Abstract
Study design
Case report.
Objectives
To describe the first reported use of corrective surgery combined with C7 vertebral column resection (VCR) to treat an extremely rare case of severe, rigid cervical kyphotic deformity associated with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.
Background data
Spinal lesions reportedly occur in 32–45% in SAPHO syndrome. However, bone and joint lesions are usually repaired such that severe joint destruction is rare. Therefore, there have been few reported cases of surgical treatment for spinal lesions.
Methods
A 22-year-old woman had been diagnosed with cervical kyphotic deformity associated with SAPHO syndrome. She had difficulty looking upward. On radiography, the C4–C7 vertebral bodies were fused and exhibited severe rigid kyphotic deformity. Right convex scoliosis with a Cobb angle of 22° was apparent at C5–T2, and the C2–C7 angle of kyphosis was 75°. Corrective three-stage surgery was carried out from the anterior, posterior, and anterior, with C7 VCR.
Results
The C2–T1 angle improved to 21° and the patient was capable of looking up. At present, 2 years postoperatively, complete bony fusion has been achieved. Her cervical spine function and quality of life were markedly improved.
Conclusion
Only a very limited number of patients have a deformity sufficiently severe that VCR of cervical vertebra is required, but as this is the technique that provides the greatest multiplanar alignment correction, its choice in the present case was appropriate.
Level of evidence
IV.
References
Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME (2012) The SAPHO syndrome. Semin Arthritis Rheum 42(3):254–265. https://doi.org/10.1016/j.semarthrit.2012.05.006
Hayem G, Bouchaud-Chabot A, Benali K, Roux S, Palazzo E, Silbermann-Hoffman O, Kahn MF, Meyer O (1999) SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum 29(3):159–171. https://doi.org/10.1016/s0049-0172(99)80027-4
Li C, Zuo Y, Wu N, Li L, Li F, Zhang W, Xu W, Zhao X, Jing H, Pan Q, Zhou W, Shi X, Fan Y, Wang J, Liu S, Liu Z, Zhang F, Zeng X, Chen H, Zhang S, Liu J, Qiu G, Wu Z, Dong Z, Zhang W (2016) Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome: a single centre study of a cohort of 164 patients. Rheumatology (Oxford) 55(6):1023–1030. https://doi.org/10.1093/rheumatology/kew015
Toussirot E, Dupond JL, Wendling D (1997) Spondylodiscitis in SAPHO syndrome. A series of eight cases. Ann Rheumatic Dis 56(1):52–58. https://doi.org/10.1136/ard.56.1.52
Harifi G, Belkhou A, El Bouchti I, Ouali Idrissi M, Cherif Idrissi N, Ousehal A, El Hassani S (2012) C1–C2 spondylodiscitis in an adult with SAPHO syndrome: an unusual presentation. Rheumatol Int 32(2):445–447. https://doi.org/10.1007/s00296-009-1274-z
Mateo L, Sanint J, Rodríguez Muguruza S, Martínez Morillo M, Pérez Andrés R, Domenech Puigcerver S (2017) SAPHO syndrome presenting as an osteolytic lesion of the neck. Reumatología Clínica (English Edition) 13(1):44–47. https://doi.org/10.1016/j.reumae.2015.11.022
Miyamoto M, Shirai Y, Nakayama Y, Gembun Y, Kitagawa Y, Nanbu A (2000) A case report of synovitis, acne, pustulosis, hyperostosis and osteitis syndrome presenting with spondylodiscitis. Journal of Nippon Medical School = Nippon Ika Daigaku zasshi 67(3):191–197. https://doi.org/10.1272/jnms.67.191
Takigawa T, Tanaka M, Nakanishi K, Misawa H, Sugimoto Y, Takahata T, Nakahara H, Nakahara S, Ozaki T (2008) SAPHO syndrome associated spondylitis. Eur Spine J 17(10):1391–1397. https://doi.org/10.1007/s00586-008-0722-x
