Optic Disc Pit-Associated Maculopathy

Optic disc pit is a rare congenital defect of the optic nerve head, often leading to maculopathy characterized by intraretinal and subretinal fluid accumulation. This chapter presents an overview of optic disc pit-associated maculopathy, addressing its clinical features, pathogenesis, disease progression, differential diagnosis, treatment strategies, and surgical outcomes. Pathogenesis theories implicate both cerebrospinal fluid and vitreous fluid as potential sources. Disease progression involves fluid accumulation leading to schisis-like separation, outer lamellar hole formation, and subretinal fluid accumulation. Surgical intervention with pars plana vitrectomy and gas tamponade is recommended for maculopathy. Various additional techniques have been proposed, including laser to the optic nerve edge and stuffing of the nerve pit with inverted internal limiting membrane tissue. Surgical outcomes show slow visual improvement in most cases, though photoreceptor defects can influence recovery potential.