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December 1957

Hirschsprung's Disease: A Lethal Problem in Early Infancy

Author Affiliations

Dallas, Texas

AMA Arch Surg. 1957;75(6):906-913. doi:10.1001/archsurg.1957.01280180038006

Hirschsprung's disease is particularly devastating to children under 6 months of age, and, contrary to popular opinion, relatively few survive early infancy to develop eventually the classical and better-known clinical picture originally described by Hirschsprung.1 Even since 1948, when Swenson and Bill published their original work on the development of a curative surgical operation to correct this anomaly,2 the high mortality among young infants with this disease has continued as a shocking paradox to the many subsequent glowing reports on the successful management of this problem in slightly older children.

The term Hirschsprung's disease implies herein the specific functional disorder of bowel caused by congenital dysplasia of the myenteric nervous plexus. The indefinite term megacolon is used only in its literal sense, meaning an enlarged and hypertrophied colon. Idiopathic megacolon, as defined by Bodian, Stephens, and Ward,3 and megacolon due to a distal organic obstructive lesion will