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非综合征型颅缝早闭症的手术治疗与并发症解析

  • 陈锦泉 ,
  • 王晓强
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  • 上海交通大学医学院附属新华医院小儿神经外科,上海 200082
王晓强,主任医师,博士;电子信箱:Wangxiaoqiang@xinhuamed.com.cn

收稿日期: 2025-11-19

  录用日期: 2026-01-08

  网络出版日期: 2026-05-28

基金资助

上海市促进产业高质量发展专项资金先导产业创新发展项目(RZCYA101250994)

Analysis of surgical treatment and complications of non-syndromic craniosynostosis

  • Chen Jinquan ,
  • Wang Xiaoqiang
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  • Department of Pediatric Neurosurgery, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200082, China
Wang Xiaoqiang, E-mail: Wangxiaoqiang@xinhuamed.com.cn.

Received date: 2025-11-19

  Accepted date: 2026-01-08

  Online published: 2026-05-28

Supported by

Shanghai Special Fund for Promoting High-Quality Industrial Development-Pilot Industry Innovation and Development Program(RZCYA101250994)

摘要

非综合征型颅缝早闭症(non-syndromic craniosynostosis,NSC)是儿童期常见的先天性发育畸形。其特征为一条或多条颅缝过早融合,进而引发颅骨形态异常、颅内压升高,以及一系列神经发育问题。根据受累颅缝的差异,NSC可表现为舟状头、三角头、前斜头、短头及后斜头等多种异常头型。目前,手术治疗仍是针对NSC唯一有效的干预手段,旨在解除颅缝的融合状态、扩大颅腔容积并恢复颅颌部的正常形态。近年来,手术理念与技术持续发展,主要术式包括内镜辅助下早闭颅缝骨条切除术、颅骨重塑术(如Pi式手术及传统的大范围全颅盖切开松解重塑术),以及弹簧辅助颅骨重塑术。内镜辅助下早闭颅缝骨条切除术适用于确诊的月龄≤3个月的低龄患儿,该术式创伤小、恢复快,但术后颅骨塑形依赖佩戴头盔。Pi式手术和全颅盖重塑术可在术中即时实现显著的颅腔容积扩大与形态矫正,是目前应用于3月龄以上患儿最为广泛的术式。弹簧辅助颅骨重塑术则通过持续的牵张力实现颅腔的渐进性扩容,但需二次手术以取出植入装置。术式选择需综合患儿的年龄、畸形类型、颅内压水平及机构技术条件等因素,并且离不开多学科团队的评估与协作。尽管NSC手术总体安全性较高,但在长期随访过程中,术后复发与颅骨缺损仍是最为关键且具有重要临床意义的并发症,其中再骨化过程的异常被视为导致这些并发症的关键机制。未来研究应在明确术后再骨化机制的基础上,进一步整合个体化手术规划、新型生物材料应用,以及长期神经认知与生活质量结局的评估,以推动NSC的治疗模式从形态矫正向长期功能优化转变。

本文引用格式

陈锦泉 , 王晓强 . 非综合征型颅缝早闭症的手术治疗与并发症解析[J]. 上海交通大学学报(医学版), 2026 , 46(5) : 665 -671 . DOI: 10.3969/j.issn.1674-8115.2026.05.013

