PEDIATRICS Vol. 21 No. 2 February 1958, pp. 261-266
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CONGENITAL ABNORMALITIES OF INTESTINAL INNERVATION

Absence of Innervation of Jejunum, Ileum and Colon in Siblings

Joseph D. Boggs M.D.1 and James M. Kidd M.D.1

1 Department of Pathology, Children's Memorial Hospital, Chicago, and Northwestern University Medical School

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.

Submitted on November 5, 1956
Accepted on August 27, 1957