Hirschsprung’s disease is one of the most important and most fascinating diseases in paediatric surgery. Our understanding of Hirschsprung’s disease is developing rapidly, not only in relation to its pathophysiology and the development of new surgical techniques, but especially in relation to new genetic findings. A first comprehensive description of the pathophysiology, clinical symptoms, diagnosis and therapy of Hirschsprung’s disease was outlined in 1970 by Theodor Ehrenpreis, Professor of Pediatric Surgery at the Karolinska Institute, Stockholm, Sweden, in a booklet entitled “Hirschsprung’s Disease”. The booklet of 176 pages was dedicated to Harald Hirschsprung (1830–1916) of Copenhagen, Denmark, and to Ovar Swenson of Chicago, Illinois, USA, the two pioneers in the study of Hirschsprung’s disease
