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April 1968

Congenital Aganglionic Megacolon (Hirschsprung's Disease): Diagnosis, Management, and Complications

Author Affiliations

Iowa City
From the Department of Surgery, University of Iowa College of Medicine, Iowa City.

Arch Surg. 1968;96(4):554-562. doi:10.1001/archsurg.1968.01330220070013

CONGENITAL aganglionosis of the colon (Hirschsprung's disease) is the most common congenital, nonmechanical cause of intestinal obstruction. In England its incidence has been estimated at 1:2,000 births1 and in the United States 1:5,000 births.2 It has a strong male sex preponderance of 4 to 5:1, but curiously enough, females with Hirschsprung's disease tend to have greater lengths of aganglionic bowel than males. There is a mild familial pattern to the inheritance of Hischsprung's disease, and a statistically significant correlation with mongolism,3 although its exact mode of inheritance is unknown.

The purpose of this paper is to review 30 patients with biopsy-proven Hirschsprung's disease who were seen at the University of Iowa Hospitals during the past six years.

Materials and Methods  Of the 30 patients with congenital aganglionosis, 26 were males. The four female patients had only conventional extent of aganglionosis (rectum and sigmoid), not showing the reported

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