Alaska Birth Defects Registry
Cleft Conditions
Alaska, 2007-2015
Background
Cleft conditions, commonly referred to as orofacial clefts, includes all cleft lip and cleft palate birth defects [1]. Orofacial clefts occur when a baby’s lip and/or palate do not form properly during early pregnancy. Cleft lip occurs when the lip does not come together during development, separating the lip either on one side (unilateral) or on both sides (bilateral) [2]. Cleft palate occurs when the roof of the mouth fails to join together resulting in an opening in either the front of the palate, the back of the palate, or both [1]. The lip and palate develop separately during pregnancy, therefore, a baby can have either cleft lip, cleft palate, or both cleft lip and cleft palate [2]. Orofacial clefts are one of the most common birth defects in the United States [2, 3]. While the exact cause for most cases of orofacial clefts is unknown. It is believed that a combination of environmental factors, mothers behaviors during early pregnancy (what she eats/drinks and medicine consumed), and genetic factors may contribute to orofacial clefts [1, 2].
Methodology
The Alaska Birth Defects Registry (ABDR) registers birth defects as reported from health care providers using International Classification of Disease (ICD) billing codes. The use of these ICD codes can lead to misclassification of diagnosed conditions. Prior to this report, all prevalence estimates were based on the number of unique children reported to ABDR with an ICD code representing a specified condition regardless of case confirmation status.
The estimates in this report were derived by conducting medical record review and case confirmation of a random sample of cases of the condition reported to ABDR. The confirmation probability from the sample was used to develop informed estimates of the actual diagnosed defect prevalence. See defect prevalence calculation for a complete explanation of the methodology used for generating estimates.
While this report provides the most precise estimates, caution should be used in interpreting prevalence estimates based on conditions with <30 annual reports, as they are potentially vulnerable to large year-to-year variation.
For explanations of table columns see column descriptions.
All Oral Facial Clefts
According to the 2014 annual report released by the National Birth Defects Prevention Network (NBDPN), the prevalence of all orofacial clefts for 29 U.S. States from 2007-2011 was 14.5 per 10,000 live births.[3]
In Alaska, during 2007-2015, the prevalence of all orofacial clefts was 32.4 per 10,000 live births.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Total | 406 | 330.7 | 101978 | 32.4 (29.1, 36.0) |
| Notes: 95% CI = 95% Confidence Interval | ||||
Cleft Lip
Cleft Lip Prevalence
According to the 2014 annual report released by the National Birth Defects Prevention Network (NBDPN), the prevalence of cleft lip was for 29 U.S. States from 2007-2011 was 3.1 per 10,000 live births.[3]
In Alaska, during 2007-2015, the prevalence of cleft lip was 4.9 per 10,000 live births.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Total | 107 | 49.5 | 101978 | 4.9 (3.6, 6.4) |
| Notes: 95% CI = 95% Confidence Interval | ||||
Cleft Lip Trend
Prevalence per 10,000 births of Cleft Lip during 2007-2015 by three-year moving averages, with 95% confidence interval band and Poisson estimated fitted line. 
