Marfan Syndrome – Page 37 of 50

Peripapillary staphyloma or scleral ectasia is a rare congenital anomaly of the optic nerve. It occurs when the most posterior portion of the sclera fails to develop during embryogenesis.⁴ Patients with this condition have a normal or nearly normal optic nerve head that lies at the bottom of a deep excavation (Staphyloma) and appears to be receded behind the plane of the retina. This is best appreciated when viewed stereoscopically (see top left image). The excavation of peripapillary staphyloma can be seen ultrasonically (see top right image) as a concave depression at the position of the optic nerve head. This depression might be mistaken for optic nerve cupping. However, the standard 10 MHz transducer rarely has the capability to resolve optic nerve cupping.