Congenital Malformations Registry - Summary Report, 2008

Section III: Prevalence of Selected Malformations by Sex and Race/Ethnicity

Introduction to Table

The malformations presented in this section were selected because of the frequency with which they were reported and/or their clinical significance. Rates are per 10,000 live births. The sex ratio is calculated by dividing the rate in males by the rate in females. The malformation rates presented in this report may not be comparable to earlier reports. Previous reports from 1989 to 1991 did not use birth certificate matched cases; thus, the race and birth weight from the birth certificate were not available. Birth weight data are useful to calculate the rate of some malformations such as patent ductus arteriosus. In some cases, these conditions can result from being preterm rather than actually having a malformation. Racial data in this report also may not be comparable because race is defined by maternal race from the birth certificate. In the earlier reports, race was defined by what was reported on the CMR form, which may differ from what is recorded on the birth certificate.

Fluctuations in specific malformation prevalence should be interpreted with caution, especially differences in the "other" race category since the numbers in this group are small. In addition, several malformations were added in 1992 as a result of the change to the BPA coding system. Previously, these could not be distinguished using the ICD-9 codes. However, since ICD-9 codes are more familiar to most vendors, the ICD-9 code is given on the table with the named malformation. See Appendix 4 for further information on the BPA codes.

Children with Selected Malformations Prevalence per 10,000 Live Births by Sex and Race/Ethnicity
2008 Births – New York State Residents
ICD-9 Malformation Total Number Total Prevalence Male Female Ratio (M/F) Non-Hispanic White Non-Hispanic Black Hispanic Other/ Unknown Race
243 Congenital hypothyroidism 133 5.4 5.3 5.5 1.0 4.2 9.2 5.1 5.7
270.1 Phenylketonuria 10 0.4 0.6 0.3 2.2 0.3 0.5 0.2 1.1
277.0 Cystic fibrosis 44 1.8 1.7 1.8 0.9 1.9 1.7 2.2 0.4
282.6 Sickle-cell anemia 161 6.5 6.3 6.8 0.9 0.4 31.1 3.9 2.8
740.0 Anencephalus 11 0.4 0.7 0.2 4.3 0.3 0.5 0.8 0.0
741.0 Spina bifida with hydrocephalus 24 1.0 0.8 1.2 0.7 1.2 1.5 0.5 0.4
741.9 Spina bifida without hydrocephalus 22 0.9 0.9 0.8 1.1 1.0 0.7 0.8 0.7
742.0 Encephalocele 28 1.1 1.2 1.1 1.1 0.8 1.0 1.0 2.8
