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- Tamara Feldman, MD
- Barry K. Wershil, MD
- The Children’s Hospital at Montefiore
Bronx, NY
Characterized by the congenital absence of ganglion cells in the myenteric and submucosal plexuses of the intestine, Hirschsprung disease (HSD) results in abnormalities of intestinal motility that manifest most commonly as colonic obstruction. The aganglionic segment begins at the internal anal sphincter and extends a variable distance proximally. A simple, practical classification of HSD divides it into two types: short segment (S-HSD) and long segment (L-HSD). In the more common S-HSD (75% to 80% of cases), the aganglionic segment is located distal to the splenic flexure and often is limited to the rectosigmoid area. In L-HSD (20% of cases), the aganglionic segment includes and extends proximal to the splenic flexure, sometimes affecting the entire colon (total colonic aganglionosis), and in rare instances, the entire large and small intestine (total intestinal aganglionosis). A controversial variant of HSD …
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