ABSTRACT

Hirschsprung’s disease (HD) is characterized by an absence of ganglion cells in the nerve plexuses of the distal large bowel. The lack of ganglion cells produces a functional obstruction and leads to dilatation of the bowel that is proximal to the aganglionic zone. The commonly quoted incidence of HD is 1 in 5000. The classic description of HD was presented by Danish pediatrician, Harald Hirschsprung, in 1886. The absence of ganglion cells in the distal large bowel was first reported in the early 1900s but the crucial role of this finding as the primary pathology was not appreciated until the late 1940s. In 1948, the first successful operation for HD was performed by Orvar Swenson and Alexander Bill.