Functional Outcome in Children with Hirschsprung's Disease or Imperforate Anus

Various outcomes following operative therapy for Hirschsprung's disease and anorectal malformations have been reported. Operative techniques for anorectal reconstruction have been modified several times in the past. Repair of anorectal malformations have been performed through a posterior sagittal approach since the 1980s. This allows an anatomically correct reconstruction of the anorectal canal. Abdominoperineal or sacroabdominoperineal pull-through procedures, as the classical operative techniques, have been abandoned by most surgeons. Rectosigmoidectomy with colo-anal anastomosis, as described by Swenson and Bill in 1948, as well as the retro-rectal pull-through (Duhamel) and the endorectal pull-through (Soave) are still frequently used in surgery for Hirschsprung's disease. The development of the transanal endorectal pull-through with (Georgeson) or without (de la Torre) laparoscopic assistance has eliminated the necessity of laparotomy in selected cases. Despite significant progress in the understanding of the pathophysiology of and therapy for Hirschsprung's disease and anorectal malformations, the functional results remain unsatisfactory. Functional problems occur already in early childhood and decrease the quality of life significantly. Although complications resolve with time and the quality of life normalises in adolescence and adulthood, this might be mainly due to an adaptation strategy by the patients. For the future, a standardised and prospective study design is necessary to compare different procedures and to provide a basis for the further development of therapeutic strategies.