Transanal one-stage Soave procedure for infants with Hirschsprung's disease

Purpose: Many centers perform a one-stage pull-through procedure for Hirsch sprung's disease (HD) diagnosed in infancy. The authors have developed a on e-stage pull-through procedure using a transanal approach that eliminates t he need for intraabdominal dissection. Methods: Nine children aged 3 weeks to 18 months with biopsy-proven HD unde rwent a transanal pull-through procedure over a 13-month period. A rectal m ucosectomy was performed starting 0.5 cm proximal to the dentate line, and extending proximally to the level of the intraperitoneal rectum. In the fir st eight children, intraperitoneal position was confirmed with a laparoscop e placed through a 3- to 5-mm port in the base of the umbilicus. The muscul ar sleeve was divided circumferentially to allow full-thickness mobilizatio n of the rectosigmoid junction. Manual transanal traction permitted direct visualization and division of mesenteric vessels with transanal mobilizatio n above the transition zone. Ganglion cells were confirmed by frozen sectio n, and the bowel was transected. The rectal muscular cuff was divided longi tudinally, and the anastomosis was completed. The laparoscope confirmed ori entation and adequate hemostasis. In a ninth patient, the identical procedu re was performed, but with the laparoscope used only for confirmation at th e end of the procedure. Results: Operative time, including frozen sections, averaged 194 minutes (r ange, 169 to 250 minutes), and the average length of bowel resected was 12 cm (range, 7.5 to 22 cm). Four of the nine patients were discharged on post operative day (POD) 1, four on POD 2, and one patient with Down's syndrome was discharged on POD 6. Median follow-up was 6 months (range, 3 to 14 mont hs). One death occurred 2.5 months postoperatively secondary to sudden infa nt death syndrome. Complications included postoperative apnea spells (n = 1 ), mild enterocolitis (n = 2), constipation (n = 1), anastomotic stricture (n = 1), and muscular cuff narrowing (n = 1); each responded to nonoperativ e management. Stool output has ranged from four to eight per day. Conclusion: A one-stage pull-through for HD can be performed successfully u sing a transanal approach without intraperitoneal dissection. This procedur e is associated with excellent clinical results and permits early postopera tive feeding, early hospital discharge, and no visible scars. J Pediatr Sur g 34:148-152. Copyright (C) 1999 by W.B. Saunders Company.