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Zentralbl Chir 2009; 134(6): 507-513
DOI: 10.1055/s-0029-1224696
Übersicht

© Georg Thieme Verlag Stuttgart ˙ New York

Funktioneller Outcome von Kindern mit Morbus Hirschsprung und Analatresie

Functional Outcome in Children with Hirschsprung’s Disease or Imperforate AnusF. Obermayr1 , J. Fuchs1
  • 1Universitätsklinik für Kinder- und Jugendmedizin, Abteilung für Kinderchirurgie, Tübingen, Deutschland
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Publication History

Publication Date:
17 December 2009 (online)

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Zusammenfassung

Die funktionellen Ergebnisse nach operativer Therapie von Kindern mit Morbus Hirschsprung und anorektalen Malformationen werden in der Literatur sehr unterschiedlich bewertet. Zahlreiche Modifikationen der Operationstechniken zur Rekonstruktion des Anorektums wurden in der Vergangenheit beschrieben. Anorektale Malformationen werden seit den 1980er-Jahren vorwiegend über einen posterioren sagittalen Zugang korrigiert, was eine anatomiegerechte Rekonst­ruktion des Analkanals erlaubt. Der abdomino­perineale Durchzug oder der sakroabdominoperineale Durchzug, als klassische Operationsverfahren, sind in den Hintergrund getreten. Für die Therapie des Morbus Hirschsprung finden neben der von Swenson und Bill beschriebenen Rektosigmoidektomie mit koloanaler Anastomose der retrorektale Durchzug nach Duhamel und der endo­rektale Durchzug nach Soave Anwendung. Die Weiterentwicklung des endorektalen Durchzuges durch Georgeson und de la Torre erlaubt heute einen transanalen endorektalen Durchzug ohne die Notwendigkeit einer Laparotomie. Trotz der Fortschritte im pathophysiologischen Verständnis und der Therapie des Morbus Hirschsprung und der anorektalen Malformationen sind die funktionellen Ergebnisse nicht befriedigend. Die persistierende Obstipation, die Stuhl­inkontinenz und beim Morbus Hirschsprung die Enterokolitis stellen die häufigsten postopera­tiven Komplikationen nach anorektaler Rekonst­ruktion dar. Die funktionellen Probleme treten bereits im Kindesalter auf und senken die Lebensqualität der Patienten signifikant. Zwar kommt es im Laufe der Zeit zu einer Verbesserung der anorektalen Funktion und zu einer Normalisierung der Lebensqualität, jedoch scheint dies durch eine Anpassungsstrategie der Patienten bedingt zu sein. Ein standardisiertes und prospektives Studiendesign ist notwendig, um in Zukunft verschiedene Serien miteinander vergleichen und eine Basis für eine Weiterentwicklung der therapeutischen Ansätze schaffen zu können.

Abstract

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.

Schlüsselwörter

Morbus Hirschsprung - Analatresie - Outcome - Komplikationen

Key words

Hirschprung’s disease - imperforate anus - abdominal surgery - anorectal malformations - outcome - complications

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Dr. med. F. Obermayr

Universitätsklinik für Kinder- und Jugendmedizin

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72074 Tübingen

Deutschland

Phone: ++49 / (0)70 71 / 2 98 66 21

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Email: florian.obermayr@med.uni-tuebingen.de