Kurzdarmsyndrom und Darmversagen: Diagnostik und Therapie

Zusammenfassung

Das chronische Darmversagen ist eine seltene Krankheitsentität, die durch einen Verlust an Darmlänge oder Agenesie (Kurzdarmsyndrom) oder durch funktionelle Störungen der Mukosa bzw. der Motilität des Dünndarms bedingt sein kann. Die Inzidenz für ein Kurzdarmsyndrom liegt bei ca. 2 – 3 Patienten/1 Mio./Jahr, die Prävalenz bei ca. 4 – 5/1 Mio./Jahr. Das irreversible chronische Darmversagen geht mit der Notwendigkeit einer total parenteralen Ernährung einher. Das mit dem Kurzdarmsyndrom verbundene Malassimilationssyndrom resultiert in spezifischen medizinischen Anforderungen und einer Vielzahl von potenziellen Komplikationen, die aufgrund ihrer Komplexität und Seltenheit eine Behandlung in spezialisierten Kliniken notwendig machen. Kann unter begleitender konservativer Therapie keine enterale Autonomie hergestellt werden, sodass eine lebenslange Abhängigkeit von partiell oder total parenteraler Ernährung besteht, so steht die Vermeidung von schwerwiegenden Komplikationen, wie z. B. der mit dem Darmversagen assoziierten cholestatischen Hepatopathie (IFALD; Intestinal Failure Associated Liver Disease) und schweren katheterassoziierten Komplikationen im Vordergrund. In den letzten Jahren hat sich die Darmtransplantation als erfolgreiche Behandlungsoption für Patienten mit kompliziert verlaufendem, irreversiblem chronischem Darmversagen etabliert.

Abstract

Chronic intestinal failure is a rare disease entity which either results from short bowel syndrome due to agenesia or the loss of small intestine during surgery, or from functional disorders of the mucosa, or motility disorders. The incidence of intestinal failure is approximately 2 – 3 per million population, the estimated prevalence is 4 – 5 per million population. Irreversible intestinal failure requires total parenteral nutrition and is associated with a state of malassimilation which results in special medical requirements and specific potential complications which require treatment in specialized centers. If enteral autonomy cannot be re-established and life-long dependency on parenteral nutrition is indicated, the avoidance of typical complications related to intestinal failure, such as intestinal failure associated liver disease (IFALD) and catheter-related complications is of major concern. Intestinal transplantation has emerged as successful therapeutic option for those patients with complicated irreversible intestinal failure over the last years.

Schlüsselwörter

Kurzdarmsyndrom - chronisches Darmversagen - intestinale Adaptation - parenterale Ernährung - Darmtransplantation

Keywords

short bowel syndrom - intestinal failure - intestinal adaptation - parenteral nutrition - intestine transplantation

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