Severe kyphoscoliosis secondary to neurofibromatosis. Case presentation
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Published:
Nov 18, 2020
Keywords:
Neurofibromatosis, scoliosis, resection, spine
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Abstract
Dystrophic scoliosis in neurofibromatosis is identifiable by being an acute-angle kyphoscoliosis involving a short segment of the spine and producing severe deformity that associated with the dystrophic changes of the spine result in real surgical challenges.We report the clinical case of a 15-year male with severe dystrophic kyphoscoliosis at the thoracolumbar area, with apex at T9, scoliosis with a Cobb angle of 107 °, and segmental kyphosis of 110.7°. The patient underwent a three-stage surgery, performed through a posterior approach, involving a vertebral column resection (VCR) and titanium mesh replacement, and achieving a kyphosis correction of 56% and a scoliosis correction of 59.8%. The patient experienced no major complications nor sequelae and had a favorable course. The VCR is a powerful and demanding surgical technique that allows for the management of the complex kyphoscoliosis deformity to achieve spinal balance; however, it is not without complications, especially neurological and pulmonary complications, which may be unavoidable. Our patient’s quality of life has improved significantly.
Key words: Neurofibromatosis; scoliosis; resection; spineLevel of Evidence: IV
Key words: Neurofibromatosis; scoliosis; resection; spineLevel of Evidence: IV
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How to Cite
Nuñez Alvarado, L. E., Morales Vásquez, E., & Macchiavello Falcon, R. (2020). Severe kyphoscoliosis secondary to neurofibromatosis. Case presentation. Revista De La Asociación Argentina De Ortopedia Y Traumatología, 85(4), 393-402. https://doi.org/10.15417/issn.1852-7434.2020.85.4.1015
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Case Presentations
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References
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comparison between 3-column osteotomy techniques in 105 consecutive spinal deformity procedures. Spine (Phila Pa1976) 2012;37(14):1198-210. https://doi.org/10.1097/BRS.0b013e31824fffde
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the efficacy and safety of posterior-only vertebral column resection (PVCR) for the treatment of angular and
isolated congenital kyphosis. Eur Spine J 2017;26(7):1817-25. https://doi.org/10.1007/s00586-015-4344-9
19. Sacramento-Domínguez C, Yagi M, Ayamga J, Nemani VM, Akoto H, Mahmud R, et al. Apex of deformity for
three column osteotomy. Does it matter in the occurrence of complications? FOCOS Spine Research Group. Spine J 2015;15(11):2351-9. https://doi.org/10.1016/j.spinee.2015.07.010
20. Sheha ED, Kim HJ, Cunningham ME. Vertebral column resection for complex spinal deformity. Semin Spine Surg 2017;29(4):175-83. https://doi.org/10.1053/j.semss.2017.08.002
21. Wang H, Guo J, Wang S, Yang Y, Zhang Y, Qiu G, Zhang J. Instrumentation failure after posterior vertebral column resection in adult spinal deformity. Spine (Phila Pa 1976) 2017;42(7):471-8.
https://doi.org/10.1097/BRS.0000000000001844
22. Sravisht Iyer, Venu M. Nemani, Han Jo Kim. A review of complications and outcomes following vertebral column resection in adults. Asian Spine J 2016;10(3):601-9. https://doi.org/10.4184/asj.2016.10.3.601
23. Climent JM, Bagó J, Rodríguez-Ruiz C, Sánchez-Raya J, Mulet S, Cholbi F. Nueva estrategia para mejorar la
medida de la calidad de vida en la escoliosis idiopática: adición de la dimensión de deformidad percibida al
cuestionario de la Scoliosis Research Society (SRS-22). Rehabilitación 2011;45(3):228-32.
https://doi.org/10.1016/j.rh.2011.04.007
24. Merola AA, Haher TR, Brkaric M, Panagopoulos G, Mathur S, Kohani O, et al. A multicenter study of the outcomes of the surgical treatment of adolescent idiopathic scoliosis using the Scoliosis Research Society (SRS) outcome instrument. Spine (Phila Pa 1976) 2002;27(18):2046-51. https://doi.org/10.1097/00007632-200209150-00015
2. Bersusky E. Deformidades vertebrales por neurofibromatosis. Rev Asoc Argent Ortop Traumatol 1999;64(4):263-9. https://www.aaot.org.ar/revista/1993_2002/1999/1999_4/640401.pdf
3. Coca Pérez A, Heredero JJ, Burgos J, Ferrero A, Aparicio-Meix JM. Neurofibromatosis tipo 1: escoliosis distrófica y tumor paravertebral. Rev Esp Pediatr 2004;60(3):243-5. https://pesquisa.bvsalud.org/portal/resource/pt/ibc-37743
4. Woodrow C, Clarke A, Amirfey R. Neurofibromatosis. Orthop Trauma 2015;29(3):206-10.
https://doi.org/10.1016/j.mporth.2015.02.004
5. Patel N B, Stacy GS. Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol
2012;199(1):W99-106. https://doi.org/10.2214/AJR.11.7811
6. Crawford AH, Herrera-Soto J. Scoliosis associated with neurofibromatosis. Orthop Clin North Am 2007;38(4):553-62. https://doi.org/10.1016/j.ocl.2007.03.008
7. Haher TR, Gorup JM, Shin TM, Homel P, Merola AA, Grogan DP, et al. Results of the Scoliosis Research Society
instrument for evaluation of surgical outcome in adolescent idiopathic scoliosis. Spine 1999;24(14):1435-40.
