Total colonic aganglionosis with or without small bowel involvement: A changing profile

Background/Purpose: To identify recent trends in the diagnosis and treatment of total colonic aganglionosis with or without small bowel involvement (TCSA), the authors analyzed the findings in 107 patients who had TCSA seen between 1988 and 1992 at 147 medical institutions throughout Japan and compared the results with those of a previous survey conducted between 1978 and 1982. Results: The estimated incidence of total colonic aganglionosis was 1 in 58,084 live births, the male to female ratio was 1.5:1, and the incidence of associated anomalies was 15%. These findings were all very similar to those of the previous survey. Ten years ago, 83.6% of all patients underwent Martin's procedure. In the recent survey, this rate had fallen to 52.1%, and a right colon Batch method has also been developed as a new procedure. A marked decrease in the overall mortality rate from 40.9% to 21.5% was observed. However, a high mortality rate persists in those cases with small bowel involvement (33.3%); most such cases are complicated with enterocolitis. Conclusions: Although the general features of TCSA were similar to those in the previous survey, a substantial improvement in the treatment results for TCSA has occurred. However, further efforts are still required both to prevent and more effectively to treat enterocolitis, especially in cases involving any aganglionosis extending into small bowel.