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線状皮脂腺母斑症候群の2症例に眼科的検索を行い,広範な母斑を伴う1例の同側眼底に,乳頭上鼻側方向に脈絡膜の低色素を主病変とし,同部の脈絡膜,強膜の低形成が混在するという形成異常,ならびに乳頭の生理的陥凹の拡大を認め考察を加えた.
We observed 2 cases of linear nevus sebaceous syndrome with the triad of midfacial nevus sebaceus, seizures, and mental retardation.
The first case, a 11-year-old male, presented with nevus sebaceus of Jadassohn in the left facial hemi-sphere, focal EEG abnormality in the left occipital cortex and marked dilatation of the left temporal subarachnoideal space. The left fundus was affected with marked disc cupping, sectorial hypopigmen-tation of the choroid extending upper-nasal from the disc, and scattered patches of hypoplasia of the choroid and the sclera.
The second case was a 10-year-old male with the above triad associated with hemangioma in theforearm and normal ocular findings.
The observed wide spectrum of disorders involv-ing the brain, skin and the eye seemed to represent the multiple ectodermal and mesodermal malfor-mation in a rare congenital neurocutaneous syndrome.
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