Background: Since Wiethe first described the clinical presentation of two optic disc depressions in a 62-year-old woman in 1882, there have been many studies addressing what later become known as the "optic disc pit." The main complication of this condition, termed optic disc pit maculopathy, is associated with visual deterioration. Treatment of optic disc pit maculopathy remains challenging.
Methods: Here we review the body of literature that documents the clinical findings, pathophysiology, histology, main complications, treatment options, special features and presentations, and differential diagnosis of optic disc pit.
Results: The source of the intraretinal fluid in optic disc pit maculopathy remains controversial. Four possible sources of this fluid have been proposed: fluid from the vitreous cavity; cerebrospinal fluid originating from the subarachnoid space; fluid from leaky blood vessels at the base of the pit; and fluid from the orbital space surrounding the dura.
Conclusions: Optic disc pits are a very rare clinical entity, affecting approximately one in 11,000 people. Patients with congenital optic disc pit sometimes remain asymptomatic, but 25% to 75% present with visual deterioration in their 30s or 40s after developing macular schisis and detachment. The most widely accepted treatment for such patients is a surgical approach involving pars plana vitrectomy with or without internal limiting membrane peeling, with or without endolaser photocoagulation and C3F8 endotamponade.