Etiology

Hirschsprung's disease is characterized by the congenital absence of ganglionic cells in the submu-cosal and myenteric plexuses in the distal bowel, and variable proportion of the colon proximally. The embryonic disorder is a lack of the craniocaudal migration, differentiation and maturation of neurob-lasts from the neural crests. The earlier the migration ceases, the longer the aganglionic segment will be. The aganglionic segment is permanently contracted, causing dilatation of its proximal tract.3

Hirschsprung's disease may be classified according to the length of the aganglionic segment: the classic form (short segment in 70-75% of cases) is limited to the rectum and sigmoid colon; the long segment, or subtotal colonic disease (10-15%), generally involves the bowel up to the splenic flexure; total colonic aganglionosis (TCA; 3-6%) may extend to involve a variable amount of the short bowel; and total intestinal aganglionosis is sometimes associated with intestinal malrotation or volvulus.4 Ultrashort-segment aganglionosis is considered a functional alteration, without any detectable histological finding. Although longer aganglionic segments tend to produce more dramatic symptoms, some patients with even short-segment disease deteriorate rapidly.5

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