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Diagnosis and Treatment of Congenital Scoliosis

Year 2019, Issue: 2, 148 - 155, 01.06.2019

Çağlar Yılgör İrem Nur Eroğlu Nuray Söğünmez Kadir Abul Ahmet Alanay

Abstract

Congenital scoliosis is the most common congenital spinal deformity caused by a failure in the formation or segmentation or both. Clinical manifestations may vary from stable hemivertebra to complex deformities that can lead to pulmonary, cardiac, genitourinary and neurological complications. Other system anomalies may accompany as well. Prenatal ultrasound, physical examination and x-rays are used for diagnosis. MR is helpful in detecting accompanying pathologies. CT may be useful in preoperative planning. The treatment plan is tailored to each patient according to location and magnitude of the curve, patient age, type of the deformity and the anticipated natural history. Treatment consists of controlled observation, conservative and surgical methods. Hemivertebrectomy and limited fusion are recommended at an early age for single hemivertebra. Hemiepiphysiodesis, distraction-based non-fusion methods or combinations of these may be used for more complex deformities. Technological advances today allow for concurrent surgeries for spinal deformity and intradural pathologies. Corrective osteotomies may be required in neglected severe cases. Due to the challenging technique and risk of neurological injury, corrective osteotomies necessitate well-equipped centers and experienced surgeons

