MORE THAN 10 YEARS FOLLOW-UP OF TOTAL COLONIC AGANGLIONOSIS - SEVERE IRON-DEFICIENCY ANEMIA AND GROWTH-RETARDATION

Seven cases of total colonic aganglionosis were reviewed with,a follow -up period of 10 to 26 years, focusing on the relationship between the length of aganglionic ileum and postoperative metabolic disorders. Pu lled-through ileum ranged from 0 to 65 cm from the ileocecal valve, an d suprapelvic side-to-side anastomosis was performed between the pulle d-through ileum and 17 to 40 cm of aganglionic colon (left side and tr ansverse colon, four; right side colon, one; no colonic patch, two). H emoglobin level in three out of four patients with ileal involvement o f more than 25 cm was below 11 g/dL (10.9, 7.7, 6.6 g/dL, respectively ). Serum iron lever was less than 30 mu g/dL (27, 21, 20, 18 mu g/dL, respectively) in four out of five patients with ileal involvement of m ore than 10 cm. Serum vitamin B12 level was below 100 (100, 46 pg/dL, respectively) in two patients whose purled-through ileum was 45 cm and 65 cm, respectively from the ileocecal valve. Ore patient needs perio dical parenteral iron therapy and one was treated as megaloblastic ane mia. In the patients with ileal involvement of more than 25 cm, both w eight and height for age are very low at less than the fifth percentil e, except for one patient whose side patch was at the right colon. One patient still needs parenteral nutritional support. Severe iron defic iency anemia, low level of B12, and growth retardation are apparent in the patients with total colonic aganglionosis with ileal involvement. Colonic side-to-side anastomosis does not substitute for the toss of terminal ileum. Copyright (C) 1997 by W.B. Saunders Company