742.1 Microcephalus 143 5.8 4.9 6.8 0.7 3.5 8.5 8.0 7.1
742.2 Agyria & lissencephaly 5 0.2 0.3 0.1 3.8 0.1 0.0 0.2 1.1
742.2 Anomalies of corpus callosum 58 2.4 2.4 2.3 1.1 2.3 2.2 2.9 1.8
742.2 Holoprosencephaly 13 0.5 0.6 0.5 1.1 0.7 0.5 0.5 0.0
742.3 Congenital hydrocephalus 183 7.4 8.6 6.2 1.4 6.9 9.4 7.3 7.1
742.4 Porencephaly 12 0.5 0.8 0.2 4.7 0.2 0.7 1.2 0.0
742.5 Congenital tethered cord 50 2.0 2.1 1.9 1.1 2.3 1.7 1.7 1.8
743.0 Anophthalmos 4 0.2 0.2 0.2 0.9 0.3 0.2 0.0 0.0
743.1 Microphthalmos 26 1.1 0.8 1.3 0.6 0.6 1.2 2.0 0.7
743.2 Glaucoma 25 1.0 1.1 0.9 1.2 0.4 2.0 1.4 1.4
743.3 Absence of lens 1 0.0 0.0 0.1 0.0 0.1 0.0 0.0 0.0
743.3 Congenital cataract 53 2.1 1.4 2.9 0.5 2.0 3.0 2.2 1.4
743.45 Aniridia 5 0.2 0.2 0.2 1.4 0.1 0.2 0.5 0.0
743.46 Coloboma of iris 5 0.2 0.2 0.2 1.4 0.1 0.0 0.5 0.4
744.0 Anotia/microtia 30 1.2 1.3 1.2 1.1 1.3 0.7 1.4 1.1
745.0 Common truncus 16 0.6 0.9 0.4 2.1 0.7 1.5 0.3 0.0
745.1 Transposition of great vessels 86 3.5 3.8 3.2 1.2 3.4 3.2 3.6 4.2
745.2 Tetralogy of Fallot 128 5.2 5.4 4.9 1.1 5.1 7.0 3.9 5.7
745.3 Common ventricle 20 0.8 0.9 0.8 1.2 0.5 2.0 0.7 0.7
745.4 Ventricular septal defect 1,060 43.0 38.9 47.3 0.8 45.8 44.0 40.7 34.3
745.5 Ostium secundum type atrial septal def. 1,040 42.2 43.0 41.3 1.0 32.7 74.3 37.8 45.3
745.6 Endocardial cushion defects 100 4.1 3.4 4.8 0.7 3.8 7.2 3.2 2.5
746.0 Atresia/stenosis of pulmonary valve 219 8.9 9.3 8.4 1.1 8.1 11.9 8.5 8.5
746.1 Tricuspid atresia/stenosis/hypoplasia 13 0.5 0.7 0.3 2.1 0.3 1.2 0.5 0.7
746.2 Ebstein's anomaly 14 0.6 0.6 0.6 0.9 0.6 0.2 0.5 1.1
746.3 Congenital stenosis of aortic valve 34 1.4 1.9 0.8 2.3 1.7 0.2 1.5 1.4
746.7 Hypoplastic left heart syndrome 62 2.5 3.3 1.7 2.0 3.1 2.2 2.0 1.4
746.85 Anomalies of coronary artery 24 1.0 0.9 1.0 0.9 0.8 2.0 1.0 0.4
747.0 Patent ductus arteriosis 986 40.0 40.3 39.6 1.0 39.0 60.4 30.5 34.7
747.10 Coartation of aorta 148 6.0 6.3 5.7 1.1 6.5 6.5 4.9 5.7
747.41 Total anomalous pulmonary venus connect. 27 1.1 1.3 0.8 1.6 0.8 1.0 1.9 1.1
762.8 Amniotic bands 11 0.4 0.6 0.3 1.7 0.4 0.7 0.5 0.0
771.1 Congenital cytomegalovirus infection 23 0.9 0.7 1.2 0.6 0.6 2.2 1.0 0.4
771.2 Other congenital infections 49 2.0 2.4 1.6 1.5 2.1 2.0 2.0 1.4
748.0 Choanal atresia 48 1.9 2.2 1.7 1.3 2.6 2.0 1.4 0.4
748.5 Agenesis/hypoplasia of lung 42 1.7 1.9 1.5 1.3 1.4 2.0 2.2 1.4
749.0 Cleft palate 128 5.2 4.2 6.3 0.7 6.2 4.0 4.4 4.2
749.1 Cleft lip 57 2.3 3.4 1.2 2.9 2.4 1.5 2.2 3.2
749.2 Cleft palate & lip 113 4.6 5.5 3.6 1.5 5.5 2.7 4.7 2.8