Nakamae T, Yamada K, Tsuchida Y, Adachi N, Fujimoto Y (2019) Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome with destructive spondylitis: a case report. Spine Surg Relat Res 3(3):267–269. https://doi.org/10.22603/ssrr.2018-0035
Nakamura J, Yamada K, Mitsugi N, Saito T (2010) A case of SAPHO syndrome with destructive spondylodiscitis suspicious of tuberculous spondylitis. Mod Rheumatol 20(1):93–97. https://doi.org/10.1007/s10165-009-0234-5
Takigawa T, Tanaka M, Nakahara S, Sugimoto Y, Ozaki T (2008) SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically. Eur Spine J 17(Suppl 2):S331–337. https://doi.org/10.1007/s00586-008-0663-4
Ames CP, Smith JS, Scheer JK, Shaffrey CI, Lafage V, Deviren V, Moal B, Protopsaltis T, Mummaneni PV, Mundis GM Jr, Hostin R, Klineberg E, Burton DC, Hart R, Bess S, Schwab FJ (2013) A standardized nomenclature for cervical spine soft-tissue release and osteotomy for deformity correction: clinical article. J Neurosurg Spine 19(3):269–278. https://doi.org/10.3171/2013.5.spine121067
Fukui M, Chiba K, Kawakami M, Kikuchi S, Konno S, Miyamoto M, Seichi A, Shimamura T, Shirado O, Taguchi T, Takahashi K, Takeshita K, Tani T, Toyama Y, Wada E, Yonenobu K, Tanaka T, Hirota Y, Subcommittee on Low Back P, Cervical Myelopathy Evaluation of the Clinical Outcome Committee of the Japanese Orthopaedic A (2007) An outcome measure for patients with cervical myelopathy: Japanese Orthopaedic Association Cervical Myelopathy Evaluation Questionnaire (JOACMEQ): Part 1. J Orthop Sci 12(3):227–240. https://doi.org/10.1007/s00776-007-1118-1
Miura K, Kadone H, Koda M, Kumagai H, Nagashima K, Fujii K, Noguchi H, Funayama T, Abe T, Furuya T, Yamazaki M (2018) Three-dimensional gait analysis reveals dynamic alignment change in a patient with dropped head syndrome: a case report. J Clin Neurosci 48:106–108. https://doi.org/10.1016/j.jocn.2017.10.075
Sheha ED, Gandhi SD, Colman MW (2019) 3D printing in spine surgery. Ann Transl Med 7(Suppl 5):S164. https://doi.org/10.21037/atm.2019.08.88
Yamazaki M, Okawa A, Akazawa T, Koda M (2007) Usefulness of 3-dimensional full-scale modeling for preoperative simulation of surgery in a patient with old unilateral cervical fracture-dislocation. Spine (Phila Pa 1976) 32 (18):E532–536. https://doi.org/10.1097/BRS.0b013e318133fcdf
Yamazaki M, Okawa A, Kadota R, Mannoji C, Miyashita T, Koda M (2009) Surgical simulation of circumferential osteotomy and correction of cervico-thoracic kyphoscoliosis for an irreducible old C6–C7 fracture dislocation. Acta Neurochir (Wien) 151(7):867–872. https://doi.org/10.1007/s00701-009-0388-0
Funayama T, Noguchi H, Kumagai H, Yoshioka T, Yamazaki M (2020) A novel unidirectional porous beta-tricalcium phosphate bone substitute in orthopedic surgery: a technical note and case illustrations. Cureus 12(3):e7465. https://doi.org/10.7759/cureus.7465
Noguchi H, Funayama T, Koda M, Iijima Y, Kumagai H, Ishikawa T, Aiba A, Abe T, Nagashima K, Miura K, Izawa S, Maki S, Furuya T, Yamazaki M (2019) A unidirectional porous beta-tricalcium phosphate material (Affinos(R)) for reconstruction of bony defects after excision of fibular bone for spinal surgery graft. J Clin Neurosci 66:71–76. https://doi.org/10.1016/j.jocn.2019.05.021
Kubota S, Abe T, Koda M, Kadone H, Shimizu Y, Mataki Y, Noguchi H, Fujii K, Marushima A, Funayama T, Kawamoto H, Hada Y, Sankai Y, Yamazaki M (2018) Application of a newly developed upper limb single-joint hybrid assistive limb for postoperative C5 paralysis: an initial case report indicating its safety and feasibility. J Clin Neurosci 50:268–271. https://doi.org/10.1016/j.jocn.2018.01.038
