Skip to main content
SpringerLink
Log in

The cervical spine in the Klippel-Feil syndrome

A report of 57 cases

  • Published:
International Orthopaedics Aims and scope Submit manuscript

Summary

We report 57 cases of the Klippel-Feil syndrome. Five had type I congenital cervical fusion, 48 type II and 4 type III. The symptoms and signs varied considerably. Patients with C2–C3 fusion often had symptoms associated with odontoid dysplasia and occipito-cervical instability. Twenty-one had progressive neurological symptoms and 19 were operated on: 3 had occiput-C3 posterior arthrodeses, 2 a single level fusion, 1 a laminectomy and anterior arthrodesis, and 13 had laminoplasties. Degenerative changes at the unfused segment and a narrow bony canal are high risk factors in the development of neurological compromise.

Résumé

Les auteurs rapportent 57 cas de syndrome de Klippel-Feil. D'après la classification de Feil, 5 des patients avaient une fusion de type I, 48 une fusion de type II et 4 une fusion de type de III. Il n'y avait pas de prévalence sexuelle notable et les symptômes étaient très variés. Les patients avec fusion C2–C3, présentaient souvent une dysplasie de l'apophyse ondotoïde avec instabilité cervicooccipitale. 21 patients ont eu des déficits neurologiques progressifs et 19 d'entre-eux ont du être opérés: 3 pour une arthrodèse postérieure occipito-C3, 2 pour une arthrodèse antérieure en un seul niveau, 1 par laminectomie et arthrodèse antérieure et 13 par laminoplasie. En plus de la pathologie dégénérative se développant au niveau du segment non fusionné, un canal cervical étroit au niveau du segment atteint de syndrome de Klippel-Feil représente un facteur de risque important de troubles neurologiques.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Baba H, Furusawa N, Imura S, Kawahara N, Tsuchiya H, Tomita K (1993) Late radiographic findings after anterior cervical fusion for spondylotic myeloradiculopathy. Spine 18: 2167–2173

    Article  PubMed  CAS  Google Scholar 

  2. Baba H, Furusawa N, Imura S, Kawahara N, Tomita K (1994) Laminoplasty following anterior cervical fusion for spondylotic myeloradiculopathy. Int Orthop 18: 1–5

    PubMed  CAS  Google Scholar 

  3. Chandraraj S, Briggs C (1992) Failure of somite differentiation at the craniovertebral region as a cause of occipitalization of the atlas. Spine 17: 1249–1251

    PubMed  CAS  Google Scholar 

  4. Ducker TB (1990) Cervical myeloradiculopathy: Klippel-Feil deformity. J Spinal Disord 3: 439–444

    PubMed  CAS  Google Scholar 

  5. Elster AD (1984) Quadriplegia after minor trauma in the Klippel-Feil syndrome. A case report and review of the literature. J Bone Joint Surg [Am] 66: 1473–1474

    PubMed  CAS  Google Scholar 

  6. Epstein NE, Epstein JA, Zikha A (1984) Traumatic myelopathy in a seventeen-year-old child with cervical stenosis (without fracture or dislocation) and a C2–C3 Klippel-Feil fusion: a case report. Spine 9: 344–347

    PubMed  CAS  Google Scholar 

  7. Feil A (1919) L'absence et la diminution des vertèbres cervicales (étude clinique et pathogénique): le syndrome de réduction numérique cervicale, Thèses de Paris

  8. Fielding JW, Hensinger RN, Hawkins RJ (1980) Osodontoideum. J Bone Joint Surg [Am] 62: 376–383

    PubMed  CAS  Google Scholar 

  9. Fielding JW, Hensinger RN, Hawkins RJ (1986) The cervical spine. In: Lowel WW, Winter RB (eds) Pediatric orthopaedics, 2nd edn. Lippincott, Philadelphia

    Google Scholar 

  10. Hall JE, Simmons ED, Danylchuk K, Barnes PD (1990) Instability of the cervical spine and neurologic involvement in Klippel-Feil syndrome: a case report. J Bone Joint Surg [Am] 72: 460–462

    PubMed  CAS  Google Scholar 

  11. Hensinger RN (1992) Congenital anomalies of the cervical spine. In: Rothman RH, Simeone FA (eds) The spine. Saunders, Philadelphia

    Google Scholar 

  12. Hensinger RN, Lang JE, MacEwen GD (1974) Klippel-Feil syndrome: a constellation of associated anomalies. J Bone Joint Surg [Am] 56: 1242–1253

    Google Scholar 

  13. Jeanneret B, Magerl F (1990) Congenital fusion C0–C2 associated with spondylolysis of C2. J Spinal Disord 3: 413–417

    PubMed  CAS  Google Scholar 

  14. Klippel M, Feil A (1912) Un cas d'absence des vertèbres cervicales avec cage thoracique remontant jusqu'à la base du crâne. Nouv Icon Salpétrière 25: 223–250

