Department of Cardiology, Shanghai Ninth People's Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, PR China.
PLoS One. 2013;8(1):e54726. doi: 10.1371/journal.pone.0054726. Epub 2013 Jan 21.
A 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.
All children with orofacial clefts, which were sought out from the Health Information System of Shanghai Ninth People's Hospital between 1(st) Jan 2009 and 30(th) 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.
As 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.
中国有很高的唇腭裂发病率，但目前尚无数据表明唇腭裂类型与其他畸形的发病率之间存在关联。本研究旨在评估不同类型的唇腭裂与先天性心脏病和其他器官畸形的发病率之间的关系。
本研究纳入了 2009 年 1 月 1 日至 2011 年 12 月 30 日期间在上海第九人民医院健康信息系统中发现的所有唇腭裂患儿。所有患儿均接受了详细检查，并根据唇腭裂表型进行分组。记录其他器官畸形的数量和类型，并使用 SPSS 17.0 进行统计学分析。在报告的 2180 例唇腭裂病例中，有 657 例（30.1%）存在其他先天性异常，其中腭裂（47.9%（329/687））明显多于唇裂（10.6%（80/755））或唇腭裂（33.6%（248/738））（P<0.01）。在亚组中，单侧唇腭裂的伴发畸形发生率明显高于双侧唇腭裂（P<0.01）。最常见的畸形是先天性心脏病，占所有畸形的 45.1%（296/657）。中枢神经系统疾病（14.3%（94/657））和骨骼异常（13.1%（86/657））也经常与唇腭裂相关。此外，心脏最常见的缺陷是房间隔缺损，占所有先天性心脏病的 39.7%（118/296）。
鉴于中国东部地区腭裂患儿心脏缺陷和其他器官异常的高发生率，在进行任何手术矫正之前，应特别注意这些患儿，并建议对腭裂患儿进行超声心动图检查。
