LB16.1 Hirschsprung disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

This is a developmental anomaly affecting the intestinal tract characterised by congenital absence of myenteric ganglion cells (aganglionosis) in a segment of the large bowel. Due to the absence of intrinsic innervation of the muscle layers of the affected segment, there is a loss of motor function. This results in an abnormally large or dilated colon (congenital megacolon) with intestinal occlusion or constipation. This condition becomes evident shortly after birth.

synonyms

• Hirschsprung disease
• Aganglionic megacolon
• aganglionosis
• congenital aganglionic megacolon
• congenital megacolon
• aganglionosis of colon
• bowel aganglionosis
• colon aganglionosis
• colonic aganglionosis
• Hirschsprung megacolon
• pelvirectal achalasia
• Entire colon Hirschsprung disease
• Total aganglionosis of colon and intestine
• Extensive aganglionosis Hirschsprung disease
• Long segment Hirschsprung disease
• Rectosigmoid Hirschsprung disease
• Short segment Hirschsprung disease

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