Chapter XVII

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

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


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


Q39	Congenital malformations of oesophagus
Q39.0	Atresia of oesophagus without fistula
	Atresia of oesophagus NOS
Q39.1	Atresia of oesophagus with tracheo-oesophageal fistula
	Atresia of oesophagus with broncho-oesophageal fistula
Q39.2	Congenital tracheo-oesophageal fistula without atresia
	Congenital tracheo-oesophageal fistula NOS
Q39.3	Congenital stenosis and stricture of oesophagus
Q39.4	Oesophageal web
Q39.5	Congenital dilatation of oesophagus
Q39.6	Diverticulum of oesophagus
	Oesophageal pouch
Q39.8	Other congenital malformations of oesophagus
	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
	Congenital or infantile:
	· constriction · hypertrophy · spasm · stenosis · stricture		} } } } }	of pylorus
Q40.1	Congenital hiatus hernia
	Displacement of cardia through oesophageal hiatus
	Excludes:	congenital diaphragmatic hernia ( Q79.0 )
Q40.2	Other specified congenital malformations of stomach
	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
	Congenital:
	· anomaly · deformity		} }	NOS of upper alimentary tract


Q41	Congenital absence, atresia and stenosis of small intestine
	Includes:	congenital obstruction, occlusion and stricture of small intestine or intestine NOS
	Excludes:	meconium ileus ( E84.1 )
Q41.0	Congenital absence, atresia and stenosis of duodenum
Q41.1	Congenital absence, atresia and stenosis of jejunum
	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
	Congenital absence, atresia and stenosis of intestine NOS


Q42	Congenital absence, atresia and stenosis of large intestine
	Includes:	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
	Imperforate rectum
Q42.2	Congenital absence, atresia and stenosis of anus with fistula
Q42.3	Congenital absence, atresia and stenosis of anus without fistula
	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's diverticulum
	Persistent: · omphalomesenteric duct · vitelline duct
Q43.1	Hirschsprung's disease
	Aganglionosis Congenital (aganglionic) megacolon
Q43.2	Other congenital functional disorders of colon
	Congenital dilatation of colon
Q43.3	Congenital malformations of intestinal fixation
	Congenital adhesions [bands]: · omental, anomalous · peritoneal Jackson's 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
	Excludes:	congenital fistula: · rectovaginal ( Q52.2 ) · urethrorectal ( Q64.7 ) pilonidal fistula or sinus ( L05.- ) with absence, atresia and stenosis ( Q42.0 , Q42.2 )
Q43.7	Persistent cloaca
	Cloaca NOS
Q43.8	Other specified congenital malformations of intestine
	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
		Congenital absence of gallbladder
Q44.1		Other congenital malformations of gallbladder
		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
		Accessory hepatic duct Congenital malformation of bile duct NOS Duplication: · biliary duct · cystic duct
Q44.6		Cystic disease of liver
		Fibrocystic disease of liver
Q44.7		Other congenital malformations of liver
		Accessory liver Alagille's syndrome Congenital: · absence of liver · hepatomegaly · malformation of liver NOS


Q45	Other congenital malformations of digestive system
	Excludes:	congenital: · diaphragmatic hernia ( Q79.0 ) · hiatus hernia ( Q40.1 )
Q45.0	Agenesis, aplasia and hypoplasia of pancreas
	Congenital absence of pancreas
Q45.1	Annular pancreas
Q45.2	Congenital pancreatic cyst
Q45.3	Other congenital malformations of pancreas and pancreatic duct
	Accessory pancreas Congenital malformation of pancreas or pancreatic duct NOS
	Excludes:	diabetes mellitus: · congenital ( E10.- ) · neonatal ( P70.2 ) fibrocystic disease of pancreas ( E84.- )
Q45.8	Other specified congenital malformations of digestive system
	Absence (complete)(partial) of alimentary tract NOS
	Duplication Malposition, congenital		} }	of digestive organs NOS
Q45.9	Congenital malformation of digestive system, unspecified
	Congenital:
	· anomaly · deformity		} }	NOS of digestive system