| Estimate | Std. Error | t value | Pr(>|t|) |
|---|---|---|---|
| 0.03888 | 0.01686 | 2.30637 | 0.06922 |
| Reports | Defects | Births | Prevalence (95% CI) | Predicted Prevalence† | |
|---|---|---|---|---|---|
| 2007-2009 | 10.7 | 5.7 | 11261.7 | 5.0 (2.1, 10.8) | 4.4 |
| 2008-2010 | 9.7 | 5.2 | 11404.0 | 4.5 (1.8, 9.8) | 4.6 |
| 2009-2011 | 9.3 | 4.9 | 11404.7 | 4.3 (1.6, 9.4) | 4.8 |
| 2010-2012 | 11.0 | 5.1 | 11354.0 | 4.5 (1.8, 9.8) | 5.0 |
| 2011-2013 | 13.3 | 6.0 | 11349.0 | 5.2 (2.3, 11.1) | 5.2 |
| 2012-2014 | 14.0 | 6.2 | 11334.7 | 5.5 (2.3, 11.1) | 5.4 |
| 2013-2015 | 14.0 | 6.7 | 11377.0 | 5.8 (2.5, 11.5) | 5.6 |
|
Notes: Each row is based on three-year moving averages; Prevalence reported per 10,000 live births; 95% CI=95% Confidence Interval † Estimated rate based on Poisson model |
|||||
Cleft Lip Regional Distribution
Distribution of cleft lip in Alaska by Public Health Region of maternal residence at the time of birth. A description of regional breakdowns can be found here. Data suppressed for # of reports < 6.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Anchorage | 31 | 13.3 | 41815 | 3.2 (1.8, 5.3) |
| Gulf Coast | 11 | 4.5 | 6313 | 7.1 (2.6, 16.2) |
| Interior | 20 | 8.3 | 18534 | 4.5 (2.2, 8.5) |
| Mat-Su | 11 | 4.6 | 12081 | 3.8 (1.3, 8.5) |
| Northern | 7 | 2.9 | 7114 | 4.1 (0.9, 10.2) |
| Southeast | 7 | 2.9 | 6375 | 4.6 (1, 11.3) |
| Southwest | 20 | 8.1 | 9746 | 8.3 (4.2, 16.2) |
| Notes: Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Cleft Lip Demographics
Some subgroups may be more at risk for having a baby with a cleft lip. This section provides the descriptive epidemiology of specified maternal, birth, and child characteristics identified from the birth certificate.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Sex | ||||
| Female | 39 | 16.6 | 49462 | 3.4 (2.0, 5.2) |
| Male | 68 | 28.1 | 52516 | 5.3 (3.7, 7.7) |
| Birth weight (grams) | ||||
| <2500 | 11 | 4.5 | 5925 | 7.5 (2.7, 17.3) |
| 2500+ | 96 | 40.2 | 95890 | 4.2 (3.1, 5.7) |
| Maternal age | ||||
| 12-19 | 10 | 4.1 | 8043 | 5.1 (2.0, 12.7) |
| 20-24 | 40 | 16.4 | 28015 | 5.9 (3.5, 9.3) |
| 25-29 | 23 | 9.9 | 30852 | 3.2 (1.6, 5.5) |
| 30-34 | 23 | 9.6 | 22480 | 4.3 (2.1, 7.6) |
| 35-39 | - | - | 10015 | 3.4 (1.1, 8.8) |
| 40+ | - | - | 2543 | - |
| Maternal race | ||||
| Alaska Native/American Indian | 48 | 19.5 | 25801 | 7.6 (4.7, 11.5) |
| Asian/Pacific Islander | - | - | 9399 | 2.8 (0.7, 7.7) |
| Black | - | - | 4134 | - |
| White | 49 | 20.9 | 61287 | 3.4 (2.1, 5.0) |
| Maternal education (years) | ||||
| <12 | 13 | 5.4 | 9778 | 5.5 (2.2, 11.9) |
| 12 | 53 | 21.8 | 36724 | 5.9 (3.8, 8.7) |
| 12+ | 40 | 17.1 | 52380 | 3.3 (2.0, 5.2) |
| Marital status | ||||
| Married | 54 | 22.9 | 64510 | 3.5 (2.3, 5.2) |
| Unmarried | 52 | 21.4 | 37078 | 5.8 (3.7, 8.7) |
| Maternal smoking use | ||||
| Reported smoking | 26 | 10.6 | 14467 | 7.3 (3.8, 12.7) |
| Reported not smoking | 77 | 32.5 | 85927 | 3.8 (2.6, 5.3) |
| Medicaid (mother or child) | ||||
| Medicaid | 83 | 33.9 | 51554 | 6.6 (4.6, 9.0) |
| non-Medicaid | 24 | 10.8 | 50311 | 2.1 (1.1, 3.7) |
| Father on birth certificate | ||||
| None | 6 | 2.5 | 5559 | 4.5 (1.1, 13.0) |
| Present | 101 | 42.2 | 96419 | 4.4 (3.2, 5.9) |
| Notes: Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Cleft Palate
Cleft Palate Prevalence
According to the 2014 annual report released by the National Birth Defects Prevention Network (NBDPN), the prevalence of cleft palate for 29 U.S. States from 2007-2011 was 5.9 per 10,000 live births.[3]
In Alaska, during 2007-2015, the prevalence of cleft palate was 13.6 per 10,000 live births.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Total | 202 | 138.5 | 101978 | 13.6 (11.5, 16.0) |
| Notes: 95% CI = 95% Confidence Interval | ||||
Cleft Palate Trend
Prevalence per 10,000 births of cleft palate during 2007-2015 by three-year moving averages, with 95% confidence interval band and Poisson estimated fitted line.
