Successful nutrition management of megacystis-microcolon-intestinal hypoperistalsis syndrome--a case report.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis-microcolon -intestinal hypoperistalsis syndrome (MMIHS ), a visceral myopathy causing intestinal obstruction in the newborn , is a generally fatal condition , with death being secondary either to sepsis or to malnutrition if long -term intravenous feeding is not provided . A patient with MMIHS is described who has been raised by total parenteral nutrition (TPN ) for seven years since her birth . Severe hepatic dysfunction was encountered in early infancy , which gradually cleared after the initiation of milk feeding by mouth , although the milk could not be absorbed because of the high -output jejunostomy . The patient also experienced a bone disease similar to scurvy but caused by copper deficiency at the age of 9 months . The central venous catheter now in situ is the 25 th one for the patient . When these catheters were evaluated , the Broviac proved more efficacious than the traditional Silastic for use in long -term TPN . The patient does not yet have normal bowel function and still requires TPN .