Altersabhängige Viszeralmedizin: pädiatrische Viszeralmedizin – viszeralmedizinische Pädiatrie – Überlegungen zum Kurzdarmsyndrom im Kindesalter

Zusammenfassung

Die Ursachen für ein Kurzdarmsyndrom im Kindesalter sind vielfältig, die Häufigkeit gering. Die wesentlichen Gründe für die Entstehung sind ausgedehnte Darmresektionen nach angeborenen Fehlbildungen (Gastroschisis, intestinale Atresie oder Dysplasie) oder ischämischen Episoden (Volvulus). Neben diesen Formen sind auch funktionelle Ausprägungen zu beachten, welche durch oralnahe Stomaanlage entstehen können. Die Unterscheidung in temporäre und permanente Formen ist essenziell für den Ansatz der Behandlung. Bei Verlust von organischer Resorptionsfläche sind eine Vielzahl von pathologischen Veränderungen zu erwarten und zu behandeln. Aus pädiatrischer Sicht ist die Möglichkeit der intestinalen Adaptation einzuschätzen. Die weiteren Therapieoptionen bestehen in einem parenteralen und/oder enteralen Ernährungsregime sowie der Vermeidung drohender Komplikationen (z. B. Laktatazidose). Die chirurgischen Therapieoptionen bestehen im Wesentlichen aus der LILT-Methode nach Bianchi und oder der segmentalen Verengung durch das STEP-Verfahren. Die operative Anlage von antiperistaltischen Segmenten ist verlassen worden. Die Dünndarmtransplantation stellt mit der Verbesserung der Ergebnisse in den letzten Jahren nunmehr auch eine Option für das Kindesalter dar. Die wesentliche Schwierigkeit und somit auch die Hauptkomplikation als limitierender Faktor ist der Gefäßzugang bei der dauernden TPN-Behandlung (TPN: Totale Parenterale Ernährung). Hier sind oftmals die entscheidenden Gründe für die Letalität dieser Kinder zu finden.

Abstract

There are several reasons for the possible development of a short bowel syndrome, which, however, occurs only rarely. The main causes consist of extended intestinal resections in cases of congenital anomalies (e.g., gastroschisis, intestinal atresia or dysplasia) or ischaemic lesions due to a volvulus. In addition, an intestinal stoma at a more upper segment of the GI tract can result in the functional manifestation of a short bowel syndrome. The differentiation between temporary and persisting types is essential for initiation of an adequate treatment. Loss or exclusion of organic resorption area at the inner surface of the (small) intestine can be associated with numerous pathological consequences requiring treatment. As a principle consideration from the paediatric point of view, the potential of intestinal adaptation needs to be assessed. Basic conservative treatment options are parenteral and enteral nutrition regimens, in particular, to prevent complications (such as D-lactate acidosis). The main surgical approaches are the procedures called LILT (longitudinal intestinal lengthening and tailoring) according to Bianchi and STEP (serial transverse enteroplasty). The technique to create intestinal segments of antiperistalsis has been abandoned. Because of the encouraging results of intestinal transplantation, this novel treatment option has gained greater attention over the past few years and is now also an option for paediatric patients. The limiting factor and thus major complication is the central venous catheter for long-term treatment. Catheter-related complications are still the main reason for a considerable mortality in these children.

Schlüsselwörter

Kurzdarmsyndrom - Kindesalter - Totale Parenterale Ernährung - Chirurgische Optionen - zentralvenöse Katheter

Key words

short bowel syndrome - childhood - total parenteral nutrition - surgical options - central venous catheter

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