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Chronic Intestinal Pseudo-obstruction Syndrome: Surgical Approach and Intestinal Transplantation

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Pediatric Neurogastroenterology

Part of the book series: Clinical Gastroenterology ((CG))

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Abstract

Chronic intestinal pseudo-obstruction syndrome (CIPOS) is a rare and severe syndrome representing one of the main causes of intestinal failure (IF). Its diagnosis is based on typical clinical manifestations, radiological evidence of distended bowel loops with air-fluid levels, and the exclusion of any organic obstruction of the gut lumen. In children, most cases are idiopathic and sporadic. Based on histological features CIPOS is classified into two main groups: neuropathies and myopathies. It is characterized by impairment of physical growth and development and requires usually parenteral nutrition for periods or for long-term. Accompanying uropathies must be sought in patients with CIPOS. Long-term outcomes are generally poor despite surgical and medical therapies and characterized by disabling and potentially life-threatening complications. Treatment of CIPOS involves nutritional, pharmacological, and surgical therapies but is often frustrating and does not change the natural course in the majority of cases. Medical management remains difficult and prognosis poor. Surgery is performed in a variety of situations in pediatric patients but surgical options must be evaluated carefully. There is no consensus regarding indications and procedures. They include surgery for diagnosis and intestinal full-thickness biopsies, for performing enterostomy (preferably an ileostomy), for resuming repeated obstruction episodes. Repeated surgery may be deleterious in providing peritoneal adhesions and subsequent additional obstruction episodes. Two types of surgery are “extreme.” One is subtotal enterectomy that may improve the quality of life (QOL) by decreasing the obstruction, but it leads to permanent parenteral nutrition with subsequent risk of complications. The other one is intestinal transplantation (ITx) with or without the liver. Multivisceral (stomach, duodenum, pancreas, small intestine, and part of colon) or modified multivisceral Tx (without the liver) have been successfully performed with long-term patient or graft survival being >50 %. Beside the classical indications for ITx (repeated sepsis, liver fibrosis, or cirrhosis, high fluid losses, etc.) poor QOL may be considered. A trained multidisciplinary team, including surgeons, gastroenterologists, nutritionists, and a home PN coordinator, should assume the management of these patients which may involve a PN program and transplant surgery.

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Authors and Affiliations

  1. Department of Pediatric, Gastroenterology Hepatology and Nutrition, National Reference Center for Rare Digestive Diseases, Hospital Necker-Enfants Malades, University of Paris Descartes, 149 rue de Sèvres, 75015, Paris, France

    Olivier Goulet M.D., Ph.D.

  2. Pediatric Surgery, Hôpital Necker-Enfants Malades, University of Paris, Paris, France

    Sabine Irtan M.D.

Authors
  1. Olivier Goulet M.D., Ph.D.
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  2. Sabine Irtan M.D.
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Correspondence to Olivier Goulet M.D., Ph.D. .

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Editors and Affiliations

  1. Sainte-Justine Hospital, Department of Pediatrics, Division of Pediatric Gastroenterology, Université de Montréal, Montreal, H3T 1C5, Québec, Canada

    Christophe Faure

  2. Nationwide Children's Hospital, Division of Pediatric Gastroenterology, The Ohio State University, Columbus, 43205, Ohio, USA

    Carlo Di Lorenzo

  3. Gastroenterology and Neural Development, University College London, Institute of Child Health, Great Ormond Street Hospital for Children, London, WC1N 1EH, United Kingdom

    Nikhil Thapar

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Goulet, O., Irtan, S. (2013). Chronic Intestinal Pseudo-obstruction Syndrome: Surgical Approach and Intestinal Transplantation. In: Faure, C., Di Lorenzo, C., Thapar, N. (eds) Pediatric Neurogastroenterology. Clinical Gastroenterology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-709-9_46

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  • DOI: https://doi.org/10.1007/978-1-60761-709-9_46

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