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伴有皮肤表现的罕见骶尾部胚胎发育畸形。

Unusual sacrococcygeal embryologic malformations with cutaneous manifestations.

作者信息

Harrist T J, Gang D L, Kleinman G M, Mihm M C, Hendren W H

出版信息

Arch Dermatol. 1982 Sep;118(9):643-8.

PMID:7114865
Abstract

Two unusual sacrococcygeal neuroepithelial heterotopias manifested as masses associated with cutaneous signs. In a 13-month-old infant, a cystic coccygeal medullary vestige was associated with a midline epidermal nevus. In another patient, a lipomeningocele with neuroepithelial heterotopia manifested as a skin tag and mass in the right buttock. In both cases, the malformations probably resulted from abnormal canalization and retrograde differentiation of the distal neural tube. Cystic coccygeal medullary vestige results from dilation of a persistent ependymal cyst present commonly in neonates at the distal part of the coccyx. The lipomeningocele appears to have arisen from an aberrantly formed ependymal canal. The embryologic events that gave rise to the lesions, the differential diagnosis of postrectal masses, and the common association of midline lesions of skin and soft tissue with neural defects are stressed.

摘要

两例罕见的骶尾部神经上皮异位表现为与皮肤体征相关的肿块。在一名13个月大的婴儿中,一个囊性尾髓遗迹与中线表皮痣相关。在另一名患者中,伴有神经上皮异位的脂肪瘤样脊膜膨出表现为右臀部的皮赘和肿块。在这两个病例中,畸形可能是由于远端神经管的异常管道形成和逆行分化所致。囊性尾髓遗迹是由新生儿常见的尾骨远端持续存在的室管膜囊肿扩张引起的。脂肪瘤样脊膜膨出似乎起源于异常形成的室管膜管。文中强调了导致这些病变的胚胎学事件、直肠后肿块的鉴别诊断以及皮肤和软组织中线病变与神经缺陷的常见关联。

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Unusual sacrococcygeal embryologic malformations with cutaneous manifestations.伴有皮肤表现的罕见骶尾部胚胎发育畸形。
Arch Dermatol. 1982 Sep;118(9):643-8.
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Teratology. 1982 Apr;25(2):201-13. doi: 10.1002/tera.1420250209.
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