Keywords

Spine congenital abnormalities scoliosis kyphosis hemivertebrae

References

  • Arlet V, Odent T, Aebi M. Congenital scoliosis. Eur Spine J 2003;12:456- 63. [CrossRef]
  • Tsirikos AI, McMaster MJ. Congenital anomalies of the ribs and chest wall associated with congenital deformities of the spine. J Bone Joint Surg Am 2005;87:2523-36. [CrossRef]
  • Giampietro PF. Genetic aspects of congenital and idiopathic scoliosis. Scientifica (Cairo) 2012;2012:152365. [CrossRef]
  • Pahys JM, Guille JT. What’s New in Congenital Scoliosis? J Pediatr Orthop 2018;38:e172-e9. [CrossRef] https://doi.org/10.1097/ BPO.0000000000000922.
  • Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J 2005;5:564-76. [CrossRef]
  • Grimme JD, Castillo M. Congenital anomalies of the spine. Neuroimaging Clin N Am 2007;17:1-16. [CrossRef]
  • Hedequist D, Emans J. Congenital scoliosis: a review and update. J Pediatr Orthop 2007;27:106-16. [CrossRef]
  • Li Z, Yu X, Shen J. Environmental aspects of congenital scoliosis. Environ Sci Pollut Res Int 2015;22:5751-5. [CrossRef]
  • Burnei G, Gavriliu S, Vlad C, et al. Congenital scoliosis: an up-to-date. J Med Life 2015;8:388-97.
  • Hedequist DJ, Emans JB. The correlation of preoperative three- dimensional computed tomography reconstructions with operative findings in congenital scoliosis. Spine 2003;28:2531-4; discussion 1. [CrossRef]
  • Tauchi R, Tsuji T, Cahill PJ, et al. Reliability analysis of Cobb angle measurements of congenital scoliosis using X-ray and 3D-CT images. Eur J Orthop Surg Traumatol 2016;26:53-7. [CrossRef]
  • Hedequist D, Emans J. Congenital scoliosis. J Am Acad of Orthop Surg 2004;12:266-75.
  • Jaskwhich D, Ali RM, Patel TC, Green DW. Congenital scoliosis. Curr Opin Pediatr 2000;12:61-6.
  • McMaster MJ, Ohtsuka K. The natural history of congenital scoliosis. A study of two hundred and fifty-one patients. J Bone Joint Surg Am 1982;64:1128-47.
  • Johal J, Loukas M, Fisahn C, Chapman JR, Oskouian RJ, Tubbs RS. Hemivertebrae: a comprehensive review of embryology, imaging, classification, and management. Child’s Nerv Syst 2016;32:2105-9. [CrossRef]
  • Feng Y, Hai Y, Zhao S, Zang L. Hemivertebra resection with posterior unilateral intervertebral fusion and transpedicular fixation for congenital scoliosis: results with at least 3 years of follow-up. Eur Spine J 2016;25:3274-81. [CrossRef]
  • Shawen SB, Belmont PJ, Jr., Kuklo TR, et al. Hemimetameric segmental shift: a case series and review. Spine 2002;27:E539-44. [CrossRef]
  • Basu PS, Elsebaie H, Noordeen MH. Congenital spinal deformity: a comprehensive assessment at presentation. Spine 2002;27:2255-9. [CrossRef]
  • Marks DS, Qaimkhani SA. The natural history of congenital scoliosis and kyphosis. Spine 2009;34:1751-5. [CrossRef]
  • Balioglu MB, Akman YE, Ucpunar H, et al. Sacral agenesis: evaluation of accompanying pathologies in 38 cases, with analysis of long-term outcomes. Child’s Nerv Syst 2016;32:1693-702. [CrossRef]
  • Dubousset J, Herring JA, Shufflebarger H. The crankshaft phenomenon. J Pediatr Orthop 1989;9:541-50.
  • Kesling KL, Lonstein JE, Denis F, et al. The crankshaft phenomenon after posterior spinal arthrodesis for congenital scoliosis: a review of 54 patients. Spine 2003;28:267-71. [CrossRef]
  • Zhuang Q, Zhang J, Li S, Wang S, Guo J, Qiu G. One-stage posterior- only lumbosacral hemivertebra resection with short segmental fusion: a more than 2-year follow-up. Eur Spine J 2016;25:1567-74. [CrossRef]
  • Guo J, Zhang J, Wang S, et al. Surgical outcomes and complications of posterior hemivertebra resection in children younger than 5 years old. J Orthop Surg Res 2016;11:48. [CrossRef]
  • Piantoni L, Francheri Wilson IA, Tello CA, et al. Hemivertebra Resection With Instrumented Fusion by Posterior Approach in Children. Spine Deform 2015;3:541-8. [CrossRef]
  • Hedequist D, Emans J, Proctor M. Three rod technique facilitates hemivertebra wedge excision in young children through a posterior only approach. Spine 2009;34:E225-9. [CrossRef]
  • Ruf M, Harms J. Hemivertebra resection by a posterior approach: innovative operative technique and first results. Spine 2002;27:1116-23.
