Encephalocraniocutaneous lipomatosis. Report of two cases and a review of the literature.

Neurofibromatosis Pub Date : 1989-01-01
J S Bamforth, V M Riccardi, P Thisen, D Chitayat, J M Friedman, J Caruthers, J G Hall
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Abstract

We describe 2 patients with unilateral facial and cerebral malformations consistent with encephalocraniocutaneous lipomatosis. The characteristic facial appearance seems to develop with age, possibly as a consequence of increased intracranial pressure. However, the clinical data suggest that this condition arises as a result of a disruption of cephalic neural crest and anterior neural tube.

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Encephalocraniocutaneous lipomatosis。报告两例病例并复习文献。
我们描述了2例伴有单侧面部和大脑畸形的患者,这些畸形与脑颅皮脂肪瘤病一致。面部特征似乎随着年龄的增长而发展,可能是颅内压升高的结果。然而,临床资料表明,这种情况的出现是由于头神经嵴和前神经管的破坏。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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