Long-Term Results of the Treatment of Total Colonic Aganglionosis with Two Different Techniques

Aim: Aim of this study was to assess the long-term results of the treatment of total colonic aganglionosis (TCA) with 2 different techniques in terms of growth, continence and quality of life (QOL). Patients and Methods: Forty-one patients treated for TCA between 1972 and 2007 were reviewed retrospectively with special attention paid to the length of aganglionosis, complications, growth, continence and QOL. Until 1992, patients underwent subtotal colonic resection and side-to-side ileosigmoid anastomosis (modified Martin). Since 7992, straight ileo-anal pull-through was preferred. At the end of follow-up (median 18 years, range 1-35), the height and weight, continence and QOL (scoring feeding habits, school/work performance, family life and professional development) were assessed by clinical visit or phone interview. Results: Twenty-eight patients were male and 13 female. Six had total intestinal aganglionosis and were excluded from this review together with the 2 who died before definitive treatment. The 33/41 persons in whom aganglionosis had involved less than 50 cm above the ileocecal valve and who had been considered suitable for the reestablishment of transanal fecal flow were included. Eighteen children underwent a modified Martin and 15 straight ileo-anal pull-through. Postoperative intestinal obstruction occurred in 4 cases, prolapse and prolonged TPN requirement in 2, and wound disruption and fistula in 1. Thirteen patients (39%) had postoperative enterocolitis. Two children died after operation (1 wound disruption with sepsis and 1 pneumonia). Out of 31 survivors, 57 % and 53 % were > p50 with regard to height and weight whereas only 15% and 19% were < p3 respectively. Only half the patients had more than 3 bowel movements per day and the median Wildhaber continence score(normal =14) was 11 (range 6-14). Both types of operations resulted in comparable defecation and continence patterns. QOL was rated as good in all cases but one. All patients but 2 attended high school, 8 attend university, 4 are employed and 1 is married and has 2 daughters. Social life is normal except for 1 patient who perceives his disease as a burden. Conclusions: Patients with TCA amenable to reestablishment of the transanal fecal flow can have adequate growth, normal feeding, reasonably good continence and satisfactory QOL. However, complications and enterocolitis are frequent. A modified Martin's procedure was performed as well as straight ileo-anal pull-through with little influence on the long-term outcome.