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Abstract #21445 Published in IGR 10-3
Bilateral Sturge-Weber-Krabbe syndrome. A case report
Ouaggag B; Baha Ali T; Gaboune L; Benfdil N; Moutaouakil ABulletin de la Société Belge d'Ophtalmologie 2008; 19-23
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. LA: French
Dr. B. Ouaggag, Service d'Ophtalmologie, CHU Mohammed VI-Marrakech, Maroc. ouaggagb@yahoo.fr
Classification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
9.1.2 Juvenile glaucoma (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
