ABSTRACT
Hirschsprung disease (HD) has been recognized since 1691, when Dutch anatomist Frederic Ruysch described a case of congenital megacolon at autopsy. Over the ensuing 300 years, the surgeons who struggle to treat this condition have slowly grown to understand it [1]. Harald Hirschsprung, [2] the Danish pediatrician whose name the disorder bears, described two patients who died of chronic constipation and megacolon in 1887. Tittle noted an absence of ganglia in the distal bowel in 1901. He was followed by Tiffin et al. in 1940, who proposed that the aganglionic bowel lacked peristalsis, which accounted for the symptoms. However, it was not until the pioneering work of Swenson and Bill [3], who performed the first surgical correction for the disorder in 1948, that effective treatment became available. Despite the amount of energy expended on Hirschsprung disease by the medical community, its etiology has not yet been completely defined, and the dysfunction that it manifests still occasionally leads to “cures” that are less than satisfying. It is to be hoped that the biochemical elucidation of
200 Lally
the disorder during the last decade will allow a clearer understanding of the pathology.
