Chapter XVII
Congenital malformations, deformations and chromosomal abnormalities
(Q00-Q99)

Rules Conventions GCS Guidance

Other congenital malformations of the digestive system
(Q38-Q45)

Q38 Other congenital malformations of tongue, mouth and pharynx

Excl.:
macrostomia ( Q18.4)
microstomia ( Q18.5)
Q38.0 Congenital malformations of lips, not elsewhere classified
Incl.:
Congenital:
  • fistula of lip
  • malformation of lip NOS
Van der Woude syndrome
Excl.:
cleft lip ( Q36.-)
cleft lip
  • with cleft palate (Q37.-)
macrocheilia ( Q18.6)
microcheilia ( Q18.7)
Q38.1 Ankyloglossia
Incl.:
Tongue tie
Q38.2 Macroglossia
Q38.3 Other congenital malformations of tongue
Incl.:
Aglossia
Bifid tongue
  • Congenital:
    • adhesion
    • fissure
    • malformation NOS
  • of tongue
Hypoglossia
Hypoplasia of tongue
Microglossia
Q38.4 Congenital malformations of salivary glands and ducts
Incl.:
  • Absence
  • Accessory
  • Atresia
  • (of) salivary gland or duct
Congenital fistula of salivary gland
Q38.5 Congenital malformations of palate, not elsewhere classified
Incl.:
Absence of uvula
Congenital malformation of palate NOS
High arched palate
Excl.:
cleft palate ( Q35.-)
cleft palate
Q38.6 Other congenital malformations of mouth
Incl.:
Congenital malformation of mouth NOS
Q38.7 Pharyngeal pouch
Incl.:
Diverticulum of pharynx
Excl.:
pharyngeal pouch syndrome ( D82.1)
Q38.8 Other congenital malformations of pharynx
Incl.:
Congenital malformation of pharynx NOS

Q39 Congenital malformations of oesophagus

Q39.0 Atresia of oesophagus without fistula
Incl.:
Atresia of oesophagus NOS
Q39.1 Atresia of oesophagus with tracheo-oesophageal fistula
Incl.:
Atresia of oesophagus with broncho-oesophageal fistula
Q39.2 Congenital tracheo-oesophageal fistula without atresia
Incl.:
Congenital tracheo-oesophageal fistula NOS
Q39.3 Congenital stenosis and stricture of oesophagus
Q39.4 Oesophageal web
DCS.XI.1
Q39.5 Congenital dilatation of oesophagus
Q39.6 Diverticulum of oesophagus
Incl.:
Oesophageal pouch
Q39.8 Other congenital malformations of oesophagus
Incl.:
  • Absent
  • Congenital displacement
  • Duplication
  • (of) oesophagus
Q39.9 Congenital malformation of oesophagus, unspecified

Q40 Other congenital malformations of upper alimentary tract

Q40.0 Congenital hypertrophic pyloric stenosis
Incl.:
  • Congenital or infantile:
    • constriction
    • hypertrophy
    • spasm
    • stenosis
    • stricture
  • of pylorus
Q40.1 Congenital hiatus hernia
Incl.:
Displacement of cardia through oesophageal hiatus
Excl.:
congenital diaphragmatic hernia ( Q79.0)
Q40.2 Other specified congenital malformations of stomach
Incl.:
Congenital:
  • displacement of stomach
  • diverticulum of stomach
  • hourglass stomach
Duplication of stomach
Megalogastria
Microgastria
Q40.3 Congenital malformation of stomach, unspecified
Q40.8 Other specified congenital malformations of upper alimentary tract
Q40.9 Congenital malformation of upper alimentary tract, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
  • NOS of upper alimentary tract

Q41 Congenital absence, atresia and stenosis of small intestine

Incl.:
congenital obstruction, occlusion and stricture of small intestine or intestine NOS
Excl.:
meconium ileus ( E84.1)
Q41.0 Congenital absence, atresia and stenosis of duodenum
Q41.1 Congenital absence, atresia and stenosis of jejunum
Incl.:
Apple peel syndrome
Imperforate jejunum
Q41.2 Congenital absence, atresia and stenosis of ileum
Q41.8 Congenital absence, atresia and stenosis of other specified parts of small intestine
Q41.9 Congenital absence, atresia and stenosis of small intestine, part unspecified
Incl.:
Congenital absence, atresia and stenosis of intestine NOS

