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神經皮膚黑病變合併先天性水腦症―一病例報告―

Neurocutaneous Melanosis with Congenital Hydrocephalus–A Case Report-

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摘要


神經皮膚黑病變(neurocutaneous melanosis)是合併皮膚及中樞神經系統的先天性異常中最罕見的疾病。本文報告的病例為28天大之男嬰,出生即有全身性大小不一之散播狀黑色母斑,且頭部較大。腦部超音波與電腦斷層攝影檢查發現有水腦與左後顱窩的巨大腫塊。開顱手術取出之黑色腫瘤及皮膚之母斑。經病理組織與電子顯微鏡檢查,診斷為此病。在本國尚為同樣之病例報告,故提出並加以討論。

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並列摘要


Neurocutaneous melanosis is a rare disease of the combined congenital abnormalities of the skin and central nervous system. We report a 28-day-old male newborn who had a large head and multiple pigmented skin nevi scattered, over the whole body at birth. He was admitted to the Department of Pediatrics of the Kaohsiung Medical College because of increasing head circumference. Transillumination of the scalp suggested the presence of hydrocephalus. Plain skull X-ray film showed a marked separation of the sutures. Eçhoencephalogram and CT scan of the brain identified a mass of high density in the left posterior fossa and marked dilatation of the ventricles. The mass was removed by left suboccipital craniectomy and was found to be melanosis. Both electron microscopic bud histological findings f the skin lesions and the mass indicated neurocutaneous melanosis.

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