先天性巨结肠同源病：手术方法的选择

The choice of operative procedures for Hirschsprungs disease allied diseases in children

目的对先天性巨结肠同源病的手术处理方式做探讨。方法2008年1月至2010年12月45例巨结肠同源病行根治术，平均年龄（35．5±5）个月，其中节细胞减少症（HG）33例（有既往手术史4例，12．1％），肠神经元发育不良症（IND）12例（有既往手术史4例，33．33％）。所有患儿均在术后1、3、6及12个月进行随访，记录患儿术后排便功能并与术前结果相比较。结果HG组33例中17例（52％）经开腹手术，16例（48％）腹腔镜辅助或经肛门拖出术。结肠切除范围：左半切除28例（84．8％），次全切除5例（15．2％）；12例IND组全部经开腹手术，结肠左半切除4例（33．3％），次全切除8例（66．7％）。术后随访：所有患儿便秘症状消失，无手术死亡及严重并发症发生。不同术式组中均有少数患儿持续存在污粪现象，1年期随访经肛门手术显著高于开腹手术（P〈0．05）。出现术后污粪患儿施行肛管直肠测压术前肛管静息压（66．5±11．67）mmHg，术后3个月为（52．17±0．31）mmHg较术前明显下降，但至术后6个月～1年后测压为（58±5．7）mmHg，与术前相比均无显著差异。结论同源病的手术应根据不同病理类型和临床情况选择手术方式：原发性HG病变可采用直接经肛门拖出或腹腔镜辅助游离术。既往曾经肛门直肠手术或年长、晚期患儿应采取保留肛管直肠括约肌形态和功能完整性的术式。IND患儿均需行根治性次全切除术，不主张采用简单的经肛门拖出术式而应采用盆腔内的低位吻合术式。经肛门或腹腔镜辅助经肛门拖出术式组1年期随访时污粪率要高于非拖出术组，左半切或次全切方式对术后是否污粪无影响。

Objective To determine operative procedures for Hirschsprung＇s disease allied diseases （HAD） in children. Methods From Jun. 2008 to Dec. 2010, 45 consecutive patients with an average ages of 35.5 ± 5 months underwent radical surgery for HAD in one surgical group, including 33 hypoganglionosis （HG） and 12 intestinal neuronal dysplasia （IND）. All patients have been followed up at 1, 3, 6 and 12 months after surgery. Defecation function were studied and compared with that before surgery. Results Among the 33 HG patients, 17 patients （52%） underwent open surgery, the other 16 （48%） underwent transanal or laparoscopic-assisted pull-through surgery including left colectomy on 28 （84. 8%） and subtotal colectomy on 5 （15.2%）. All IND patients underwent open surgery, and left colectomy were performed on 4（33. 3%） and subtotal colectomy on 8 （66. 7%）. Constipation disappeared in all patients during follow-up after surgery. No death and severe complication was noted. The incidence of soiling was significantly higher in patients with transanal pull-through procedure than that patients underwent open surgery 1 year after surgery （P〈0. 05）. Anorectal manometry for the patients with soiling showed the parameter of manometry was decreased markedly at the 3rd month after surgery is compared with preoperative manometry（52. 17 ± 0. 31） mmHg vs. （66. 5 ± 11.67）mmHg. However, manometry at 6th or 12th postoperative month is（58 ± 5. 7）mmHg was not different with preoperative manometry. Conclusions The choice of operative procedures for HAD should be based on different pathological types and clinical conditions. Primary HG lesions can be removed directly by a simple transanal pull-through or laparoscopic-assisted surgery. For HG patients with previous anorectal surgery or in older children, the surgical procedures of saving the anorectal sphincter morphology and function should be chosen. For IND children, radical subtotal colectomy and low level anastomosis within pelvic should be performed, It was not advocated using a simple transanal pull-through procedure for IND children. The rate of soiling in transanal pull-through group was higher than that in open surgery group 1 year after surgery. Neither left nor subtotal colectomy was associated with the incidence of postoperative soiling.