Hirschsprun's Disease

a congenital disease manifested by hypertrophy and dilatation of the central (proximal) section of the large intestine. It was first described in 1887 by the Danish physician H. Hirschsprung (1830-1916).

Hirschsprung’s disease is caused by a congenital defect in development—a deficiency of ganglia of the parasympathetic (Auerbach’s) neural plexus in the terminal (distal) section of the large intestine, as a result of which peristalsis is absent in that section and the patency of the intestine is disturbed. There is compensatory dilatation and hypertrophy of the higher section of the colon. Hirschsprung’s disease is manifested by persistent constipation and abdominal distention from the day of birth. The diagnosis is confirmed by X ray. If necessary, a biopsy of the wall of the rectum is performed. Treatment involves cleansing enemas, mineral oil taken internally, and abdominal massage and digital dilatation of the rectal sphincter. If the patient’s condition is serious and it is impossible to rid the colon of the fecal mass, surgery is performed. (In children up to one year old, this involves removal of the affected section of the intestine and creation of an artificial anus.)