Abstract

Non-syndromic craniosynostosis (NSC) is a common congenital developmental malformation in childhood. It is characterized by the premature fusion of one or more cranial sutures, which in turn leads to abnormal skull morphology, increased intracranial pressure, and a series of neurodevelopmental problems. Depending on the different cranial sutures involved, NSC can present with various abnormal head shapes, such as scaphocephaly, trigonocephaly, anterior plagiocephaly, brachycephaly, and posterior plagiocephaly. Currently, surgical treatment remains the only effective intervention for NSC, aiming to release the fused cranial sutures, expand the cranial cavity volume, and restore normal cranio-maxillofacial morphology. In recent years, surgical concepts and techniques have been continuously evolving. The main surgical procedures include endoscopic-assisted strip craniectomy for prematurely closed cranial sutures, cranial vault remodeling (such as the Pi procedure and traditional extensive calvarial vault incision, release, and remodeling), and spring-assisted cranial vault remodeling. Endoscopic-assisted strip craniectomy is suitable for infants diagnosed within 3 months of age. This procedure is minimally invasive and allows rapid recovery; however, postoperative cranial remodeling depends on helmet therapy. The Pi procedure and total cranial vault remodeling can achieve immediate and significant expansion of cranial cavity volume and morphological correction during the operation, and are currently the most widely used surgical procedures for children over 3 months old. Spring-assisted cranial vault remodeling achieves gradual expansion of the cranial cavity through continuous traction forces, but a second operation is required to remove the implanted devices. The selection of surgical procedures needs to comprehensively consider factors such as the child′s age, type of deformity, intracranial pressure level, and the technical conditions of the institution, and also relies on the evaluation and collaboration of a multidisciplinary team. Although NSC surgery is generally highly safe, during long-term follow-up, postoperative recurrence and skull defects are still the most critical and clinically significant complications. Abnormalities in the reossification process are regarded as the key mechanisms leading to these complications. Future research should, on the basis of clarifying the mechanism of postoperative reossification, further integrate individualized surgical planning, the application of new biomaterials, and the evaluation of long-term neurocognitive and quality-of-life outcomes, so as to promote the transformation of NSC treatment from morphological correction to long-term functional optimization.