Nassr A, Khan MH, Ali MH, Espiritu MT, Hanks SE, Lee JY, Donaldson WF, Kang JD (2009) Donor-site complications of autogenous nonvascularized fibula strut graft harvest for anterior cervical corpectomy and fusion surgery: experience with 163 consecutive cases. Spine J 9(11):893–898. https://doi.org/10.1016/j.spinee.2009.04.020
Windom RE, Sanford JP, Ziff M (1961) Acne conglobata and arthritis. Arthritis Rheum 4:632–635. https://doi.org/10.1002/art.1780040609
Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A (1987) Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases. Revue du rhumatisme et des maladies osteo-articulaires 54 (3):187–196
Sasaki T (1967) A case with osteomyelitis of the bilateral clavicles associated with pustulosis palmaris et plantaris. Rinsho Seikeigeka 2:333–337
Sonozaki H, Mitsui H, Miyanaga Y, Okitsu K, Igarashi M, Hayashi Y, Matsuura M, Azuma A, Okai K, Kawashima M (1981) Clinical features of 53 cases with pustulotic arthro-osteitis. Ann Rheum Dis 40(6):547–553. https://doi.org/10.1136/ard.40.6.547
Benhamou CL, Chamot AM, Kahn MF (1988) Synovitis-acne-pustulosis hyperostosis-osteomyelitis syndrome (SAPHO). A new syndrome among the spondyloarthropathies? Clinical and experimental rheumatology 6 (2):109–112
Tan LA, Riew KD, Traynelis VC (2017) Cervical spine deformity-part 1: biomechanics, radiographic parameters, and classification. Neurosurgery 81(2):197–203. https://doi.org/10.1093/neuros/nyx249
Safaee MM, Tan LA, Riew KD (2020) Anterior osteotomy for rigid cervical deformity correction. J Spine Surg 6(1):210–216. https://doi.org/10.21037/jss.2019.12.10
Tan LA, Riew KD, Traynelis VC (2017) Cervical spine deformity-part 2: management algorithm and anterior techniques. Neurosurgery 81(4):561–567. https://doi.org/10.1093/neuros/nyx388
Tan LA, Riew KD, Traynelis VC (2017) Cervical spine deformity-part 3: posterior techniques, clinical outcome, and complications. Neurosurgery 81(6):893–898. https://doi.org/10.1093/neuros/nyx477
Tobin MK, Birk DM, Rangwala SD, Siemionow K, Schizas C, Neckrysh S (2017) T-1 pedicle subtraction osteotomy for the treatment of rigid cervical kyphotic deformity: report of 4 cases. J Neurosurg Spine 27(5):487–493. https://doi.org/10.3171/2016.8.SPINE121065
Wollowick AL, Kelly MP, Riew KD (2012) Pedicle subtraction osteotomy in the cervical spine. Spine (Phila Pa 1976) 37 (5):E342–E348. https://doi.org/10.1097/BRS.0b013e318245bcd4
Han K, Lu C, Li J, Xiong GZ, Wang B, Lv GH, Deng YW (2011) Surgical treatment of cervical kyphosis. Eur Spine J 20(4):523–536. https://doi.org/10.1007/s00586-010-1602-8
Nottmeier EW, Deen HG, Patel N, Birch B (2009) Cervical kyphotic deformity correction using 360-degree reconstruction. J Spinal Disord Tech 22(6):385–391. https://doi.org/10.1097/BSD.0b013e318180e672
Kim HJ, Piyaskulkaew C, Riew KD (2015) Comparison of Smith-Petersen osteotomy versus pedicle subtraction osteotomy versus anterior–posterior osteotomy types for the correction of cervical spine deformities. Spine (Phila Pa 1976) 40 (3):143–146. https://doi.org/10.1097/BRS.0000000000000707
Diebo BG, Shah NV, Solow M, Challier V, Paulino CB, Passias PG, Lafage R, Schwab FJ, Kim HJ, Lafage V (2018) Adult cervical deformity: radiographic and osteotomy classifications. Orthopade 47(6):496–504. https://doi.org/10.1007/s00132-018-3581-0
Dru AB, Lockney DT, Vaziri S, Decker M, Polifka AJ, Fox WC, Hoh DJ (2019) Cervical spine deformity correction techniques. Neurospine 16(3):470–482. https://doi.org/10.14245/ns.1938288.144
Garg B, Mehta N, Vatsya P (2020) Surgical strategy for correction of severe, rigid, post-tubercular cervical kyphosis: an experience of two cases. Spine Deform. https://doi.org/10.1007/s43390-020-00104-6
