Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, WA, United States.
Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, OH, United States; Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, OH, United States.
Semin Pediatr Surg. 2022 Apr;31(2):151164. doi: 10.1016/j.sempedsurg.2022.151164. Epub 2022 Apr 18.
After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.
先天性巨结肠患儿接受手术后，应当生长良好，能够控制排便，避免反复出现腹胀和结肠炎。但实际上，仍有相当一部分患儿术后会出现上述情况。许多临床医生对这些结果感到困惑，因为他们认为这些患儿接受的手术在技术上是令人满意的。本文提出了一种针对先天性巨结肠经肛门根治术后效果不佳患儿的评估和治疗的方法。术后存在问题的先天性巨结肠患儿可能存在以下三种情况：（1）大便失禁，（2）梗阻症状，（3）反复发作的结肠炎（梗阻症状的一种更为严重的类型）。在采用系统的诊断方法后，几乎可以对所有患儿实施有效的治疗。患儿可能需要接受药物治疗（行为干预、饮食改变、泻药或机械性结肠排空），在明确特定的解剖或病理原因后需要进行再次手术，或者在非弛缓性括约肌导致梗阻症状或反复发作结肠炎时，需要注射肉毒毒素。
