Total Colonic Aganglionosis: a Rare Entity in Children

ABSTACT Total colonic aganglionosis being an unusual form of Hirschsprung’s disease is described in two children. Both the patients were male, one 8-year old and the other 12-year old. They presented with features of sub acute intestinal obstruction preceded by a past history of chronic constipation since birth and operative intervention for similar complaints few years back. A high index of suspicion prompted histopathological examination of colonic biopsies and the diagnosis of total colonic aganglionosis was confirmed. Two stage Martin’s Duhamel retrorectal pull through resulted in satisfactory recovery. Total colonic aganglionosis represents a significant challenge for pediatric surgeons. Long-term results are suboptimal, complications are very common. The goal is to provide the patient with a good quality of life. Authors review their experience with this rare entity and the pertinent literature.