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)