THE WALKING THRU THE PAST AND THE PRESENT OF HIRSCHSPRUNG DISEASE

Abstract

Hirschsprung’s disease or congenital megacolon is one of the differential diagnoses of chronic constipation mostly in infancy and may indeed represent a challenge for pediatricians, pediatric surgeons, and pediatric pathologists. The diagnosis relies clearly on the identification of the absence of ganglion cells at the plexuses (submucous and myentericus) of the bowel wall. Hirschsprung’s disease) is usually located at the terminal (distal) rectum with potential pre-terminal or proximal extension to the less distal large bowel (sigmoid colon). Astonishingly, there is some evidence that Hindu surgeons of prehistoric India may have been exposed and had considerable knowledge about Hirschsprung’s disease), but this disease is notoriously and eponymously named to Dr. Harald Hirschsprung (1830-1916), who brilliantly presented two infants with fatal constipation at the Berlin conference of the German Society of Pediatrics more than one century ago. Hirschsprung’s disease has been originally called “Die Hirschsprungsche Krankheit”). More than 100 years following his meticulous and broad description, Hirschsprung’s disease is still a puzzling disease for both diagnosis and treatment. Hirschsprung’s disease remains a critical area of clinical pediatrics and pediatric surgery and an intense area of investigation for both molecular and developmental biologists.