    Google Scholar 

  15. Lee CK, Weiss CK (1981) Isolated congenital cervical block vertebrae below the axis with neurologic symptoms. Spine 6: 118–124

    PubMed  CAS  Google Scholar 

  16. Louw JA, Albertse H (1987) Traumatic quadriplegia after minor trauma in the Klippel-Feil syndrome [Letter]. S Afr Med J 72: 889–890

    PubMed  CAS  Google Scholar 

  17. Nagib MG, Maxwell RE, Chou SN (1984) Identification and management of high-risk patients with Klippel-Feil syndrome. J Neurosurg 61: 525–530

    Google Scholar 

  18. Nagib MG, Maxwell RE, Chou SN (1985) Klippel-Feil syndrome in children: clinical features and management. Child Neurol System 1: 255–263

    Article  CAS  Google Scholar 

  19. Odom GL, Finney W, Woodhall (1958) Cervical disc lesions. JAMA 166: 23–28

    CAS  Google Scholar 

  20. Ohki I, Ohta H, Saitoh A (1993) Cervical myelopathy associated with Klippel-Feil syndrome. Proc Jpn Orthop Assoc 66: S69

  21. Pizzutillo PD (1983) Klippel-Feil syndrome. In: Bailey RW, Sherk HH, Dunn EJ et al (eds) The cervical spine. Lippincott, Philadelphia

    Google Scholar 

  22. Pizzutillo PD, Woods MW, Nicholson I (1987) Risk factors in the Klippel-Feil syndrome. Orthop Trans 11: 473

    Google Scholar 

  23. Prusik VR, Samberg LC (1985) Klippel-Feil syndrome associated with spinal stenosis: a case report. J Bone Joint Surg [Am] 67: 161–164

    Google Scholar 

  24. Ritterbusch JF, McGInty LD, Spar J, Orrison WW (1991) Magnetic resonance imaging for stenosis and subluxation in Klippel-Feil syndrome. Spine 16: S539-S541

    PubMed  CAS  Google Scholar 

  25. Sherk HH, Dawoud S (1981) Congenital os odontoideum with Klippel-Feil syndrome and fatal atlanto-axial instability: report of a case. Spine 6: 42–45

    PubMed  CAS  Google Scholar 

  26. Sirasaki N, Okada K, Oka S, Hosono N, Yonenobu K, Ono K (1991) Os odontoideum with posterior atlantoaxial instability. Spine 16: 706–715

    Google Scholar 

  27. Smith MD, Phillips WA, Hensinger RN (1991) Fusion of the upper cervical spine in children and adolescents: an analysis of 17 patients. Spine 16: 695–701

    PubMed  CAS  Google Scholar 

  28. Sperings ELH, Braakman R (1982) The management of os odontoideum: analysis of 37 cases. J Bone Joint Surg [Br] 64: 422–428

    Google Scholar 

  29. Warner WC Jr (1991) Cervical spine anomalies. In: Canale ST, Beaty JH (eds) Operative pediatric orthopaedics, Mosby Year Book, St Louis

    Google Scholar 

  30. Weisy GM (1983) Trauma to the anomalous cervical spine. Spine 8: 225–227

    Google Scholar 

  31. White AA, Panjabi MM (1990) Practical biomechanics of spine trauma. In: White AA, Panjabi MM (eds) Clinical biomechanics of the spine, 2nd edn. Lippincott, Philadelphia

    Google Scholar 

  32. Winter RB, Moe JH (1984) The incidence of Klippel-Feil syndrome in patients with congenital scoliosis and kyphosis. Spine 9: 363–366

    PubMed  CAS  Google Scholar 

  33. Wolf BS, Khilnani M, Malis L (1965) The sagittal diameter of the cervical canal and its significance in cervical spondylosis. J Mt Sinai Hosp 23: 283–292

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Orthopaedic Surgery, Fukui Medical School, Shimoaizuki 23, Matsuoka, 910-11, Fukui, Japan

    H. Baba, Y. Maezawa, N. Furusawa, Q. Chen & S. Imura

  2. Department of Orthopaedic Surgery, School of Medicine, Kanazawa University, Kanazawa, Japan

    K. Tomita

Authors
  1. H. Baba
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Y. Maezawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. N. Furusawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Q. Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Imura
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. K. Tomita
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Baba, H., Maezawa, Y., Furusawa, N. et al. The cervical spine in the Klippel-Feil syndrome. International Orthopaedics 19, 204–208 (1995). https://doi.org/10.1007/BF00185222

Download citation

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00185222

Keywords

Search

Navigation