TABLE A1.
Birth defects and organ systems as defined by the National Birth Defects Prevention Network (NBDPN) by disease classification codes
| Birth defects by organ systema | ICD-9-CM Codesa | ICD-10-CM Codesa | CDC/BPA Codesa |
|---|---|---|---|
| Congenital anomalies (740–759)/congenital malformations, deformations and chromosomal abnormalities (Q00–Q99) | 740–759 | Q00–Q99 | 740–759 |
| Central nervous system (740–742, Q00–07) | 740–742 | Q00–07 | 740–742 |
| Anencephaly | 740.0–740.1 | Q00.0–Q00.1 | 740.00–740.10 |
| Encephalocele | 742.0 | Q01.0–Q01.9 | 742.00–742.09 |
| Holoprosencephaly | 742.2 | Q04.2 | 742.26 |
| Spina bifida without anencephaly | 741.0, 741.9 w/o 740.0–740.1 | Q05.0–Q05.9, Q07.01, Q07.03 w/o Q00.0–Q00.1 | 741.00–741.99 w/o 740.00–740.10 |
| Eye (743, Q10–15) | 743 | Q10–Q15 | 743 |
| Anophthalmia/microphthalmia | 743.0, 743.1 | Q11.0–Q11.2 | 743.00–743.10 |
| Congenital cataract | 743.30–743.34 | Q12.0 | 743.32 |
| Ear, face, neck (744, Q16–18) | 744 | Q16–Q18 | 744 |
| Anotia/microtia | 744.01, 744.23 | Q16.0, Q17.2 | 744.01, 744.21 |
| Cardiovascular (745–747, Q20–28) | 745–747 | Q20–Q28 | 745–747 |
| Aortic valve stenosis | 746.3 | Q23.0 | 746.3 |
| Atrial septal defect | 745.5 | Q21.1 | 745.51–745.59 |
| Atrioventricular septal defect | 745.60, .61, .69 | Q21.2 | 745.60–745.69, 745.487 |
| Coarctation of aorta | 747.10 | Q25.1 | 747.10–747.19 |
| Common truncus (truncus arteriosus or TA) | 745.0 | Q20.0 | 745.00 only (excluding 745.01) |
| Double outlet right ventricle (DORV) | 745.11 | Q20.1 | 745.13–745.15 |
| Ebstein anomaly | 746.2 | Q22.5 | 746.20 |
| Hypoplastic left heart syndrome | 746.7 | Q23.4 | 746.7 |
| Interrupted aortic arch (IAA) | 747.11 | Q25.2, Q25.4 | 747.215–747.217, 747.285 |
| Pulmonary valve atresia and stenosis | 746.01, 746.02 | Q22.0, Q22.1 | 746.00, 746.01 |
| Single ventricle | 745.3 | Q20.4 | 745.3 |
| Tetralogy of Fallot (TOF) | 745.2 | Q21.3 | 745.20–745.21, 747.31 |
| Total anomalous pulmonary venous connection (TAPVC) | 747.41 | Q26.2 | 747.42 |
| Transposition of the great arteries (TGA) | 745.10, .12, .19 | Q20.3, Q20.5 | 745.10–745.12, 745.18–745.19 |
| Tricuspid valve atresia and stenosis | 746.1 | Q22.4 | 746.100, 746.106 (excluding 746.105) |
| Ventricular septal defect | 745.4 | Q21.0 | 745.40–745.49 (excluding 745.487, 745.498) |
| Respiratory (748, Q30–34) | 748 | Q30–Q34 | 748 |
| Choanal atresia | 748.0 | Q30.0 | 748.0 |
| Orofacial clefts (749, Q35–37) | 749 | Q35–Q37 | 749 |
| Cleft lip alone (without cleft palate) | 749.1 | Q36.0–Q36.9 | 749.10–749.19 |
| Cleft lip with cleft palate | 749.20–749.25 | Q37.0–Q37.9 | 749.20–749.29 |
| Cleft palate alone (without cleft lip) | 749.0 | Q35.1–Q35.9 | 749.00–749.09 |
| Upper gastrointestinal (750, Q38–40) | 750 | Q38–Q40 | 750 |
| Esophageal atresia/tracheoesophageal fistula | 750.3 | Q39.0–Q39.4 | 750.30–750.35 |
| Lower gastrointestinal (751, Q41–45) | 751 | Q41–Q45 | 751 |
| Biliary atresia | 751.61 | Q44.2–Q44.3 | 751.65 |
| Rectal and large intestinal atresia/stenosis | 751.2 | Q42.0–Q42.9 | 751.20–751.24 |
| Small intestinal atresia/stenosis | 751.1 | Q41.0–Q41.9 | 751.10–751.19 |
| Genital (752, Q50–56) | 752 | Q50–Q56 | 752 |
| Hypospadias | 752.61 | Q54.0–Q54.9 (excluding Q54.4) | 752.60–752.62 (excluding 752.61 and 752.621) |
| Renal (753, Q60–64) | 753 | Q60–Q64 | 753 |
| Bladder exstrophy | 753.5 | Q64.10, Q64.19 | 753.5 |
| Cloacal exstrophy | 751.5 | Q64.12 | 751.555 |
| Congenital posterior urethral valves | 753.6 | Q64.2 | 753.60 |
| Renal agenesis/hypoplasia | 753.0 | Q60.0–Q60.6 | 753.00–753.01 |
| Musculoskeletal (754, Q65–68) | 754 | Q65–Q68 | 754 |
| Clubfoot | 754.51, 754.70 | Q66.0, Q66.89 | 754.50, 754.73 (excluding 754.735) |
| Limbs (755, Q69–74) | 755 | Q69–Q74 | 755 |
| Limb deficiencies (reduction defects) | 755.2–755.4 | Q71.0–Q71.9, Q72.0–Q72.9, Q73.0–Q73.8 | 755.20–755.49 |
| Other musculoskeletal (756, Q75–79) | 756 | Q75–Q79 | 756 |
| Craniosynostosis | No specific code | Q75.0 | 756.00–756.03 |
| Diaphragmatic hernia | 756.6 | Q79.0, Q79.1 | 756.610–756.617 |
| Gastroschisis | 756.73 | Q79.3 | 756.71 |
| Omphalocele | 756.72 | Q79.2 | 756.70 |
| Skin (757, Q80–84) | 757 | Q80–Q84 | 757 |
| Chromosomal (758, Q90–99) | 758 | Q90 –Q99 | 758 |
| Deletion 22 q11.2 | 758.32 | Q93.81 | 758.37 |
| Trisomy 13 | 758.1 | Q91.4–Q91.7 | 758.10–758.19 |
| Trisomy 18 | 758.2 | Q91.0–Q91.3 | 758.20–758.29 |
| Trisomy 21 (down syndrome)b | 758.0 | Q90.0–Q90.9 | 758.00–758.09 |
| Turner syndrome | 758.6 | Q96.0–Q96.9 | 758.60–758.69 |
| Other (759, Q85–89) | 759 | Q85–Q89 | 759 |
a
Birth defect surveillance programs may have modified the requested code ranges used to define a select defect as necessary. Programs provided the code ranges where they differed from those requested by the National Birth Defects Prevention Network (NBDPN). If a program defined a defect using a different code range then the created estimates use the program-specific code range, where no alternate code range was specified the NBDPN code range was used. ICD-9-CM: International Classification of Diseases, ninth Revision, Clinical Modification; ICD-10-CM: International Classification of Diseases, 10th Revision, Clinical Modification; CDC/BPA: Centers for Disease Control and Prevention / British Pediatric Association Classification of Diseases.