Which of the following statements about Hirschsprung's disease is/are true?

A. There are no ganglion cells seen in Auerbach's plexus.

B. There is an increased incidence of Down syndrome.

D. It may be associated with enterocolitis.

E. It may involve the small intestine. 

DISCUSSION: The affected segment of bowel in patients with Hirschsprung's disease has hypertrophic nerves in Auerbach's intermyenteric plexus, but no ganglion cells are present. Ganglion cells are also absent in Meissner's submucosal plexus. Some 3% to 5% of babies with Hirschsprung's disease also have Down syndrome. Hirschsprung's disease should be suspected in infants with Down syndrome that manifest evidence of abdominal distension and constipation. Hirschsprung's disease is much more common in boys (4:1). The enterocolitis of Hirschsprung's disease is a condition associated with delay in diagnosis, low bowel obstruction, severe abdominal distension, explosive diarrhea, and colonic mucosal ulceration. The course may be fulminant. This complication is associated with increased morbidity and mortality. Bacterial translocation and endotoxemia may complicate the condition. Treatment includes nasogastric suction, intravenous fluids, antibiotics, and rectal tube decompression of the obstructed rectosigmoid segment. In approximately 10% of cases aganglionosis extends into varying lengths of small bowel. In rare instances, the entire small bowel and colon may be aganglionic.