Retinal findings in pediatric idiopathic intracranial hypertension

Abstract

Background

Pediatric and adult idiopathic intracranial hypertension (IIH) have distinct clinical features. Retinal manifestations associated with adult IIH are well known and can limit visual function; however, data detailing these manifestations in pediatric IIH are sparse. The purpose of this study was to identify the range and effects of retinal manifestations in pediatric IIH focusing on associated visual and structural outcomes.

Methods

The medical records of IIH patients from an academic children’s hospital between 1998 and 2022 were reviewed retrospectively. Patients meeting diagnostic criteria for IIH without other concomitant retinal disease of alternate etiology were included. We evaluated demographics, visual function, and structural correlates longitudinally, and discernible retinal pathology based on fundus photography and optical coherence tomography was recorded.

Results

Of the 253 patients with IIH, 24 (9.5%) had retinal manifestations, including 20 (7.9%) with retinal folds, 15 (5.9%) with macular exudates, 11 (4.3%) with subretinal fluid and 1 (0.4%) with a choroidal neovascular membrane. Baseline (P < 0.001) and final (P = 0.013) visual acuities were significantly lower in patients with retinal findings than those without. Eight patients (33.3%) had persistent retinal damage after the resolution of optic nerve edema. The presence of optic nerve atrophy after treatment was significantly higher in patients with retinal findings than those without (P < 0.001).

Conclusions

In our study cohort, retinal pathology was seen in a minority of pediatric IIH patients and persisted in many cases following resolution of papilledema. Retinal pathology in pediatric IIH was associated with worse visual function and outcomes and likely reflects worse disease severity. The extent to which retinal disease in IIH might contribute to vision loss remains unclear.