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. 2023 Jun 1;115(10):980-997.
doi: 10.1002/bdr2.2176. Epub 2023 Apr 26.

A multi-program analysis of cleft lip with cleft palate prevalence and mortality using data from 22 International Clearinghouse for Birth Defects Surveillance and Research programs, 1974-2014

Niall Mc Goldrick  1 , Gavin Revie  1 , Boris Groisman  2 , Paula Hurtado-Villa  3 , Antonin Sipek  4 , Babak Khoshnood  5 , Anke Rissmann  6 , Saeed Dastgiri  7 , Danielle Landau  8 , Giovanna Tagliabue  9 , Anna Pierini  10 , Miriam Gatt  11 , Osvaldo M Mutchinick  12 , Laura Martínez  13 , Hermein E K de Walle  14 , Elena Szabova  15 , Jorge Lopez Camelo  16 ; ECEMC Peripheral Group; Karin Källén  17 , Margery Morgan  18 , Wladimir Wertelecki  19 , Amy Nance  20 , Erin B Stallings  21 , Wendy N Nembhard  22 , Peter Mossey  1
Affiliations

A multi-program analysis of cleft lip with cleft palate prevalence and mortality using data from 22 International Clearinghouse for Birth Defects Surveillance and Research programs, 1974-2014

Niall Mc Goldrick et al. Birth Defects Res. .

Abstract

Background: Cleft lip with cleft palate (CLP) is a congenital condition that affects both the oral cavity and the lips. This study estimated the prevalence and mortality of CLP using surveillance data collected from birth defect registries around the world.

Methods: Data from 22 population- and hospital-based surveillance programs affiliated with the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) in 18 countries on live births (LB), stillbirths (SB), and elective terminations of pregnancy for fetal anomaly (ETOPFA) for CLP from 1974 to 2014 were analyzed. Prevalence and survival (survival for LB only) estimates were calculated for total and subclassifications of CLP and by pregnancy outcome.

Results: The pooled prevalence of total CLP cases was 6.4 CLP per 10,000 births. The prevalence of CLP and all of the pregnancy outcomes varied across programs. Higher ETOPFA rates were recorded in most European programs compared to programs in other continents. In programs reporting low ETOPFA rates or where there was no ascertainment of ETOPFA, the rate of CLP among LB and SB was higher compared to those where ETOPFA rates were ascertained. Overall survival for total CLP was 91%. For isolated CLP, the survival was 97.7%. CLP associated with multiple congenital anomalies had an overall survival of 77.1%, and for CLP associated with genetic/chromosomal syndromes, overall survival was 40.9%.

Conclusions: Total CLP prevalence reported in this study is lower than estimates from prior studies, with variation by pregnancy outcomes between programs. Survival was lower when CLP was associated with other congenital anomalies or syndromes compared to isolated CLP.

Keywords: congenital anomalies; craniofacial abnormalities; mortality; prevalence; surveillance.

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Figures

FIGURE 1
FIGURE 1
Pooled prevalence per 10,000 births of total cleft lip with the cleft palate for each participating ICBDSR program for the period 1974–2014.
FIGURE 2
FIGURE 2
Prevalence by year of undifferentiated cleft lip with the cleft palate.
FIGURE 3
FIGURE 3
Quality indicators used by authors to assess data quality of data sets and inform inclusion criteria.
See this image and copyright information in PMC

References

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