| Estimate | Std. Error | t value | Pr(>|t|) |
|---|---|---|---|
| 0.01840 | 0.00693 | 2.65612 | 0.04509 |
| Reports | Defects | Births | Prevalence (95% CI) | Predicted Prevalence† | |
|---|---|---|---|---|---|
| 2007-2009 | 19.0 | 14.3 | 11261.7 | 12.8 (7.2, 20.5) | 13.0 |
| 2008-2010 | 22.0 | 15.6 | 11404.0 | 13.7 (8.0, 21.7) | 13.2 |
| 2009-2011 | 22.7 | 15.6 | 11404.7 | 13.7 (8.0, 21.7) | 13.5 |
| 2010-2012 | 23.7 | 15.7 | 11354.0 | 13.8 (8.1, 21.7) | 13.7 |
| 2011-2013 | 22.0 | 15.0 | 11349.0 | 13.2 (7.6, 21.0) | 14.0 |
| 2012-2014 | 23.7 | 15.9 | 11334.7 | 14.0 (8.3, 22.2) | 14.2 |
| 2013-2015 | 24.7 | 17.1 | 11377.0 | 15.0 (9.2, 23.6) | 14.5 |
|
Notes: Each row is based on three-year moving averages; Prevalence reported per 10,000 live births; 95% CI=95% Confidence Interval † Estimated rate based on Poisson model |
|||||
Cleft Palate Regional Distribution
Distribution of cleft palate in Alaska by Public Health Region of maternal residence at the time of birth. A description of regional breakdowns can be found here. Data suppressed for # of reports < 6.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Anchorage | 72 | 49.5 | 41815 | 11.8 (8.9, 15.5) |
| Gulf Coast | 20 | 12.6 | 6313 | 20 (11, 33.2) |
| Interior | 34 | 23.1 | 18534 | 12.5 (8.3, 18.6) |
| Mat-Su | 12 | 9.4 | 12081 | 7.8 (4, 14.1) |
| Northern | 22 | 13.9 | 7114 | 19.5 (10.8, 31.2) |
| Southeast | 13 | 8.7 | 6375 | 13.6 (6.5, 24.7) |
| Southwest | 29 | 18.4 | 9746 | 18.9 (11.7, 29.2) |
| Notes:Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Cleft Palate Demographics
Some subgroups may be more at risk for having a baby with a cleft palate. This section provides the descriptive epidemiology of specified maternal, birth, and child characteristics identified from the birth certificate.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Sex | ||||
| Female | 94 | 63.5 | 49462 | 12.8 (10.0, 16.3) |
| Male | 108 | 72.1 | 52516 | 13.7 (10.9, 17.3) |
| Birth weight (grams) | ||||
| <2500 | 29 | 17.6 | 5925 | 29.6 (18.0, 45.9) |
| 2500+ | 173 | 118.0 | 95890 | 12.3 (10.3, 14.7) |
| Maternal age | ||||
| 12-19 | 23 | 14.7 | 8043 | 18.2 (10.4, 29.2) |
| 20-24 | 53 | 35.9 | 28015 | 12.8 (9.0, 17.4) |
| 25-29 | 57 | 38.7 | 30852 | 12.6 (9.0, 16.9) |
| 30-34 | 43 | 29.0 | 22480 | 12.9 (9.0, 18.5) |
| 35-39 | - | - | 10015 | 13.9 (7.6, 22.2) |
| 40+ | - | - | 2543 | - |
| Maternal race | ||||
| Alaska Native/American Indian | 95 | 58.9 | 25801 | 22.8 (17.4, 29.1) |
| Asian/Pacific Islander | 12 | 8.8 | 9399 | 9.3 (4.4, 16.8) |
| Black | 6 | 4.3 | 4134 | 10.3 (3.9, 24.8) |
| White | 87 | 62.2 | 61287 | 10.2 (7.9, 13.0) |
| Maternal education (years) | ||||
| <12 | 28 | 17.8 | 9778 | 18.2 (10.9, 27.8) |
| 12 | 93 | 60.2 | 36724 | 16.4 (12.7, 21.0) |
| 12+ | 74 | 53.0 | 52380 | 10.1 (7.6, 13.0) |
| Marital status | ||||
| Married | 99 | 69.6 | 64510 | 10.8 (8.5, 13.5) |
| Unmarried | 102 | 65.3 | 37078 | 17.6 (13.8, 22.3) |
| Maternal smoking use | ||||
| Reported smoking | 53 | 32.9 | 14467 | 22.7 (15.7, 31.2) |
