Neonatal-onset chronic intestinal pseudo-obstruction syndrome.

Between January 1985 and January 1990, six cases of neonatal-onset chronic intestinal pseudo-obstruction syndrome (CIPS) were identified. Failure to gain weight in six cases, abdominal distention in five, and vomiting in five were the most common presenting symptoms. The contrast studies of the gastrointestinal tract demonstrated delayed transit time in 6/6, jejunal or ileal dilatation in 1/6, megaduodenum in 1/6, dilatation 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. Except for hypoganglionosis in Patient 4, no recognizable neuropathy or myopathy was noted histopathologically. Four patients expired within 2 months after discharge. We conclude that CIPS with neonatal onset should be suspected when infants have urinary retention and abdominal distention or constipation beginning at birth or soon after. The prognosis of CIPS presenting in the newborn period appears worse than that presenting in childhood or adulthood.