Skip to main content

Pathology of the Child’s Cervical Spine and Its Clinical Implications

  • Chapter
  • First Online:
The Management of Disorders of the Child’s Cervical Spine

Abstract

Immature cervical vertebrae and their surrounding joints provide little in the way of bony support in the neck of a child; thus, the integrity of the pediatric cervical spine relies heavily on the soft tissues that surround it. This chapter discusses conditions that are associated with cervical spine pathology due to weak connective tissues, including syndromes of Marfan, Loeys-Dietz, Ehlers-Danlos, and Down. Later we touch on bone dysplasias, inflammatory conditions and infections.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Institutional subscriptions

References

  1. Pyeritz RE, McKusick VA. The Marfan syndrome: diagnosis and management. N Engl J Med. 1979;300(14):772–7.

    Article  CAS  PubMed  Google Scholar 

  2. Parrish JG. Heritable disorders of connective tissue. Proc R Soc Med. 1960;53:515.

    Google Scholar 

  3. Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991;352(6333):337–9.

    Article  CAS  PubMed  Google Scholar 

  4. Dietz HC, Pyeritz RE. Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders. Hum Mol Genet. 1995;4 Spec No:1799–809.

    Article  CAS  PubMed  Google Scholar 

  5. Eldadah ZA, Grifo JA, Dietz HC. Marfan syndrome as a paradigm for transcript-targeted preimplantation diagnosis of heterozygous mutations. Nat Med. 1995;1(8):798–803.

    Article  CAS  PubMed  Google Scholar 

  6. McKusick VA. Heritable disorders of connective tissue. 4th ed. St. Louis: CV Mosby; 1972.

    Google Scholar 

  7. Pereira L, Andrikopoulos K, Tian J, Lee SY, Keene DR, Ono R, et al. Targetting of the gene encoding fibrillin-1 recapitulates the vascular aspect of Marfan syndrome. Nat Genet. 1997;17(2):218–22.

    Article  CAS  PubMed  Google Scholar 

  8. Neptune ER, Frischmeyer PA, Arking DE, Myers L, Bunton TE, Gayraud B, et al. Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome. Nat Genet. 2003;33(3):407–11.

    Article  CAS  PubMed  Google Scholar 

  9. Robins PR, Moe JH, Winter RB. Scoliosis in Marfan’s syndrome. Its characteristics and results of treatment in thirty-five patients. J Bone Joint Surg Am. 1975;57(3):358–68.

    Article  CAS  PubMed  Google Scholar 

  10. Sponseller PD, Hobbs W, Riley LH 3rd, Pyeritz RE. The thoracolumbar spine in Marfan syndrome. J Bone Joint Surg Am. 1995;77(6):867–76.

    Article  CAS  PubMed  Google Scholar 

  11. Yetman AT, Bornemeier RA, McCrindle BW. Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death? J Am Coll Cardiol. 2003;41(2):329–32.

    Article  PubMed  Google Scholar 

  12. Keech MK, Wendt VE, Read RC, Bistue AR, Bianchi FA. Family studies of the Marfan syndrome. J Chronic Dis. 1966;19(1):57–83.

    Article  CAS  PubMed  Google Scholar 

  13. Steinberg I. A simple screening test for the Marfan syndrome. Am J Roentgenol Radium Therapy, Nucl Med. 1966;97(1):118–24.

    Article  CAS  Google Scholar 

  14. Rose PS, Levy HP, Ahn NU, Sponseller PD, Magyari T, Davis J, et al. A comparison of the Berlin and Ghent nosologies and the influence of dural ectasia in the diagnosis of Marfan syndrome. Genet Med. 2000;2(5):278–82.

    Article  CAS  PubMed  Google Scholar 

  15. Jones KB, Erkula G, Sponseller PD, Dormans JP. Spine deformity correction in Marfan syndrome. Spine (Phila Pa 1976). 2002;27(18):2003–12.

    Article  Google Scholar 

  16. Place HM, Enzenauer RJ. Cervical spine subluxation in Marfan syndrome. A case report. J Bone Joint Surg Am. 2006;88(11):2479–82.

    Article  PubMed  Google Scholar 

  17. Dickman CA, Kalani MY. Resolution of cervical syringomyelia after transoral odontoidectomy and occipitocervical fusion in a patient with basilar invagination and Type I Chiari malformation. J Clin Neurosci. 2012;19(12):1726–8.

    Article  PubMed  Google Scholar 

  18. Hobbs WR, Sponseller PD, Weiss AP, Pyeritz RE. The cervical spine in Marfan syndrome. Spine (Phila Pa 1976). 1997;22(9):983–9.

    Article  CAS  Google Scholar 

  19. Herzka A, Sponseller PD, Pyeritz RE. Atlantoaxial rotatory subluxation in patients with Marfan syndrome. A report of three cases. Spine (Phila Pa 1976). 2000;25(4):524–6.

    Article  CAS  Google Scholar 

  20. Yang JS, Sponseller PD. Severe cervical kyphosis complicating halo traction in a patient with Marfan syndrome. Spine (Phila Pa 1976). 2009;34(1):E66–9.

    Article  Google Scholar 

  21. MacKenzie JM, Rankin R. Sudden death due to atlantoaxial subluxation in Marfan syndrome. Am J Forensic Med Pathol. 2003;24(4):369–70.

