Die chronische intestinale Pseudoobstruktion: Pathogenese, Diagnostik und Therapie

Zusammenfassung

Die chronische intestinale Pseudoobstruktion (CIPO) ist ein seltenes Krankheitsbild, bei dem eine schwere intestinale Motilitätsstörung zur Behinderung des Chymustransports bis zum Vollbild eines mechanischen Ileus führt, ohne dass ein Passagehindernis vorliegt. Eine CIPO kann als primäre Erkrankung oder sekundär auftreten aufgrund muskulärer Erkrankungen, neurologischer, metabolischer und endokriner Störungen, aber auch postinfektiös, postoperativ, nach abdomineller Bestrahlung, durch Medikamente oder Noxen. Beim Vollbild ist die typische Anamnese mit frustranen Laparotomien unter dem Verdacht eines mechanischen Ileus diagnostisch wegweisend. Bei Verdacht auf eine CIPO muss mithilfe radiologischer und endoskopischer Verfahren sorgfältig nach einem mechanischen Hindernis gesucht werden. Aussagekräftige histologische Untersuchungen sind i. d. R. nur an transmuralen Darmwandbiopsien möglich. Mittels Dünndarmmanometrie kann die Diagnose einer CIPO auch im Intervall gesichert und zwischen Neuropathie und Myopathie unterschieden werden. Primäre Therapieziele sind: 1. Die Aufrechterhaltung eines adäquaten Ernährungszustandes durch orale und/oder enterale Ernährung; bei schweren Verlaufsformen kommt eine heimparenterale Ernährung und insbesondere bei Kindern als Ultima Ratio die Dünndarmtransplantation infrage. 2. Die Wiederherstellung einer intestinalen Propulsion durch Prokinetika. 3. Die Behandlung von Komplikationen wie bakterielle Fehlbesiedlung und ausgeprägte Schmerzen durch Antibiotika bzw. gezielte chirurgische Eingriffe. Unnötige Operationen sollten unbedingt vermieden werden, da sie das klinische Bild durch Bridenbildung wesentlich komplizieren können.

Abstract

Chronic intestinal pseudoobstruction (CIPO) is a rare disease in which a severe intestinal motility disorder impairs transit of chyme so that patients suffer from symptoms of a mechanical ileus without mechanical obstruction. CIPO may be a primary or secondary disorder due to muscular, neurologic, metabolic or endocrine disorders, but may also occur postinfectiously, postoperatively, following abdominal radiation or be caused by drugs or noxae. In severe cases, the typical history of (repeated) symptoms of mechanical obstruction leading to unsuccessful laparotomies will give key clues for diagnosis. If CIPO is suspected, mechanical obstruction must be searched for carefully by radiologic and endoscopic examinations. Histologic diagnosis usually demands full thickness biopsies of the intestinal wall. Small intestinal manometry allows diagnosis of CIPO even during oligosymptomatic intervals as well as differentiation among neuropathic and myopathic forms of the disease. The main therapeutic goals consist in: 1. Maintenance of an adequate nutritional state by oral and/or enteral nutrition; in severe cases home-parenteral nutrition may be required and particularly in children intestinal transplantation may be the ultima ratio. 2. Reconstitution of intestinal propulsion by prokinetic drugs. 3. Therapy of complications such as bacterial overgrowth and severe pain by antibiotics and specific surgical procedures. Unnecessary laparotomies should be strictly avoided because they may lead to adhesions and markedly complicate the clinical course.

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