参考文献

[1] Persing J A. MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis[J]. Plast Reconstr Surg, 2008, 121(4 Suppl): 1-11.
[2] Shlobin N A, Baticulon R E, Ortega C A, et al. Global epidemiology of craniosynostosis: a systematic review and meta-analysis[J]. World Neurosurg, 2022, 164: 413-423.e3.
[3] Stanton E, Urata M, Chen J F, et al. The clinical manifestations, molecular mechanisms and treatment of craniosynostosis[J]. Dis Model Mech, 2022, 15(4): dmm049390.
[4] Roth D M, Souter K, Graf D. Craniofacial sutures: signaling centres integrating mechanosensation, cell signaling, and cell differentiation[J]. Eur J Cell Biol, 2022, 101(3): 151258.
[5] 施诚仁, 蔡威, 吴晔明, 等. 新生儿外科学[M]. 2版. 上海: 世界图书出版公司, 2019.
  Shi C R, Cai W, Wu Y M, et al. Neonatal Surgery[M]. 2nd ed. Shanghai: World Publishing Corporation, 2019.
[6] Frassanito P, di Rocco C. Depicting cranial sutures: a travel into the history[J]. Childs Nerv Syst, 2011, 27(8): 1181-1183.
[7] Pontell M E, Barrero C E, Wagner C S, et al. Oxycephaly-systematic review, case presentation, and diagnostic clarification[J]. Childs Nerv Syst, 2023, 39(11): 3041-3049.
[8] Virchow R. Uber den cretinismus, namentlich in Franken, und uber pathologische schadelformen[J]. Verh Phys Med Gesellsch Wurzburg,1851, 2: 230-270.
[9] Iqbal J, Yangi K, Naseem A, et al. Surgery of craniosynostosis: a historical review[J]. Ann Med Surg (Lond), 2025, 87(4): 2234-2242.
[10] Tessier P, Delaire J, Billet J, et al. Consid′erations sur le d′eveloppement de l′orbite; ses incidences sur la croissance faciale[J]. Revue Stomatol 1965, 66: 27-39.
[11] Tessier P, Guiot G, Rougerie J, et al. Cranio-naso-orbito-facial osteotomies. hypertelorism[J]. Ann Chir Plast, 1967, 12(2): 103-118.
[12] Arnaud é. L′innovation en chirurgie craniofaciale: depuis Tessier jusqu′aux perspectives futures. D′après les témoignages de F. Ortiz-Monasterio, D. Marchac, F. Firmin et T. Wolfe[J]. Ann Chir Plast Esthe, 2010, 55(5): 363-383.
[13] Jimenez D F, Barone C M. Endoscopic craniectomy for early surgical correction of sagittal craniosynostosis[J]. J Neurosurg, 1998, 88(1): 77-81.
[14] Delye H H K, Borstlap W A, van Lindert E J. Endoscopy-assisted craniosynostosis surgery followed by helmet therapy[J]. Surg Neurol Int, 2018, 9: 59.
[15] Clayman M A, Murad G J, Steele M H, et al. History of craniosynostosis surgery and the evolution of minimally invasive endoscopic techniques: the University of Florida experience[J]. Ann Plast Surg, 2007, 58(3): 285-287.
[16] Ko J M. Genetic syndromes associated with craniosynostosis[J]. J Korean Neurosurg Soc, 2016, 59(3): 187-191.
[17] Jabs E W, Müller U, Li X, et al. A mutation in the homeodomain of the human MSX2 gene in a family affected with autosomal dominant craniosynostosis[J]. Cell, 1993, 75(3): 443-450.
[18] Goovaerts S, Hoskens H, Eller R J, et al. Joint multi-ancestry and admixed GWAS reveals the complex genetics behind human cranial vault shape[J]. Nat Commun, 2023, 14(1): 7436.
[19] Nicoletti P, Zafer S, Matok L, et al. Regulatory elements in SEM1-DLX5-DLX6 (7q21.3) locus contribute to genetic control of coronal nonsyndromic craniosynostosis and bone density-related traits[J]. Genet Med Open, 2024, 2: 101851.
[20] Justice C M, Cuellar A, Bala K, et al. A genome-wide association study implicates the BMP7 locus as a risk factor for nonsyndromic metopic craniosynostosis[J]. Hum Genet, 2020, 139(8): 1077-1090.
[21] Honeycutt J H. Endoscopic-assisted craniosynostosis surgery[J]. Semin Plast Surg, 2014, 28(3): 144-149.
[22] Weinzweig J, Baker S B, Whitaker L A, et al. Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity[J]. Plast Reconstr Surg, 2002, 110(2): 397-408.
[23] Persing J A, Babler W J, Nagorsky M J, et al. Skull expansion in experimental craniosynostosis[J]. Plast Reconstr Surg, 1986, 78(5): 594-603.
[24] Lauritzen C G K, Davis C, Ivarsson A, et al. The evolving role of springs in craniofacial surgery: the first 100 clinical cases[J]. Plast Reconstr Surg, 2008, 121(2): 545-554.
[25] Lee B S, Hwang L S, Doumit G D, et al. Management options of non-syndromic sagittal craniosynostosis[J]. J Clin Neurosci, 2017, 39: 28-34.
[26] Isaac K V, Meara J G, Proctor M R. Analysis of clinical outcomes for treatment of sagittal craniosynostosis: a comparison of endoscopic suturectomy and cranial vault remodeling[J]. J Neurosurg Pediatr, 2018, 22(5): 467-474.
[27] Ghenbot R G, Patel K B, Skolnick G B, et al. Effects of open and endoscopic surgery on skull growth and calvarial vault volumes in sagittal synostosis[J]. J Craniofac Surg, 2015, 26(1): 161-164.
[28] Le M B, Patel K, Skolnick G, et al. Assessing long-term outcomes of open and endoscopic sagittal synostosis reconstruction using three-dimensional photography[J]. J Craniofac Surg, 2014, 25(2): 573-576.