    Article  PubMed  Google Scholar 

  22. Campbell RM Jr. Spine deformities in rare congenital syndromes: clinical issues. Spine (Phila Pa 1976). 2009;34(17):1815–27.

    Article  Google Scholar 

  23. Loeys BL, Chen J, Neptune ER, Judge DP, Podowski M, Holm T, et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37(3):275–81.

    Article  CAS  PubMed  Google Scholar 

  24. Arslan-Kirchner M, Epplen JT, Faivre L, Jondeau G, Schmidtke J, De Paepe A, et al. Clinical utility gene card for: Loeys-Dietz syndrome (TGFBR1/2) and related phenotypes. Eur J Hum Genet. 2011;19(10).

    Google Scholar 

  25. Loeys BL, Schwarze U, Holm T, Callewaert BL, Thomas GH, Pannu H, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355(8):788–98.

    Article  CAS  PubMed  Google Scholar 

  26. Attias D, Stheneur C, Roy C, Collod-Béroud G, Detaint D, Faivre L, et al. Comparison of clinical presentations and outcomes between patients with TGFBR2 and FBN1 mutations in Marfan syndrome and related disorders. Circulation. 2009;120(25):2541–9.

    Article  CAS  PubMed  Google Scholar 

  27. Hughes BD, Powers CJ, Zomorodi AR. Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report. Neurosurgery. 2011;69(3):E746–55; discussion E55.

    Article  PubMed  Google Scholar 

  28. Gath A. Parental reactions to loss and disappointment: the diagnosis of Down’s syndrome. Dev Med Child Neurol. 1985;27(3):392–400.

    Article  CAS  PubMed  Google Scholar 

  29. Caird MS, Wills BP, Dormans JP. Down syndrome in children: the role of the orthopaedic surgeon. J Am Acad Orthop Surg. 2006;14(11):610–9.

    Article  PubMed  Google Scholar 

  30. Pizzutillo PD, Herman MJ. Cervical spine issues in Down syndrome. J Pediatr Orthop. 2005;25(2):253–9.

    Article  PubMed  Google Scholar 

  31. Browd SR, McIntyre JS, Brockmeyer D. Failed age-dependent maturation of the occipital condyle in patients with congenital occipitoatlantal instability and Down syndrome: a preliminary analysis. J Neurosurg Pediatr. 2008;2(5):359–64.

    Article  PubMed  Google Scholar 

  32. Matsunaga S, Imakiire T, Koga H, Ishidou Y, Sasaki H, Taketomi E, et al. Occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome. J Neurosurg. 2007;107(6 Suppl):457–9.

    PubMed  Google Scholar 

  33. Al-Habib A, AlAqeel A. C2/3 instability: unusual cause of cervical myelopathy in a child with Down syndrome. Childs Nerv Syst. 2013;29(1):163–5.

    Article  PubMed  Google Scholar 

  34. Tassone JC, Duey-Holtz A. Spine concerns in the Special Olympian with Down syndrome. Sports Med Arthrosc Rev. 2008;16(1):55–60.

    Article  PubMed  Google Scholar 

  35. Hedequist D, Bekelis K, Emans J, Proctor MR. Single stage reduction and stabilization of basilar invagination after failed prior fusion surgery in children with Down’s syndrome. Spine (Phila Pa 1976). 2010;35(4):E128–33.

    Article  Google Scholar 

  36. Hopwood JJ, Morris CP. The mucopolysaccharidoses. Diagnosis, molecular genetics and treatment. Mol Biol Med. 1990;7(5):381–404.

    CAS  PubMed  Google Scholar 

  37. Leroux S, Muller JB, Boutaric E, Busnel A, Lemouel F, Andro-Garçon M, et al. Hurler syndrome: early diagnosis and treatment. Arch Pediatr. 2014;21(5):501–506. French.

    Article  CAS  PubMed  Google Scholar 

  38. Jin WD, Jackson CE, Desnick RJ, Schuchman EH. Mucopolysaccharidosis type VI: identification of three mutations in the arylsulfatase B gene of patients with the severe and mild phenotypes provides molecular evidence for genetic heterogeneity. Am J Hum Genet. 1992;50(4):795–800.

    CAS  PubMed  PubMed Central  Google Scholar 

  39. Scriver CR, Sly WS. The metabolic and molecular basis of inherited disease. New York: McGraw-Hill; 2008.

    Google Scholar 

  40. Haddad FS, Jones DH, Vellodi A, Kane N, Pitt MC. Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses. J Bone Joint Surg Br. 1997;79(4):576–82.

    Article  CAS  PubMed  Google Scholar 

  41. Odunusi E, Peters C, Krivit W, Ogilvie J. Genu valgum deformity in Hurler syndrome after hematopoietic stem cell transplantation: correction by surgical intervention. J Pediatr Orthop. 1999;19(2):270–4.

    Article  CAS  PubMed  Google Scholar 

  42. Kopits SE. Orthopedic complications of dwarfism. Clin Orthop Relat Res. 1976;114:153–79.

    Google Scholar 

  43. White KK, Karol LA, White DR, Hale S. Musculoskeletal manifestations of Sanfilippo syndrome (mucopolysaccharidosis type III). J Pediatr Orthop. 2011;31(5):594–8.

