Abstract
BackgroundA high incidence of orofacial clefts is reported in China, but no data has shown the relation between cleft types and the incidence of other defects so far. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts.Methodology and Principal FindingsAll children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1st Jan 2009 and 30th Dec 2011, were enrolled in this study. All subjects underwent a thorough examination and grouped by the cleft phenotype. The numbers and types of other organic defects were recorded and analyzed statistically using SPSS 17.0. Of 2180 cases reported as having orofacial clefts, 657 (30.1%) had other congenital abnormalities, which were significantly more common in cleft palate (47.9% (329/687)) than that in cleft lip (10.6% (80/755)) or cleft lip and palate (33.6% (248/738)) (P<0.01). In subgroups, unilateral cleft lip and palate had a statistically higher incidence of associated abnormalities than bilateral cleft lip and palate (P<0.01). The most common malformation was congenital heart disease, which counted 45.1% (296/657) of all malformations. Disorders of the central nervous system (14.3%(94/657)) and Skeletal anomalies (13.1%(86/657)) were also frequently associated. Additionally, the most common defect in heart was atrial septal defect, which was 39.7% (118/296) of all congenital heart diseases.Conclusions and SignificanceAs the high incidence of heart defects and other organic abnormalities in the children with cleft palate in Eastern China, special attention should be paid to them and echocardiography should be a proposed examination in the evaluation of children with cleft palate before any surgical correction being executed.
Highlights
According to anatomical, genetic and embryological findings, orofacial clefts (OCs) are commonly divided into isolated cleft lip (CL), isolated cleft palate (CP), and cleft lip and palate (CLP)
Vallino-Napoli reported that one third of the 2022 oral clefts patients had other birth defects and the prevalence rate of CL/P was higher than that of CP from 1983 to 2000 in Victoria, Australia [1]
Beriaghi reported that 32.2% of all cleft patients had other associated congenital malformations, which were more common in CP (38.7%) than in CL/P(26.4%) from 1980 to 2000 in USA [2]
Summary
Genetic and embryological findings, orofacial clefts (OCs) are commonly divided into isolated cleft lip (CL), isolated cleft palate (CP), and cleft lip and palate (CLP). OCs are often associated with other congenital abnormalities or organ defects. Beriaghi reported that 32.2% of all cleft patients had other associated congenital malformations, which were more common in CP (38.7%) than in CL/P(26.4%) from 1980 to 2000 in USA [2]. Luijsterburg reported that 10% of all cleft patients showed additional abnormalities of the head and neck area and 13% displayed congenital anomalies of other systems from 1997 to 2006 in Netherlands [3]. Aqrabawi reported that congenital heart disease(CHD) was the most common associated anomalies (47%), followed by skeletal abnormalities (13%) and renal anomalies(10%) among Jordanian infants from 2000 to 2005 [4]. The aim of this study is to assess the incidence of congenital heart diseases and other organic defects associated with different types of orofacial clefts
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