Alaska Birth Defect Reported Prevalence (per 10,000 live births), 2004-2012

Current nationally reportable major congenital anomalies (N=47)

Defect 2004-2008* 2005-2009* 2006-2010* 2007-2011 2008-2012 p-value
Cardiovascular
  Aortic valve stenosis 1.3 - 1.1 1.4 2.0 0.3027
  Atrail spetal defect 139.2 148.6 157.4 158.0 160.2 0.0256
  Atrioventricular septal defect 5.4 6.2 6.1 7.3 6.4 0.1779
  Coarctation of the aorta 5.4 4.6 5.4 5.3 4.4 0.5386
  Common truncus 2.0 2.2 1.8 2.5 2.8 0.1439
  Double outlet right ventricle - - - 2.1 1.8 -
  Ebstein anomaly - - 1.4 1.2 1.6 0.6893
  Hypoplastic left heart syndrome 2.6 2.6 2.7 2.0 1.2 0.0922
  Inerrupted aortic arch - - - 4.3 4.4 -
  Pulmonary valve atresia and stenosis 13.9 14.6 15.1 14.4 14.0 0.9832
  Single ventricle - - - 1.4 0.9 -
  Teratology of fallot 5.9 5.5 5.6 6.0 5.3 0.5854
  Total anomalous pulmonary venous connection - 2.0 2.2 1.8 1.8 0.2496
  Transposition of the great arteries 4.6 4.4 4.7 4.6 3.7 0.2631
  Tricuspid valve atresia and stenosis 1.9 1.6 1.1 1.1 - 0.0530
  Ventricular septal defect 98.7 102.0 99.1 101.9 101.2 0.3909
Central Nervous System
  Anencephalus - - 2.0 2.5 2.1 0.8086
  Spina bifida without anencephalus 7.6 6.4 5.6 3.7 2.7 0.0045
  Encephalocele 4.8 4.4 4.1 3.2 2.8 0.0051
  Holoprosencephaly - - - 9.2 10.1 -
Chromosomal
  Deletion 22 q11 - - - 0.9 - -
  Trisomy 13 1.5 1.5 1.3 1.2 1.6 0.9765
  Trisomy 18 2.2 2.2 1.8 2.1 2.1 0.7222
  Trisomy 21 (Down syndrome) 15.8 15.9 15.6 16.2 17.8 0.1228
  Turner syndrome* - - - - 2.6 -
Ear
  Anotia / microtia 2.2 3.3 3.9 3.7 4.1 0.0730
Eye
  Anophthalmia / microphthalmia 4.1 2.7 2.0 1.4 1.6 0.0135
  Congenital cataract 5.2 5.5 5.7 5.2 5.5 0.7660
Gastrointestinal
  Biliary atresia 3.0 2.9 3.2 2.3 2.5 0.2249
  Esophageal atresia / tracheoesophageal fistula 2.6 2.9 2.7 2.7 2.7 0.7821
  Rectal and large intestinal atresia / stenosis 8.9 9.5 10.4 10.5 9.9 0.1631
  Small intestinal atresia / stenosis - - - 7.5 6.6 -
Genitourinary
  Bladder exstrophy - 1.1 - - - -
  Cloacal exstrophy - - - - - -
  Congenital posterior urethral valves - - - 7.5 8.7 -
  Hypospadias** 99.9 104.6 105.6 112.6 113.6 0.0063
  Renal agenesis / hypoplasia 7.2 - 7.5 7.8 9.1 0.1220
Musculoskeletal
  Clubfoot - - - 35.8 36.9 -
  Craniosynostosis - - - - - -
  Diaphragmatic hernia 6.3 6.6 5.7 5.7 5.5 0.0534
  Gastroschisis - - - 3.9 5.2 -
  Limb deficiencies - - - 11.9 11.7 -
  Omphalocele - - - 13.9 12.4 -
Orofacial
  Choanal atresia 2.6 2.2 2.7 3.0 3.2 0.0916
  Cleft lip with celft palate - - - 7.8 8.7 -
  Cleft lip alone - - - 4.1 3.9 -
  Cleft palate alone 17.7 15.3 17.7 14.1 13.1 0.1220
Ref: 95% CI = 95% Confidence Interval; g = grams

*per 10,000 live female births

**per 10,000 live male births

Table Notes:

  1. Missing data is the result of suppression and/or non-data collection in early years.

  2. Five-year estimates are based on historical estimates reported to the National Birth Defects Prevention Network. Although p-value estimates are provided to assess the trend (increasing or decreasing) over time, caution should be used in interpretation as data collection methods and data aggregation may vary leading to systematic differences. Further, from the 2007-2011 estimate and on reports are only included if reported before the age of three years. For conditions that tend to have a later diagnosis and reporting estimates in the more recent years may be systematically lower.

  3. The p-value estimates the probability that the observed slope is different from a slope of zero. By convention an alpha of 0.05 is used to determine significance; p-values are only calculated for defects with 3 or more data points. The p-value estimate is calculated to model the observed rates over time using a generalized linear model with a Poisson distribution and offset of the natural log (ln) of the birth population size under the form:

\[ ln \frac a n = \beta_0 + \beta_1 X_1 + \ln(n) \]

Resources
National Birth Defects Prevention Network
Centers for Diesase Control and Prevention

Contact
Alaska Birth Defects Registry (ABDR)
3601 C Street, Suite 358
Anchorage, AK 99503
(907) 269-3400 phone
(907) 754-3529 fax
hssbirthdefreg@alaska.gov

Updated: March 16, 2017
Code source: R:\ABDR\Analysis_New\Published Data\trend04_12.Rmd