Nutritional and Surgical Management of Pediatric Intestinal Motility Disorders
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.
KeywordsParenteral Nutrition Plant Sterol Short Bowel Syndrome Medium Chain Triglyceride Intestinal Transplantation
List of Abbreviations
Chronic intestinal pseudo-obstruction
Short bowel syndrome
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