  • Chang DG, Kim JH, Ha KY, Lee JS, Jang JS, Suk SI. Posterior hemivertebra resection and short segment fusion with pedicle screw fixation for congenital scoliosis in children younger than 10 years: greater than 7-year follow-up. Spine 2015;40:E484-91. [CrossRef]
  • Uzumcugil A, Cil A, Yazici M, et al. The efficacy of convex hemiepiphysiodesis in patients with iatrogenic posterior element deficiency resulting from diastematomyelia excision. Spine 2003;28:799-805.
  • Kieffer J, Dubousset J. Combined anterior and posterior convex epiphysiodesis for progressive congenital scoliosis in children aged < or = 5 years. Eur Spine J 1994;3:120-5.
  • Bekmez S, Demirkiran HG, Yilmaz G, et al. Convex Hemiepiphysiodesis: Posterior/anterior in-situ Versus Posterior-only With Pedicle Screw Instrumentation: An Experimental Simulation in Immature Pigs. J Pediatr Orthop 2016;36:847-52. [CrossRef]
  • Andrew T, Piggott H. Growth arrest for progressive scoliosis. Combined anterior and posterior fusion of the convexity. J Bone Joint Surg Br 1985;67:193-7.
  • Bradford DS. Partial epiphyseal arrest and supplemental fixation for progressive correction of congenital spinal deformity. J Bone Joint Surg Am 1982;64:610-4.
  • Keller PM, Lindseth RE, DeRosa GP. Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report. Spine 1994;19:1933-9.
  • Uzumcugil A, Cil A, Yazici M, et al. Convex growth arrest in the treatment of congenital spinal deformities, revisited. J Pediatr Orthop 2004;24:658-66.
  • Walhout RJ, van Rhijn LW, Pruijs JE. Hemi-epiphysiodesis for unclassified congenital scoliosis: immediate results and mid-term follow-up. Eur Spine J 2002;11:543-9. [CrossRef]
  • Demirkiran G, Dede O, Ayvaz M, Bas CE, Alanay A, Yazici M. Convex Instrumented Hemiepiphysiodesis With Concave Distraction: A Treatment Option for Long Sweeping Congenital Curves. J Pediatr Orthop 2016;36:226-31. [CrossRef]
  • Cheung KM, Zhang JG, Lu DS, K Luk KD, Y Leong JC. Ten-year follow- up study of lower thoracic hemivertebrae treated by convex fusion and concave distraction. Spine 2002;27:748-53.
  • Alanay A, Dede O, Yazici M. Convex instrumented hemiepiphysiodesis with concave distraction: a preliminary report. Clin Orthop Relat Res 2012;470:1144-50. [CrossRef]
  • Elsebai HB, Yazici M, Thompson GH, et al. Safety and efficacy of growing rod technique for pediatric congenital spinal deformities. J Pediatr Orthop 2011;31:1-5. [CrossRef]
  • Yazici M, Emans J. Fusionless instrumentation systems for congenital scoliosis: expandable spinal rods and vertical expandable prosthetic titanium rib in the management of congenital spine deformities in the growing child. Spine 2009;34:1800-7. [CrossRef]
  • Emans JB, Caubet JF, Ordonez CL, Lee EY, Ciarlo M. The treatment of spine and chest wall deformities with fused ribs by expansion thoracostomy and insertion of vertical expandable prosthetic titanium rib: growth of thoracic spine and improvement of lung volumes. Spine 2005;30:S58-68.
  • Campbell RM Jr., Smith MD, Mayes TC, et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am 2003;85-A:399-408.
  • Ozturk C, Alanay A, Ganiyusufoglu K, Karadereler S, Ulusoy L, Hamzaoglu A. Short-term X-ray results of posterior vertebral column resection in severe congenital kyphosis, scoliosis, and kyphoscoliosis. Spine 2012;37:1054-7. [CrossRef]
  • Lenke LG, O’Leary PT, Bridwell KH, Sides BA, Koester LA, Blanke KM. Posterior vertebral column resection for severe pediatric deformity: minimum two-year follow-up of thirty-five consecutive patients. Spine 2009;34:2213-21. [CrossRef]
  • Larson AN, Polly DW Jr., Guidera KJ, et al. The accuracy of navigation and 3D image-guided placement for the placement of pedicle screws in congenital spine deformity. J Pediatr Orthop 2012;32:e23- 9. [CrossRef]
  • Yilgor C, Sogunmez N, Dalla M, et al. Eş Zamanlı İntradural Spinal Patoloji ve Pediatrik Spinal Deformite Cerrahisi. Türk Nöroşirürji Derneği 31 Bilimsel Kongresi; 2017; Antalya.
  • Weiss HR, Goodall D. Rate of complications in scoliosis surgery - a systematic review of the Pub Med literature. Scoliosis 2008;3:9. [CrossRef]
  • Ruf M, Jensen R, Letko L, Harms J. Hemivertebra resection and osteotomies in congenital spine deformity. Spine 2009;34:1791-9. [CrossRef]

