先天性唇瘘又名Dermarguay氐综合症,患者多伴有唇裂和腭裂,此症比较罕见,发生率约1/15000～100000。遗传是个重要因素,有不同程度常染色体显性遗传。发病机制为:胚胎时期上颌突,球突和下颌突的游离缘为未来口唇基础,如该缘侧沟未消失可出现先天性唇瘘,多见于下唇。近年我科收治1例报告如下: Congenital lip fistula, also known as Dermarguay's syndrome, many patients with cleft lip and cleft palate, the disease is relatively rare, the incidence of about 1/15000 ~ 100000. Genetics is an important factor, with varying degrees of autosomal dominant inheritance. Pathogenesis: embryonic period maxillary process, bulbar and mandibular process free edge for the future lip base, such as the edge of the groove does not disappear can appear congenital lip fistula, more common in the lower lip. In recent years, our department received a report as follows: