Introduction

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 [1]. 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