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Published on: September 16, 2020
Retinal findings in pediatric idiopathic intracranial hypertension
Michael T Curley1, Gena Heidary2, Eric D Gaier3
1Department of Ophthalmology, Boston Medical Center, Boston, Massachusetts.
Insights
Retinal issues in pediatric idiopathic intracranial hypertension (IIH) affect vision and structure, persisting even after papilledema resolves. These findings suggest more severe IIH disease in affected children.
Area of Science:
- Ophthalmology
- Pediatric Neurology
- Neuro-ophthalmology
Background:
- Idiopathic intracranial hypertension (IIH) presents differently in children and adults.
- Adult IIH retinal manifestations are known, but pediatric data is limited.
- This study investigates retinal changes in pediatric IIH.
Purpose of the Study:
- To identify the spectrum of retinal manifestations in pediatric idiopathic intracranial hypertension (IIH).
- To assess the impact of these retinal findings on visual function.
- To evaluate structural outcomes associated with pediatric IIH and its retinal complications.
Main Methods:
- Retrospective review of medical records from 1998-2022 at a children's hospital.
- Inclusion criteria: pediatric patients diagnosed with IIH without other retinal diseases.
- Evaluation included demographics, visual function, structural correlates, fundus photography, and optical coherence tomography.
Main Results:
- 24 out of 253 (9.5%) pediatric IIH patients had retinal manifestations.
- Common findings included retinal folds (7.9%), macular exudates (5.9%), and subretinal fluid (4.3%).
- Patients with retinal findings had significantly worse visual acuity and higher rates of optic nerve atrophy, with persistent damage in some cases.
Conclusions:
- Retinal pathology occurs in a subset of pediatric IIH patients and can persist post-treatment.
- Pediatric IIH with retinal involvement is associated with poorer visual outcomes and may indicate greater disease severity.
- The precise contribution of retinal disease to vision loss in pediatric IIH requires further investigation.
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.
