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目的:回顾性分析总结综合征型颅缝早闭症Le Fort Ⅲ型截骨术和面中部前徙牵引的初步疗效、围术期管理和并发症发生，为临床提供经验借鉴。方法:2017年10月至2020年1月在北京大学国际医院口腔颌面外科单纯行Le Fort Ⅲ型截骨牵引前徙术的综合征型颅缝早闭症患者20例，其中男性11例，女性9例；中位年龄 7岁（1.5~15岁）。术前经多学科评估提出风险防范预案，并进行术前干预治疗。专科检查获取SNA、气道容积、多导睡眠监测（polysomnography，PSG）、眼球突度及咬合关系诊断数据，通过虚拟手术规划制订截骨和牵引手术方案。术后1周及3、6、12个月拍摄CT，进行PSG和眼球突度检查，评估疗效，并总结综合征类型、多学科联合诊治（multiple disciplinary treatment，MDT）干预治疗、并发症的发生与结局。结果:20例患者中，Crouzon综合征15例，Pfeiffer综合征5例；18例以睡眠呼吸暂停为第一主诉，2例以突眼症第一主诉。8例患者进行术前干预性治疗，包括4例腺样体手术治疗，2例持续气道正压通气治疗，2例上颌扩弓治疗。手术相关并发症以意外骨折最多，占70%（14/20例），其他并发症包括脑脊液漏2例，颈内动脉海绵窦瘘1例，术后低钠血症5例，哭闹综合征2例，伤口感染2例，下眼睑倒睫4例，鼻畸形1例；3例实施了非计划二次手术。术后半年的SNA、气道容积及平均SpOn 2均显著大于术前（SNA：n F=10.094，n P=0.001；气道容积：n F=5.126，n P<0.001；平均SpOn 2：n F=10.782，n P=0.001），眼球突度和AHI均显著低于术前（眼球突度：n F=6.729，n P=0.010；AHI：n F=18.468，n P0.05）。n 结论:MDT围术期安全评估和早期干预是综合征性颅缝早闭症Le Fort III截骨牵引前徙术的有效诊疗模式，手术相关并发症以局部为主，全身并发症可控。“,”Objective:To summarize the preliminary efficacy, perioperative management and complications of Le Fort Ⅲ osteotomy and midface distraction in patients with syndromic craniosynostosis by retrospective analysis, and to provide clinical experience for reference.Methods:From October 2017 to January 2020, 20 patients with syndromic craniosynostosis underwent Le Fort Ⅲ osteotomy and distraction in The Department of Oral and maxillofacial Surgery of Peking University International Hospital, including 11 males and 9 females. The median age was 7 years (1.5 to 15 years). Preoperative risk prevention plan was put forward by multidisciplinary evaluation, and preoperative intervention was carried out. The diagnostic data of SNA, airway volume, polysomnography (PSG), ophthalmology and occlusal relationship were obtained through specialized examination, and osteotomy and distraction surgical plan was formulated through virtual surgical planning. CT was taken 1 week and 3, 6, 12 months after operation, PSG and eye protrudence examination were conducted to evaluate the therapeutic effect, syndrome type, multiple disciplinary treatment (MDT) intervention, occurrence and outcome of complications were summarized.Results:There were 15 cases of Crouzon syndrome and 5 cases of Pfeiffer syndrome. Sleep apnea was the first complaint in 18 cases and exophthalmia in 2 cases. Preoperative interventional therapy included 4 cases of adenoid surgery, 2 cases of continuous positive airway pressure (CPAP) and 2 cases of maxillary expansion. The most common surgical complications were accidental fracture (14/20 cases, 70%), cerebrospinal fluid fistula (2 cases), internal carotid cavernous sinus fistula (1 case), postoperative hyponatraemia (5 cases), crying syndrome (2 cases), wound infection (2 cases), trichiasis of lower eyelid (4 cases), and nasal malformation (1 case). Three cases underwent unplanned secondary surgery. SNA, airway volume and mean SpOn 2 six months after operation were significantly higher than those before operation (SNA: n F=10.094, n P=0.001; airway volume: n F=5.126, n P<0.001; mean SpOn 2: n F= 10, 782, n P=0.001), and the protrusion and AHI were significantly lower than those before surgery (protrusion: n F=6.729, n P=0.010; AHI: n F=18.468, n P0.05).n Conclusions:Perioperative safety assessment and early intervention of MDT is an effective diagnosis and treatment model of Le Fort Ⅲ osteotomy and distraction for syndromic craniosynosis. The operative complications are mainly local, and systemic complications are controllable.