Langer J C, Rollins M D, Levitt M, Gosain A, Torre L de la, Kapur R P, Cowles R A, Horton J, Rothstein D H, Goldstein A M
Division of General and Thoracic Surgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada.
Division of Pediatric General and Thoracic Surgery, Hospital for Sick Children, Rm 1524, 555 University Ave, Toronto, ON, M5G 1X8, Canada.
Pediatr Surg Int. 2017 May;33(5):523-526. doi: 10.1007/s00383-017-4066-7. Epub 2017 Feb 8.
Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.
尽管大多数患有先天性巨结肠症的儿童最终预后良好，但许多患儿在拖出式手术后仍会出现各种持续存在的问题。最常见的问题包括梗阻症状、大便失禁、小肠结肠炎和发育不良。本指南的目的是针对先天性巨结肠症患儿术后梗阻症状的管理提出一种合理的方法。美国小儿外科协会理事会成立了一个先天性巨结肠症兴趣小组。然后通过小组讨论、文献综述和专家共识，总结了关于先天性巨结肠症拖出式手术后梗阻症状患儿的病因、诊断方法和治疗方法的当前知识状况。拖出式手术后梗阻症状的病因包括机械性梗阻；持续性或获得性无神经节细胞症、神经节细胞减少症或过渡区拖出；内括约肌失弛缓症；含有神经节细胞的近端肠道动力紊乱；或由憋便行为引起的功能性巨结肠。本文提出了一种先天性巨结肠症拖出式手术后梗阻症状的诊断和管理算法。对先天性巨结肠症拖出式手术后出现梗阻症状的患者进行诊断和管理的逐步、逻辑方法有助于治疗。证据级别为V级。
