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克鲁宗综合征的治疗算法。

Algorithm to the treatment of Crouzon syndrome.

作者信息

Raffaini Mirco, Magri Alice Sara, Genitori Lorenzo, Arcuri Francesco

机构信息

"Facesurgery Center", Parma, Italy.

Service of Pediatric Neurosurgery, Hospital for Sick Children Anna Meyer, Florence, Italy.

出版信息

J Craniomaxillofac Surg. 2022 Feb;50(2):124-133. doi: 10.1016/j.jcms.2021.11.008. Epub 2021 Nov 26.

DOI:10.1016/j.jcms.2021.11.008
PMID:34857441
Abstract

Clinical reports regarding the entire surgical sequence in Crouzon syndrome from the neonatal period to the adult age are rare. The purpose of this study is to trace an operative algorithm with a long term follow up in a homogenous group of patients affected by Crouzon syndrome. A retrospective review was conducted for all patients affected by Crouzon syndrome who completed the entire surgical sequence. 7 Crouzon patients (4 females, 3 males) completed the entire surgical sequence at different ages: fronto-orbital advancement (0.9 years), LF III distraction osteogenesis (11.5 years) and orthognathic surgery (18 years). The mean age at the last follow up was 19.3 years; normalization of the face was obtained in all cases with improvement of the respiratory problems. After orthognathic surgery, all patients had stable occlusion. A one-year postoperative CBCT scan revealed almost complete ossification of all osteotomy sites. Frontoorbital advancement and modified Le Fort III distraction osteogenesis are reliable surgical procedures. SARME and conventional orthognathic surgery with reductive genioplasty and fat grafting are performed at the end of the surgical sequence to enhance facial aesthetics.

摘要

关于从新生儿期到成年期克鲁宗综合征整个手术流程的临床报告很少。本研究的目的是在一组受克鲁宗综合征影响的同质患者中追踪一种具有长期随访的手术算法。对所有完成整个手术流程的克鲁宗综合征患者进行了回顾性研究。7例克鲁宗综合征患者(4例女性,3例男性)在不同年龄完成了整个手术流程:额眶前移术(0.9岁)、改良Le Fort III型牵引成骨术(11.5岁)和正颌手术(18岁)。最后一次随访时的平均年龄为19.3岁;所有病例面部均恢复正常,呼吸问题得到改善。正颌手术后,所有患者咬合稳定。术后一年的CBCT扫描显示所有截骨部位几乎完全骨化。额眶前移术和改良Le Fort III型牵引成骨术是可靠的手术方法。序列治疗的最后阶段采用骨锚式上颌骨牵张成骨术(SARME)和常规正颌手术联合颏部成形术及脂肪移植术,以改善面部美观。

相似文献

1
Algorithm to the treatment of Crouzon syndrome.克鲁宗综合征的治疗算法。
J Craniomaxillofac Surg. 2022 Feb;50(2):124-133. doi: 10.1016/j.jcms.2021.11.008. Epub 2021 Nov 26.
2
Midface correction in patients with Crouzon syndrome is Le Fort III distraction osteogenesis with a rigid external distraction device the gold standard?颅面骨发育不全综合征患者的中面部矫正采用 Le Fort III 骨切开术联合刚性外牵引器,是金标准吗?
J Craniomaxillofac Surg. 2019 Mar;47(3):420-430. doi: 10.1016/j.jcms.2018.11.028. Epub 2018 Dec 31.
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A Rare Orbital Complication of Eye Exodeviation With Limited Abduction During Monobloc Le Fort III Distraction Osteogenesis.单块式Le Fort III型牵张成骨术中伴外展受限的眼球外斜视罕见眼眶并发症。
Cleft Palate Craniofac J. 2015 Jul;52(4):489-93. doi: 10.1597/14-013. Epub 2014 Jul 9.
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Le Fort III external midface distraction: surgical outcomes and skeletal stability.勒福Ⅲ型面中部外牵张成骨:手术效果与骨骼稳定性
J Craniofac Surg. 2012 May;23(3):896-900. doi: 10.1097/SCS.0b013e31824e2549.
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Long-term follow-up of a patient diagnosed with Crouzon syndrome who underwent Le Fort I and III distraction osteogenesis using a rigid external distractor system.采用硬性外牵张器系统行 Le Fort I 和 III 牵张成骨术治疗 Crouzon 综合征患者的长期随访。
Angle Orthod. 2023 Nov 1;93(6):736-746. doi: 10.2319/011823-40.1.
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The Le Fort III osteotomy: to distract or not to distract?勒福Ⅲ型截骨术:是否进行牵张成骨?
Plast Reconstr Surg. 2001 Apr 15;107(5):1091-103; discussion 1104-6. doi: 10.1097/00006534-200104150-00001.
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Monobloc Le Fort III Distraction Osteogenesis for Correction of Severe Fronto-orbital and Midface Hypoplasia in Pediatric Crouzon Syndrome.单块式勒福Ⅲ型牵引成骨术治疗小儿克鲁宗综合征严重额眶及面中部发育不全
Cleft Palate Craniofac J. 2016 Jan;53(1):118-25. doi: 10.1597/14-210. Epub 2015 Feb 4.
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Modified Le Fort III osteotomy: A simple solution to severe midfacial hypoplasia.改良 Le Fort III 截骨术:治疗严重面中部发育不全的简单方法。
J Craniomaxillofac Surg. 2018 May;46(5):837-843. doi: 10.1016/j.jcms.2018.03.010. Epub 2018 Mar 21.
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Additional orthognathic surgery following Le Fort III and monobloc advancement.Le Fort III 型截骨术后同期行正颌外科手术和单颌骨前移术。
Int J Oral Maxillofac Surg. 2011 Jul;40(7):679-84. doi: 10.1016/j.ijom.2011.02.014. Epub 2011 Mar 12.
10
Treatment of the Patient With Crouzon Syndrome With Orthognathic Surgery.
J Craniofac Surg. 2020 May/Jun;31(3):806-808. doi: 10.1097/SCS.0000000000006177.

引用本文的文献

1
Ocular manifestations and treatment progress of Crouzon syndrome.Crouzon 综合征的眼部表现及治疗进展。
Int Ophthalmol. 2024 Sep 5;44(1):367. doi: 10.1007/s10792-024-03293-5.
2
Adenoidectomy in a child with Crouzon syndrome complicated with severe obstructive sleep apnea: Case report and review of literature.儿童颅缝早闭综合征伴严重阻塞性睡眠呼吸暂停行腺样体切除术:病例报告及文献复习。
Medicine (Baltimore). 2024 Jun 7;103(23):e38534. doi: 10.1097/MD.0000000000038534.
3
[Application and development of orthognathic surgery in treatment of syndromic craniosynostosis].
正颌外科手术在综合征性颅缝早闭治疗中的应用与进展
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2023 Jul 15;37(7):879-884. doi: 10.7507/1002-1892.202302102.