Konjenital Skolyoz Tanı ve Tedavisi

Year 2019, Issue: 2, 148 - 155, 01.06.2019

Çağlar Yılgör İrem Nur Eroğlu Nuray Söğünmez Kadir Abul Ahmet Alanay

Abstract

Omurganın en sık görülen konjenital deformitesi konjenital skolyozdur. Deformiteyi oluşturan sebep, oluşum veya ayrışma kusuru veya bu iki bozukluğun birlikte olduğu karma tiptir. Kliniğe yansıması, stabil hemivertebradan, pulmoner, kardiyak, genitoüriner ve nörolojik komplikasyonlara yol açabilen karmaşık ve ilerleyici deformitelere kadar geniş bir aralıktadır. Tabloya sıklıkla diğer organ sistemlerindeki anomaliler eşlik eder. Tanıda; prenatal ultrason, fizik muayene ve röntgen kullanılır. Manyetik rezonans eşlik eden intradural patolojilerin ortaya konulmasında yardımcıdır. Bilgisayarlı tomografi cerrahi planlamada yardımcı olabilir. Tedavi planı eğriliğin yeri ve derecesi, hastanın yaşı, deformitenin tipine ve öngörülen ilerleme beklentisine göre her hastaya özel olarak yapılır. Tedavi temel olarak kontrollü gözlem, konservatif ve cerrahi tedavilerden oluşur. Tek hemivertebra varlığında genellikle erken yaşta hemivertebrektomi ve limitli füzyon önerilir. Daha karmaşık deformitelerde hemiepifizyodez, distraksiyon temelli füzyonsuz cerrahi yöntemler veya bunların kombinasyonları kullanılabilir. Günümüzde teknolojik ilerlemeler sayesinde spinal deformite ve intradural patolojilerin cerrahisi eş zamanlı olarak güvenli şekilde yapılabilmektedir. İhmal edilmiş ileri deformitelerde düzeltici osteotomiler gerekebilir. Düzeltici osteotomiler barındırdıkları nörolojik yaralanma riski ve uygulamadaki teknik zorluklar nedeniyle donanımlı merkezlerde deneyimli cerrahlar tarafından gerçekleştirilmelidir

