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

Background: Cleft lip with cleft palate (CLP) is a congenital condition thataffects both the oral cavity and the lips. This study estimated the prevalenceand mortality of CLP using surveillance data collected from birth defect registries around the world.Methods: Data from 22 population- and hospital-based surveillance programsaffiliated with the International Clearinghouse for Birth Defects Surveillanceand Research (ICBDSR) in 18 countries on live births (LB), stillbirths (SB), andelective terminations of pregnancy for fetal anomaly (ETOPFA) for CLP from1974 to 2014 were analyzed. Prevalence and survival (survival for LB only) estimates were calculated for total and subclassifications of CLP and by pregnancyoutcome.Results: The pooled prevalence of total CLP cases was 6.4 CLP per 10,000births. The prevalence of CLP and all of the pregnancy outcomes varied acrossprograms. Higher ETOPFA rates were recorded in most European programscompared to programs in other continents. In programs reporting low ETOPFA rates or where there was no ascertainment of ETOPFA, the rate ofCLP among LB and SB was higher compared to those where ETOPFA rateswere ascertained. Overall survival for total CLP was 91%. For isolated CLP, thesurvival was 97.7%. CLP associated with multiple congenital anomalies had anoverall survival of 77.1%, and for CLP associated with genetic/chromosomalsyndromes, 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.