2021 Congenital Anomaly Codes (Appendix C)

Description:

This is Appendix C from the Manual of Operations. It is a list of the Congenital Anomaly Codes reported to VON in the VLBW and Expanded databases.

Congenital Anomaly Codes

 

Central Nervous System Anomalies

Code        Description

101           Anencephaly

102           Meningomyelocele

103           Hydranencephaly

104           Congenital Hydrocephalus

105           Holoprosencephaly

106           Encephalocele

901           Other lethal or life threatening central nervous system anomaly not listed above                                   (description required)

 

Congenital Heart Anomalies

Code        Description

201          Truncus Arteriosus

202          Transposition of the Great Vessels

203          Tetralogy of Fallot with or without Pulmonary Atresia

204          Single Ventricle

205          Double Outlet Right Ventricle

206          Complete Atrio-Ventricular Canal

207          Pulmonary Atresia with Intact Ventricular Septum

208          Tricuspid Atresia

209          Hypoplastic Left Heart Syndrome

210          Interrupted Aortic Arch

211          Total Anomalous Pulmonary Venous Return

212          Pentalogy of Cantrell (Thoraco-Abdominal Ectopia Cordis)

213          Coarctation of the Aorta requiring surgical or medical intervention

214          Atrial Septal Defect requiring surgical or medical intervention

215          Ventricular Septal Defect requiring surgical or medical intervention

216          Arrythmia requiring surgical or medical intervention

217          Ebstein’s Anomaly requiring surgical or medical intervention

218          Pulmonary Valvular Stenosis requiring surgical or medical intervention

902          Other lethal or life threatening heart anomaly not listed above (description required)

 

Gastro-Intestinal Anomalies

Code        Description

301           Cleft Palate

302           Tracheo-Esophageal Fistula

303           Esophageal Atresia

304           Duodenal Atresia

305           Jejunal Atresia

306           Ileal Atresia

307           Atresia of Large Bowel or Rectum

308           Imperforate Anus

309           Omphalocele

310           Gastroschisis

311           Biliary Atresia

312           Malrotation

313           Hirschsprung’s Disease requiring surgical or medical intervention

314           Sacrococcygeal teratoma requiring surgical or medical intervention

903           Other lethal or life threatening gastro-intestinal anomaly not listed above (description                           required)

 

Genito-Urinary Anomalies

Code        Description

401           Bilateral Renal Agenesis 

402           Bilateral Polycystic, Multicystic, or Dysplastic Kidneys

403           Obstructive Uropathy with Congenital Hydronephrosis

404           Exstrophy of the Urinary Bladder

904           Other lethal or life threatening genito-urinary anomaly not listed above (description                             required)

 

Chromosomal Anomalies

Code        Description

501           Trisomy 13

502           Trisomy 18

503           Trisomy 21

504           Other chromosomal anomaly not listed above (description required)

505           Triploidy

 

Other Congenital Anomalies

Code        Description

601           Skeletal Dysplasia (description required)

602           Congenital Diaphragmatic Hernia

603           Hydrops Fetalis with anasarca and one or more of the following: ascites, pleural effusion,                       pericardial effusion

604           Oligohydramnios Sequence including all 3 of the following: (1) Oligohydramnios                                     documented by antenatal ultrasound 5 or more days prior to delivery, (2) evidence of fetal                   constraint on postnatal physical exam (such as Potter’s facies, contractures, or positional                       deformities of limbs), and (3) postnatal respiratory failure requiring endotracheal intubation                   and assisted ventilation.

605           Inborn Error of Metabolism (description required)

606           Myotonic Dystrophy requiring endotracheal intubation and assisted ventilation

607           Conjoined Twins

608           Tracheal Agenesis or Atresia

609           Thanatophoric Dysplasia Types 1 and 2

610           Hemoglobin Barts

 

Pulmonary Anomalies

Code        Description

701           Congenital Cystic Adenomatoid Malformation of the Lung

907           Other lethal or life threatening pulmonary anomaly not listed above (description required)

 

Other Lethal or Life Threatening Anomalies

Code        Description

100           Other lethal or life threatening anomalies not listed above (description required)