PDF(Pubmed)
Abstract：
Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system (ENS), although data in humans are lacking. We here describe the first case of enteric degenerative neuropathy related to a congenital obstruction of the gut. A 3-year and 9-mo old girl began to complain of vomiting, abdominal distension, constipation with air-fluid levels at plane abdominal radiology. Her subsequent medical history was characterized by 3 operations: the first showed dilated duodeno-jejunal loops in the absence of occlusive lesions; the second (2 years later) was performed to obtain full-thickness biopsies of the dilated intestinal loops and revealed hyperganglionosis at histopathology; the third (9 years after the hyperganglionosis was identified) disclosed a Ladd\'s band which was removed and the associated gut malrotation was corrected. Repeated intraoperative full-thickness biopsies showed enteric degenerative neuropathy along with reduced interstitial cells of Cajal network in dilated loops above the obstruction and a normal neuromuscular layer below the Ladd\'s band. One year after the latest surgery the patient tolerated oral feeding and did well, suggesting that congenital (partial) mechanical obstruction of the small bowel in humans can evoke progressive adaptive changes of the ENS which are similar to those found in animal models of intestinal mechanical occlusion. Such ENS changes mimic neuronal abnormalities observed in intestinal pseudo-obstruction.
摘要：
实验证据表明，肠道的慢性机械性亚闭塞可能会损害肠神经系统（ENS），尽管人类缺乏数据。我们在这里描述了第一例与先天性肠梗阻有关的肠退行性神经病。一名3岁和9个月大的女孩开始抱怨呕吐，腹胀,在平面腹部放射学上有空气-液体水平的便秘。她随后的病史以3次手术为特征：第一次显示十二指肠空肠扩张环，没有闭塞性病变；第二次（2年后）进行了扩张的肠loop的全层活检，并在组织病理学上发现了节骨病；第三次（发现节骨病后9年）发现了Ladd\'s带，该带被去除，并纠正了相关的肠旋转不良。重复的术中全层活检显示肠退行性神经病变，梗阻上方扩张的环和Ladd带下方正常的神经肌肉层的Cajal网络间质细胞减少。最近一次手术一年后，患者耐受口服喂养，表现良好，提示人类小肠的先天性（部分）机械性阻塞可以引起ENS的进行性适应性变化，这与在肠机械性阻塞动物模型中发现的相似。这样的ENS变化模拟在肠假性梗阻中观察到的神经元异常。
