Review Article

Birth Defects Surveillance in the United States: Challenges and Implications of International Classification of Diseases, Tenth Revision, Clinical Modification Implementation

Table 3

Birth defects list with ICD-9-CM, ICD-10-CM, and CDC/BPA codes, National Birth Defects Prevention Network [41].

Birth defectsICD-9-CM codesCDC/BPA codesICD-10-CM codes

Central nervous system
 Anencephalus740.0-740.1740.00–740.10Q00.0-Q00.1
 Spina bifida without anencephalus741.0–741.9 without 740.0–740.10741.00–741.99
without 740.0–740.10
Q05.0–Q05.9, Q07.01, Q07.03 without Q00.0-Q00.1
 Hydrocephalus without spina bifida742.3 without 741.0, 741.9742.30–742.39
without 741.00–741.99
Q03.0–Q03.9
 Encephalocele742.0742.00–742.09Q01.0–Q01.9
 Microcephalus742.1742.10Q02
Eye
 Anophthalmia/microphthalmia743.0, 743.1743.00–743.10Q11.0–Q11.2
 Congenital cataract743.30–743.34743.32 Q12.0
 Aniridia743.45743.42Q13.1
Ear
 Anotia/microtia744.01, 744.23744.01, 744.21Q16.0, Q16.1
Cardiovascular
 Common truncus745.0745.00Q20.0
 Transposition of great arteries745.10, 745.11,
745.12, 745.19
745.10–745.19
(exclude 745.13, 745.15, and 745.18)
Q20.1, Q20.3, Q20.5
 Tetralogy of Fallot745.2745.20-745.21, 747.31Q21.3
 Ventricular septal defect745.4745.40–745.49 (exclude 745.487, 745.498)Q21.0
 Atrial septal defect745.5745.51–745.59 Q21.1
 Atrioventricular septal defect (endocardial
 cushion defect)
745.60, 745.61, 745.69745.60–745.69, 745.487Q21.2
 Pulmonary valve atresia and stenosis746.01, 746.02746.00-746.01Q22.0, Q22.1
 Tricuspid valve atresia and stenosis746.1746.10 (exclude 746.105) Q22.4
 Ebstein’s anomaly746.2746.20Q22.5
 Aortic valve stenosis746.3746.30Q23.0
 Hypoplastic left heart syndrome746.7746.70Q23.4
 Patent ductus arteriosus747.0747.00Q25.0
 Coarctation of aorta747.10747.10–747.19Q25.1
 Total anomalous pulmonary venous return
 (TAPVR)
747.41747.42Q26.2
Orofacial
 Cleft palate without cleft lip749.0749.00–749.09Q35.0–Q35.9
 Cleft lip with and without cleft palate749.1, 749.2749.10–749.29Q36.0–Q36.9,
Q37.0–Q37.9
 Choanal atresia748.0748.0Q30.0
Gastrointestinal
 Esophageal atresia/tracheoesophageal fistula750.3750.30–750.35Q39.0–Q39.4
 Rectal and large intestinal atresia/stenosis751.2751.20–751.24Q42.0–Q42.9
 Pyloric stenosis750.5750.51Q40.0
 Hirschsprung’s disease (congenital megacolon)751.3751.30–751.34Q43.1
 Biliary atresia751.61751.65Q44.2-Q44.3
Genitourinary
 Renal agenesis/hypoplasia753.0753.00-753.01Q60.0–Q60.6
 Bladder exstrophy753.5753.50Q64.10, Q64.19
 Obstructive genitourinary defect753.2, 753.6753.20–753.29 and
753.60–753.69
Q62.0–Q62.3 and Q64.2-Q64.3
 Hypospadias752.61752.60–752.62 (exclude 752.61 and 752.621)Q54.0–Q54.9 (exclude Q54.4)
 Epispadias752.62752.61Q64.0
Musculoskeletal
 Reduction deformity, upper limbs755.20–755.29755.20–755.29Q71.0–Q71.9
 Reduction deformity, lower limbs755.30–755.39755.30–755.39Q72.0–Q72.9
 Gastroschisis*756.79756.71Q79.3
 Omphalocele**756.79756.70Q79.2
 Congenital hip dislocation754.30, 754.31, 754.35754.30Q65.0–Q65.2
 Diaphragmatic hernia756.6756.61Q79.0-Q79.1
Chromosomal
 Patau syndrome (trisomy 13)758.1758.10–758.19Q91.4–Q91.7
 Down syndrome (trisomy 21)758.0758.00–758.09Q90.0–Q90.9
 Edwards syndrome (trisomy 18)758.2758.20–758.29Q91.0–Q91.3
Other
 Fetus or newborn affected by maternal alcohol
 use
760.71760.71Q86.0
 Amniotic bandsNo code658.80No code

Note.
ICD-9-CM: International Classification of Diseases, Ninth Edition, Clinical Modification.
ICD-10-CM: International Classification of Diseases, Tenth Edition, Clinical Modification.
CDC/BPA codes: Centers of Disease Control and Prevention/British Paediatric Association.
*756.79 started being coded as 756.73 as of 10/1/2009.
**756.79 started being coded as 756.72 as of 10/1/2009.