These rare congenital defects can be found in all ages and both sexes. Their origins are unclear however they are most likely due to closure failure of the optic fissure.
They are usually unilateral ~90% of the time and solitary however multiple pits can be observed in the same nerve.
Patients may be asymptomatic or have significant central visual loss due to progression to Optic Disc Pit Maculopathy (ODPM), which usually occurs in adulthood.
They often appear as yellow/black or grey depressions in the optic disc, however can be very subtle and only picked up on OCT
When maculopathy occurs, juxtapapillary laser or surgery is indicated. Surgical techniques are conflicting indicating no universal solution but all include vitrectomy with PVD induction. Combined with vitrectomy, some surgeons opt to add either ILM peeling, inner retinal fenestrations or plugging of the pit with ILM, glue or donor sclera .
Generally success rates are equal with results taking months to show regression of the subretinal or intraretinal fluid.
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