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目的:通过对Van der Woude综合征(VWS)的主要临床表现-下唇瘘的研究,总结其主要治疗方法。方法:收集沈阳市2000~2012年共9例VWS患者,对其主要临床表现及治疗方法进行统计。结果:9例下唇瘘的临床表现中4例伴有左侧唇、腭裂,4例伴有双侧唇、腭裂;经过手术治疗所有VWS患者的颌面畸形都得到了有效的纠正。结论:VWS是常染色体显性遗传病,其临床表现以下唇瘘为主,并常伴有唇、腭裂的发生。对该综合征的治疗手段应以纠正其颌面畸形的外科手术为主。
OBJECTIVE: To summarize the main treatment methods of the lower lip fistula, the main clinical manifestations of Van der Woude syndrome (VWS). Methods: A total of 9 VWS patients from 2000 to 2012 in Shenyang were collected and their main clinical manifestations and treatment methods were analyzed. Results: In the 9 cases of lower lip fistula, 4 cases were accompanied by left lip and cleft palate and 4 cases with bilateral lip and cleft palate. The maxillofacial deformity in all VWS patients was effectively corrected. Conclusion: VWS is an autosomal dominant genetic disease. Its clinical manifestations are mainly lip fistula, and often accompanied by the occurrence of lip and cleft palate. The treatment of the syndrome should be based on the correction of maxillofacial surgery based surgery.