Early diagnosis and treatment of Hirschsprung's disease in New Mexico.

Four decades ago, the mortality of congenital aganglionosis of the distal part of the colon (Hirschsprung's disease) approached 100 per cent. The development of diagnostic methods and curative operations lowered the mortality to 5 to 10 per cent. Enterocolitis remained the major cause of death. In this series of 18 children with Hirschsprung's disease, the majority were diagnosed as neonates. Although enterocolitis was present at diagnosis in four (22 per cent), all children survived after prompt surgical therapy. Neonatal diagnosis and treatment may prevent the development of fatal enterocolitis, virtually eliminating the mortality of Hirschsprung's disease.

Congenital defects of the abdominal wall. A review of the experience in New Mexico.

Omphalocele, umbilical cord hernia, and gastroschisis are surgically correctable defects of the abdominal wall. Each of these defects has a distinct embryologic basis that results in a characteristic clinical picture. Twenty-five infants with congenital defects of the abdominal wall were treated at the University of New Mexico Hospital in the past four years. Six infants had omphalocele, one had umbilical cord hernia, and 18 had gastroschisis. Survival among infants who underwent a corrective operation was as follows: omphalocele, 50%; umbilical cord hernia, 100%; and gastroschisis, 82%. Long-term survival for the entire group was 72% (18/25). Gastroschisis, which had a lower incidence of major associated anomalies, had a better prognosis than omphalocele. The mortality of congenital abdominal wall defects was related to presence of severe associated anomalies and to poor clinical condition on admission. Prompt and informed initial care may increase the chance of survival.