2019 Congenital Anomaly Codes (Appendix C)

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)