Sturge-Weber disease: value of the topographic analysis of the facial angioma for the diagnosis of associated pial angioma
Dulac, O.; Larregue, M.; Roger, J.; Arthuis, M.
Archives Francaises de Pediatrie 39(3): 155-158
1982
ISSN/ISBN: 0003-9764 PMID: 7103671 Document Number: 185725
A retrospective study was undertaken in 47 children with congenital capillary naevus of the face in order to appreciate the predictive value of the topography of the facial angioma for the diagnosis of associated pial angioma. In patients with Sturge-Weber disease, facial angioma concerned the whole upper eyelid, extending to the forehead and/or cheek. Less often, it was localized to the root of the nose; in such cases it was a port-wine, homogeneous angioma, with clearcut outlines, quite different from the angiomatous dots frequently seen in this area in neonates. Except for these rare cases, angiomas concerned only half or less of the upper eyelid and no pial angioma was associated. Finally, congenital capillary naevi with this topography typical of Sturge-Weber disease were frequently associated with pial angioma.