AN EPIDEMIOLOGIC-STUDY OF HIRSCHSPRUNGS-DISEASE AND ADDITIONAL ANOMALIES

The incidence of Hirschsprung's disease was studied in approximately 1 .5 million consecutive live births in Denmark by hospital records. A d iagnosis of Hirschsprung's disease required a histologic verified abse nce of ganglion cells in either biopsy or surgical colonic specimens. The incidence of Hirschsprung's disease was found to be 0.140 per 1000 live births (1:7,165) with a male:female ratio of 4.1:1 in short segm ent, and 2.4:1 in long segment Hirschsprung's disease (p = 0.36). Mate rnal age and birth order were unimportant factors. The association of Hirschsprung's disease and Down's syndrome was seen in 9 of the 207 pa tients and may represent a real association, whereas the association w ith congenital heart defects seen in 2% (not including patients with D own's syndrome) is more doubtful. A mortality of 16% among the patient s with Hirschsprung's disease emphasizes the extreme importance of ear ly diagnosis.