Total Colon Aganglionosis
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Hirschsprung disease (HD) is a common cause of lower intestinal obstruction in the newborn. Patients with HD can be classified based on extent of aganglionosis - classical segment disease refers to those with aganglionosis that does not extend beyond the upper sigmoid colon, long segment disease refers to those with aganglionosis extending to the splenic flexure or transverse colon and total colonic aganglionosis (TCA) refers to those in whom the aganglionosis extends to the entire colon and may also extend up to 30 cm proximally into the distal small bowel . Total intestinal aganglionosis, the rarest form, refers to infants with absence of ganglion cells from the duodenum distally. This topic covers the distinct considerations in the management of TCA.
see also Hirschsprung disease for a complete discussion of the entire spectrum of disease
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Last updated: September 7, 2018
Gosain, Ankush, and Andrew Zigman. "Total Colon Aganglionosis." Pediatric Surgery NaT, American Pediatric Surgical Association, 2018. APSA Webapp, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829568/all/Total_Colon_Aganglionosis.
Gosain A, Zigman A. Total Colon Aganglionosis. In: Waldhausen J, Powell D, Hirschl R, eds. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829568/all/Total_Colon_Aganglionosis. Accessed May 22, 2019.
Gosain, A., & Zigman, A. (2018). Total Colon Aganglionosis. In Waldhausen, J., Powell, D., & Hirschl, R. (Eds.), Pediatric Surgery NaT. Available from https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829568/all/Total_Colon_Aganglionosis
Gosain A, Zigman A. Total Colon Aganglionosis [Internet]. In: Waldhausen J, Powell D, Hirschl R, editors. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. [cited 2019 May 22]. Available from: https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829568/all/Total_Colon_Aganglionosis.
TY - ELEC
T1 - Total Colon Aganglionosis
ID - 829568
A1 - Gosain,Ankush,MD, PhD
AU - Zigman,Andrew,MD, CM
ED - Waldhausen,John,
ED - Powell,David,
ED - Hirschl,Ron,
Y1 - 2018/09/07/
BT - Pediatric Surgery NaT
UR - https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829568/all/Total_Colon_Aganglionosis
PB - American Pediatric Surgical Association
DB - APSA Webapp
DP - Unbound Medicine