    Article  PubMed  Google Scholar 

  44. Thorne JA, Javadpour M, Hughes DG, Wraith E, Cowie RA. Craniovertebral abnormalities in Type VI mucopolysaccharidosis (Maroteaux-Lamy syndrome). Neurosurgery. 2001;48(4):849–52; discussion 852–3.

    CAS  PubMed  Google Scholar 

  45. Beck M, Arn P, Giugliani R, Muenzer J, Okuyama T, Taylor J, et al. The natural history of MPS I: global perspectives from the MPS I Registry. Genet Med. 2014;16(10):759–65.

    Article  PubMed  PubMed Central  Google Scholar 

  46. White KK, Sousa T. Mucopolysaccharide disorders in orthopaedic surgery. J Am Acad Orthop Surg. 2013;21(1):12–22.

    Article  PubMed  Google Scholar 

  47. Houten JK, Kinon MG, Goodrich JT. Morquio’s syndrome and craniocervical instability. Pediatr Neurosurg. 2011;47(3):238–40.

    Article  PubMed  Google Scholar 

  48. Solanki GA, et al. Spinal involvement in mucopolysaccharidosis IVA (Morquio-Brailsford or Morquio A syndrome): presentation, diagnosis and management. J Inherit Metab Dis. 2013;36(2):339–55.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. White KK, Steinman S, Mubarak SJ. Cervical stenosis and spastic quadriparesis in Morquio disease (MPS IV). A case report with twenty-six-year follow-up. J Bone Joint Surg Am. 2009;91(2):438–42.

    Article  PubMed  Google Scholar 

  50. Solanki GA, Alden TD, Burton BK, Giugliani R, Horovitz DD, Jones SA, et al. A multinational, multidisciplinary consensus for the diagnosis and management of spinal cord compression among patients with mucopolysaccharidosis VI. Mol Genet Metab. 2012;107(1–2):15–24.

    Article  CAS  PubMed  Google Scholar 

  51. Souillet G, Guffon N, Maire I, Pujol M, Taylor P, Sevin F, et al. Outcome of 27 patients with Hurler’s syndrome transplanted from either related or unrelated haematopoietic stem cell sources. Bone Marrow Transplant. 2003;31(12):1105–17.

    Article  CAS  PubMed  Google Scholar 

  52. Noh H, Lee JI. Current and potential therapeutic strategies for mucopolysaccharidoses. J Clin Pharm Ther. 2014;39(3):215–24.

    Article  CAS  PubMed  Google Scholar 

  53. Illsinger S, et al. Scheie syndrome: enzyme replacement therapy does not prevent progression of cervical myelopathy due to spinal cord compression. J Inherit Metab Dis. 2009;32(Suppl 1):S321–5.

    Article  PubMed  Google Scholar 

  54. Kirkpatrick K, Ellwood J, Walker RW. Mucopolysaccharidosis type I (Hurler syndrome) and anesthesia: the impact of bone marrow transplantation, enzyme replacement therapy, and fiberoptic intubation on airway management. Paediatr Anaesth. 2012;22(8):745–51.

    Article  PubMed  Google Scholar 

  55. Vougioukas VI, Berlis A, Kopp MV, Korinthenberg R, Spreer J, van Velthoven V. Neurosurgical interventions in children with Maroteaux-Lamy syndrome. Case report and review of the literature. Pediatr Neurosurg. 2001;35(1):35–8.

    Article  CAS  PubMed  Google Scholar 

  56. Khan SA, Sehat K, Calthorpe D. Cervical cord compression in an elderly patient with Hurler’s syndrome: a case report. Spine (Phila Pa 1976). 2003;28(16):E313–5.

    Article  Google Scholar 

  57. Baratela WA, Bober MB, Thacker MM, Belthur MV, Oto M, Rogers KJ, et al. Cervicothoracic myelopathy in children with Morquio syndrome a: a report of 4 cases. J Pediatr Orthop. 2014;34(2):223–8.

    Article  PubMed  Google Scholar 

  58. Dede O, Thacker MM, Rogers KJ, Oto M, Belthur MV, Baratela W, et al. Upper cervical fusion in children with Morquio syndrome: intermediate to long-term results. J Bone Joint Surg Am. 2013;95(13):1228–34.

    Article  PubMed  Google Scholar 

  59. Dickerman RD, Colle KO, Bruno CA Jr, Schneider SJ. Craniovertebral instability with spinal cord compression in a 17-month-old boy with Sly syndrome (mucopolysaccharidosis type VII): a surgical dilemma. Spine (Phila Pa 1976). 2004;29(5):E92–4.

    Article  Google Scholar 

  60. Riccardi VM. Neurofibromatosis: past, present, and future. N Engl J Med. 1991;324(18):1283–5.

    Article  CAS  PubMed  Google Scholar 

  61. Cooper J, Giancotti FG. Molecular insights into NF2/Merlin tumor suppressor function. FEBS Lett. 2014;588(16):2743–52.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Alman AB, Goldberg MJ. Syndromes of orthopaedic importance. In: Morrissy WS, editor. Lovell and Winter’s pediatric orthopaedics. Philadelphia: Lippincott Williams & Wilkins; 2006. p. 261–7.

