Witherow Helen, Dunaway David, Evans Robert, Nischal Ken K, Shipster Caroleen, Pereira Valerie, Hearst Daniella, White Michelle, Jones Barry M, Hayward Richard
London, United Kingdom From the Department of Craniofacial Surgery, Great Ormond Street Hospital, and the Thames Regional Cleft Service.
Plast Reconstr Surg. 2008 Apr;121(4):1311-1322. doi: 10.1097/01.prs.0000305538.75347.52.
Craniofacial dysostosis syndromes produce multisutural synostoses combined with severe midfacial retrusion. This may cause serious functional problems, including airway obstruction, exposure of the eyes, visual pathway dysfunction, and raised intracranial pressure. Early midface advancement may be necessary to address these issues. Distraction osteogenesis has provided the facility to achieve significant advances safely and is often in excess of that which is achievable by conventional means.
A retrospective study of 20 patients with craniofacial dysostosis and severe midface hypoplasia who underwent monobloc advancement osteotomies using the rigid external distractor system principally for functional reasons was undertaken. The multidisciplinary management and outcome measures of these patients were recorded.
The midface was distracted an average of 16.4 mm, with a range of 12 to 22 mm. Ocular protection was achieved in all patients with preoperative exposure keratopathy and/or globe subluxation. Improvements in optic disc swelling and pattern visually evoked potentials were seen in those patients with threatened visual impairment. Improvement in airway obstruction was seen in those patients with abnormal polysomnography. Decannulation was achieved in five of the seven patients with tracheostomies. Fifty percent had a reduction in hyponasality, and the visual appearance of speech was improved. Complications included persistent cerebrospinal fluid leakage, acquired hypernasality in 25 percent, cranial bone loss, and sinus formation requiring surgical revision.
Monobloc distraction osteogenesis results in good aesthetic and functional outcomes. The relatively high rate of complications remains a concern, and further adaptations of technique are needed to reduce the risks of this procedure.
颅面骨发育不全综合征会导致多缝早闭，并伴有严重的面中部后缩。这可能会引发严重的功能问题，包括气道阻塞、眼部暴露、视觉通路功能障碍以及颅内压升高。早期进行面中部前移可能是解决这些问题的必要手段。牵引成骨技术为安全实现显著的前移提供了便利，且通常能达到传统方法难以企及的效果。
对20例患有颅面骨发育不全且面中部严重发育不全的患者进行回顾性研究，这些患者主要因功能原因采用坚固外固定牵引系统进行整块前移截骨术。记录这些患者的多学科管理及结果指标。
面中部平均牵引16.4毫米，范围为12至22毫米。所有术前患有暴露性角膜病变和/或眼球半脱位的患者均实现了眼部保护。那些有视觉损害风险的患者，视盘肿胀和图形视觉诱发电位有所改善。多导睡眠图异常的患者气道阻塞情况得到改善。7例气管切开患者中有5例实现了拔管。50%的患者鼻音减轻，语音的视觉外观得到改善。并发症包括持续性脑脊液漏、25%的患者出现获得性鼻音过重、颅骨缺失以及需要手术修复的鼻窦形成。
整块牵引成骨术可带来良好的美学和功能效果。相对较高的并发症发生率仍然令人担忧，需要进一步改进技术以降低该手术的风险。
