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Kinder- und Jugendmedizin 2018; 18(01): 11-18
DOI: 10.1055/s-0038-1636715
Gastroenterologie/Kinderchirurgie
Schattauer GmbH

Morbus Hirschsprung

Was sollte der Pädiater wissen?Hirschsprung diseasewhat should the pediatrician know?
M. Lacher
1   Klinik und Poliklinik für Kinderchirurgie, Universitätsklinikum Leipzig
› Author Affiliations
Further Information

Publication History

Eingereicht am: 19 June 2018

angenommen am: 20 July 2017

Publication Date:
09 February 2018 (online)

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Zusammenfassung

Der Morbus Hirschsprung ist ein häufiges kinderchirurgisches Krankheitsbild.

Auch wenn eine Vielzahl von genetischen Mutationen beschrieben wurde, besteht die Trias der Diagnosestellung weiterhin in der sorgfältigen Anamnese, Kolon-Kontrastmitteluntersuchung und Rektumbiopsie.

Die Durchzugsoperation hat zum Ziel, den aganglionären Darm zu entfernen und die intestinale Passage durch Anastomosierung des innervierten Darms mit dem Analkanal unmittelbar oberhalb der Linea dentata zu rekonstruieren, sodass der Analkanal und die Sphinkterfunktion erhalten bleiben. Zu den häufigsten Techniken gehören die Operationen nach Swenson, Soave (de la Torre) und Duhamel. Diese können auch laparoskopisch assistiert durchgeführt werden.

Obwohl der Morbus Hirschsprung durch Resektion des aganglionären Darmes primär gut zu behandeln ist, ist die Langzeitmorbidität hoch. Diese wird vor allem durch die fort-bestehende Obstipation bis hin zur Stuhlinkontinenz oder rezidivierende Episoden einer Enterokolitis bestimmt. Durch eine zielgerichtete Diagnostik und multidisziplinäre Behandlung können diese Symptome meist im Verlauf mehrerer Jahre erfolgreich behandelt werden.

Der Pädiater spielt beim Erkennen und Management dieser Kinder eine wichtige Rolle.

Summary

The treatment of Hirschsprung disease is a common in pediatric surgery. Although a variety of genetic mutations have been described the triad of diagnosis consists of a thorough history, contrast enema and rectal biopsy.

The pull-through operation aims to resect aganglionic bowel and reestablish the intestinal passage by connecting healthy bowel to the anal canal just above the dentate (pecti-nate) line. While doing this the anal canal itself and the sphincter muscles have to be preserved by all means. Most common surgical techniques include the Swenson, Soave (de la Torre) and Duhamel pull-through. They can all be carried out by the help of laparoscopy.

In Hirschsprung disease the resection of aganglionic bowel is highly feasible, however long-term morbidity remains high. In later life ongoing constipation, fecal incontinence, and recurrent episodes of enterocolitis may be a problem. With a multidisciplinary approach these sequelae can be tackled successfully.

The pediatrician plays an important role in managing these children.

Schlüsselwörter

Aganglionose - Megakolon - Enterokolitis - Morbus Hirschsprung - Durchzugsoperation

Keywords

Aganglionosis - congenital megacolon - enterocolitis - Hirschsprung disease - pull-through
 

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