先天性巨结肠Duhamel术后远期功能评价

Long-Term functional evaluation of duhamel operation in children with Hirschsprung's disease

目的评价先天性巨结肠(HD)Duhamel术后远期功能效果。方法对随访复查病例均行排便造影和肛管直肠测压检查,行腹部和肛门指诊检查,评价生活质量。结果42例按照临床评分标准,“优”6例,“良好”32例,“一般”4例,无“差”病例。手术后并发创口裂开、肠粘连、小肠结肠炎等并发症与排便评分无相关性;而术后盆腔感染、吻合口瘘、肛门再次手术与排便评分有相关性(rs=0.68,0.78,0.84,P<0.05)。结论先天性巨结肠Duhamel根治术后,多数患儿能够达到社会可接受的排便功能。HD术后直肠肛门抑制性反射并不恢复。HD术后需要长期随访,应及时处理并发症如闸门综合征、黏膜脱垂、肛门狭窄等。对术后存在不同原因的排便障碍,需要进行主观和客观的综合评价。

Objective To investigate the postoperative bowel function in patients with Hirschsprung's Disease(HD). Methods Between Sep. 1979 and Sep.1993, 66 children with HD were operated in the Department of Pediatric Surgery, Shandong Provincial Hospital. Of the 42 children followed-up, 30 were examined clinically and underwent defectography and manometric study. The clinical progress and quality of life were recorded in details. Abdominal and anal examination were routinely carried out. Results The 42 patients were classified into 3 groups, according to the standard quantitative clinical scoring systems (stooling score ranges from 0 to 14). Six children (~14.3% ) in the Excellent group (score 14) had normal bowel habit; 32 children (~76.2% ) in the Good group ( score 10-13) had minor continence related social problems; 4 children (~9.5% ) in the Fair group (score 5-9) had marked limitations in social life. There was no patient in the Poor group. Stool score did not correlate with postoperative wound dehiscence、adhesions obstruction which required re-operation and Hirschsprung-associated enterocolitis. On the other hand, postoperative pelvic infection、anal anastomotic dehiscence (leakage)、and re-operation of the anus correlated with poor stooling score (correlation coefficient ~0.86 , ~0.79 , ~0.84 respectively). Anorectal manometry study showed that the anal resting pressure and voluntary sphincter force (maximal squeeze pressure minus resting pressure ) in fair group were significantly lower than that of in control group (P<~0.05 ). The length of anus and the feeling threshold were also lower. Defectography showed that the anorectal angle and rectal angle in fair group was larger than that in normal control group (P<~0.05 ). Conclusions Most of the patients have achieved a socially acceptable function of defecation which improves with time after Duhamel approach. Rectal anal inhibitory reflex (RAIR) could not be established in patient with HD after the operation. Long-term follow-up is an important component of patient's care.