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Pseudo-obstruction intestinale chroniqueChronic intestinal pseudo-obstruction

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Résumé

La pseudo-obstruction intestinale chronique (POIC) désigne un syndrome clinique évoquant une obstruction mécanique de l’intestin grêle, mais pour laquelle aucun obstacle n’est mis en évidence. Il s’agit d’une affection rare et grave, d’origine primitive (40 %) ou secondaire (60 %).

Les principaux symptômes de pseudo-obstruction sont nausées, vomissements, météorisme, douleurs abdominales et troubles du transit à type de constipation et/ou diarrhée. Les symptômes précèdent souvent pendant des années le diagnostic positif de POIC. Ils aboutissent à terme à une dénutrition et des troubles hydro-électrolytiques parfois majeurs.

La prise en charge des malades nécessite une démarche rigoureuse : suspecter le diagnostic et éliminer un obstacle organique ; rechercher sur les examens cliniques et paracliniques les anomalies en rapport avec les segments atteints ; rechercher des atteintes extra-intestinales, en particulier de nature neurogène ou myogène ; discuter au cas par cas la réalisation de biopsies intestinales chirurgicales et/ou une biopsie neuromusculaire si une cytopathie mitochondriale est suspectée.

La prise en charge est centrée sur le contrôle des symptômes et l’assistance nutritionnelle. Les prokinétiques peuvent améliorer les symptômes digestifs. La nutrition entérale doit être préférée à la nutrition parentérale. La mise en place d’une double-sonde permettant une gastrostomie d’aspiration et une jéjunostomie d’alimentation peut améliorer le confort. La nutrition parentérale est généralement définitive. La transplantation intestinale peut-être proposée dans certaines situations.

Summary

Chronic intestinal pseudo-obstruction (CIPO) is a disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Pseudo-obstruction is an uncommon condition and can result from primary (40 %) or secondary (60 %) causes.

The most common symptoms are nausea, vomiting, abdominal distension, abdominal pain and constipation or diarrhea. These symptoms are usually present many years before CIPO diagnosis. They can lead to severe electrolyte disorders and malnutrition.

Principles for management of patients with CIPO are: to establish a correct clinical diagnosis in excluding mechanical obstruction; to perform a symptomatic and physiologic assessment of the gastrointestinal tract involved; to look for extra-intestinal manifestations, especially for myopathy and neuropathy; to discuss in some cases a surgery for full-thickness intestinal biopsies, and/or a neuromuscular biopsy in case of mitochondrial cytopathy suspicion.

The management is primarily focused on symptom control and nutritional support to prevent weight loss and malnutrition. Treatment of CIPO includes prokinetic agents which may help to reduce gastrointestinal symptoms Courses of antibiotics may be needed in patients with symptoms suggestive of bacterial overgrowth. When necessary, enteral nutrition is preferred. In carefully selected patients, feeding jejunostomy with or without decompression gastrostomy may be tried. Long term parenteral nutrition should be reserved for patients who can not tolerate enteral nutrition. Intestinal transplantation can be discussed in selected patients.

Références (106)

  • D.S. Smith et al.

    Diagnosis and treatment of chronic gastroparesis and chronic intestinal pseudo-obstruction

    Gastroenterol Clin North Am

    (2003)
  • V.A. Lennon et al.

    Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma

    Gastroenterology

    (1991)
  • H.R. Lee et al.

    Paraneoplastic gastrointestinal motor dysfunction: clinical and laboratory characteristics

    Am J Gastroenterol

    (2001)
  • R. De Giorgio et al.

    Anti-HuD-induced neuronal apoptosis underlying paraneoplastic gut dysmotility

    Gastroenterology

    (2003)
  • L.A. Mueller et al.

    Mitochondrial neurogastrointestinal encephalomyopathy: manometric and diagnostic features

    Gastroenterology

    (1999)
  • M. Hirano et al.

    Mitochondrial neurogastrointestinal encephalomyopathy syndrome maps to chromosome 22q13

    32-qter. Am J Hum Genet

    (1998)
  • J. Gamez et al.

    A novel thymidine phosphorylase mutation in a Spanish MNGIE patient

    J Neurol Sci

    (2005)
  • T.M. Chang et al.

    Paralytic ileus in MELAS with phenotypic features of MNGIE

    Pediatr Neurol

    (2004)
  • S.H. Leon et al.

    Chronic intestinal pseudoobstruction as a complication of Duchenne's muscular dystrophy

    Gastroenterology

    (1986)
  • E.S. Bonapace et al.

    Whole gut transit scintigraphy in the clinical evaluation of patients with upper and lower gastrointestinal symptoms

    Am J Gastroenterol

    (2000)
  • A.J. Bredenoord et al.

