ABNORMALLY SHAPED ARTERIES IN THE INTESTINE OF CHILDREN WITH HIRSCHSPRUNGS-DISEASE - ETIOLOGIC CONSIDERATIONS RELATING TO ISCHEMIC THEORY

Abnormally shaped arteries were found in the intestines of 17 of 62 pa tients (27%) with Hirschsprung's disease. The histological characteris tics of abnormal arteries included proliferation of collagen fibers an d smooth muscle cells in the adventitia. The incidence of abnormal art eries in each type of aganglionosis was as follows: 25% (13 of 51) in short-segment aganglionosis; 0% (O of 6) in long-segment aganglionosis ; and 80% (4 of 5) in total-colon or extensive aganglionosis, The olde r the patients were at the time of resection, the higher the incidence of abnormal arteries (<1 year old, 15%; 1-3 years old, 38%; >3 years old, 75%). The abnormally shaped arteries were mostly located in the h istological transitional zone. These findings suggest the following po ssibilities: (a) the craniocaudal migration of ganglion cells was inte rrupted by intestinal ischemia in the presence of abnormal arteries in utero; (b) an ischemic episode caused both the disappearance of neura l cells and dysplasia of the artery; (c) the ganglion cells were destr oyed by mild ischemia caused by the abnormal arteries; or (d) the tens ion caused by mechanical expansion resulted in a change in the vascula r walls in the transitional zone.