| Reported not smoking | 140 | 97.3 | 85927 | 11.3 (9.3, 13.8) |
| Medicaid (mother or child) | ||||
| Medicaid | 147 | 93.7 | 51554 | 18.2 (14.7, 22.1) |
| non-Medicaid | 55 | 41.9 | 50311 | 8.3 (6.0, 11.1) |
| Father on birth certificate | ||||
| None | 16 | 10.2 | 5559 | 18.3 (9.9, 33.1) |
| Present | 186 | 125.4 | 96419 | 13.0 (10.9, 15.4) |
| Notes: Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Cleft Lip with Cleft Palate
Cleft Lip with Cleft Palate Prevalence
According to the 2014 annual report released by the National Birth Defects Prevention Network (NBDPN), the prevalence of cleft lip with cleft palate for 29 U.S. States from 2007-2011 was 5.6 per 10,000 live births.[3]
In Alaska, during 2007-2015, the prevalence of cleft lip with cleft palate was 14.0 per 10,000 live births.| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Total | 97 | 142.7 | 101978 | 14.0 (11.8, 16.4) |
| Notes: 95% CI = 95% Confidence Interval | ||||
Cleft Lip with Cleft Palate Trend
Prevalence per 10,000 births of cleft lip with cleft palate during 2007-2015 by three-year moving averages, with 95% confidence interval band and Poisson estimated fitted line.
| Estimate | Std. Error | t value | Pr(>|t|) |
|---|---|---|---|
| 0.07883 | 0.01521 | 5.18407 | 0.00351 |
| Reports | Defects | Births | Prevalence (95% CI) | Predicted Prevalence† | |
|---|---|---|---|---|---|
| 2007-2009 | 6.7 | 13.7 | 11261.7 | 12.2 (6.8, 19.7) | 10.9 |
| 2008-2010 | 7.3 | 13.7 | 11404.0 | 12.0 (6.7, 19.5) | 11.8 |
| 2009-2011 | 7.3 | 13.3 | 11404.7 | 11.6 (6.5, 19.1) | 12.8 |
| 2010-2012 | 8.3 | 14.1 | 11354.0 | 12.4 (6.9, 19.9) | 13.8 |
| 2011-2013 | 12.3 | 16.9 | 11349.0 | 14.8 (8.7, 22.8) | 15.0 |
| 2012-2014 | 15.7 | 19.0 | 11334.7 | 16.7 (10.3, 25.4) | 16.2 |
| 2013-2015 | 17.3 | 20.7 | 11377.0 | 18.2 (11.7, 27.5) | 17.5 |
|
Notes: Each row is based on three-year moving averages; Prevalence reported per 10,000 live births; 95% CI=95% Confidence Interval † Estimated rate based on Poisson model |
|||||
Cleft Lip with Cleft Palate Regional Distribution
Distribution of cleft lip with cleft palate in Alaska by Public Health Region of maternal residence at the time of birth. A description of regional breakdowns can found here. Data suppressed for # of reports < 6.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Anchorage | 32 | 21.6 | 41815 | 5.2 (3.3, 7.7) |
| Gulf Coast | 11 | 7.3 | 6313 | 11.6 (5.5, 22.8) |
| Interior | 22 | 14.7 | 18534 | 8 (4.5, 12.7) |
| Mat-Su | - | - | 12081 | - |
| Northern | 8 | 5.4 | 7114 | 7.5 (3.1, 16.4) |
| Southeast | - | - | 6375 | - |
| Southwest | 15 | 10.0 | 9746 | 10.3 (5.6, 18.9) |
| Notes:Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Cleft Lip with Cleft Palate Demographics
Some subgroups may be more at risk for having a baby with a cleft lip with cleft palate. This section provides the descriptive epidemiology of specified maternal, birth, and child characteristics identified from the birth certificate.
