SURGICAL MANAGEMENT OF TOTAL COLONIC AND PARTIAL SMALL INTESTINAL AGANGLIONOSIS

During a 10-year period, 6 cases of small intestinal aganglionosis have been encountered out of 70 patients with Hirschsprung's disease. Five children underwent Duhamel procedures and 3 have survived with good results. The other 2 succumbed to aspiration pneumonia, unexpectedly, years after surgery. The sixth died in the newborn period from aganglionic involvement extending into the jejunum. Plain film of the abdomen and especially the barium enema are invaluable in the diagnosis. Frozen sections of the bowel wall are essential, in confusing cases of neonatal intestinal obstruction. The colon should be removed at the time of definitive surgery to prevent subsequent infection, abscess formation, and abdominal distention. © 1969.