Hirschsprung disease (Q46328): Difference between revisions
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Revision as of 14:50, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | LB16.1 |
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| English | Hirschsprung disease |
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. |
