Total colonic aganglionosis: diagnosis and management in a 12-year-old boy.

Total colonic aganglionosis (TCA) is an unusual form of Hirschsprung's disease, and most of these present within the first few weeks and nearly all by the end of the first year of life. Very few cases presenting in older childhood or adulthood have been reported. Without a high index of suspicion these cases can easily be missed and, therefore, patients undergo repeated laparotomies for subacute intestinal obstruction without relief of symptoms. The diagnosis is suspected on operative findings and confirmed by histological evaluation of seromuscular biopsy specimens. Proper diagnosis will prevent loss of bowel length caused by inadvertent resection and will help in performing an optimal procedure to treat these patients. Discussed herein is another case of TCA in a 12-year-old boy with review of relevant literature.