Hirschsprung's Disease in the Newborn

Hirschsprung's disease is one of the more frequent causes of intestinal obstruction in the newborn. Improvements in the methods of diagnosis and management of patients in this group have increased the survival rate. The 3-day-old infant in Figure 1 has the signs of acute intestinal obstruction. He has become dehydrated and lethargic from fluid and electrolyte loss due to repeated bile-stained vomiting. In addition there is abdominal distension and obstipation. Many of the infants with Hirschsprung's disease present with symptoms and signs indistinguishable from other cause of intestinal obstruction such as ileal atresia. Table 1 compares the incidence of vomiting, obstipation, and abdominal distension in a group of neonates with Hirschsprung's disease and another group with ileal atresia. Figures 2 through 5 indicate the proper diagnostic methods to differentiate Hirschsprung's disease from other causes of acute intestinal obstruction in the newborn. Prompt abdominal exploration is needed in small bowel obstruction,