    Google Scholar 

  63. Akbarnia BA, Gabriel KR, Beckman E, Chalk D. Prevalence of scoliosis in neurofibromatosis. Spine (Phila Pa 1976). 1992;17(8 Suppl):S244–8.

    Article  CAS  Google Scholar 

  64. Wu F, Zhang L, Liu Z, Sun Y, Li F, Wang S, Wei F. Cervical neurofibromatosis with tetraplegia: management by cervical suspensory traction. Spine (Phila Pa 1976). 2012;37(14):E858–62.

    Article  Google Scholar 

  65. Vitale MG, Guha A, Skaggs DL. Orthopaedic manifestations of neurofibromatosis in children: an update. Clin Orthop Relat Res. 2002;401:107–18.

    Article  Google Scholar 

  66. Plotkin SR, O’Donnell CC, Curry WT, Bove CM, MacCollin M, Nunes FP. Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 2011;14(4):543–7.

    Article  PubMed  Google Scholar 

  67. Taleb FS, Guha A, Arnold PM, Fehlings MG, Massicotte EM. Surgical management of cervical spine manifestations of neurofibromatosis Type 1: long-term clinical and radiological follow-up in 22 cases. J Neurosurg Spine 2011;14(3):356–366.

    Article  PubMed  Google Scholar 

  68. Miyakoshi N, Hongo M, Kasukawa Y, Misawa A, Shimada Y. Bilateral and symmetric C1-C2 dumbbell ganglioneuromas associated with neurofibromatosis type 1 causing severe spinal cord compression. Spine J. 2010;10(4):e11–5.

    Article  PubMed  Google Scholar 

  69. Garg S, Hosalkar H, Dormans JP. Quadriplegia in a 10-year-old boy due to multiple cervical neurofibromas. Spine (Phila Pa 1976). 2003;28(17):E339–43.

    Article  Google Scholar 

  70. Ma J, Wu Z, Yang X, Xiao J. Surgical treatment of severe cervical dystrophic kyphosis due to neurofibromatosis Type 1: a review of 8 cases. J Neurosurg Spine 2011;14(1):93–98.

    Article  PubMed  Google Scholar 

  71. Yoshihara H, Abumi K, Ito M, Kotani Y, Sudo H, Takahata M. Severe fixed cervical kyphosis treated with circumferential osteotomy and pedicle screw fixation using an anterior-posterior-anterior surgical sequence. World Neurosurg. 2013;80(5):654.e17–21.

    Article  Google Scholar 

  72. Kawabata S, Watanabe K, Hosogane N, Ishii K, Nakamura M, Toyama Y, et al. Surgical correction of severe cervical kyphosis in patients with neurofibromatosis Type 1. J Neurosurg Spine. 2013;18(3):274–9.

    Article  PubMed  Google Scholar 

  73. Inoue M, Sairyo K, Sakai T, Yasui N. Significance of surgical treatment for severe dystrophic changes in the cervical spine associated with neurofibromatosis type I: a case report. J Pediatr Orthop B. 2010;19(3):270–5.

    Article  PubMed  Google Scholar 

  74. Warman ML, Cormier-Daire V, Hall C, Krakow D, Lachman R, LeMerrer M, et al. Nosology and classification of genetic skeletal disorders: 2010 revision. Am J Med Genet A. 2011;155A(5):943–68.

    Article  PubMed  CAS  Google Scholar 

  75. Shiang R, Thompson LM, Zhu YZ, Church DM, Fielder TJ, Bocian M, et al. Mutations in the transmembrane domain of FGFR3 cause the most common genetic form of dwarfism, achondroplasia. Cell. 1994;78(2):335–42.

    Article  CAS  PubMed  Google Scholar 

  76. Ippolito E, Maynard JA, Mickelson MR, Ponseti IV. Histochemical and ultrastructural study of the growth plate in achondroplasia. Basic Life Sci. 1988;48:61–71.

    CAS  PubMed  Google Scholar 

  77. Wynne-Davies R, Walsh WK, Gormley J. Achondroplasia and hypochondroplasia. Clinical variation and spinal stenosis. J Bone Joint Surg Br. 1981;63B(4):508–15.

    CAS  PubMed  Google Scholar 

  78. Harrod MJ, Friedman JM, Currarino G, Pauli RM, Langer LO Jr. Genetic heterogeneity in spondyloepiphyseal dysplasia congenita. Am J Med Genet. 1984;18(2):311–20.

    Article  CAS  PubMed  Google Scholar 

  79. LeDoux MS, Naftalis RC, Aronin PA. Stabilization of the cervical spine in spondyloepiphyseal dysplasia congenita. Neurosurgery. 1991;28(4):580–3.

    Article  CAS  PubMed  Google Scholar 

  80. Al Kaissi A, Chehida FB, Ghachem MB, Klaushofer K, Grill F. A hypoplastic atlas and long odontoid process in a girl manifesting phenotypic features resembling spondyloepimetaphyseal dysplasia joint laxity syndrome. Skelet Radiol. 2008;37(5):469–73.

    Article  Google Scholar 

  81. Simon M, Campos-Xavier AB, Mittaz-Crettol L, Valadares ER, Carvalho D, Speck-Martins CE, et al. Severe neurologic manifestations from cervical spine instability in spondylo-megaepiphyseal-metaphyseal dysplasia. Am J Med Genet C Semin Med Genet. 2012;160C(3):230–7.

