectomy, endophotocoagulation, perfluoropropane gas tamponade and positioning was performed. Recurrence was observed 21 days postoperatively, a second vitrectomy was performed completing the peripapillary photocoagulation, using 1000 cS silicone oil as a buffer. Neurosensory retinal application was achieved, with finger-counting vision.