https://doi.org/10.1097/00007632-199907150-00008
8. Boachie-Adjei O, Yagi M, Sacramento-Dominguez C, Akoto H, Cunningham ME, Gupta M, et al. Surgical
risk stratification based on preoperative risk factors in severe pediatric spinal deformity surgery. Spine Deform
2014;2(5):340-9. https://doi.org/10.1016/j.jspd.2014.05.004
9. Tejeda Barreras M. Esteroides en lesión medular postraumática aguda. Columna 2011;1(2):39-42.
https://www.medigraphic.com/pdfs/columna/col-2011/col112d.pdf
10. Motono N, Kawaguchi M, Kawaharab N, Uramoto H. Case report of surgical treatment of scoliosis caused by
neurofibroma located posterior mediastinum. Int J Surg Case Rep 2018;53:168-70.
https://doi.org/10.1016/j.ijscr.2018.10.071
11. Tsirikos AI, Saifuddin A, Noordeen MH. Spinal deformity in neurofibromatosis type-1: diagnosis and treatment. Eur Spine J 2005;14(5):427-39. https://doi.org/10.1007/s00586-004-0829-7
12. Larson AN, Ledonio CGT, Brearley AM, Sucato DJ, Carreon LY, Crawford AH, et al. Predictive value and
interrater reliability of radiographic factors in neurofibromatosis patients with dystrophic scoliosis. Spine Deform
2018;6(5):560-7. https://doi.org/10.1016/j.jspd.2018.02.011
13. Lenke LG, Newton PO, Sucato DJ, Shufflebarger HL, Emans JB, Sponseller PD, et al. Complications after
147 consecutive vertebral column resections for severe pediatric spinal deformity. Spine (Phila Pa 1976)
2013;38(2):119-32. https://doi.org/10.1097/BRS.0b013e318269fab1
14. Schwab F, Blondel B, Chay E, Demakakos J, Lenke L, Tropiano P, et al. The comprehensive anatomical spinal
osteotomy classification. Neurosurgery 2014;74(1):112-20. https://doi.org/10.1227/01.neu.0000462076.73701.09
15. Uribe JS, Schwab F, Mundis GM, Xu DS, Januszewski J, Kanter AS, et al. The comprehensive anatomical spinal
osteotomy and anterior column realignment classification. J Neurosurg Spine 2018;29(5):565-75.
https://doi.org/10.3171/2018.4.SPINE171206
16. Atici Y, Balioglu MB, Kargin D, Mert M, Albayrak A, Kaygusuz MA. Analysis of complications following posterior
vertebral column resection for the treatment of severe angular kyphosis greater than 100°. Acta Orthop Traumatol Turc 2017;51(3):201-8. https://doi.org/10.1016/j.aott.2017.02.015
17. Auerbach JD, Lenke LG, Bridwell KH, Sehn JK, Milby AH, Bumpass D, et al. Major complications and
comparison between 3-column osteotomy techniques in 105 consecutive spinal deformity procedures. Spine (Phila Pa1976) 2012;37(14):1198-210. https://doi.org/10.1097/BRS.0b013e31824fffde
18. Wang S, Aikenmu K, Zhang J, Qiu G, Guo J, Zhang Y, Weng, X. The aim of this retrospective study is to evaluate
the efficacy and safety of posterior-only vertebral column resection (PVCR) for the treatment of angular and
isolated congenital kyphosis. Eur Spine J 2017;26(7):1817-25. https://doi.org/10.1007/s00586-015-4344-9
19. Sacramento-Domínguez C, Yagi M, Ayamga J, Nemani VM, Akoto H, Mahmud R, et al. Apex of deformity for
three column osteotomy. Does it matter in the occurrence of complications? FOCOS Spine Research Group. Spine J 2015;15(11):2351-9. https://doi.org/10.1016/j.spinee.2015.07.010
20. Sheha ED, Kim HJ, Cunningham ME. Vertebral column resection for complex spinal deformity. Semin Spine Surg 2017;29(4):175-83. https://doi.org/10.1053/j.semss.2017.08.002
21. Wang H, Guo J, Wang S, Yang Y, Zhang Y, Qiu G, Zhang J. Instrumentation failure after posterior vertebral column resection in adult spinal deformity. Spine (Phila Pa 1976) 2017;42(7):471-8.
https://doi.org/10.1097/BRS.0000000000001844
22. Sravisht Iyer, Venu M. Nemani, Han Jo Kim. A review of complications and outcomes following vertebral column resection in adults. Asian Spine J 2016;10(3):601-9. https://doi.org/10.4184/asj.2016.10.3.601
23. Climent JM, Bagó J, Rodríguez-Ruiz C, Sánchez-Raya J, Mulet S, Cholbi F. Nueva estrategia para mejorar la
medida de la calidad de vida en la escoliosis idiopática: adición de la dimensión de deformidad percibida al
cuestionario de la Scoliosis Research Society (SRS-22). Rehabilitación 2011;45(3):228-32.
https://doi.org/10.1016/j.rh.2011.04.007
24. Merola AA, Haher TR, Brkaric M, Panagopoulos G, Mathur S, Kohani O, et al. A multicenter study of the outcomes of the surgical treatment of adolescent idiopathic scoliosis using the Scoliosis Research Society (SRS) outcome instrument. Spine (Phila Pa 1976) 2002;27(18):2046-51. https://doi.org/10.1097/00007632-200209150-00015