LB16.1 Hirschsprung disease
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
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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