Combined therapy of a circumscribed choroidal hemangioma in contralateral Sturge-Weber syndrome with acetazolamide and lasercoagulation

Background In Sturge-Weber syndrome choroidal hemangiomas occur in up to 55%. With a few exceptions it is a diffuse hemangioma and it always affects the eye ipsilateral to the naevus flammeus. To the best of our knowledge, a contralateral circumscribed choroidal hemangioma has not yet been described. His:tory and clinical findings We present a 34-year-old male caucasian with a left-sided naevus flammeus and a right-sided circumscribed choroidal hemangioma. Ipsilateral to the naevus; the left eye showed typical vascular anomalies of the conjunctiva and episclera as well as in the anterior chamber angle with anterior synechia formation and circumscribed iris atrophy without secondary glaucoma. Retinal and choroidal vasculature was normal. In the contralateral eye the anterior segment was normal, but there was a juxtafoveolar circumscribed choroidal hemangioma of about 7.5 x 4.5 mm along the superior temporal vascular arcade. The tumor was detected when vision dropped to 0.4 due to an exsudative retinal detachment over the tumor involving the fovea. Therapy and clinical course Complete regression of the exsudative detachment occured under oral Acetazolamide (1000 mg/d), but after dose reduction to 500 mg/d the exsudation recurred. Thereafter, the surface of the tumor was treated with argongreen grid lasercoagulation in 3 sessions under Acetazolamide cover. Following scar formation the retina has stayed completely attached even after discontinuation of oral Acetazolamide therapy with a follow-up of now 7 months. Conclusions Visual prognosis after exsudative macula detachment is depended on rapid retinal reattachment. Therefore early and effective treatment is mandatory. Systemic application of Acetazolamid (1000 mg/d orally) led to a rapid reattachment of the retina with an excellent visual result. However, dose-reduction was followed by an immediate recurrency. We therefore suggest to perform an Argongreen grid lasercoagulation of the tumor surface while the patient is still under systemic Aceazolamid. This strategy has proven successful in this case.