    Article  PubMed  Google Scholar 

  82. Hastbacka J, Sistonen P, Kaitila I, Weiffenbach B, Kidd KK, de la Chapelle A. A linkage map spanning the locus for diastrophic dysplasia (DTD). Genomics. 1991;11(4):968–73.

    Article  CAS  PubMed  Google Scholar 

  83. Hastbacka J, de la Chapelle A, Mahtani MM, Clines G, Reeve-Daly MP, Daly M, et al. The diastrophic dysplasia gene encodes a novel sulfate transporter: positional cloning by fine-structure linkage disequilibrium mapping. Cell. 1994;78(6):1073–87.

    Article  CAS  PubMed  Google Scholar 

  84. Remes VM, Marttinen EJ, Poussa MS, Helenius IJ, Peltonen JI. Cervical spine in patients with diastrophic dysplasia–radiographic findings in 122 patients. Pediatr Radiol. 2002;32(9):621–8.

    Article  PubMed  Google Scholar 

  85. Winer N, Kyndt F, Paumier A, David A, Isidor B, Quentin M, et al. Prenatal diagnosis of Larsen syndrome caused by a mutation in the filamin B gene. Prenat Diagn. 2009;29(2):172–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  86. Roopesh Kumar VR, Madhguiri VS, Sasidharan GM, Gundamaneni SK, Yadav AK. Larsen syndrome with C3-C4 spondyloptosis and atlantoaxial dislocation in an adult. Spine (Phila Pa 1976). 2013;38(1):E43–7.

    Article  CAS  Google Scholar 

  87. Deere M, Sanford T, Francomano CA, Daniels K, Hecht JT. Identification of nine novel mutations in cartilage oligomeric matrix protein in patients with pseudoachondroplasia and multiple epiphyseal dysplasia. Am J Med Genet. 1999;85(5):486–90.

    Article  CAS  PubMed  Google Scholar 

  88. Shetty GM, Song HR, Unnikrishnan R, Suh SW, Lee SH, Hur CY. Upper cervical spine instability in pseudoachondroplasia. J Pediatr Orthop. 2007;27(7):782–7.

    Article  PubMed  Google Scholar 

  89. Meyer J, Südbeck P, Held M, Wagner T, Schmitz ML, Bricarelli FD, et al. Mutational analysis of the SOX9 gene in campomelic dysplasia and autosomal sex reversal: lack of genotype/phenotype correlations. Hum Mol Genet 1997;6(1):91–98.

    Article  CAS  PubMed  Google Scholar 

  90. Lekovic GP, Rekate HL, Dickman CA, Pearson M. Congenital cervical instability in a patient with camptomelic dysplasia. Childs Nerv Syst. 2006;22(9):1212–4.

    Article  PubMed  Google Scholar 

  91. Bams-Mengerink AM, Koelman JH, Waterham H, Barth PG, Poll-The BT. The neurology of rhizomelic chondrodysplasia punctata. Orphanet J Rare Dis. 2013;8:174.

    Article  PubMed  PubMed Central  Google Scholar 

  92. Violas P, Fraisse B, Chapuis M, Bracq H. Cervical spine stenosis in chondrodysplasia punctata. J Pediatr Orthop B. 2007;16(6):443–5.

    Article  PubMed  Google Scholar 

  93. Khandanpour N, Connolly DJ, Raghavan A, Griffiths PD, Hoggard N. Craniospinal abnormalities and neurologic complications of osteogenesis imperfecta: imaging overview. Radiographics 2012;32(7):2101–2112.

    Article  PubMed  Google Scholar 

  94. Leng LZ, Shajari M, Hartl R. Management of acute cervical compression fractures in two patients with osteogenesis imperfecta. Spine (Phila Pa 1976). 2010;35(22):E1248–52.

    Article  Google Scholar 

  95. Arroyo IL, Barron KS, Brewer EJ Jr. Spinal cord compression by epidural lipomatosis in juvenile rheumatoid arthritis. Arthritis Rheum. 1988;31(3):447–51.

    Article  CAS  PubMed  Google Scholar 

  96. Sherk HH, Pasquariello PS, Watters WC. Multiple dislocations of the cervical spine in a patient with juvenile rheumatoid arthritis and Down’s syndrome. Clin Orthop Relat Res. 1982;162:37–40.

    Google Scholar 

  97. Lawrence RC, Helmick CG, Arnett FC, Deyo RA, Felson DT, Giannini EH, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum. 1998;41(5):778–99.

    Article  CAS  PubMed  Google Scholar 

  98. Hensinger RN, DeVito PD, Ragsdale CG. Changes in the cervical spine in juvenile rheumatoid arthritis. J Bone Joint Surg Am. 1986;68(2):89–98.

    Article  Google Scholar 

  99. Uziel Y, Rathaus V, Pomeranz A, Solan H, Wolach B. Torticollis as the sole initial presenting sign of systemic onset juvenile rheumatoid arthritis. J Rheumatol. 1998;25(1):166–8.

    CAS  PubMed  Google Scholar 

  100. Rawlins BA, Girardi FP, Boachie-Adjei O. Rheumatoid arthritis of the cervical spine. Rheum Dis Clin N Am. 1998;24(1):55–65.

