Spectral-domain optical coherence tomography of peripapillary staphyloma

Abstract

Dear Editor,Peripapillary staphyloma is an extremely rare, nonhe-reditary condition in which the optic disc sits at thebase of surrounding staphylomaous walls, accompaniedby the adjacent atrophic choroid and retinal pigmentepithelium [2, 3]. Optical coherence tomography (OCT)findings of other cavitary optic disc malformations havebeen reported [1, 4, 5], however, there has been no casereport to show spectral-domain OCT findings under thename of peripapillary staphyloma.A 4-year-old boy presented with poor visual acuityand strabismus in the right eye. His birth, developmen-tal, and medical histories were unremarkable. Visualacuity was finger count in the right eye and 20/30 inthe left eye. The Krimsky test showed a right exotropiaof 20 prism diopters. A right relative afferent pupillarydefect was present. Cycloplegic refraction showed -6.50Dsph -3.00 Dcyl x180A in the right eye and -1.25 Dsph-2.00 Dcyl x 180A in the left eye. Fundus examinationdisclosed a staphylomatous defect around the opticpapilla and peripapillary atrophy (Fig. 1). Spectral-domain OCT (Spectralis OCT, Heidelberg Engineering,Inc., Heidelberg, Germany) showed a deeply seated rightoptic nerve with multiple intraretinal cystic cavities alongthe temporal tuft of staphyloma (Fig. 2). The OCT-measured depth of staphyloma was more than 1 mm.The left fundus was normal.This patient has an optic disc malformation mostcompatible with peripapillary staphyloma, which can beoften confused with other excavated lesions of the opticdisc. The morning glory disc is shallower, with a centralglial tuft, an anomalous enlarged optic disc, andincreased, straightened vessels [2, 5]. OCT of themorning glory disc showed increased optic nerve headdimensions, increased retinal nerve fiber layer thickness,and reduced macular thickness [5]. In optic disc colo-boma, the optic disc contains an excavation, oftenaccompanied by iris or retina coloboma, and systemicdisease is frequently present [2, 4]. OCT of optic disccoloboma revealed signs of a connection between theperineural space and the inner retinal layers on thetemporal optic disc border, as well as schisis-like changesextending from the disc to the macula, with cystoiddegeneration [4]. In this study, a third generationspectral-domain OCT provided a distinct visualization of