Keywords

Omurga doğumsal anomaliler skolyoz kifoz hemivertebra

References

  • Arlet V, Odent T, Aebi M. Congenital scoliosis. Eur Spine J 2003;12:456- 63. [CrossRef]
  • Tsirikos AI, McMaster MJ. Congenital anomalies of the ribs and chest wall associated with congenital deformities of the spine. J Bone Joint Surg Am 2005;87:2523-36. [CrossRef]
  • Giampietro PF. Genetic aspects of congenital and idiopathic scoliosis. Scientifica (Cairo) 2012;2012:152365. [CrossRef]
  • Pahys JM, Guille JT. What’s New in Congenital Scoliosis? J Pediatr Orthop 2018;38:e172-e9. [CrossRef] https://doi.org/10.1097/ BPO.0000000000000922.
  • Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J 2005;5:564-76. [CrossRef]
  • Grimme JD, Castillo M. Congenital anomalies of the spine. Neuroimaging Clin N Am 2007;17:1-16. [CrossRef]
  • Hedequist D, Emans J. Congenital scoliosis: a review and update. J Pediatr Orthop 2007;27:106-16. [CrossRef]
  • Li Z, Yu X, Shen J. Environmental aspects of congenital scoliosis. Environ Sci Pollut Res Int 2015;22:5751-5. [CrossRef]
  • Burnei G, Gavriliu S, Vlad C, et al. Congenital scoliosis: an up-to-date. J Med Life 2015;8:388-97.
  • Hedequist DJ, Emans JB. The correlation of preoperative three- dimensional computed tomography reconstructions with operative findings in congenital scoliosis. Spine 2003;28:2531-4; discussion 1. [CrossRef]
  • Tauchi R, Tsuji T, Cahill PJ, et al. Reliability analysis of Cobb angle measurements of congenital scoliosis using X-ray and 3D-CT images. Eur J Orthop Surg Traumatol 2016;26:53-7. [CrossRef]
  • Hedequist D, Emans J. Congenital scoliosis. J Am Acad of Orthop Surg 2004;12:266-75.
  • Jaskwhich D, Ali RM, Patel TC, Green DW. Congenital scoliosis. Curr Opin Pediatr 2000;12:61-6.
  • McMaster MJ, Ohtsuka K. The natural history of congenital scoliosis. A study of two hundred and fifty-one patients. J Bone Joint Surg Am 1982;64:1128-47.
  • Johal J, Loukas M, Fisahn C, Chapman JR, Oskouian RJ, Tubbs RS. Hemivertebrae: a comprehensive review of embryology, imaging, classification, and management. Child’s Nerv Syst 2016;32:2105-9. [CrossRef]
  • Feng Y, Hai Y, Zhao S, Zang L. Hemivertebra resection with posterior unilateral intervertebral fusion and transpedicular fixation for congenital scoliosis: results with at least 3 years of follow-up. Eur Spine J 2016;25:3274-81. [CrossRef]
  • Shawen SB, Belmont PJ, Jr., Kuklo TR, et al. Hemimetameric segmental shift: a case series and review. Spine 2002;27:E539-44. [CrossRef]
  • Basu PS, Elsebaie H, Noordeen MH. Congenital spinal deformity: a comprehensive assessment at presentation. Spine 2002;27:2255-9. [CrossRef]
  • Marks DS, Qaimkhani SA. The natural history of congenital scoliosis and kyphosis. Spine 2009;34:1751-5. [CrossRef]
  • Balioglu MB, Akman YE, Ucpunar H, et al. Sacral agenesis: evaluation of accompanying pathologies in 38 cases, with analysis of long-term outcomes. Child’s Nerv Syst 2016;32:1693-702. [CrossRef]
  • Dubousset J, Herring JA, Shufflebarger H. The crankshaft phenomenon. J Pediatr Orthop 1989;9:541-50.
  • Kesling KL, Lonstein JE, Denis F, et al. The crankshaft phenomenon after posterior spinal arthrodesis for congenital scoliosis: a review of 54 patients. Spine 2003;28:267-71. [CrossRef]
  • Zhuang Q, Zhang J, Li S, Wang S, Guo J, Qiu G. One-stage posterior- only lumbosacral hemivertebra resection with short segmental fusion: a more than 2-year follow-up. Eur Spine J 2016;25:1567-74. [CrossRef]
  • Guo J, Zhang J, Wang S, et al. Surgical outcomes and complications of posterior hemivertebra resection in children younger than 5 years old. J Orthop Surg Res 2016;11:48. [CrossRef]
  • Piantoni L, Francheri Wilson IA, Tello CA, et al. Hemivertebra Resection With Instrumented Fusion by Posterior Approach in Children. Spine Deform 2015;3:541-8. [CrossRef]
  • Hedequist D, Emans J, Proctor M. Three rod technique facilitates hemivertebra wedge excision in young children through a posterior only approach. Spine 2009;34:E225-9. [CrossRef]
  • Ruf M, Harms J. Hemivertebra resection by a posterior approach: innovative operative technique and first results. Spine 2002;27:1116-23.
  • Chang DG, Kim JH, Ha KY, Lee JS, Jang JS, Suk SI. Posterior hemivertebra resection and short segment fusion with pedicle screw fixation for congenital scoliosis in children younger than 10 years: greater than 7-year follow-up. Spine 2015;40:E484-91. [CrossRef]
