Walsh & Hoyt: Peripapillary Staphyloma

Peripapillary staphyloma is an extremely rare, usually unilateral anomaly, in which a deep fundus excavation surrounds the optic disc. In this condition, the disc is seen at the bottom of the excavated defect and may appear normal or show temporal pallor. The walls and margin of the defect may show atrophic changes in the retinal pigment epithelium (RPE) and choroid. Unlike the morning glory disc anomaly, there is no central glial tuft overlying the disc, and the retinal vascular pattern remains normal, apart from reflecting the essential contour of the lesion. The staphylomatous excavation in peripapillary staphyloma is also notably deeper than that seen in the morning glory disc anomaly. Several cases of contractile peripapillary staphyloma have been documented. Seybold and Rosen described a patient who had transient visual obscurations in an eye with an atypical peripapillary staphyloma.