Aganglionosis Involving the Entire Colon and a Variable Length of Small Bowel

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Abstract
Hirschsprung''s disease of the entire colon and a variable length of small intestine was reported in at least 79 patients. The clinical pattern is usually one of incomplete intestinal obstruction of the full-term newborn infant. The radiographic pattern includes air-fluid levels, delayed small-bowel motility with oral contrast agent eventually reaching the colon, dilatation of small bowel as outlined by air or medium with a colon and terminal ileum of lesser diameter, fecaliths in the small bowel, adherent meconium or inspissated feces in proximal colon and distal ileum, rarely calcified, an adult configuration of the colon either lacking redundant flexures or medially displaced, with gross foreshortening on subsequent colon studies, irregular contractions in the descending colon, and "false" transition zones in transverse or sigmoid colon. The proximal extent of aganglionosis must be determined by muscle biopsy.