Bilateral circumscribed choroidal hemangioma in an otherwise healthy individual

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

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.

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.

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.