Hirschsprung's disease in the newborn.

Hirschsprung's disease has become a more common cause of newborn intestinal obstruction in the past 30 years. In a group of 137 newborns with intestinal obstruction the most common diagnosis was necrotizing enterocolitis. The second most common cause, however, was Hirschsprung's disease. We have reviewed 26 infants with aganglionosis who presented at 32 days of age or less between 1972 and 1978. The average age at presentation was 8.3 days. There were 21 males and five females. Five infants had long-segment or total colonic disease. There was only one premature infant (34 weeks, 1840 g). The mean birthweight in the series was 3.6 kg. Six children had a family history of a congenital anomaly (23%). Three of these had a family history of Hirschsprung's disease (12%). Nine infants (35%) had associated congenital anomalies. Four of these newborns had Down's syndrome, and all four had a cardiac anomaly as well. Fifteen newborns presented with emesis (58%) which was bilious in nine (35%) cases. Seventeen babies (65%) had abdominal distension at the time of presentation. Eleven infants passed a meconium stool by 24 hours of age (42%), and 15 had passed meconium by 48 hours (58%). Twenty-two of 24 (92%) barium enema examinations available prior to diagnosis were diagnostic of Hirschsprung's disease. All of the 23 suction rectal biopsies were positive. All 26 patients underwent a colostomy or ileostomy following diagnosis. There was no enterocolitis and no mortality. All 26 patients have had an endorectal pullthrough performed at a mean age of 11.8 months without major complications.(ABSTRACT TRUNCATED AT 250 WORDS)