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Congenital scoliosis

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Abstract

Congenital scoliosis is the most frequent congenital deformity of the spine. Congenital curvatures are due to anomalous development of the vertebrae (failure of formation and/or segmentation). Congenital scoliosis is believed to be related to an insult to the fetus during spine embryological development, and associated malformations (heart, spinal cord, kidney...) are frequently observed. A perfect understanding of the natural history of the deformity and the treatment principles will allow best management of these complex spine deformities. New imaging techniques like three-dimensional computed tomography (CT) and magnetic resonance imaging (MRI) are important tools for analyzing the underlying deformity and understanding the evolution of the complex deformities. The mainstay of treatment is either observation or, in case of curve progression (>10°/year), surgery. Different surgeries are described with two main principles: (1) prophylactic surgeries like hemiepiphysiodesis or in situ fusions that will prevent worsening or allow progressive correction over time, and (2) corrective surgeries, with spinal fusion with or without spinal resection. Exceptional procedures (e.g. spinal column resection or halo distraction) can be attempted in cases of very severe deformity. Congenital curves must be carefully observed to choose the least invasive procedure at the right time and to minimize spinal cord risks.

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References

  1. Arlet V, Papin P, Marchesi D (1999) Halo femoral traction and sliding rods in the treatment of a neurologically compromised congenital scoliosis: technique. Eur Spine J 8:329–331

    Article  CAS  PubMed  Google Scholar 

  2. Beals R, Robbins JR, Rolfe B (1993) Anomalies associated with vertebral malformations. Spine 18:1329–1332

    CAS  PubMed  Google Scholar 

  3. Benacerraf BR, Greene MF, Barss VA (1986) Prenatal sonographic diagnosis of congenital hemivertebra. J Ultrasound Med 5:257–259

    CAS  PubMed  Google Scholar 

  4. Bradford DS, Boachie-Adjei O (1990) One-stage anterior and posterior hemivertebral resection and arthrodesis for congenital scoliosis. J Bone Joint Surg Am 72:536–540

    CAS  PubMed  Google Scholar 

  5. Bradford DS, Tribus CB (1997) Vertebral column resection for the treatment of rigid coronal decompensation. Spine 22:1590

    Article  CAS  PubMed  Google Scholar 

  6. Bradford DS, Heithoff KB, Cohen M (1991) Intraspinal abnormalities and congenital spine deformities: a radiographic and MRI study. J Pediatr Orthop 11:36–41

    CAS  PubMed  Google Scholar 

  7. Callahan BC, Georgopoulos G, Eilert RE (1997) Hemivertebral excision for congenital scoliosis. J Pediatr Orthop J 17:96–99

    Article  CAS  Google Scholar 

  8. Campbell RM, Vocke AK (2001) Growth of the thoracic spine in congenital scoliosis after expansion thoracoplasty. Scoliosis Research Society 36th annual meeting. Cleveland, Ohio

  9. Cheung KM, Zhang JG, Lu DS, K Luk KD, Y Leong JC (2002) Ten-year follow-up study of lower thoracic hemivertebrae treated by convex fusion and concave distraction. Spine 27:748–753

    Article  PubMed  Google Scholar 

  10. Chirpaz-Cerbat JM, Michel F, Berard J, Onimus M, Michel CR (1993) Early and semi-early surgery for scoliosis caused by hemivertebrae—indications and results. Eur J Pediatr Surg 3:144–153

    CAS  PubMed  Google Scholar 

  11. Dubousset J, Katti E, Seringe R (1993) Epiphysiodesis of the spine in young children for congenital spinal deformations. J Pediatr Orthop Part B 1–292:123–130

  12. Hefti F (2002) Congenital anomalies of the spine. Orthopade 31:34–43

    Article  CAS  PubMed  Google Scholar 

  13. Holte DC, Winter RB, Lonstein JE, Denis F (1995) Excision of hemivertebrae and wedge resection in the treatment of congenital scoliosis. J Bone Joint Surg Am 77:159–171

    CAS  PubMed  Google Scholar 

  14. Keller PM, Lindseth RE, DeRosa GP (1994) Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report. Spine 19:1933–1939

    Google Scholar 

  15. Kieffer J, Dubousset J (1994) Combined anterior and posterior convex epiphysiodesis for progressive congenital scoliosis in children aged ≤5 years. Eur Spine J 3:120–125

    CAS  PubMed  Google Scholar 

  16. Klemme WR, Polly DW Jr, Orchowski JR (2001) Hemivertebral excision for congenital scoliosis in very young children. J Pediatr Orthop 21:761–764

