Clinical sxs. Hirschsprung’s disease (congenital megacolon) is caused by failed migration of colonic ganglion cells in utero. Varying lengths of the distal colon are unable to relax, causing functional colonic obstruction.Can vary from very short segment to entire large bowel. Obstruction noted usually in infancy, Sometimes recognition is later due to intractable constipation.
Hirschsprung's ... Disease is most often diagnosed in young infants, occasionally in older kids or adults. The normal nerve cells (ganglion cells) are missing from the distal colorectum, resulting in severe constipation or functional bowel obstruction. The DX can be suggested by a barium enema showing a "transition zone." the definitive DX is made with a rectal biopsy showing absence of ganglion cells.