Peripapillary staphyloma: clinical features and visual outcome in 19 cases

To investigate the clinical features and visual outcome of patients with peripapillary staphyloma.

The medical records of patients diagnosed as having peripapillary staphyloma at Seoul National University Children's Hospital, Seoul, Korea, between January 1, 1991, and December 31, 2003, were reviewed. Visual acuity and associated ocular and systemic abnormalities were recorded.

Nineteen patients (21 eyes) with peripapillary staphyloma were included in the study. The mean age at initial examination was 21 months, and 42% (8/19) of the patients were girls. Two patients (11%) had bilateral peripapillary staphylomas. Only 2 eyes had visual acuity better than 20/200 at the last examination, and 7 eyes had severe myopia of more than -6 diopters. After occlusion therapy, 1 patient achieved a visual improvement from light perception to 20/30. Congenital cataract and persistent pupillary membrane were present in 2 eyes each at initial examination. Complications observed during the follow-up period included acquired total cataract in 2 eyes and retinal detachment in 3 eyes. Scleral encircling buckling was performed in 1 eye with retinal detachment, and subsequently the retina was reattached. Only 2 patients had accompanying systemic abnormalities.

Rarely, patients with peripapillary staphyloma can achieve significant visual improvement by occlusion therapy, although visual outcome is generally poor. Because associated ocular disease and refractive errors are not infrequent, complete ophthalmic examinations and regular follow-up are necessary.