Q42 Congenital absence, atresia and stenosis of large intestine

Incl.:
congenital obstruction, occlusion and stricture of large intestine
Q42.0 Congenital absence, atresia and stenosis of rectum with fistula
Q42.1 Congenital absence, atresia and stenosis of rectum without fistula
Incl.:
Imperforate rectum
Q42.2 Congenital absence, atresia and stenosis of anus with fistula
Q42.3 Congenital absence, atresia and stenosis of anus without fistula
Incl.:
Imperforate anus
Q42.8 Congenital absence, atresia and stenosis of other parts of large intestine
Q42.9 Congenital absence, atresia and stenosis of large intestine, part unspecified

Q43 Other congenital malformations of intestine

Q43.0 Meckel diverticulum
Incl.:
Persistent:
  • omphalomesenteric duct
  • vitelline duct
Q43.1 Hirschsprung disease
Incl.:
Aganglionosis
Congenital (aganglionic) megacolon
Q43.2 Other congenital functional disorders of colon
Incl.:
Congenital dilatation of colon
Q43.3 Congenital malformations of intestinal fixation
Incl.:
Congenital adhesions [bands]:
  • omental, anomalous
  • peritoneal
Jackson membrane
Malrotation of colon
  • Rotation:
    • failure of
    • incomplete
    • insufficient
  • of caecum and colon
Universal mesentery
Q43.4 Duplication of intestine
Q43.5 Ectopic anus
Q43.6 Congenital fistula of rectum and anus
Excl.:
congenital fistula:
pilonidal fistula or sinus ( L05.-)
with absence, atresia and stenosis ( Q42.0, Q42.2)
Q43.7 Persistent cloaca
Incl.:
Cloaca NOS
Q43.8 Other specified congenital malformations of intestine
Incl.:
Congenital:
  • blind loop syndrome
  • diverticulitis, colon
  • diverticulum, intestine
Dolichocolon
Megaloappendix
Megaloduodenum
Microcolon
Transposition of:
  • appendix
  • colon
  • intestine
Q43.9 Congenital malformation of intestine, unspecified

Q44 Congenital malformations of gallbladder, bile ducts and liver

Q44.0 Agenesis, aplasia and hypoplasia of gallbladder
Incl.:
Congenital absence of gallbladder
Q44.1 Other congenital malformations of gallbladder
Incl.:
Congenital malformation of gallbladder NOS
Intrahepatic gallbladder
Q44.2 Atresia of bile ducts
Q44.3 Congenital stenosis and stricture of bile ducts
Q44.4 Choledochal cyst
Q44.5 Other congenital malformations of bile ducts
Incl.:
Accessory hepatic duct
Congenital malformation of bile duct NOS
Duplication:
  • biliary duct
  • cystic duct
Q44.6 Cystic disease of liver
Incl.:
Fibrocystic disease of liver
Q44.7 Other congenital malformations of liver
Incl.:
Accessory liver
Alagille syndrome
Congenital:
  • absence of liver
  • hepatomegaly
  • malformation of liver NOS

Q45 Other congenital malformations of digestive system

Excl.:
congenital:
  • diaphragmatic hernia (Q79.0)
  • hiatus hernia (Q40.1)
Q45.0 Agenesis, aplasia and hypoplasia of pancreas
Incl.:
Congenital absence of pancreas
Q45.1 Annular pancreas
Q45.2 Congenital pancreatic cyst
Q45.3 Other congenital malformations of pancreas and pancreatic duct
Incl.:
Accessory pancreas
Congenital malformation of pancreas or pancreatic duct NOS
Excl.:
diabetes mellitus:
fibrocystic disease of pancreas ( E84.-)
Q45.8 Other specified congenital malformations of digestive system
Incl.:
Absence (complete)(partial) of alimentary tract NOS
  • Duplication
  • Malposition, congenital
  • of digestive organs NOS
Q45.9 Congenital malformation of digestive system, unspecified
Incl.:
  • Congenital:
    • anomaly
    • deformity
  • NOS of digestive system