ABSTRACT

Hirschsprung’s disease is a heterogeneous genetic disorder, resulting from an anomaly of the enteric nervous system of neural crest cell origin, characterized by the absence of parasympathetic intrinsic ganglionic cells in the submucosal and myenteric plexuses. It is regarded as the consequence of the premature arrest of the craniocaudal migration of vagal neural crest cells in the hindgut, between the 5th and 12th weeks of gestation, to form the enteric nervous system, and is therefore considered a neurocristopathy.1