    Article  CAS  Google Scholar 

  101. Jurriaans E, Singh NP, Finlay K, Friedman L. Imaging of chronic recurrent multifocal osteomyelitis. Radiol Clin N Am. 2001;39(2):305–27.

    Article  CAS  PubMed  Google Scholar 

  102. Kahn MF, Chamot AM. SAPHO syndrome. Rheum Dis Clin N Am. 1992;18(1):225–46.

    CAS  Google Scholar 

  103. Yu L, Kasser JR, O’Rourke E, Kozakewich H. Chronic recurrent multifocal osteomyelitis. Association with vertebra plana. J Bone Joint Surg Am. 1989;71(1):105–12.

    Article  CAS  PubMed  Google Scholar 

  104. Gorham LW, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone); its relation to hemangiomatosis. J Bone Joint Surg Am. 1955;37-A(5):985–1004.

    Article  CAS  PubMed  Google Scholar 

  105. Martin JC, Desoysa R, O’Sullivan MM, Silverstone E, Williams H. Chronic recurrent multifocal osteomyelitis: spinal involvement and radiological appearances. Br J Rheumatol. 1996;35(10):1019–21.

    Article  CAS  PubMed  Google Scholar 

  106. Demharter J, Bohndorf K, Michl W, Vogt H. Chronic recurrent multifocal osteomyelitis: a radiological and clinical investigation of five cases. Skelet Radiol 1997;26(10):579–588.

    Article  CAS  Google Scholar 

  107. Chun CS. Chronic recurrent multifocal osteomyelitis of the spine and mandible: case report and review of the literature. Pediatrics. 2004;113(4):e380–4.

    Article  PubMed  Google Scholar 

  108. Khanna G, Sato TS, Ferguson P. Imaging of chronic recurrent multifocal osteomyelitis. Radiographics. 2009;29(4):1159–77.

    Article  PubMed  Google Scholar 

  109. Jansson AF, Grote V, Group ES. Nonbacterial osteitis in children: data of a German Incidence Surveillance Study. Acta Paediatr. 2011;100(8):1150–7.

    Article  PubMed  Google Scholar 

  110. El-Shanti HI, Ferguson PJ. Chronic recurrent multifocal osteomyelitis: a concise review and genetic update. Clin Orthop Relat Res. 2007;462:11–9.

    Article  PubMed  Google Scholar 

  111. Beck C, Morbach H, Beer M, Stenzel M, Tappe D, Gattenlöhner S, et al. Chronic nonbacterial osteomyelitis in childhood: prospective follow-up during the first year of anti-inflammatory treatment. Arthritis Res Ther. 2010;12(2):R74.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  112. Jurik AG, Egund N. MRI in chronic recurrent multifocal osteomyelitis. Skelet Radiol. 1997;26(4):230–8.

    Article  CAS  Google Scholar 

  113. King SM, Laxer RM, Manson D, Gold R. Chronic recurrent multifocal osteomyelitis: a noninfectious inflammatory process. Pediatr Infect Dis J 1987;6(10):907–911.

    Article  CAS  PubMed  Google Scholar 

  114. Kozlowski K, Masel J, Harbison S, Yu J. Multifocal chronic osteomyelitis of unknown etiology. Report of five cases. Pediatr Radiol. 1983;13(3):130–6.

    Article  CAS  PubMed  Google Scholar 

  115. Kayani I, Syed I, Saifuddin A, Green R, MacSweeney F. Vertebral osteomyelitis without disc involvement. Clin Radiol. 2004;59(10):881–91.

    Article  CAS  PubMed  Google Scholar 

  116. Mortensson W, Edeburn G, Fries M, Nilsson R. Chronic recurrent multifocal osteomyelitis in children. A roentgenologic and scintigraphic investigation. Acta Radiol. 1988;29(5):565–70.

    Article  CAS  PubMed  Google Scholar 

  117. Schilling F, Fedlmeier M, Eckardt A, Kessler S. Vertebral manifestation of chronic recurrent multifocal osteomyelitis (CRMO). Rofo. 2002;174(10):1236–42. German.

    Article  CAS  PubMed  Google Scholar 

  118. Anderson SE, Heini P, Sauvain MJ, Stauffer E, Geiger L, Johnston JO, et al. Imaging of chronic recurrent multifocal osteomyelitis of childhood first presenting with isolated primary spinal involvement. Skelet Radiol. 2003;32(6):328–36.

    Article  CAS  Google Scholar 

  119. Carr AJ, Cole WG, Roberton DM, Chow CW. Chronic multifocal osteomyelitis. J Bone Joint Surg Br. 1993;75(4):582–91.

    CAS  PubMed  Google Scholar 

  120. Baulot E, Bouillien D, Giroux EA, Grammont PM. Chronic recurrent multifocal osteomyelitis causing spinal cord compression. Eur Spine J 1998;7(4):340–343.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  121. Wiley AM, Trueta J. The vascular anatomy of the spine and its relationship to pyogenic vertebral osteomyelitis. J Bone Joint Surg Br. 1959;41-B:796–809.

    CAS  PubMed  Google Scholar 

  122. Crock HV, Goldwasser M. Anatomic studies of the circulation in the region of the vertebral end-plate in adult Greyhound dogs. Spine (Phila Pa 1976). 1984;9(7):702–6.

