Defect 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 p-value
Cardiovascular
  Aortic valve stenosis - - 3.5 3.5 - 3.6 1.7 - 4.4 2.7 0.8143
  Atrail spetal defect 158.9 158.7 168.4 158.2 156.1 174.0 193.5 162.7 170.4 110.3 0.5412
  Atrioventricular septal defect 7.2 8.7 6.2 6.1 9.6 3.6 2.6 7.9 2.6 4.4 0.1156
  Coarctation of the aorta 6.3 3.5 2.6 7.0 9.6 1.8 6.1 2.6 7.1 2.7 0.7333
  Common truncus 2.7 3.5 1.8 - 3.5 5.4 2.6 - - - 0.4789
  Double outlet right ventricle 2.7 2.6 2.6 - 2.6 - 1.7 2.6 2.6 1.8 0.1873
  Ebstein anomaly - - - 4.3 - 1.8 - - - 1.8 0.4019
  Hypoplastic left heart syndrome 3.6 1.7 2.6 2.6 - - 2.6 2.6 1.8 - 0.3619
  Inerrupted aortic arch 7.2 2.6 5.3 3.5 7.0 8.9 7.0 6.1 9.7 4.4 0.3345
  Pulmonary valve atresia and stenosis 16.2 13.9 15.9 19.1 11.3 17.0 14.8 8.7 9.7 8.9 0.0325
  Single ventricle 2.7 1.7 - 1.7 1.7 1.8 3.5 2.6 1.8 - 0.7634
  Teratology of fallot 6.3 5.2 6.2 4.3 10.5 3.6 7.0 6.1 2.6 1.8 0.2415
  Total anomalous pulmonary venous connection - 5.2 2.6 - - - - 1.7 1.8 1.8 0.0453
  Transposition of the great arteries 6.3 4.4 2.6 5.2 7.0 4.5 1.7 4.4 2.6 1.8 0.0919
  Tricuspid valve atresia and stenosis 2.7 2.6 - - - - - - - - -
  Ventricular septal defect 106.8 105.5 105.8 86.9 110.8 108.9 119.4 78.7 98.9 79.1 0.2120
Central Nervous System
  Anencephalus 2.7 1.7 4.4 1.7 1.7 - - - 1.8 1.8 0.3582
  Spina bifida without anencephalus 9.0 - 6.2 5.2 3.5 2.7 1.7 6.1 8.8 2.7 0.3983
  Encephalocele 4.5 5.2 1.8 - 4.4 2.7 1.7 - - 1.8 0.0896
  Holoprosencephaly 10.8 9.6 7.1 11.3 10.5 15.2 13.9 19.2 15.0 9.8 0.1064
Chromosomal
  Deletion 22 q11 1.8 - - 3.5 1.7 - 1.7 1.7 3.5 - 0.6924
  Trisomy 13 - 3.5 1.8 - - 1.8 - - - - 0.4868
  Trisomy 18 3.6 1.7 1.8 - 2.6 3.6 1.7 1.7 2.6 - 0.7070
  Trisomy 21 (Down syndrome) 15.3 21.8 14.1 15.6 19.2 23.2 16.6 7.9 11.5 16.9 0.3523
  Turner syndrome* 5.6 3.6 - 3.6 3.6 9.1 - - 9.1 5.5 0.2498
Ear
  Anotia / microtia 3.6 1.7 7.1 3.5 4.4 4.5 3.5 5.2 3.5 2.7 0.8775
Eye
  Anophthalmia / microphthalmia 1.8 - 4.4 - - 2.7 - - - 2.7 0.9960
  Congenital cataract 4.5 6.1 7.9 7.8 7.8 5.4 3.5 5.2 5.3 2.7 0.1339
Gastrointestinal
  Biliary atresia - 5.2 3.5 2.6 - 1.8 - 1.7 - 5.3 0.8858
  Esophageal atresia / tracheoesophageal fistula - 4.4 4.4 1.7 1.7 - 3.5 5.2 1.8 2.7 0.6298
  Rectal and large intestinal atresia / stenosis 12.6 10.5 11.5 7.8 11.3 9.8 4.4 7.0 7.9 4.4 0.0091
  Small intestinal atresia / stenosis 6.3 9.6 4.4 7.0 10.5 1.8 6.1 5.2 5.3 6.2 0.4244
Genitourinary
  Bladder exstrophy - - 1.8 - - - - - - - -
  Congenital posterior urethral valves 15.3 17.4 15.0 15.6 14.0 13.4 7.0 7.9 - 2.7 0.0039
  Hypospadias** 114.1 99.2 115.5 122.6 127.7 111.7 137.9 89.8 117.1 112.5 0.9636
  Renal agenesis / hypoplasia 8.1 12.2 11.5 7.0 7.0 13.4 4.4 7.0 15.9 4.4 0.6959
Musculoskeletal
  Clubfoot 35.9 43.6 37.9 38.2 39.2 41.1 32.2 33.2 33.6 27.6 0.0199
  Craniosynostosis - - - 1.7 1.7 - 2.6 6.1 14.1 25.8 0.0022
  Diaphragmatic hernia 6.3 8.7 7.1 3.5 5.2 6.2 5.2 3.5 2.6 - 0.0261
  Gastroschisis 9.9 7.0 4.4 7.0 10.5 6.2 9.6 7.0 7.9 6.2 0.8135
  Limb deficiencies 13.5 16.6 14.1 10.4 8.7 10.7 6.1 14.9 6.2 15.1 0.3834
  Omphalocele 20.7 12.2 11.5 11.3 14.8 13.4 11.3 14.9 24.7 86.2 0.0394
Orofacial
  Choanal atresia 2.7 2.6 - 7.0 2.6 3.6 3.5 1.7 3.5 1.8 0.5212
  Cleft lip with celft palate 4.5 7.0 7.1 6.1 6.1 9.8 17.4 14.9 14.1 8.0 0.0336
  Cleft lip alone 9.0 12.2 8.8 7.0 11.3 13.4 13.1 13.1 11.5 7.1 0.6783
  Cleft palate alone 26.0 26.2 25.6 33.0 27.0 21.4 20.9 22.7 21.2 25.8 0.1738
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. Prevalence rate estimates are based on reports to the Alaska Birth Defect Registry. 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 over time. Reports of defects are only included if diagnosed before the age of three years. For conditions that are commonly diagnosed after the first three years of life, reporting estimates 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

Updated: August 3, 2020
Code source: R:\ABDR\Analysis_New\Published Data\trend07_16.Rmd