Hirschsprung’s Disease: A Historical Perspective — 1691–2005

Abstract

Hirschsprung’s disease is a common cause of neonatal intestinal obstruction that is of great interest to pediatric surgeons throughout the world. Prior reports concerning the historical origins ascribe the initial description of this condition to Fredericus Ruysch, a Dutch anatomist in Amsterdam in 1691 [20, 33, 91, 137]. He described a 5-year-old girl with abdominal pain who did not respond to the “usual treatment of the day to relieve pain, pass wind and kill worms”. She eventually died. The information regarding the patient was incomplete in regard to the events that occurred at the time of her birth and except for enormous dilatation of the colon, the autopsy findings were not clearly described. Although this may have represented a case of Hirschsprung’s disease there was inadequate evidence to be sure of the actual diagnosis [33]. Similarly, Domenico Battini in Italy in 1800 described a child whom he followed for 10 years with severe constipation who eventually died and demonstrated severe colonic dilatation at autopsy consistent with, but not pathognomonic of, megacolon [39]. An additional report by Ebers in 1836 noted a 17-year-old boy with a history of constipation “since early youth” who died [33].