    Article  CAS  Google Scholar 

  123. Whalen JL, Parke WW, Mazur JM, Stauffer ES. The intrinsic vasculature of developing vertebral end plates and its nutritive significance to the intervertebral discs. J Pediatr Orthop. 1985;5(4):403–10.

    Article  CAS  PubMed  Google Scholar 

  124. Parke WW, Rothman RH, Brown MD. The pharyngovertebral veins: an anatomical rationale for Grisel’s syndrome. J Bone Joint Surg Am. 1984;66(4):568–74.

    Article  CAS  PubMed  Google Scholar 

  125. Zigler JE, Bohlman HH, Robinson RA, Riley LH, Dodge LD. Pyogenic osteomyelitis of the occiput, the atlas, and the axis. A report of five cases. J Bone Joint Surg Am 1987;69(7):1069–1073.

    Article  CAS  PubMed  Google Scholar 

  126. Nyberg DA, ffrey RB, Brant-Zawadzki M, Federle M, Dillon W. Computed tomography of cervical infections. J Comput Assist Tomogr. 1985;9(2):288–96.

    Article  CAS  PubMed  Google Scholar 

  127. Kemp HB, Jackson JW, Jeremiah JD, Hall AJ. Pyogenic infections occurring primarily in intervertebral discs. J Bone Joint Surg Br. 1973;55(4):698–714.

    CAS  PubMed  Google Scholar 

  128. Sapico FL, Montgomerie JZ. Pyogenic vertebral osteomyelitis: report of nine cases and review of the literature. Rev Infect Dis. 1979;1(5):754–76.

    Article  CAS  PubMed  Google Scholar 

  129. Fernandez M, Carrol CL, Baker CJ. Discitis and vertebral osteomyelitis in children: an 18-year review. Pediatrics. 2000;105(6):1299–304.

    Article  CAS  PubMed  Google Scholar 

  130. Bruschwein DA, Brown ML, McLeod RA. Gallium scintigraphy in the evaluation of disk-space infections: concise communication. J Nucl Med. 1980;21(10):925–7.

    CAS  PubMed  Google Scholar 

  131. Norris S, Ehrlich MG, McKusick K. Early diagnosis of disk space infection with 67Ga in an experimental model. Clin Orthop Relat Res. 1979;144:293–8.

    Google Scholar 

  132. Haase D, Martin R, Marrie T. Radionuclide imaging in pyogenic vertebral osteomyelitis. Clin Nucl Med. 1980;5(12):533–7.

    Article  CAS  PubMed  Google Scholar 

  133. Modic MT, Feiglin DH, Piraino DW, Boumphrey F, Weinstein MA, Duchesneau PM, et al. Vertebral osteomyelitis: assessment using MR. Radiology. 1985;157(1):157–66.

    Article  CAS  PubMed  Google Scholar 

  134. Bruns J, Maas R. Advantages of diagnosing bacterial spondylitis with magnetic resonance imaging. Arch Orthop Trauma Surg. 1989;108(1):30–5.

    Article  CAS  PubMed  Google Scholar 

  135. Digby JM, Kersley JB. Pyogenic non-tuberculous spinal infection: an analysis of thirty cases. J Bone Joint Surg Br. 1979;61(1):47–55.

    CAS  PubMed  Google Scholar 

  136. Frederickson B, Yuan H, Olans R. Management and outcome of pyogenic vertebral osteomyelitis. Clin Orthop Relat Res. 1978;131:160–7.

    Google Scholar 

  137. Eismont FJ, Bohlman HH, Soni PL, Goldberg VM, Freehafer AA. Pyogenic and fungal vertebral osteomyelitis with paralysis. J Bone Joint Surg Am. 1983;65(1):19–29.

    Article  CAS  PubMed  Google Scholar 

  138. Emery SE, Chan DP, Woodward HR. Treatment of hematogenous pyogenic vertebral osteomyelitis with anterior debridement and primary bone grafting. Spine (Phila Pa 1976). 1989;14(3):284–91.

    CAS  Google Scholar 

  139. Forsythe M, Rothman RH. New concepts in the diagnosis and treatment of infections of the cervical spine. Orthop Clin North Am. 1978;9(4):1039–51.

    CAS  PubMed  Google Scholar 

  140. Aulisa L, Pitta L, Aulisa AG, Mastantuoni G, Pola E, Leone A. Lumbar nerve root “walled” by a calcified herniated mass in a young patient. Childs Nerv Syst. 2003;19(5–6):384–6.

    Article  CAS  PubMed  Google Scholar 

  141. Harvet G, De Pontual L, Neven B, Mary P, Letamendia Richard E, Nathanson M, et al. Paediatric intervertebral calcifications: two cases report and review of the literature. Arch Pediatr. 2004;11(12):1457–61. French.

    Article  CAS  PubMed  Google Scholar 

  142. Gerlach R, Zimmermann M, Kellermann S, Lietz R, Raabe A, Seifert V. Intervertebral disc calcification in childhood–a case report and review of the literature. Acta Neurochir. 2001;143(1):89–93.

    Article  CAS  PubMed  Google Scholar 

  143. Ginalski JM, Landry M, Gudinchet F, Schnyder P. Is tomography of intervertebral disc calcification useful in children? Pediatr Radiol 1992;22(1):59–61.

