ABSTRACT
A case of Sturge-Weber Syndrome is presented in a male neonate with severe gangrenous ulcerative stomatitis involving the angiomatous skin lesion and leaving sequele of cleft lip and nasal disfiguration.
Subject(s)
Humans , Infant, Newborn , Male , Cleft Lip , Congenital Abnormalities , Gingivitis, Necrotizing Ulcerative , Skin , Sturge-Weber SyndromeABSTRACT
The so called MOYAMOYA disease is characterized by idiopathic progressive bilateral occlusions of internal carotid arteries near the supraclinoid portion, with a distinctive picture of abnormal net like vessels in the base of the brain. The term MOYAMOYA is a Japanese expression for "PUFF OF A SMOKE", suggested by Suzuki & Takaku in 1969. We have experienced typical 3 cases of so called MOYAMOYA disease during 1979, which were confirmed by bilateral carotid angiography. All 3 cases were involved bilateral sides of the brain and the exact causes were not detected in spite of various laboratory examinations. All of our cases were compatible with the criteria of Moyamoya disease, suggested by Takeuchi in 1978. In addition to the case report a review of history, etiology & pathogenesis of Moyamoya Disease were discussed.