Hirschsprung disease

Description

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.

Index terms

Hirschsprung disease
Aganglionic megacolon
Hirschsprung megacolon

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aganglionosis
aganglionosis of colon
bowel aganglionosis
colon aganglionosis
colonic aganglionosis
congenital aganglionic megacolon
congenital 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

Exclusions from above levels

LB17.2 – Persistent cloaca
LB17.Y – Congenital atresia of rectum
http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

LB16.0 – Congenital absence, atresia or stenosis of large intestine
LB16.2 – Immature ganglionosis of large intestine
LB16.3 – Congenital hypoganglionosis of large intestine
LB16.Y – Other specified structural developmental anomalies of large intestine
LB16.Z – Structural developmental anomalies of large intestine, unspecified

Foundation entity

Hirschsprung disease – http://id.who.int/icd/entity/1772690306

Part of: LB16: Structural developmental anomalies of large intestine › 20: Developmental anomalies