Abstract
Chronic intestinal pseudo-obstruction (CIPO) is a diagnosis which encompasses a heterogeneous group of rare disorders involving intrinsic enteric nerves, muscles, and/or connective tissues which impairs gastrointestinal motility. CIPO is in a general sense the motility disorder with the greatest morbidity and mortality per patient. One third of those with congenital CIPO require total parenteral nutrition (TPN), and it is those infants and children who are at risk for serious complications such as sepsis, thrombo-embolic disease, TPN-associated liver disease, and gallstones.
Factors relating to better outcomes include maintaining good nutrition, avoiding acute intercurrent illness, enteral nutrition support, and avoiding surgery. Clinicians strive first to support normal growth and development. Second, clinicians strive to utilize the gastrointestinal tract for nutrition support rather than using TPN. Treatment of abdominal distension, abdominal pain, nausea and vomiting often require decompression of dilated bowel as a first step. Decompression may be accomplished by gastrostomy or ileostomy. Improvements in management may include bowel transplantation or stem cell therapy to replace diseased nerves or muscle.
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Hyman, P.E., Thapar, N. (2013). Chronic Intestinal Pseudo-Obstruction. 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_22
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