| Reports | Defects | Births | Prevalence (95% CI) | |
|---|---|---|---|---|
| Sex | ||||
| Female | 37 | 25.0 | 49462 | 5.0 (3.3, 7.2) |
| Male | 60 | 40.2 | 52516 | 7.7 (5.6, 10.4) |
| Birth weight (grams) | ||||
| <2500 | 16 | 10.6 | 5925 | 17.9 (9.3, 31.0) |
| 2500+ | 81 | 54.6 | 95890 | 5.7 (4.3, 7.3) |
| Maternal age | ||||
| 12-19 | - | - | 8043 | 7.5 (3.5, 16.2) |
| 20-24 | 33 | 22.1 | 28015 | 7.9 (5.2, 11.9) |
| 25-29 | 18 | 12.2 | 30852 | 4.0 (2.2, 6.8) |
| 30-34 | 20 | 13.5 | 22480 | 6.0 (3.4, 9.9) |
| 35-39 | 14 | 9.4 | 10015 | 9.3 (4.8, 17.1) |
| 40+ | - | - | 2543 | - |
| Maternal race | ||||
| Alaska Native/American Indian | 42 | 28.0 | 25801 | 10.9 (7.5, 15.7) |
| Asian/Pacific Islander | - | - | 9399 | 4.3 (1.7, 10.9) |
| Black | - | - | 4134 | - |
| White | 45 | 30.4 | 61287 | 5.0 (3.4, 7.0) |
| Maternal education (years) | ||||
| <12 | 15 | 10.0 | 9778 | 10.2 (5.6, 18.8) |
| 12 | 41 | 27.5 | 36724 | 7.5 (5.1, 10.7) |
| 12+ | 37 | 25.0 | 52380 | 4.8 (3.2, 7.0) |
| Marital status | ||||
| Married | 53 | 35.7 | 64510 | 5.5 (3.9, 7.5) |
| Unmarried | 43 | 28.8 | 37078 | 7.8 (5.2, 10.9) |
| Maternal smoking use | ||||
| Reported smoking | 18 | 12.0 | 14467 | 8.3 (4.8, 14.5) |
| Reported not smoking | 74 | 49.8 | 85927 | 5.8 (4.3, 7.5) |
| Medicaid (mother or child) | ||||
| Medicaid | 71 | 47.5 | 51554 | 9.2 (6.9, 12.1) |
| non-Medicaid | 26 | 17.7 | 50311 | 3.5 (2.1, 5.4) |
| Father on birth certificate | ||||
| None | - | - | 5559 | - |
| Present | - | - | 96419 | 6.4 (4.9, 8.1) |
| Notes: Prevalence reported per 10,000 live births; Data suppressed for # of reports < 6; 95% CI = 95% Confidence Interval | ||||
Technical notes
Column descriptions
# Reports: Unless otherwise noted, the number of unique reports of the defect received by ABDR during the specified birth year(s). Each report represents a unique child with the specified defect.
