A Case of Ophthalmofacial-Diencephalic Arteriovenous Malformation: A Variant of Bonnet-Dechaume-Blanc Syndrome

ABSTRACT

Authors experienced a rare case of ophthalmofacial-diencephalic arteriovenous malformation, named Bonnet-Dechaume-Blanc syndrome in 12 year-old girl. Clinically patient had an episode of frequent bleeding from the nose and gingival. Also she was left blindness of which the ophthalmologist gave the warning for cerebral hemorrhage because of the tangled, engorged vessels in left fundus. The multiple nidus on angiography revealed on left intraorbital, maxillopalatal, hypothalamic and basal ganglia area. Surgery underwent for huge intracerebral hematoma. The patient passed away due to rebleeding 15 months after initial hemorrhage.