Segmental aganglionosis of the appendix.

Ignoring the pathologist's dictum that the appendix is not reliable for the identification of ganglion cells in suspected cases of total colonic aganglionosis, the author (K.D.A.) performed an ileostomy in a child whose appendix contained no ganglion cells. The ascending colon was later found to be ganglionic. The appendices from 36 cases of Hirschsprung's disease were examined and the number of ganglion cells in 10 high power fields was compared with 10 appendices from patients who did not have Hirschsprung's disease. There were no ganglion cells in any of the patients with total aganglionosis of the colon. Twenty-two patients with Hirschsprung's disease involving the sigmoid colon had an average of 3.0 ganglion cells per high power field (range 0.5 to 5.1). Five patients with longer segment aganglionosis had 2.2 cells per high power field (range 0 to 4.1). The index case fell into this group and was serially sectioned without finding any ganglion cells. The control group averaged 3.41 cells per high power field (range 1.4 to 5.9). There was no significant difference between the control group and the groups with less than total colonic aganglionosis. It is speculated that this case represents segmental Hirschsprung's disease with the skip area in the ascending colon.