NEONATAL-ONSET CHRONIC INTESTINAL PSEUDOOBSTRUCTION SYNDROME

Between January 1985 and January 1990, six cases of neonatal-onset chr onic intestinal pseudo-obstruction syndrome (CIPS) were identified. Fa ilure to gain weight in six cases, abdominal distention in five, and v omiting in five were the most common presenting symptoms. The contrast studies of the gastrointestinal tract demonstrated delayed transit ti me in 6/6, jejunal or ileal dilatation in 1/6, megaduodenum in 1/6, di latation of the colon with barium retention in 4/6, and microcolon in 1/6, Urinary tract involvement was noted in three patients. Laparotomy , performed in three patients, revealed no mechanical obstruction. Exc ept for hypoganglionosis in Patient 4, no recognizable neuropathy or m yopathy was noted histopathologically. Four patients expired within 2 months after discharge. We conclude that CIPS with neonatal. onset sho uld be suspected when infants have urinary retention and abdominal dis tention or constipation beginning at birth or soon after, The prognosi s of CIPS presenting in the newborn period appears worse than that pre senting in childhood or adulthood.