# Defects: The estimated true number of reports that are diagnosed defects based on medical record review and case confirmation.
# Births: The number of live births among Alaskan residents that occurred in Alaska during the specified birth year(s).
Prevalence (95% CI): The estimated diagnosed prevalence of the condition and corresponding 95% Confidence Interval. (For information on how the defect prevalence was estimated see below).
Defect prevalence calculation
The estimated defect prevalence was calculated using a Bayesian approach based on the reported prevalence, PPV and 1-NPV (see formula below).
Through medical records review and case confirmation of a random sample of reported cases, the defect prevalence is calculated as:
\[PPV (Positive Predictive Value) = p(defect|report)\] \[NPV (Negative Predictive Value) = p(\overline{defect}|\overline{report})\]
\[p(defect) \approx [p(report)\cdot PPV]+[p(\overline{report})\cdot (1-NPV)]\]
For cleft conditions, many cases were determined to be a different cleft defect than what was reported. Therefore, an additional intra-conditional probability was calculated and applied to the prevalence estimates. This probability accounts for the misclassified cleft conditions that were reported. In addition, a number of individuals were reported for more than one, or all three possible cleft conditions. For these individuals a separate PPV and NPV was calculated and applied.
Defect prevalence estimates are a more accurate estimation of the actual diagnosed prevalance of birth defects compared to the reported prevalance estimates in Alaska. ABDR obtains reports from medical providers using International Classification of Disease (ICD) codes that are extracted from individual systems which when aggregated may not reflect true diagnostics. Caution should be used when interpreting and comparing the reported prevalence estimates with national estimates.
See Data analysis methods for more information.
P-value estimate
To evaluate the trend over time and account for under/over-dispersion we constructed a quasi-Poisson regression model. This model assumes the variance is a linear function of the mean, and models the estimated number of annual defects by year with a natural log (ln) offset of the annual births. P-values < 0.05 are considered significant, which indicates that the predicted slope is significantly different from a slope of zero.
Data suppression
For region and demographic data tables, values are suppressed based on the number of reports received during the observation period. Counts less than 6 are suppressed (as indicated by ‘-’ in the table). For regions or demographics with only one cell count suppressed a second is suppressed to eliminate the ability to back-calculate the estimate.
References
[1] Facts about Cleft Lip and Cleft Palate | CDC. (2017). Centers for Disease Control and Prevention. Retrieved 22 March 2019, from https://www.cdc.gov/ncbddd/birthdefects/cleftlip.html
[2] Introduction to Cleft & Craniofacial Conditions - ACPA Family Services. (2019). Cleftline.org. Retrieved 22 March 2019, from https://cleftline.org/family-resources/introduction-to-cleft-craniofacial-conditions/
[3] Mai, C. T., Cassell, C. H., Meyer, R. E., Isenburg, J., Canfield, M. A., Rickard, R., … & Cho, S. J. (2014). Birth defects data from population-based birth defects surveillance programs in the United States, 2007 to 2011: Highlighting orofacial clefts. Birth Defects Research Part A: Clinical and Molecular Teratology, 100(11), 895-904.
Resources
Centers for Disease Control and Prevention
Genetic and Rare Diseases Information Center
National Birth Defects Prevention Network Mayo Clinic: Cleft lip and cleft palate Alaska’s Cleft Lip and Palate Program Seattle Children’s
Suggested Citation
State of Alaska Department of Health and Social Services, Division of Public Health, Section of Women’s, Children’s, and Family Health. Alaska Birth Defects Registry Condition Report: Cleft Conditions, Alaska, 2007-2015. Updated October 30, 2019. Available at: http://rpubs.com/AK_ABDR/cleft_Conds.
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: October 30, 2019
Code source: R:\ABDR\Analysis_New\ABDR_CASECONF\cond_reports\Published_reports\cleft_conds07_15.Rmd
