Two cases are presented in which there was an absence of Auerbach's ganglia and nerves in the intestine from the duodenojejunal junction to the anus. This condition represents an unusual variant of Hirschsprung's disease, differing in the widespread nature and distribution of nervous-tissue defect.

It is suggested that the pathogenesis of this defect may lie in a failure of nerve fibers to invade the primitive gut wall, with failure of synapse formation and of development of intrinsic intestinal ganglia.

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