Peripapillary staphyloma

Definition

A rare congenital optic disc excavation characterized by deep fundus excavation of chorioretinal atrophy surrounding a relatively normal appearing optic disc. Retinal vasculature is normal, and retinochoroidal coloboma and glial anomalies are absent. Patients present with mostly unilateral markedly reduced visual acuity. Association with other congenital defects or systemic diseases is uncommon.

Disease data

Classification

Morphological anomaly

ORPHA code

519400

OMIM code

ICD10 code

Q14.2

ICD11 code

*Soruce