Nutrition Status And Quality Of Life In Patients With Total Colonic Aganglionosis

Objective: Over the past decades, the quality of life (QOL) of patients with total colonic aganglionosis (TCA) has been significantly improved. This study is to evaluate the long-term outcome of TCA patients in our hospital. Materials: We identified eleven patients with TCA treated from 1999 to 2010. Clinical follow-up data was collected by in-person or telephone interviews. Anorectal function and QOL were evaluated by the Kelly scoring system and Quality of Life Scoring Criteria, respectively. Laboratory results were reviewed and compared with non-TCA patients, including patients with rectosigmoidaganglionosis (RS) or non-Hirschsprung's disease who had colon resection. Results: The defecation frequency of 10 patients was 3 to 6 times per day. All children had Kelly's score of 5 or 6 for anorectal function. Nine patients had satisfactory QOL score and the other 2 patients had fair QOL score. The gamma-GT levels in TCA patients were significantly lower than that in non-TCA patients. LDL levels in TCA patients were lower than normal range but of no significant difference compared with non-TCA patients. Conclusions: The long-term outcome of TCA patients was satisfactory in terms of anorectal function and QOL. However, the reduced level of.-GT and LDL requires further investigations as they might be indicators of potential inflammation for patients with TCA.