J Korean Assoc Pediatr Surg.  1998 Jun;4(1):74-78. 10.13029/jkaps.1998.4.1.74.

Total Colonic Aganglionosis With Skip Area

• Lee SK
• Lee WY
• Kim HH

• KMID: 2422231
• DOI: http://doi.org/10.13029/jkaps.1998.4.1.74

Abstract

Early recognition and surgical treatment of Hirschsprung's disease prevents serious mortality and morbidity from enterocolitis and obstruction. Usually this disease is characterized by a single aganglionic segment of the colon extending distally to the anal margin. In surgical treatment, the surgeon performs a frozen section biopsy to confirm whether there are ganglion cells. If there are intervening ganglionic sites in aganglionic bowel, there may be confusion in diagnosis and treatment. The authors experienced one case of total colonic aganglionosis with skip area. A transverse loop colostomy was performed on a 7 day-old male baby with colon perforation due to Hirschsprung's disease. But intestinal obstruction persisted and required two more operations to find the true nature of the disease. There were aganglionic segments from the anal margin to the terminal ileum 3.7cm proximal to the ileocecal valve. The entire transverse colon and appendix were normally ganglionated.

Keyword

Total colonic aganglionosis; Skip area

MeSH Terms

Appendix
Biopsy
Colon
Colon, Transverse
Colostomy
Diagnosis
Enterocolitis
Frozen Sections
Ganglion Cysts
Hirschsprung Disease*
Humans
Ileocecal Valve
Ileum
Intestinal Obstruction
Male
Mortality