Abstract

Congenital aganglionosis or Hirschrung's disease is a congenital absence of intramural parasympathic ganglion cells in the distal gastrointestinal tract, associated with nonfunction of the affected segment and subsequent proximal colonic obstruction. The diagnosis of Hirschprung's disease requiıes histological demonstration of aganglionosis in rectal tissue. Definitive treatment involves resecting and bypassing the aganglionic segment and bringing normally innervated colon distally to the internal sphincter. This review presents the state of our recent knowledge of classical agangliosis and related disorders which include hypoganglionosis, intestinal neuronal dysplasia, and so on. We want to emphasize the morphological differentiation of these neuronal malformations.