Nyarenchi Obed M, Scherer Andrea, Wilson Saul, Fulkerson Daniel H
Indiana University School of Medicine, Indianapolis, IN, USA.
Childs Nerv Syst. 2014 Feb;30(2):337-43. doi: 10.1007/s00381-013-2195-4. Epub 2013 Jun 13.
Cloacal exstrophy is a rare condition characterized by a defect in the abdominal wall with extrusion of malformed abdominal contents. The normal separation of the genitourinary and gastrointestinal systems does not occur. While patients with cloacal exstrophy have a high incidence of spinal defects, cranial defects are rare. Chiari malformation has been rarely reported in children with cloacal exstrophy, although the embryogenesis is unknown.
In this report, the authors describe a child with cloacal exstrophy and a large myelocystocele. The child also had an extensive Chiari II malformation.
We review the relevant embryology and suggest a possible mechanism for Chiari formation in this patient.
泄殖腔外翻是一种罕见的病症，其特征为腹壁存在缺损，伴有畸形腹腔内容物的外突。泌尿生殖系统和胃肠道系统未正常分离。虽然泄殖腔外翻患者脊柱缺陷的发生率较高，但颅骨缺陷却很罕见。尽管泄殖腔外翻患儿的胚胎发育过程尚不清楚，但关于Chiari畸形的报道却很少。
在本报告中，作者描述了一名患有泄殖腔外翻和巨大脊髓脊膜膨出的儿童。该患儿还患有广泛的Chiari II畸形。
我们回顾了相关胚胎学知识，并提出了该患者Chiari畸形形成的可能机制。
