Bipartition截骨治疗眶距增宽症合并颌面部发育畸形

Bipartition osteotomy for treating orbital hypertelorism with maxillofacial malformation

目的探讨Bipartition截骨术结合颅骨重塑治疗眶距增宽症伴发面中部发育不良的临床效果。方法回顾性分析2005年7月和2018年8月上海交通大学医学院附属第九人民医院收治的2例眶距增宽症合并颌面部发育畸形患者的临床资料。男1例,女1例,年龄分别为17、19岁。术前采集患者全头颅CT数据,进行三维重建,对手术进行设计,并进行手术模拟,确定术中去骨量及V形截骨线角度。手术采用经颅内径路,额部颅骨开窗,完成颅面整体Monobloc联合截骨游离整块颅-眶-上颌骨组织后,再根据术前三维设计的方案行Bipartition截骨V形截除眶间骨块及沿中线纵向劈开硬腭,最终使颅面骨分为2段完全游离的骨块,重塑额部,将双侧眶-上颌骨块内旋并固定,以肋软骨隆鼻。术后随访观察患者恢复情况、并发症发生情况,以及眼球活动、视力、嗅觉、鼻部形态、骨性眶间距(IOD)、眶距增宽外观形态的改善程度。结果2例患者均去除眶鼻骨15 mm;IOD分别缩窄为17 mm和19 mm。2例患者均在术后出现轻度脑脊液漏,分别去枕平卧5 d和8 d后自愈。男患者出现冠状切口处局部皮肤感染,换药1周后痊愈。女患者术后1周鼻尖近鼻小柱部分破溃,清创修整后痊愈。随访4~11个月,2例患者的眼球活动可,视力可,无复视,辐辏功能正常,嗅觉正常。鼻部重建形态可,眶距增宽的外观形态得到有效纠正,但鼻部形态及内眦赘皮仍需进一步修整。结论Bipartition截骨术能有效治疗面中部过宽,通过两侧颅-眶-颧-上颌骨块内旋固定可使高拱形腭降低,使上颌咬合平面平坦。

Objective To summarize the experience of Bipartition osteotomy combined with craniofacial bone remodeling in the treatment of orbital hypertelorism and midfacial dysplasia.Methods A retrospective analysis was done by the clinical data of two patients with orbital hypertelorism with maxillofacial malformation treated in the Shanghai Ninth People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine in July 2005 and August 2018.The Bipartition osteotomy via intracranial approach were applied for the two patients,one male and one female.The two patients aged 17 and 19,were suffered from degreeⅠorbital hypertelorism[interorbital distance(IOD)32 mm and 34 mm].With a frontal bone fenestration,the Monobloc osteotomy was done firstly to dislocate the cranio-orbital-maxilla bone graft,then the Bipartition osteotomy was performed according to the preoperative design through 3D computer aided design(CAD)and a V-shape bone graft was removed from the interorbital bone graft to split the hard palate longitudinally along the midline.After all,the whole facial bone was separated to two blocks:bilateral orbital-maxillary segments.Finally the midface had been remodeled by the bilateral orbital-maxillary segments which rotated and fixed internally.Self-rib nasal augmentation was done later.Patents’complications,eye movement,visual acuity,olfactory sensation,nasal shape,IOD were measured through CT scan and the appearances were observed in the postoperative follow-up to determine the degree of improvement.Results 15 mm in width of interorbital bone was resected in both of the two patients,respectively.Postoperative IOD reduced by 17 mm,19 mm,respectively.Mild cerebrospinal fluid leakage occurred in both patients after operation.They recovered after 5,8 days of pillow-free horizontal position,respectively.The male patient developed local skin infection at the coronal incision and recovered after dressing change for 1 week.One week after operation,the female patient’s nose tip was partially broken near the nasal columella,and recovered after debridement and repairment.Follow up for 4-11 months showed that the eye movement,visual acuity,normal convergence and olfactory sensation were normal and no diplopia of 2 patients.The nasal appearances and orbital hypertelorisum of them were corrected obviously by follow-up after 4 months,but the nasal morphology and epicanthus still need further improvement.Conclusions The Bipartition osteotomy can effectively treat the orbital hypertelorisum through the internal rotation fixation of bilateral orbital-maxillary segments,that makes the high arch palate lower and the occlusal plane of the upper jaw flattened.