Abstract
Purpose
To evaluate clinical and radiographic outcomes after surgical scoliosis correction and posterior instrumented fusion in SMF patients.
Methods
A single-center medical database was reviewed to identify MF patients who presented with scoliosis from 2000 to 2015. Patients who underwent spinal fusion surgery were included. Demographic, operative and clinical data were reviewed, and the preoperative, postoperative, and latest follow-up radiographic parameters were compared.
Results
Twelve patients were identified (2 males, 10 females) with an average age at surgery of 14.4 ± 2.6 years. Comorbidities were found in 84.6%. Most patients (90.9%) presented with a right thoracic curve. The average preoperative Cobb angle was 75.6 ± 15.5 degrees. Posterior instrumented spinal fusion was performed in all patients (1 hook/pedicular screw and 11 pedicle screws only). The average follow-up period was 6.8 ± 3.1 years. The mean postoperative Cobb angle after surgery and at the final follow-up was 33.4 ± 18.0 degrees and 35.5 ± 18.4 degrees, respectively. There was a statistically significant difference among the preoperative and postoperative Cobb angles (p < 0.001), but no significant difference among the sagittal angles. Two perioperative complications including superficial wound infection and broken rods were observed.
Conclusions
Posterior scoliosis correction and instrumented spinal fusion resulted in a satisfactory outcome in MF patients. Perioperative complications are not uncommon; however, no neurological complication or spinal decompensation was observed in this study.
Level of evidence
IV.
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Abbreviations
- MF:
-
Marfan syndrome
- SMF:
-
Marfan patients with scoliosis
- AP:
-
Anteroposterior
References
Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, Puffenberger EG, Hamosh A, Nanthakumar EJ, Curristin SM et al (1991) Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature 352:337–339. https://doi.org/10.1038/352337a0
Tsipouras P, Del Mastro R, Sarfarazi M, Lee B, Vitale E, Child AH, Godfrey M, Devereux RB, Hewett D, Steinmann B et al (1992) Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. The International Marfan Syndrome Collaborative Study. N Engl J Med 326:905–909. https://doi.org/10.1056/NEJM199204023261401
Jones KB, Myers L, Judge DP, Kirby PA, Dietz HC, Sponseller PD (2005) Toward an understanding of dural ectasia: a light microscopy study in a murine model of Marfan syndrome. Spine 30:291–293. https://doi.org/10.1097/01.brs.0000152166.88174.1c ((Phila Pa 1976))
Pyeritz RE, McKusick VA (1979) The Marfan syndrome: diagnosis and management. N Engl J Med 300:772–777. https://doi.org/10.1056/NEJM197904053001406
De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE (1996) Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 62:417–426. https://doi.org/10.1002/(SICI)1096-8628(19960424)62:4%3c417::AID-AJMG15%3e3.0.CO;2-R
Sponseller PD, Hobbs W, Riley LH 3rd, Pyeritz RE (1995) The thoracolumbar spine in Marfan syndrome. J Bone Joint Surg Am 77:867–876. https://doi.org/10.2106/00004623-199506000-00007
Joseph KN, Kane HA, Milner RS, Steg NL, Williamson MB Jr, Bowen JR (1992) Orthopedic aspects of the Marfan phenotype. Clin Orthop Relat Res. https://doi.org/10.1097/00003086-199204000-00031
Taylor LJ (1987) Severe spondylolisthesis and scoliosis in association with Marfan’s syndrome. Case report and review of the literature. Clin Orthop Relat Res 221:207–211
Savini R, Cervellati S, Beroaldo E (1980) Spinal deformities in Marfan’s syndrome. Ital J Orthop Traumatol 6:19–40
Glard Y, Launay F, Edgard-Rosa G, Collignon P, Jouve JL, Bollini G (2008) Scoliotic curve patterns in patients with Marfan syndrome. J Child Orthop 2:211–216. https://doi.org/10.1007/s11832-008-0095-z
Birch JG, Herring JA (1987) Spinal deformity in Marfan syndrome. J Pediatr Orthop 7:546–552. https://doi.org/10.1097/01241398-198709000-00009
