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Enteric neuropathology of congenital intestinal obstruction: A case report.

机译:先天性肠梗阻的肠神经病理学:一例报告。

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摘要

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.
机译:实验证据表明,尽管缺乏人体数据,但肠的慢性机械性咬合可能会损害肠神经系统(ENS)。我们在这里描述与先天性肠梗阻有关的肠道退行性神经病的第一例。一个3岁9个月大的女孩开始抱怨呕吐,腹胀,在腹部平面放射学检查时出现便秘并伴有气液。她的后续病史以3例手术为特征:第一例显示在没有闭塞性病变的情况下十二指肠-空肠扩张。进行第二次(2年后),以获取扩张后的肠loop的全层活检,并在组织病理学上发现高神经节病。第三例(确定为神经节过度增生后的9年)披露了一条Ladd's乐队,该乐队被移除并纠正了相关的肠道畸形。重复的术中全厚度活检显示肠退行性神经病变,并在梗阻上方的扩张环和Ladd谱带下方的正常神经肌肉层中减少了Cajal网络的间质细胞。最近一次手术后一年,患者忍受了口服喂养,并且表现良好,这表明人的小肠先天性(部分性)机械阻塞可引起ENS的进行性适应性改变,类似于肠道机械性闭塞动物模型中发现的这种改变。 。这样的ENS改变了在肠假性梗阻中观察到的模拟神经元异常。

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