Nutritional and Surgical Management of Pediatric Intestinal Motility Disorders

  • Mikko P. Pakarinen
  • Laura Merras-Salmio
  • Annika Mutanen
Reference work entry


Intestinal motility disorders are increasingly recognized conditions characterized by impaired propulsion of enteric contents in the absence of mechanical bowel occlusion. Clinical presentation ranges from mild symptoms to rare disabling and life-threatening disorders. A significant proportion of patients require long-term parenteral nutrition (PN) to survive. Chronic intestinal pseudo-obstruction (CIPO) and Hirschsprung disease (HD) are among the most common reasons for pediatric intestinal failure (IF) with high mortality. CIPO involves variable defects of the intestinal neuromuscular apparatus. Besides extended aganglionosis, HD may result in IF due to persistent generalized dysmotility of the remaining ganglionic intestine. The management of severe intestinal motility disorders is challenging, the main goals being improvement of intestinal passage while maintaining of adequate nutrition and growth. These goals are best achieved by multidisciplinary approach. Because effectiveness of prokinetic medication alone is often poor, the mainstay of treatment is nutritional support. Every effort should be made to avoid life-threatening complications of long-term PN, such as IF-associated liver disease (IFALD), frequent septicemia, and loss of venous access sites when intestinal transplantation (IT) serves as a salvage therapy. With critical patient selection, also non-transplant surgery can effectively relieve intestinal obstruction, facilitate enteral tolerance, aid to avoid development of IFALD, and provide prerequisite for weaning off PN.


Parenteral Nutrition Plant Sterol Short Bowel Syndrome Medium Chain Triglyceride Intestinal Transplantation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations


Chronic intestinal pseudo-obstruction


Hirschsprung disease


Intestinal failure


Intestinal transplantation


Parenteral nutrition


Short bowel syndrome


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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Mikko P. Pakarinen
    • 1
  • Laura Merras-Salmio
    • 2
  • Annika Mutanen
    • 1
  1. 1.Section of Pediatric Surgery, Pediatric Liver and Gut Research GroupChildren’s Hospital, Helsinki University Central Hospital, University of HelsinkiHelsinkiFinland
  2. 2.Section of Pediatric GastroenterologyChildren’s Hospital, Helsinki University Central Hospital and University of HelsinkiHelsinkiFinland

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