  • Uzumcugil A, Cil A, Yazici M, et al. The efficacy of convex hemiepiphysiodesis in patients with iatrogenic posterior element deficiency resulting from diastematomyelia excision. Spine 2003;28:799-805.
  • Kieffer J, Dubousset J. Combined anterior and posterior convex epiphysiodesis for progressive congenital scoliosis in children aged < or = 5 years. Eur Spine J 1994;3:120-5.
  • Bekmez S, Demirkiran HG, Yilmaz G, et al. Convex Hemiepiphysiodesis: Posterior/anterior in-situ Versus Posterior-only With Pedicle Screw Instrumentation: An Experimental Simulation in Immature Pigs. J Pediatr Orthop 2016;36:847-52. [CrossRef]
  • Andrew T, Piggott H. Growth arrest for progressive scoliosis. Combined anterior and posterior fusion of the convexity. J Bone Joint Surg Br 1985;67:193-7.
  • Bradford DS. Partial epiphyseal arrest and supplemental fixation for progressive correction of congenital spinal deformity. J Bone Joint Surg Am 1982;64:610-4.
  • Keller PM, Lindseth RE, DeRosa GP. Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report. Spine 1994;19:1933-9.
  • Uzumcugil A, Cil A, Yazici M, et al. Convex growth arrest in the treatment of congenital spinal deformities, revisited. J Pediatr Orthop 2004;24:658-66.
  • Walhout RJ, van Rhijn LW, Pruijs JE. Hemi-epiphysiodesis for unclassified congenital scoliosis: immediate results and mid-term follow-up. Eur Spine J 2002;11:543-9. [CrossRef]
  • Demirkiran G, Dede O, Ayvaz M, Bas CE, Alanay A, Yazici M. Convex Instrumented Hemiepiphysiodesis With Concave Distraction: A Treatment Option for Long Sweeping Congenital Curves. J Pediatr Orthop 2016;36:226-31. [CrossRef]
  • Cheung KM, Zhang JG, Lu DS, K Luk KD, Y Leong JC. Ten-year follow- up study of lower thoracic hemivertebrae treated by convex fusion and concave distraction. Spine 2002;27:748-53.
  • Alanay A, Dede O, Yazici M. Convex instrumented hemiepiphysiodesis with concave distraction: a preliminary report. Clin Orthop Relat Res 2012;470:1144-50. [CrossRef]
  • Elsebai HB, Yazici M, Thompson GH, et al. Safety and efficacy of growing rod technique for pediatric congenital spinal deformities. J Pediatr Orthop 2011;31:1-5. [CrossRef]
  • Yazici M, Emans J. Fusionless instrumentation systems for congenital scoliosis: expandable spinal rods and vertical expandable prosthetic titanium rib in the management of congenital spine deformities in the growing child. Spine 2009;34:1800-7. [CrossRef]
  • Emans JB, Caubet JF, Ordonez CL, Lee EY, Ciarlo M. The treatment of spine and chest wall deformities with fused ribs by expansion thoracostomy and insertion of vertical expandable prosthetic titanium rib: growth of thoracic spine and improvement of lung volumes. Spine 2005;30:S58-68.
  • Campbell RM Jr., Smith MD, Mayes TC, et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am 2003;85-A:399-408.
  • Ozturk C, Alanay A, Ganiyusufoglu K, Karadereler S, Ulusoy L, Hamzaoglu A. Short-term X-ray results of posterior vertebral column resection in severe congenital kyphosis, scoliosis, and kyphoscoliosis. Spine 2012;37:1054-7. [CrossRef]
  • Lenke LG, O’Leary PT, Bridwell KH, Sides BA, Koester LA, Blanke KM. Posterior vertebral column resection for severe pediatric deformity: minimum two-year follow-up of thirty-five consecutive patients. Spine 2009;34:2213-21. [CrossRef]
  • Larson AN, Polly DW Jr., Guidera KJ, et al. The accuracy of navigation and 3D image-guided placement for the placement of pedicle screws in congenital spine deformity. J Pediatr Orthop 2012;32:e23- 9. [CrossRef]
  • Yilgor C, Sogunmez N, Dalla M, et al. Eş Zamanlı İntradural Spinal Patoloji ve Pediatrik Spinal Deformite Cerrahisi. Türk Nöroşirürji Derneği 31 Bilimsel Kongresi; 2017; Antalya.
  • Weiss HR, Goodall D. Rate of complications in scoliosis surgery - a systematic review of the Pub Med literature. Scoliosis 2008;3:9. [CrossRef]
  • Ruf M, Jensen R, Letko L, Harms J. Hemivertebra resection and osteotomies in congenital spine deformity. Spine 2009;34:1791-9. [CrossRef]
There are 49 citations in total.

Details

Primary Language Turkish
Journal Section Collection
Authors

Çağlar Yılgör

İrem Nur Eroğlu

Nuray Söğünmez

Kadir Abul

Ahmet Alanay

Publication Date June 1, 2019
Published in Issue Year 2019Issue: 2

Cite

EndNote Yılgör Ç, Eroğlu İN, Söğünmez N, Abul K, Alanay A (June 1, 2019) Konjenital Skolyoz Tanı ve Tedavisi. Acıbadem Üniversitesi Sağlık Bilimleri Dergisi 2 148–155.