Defect 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 p-value
Cardiovascular
  Aortic valve stenosis - - 3.5 3.5 - 3.6 1.7 - 4.4 2.7 2.9 0.61
  Atrail spetal defect 158.9 158.7 168.4 158.2 156.1 174.0 193.5 162.7 170.4 110.3 121.0 0.17
  Atrioventricular septal defect 7.2 8.7 6.2 6.1 9.6 3.6 2.6 7.9 2.6 4.4 2.9 0.04
  Coarctation of the aorta 6.3 3.5 2.6 7.0 9.6 1.8 6.1 2.6 7.1 2.7 4.8 0.69
  Common truncus 2.7 3.5 1.8 - 3.5 5.4 2.6 - - - - 0.47
  Double outlet right ventricle 2.7 2.6 2.6 - 2.6 - 1.7 2.6 2.6 1.8 - 0.18
  Ebstein anomaly - - - 4.3 - 1.8 - - - 1.8 - 0.45
  Hypoplastic left heart syndrome 3.6 1.7 2.6 2.6 - - 2.6 2.6 1.8 - - 0.38
  Inerrupted aortic arch 7.2 2.6 5.3 3.5 7.0 8.9 7.0 6.1 9.7 4.4 - 0.33
  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 16.2 0.10
  Single ventricle 2.7 1.7 - 1.7 1.7 1.8 3.5 2.6 1.8 - - 0.76
  Teratology of fallot 6.3 5.2 6.2 4.3 10.5 3.6 7.0 6.1 2.6 1.8 1.9 0.09
  Total anomalous pulmonary venous connection - 5.2 2.6 - - - - 1.7 1.8 1.8 2.9 0.11
  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 1.9 0.03
  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 62.9 0.04
Central Nervous System
  Anencephalus 2.7 1.7 4.4 1.7 1.7 - - - 1.8 1.8 1.9 0.30
  Spina bifida without anencephalus 9.0 - 6.2 5.2 3.5 2.7 1.7 6.1 8.8 2.7 3.8 0.29
  Encephalocele 4.5 5.2 1.8 - 4.4 2.7 1.7 - - 1.8 - 0.10
  Holoprosencephaly 10.8 9.6 7.1 11.3 10.5 15.2 13.9 19.2 15.0 9.8 9.5 0.38
Chromosomal
  Deletion 22 q11 1.8 - - 3.5 1.7 - 1.7 1.7 3.5 - 1.9 0.95
  Trisomy 13 - 3.5 1.8 - - 1.8 - - - - - 0.52
  Trisomy 18 3.6 1.7 1.8 - 2.6 3.6 1.7 1.7 2.6 - - 0.71
  Trisomy 21 (Down syndrome) 15.3 21.8 14.1 15.6 19.2 23.2 16.6 7.9 11.5 16.9 19.1 0.48
  Turner syndrome* 5.6 3.6 - 3.6 3.6 9.1 - - 9.1 5.5 3.9 0.56
Ear
  Anotia / microtia 3.6 1.7 7.1 3.5 4.4 4.5 3.5 5.2 3.5 2.7 6.7 0.60
Eye
  Anophthalmia / microphthalmia 1.8 - 4.4 - - 2.7 - - - 2.7 4.8 0.54
  Congenital cataract 4.5 6.1 7.9 7.8 7.8 5.4 3.5 5.2 5.3 2.7 4.8 0.10
Gastrointestinal
  Biliary atresia - 5.2 3.5 2.6 - 1.8 - 1.7 - 5.3 4.8 0.84
  Esophageal atresia / tracheoesophageal fistula - 4.4 4.4 1.7 1.7 - 3.5 5.2 1.8 2.7 3.8 0.75
  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 3.8 0.00
  Small intestinal atresia / stenosis 6.3 9.6 4.4 7.0 10.5 1.8 6.1 5.2 5.3 6.2 3.8 0.24
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 1.9 0.00
  Hypospadias** 114.1 99.2 115.5 122.6 127.7 111.7 137.9 89.8 117.1 112.5 96.7 0.44
  Renal agenesis / hypoplasia 8.1 12.2 11.5 7.0 7.0 13.4 4.4 7.0 15.9 4.4 8.6 0.62
Musculoskeletal
  Clubfoot 35.9 43.6 37.9 38.2 39.2 41.1 32.2 33.2 33.6 27.6 31.4 0.01
  Craniosynostosis - - - 1.7 1.7 - 2.6 6.1 14.1 25.8 30.5 0.01
  Diaphragmatic hernia 6.3 8.7 7.1 3.5 5.2 6.2 5.2 3.5 2.6 - 4.8 0.03
  Gastroschisis 9.9 7.0 4.4 7.0 10.5 6.2 9.6 7.0 7.9 6.2 9.5 0.92
  Limb deficiencies 13.5 16.6 14.1 10.4 8.7 10.7 6.1 14.9 6.2 15.1 5.7 0.14
  Omphalocele 20.7 12.2 11.5 11.3 14.8 13.4 11.3 14.9 24.7 86.2 22.9 0.14
Orofacial
  Choanal atresia 2.7 2.6 - 7.0 2.6 3.6 3.5 1.7 3.5 1.8 2.9 0.46
  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 11.4 0.05
  Cleft lip alone 9.0 12.2 8.8 7.0 11.3 13.4 13.1 13.1 11.5 7.1 9.5 0.96
  Cleft palate alone 26.0 26.2 25.6 33.0 27.0 21.4 20.9 22.7 21.2 25.8 18.1 0.05
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, 2021
Code source: R:\ABDR\Analysis_New\Published Data\trend07_17.Rmd