Total Colonic Aganglionosis With Skip Area

ABSTRACT

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is chractrized by a single aganglionic segment of colon extending distally to anal margin. In surgical treatment, the surgeon performs frozen biopsy to confirm whether there are ganglion cells or not. If there are intervening ganglionic area in aganglionic bowel, there are much confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was made to a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and it took 2 more operations to find out the ture nature of the disease. There were aganglionic segments from anal margin to terminal ileum 3.7cm proximal from ileocecal valve. But the entire transverse colon and appendix were normally ganglionated.