대한대장항문학회

Purpose : In small portion of patients with Hirschsprung's disease, the aganglionic stagment extends only up to the mid-rectum. This report describes an innovative and effective posterior sagittal anorectal myectomy for curative repair of ultrashort<br>segment Hirschsprung's disease in neonates.<br>Methods : The procedure was performed on ten patients with ultrashort segment Hirschsprung's disease between 1995 to 1998. The procedure was performed by making a sagittal incision in midline posterior perineum to expose the posterior rectum.4<br>longitudinal strip of muscular layer is removed from the aganglionic portion of the anorectum from the upper rectum to the internal sphincter. The patients were followed postoperatively to determine the effectiveness of the procedure and to observe the<br>presence of any complications.<br>Results : Seven patients (70.0%) were under three months old. Operative biopsy showed that four patients had aganglionosis in the upper rectum, three patients in the middle rectum and three patients in the lower rectum. The posterior sagittal anorectal<br>myectomy was successful in the treatment of ultrashort segment Hirschsprung's disease- symptom recurred for one patient due to total aganglionosis and for another patients due to rectosigmoid aganglionosis. These two patients received the<br>staplingprocedure after colostomy. Some transient complications included mucosal perforation during surgery (30.0%) and incisional wound infection (20.0%).<br>Conclusions : For those neonates with ultrashort segment Hirschsprung's disease, the posterior sagittal autorectal myectomy should be considered a safe and effective method for treatment and confirmatory diagnosis. In addition, preliminary colostomy is<br>not required prior to this procedure.<br>