[Bilateral Sturge-Weber-Krabbe syndrome. A case report].
Sturge-Weber-Krabbe syndrome is a rare congenital neuro-oculo-cutaneous disease, characterized by an unilateral facial vascular nevus which affects at least the first branch of the trigeminal nerve, associated with an ipsilateral leptomeningeal angioma and ipsilateral vascular lesions of the choroid sometimes leading to glaucoma. Variants of this classical presentation have been described in the literature, some of which have prognosis significance. We report an unusual case of an 11-year-old male with a serious bilateral glaucoma associated with a bilateral facial nevus flammeus and cerebral calcifications without neurological symptoms. Epidemiological, clinical, prognosis peculiarities and management of this syndrome are discussed.
Service d'Ophtalmologie, CHU Mohammed VI-Marrakech, Maroc. ouaggagb@yahoo.fr
B
B
Ouaggag
T
T
Baha Ali
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L
Gaboune
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N
Benfdil
A
A
Moutaouakil
D001927:Brain Diseases / Q000175:diagnosis; D002114:Calcinosis / Q000175:diagnosis; D002648:Child; D003937:Diagnosis, Differential; D005901:Glaucoma / Q000175:diagnosis; D006801:Humans; D008297:Male; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*