纠正典型的 Apert 面容：分体式截骨与单颌骨牵张术相结合。
Correcting the typical Apert face: combining bipartition with monobloc distraction.
机构信息
London, United Kingdom From the Department of Craniofacial Surgery, Great Ormond Street Hospital, and Cavendish Imaging.
出版信息
Plast Reconstr Surg. 2013 Feb;131(2):219e-230e. doi: 10.1097/PRS.0b013e3182778882.
BACKGROUND
Bipartition distraction is a novel procedure combining frontofacial bipartition and monobloc distraction. Apert syndrome and other syndromic craniofacial dysostoses are often characterized by hypertelorism, with a negative canthal axis and counterrotated orbits. Central midface hypoplasia can result in a biconcave face in both midsagittal and axial planes. Bipartition distraction can correct these facial abnormalities.
METHODS
Twenty patients (19 Apert syndrome patients and one Pfeiffer syndrome patient, aged 1.6 to 21 years) underwent bipartition distraction. Severity of appearance was graded preoperatively and postoperatively as mild, moderate, or severe. Functional problems were documented by a multidisciplinary team. Central and lateral midface skeletal advancement were measured. Follow-up ranged from 15 months to 7 years.
RESULTS
Bipartition distraction consistently produced more central than lateral facial advancement. Mean central advancement was 13.2 ± 5.9 mm at sella-nasion and 11.7 ± 5.4 mm at sella-A point. Lateral advancement was 4.7 ± 2.8 mm. Unbending the face improved aesthetic appearance. Airway function, eye exposure, and elevated intracranial pressure were improved. Complications included six temporary cerebrospinal fluid leaks (four needing a lumbar drain), five patients with postoperative seizures, five patients requiring Rigid External Distraction frame repositioning, one palatal fistula, one velopharyngeal incompetence, five pin-site infections, one abscess under frontal bone, three cases of sepsis, nine patients with worsened strabismus, two patients with enophthalmos, one patient with partial visual field loss, and three patients who required reintubation because of aspiration.
CONCLUSIONS
: Bipartition distraction is an effective procedure with which to differentially advance the central face in Apert syndrome. It improves both function and aesthetics.
CLINICAL QUESTION/LEVEL OF EVIDENCE: : Therapeutic, IV.
背景
分体式牵张是一种新的手术方法，结合了额面分体式和整块牵张。Apert 综合征和其他综合征性颅面发育不良症常表现为内眦距过宽，内眦轴呈负向，眼球旋转。中面部发育不全可导致矢状面和轴位的双凹面。分体式牵张术可以矫正这些面部畸形。
方法
20 例患者（19 例 Apert 综合征患者和 1 例 Pfeiffer 综合征患者，年龄 1.6 至 21 岁）接受了分体式牵张术。术前和术后的外观严重程度分级为轻度、中度或重度。由多学科团队记录功能问题。测量了中面部和侧面部的骨骼前徙距离。随访时间为 15 个月至 7 年。
结果
分体式牵张术始终能使中面部比侧面部获得更多的前徙。蝶鞍-鼻根（sella-nasion）的平均中面部前徙距离为 13.2±5.9mm，蝶鞍-前点（sella-A point）为 11.7±5.4mm。侧面部前徙距离为 4.7±2.8mm。矫正颜面畸形改善了美观。气道功能、眼球暴露度和颅内压增高均得到改善。并发症包括 6 例暂时性脑脊液漏（4 例需行腰椎引流）、5 例术后癫痫、5 例需要重新定位 Rigid External Distraction 外固定架、1 例腭裂瘘、1 例软腭咽闭合不全、5 例针道感染、1 例额骨下脓肿、3 例脓毒症、9 例斜视加重、2 例眼球内陷、1 例部分视野缺失、3 例因误吸需要重新插管。
结论
分体式牵张术是一种有效的方法，可使 Apert 综合征的中面部获得差异前徙，改善功能和美观。
临床问题/证据水平：治疗性，IV 级。
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