Repeated pull-through surgery for complicated Hirschsprung's disease--principles derived from clinical experience.

Background: In some patients, an initial pull-through procedure for Hirschsprung's disease fails, and obstructive symptoms persist or recur. Then a repeated pull-through operation may be necessary.

Methods: Seventeen patients with Hirschsprung's disease aged 2 to 9 years (median, 4.6 years) have undergone a repeated pull-through procedure because of unresponsive symptoms after an initial operation. The initial procedure was Soave in 3 patients, Rehbein in 13 patients, and Duhamel in 1 patient. Surgical revision was indicated by incomplete resection of the transition zone in 16 patients, anastomotic strictures in 9 patients, and fistulas in 2 patients. All 17 patients have undergone Redo Duhamel pull-through procedure. Median follow-up after Redo operation was 9 years (range, 1-23 years).

Results: In 15 patients, the stooling pattern normalized immediately after Redo procedure. Two, including 1 with Down's syndrome, are prone to constipation with occasional use of laxatives. Soiling is seen in the patient with Down's syndrome, but only with episodes of diarrhea. In spite of large formation of scars surrounding the neoanorectum in most patients, Duhamel pull-through reconstruction was possible in all children of this series.

Conclusions: The predominant cause for persistent or recurrent unresponsive obstructive symptoms after initial pull-through procedure is incomplete resection of the transition zone. Less frequently, anastomotic strictures, rigidity of the anorectal cuff, and fistulas cause obstruction. Preoperative workup must focus on these complications. The courses after initial pull-through procedure show that laxatives, Malone procedure, dilatations of the anorectum, myectomy, V-Y-plasty, and injections of botulinum toxin cannot eliminate the mechanical or functional obstruction. Although a large formation of scars in the pelvis resulting from the initial operation, myectomies, dilatations, and other surgical modalities render a Redo revision more difficult, Redo Duhamel pull-through procedure is able to provide the definitive solution to the problem.