Sponseller PD, Bhimani M, Solacoff D, Dormans JP (2000) Results of brace treatment of scoliosis in Marfan syndrome. Spine 25:2350–2354. https://doi.org/10.1097/00007632-200009150-00013 ((Phila Pa 1976))
Robins PR, Moe JH, Winter RB (1975) Scoliosis in Marfan’s syndrome. Its characteristics and results of treatment in thirty-five patients. J Bone Joint Surg Am 57:358–368
von Kodolitsch Y, Kutsche K (2015) Interpretation of sequence variants of the FBN1 gene: analog or digital? A commentary on the decreased frequency of FBN1 missense variants in Ghent criteria-positive Marfan syndrome and characterization of novel FBN1 variants. J Hum Genet 60:465–466. https://doi.org/10.1038/jhg.2015.95
Beighton P, Solomon L, Soskolne CL (1973) Articular mobility in an African population. Ann Rheum Dis 32:413–418. https://doi.org/10.1136/ard.32.5.413
Lenke LG, Betz RR, Harms J, Bridwell KH, Clements DH, Lowe TG, Blanke K (2001) Adolescent idiopathic scoliosis: a new classification to determine the extent of spinal arthrodesis. J Bone Joint Surg Am 83:1169–1181
Child AH (2017) Non-cardiac manifestations of Marfan syndrome. Anna cardiothorac surg 6:599–609. https://doi.org/10.21037/acs.2017.10.02
Sponseller PD, Sethi N, Cameron DE, Pyeritz RE (1997) Infantile scoliosis in Marfan syndrome. Spine 22:509–516. https://doi.org/10.1097/00007632-199703010-00008 ((Phila Pa 1976))
Demetracopoulos CA, Sponseller PD (2007) Spinal deformities in Marfan syndrome. Orthop Clin North Am 38:563–572. https://doi.org/10.1016/j.ocl.2007.04.003
Wang B, Cao XB, Qiu Y, Qian BP, Sun X, Huang AB, Zhu ZZ, Yu Y, Zhu F, Ma WW (2010) Pulmonary dysfunction patterns in patients with Marfan and Marfanoid syndrome associated with scoliosis and the influencing factors. Zhonghua Wai Ke Za Zhi 48:686–689
Gjolaj JP, Sponseller PD, Shah SA, Newton PO, Flynn JM, Neubauer PR, Marks MC, Bastrom TP (2012) Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: learning from the differences. Spine 37:1558–1565. https://doi.org/10.1097/BRS.0b013e3182541af3 ((Phila Pa 1976))
Shirley ED, Sponseller PD (2009) Marfan syndrome. J Am Acad Orthop Surg 17:572–581. https://doi.org/10.5435/00124635-200909000-00004
Liang W, Yu B, Wang Y, Li Z, Qiu G, Shen J, Zhang J (2015) Comparison of posterior correction results between Marfan syndrome scoliosis and adolescent idiopathic scoliosis-a retrospective case-series study. J Orthop Surg Res 10:73. https://doi.org/10.1186/s13018-015-0210-z
Nelson JD (1958) The Marfan syndrome, with special reference to congenital enlargement of the spinal canal. Br J Radiol 31:561–564. https://doi.org/10.1259/0007-1285-31-370-561
Lipton GE, Guille JT, Kumar SJ (2002) Surgical treatment of scoliosis in Marfan syndrome: guidelines for a successful outcome. J Pediatr Orthop 22:302–307
Li ZC, Liu ZD, Dai LY (2011) Surgical treatment of scoliosis associated with Marfan syndrome by using posterior-only instrumentation. J Pediatr Orthop B 20:63–66. https://doi.org/10.1097/BPB.0b013e328341bcc9
Kurucan E, Bernstein DN, Ying M, Li Y, Menga EN, Sponseller PD, Mesfin A (2019) Trends in spinal deformity surgery in Marfan syndrome. Spine J 19:1934–1940. https://doi.org/10.1016/j.spinee.2019.08.005
Qiao J, Xu L, Liu Z, Zhu F, Qian B, Sun X, Zhu Z, Qiu Y, Jiang Q (2016) Surgical treatment of scoliosis in Marfan syndrome: outcomes and complications. Eur Spine J 25:3288–3293. https://doi.org/10.1007/s00586-016-4579-0
Palmisani M, Dema E, Rava A, Palmisani R, Girardo M, Cervellati S (2019) Surgical treatment of spinal deformities in Marfan syndrome: Long-term follow-up results using different instrumentations. J Craniovertebr Junction Spine 10:172–178. https://doi.org/10.4103/jcvjs.JCVJS_68_19
Suk SI, Kim JH, Cho KJ, Kim SS, Lee JJ, Han YT (2007) Is anterior release necessary in severe scoliosis treated by posterior segmental pedicle screw fixation? Eur Spine J 16:1359–1365. https://doi.org/10.1007/s00586-007-0334-x
