Bilateral choroidal folds and optic neuropathy: a variant of the crowded disk syndrome?

PURPOSE: To describe the clinical features of the syndrome that includes bilateral choroidal folds and optic neuropathy and to propose a novel etiology to explain this presentation.
DESIGN: Observational small case series. PARTICIPANTS: Three patients.
METHODS: Three patients received an extensive clinical work-up including complete ocular examination, A and B scan ultrasonography, Humphrey visual field analysis, and imaging of the brain and orbit. Neurological consultation was obtained in each patient two of whom underwent lumbar puncture. The findings of this detailed investigation are reported.
RESULTS: Each patient demonstrated bilateral choroidal folds with optic disk congestion and leakage in one eye and optic atrophy in the fellow eye. Extensive ocular and extraocular investigation failed to uncover a specific etiology. Pseudotumor cerebri, a well-documented cause of papilledema and choroidal folds, was ruled out in each case. Each patient did demonstrate variable degrees of hyperopia and shortened axial lengths.
CONCLUSIONS: The syndrome of bilateral choroidal folds and optic neuropathy may have various etiologies. Pseudotumor cerebri needs to be definitively ruled out. Normal neuro-ophthalmological investigation including lumbar puncture may indicate an alternative cause. Idiopathic acquired hyperopia in middle-aged patients who are hyperopic with shortened axial lengths may be associated with choroidal folds and a constricted scleral canal causing optic disk congestion and complicated by nonarteritic anterior ischemic optic neuropathy due to a crowded disk.