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
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#		 Excludes: lingual tonsil (K14.88 )
macrostomia ( Q18.4)
median rhomboid glossitis (K14.2 )
microstomia ( Q18.5)
persistent thyroglossal or thyrolingual duct (Q89.20 )
plicated tongue (K14.5 )
 
thyroglossal:
cyst (Q89.21 )
fistula (Q89.21 )
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
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Excludes: lingual thyroid (Q89.23 )
 
                       persistent thyroglossal or thyrolingual duct (Q89.20 )
 
thyroglossal:
· cyst (Q89.21 )
· fistula (Q89.21 )
#Q38.30 Aglossia
#Q38.31 Bifid tongue
#Q38.32 Adhesion of tongue, congenital
#Q38.33 Fissure of tongue, congenital
#Q38.34 Microglossia
#Q38.38 Other specified congenital malformations of tongue
#Q38.39 Congenital malformation of tongue, unspecified
Q38.4 Congenital malformations of salivary glands and ducts
#Q38.40 Absence of salivary gland or duct
#Q38.41 Accessory salivary gland or duct
#Q38.42 Atresia of salivary gland or duct
#Q38.43 Congenital fistula of salivary gland
#Q38.48 Other specified congenital malformations of salivary glands and ducts
#Q38.49 Congenital malformation of salivary glands and ducts,unspecified
Q38.5 Congenital malformations of palate, not elsewhere classified
Excludes: cleft palate ( Q35.-)
· with cleft lip ( Q37.-)
#Q38.50 Absence of uvula
#Q38.51 High arched palate
#Q38.58 Other specified congenital malformations of palate
#Q38.59 Congenital malformation of palate, unspecified
Congenital malformation of palate NOS
Q38.6 Other congenital malformations of mouth
Congenital malformation of mouth NOS
#Q38.60 Fordyce condition [Fordyce disease]
#Q38.61 White sponge naevus [leukokeratosis oris]
#Q38.68 Other specified congenital malformations of mouth
#Q38.69 Congenital malformation of mouth, unspecified
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
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(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
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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
#Q40.4 Congenital microvillous atrophy
Microvillous inclusion disease
Q40.8 Other specified congenital malformations of upper alimentary tract
Q40.9 Congenital malformation of upper alimentary tract, unspecified
Congenital:
· anomaly
· deformity
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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
#Q41.00 Duodenal web
#Q41.08 Other congenital absence, atresia and stenosis of duodenum
#Q41.09 Congenital absence, atresia and stenosis of duodenum,unspecified
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
Ladd's band
Malrotation of colon
Rotation:
· failure of
· incomplete
· insufficient
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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
# Caroli disease
Fibrocystic disease of liver
Q44.7 Other congenital malformations of liver
#Q44.70 Alagille's syndrome
#Q44.71 Progressive familial intrahepatic cholestasis
#Q44.78 Other congenital malformations of liver
Accessory liver
Congenital:
· absence of liver
· hepatomegaly
#Q44.79 Congenital malformation of liver, unspecified
Congenital 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
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of digestive organs NOS
Q45.9 Congenital malformation of digestive system, unspecified
Congenital:
· anomaly
· deformity
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NOS of digestive system