Jones KB, Erkula G, Sponseller PD, Dormans JP (2002) Spine deformity correction in Marfan syndrome. Spine 27:2003–2012. https://doi.org/10.1097/00007632-200209150-00008 ((Phila Pa 1976))
Pourtaheri S, Shah SA, Ditro CP, Holmes L Jr, Mackenzie WG (2016) Preoperative halo-gravity traction with and without thoracoscopic anterior release for skeletal dysplasia patients with severe kyphoscoliosis. J Child Orthop 10:135–142. https://doi.org/10.1007/s11832-016-0721-0
Rinella AS, Lenke LG, Whitaker C, Kim Y, Park S-S, Peelle MW, Edwards CC, Bridwell KH (2005) Perioperative halo-gravity traction in the treatment of severe scoliosis and kyphosis. Spine 30:475–482
Sponseller PD, Takenaga RK, Newton P, Boachie O, Flynn J, Letko L, Betz R, Bridwell K, Gupta M, Marks M, Bastrom T (2008) The use of traction in the treatment of severe spinal deformity. Spine 33:2305–2309. https://doi.org/10.1097/BRS.0b013e318184ef79 ((Phila Pa 1976))
Suk SI, Kim JH, Kim WJ, Lee SM, Chung ER, Nah KH (2002) Posterior vertebral column resection for severe spinal deformities. Spine 27:2374–2382. https://doi.org/10.1097/00007632-200211010-00012 ((Phila Pa 1976))
Di Silvestre M, Bakaloudis G, Lolli F, Vommaro F, Martikos K, Parisini P (2008) Posterior fusion only for thoracic adolescent idiopathic scoliosis of more than 80 degrees: pedicle screws versus hybrid instrumentation. Eur Spine J 17:1336–1349. https://doi.org/10.1007/s00586-008-0731-9
Liu S, Zhang N, Song Y, Song Z, Zhang L, Liu J, Xie E, Wu Q, Hao D (2017) Radiologic comparison of posterior release, internal distraction, final PSO and spinal fusion with one-stage posterior vertebral column resection for multi-level severe congenital scoliosis. BMC Musculoskelet Disord 18:270. https://doi.org/10.1186/s12891-017-1627-9
Mirzashahi B, Moosavi M, Rostami M (2020) outcome of posterior-only approach for severe rigid scoliosis: a retrospective report. Int J Spine Surg 14:232–238. https://doi.org/10.14444/7032
Zenner J, Hitzl W, Meier O, Auffarth A, Koller H (2014) Surgical outcomes of scoliosis surgery in Marfan syndrome. J Spinal Disord Tech 27:48–58. https://doi.org/10.1097/BSD.0b013e31824de6f1
Acknowledgements
The authors gratefully acknowledge the staff of the Siriraj Orthopedic Research Unit, and Mr. Pongsathorn Samphaotong for his assistance with statistical analysis, manuscript preparation, and journal submission process.
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CC: Design and drafting of the work. Revising it critically for important intellectual content. Final approval of the version to be published. PV: Developed and verified the analytical methods. Final approval of the version to be published. TA: Design and drafting of the work. Final approval of the version to be published. MR: Developed and verified the analytical methods. Final approval of the version to be published. SW: Design and drafting of the work. Revising it critically for important intellectual content. Final approval of the version to be published. AC: Developed and verified the analytical methods. Final approval of the version to be published. ST: Revising it critically for important intellectual content. Final approval of the version to be published. BS: Revising it critically for important intellectual content. Writing the original draft of manuscript. Final approval of the version to be published. NA: Developed and verified the formal analysis. Administrative, technical or material support. Writing-review & editing manuscript. Final approval of the version to be published. PL: Design and drafting of the work. Develop and verified the analytical methods. Writing the original draft of manuscript. Final approval of the version to be published.
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Chotigavanichaya, C., Vatidvarodom, P., Ariyawatkul, T. et al. Surgical outcome of scoliosis in patients with Marfan syndrome. Spine Deform 10, 1453–1460 (2022). https://doi.org/10.1007/s43390-022-00547-z
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DOI: https://doi.org/10.1007/s43390-022-00547-z