750.3 Tracheoesophageal fistula etc. 67 2.7 2.4 3.0 0.8 3.1 3.0 2.2 1.8
750.5 Congenital hypertrophic pyloric stenosis 626 25.4 40.3 9.7 4.2 29.7 15.4 28.8 14.2
751.1 Atresia and stenosis of small intestine 98 4.0 3.5 4.5 0.8 3.9 4.0 4.4 3.5
751.2 Atresia and stenosis of rectum or anus 107 4.3 4.7 4.0 1.2 4.4 3.2 5.4 3.2
751.3 Hirschsprungs disease 71 2.9 3.8 1.9 2.0 3.0 3.0 2.2 3.5
751.4 Anomalies of intestinal fixation 65 2.6 2.9 2.3 1.3 3.0 2.5 1.9 2.8
751.61 Biliary atresia 20 0.8 0.8 0.8 0.9 0.7 1.5 0.7 0.7
752.6 Epispadias 37 1.5 2.8 0.1 34.1 1.2 3.5 1.0 1.1
752.6 Hypospadias 1,021 41.4 80.4 0.3 321.4 55.3 33.6 27.8 22.3
753.0 Renal agenesis and dysgenesis 113 4.6 5.1 4.0 1.3 5.5 4.5 4.1 2.1
753.1 Cystic kidney disease 171 6.9 7.7 6.2 1.2 7.0 8.2 7.5 3.9
753.2 Obstructive defect renal pelvis & ureter 1,035 42.0 57.7 25.3 2.3 44.1 30.3 41.2 51.0
753.5 Extrophy of urinary bladder 4 0.2 0.2 0.2 0.9 0.3 0.0 0.0 0.0
753.6 Atresia & stenosis of urethra & bladder 41 1.7 3.2 0.1 37.9 1.1 3.5 1.4 2.1
754.3 Congenital dislocation of hip 203 8.2 3.6 13.1 0.3 9.6 4.0 8.1 8.5
754.51 Talipes equinovarus 139 5.6 5.8 5.5 1.0 5.9 5.5 6.8 2.5
755.2 Reduction deformities of upper limb 72 2.9 3.2 2.6 1.3 3.7 2.0 3.2 0.4
755.3 Reduction deformities of lower limb 29 1.2 1.2 1.2 1.0 1.2 1.7 1.0 0.7
755.8 Arthrogryposis multiplex congenita 19 0.8 0.8 0.8 1.1 0.4 2.2 0.5 0.7
756.0 Craniosynostosis 109 4.4 5.8 2.9 2.0 5.7 2.0 4.6 2.1
756.0 Goldenhar syndrome 13 0.5 0.6 0.4 1.5 0.5 0.0 1.2 0.0
756.4 Chonodrodystrophy 49 2.0 1.9 2.1 0.9 1.7 3.7 2.2 0.4
756.51 Osteogenesis imperfecta 22 0.9 0.9 0.9 0.9 0.7 1.2 0.3 2.5
756.6 Diaphragmatic hernia 65 2.6 2.6 2.7 1.0 3.1 2.5 2.2 1.8
756.7 Gastroschisis 82 3.3 3.6 3.1 1.2 3.4 2.2 4.2 2.8
756.7 Omphalocele 23 0.9 0.9 0.9 1.0 1.1 0.5 1.2 0.4
756.7 Prune belly 5 0.2 0.4 0.0 . 0.2 0.2 0.0 0.7
758.0 Down syndrome 296 12.0 11.5 12.5 0.9 12.0 15.2 12.7 6.0
758.1 Patau syndrome 18 0.7 0.9 0.5 1.9 1.0 0.2 0.8 0.0
758.2 Edwards syndrome 44 1.8 1.1 2.5 0.4 1.2 2.7 2.7 1.1
758.6 Gonadal dysgenesis 17 0.7 0.1 1.3 0.1 0.6 0.5 1.0 0.7
758.7 Klinefelter syndrome 22 0.9 1.7 0.0 . 0.7 0.7 1.5 0.7
759.3 Situs inversus 26 1.1 0.9 1.2 0.8 0.8 2.0 0.7 1.8
760.71 Fetal alcohol syndrome 11 0.4 0.6 0.3 1.7 0.2 0.7 0.8 0.4
762.8 Amniotic bands 11 0.4 0.6 0.3 1.7 0.4 0.7 0.5 0.0
771.1 Congenital cytomegalovirus infection 23 0.9 0.7 1.2 0.6 0.6 2.2 1.0 0.4
771.2 Other congenital infections 49 2.0 2.4 1.6 1.5 2.1 2.0 2.0 1.4