Scleral Cyst Associated with Anomalous Tilted Configuration of the Optic Nerve Head: A Case Report

There is limited published data currently available on scleral cysts in the posterior pole. Here, we detail the case of a patient who was suspected to have a peripapillary scleral cyst imprinting the optic nerve head (ONH) profile. The 52-year-old asymptomatic and otherwise healthy woman presented with unilateral ONH blurred margins of the left eye for 1 year. Her best-corrected visual acuity in the right and left eye was 20/20 and 20/25, respectively. Fundus observation of the right eye revealed no significant abnormalities; on the left eye a tilted disc with blurred margins in the superior quadrants and gliosis-associated changes in the lower quadrants were identified. Structural optical coherence tomography (OCT) showed a posterior hyporeflective cystic space at the level of the ONH, and OCT-angiography revealed flow void. The differential diagnosis of ONH edema was considered and the case discussed with the neuro-ophthalmology unit. Given the clinical history, the absence of symptoms and the multimodal imaging findings, a peripapillary scleral cyst was considered to be the most likely explanation for the edematous appearance and the anomalous tilted configuration of the ONH. This case suggests that although rare, even more so in the absence of an ONH coloboma, a postequatorial scleral cyst should be considered in the differential diagnosis of ONH lesions.