面裂鼻畸形病因及临床诊疗的研究进展

Research Progress in Etiology,Diagnosis and Treatment of Nasal Deformity Caused by Craniofacial Cleft

面裂鼻畸形是指累及鼻部亚单位的先天性颅面裂隙畸形,表现为鼻部组织的部分或完全缺失。其中以正中裂(Tessier0号面裂)、旁正中裂(Tessier1号及2号面裂)及眼裂(Tessier3号面裂)为主。面裂鼻畸形病因尚不明确,目前主要有融合失败及外胚层渗入两种假说。临床上可根据鼻部亚单位理论对鼻畸形进行分析,进而制订针对性的手术方案。对合并眶距增宽的鼻背畸形,可通过眶间/鼻背截骨术对眼鼻进行同期修复,并选择合适的假体置入物或自体软骨移植物以增加鼻背高度。鼻翼畸形可选择局部皮瓣、复合组织瓣结合耳甲腔软骨移植等方法进行修复。但目前治疗方案尚无统一标准,亟待更多临床研究进一步探讨,以改善面裂鼻畸形患者的手术效果,提高患者满意度。

Nasal deformity is caused by craniofacial cleft,resulting in partial or complete loss of nasal tissue.It is a type of congenital malformation,and mainly composed of midline cleft(Tessier No.0 clefts),paramedian cleft(Tessier No.1 and No.2 clefts) and orbital cleft(Tessier No.3 clefts).The etiology is still controversial,with two hypotheses:fusion failure and ectodermal infiltration.Nasal malformation could be analyzed based on nasal subunits to make an individualized surgical plan.Nasal dorsal deformity associated with hypertelorism can be repaired by synchronous osteotomies.The alloplastic materials or autologous cartilage can be implanted or transferred to increase the nasal dorsal height.Nasal alar malformation can be repaired using local flap,composite tissue flap and/or ear cartilage transplantation.However,there is no uniform standard for the treatment yet.Therefore,further clinical research is needed to improve the surgical outcome and the satisfaction of the patients.