摘要
背景:虽然大多数先天性巨结肠(HD)患儿在拖出术后预后良好,但也有一些患儿仍存在持续性梗阻症状,这导致发病率的显著上升。目的:研究一种系统方法以检查并最终根除这些症状。方法:采用一个基于5个潜在病因的计算方法对到笔者处就医的HD拖出术后有严重梗阻症状的所有患儿进行评价并制定了适当的治疗干预措施。结果:在7年内对49例患儿进行了评价。7例采用直肠检查和钡灌肠发现有机械性肠梗阻,
Background Although most children with Hirschsprung's disease (HD) do well after pull-through surgery, some continue to have persistent obstructive symptoms that may lead to significant morbidity. The author's goal was to develop an organized algorithm for the workup and ultimate management of these symptoms. Methods All children referred to the author with severe obstructive symptoms after a pull-through for HD were evaluated using an algorithm-based on 5 potential etiologies, and appropriate therapeutic interventions were instituted. Results A total of 49 chil-drenwere evaluated over 7 years. Mechanical obstruction-was found using rectal examination and barium enema in 7, of which, 2 had resection of a Duhamel spur, and 5 had a repeat pull-through (after failed dilatation). Rectal biopsy results showed aganglionosis in 10, of whom, 8 underwent repeat pull-through, and 2 refused further surgery. Abnormal intestinal motility or intestinal neuronal dysplasia was found proximal to the aganglionic segment in 10 children using colonic and small bowel manometry or laparo-scopic biopsies; 4 of these had additional colon resected and repeat pull-through, and 6 were treated with a bowel management routine, cecostomy, or stoma formation. Internal sphincter achalasia was felt to be the primary cause of symptoms in 14, and all were treated with intrasphinc-teric botulinum toxin. Eight were found not to fall into any of the above groups and were treated medically for presumed functional megacolon; 2 of these ultimately had a colostomy. Conclusions An organized approach to the diagnosis and management of obstructive symptoms in a child after a pull-through for HD permits accurate diagnosis and effective treatment in the majority of cases.
