ICD-11 classes
13 Diseases of the digestive system
Digestive system disorders of fetus or newborn
LB16.1 — Hirschsprung disease
ICD-11 LB16.1 — 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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: