Peripapillary Retinoschisis: The Expanded Spectrum and New Insights From Multimodal Imaging

To characterize and classify different forms of peripapillary retinoschisis (PPRS), and to clarify the nomenclature of this condition.

A retrospective, multicenter, multinational case series of PPRS was performed from August 2021 to September 2024. Cases were included if they demonstrated retinoschisis contiguous with and thought to be originating from the optic disc. Demographic and clinical data collected included age, gender, visual acuity, intraocular pressure, axial length, refraction and referring symptoms. Mandatory investigations included optical coherence tomography of the optic disc and macula with radial scans, color fundus photography, and fundus autofluorescence. Select cases underwent fundus fluorescein and/or indocyanine green angiography. Retinoschisis was characterized by the meridian in relation to the optic disc, layer(s) of the retina affected and associated conditions. A literature review was performed to identify all causes of PPRS.

A total of 47 eyes from 41 patients with PPRS were identified, comprising of 22 (54%) females and a mean age of 56 years (range 14-92 years). These were classified into 9 aetiologies: Congenital Disc Abnormalities (CDA, n = 16 eyes), peripapillary chorioretinal coloboma (n = 1), peripapillary atrophy (n = 3), glaucoma (n = 7), Peripapillary Pachychoroid Syndrome (PPS, n = 4), peripapillary choroidal neovascularization (PP-CNV, n = 3), high/ pathological myopia (n = 5), vitreopapillary traction (VPT, n = 4) and idiopathic (n = 4).

The 9 aetiologies of peripapillary retinoschisis can be classified into 5 groups: non-glaucomatous optic disc abnormalities (CDA, PP-coloboma, PP-atrophy), glaucomatous optic disc abnormalities, peripapillary choroidal diseases (PPS and PP-CNV), vitreous optic disc interface abnormalities and idiopathic. A new entity, "Focal Optic Disc Dome" (FODD) was identified. Understanding the full spectrum of PPRS can assist in correct diagnosis and management.