CervicoThoracic Myelopathy In Children With Morquios Disease

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Cervico-Thoracic Myelopathy In Children With
Morquios Disease

• Mohan V. Belthur MD, Mihir M. Thacker MD, Leslie
  Grissom MD, William G. Mackenzie MD FRCS(C)

EPOS/IFPOS Combined Meeting, Sorrento, Italy 2007
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Introduction

• Morquio syndrome (Mucopolysaccharidosis Type 4)
  was simultaneously described by JF Brailsford and
  Luis Morquio in 1929.
• Spinal involvement is a major cause of morbidity
  and mortality in patients with Morquio Syndrome

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Aim

• Involvement of the upper cervical spine and the
  thoraco-lumbar spine in patients with Morquio
  syndrome is well described.
• The aim of our study was to describe compression
  of the spinal cord at the cervico-thoracic
  junction in these patients

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Methods

• Retrospective IRB approved clinical and
  radiographic review
• 16 patients with Morquio syndrome (1990-2005)
• 13/16 had been previously treated for upper
  cervical instability (fusion /-instrumentation)
• 4/16 developed a myelopathy secondary to cord
  compression at the cervico-thoracic junction
• All four had clinical and radiographic (MRI)
  evidence of cord compression at the
  cervico-thoracic region

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Methods

• Three girls and one boy
• Average age at diagnosis of cervico-thoracic
  compression was 76 months (54-112 months)

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Case Presentation

• Case of a 6 5 year old male demonstrating
  cervico-thoracic compression

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Case PresentationPost-Operative

• The patient underwent a C7-T1 laminectomy and
  posterior spinal instrumentation and fusion from
  C5-T2

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Results
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Conclusion

• Myelopathy in patients with Morquio syndrome
  secondary to cord compression at the
  Cervico-thoracic junction has not been previously
  reported in literature.
• Surgeons must be aware that cord compression can
  be seen in the upper cervical, cervico-thoracic,
  or thoracolumbar regions and needs to be
  addressed at the appropriate level.

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References

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• Hughes DG, Chadderton RD, Cowie RA, et al. MRI of
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• Kulkarni MV, Williams JC, Yeakley JW, et al.
  Magnetic resonance imaging in the diagnosis of
  the cranio-cervical manifestations of the
  mucopolysaccharidoses. Magn Reson Imaging.
  19875317-323.
• Nelson J, and Thomas PS. Clinical findings in 12
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