    Article  CAS  PubMed  Google Scholar 

  17. Lazar RD, Hall JE (1999) Simultaneous anterior and posterior hemivertebra excision. Clin Orthop 364:76–84

    PubMed  Google Scholar 

  18. Leatherman, KD, Dickson RA (1978) Two stage corrective surgery for congenital deformity of the spine. J Bone Joint Surg Br 61:324–328

    Google Scholar 

  19. Loder RT, Urquhart A, Steen H, Graziano G, Hensinger RN, Schlesinger A, Schork MA, Shyr Y (1995) Variability in Cobb angle measurements in children with congenital scoliosis. J Bone Joint Surg Br 77:768–770

    CAS  PubMed  Google Scholar 

  20. MacEwen GD, Winter RB, Hardy JH (1982) Evaluation of kidney anomalies in congenital scoliosis. J Bone Joint Surg Am 54:1451–1454

    Google Scholar 

  21. McMaster MJ (1998) Congenital scoliosis caused by a unilateral failure of vertebral segmentation with contralateral hemivertebrae. Spine 23:998–1005

    Article  CAS  PubMed  Google Scholar 

  22. McMaster MJ, Ohtsuka K (1982) The natural history of congenital scoliosis. A study of two hundred and fifty one patients. J Bone Joint Surg Am 64:1128–1147

    CAS  PubMed  Google Scholar 

  23. McMaster MJ, Singh H (2001) The surgical management of congenital kyphosis and kyphoscoliosis. Spine 26:2146–2154

    Article  CAS  PubMed  Google Scholar 

  24. Nakamura H, Matsuda H, Konishi S, Yamano Y (2002) Single-stage excision of hemivertebrae via the posterior approach alone for congenital spine deformity: follow-up period longer than ten years. Spine 27:110–115

    Article  PubMed  Google Scholar 

  25. Rothenberg S, Erickson M, Eilert R, Fitzpatrick J, Chang F, Glancy G, Georgopoulus G, Brown C (1998) Thoracoscopic anterior spinal procedures in children. J Pediatr Surg 33:1168–1170

    CAS  PubMed  Google Scholar 

  26. Ruf M, Harms J (2002) Hemivertebra resection by a posterior approach: innovative operative technique and first results. Spine 27:1116–1123

    Article  PubMed  Google Scholar 

  27. Shono Y, Abumi K, Kaneda K (2001) One-stage posterior hemivertebra resection and correction using segmental posterior instrumentation. Spine 1 26:752–757

    Article  CAS  Google Scholar 

  28. Sink EL, Karol LA, Sanders J, Birch JG, Johnston CE, Herring JA (2001) Efficacy of perioperative halo-gravity traction in the treatment of severe scoliosis in children. J Pediatr Orthop 21:519–524

    Article  CAS  PubMed  Google Scholar 

  29. Sturm PF, Chung R, Bomze SR (2001) Hemivertebra in monozygotic twins. Spine 26:1389–1391

    Article  CAS  PubMed  Google Scholar 

  30. Thompson AG, Marks DS, Sayampanathan SR, Piggott H (1995) Long-term results of combined anterior and posterior convex epiphysiodesis for congenital scoliosis due to hemivertebrae. Spine 20:1380–1385

    Google Scholar 

  31. Winter RB, Moe, JH Eilers VE (1968) Congenital scoliosis. A study of 234 patients treated and untreated. 1. Natural history. J Bone Joint Surg Am 50:1–15

    Google Scholar 

  32. Winter RB, Moe JH, Lonstein JE (1983) A review of family histories in patients with congenital spine deformities. Orthop Trans 7:32

    Google Scholar 

  33. Winter RB, Moe JH, Lonstein JE (1984) Posterior arthrodesis for congenital scoliosis. An analysis of the cases of two hundred and ninety patients, five to nineteen years old. J Bone Joint Surg Am 66:1188–1197

    CAS  PubMed  Google Scholar 

  34. Winter RB, Lonstein JE, Denis F, Sta-Ana de la Rosa H (1988) Convex growth arrest for progressive congenital scoliosis due to hemivertebrae. J Pediatr Orthop 8:633–638

    CAS  PubMed  Google Scholar 

  35. Winter RB, Lonstein JE, Boachie-Adjei O (1996) Congenital spine deformity. J Bone Joint Surg Am 78:300–311

    Google Scholar 

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Arlet, V., Odent, T. & Aebi, M. Congenital scoliosis. Eur Spine J 12, 456–463 (2003). https://doi.org/10.1007/s00586-003-0555-6

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