[Sturge-Weber syndrome: medical management of choroidal hemangiomas].
OBJECTIVE
To investigate the outcome of irradiation of complicated choroidal hemangiomas in Sturge-Weber syndrome.


METHODS
The charts of 6 patients (7 eyes) with Sturge-Weber syndrome and choroidal hemangiomas were reviewed. An exudative retinal detachment was the indication for treatment in all cases. The mean age of the 6 patients was 13 years (range, 4 to 20 years). The minimum follow-up time was 1 year. Patients were checked for initial and final best-corrected visual acuity, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. The patients were treated with radiotherapy. A total dose of 20 Grays was applied to 7 eyes: 2 with a circumscribed choroidal hemangioma underwent proton therapy and 5 with diffuse hemangioma were treated by external beam irradiation.


RESULTS
Complete resolution of the subretinal fluid was achieved in all cases with the tumor height decreased. Visual acuity improved to 1 line or more in 5 eyes and remained stable in 2 eyes. Two cases that underwent proton therapy developed radiation retinopathy.


CONCLUSIONS
External beam radiation is an effective and safe option in the management of choroidal hemangiomas complicated by retinal detachment. Based on our experience, proton therapy should be reserved for sporadic circumscribed choroidal hemangioma.
Rumen
F
F
Service d'Ophtalmologie, CHU de Bicêtre, 78, avenue du Général Leclerc, 94270 Le Kremlin Bicêtre, France.
Labetoulle
M
M
Lautier-Frau
M
M
Kirsch
O
O
Patureau
R
R
Cantalloube
A
A
Habrand
J-L
JL
Offret
H
H
Frau
E
E
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002830:Choroid Neoplasms / Q000532:radiotherapy*; D005260:Female; D006391:Hemangioma / Q000532:radiotherapy*; D006801:Humans; D008297:Male; D011878:Radiotherapy / Q000009:adverse effects; D011879:Radiotherapy Dosage; D013341:Sturge-Weber Syndrome / Q000532:radiotherapy*; D016896:Treatment Outcome