Hirschsprung disease (HSCR) constitutes a congenital absence of ganglion cells within the myenteric and submucosal plexus of a segment of the distal bowel. There is a well-recognised preponderance in males as well as a known association with certain syndromes, most commonly Trisomy 21, and causative genes, most commonly RET. While the majority (70%–80%) of patients will have disease limited to the rectum and sigmoid colon, a proportion of patients may have extended segment disease, with a loss of the male preponderance (approximately 3.5:1) in extended segment disease, total colonic aganglionosis (TCA) and small intestinal aganglionosis. HSCR usually presents in the neonatal period with signs of lower gastrointestinal obstruction (i.e., abdominal distension and failure to pass meconium), b

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