    Gastric accommodation and emptying in evaluation of patients with upper gastrointestinal symptoms

    Clin Gastroenterol Hepatol

    (2003)
  • M.D. Schuffler et al.

    Esophageal motor dysfunction in idiopathic intestinal pseudoobstruction

    Gastroenterology

    (1976)
  • M.D. Schuffler et al.

    Chronic idiopathic intestinal pseudo-obstruction caused by a degenerative disorder of the myenteric plexus: the use of Smith's method to define the neuropathology

    Gastroenterology

    (1982)
  • V. Annese et al.

    Erythromycin accelerates gastric emptying by inducing antral contractions and improved gastroduodenal coordination

    Gastroenterology

    (1992)
  • J. Tack et al.

    Effect of erythromycin on gastric motility in controls and in diabetic gastroparesis

    Gastroenterology

    (1992)
  • C.M. Prather et al.

    Tegaserod accelerates orocecal transit in patients with constipation-predominant irritable bowel syndrome

    Gastroenterology

    (2000)
  • A. Attar et al.

    Antibiotic efficacy in small intestinal bacterial overgrowth-related chronic diarrhea: a crossover, randomized trial

    Gastroenterology

    (1999)
  • V. Geromel et al.

    Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits

    Mol Genet Metab

    (2002)
  • P.G. Patrick et al.

    Endoscopic nasogastric-jejunal feeding tube placement in critically ill patients

    Gastrointest Endosc

    (1997)
  • R.W. Mc Callum et al.

    Gastric pacing improves emptying and symptoms in patients with gastroparesis

    Gastroenterology

    (1998)
  • F. Joly et al.

    Subtotal small bowel resection (SBR) in chronic intestinal pseudo-obstruction (CIPO) refractory to treatment

    Clin Nutr

    (2003)
  • C.D. Rudolph et al.

    Diagnosis and treatment of chronic intestinal pseudo-obstruction in children: report of consensus workshop

    J Pediatr Gastroenterol Nutr

    (1997)
  • M.D. Schuffler et al.

    Chronic intestinal pseudo-obstruction. A report of 27 cases and review of the literature

    Medicine (Baltimore)

    (1981)
  • R. De Giorgio et al.

    Review article: the pharmacological treatment of acute colonic pseudo-obstruction

    Aliment Pharmacol Ther

    (2001)
  • V. Stanghellini et al.

    Chronic idiopathic intestinal pseudo-obstruction: clinical and intestinal manometric findings

    Gut

    (1987)
  • B. Coulie et al.

    Intestinal pseudo-obstruction

    Annu Rev Med

    (1999)
  • R.K. Goyal et al.

    The enteric nervous system

    N Engl J Med

    (1996)
  • B. Coffin

    Troubles moteurs intestinaux

  • B. Coffin et al.

    Ileal tone in humans: effects of locoregional distensions and eating

    Am J Physiol

    (1994)
  • R. De Giorgio et al.

    Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction

    Gut

    (2004)
  • C. Di Lorenzo

    Pseudo-obstruction: current approaches

    Gastroenterology

    (1999)
  • K. De Schryver-Kecskemeti et al.

    Perineural and intraneural inflammatory infiltrates in the intestines of patients with systemic connective-tissue disease

    Arch Pathol Lab Med

    (1989)
  • G. Sarnelli et al.

    Reduced Bcl-2 Expression and Increased Myenteric Neuron Apoptosis in Patients With Idiopathic Enteric Neuropathy

    Gastroenterology

    (2005)
  • R.P. Kapur

    Hirschsprung disease and other enteric dysganglionoses

    Crit Rev Clin Lab Sci

    (1999)
  • M. Seri et al.

    Frequency of RET mutations in long- and short-segment Hirschsprung disease

    Hum Mutat

    (1997)
  • M. Camilleri

    Enteric nervous system disorders: genetic and molecular insights for the neurogastroenterologist

    Neurogastroenterol Motil

    (2001)
  • K. Robertson et al.

    Hirschsprung's disease: genetic mutations in mice and men

    Gut

    (1997)
  • C.H. Knowles et al.

    Deranged smooth muscle alpha-actin as a biomarker of intestinal pseudo-obstruction: a controlled multinational case series

    Gut

    (2004)
  • A.E. Feldstein et al.

    Chronic intestinal pseudoobstruction associated with altered interstitial cells of cajal networks

    J Pediatr Gastroenterol Nutr

    (2003)
  • K. Isozaki et al.

    Deficiency of c-kit+ cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction

    Am J Gastroenterol

    (1997)
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