Bilateral circumscribed choroidal hemangioma in an otherwise healthy individual.

Objective: To describe an extremely rare association of bilateral circumscribed hemangioma (CCH) in the absence of any other evidence of systemic abnormalities.

Methods: A 43-year-old man was referred to our institution with a diagnosis of probable unilateral hemangioma of the right eye with decreased visual acuity.

Results: Funduscopic examination of both eyes revealed one CCH in each eye. Fluorescein angiography, indocyanine green angiography, and A- and B-scan ultrasonography confirmed the diagnosis. General examination, endocrinological testing, and imagery workup did not show any abnormality. The right eye was treated with low-dose external-beam irradiation with complete recovery of visual acuity.

Conclusions: Bilateral choroidal localization of tumoral lesions raises the question about their primary or metastatic onset. To our knowledge, bilateral CCH has been reported only in association with Sturge-Weber or Klippel-Trénaunay-Weber syndrome. The bilateral CCH localization in a healthy individual represents an extremely uncommon condition, which may represent a low penetrance phenotype of Sturge-Weber syndrome.