Rare Diseases Symptoms Automatic Extraction

Understanding pediatric intestinal pseudo-obstruction: implications for nurses.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Pseudo-obstruction is actually a group of disorders resulting in bowel dysmotility in the absence of anatomic obstruction. Because of varied pathologic abnormalities of the GI tract involved in the disorder, pseudo-obstruction has been reported in the literature under different names, including hypoganglionosis, chronic adynamic ileus, pseudo-Hirschprung's disease, visceral neuropathy, visceral myopathy, and megacystis-microcolon-intestinal hypoperistalsis syndrome. Although a rare condition, its effects are severe, disabling, and life-threatening in pediatric clients. Prognosis is inversely correlated with age and is especially poor in those children with symptoms as newborns. Management involves promoting nutrition adequate for growth, treating symptoms, and preventing complications. Total parenteral nutrition is almost always required, and its long-term use is associated with chronic cholestasis and potential hepatic failure. Nurses may encounter pediatric clients with this disorder and their families in many different healthcare arenas, such as in radiology departments, ambulatory care, and acute and home care settings. The purpose of this article is to review current literature on this disease and summarize information useful to nurses.