Werner Christoph R, Stoltenburg-Didinger Gisela, Weidemann Henning, Benckert Christoph, Schmidtmann Marco, van der Voort Ivo R, Andresen Viola, Klapp Burghard F, Neuhaus Peter, Wiedenmann Bertram, Mönnikes Hubert
Department of Medicine, Division of Hepatology and Gastroenterology, Charité, Campus Virchow-Klinikum, Universitätsmedizin Berlin, Augustenburger Platz 1, Germany.
World J Gastroenterol. 2005 Sep 28;11(36):5742-5. doi: 10.3748/wjg.v11.i36.5742.
Hirschsprung's disease (HD) is a disorder associated with congenital malformation of the enteric nervous system with segmental aganglionosis. Prevailing therapy includes a resection of the affected part of the bowel. However, patients often do not obtain complete functional improvement after surgical treatment. We present the case of a 25-year-old woman who had surgical treatment of HD in early childhood. After that procedure she had clinical features of constipation for years in the end, passing of stool once a week, requiring laxatives and enemas. We diagnosed an incomplete resection of the aganglionic bowel via rectal biopsy and resected the remaining aganglionic segment. Two months after surgery the patient's bowel function improved to a frequency of 1-4 stools per day. We conclude that regular follow-up is required to identify HD patients with persistent alterations of bowel function after surgery. In patients presenting with constipation, recognition of a remaining aganglionic segment or other alterations of the enteric nervous system should be aimed at in an early stage.
先天性巨结肠（HD）是一种与肠神经系统先天性畸形及节段性无神经节细胞症相关的疾病。目前的治疗方法包括切除肠道的病变部分。然而，患者在手术治疗后往往无法获得完全的功能改善。我们报告一例25岁女性病例，她在幼儿期接受了HD手术治疗。术后多年，她一直有便秘的临床症状，每周排便一次，需要使用泻药和灌肠剂。我们通过直肠活检诊断出无神经节细胞肠段切除不完全，并切除了剩余的无神经节细胞段。术后两个月，患者的肠道功能改善至每天排便1 - 4次。我们得出结论，需要定期随访以识别术后肠道功能持续改变的HD患者。对于出现便秘的患者，应尽早识别出剩余的无神经节细胞段或肠神经系统的其他改变。
