Peripapillary Pachychoroid Syndrome

Abstract

Purpose : We performed a multi-centered retrospective study to determine the clinical features of a novel variant of central serous chorioretinopathy (CSC) in the pachychoroid spectrum of disorders. The term peripapillary pachychoroid syndrome (PPS) was proposed for this unique entity.

Methods : A total of 31 eyes with PPS from 16 patients demonstrating intraretinal cysts and thick choroid in the nasal macular region (Group 1) were recruited. Detailed medical history was reviewed and findings of ocular examination and multimodal imaging were reviewed. Macular choroidal thickness was measured on enhanced depth imaging optical coherence tomography (EDI-OCT) at several pre-defined locations. For analysis of the choroidal thickness, 17 eyes with typical CSC or pachychoroid neovasculopathy from 11 patients aged > 50 years old (Group 2) and 19 age-matched normal eyes from 12 subjects (Group 3) were included for comparisons.

Results : PPS patients were aged 71±7 years (range 58-86 years) and 13/16 (81%) were male. PPS was characterized by markedly thickened nasal macular choroid significantly greater than temporal macular choroid (P<0.0001), and significantly different from typical CSC. Associated intraretinal cysts and/or subretinal fluid was noted in the nasal macula extending from the optic disc margin in all eyes. Intraretinal cysts were often present nasal to the disc as well. Fundus autofluorescence and fluorescein angiography (FA) showed peripapillary mottling of the retinal pigment epithelium with late staining and no frank leakage in all 29 eyes with FA available. Indocyanine green angiography showed peripapillary dilated large choroidal vessels with hyperpermeability when performed. The optic nerve head was usually crowded or with small cupping or even edematous and illustrated mild late leakage in FA in half of the cases. PPS was significantly associated with old age, choroidal folds, short axial length and hyperopia.

Conclusions : PPS is a distinct variant of CSC and falls within the pachychoroid spectrum of disorders. Peripapillary choroidal congestion exhibiting a compartment-like effect was proposed as an etiologic mechanism. It is important to recognize this clinical entity, which can be confused with posterior uveitis and neuro-ophthalmologic disorders to avoid unnecessary interventions.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.