先天性肛门闭锁术后巨结肠的诊断和治疗

Diagnosis and treatment of Hirschsprung's disease after anoplasty in congenital imperforate anus.

目的 探讨先天性肛门闭锁 (IA)合并先天性巨结肠 (HD)的诊治策略 ,提高肛门成形术后腹胀、便秘的诊治水平。方法 对 1980～ 1999年 162例IA术后腹胀、便秘的 4例患儿的临床资料进行分析。结果 4例患儿中 3例为低位IA ,一期经会阴肛门成形术后出现腹胀、便秘 ,2例存在肛门狭窄 ,另 1例为高位IA并多发畸形。术前诊断为IA合并HD行巨结肠根治术 3例 ,其中 1例肛门狭窄合并急性机械性肠梗阻于术中取材冰冻诊断为HD ,术后病理诊断为继发性巨结肠 ;保守治疗 1例为肛门狭窄并继发性巨结肠 ,经扩肛和灌肠后症状消失。最后诊断为IA合并HD 2例。 4例均获得满意的治疗效果。结论 肛门狭窄是肛门成形术后腹胀、便秘的常见原因 ,IA合并HD极少见 ,可通过肛门指检。

Objective To evaluate the management of Hirschsprung's Disease (HD) coexisted with imperforate anus (IA) for improving the outcome of abdominal distend and constipation after anoplasty. Methods Four of 162 cases of IA with abdominal distend and constipation after anoplasty were presented from 1980 to 1999.Their clinical data were analyzed.Results In the four cases,3 cases underwent one stage perineal anoplasty with low IA after birth and then presented abdominal distend and constipation,two with anus stenosis,another one with high IA and multiple malformations underwent pull-through procedure for anoplasty.Among them,radical cure operation for HD was performed in three cases according to the biopsy,one of the 3 cases with anus stenosis complicated with acute mechanical intestinal obstruction,according to the frozen pathologic examination,it was HD,it was finally diagnosed as secondary megacolon by postoperative pathology,and another one with anus stenosis complicated with secondary megacolon received conservative therapy.IA coexisted with HD was defined finally in two children (1 23%).All four patients got good outcome.Conclusion Anus stenosis is the common cause in the patients with abdominal distend and constipation after anoplasty.HD coexists with IA are very rare which can be diagnosed and distinguished through anorectal examination,barum enema,biopsy and histologic-pathology investigation.