    Article  CAS  PubMed  Google Scholar 

  144. Ginalski JM, Schnyder P, Gerster JC. Spontaneous regression of intervertebral disc calcifications in a child. Clin Rheumatol. 1991;10(1):87–9.

    Article  CAS  PubMed  Google Scholar 

  145. Park SM, Kim ES, Sung DH. Cervical radiculopathy caused by neural foraminal migration of a herniated calcified intervertebral disk in childhood: a case report. Arch Phys Med Rehabil. 2005;86(11):2214–7.

    Article  PubMed  Google Scholar 

  146. Sonnabend DH, Taylor TK, Chapman GK. Intervertebral disc calcification syndromes in children. J Bone Joint Surg Br. 1982;64(1):25–31.

    CAS  PubMed  Google Scholar 

  147. Mahlfeld K, Kayser R, Grasshoff H. Permanent thoracic myelopathy resulting from herniation of a calcified intervertebral disc in a child. J Pediatr Orthop B. 2002;11(1):6–9.

    PubMed  Google Scholar 

  148. Bollini G, Bergoin M, Choux M, Padovani J. Disk calcifications in children. Apropos of 17 cases. Rev Chir Orthop Reparatrice Appar Mot. 1984;70(5):377–82. French.

    CAS  PubMed  Google Scholar 

  149. Causey AL, Evans OB, Lewis-Abney K. Intervertebral disk calcification: an unusual cause of acquired torticollis in childhood. Pediatr Emerg Care. 1996;12(5):356–9.

    Article  CAS  PubMed  Google Scholar 

  150. Herring JA, Hensinger RN. Cervical disc calcification. J Pediatr Orthop. 1988;8(5):613–6.

    Article  CAS  PubMed  Google Scholar 

  151. Coventry MB. Calcification in a cervical disc with anterior protrusion and dysphagia. A case report. J Bone Joint Surg Am. 1970;52(7):1463–6.

    Article  CAS  PubMed  Google Scholar 

  152. Jawish R, Ponet M. Dysphagia disclosing a cervical disk calcification in a child. Apropos of a case and review of the literature. Chir Pediatr. 1990;31(2):127–30. French.

    CAS  PubMed  Google Scholar 

  153. Bret P, Confavreux C, Thouard H, Pialat J. Aneurysmal bone cyst of the cervical spine: report of a case investigated by computed tomographic scanning and treated by a two-stage surgical procedure. Neurosurgery. 1982;10(1):111–5.

    CAS  PubMed  Google Scholar 

  154. Wong CC, Pereira B, Pho RW. Cervical disc calcification in children. A long-term review. Spine (Phila Pa 1976). 1992;17(2):139–44.

    Article  CAS  Google Scholar 

  155. Fielding JW, Hawkins RJ, Ratzan SA. Spine fusion for atlanto-axial instability. J Bone Joint Surg Am. 1976;58(3):400–7.

    Article  CAS  PubMed  Google Scholar 

  156. Wetzel FT, La Rocca H. Grisel’s syndrome. Clin Orthop Relat Res. 1989;240:141–52.

    Google Scholar 

  157. Van Holsbeeck EM, MacKay NN. Diagnosis of acute atlanto-axial rotatory fixation. J Bone Joint Surg Br. 1989;71(1):90–1.

    PubMed  Google Scholar 

  158. Rinaldi I, Mullins WJ Jr, Delaney WF, Fitzer PM, Tornberg DN. Computerized tomographic demonstration of rotational atlanto-axial fixation. Case report. J Neurosurg. 1979;50(1):115–9.

    Article  CAS  PubMed  Google Scholar 

  159. Pang D, Li V. Atlantoaxial rotatory fixation: part 1–biomechanics of normal rotation at the atlantoaxial joint in children. Neurosurgery. 2004;55(3):614–25; discussion 625–6.

    Article  PubMed  Google Scholar 

  160. Pang D, Li V. Atlantoaxial rotatory fixation: part 2–new diagnostic paradigm and a new classification based on motion analysis using computed tomographic imaging. Neurosurgery. 2005;57(5):941–53; discussion 941–53.

    Article  PubMed  Google Scholar 

  161. Burkus JK, Deponte RJ. Chronic atlantoaxial rotatory fixation correction by cervical traction, manipulation, and bracing. J Pediatr Orthop. 1986;6(5):631–5.

    Article  CAS  PubMed  Google Scholar 

  162. Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine (Phila Pa 1976). 1998;23(20):2174–9.

    Article  CAS  Google Scholar 

  163. Warner WC, Hedequist DJ. Cervical spine injuries in children. In: Beaty JH, Kassser JR, editors. Rockwood and Wilkins’ fractures in children. Philadelphia: Lippincott Williams & Wilkins; 2006. p. 776–816.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Daniel J. Hedequist .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer Science+Business Media LLC

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Saadat, E., Hedequist, D.J., Wright, P. (2018). Pathology of the Child’s Cervical Spine and Its Clinical Implications. In: Hedequist, D., Shah, S., Yaszay, B. (eds) The Management of Disorders of the Child’s Cervical Spine. Springer, Boston, MA. https://doi.org/10.1007/978-1-4939-7491-7_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-7491-7_3

  • Published:

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4939-7489-4

  • Online ISBN: 978-1-4939-7491-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics