Total colonic aganglionosis: surgical treatment and long-term follow-up

Abstract

Between 1966 and 1989, 165 cases of Hirschsprung's disease, of which 20 (12.1%) were total colonic aganglionosis, were surgically treated at our centre. In the first period up to 1978, in which total parenteral nutrition (TPN) was not used or was used with reservations, 6 of 11 patients died. From 1978 to 1989, 9 further cases were treated with the support of TPN and only 1 death occurred, in a child with anganglionosis up to one-half of the jejunum. The 13 surviving patients were treated with the Duhamel or Rehbein techniques; the Lester Martin procedure was not used. There were no notable complications. Follow-up of 10 of these patients, 2 of whom are now 20 years old, showed their general condition, height, weight, and food tolerance to be within normal limits. The number of daily bowel movements, on the average three, was higher than normal. Studies of serum iron transferrin, transferrin saturation, and vitamin B₁₂ and folic acid absorption showed slightly low values that did not affect the erythrocyte count, hemoglobin, or hematocrit, but which require close monitoring. Hydrogen and fecal tests were normal.