[29] Shah M N, Kane A A, Petersen J D, et al. Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children′s Hospital experience[J]. J Neurosurg Pediatr, 2011, 8(2): 165-170.
[30] Nguyen D C, Farber S J, Skolnick G B, et al. One hundred consecutive endoscopic repairs of sagittal craniosynostosis: an evolution in care[J]. J Neurosurg Pediatr, 2017, 20(5): 410-418.
[31] Massenburg B B, Tolley P D, Lee A, et al. Fronto-orbital advancement for metopic and unilateral coronal craniosynostoses[J]. Oral Maxillofac Surg Clin North Am, 2022, 34(3): 367-380.
[32] Liang Q C, Chen X, Yang B, et al. Bilateral Fronto-orbital advancement combined with cranial vault release using a free-floating bone flap technique for nonsyndromic unilateral coronal synostosis[J]. Transl Pediatr, 2023, 12(12): 2213-2221.
[33] Hansen M, Padwa B L, Scott R M, et al. Synostotic frontal plagiocephaly: anthropometric comparison of three techniques for surgical correction[J]. Plast Reconstr Surg, 1997, 100(6): 1387-1395.
[34] Esparza J, Hinojosa J, García-Recuero I, et al. Surgical treatment of isolated and syndromic craniosynostosis. results and complications in 283 consecutive cases[J]. Neurocirugia (Astur), 2008, 19(6): 509-529.
[35] Fearon J A, Yu J, Bartlett S P, et al. Infections in craniofacial surgery: a combined report of 567 procedures from two centers[J]. Plast Reconstr Surg, 1997, 100(4): 862-868.
[36] Foster K A, Frim D M, McKinnon M. Recurrence of synostosis following surgical repair of craniosynostosis[J]. Plast Reconstr Surg, 2008, 121(3): 70e-76e.
[37] McCarthy J G, Glasberg S B, Cutting C B, et al. Twenty-year experience with early surgery for craniosynostosis: Ⅰ. Isolated craniofacial synostosis: results and unsolved problems[J]. Plast Reconstr Surg, 1995, 96(2): 272-283.
[38] Jubbal K T, Agrawal N, Hollier L H Jr. Analysis of morbidity, readmission, and reoperation after craniosynostosis repair in children[J]. J Craniofac Surg, 2017, 28(2): 401-405.
[39] Branch L G, Crantford C, Cunningham T, et al. Long-term outcomes of pediatric cranial reconstruction using resorbable plating systems for the treatment of craniosynostosis[J]. J Craniofac Surg, 2017, 28(1): 26-29.
[40] Skolnick G B, Murthy S, Patel K B, et al. Long-term characterization of cranial defects after surgical correction for single-suture craniosynostosis[J]. Ann Plast Surg, 2019, 82(6): 679-685.
[41] Einhorn T A. The science of fracture healing[J]. J Orthop Trauma, 2005, 19(10 Suppl): S4-S6.
[42] Chen D, Zhao M, Mundy G R. Bone morphogenetic proteins[J]. Growth Factors, 2004, 22(4): 233-241.
[43] Prevost R, Keribin P, Batut C, et al. Management of non-syndromic craniosynostoses in France in 2015: a national survey[J]. J Craniomaxillofac Surg, 2019, 47(4): 556-560.
[44] Noordzij N, Brouwer R, van der Horst C. Incomplete reossification after craniosynostosis surgery[J]. J Craniofac Surg, 2016, 27(1): e105-e108.
[45] Thenier-Villa J L, Sanromán-álvarez P, Miranda-Lloret P, et al. Incomplete reossification after craniosynostosis surgery-incidence and analysis of risk factors: a clinical-radiological assessment study[J]. J Neurosurg Pediatr, 2018, 22(2): 120-127.
[46] Soldozy S, Ya?murlu K, Akyeampong D K, et al. Three-dimensional printing and craniosynostosis surgery[J]. Childs Nerv Syst, 2021, 37(8): 2487-2495.
[47] Elkhill C, Liu J W, Linguraru M G, et al. Geometric learning and statistical modeling for surgical outcomes evaluation in craniosynostosis using 3D photogrammetry[J]. Comput Methods Programs Biomed, 2023, 240: 107689.
[48] Uhl J F, Sufianov A, Ruiz C, et al. The use of 3D printed models for surgical simulation of cranioplasty in craniosynostosis as training and education[J]. Brain Sci, 2023, 13(6): 894.
[49] da Costa A C, Anderson V A, Holmes A D, et al. Longitudinal study of the neurodevelopmental characteristics of treated and untreated nonsyndromic craniosynostosis in infancy[J]. Childs Nerv Syst, 2013, 29(6): 985-995.
[50] Patel A, Yang J F, Hashim P W, et al. The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis[J]. Plast Reconstr Surg, 2014, 134(4): 608e-617e.
[51] Alperovich M, Runyan C M, Gabrick K S, et al. Long-term neurocognitive outcomes of spring-assisted surgery versus cranial vault remodeling for sagittal synostosis[J]. Plast Reconstr Surg, 2021, 147(3): 661-671.
[52] 蒋文怡, 董晨彬, 吴颖, 等. 颅缝早闭婴幼儿手术治疗前后发育筛查结果分析[J]. 中华小儿外科杂志, 2022, 43(10): 865-868.
  Jiang W Y, Dong C B, Wu Y, et al. Developmental screening results of infants with craniosynostosis before and after operations[J]. Chinses Journal of Pediatric Surgery, 2022, 43(10): 865-868.
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