Marty T L, Seo T, Matlak M E, Sullivan J J, Black R E, Johnson D G
University of Utah, Primary Children's Medical Center, Salt Lake City 84113-1100, USA.
J Pediatr Surg. 1995 May;30(5):655-8. doi: 10.1016/0022-3468(95)90682-7.
This study is a retrospective review of all children treated for Hirschsprung's disease over the past 22 years at a single pediatric institution. During this time 177 patients had definitive surgical reconstruction. Five children died of causes unrelated to Hirschsprung's disease, and five children died from enterocolitis after an uneventful postoperative course. Clinical follow-up information was obtained from 135 (78%). Demographic data includes the following: sex ratio 74% male, 26% female; current mean age 9.9 years; mean length of follow-up 7.9 years (range, 3 months to 21.5 years). Mean age at surgical reconstruction was 1.6 years. Definitive surgical procedures included endorectal pull-through (Soave), 21%; modified Duhamel, 67%; extended side-to-side ileocolic anastomosis, 8%; rectal myomectomy, 4%. Transition zone was within rectum or rectosigmoid region in 86%. Overall, 32% (43/135) report difficulty with fecal soiling, and 12.6% (17/135) identify this as a severe problem. These numbers include patients with trisomy 21 and total colonic aganglionosis. Severe fecal soiling was reported in 7.1% (2/28) after an endorectal pull-through, and in 12.1% (11/91) after the modified Duhamel. The difference in incidence of soiling after these two procedures is not statistically significant. However, 40% (4/10) of the patients after the long side-to-side anastomosis for total colonic aganglionosis report severe problems with fecal soiling (P = .03). Surgical reconstruction for Hirschsprung's disease provides near-normal gastrointestinal function for the majority of children, but long-term follow-up shows significant residual problems with soiling in 12.6% of the patients. This is consistent with reported experience worldwide.
本研究是对过去22年在一家儿科机构接受先天性巨结肠病治疗的所有儿童进行的回顾性分析。在此期间，177例患者接受了确定性手术重建。5例儿童死于与先天性巨结肠病无关的原因，5例儿童在术后过程平稳后死于小肠结肠炎。从135例（78%）患者处获得了临床随访信息。人口统计学数据如下：性别比例为男性74%，女性26%；当前平均年龄9.9岁；平均随访时间7.9年（范围3个月至21.5年）。手术重建时的平均年龄为1.6岁。确定性手术方式包括经直肠拖出术（Soave术），占21%；改良Duhamel术，占67%；扩大的侧侧回结肠吻合术，占8%；直肠肌瘤切除术，占4%。86%的患者移行段位于直肠或直肠乙状结肠区域。总体而言，32%（43/135）的患者报告有大便失禁问题，12.6%（17/135）的患者认为这是一个严重问题。这些数字包括21三体综合征和全结肠无神经节症患者。经直肠拖出术后7.1%（2/28）的患者报告有严重大便失禁，改良Duhamel术后12.1%（11/91）的患者报告有严重大便失禁。这两种手术方式后大便失禁发生率的差异无统计学意义。然而，全结肠无神经节症行长侧侧吻合术后40%（4/10）的患者报告有严重大便失禁问题（P = 0.03）。先天性巨结肠病的手术重建为大多数儿童提供了接近正常的胃肠功能，但长期随访显示12.6%的患者存在明显的大便失禁残留问题。这与全球报道的经验一致。