Hoh DJ, Khoueir P, Wang MY (2008) Management of cervical deformity in ankylosing spondylitis. Neurosurg Focus 24(1):E9. https://doi.org/10.3171/foc/2008/24/1/e9
Hart RA, Tatsumi RL, Hiratzka JR, Yoo JU (2008) Perioperative complications of combined anterior and posterior cervical decompression and fusion crossing the cervico-thoracic junction. Spine (Phila Pa 1976) 33(26):2887–2891. https://doi.org/10.1097/BRS.0b013e318190affe
Smith JS, Ramchandran S, Lafage V, Shaffrey CI, Ailon T, Klineberg E, Protopsaltis T, Schwab FJ, O'Brien M, Hostin R, Gupta M, Mundis G, Hart R, Kim HJ, Passias PG, Scheer JK, Deviren V, Burton DC, Eastlack R, Bess S, Albert TJ, Riew KD, Ames CP, International Spine Study G (2016) Prospective multicenter assessment of early complication rates associated with adult cervical deformity surgery in 78 patients. Neurosurgery 79(3):378–388. https://doi.org/10.1227/NEU.0000000000001129
Wewel JT, Brahimaj BC, Kasliwal MK, Traynelis VC (2019) Perioperative complications with multilevel anterior and posterior cervical decompression and fusion. J Neurosurg Spine 1–6. https://doi.org/10.3171/2019.6.SPINE198
Ogihara S, Kunogi J (2015) Single-stage anterior and posterior fusion surgery for correction of cervical kyphotic deformity using intervertebral cages and cervical lateral mass screws: postoperative changes in total spine sagittal alignment in three cases with a minimum follow-up of five years. Neurol Med Chir (Tokyo) 55(7):599–604. https://doi.org/10.2176/nmc.cr.2014-0263
Passias PG, Horn SR, Oh C, Lafage R, Lafage V, Smith JS, Line B, Protopsaltis TS, Yagi M, Bortz CA, Segreto FA, Alas H, Diebo BG, Sciubba DM, Kelly MP, Daniels AH, Klineberg EO, Burton DC, Hart RA, Schwab FJ, Bess S, Shaffrey CI, Ames CP (2020) Predicting the occurrence of postoperative distal junctional kyphosis in cervical deformity patients. Neurosurgery 86(1):E38–E46. https://doi.org/10.1093/neuros/nyz347
Passias PG, Vasquez-Montes D, Poorman GW, Protopsaltis T, Horn SR, Bortz CA, Segreto F, Diebo B, Ames C, Smith J, LaFage V, LaFage R, Klineberg E, Shaffrey C, Bess S, Schwab F, Issg (2018) Predictive model for distal junctional kyphosis after cervical deformity surgery. Spine J 18(12):2187–2194. https://doi.org/10.1016/j.spinee.2018.04.017
Acknowledgements
We thank Shinsuke Sakai for making 3D-printed actual-size model, and Shigeki Kubota and Yukiyo Shimizu for HAL rehabilitation.
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Made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data; or the creation of new software used in the work: TF, TA, HN, KM, KM, HT, MK, and MY. Drafted the work or revised it critically for important intellectual content: TF, TA, HN, KM, KM, HT, MK, and MY. Approved the version to be published: TF, TA, HN, KM, KM, HT, MK, and MY. Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: TF, TA, HN, KM, KM, HT, MK, and MY.
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Toru Funayama and Masashi Yamazaki received the research grant from Kuraray, Co. Ltd. The other authors report no conflict of interest.
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Exempted from ethics committee approval due to a case report.
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Funayama, T., Abe, T., Noguchi, H. et al. Severe, rigid cervical kyphotic deformity associated with SAPHO syndrome successfully treated with three-stage correction surgery combined with C7 vertebral column resection: a technical case report. Spine Deform 9, 285–292 (2021). https://doi.org/10.1007/s43390-020-00203-4
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DOI: https://doi.org/10.1007/s43390-020-00203-4