This paper describes the relatively benign condition of congenital peripapillary staphyloma. We discuss its management and differential diagnosis from other congenital or acquired anomalies that affect the optic nerve and may have a similar appearance. Acquired staphylomas may be associated with glaucoma or inflammation and these should be ruled out to confirm the congenital condition. On the other hand, a congenital condition can be associated with a higher occurrence of retinal detachments and the reduced vision of these eyes makes early detection of detachment more difficult by self examination, hence yearly fundus examination is warranted. We feel that differentiating the congenital peripapillary staphyloma from an acquired myopic staphyloma is an uncommon but difficult dilemma facing clinicians. Ultrasonographic measurement of axial length allows monitoring of the depth of a staphyloma and any increase in depth provides a differential diagnosis as the congenital condition is stable over time. Flicker perimetry appears to be a useful adjunct in assessing visual loss that may otherwise be overlooked using static methods.
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