Cervico-Thoracic Myelopathy In Children With Morquio's Disease
Transcript of Cervico-Thoracic Myelopathy In Children With Morquio's Disease
Cervico-Thoracic Myelopathy In Children With Morquio’s 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
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
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
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
Methods
Three girls and one boy
Average age at diagnosis of cervico-thoracic compression was 76 months (54-112 months)
Case Presentation
Case of a 6 + 5 year old male demonstrating cervico-thoracic compression
Case PresentationPost-Operative
The patient underwent a C7-T1 laminectomy and posterior spinal instrumentation and fusion from C5-T2
Results
Patient
Sex
Age at OC
fusion (months)
Age at diagnosis
of CT stenosis (months)
Age at surgery
(months)
Level of
stenosisDetails of surgery Complications/ Sequelae
1 F 33 54 56 T1-T4T1-4 laminectomy + C7-T4
Posterior spinal fusionnone
2 M 39 77 80 C7-T1Laminectomy C7-T1 +
Posterior spinal fusion C5-T2
Compression distal to the area of fusion 12 years
later. Needed T2-T6 decompression +
Posterior spinal fusion T2-T7
3 F 58 57 58 C7-T1Laminectomy T1-2 +
Posterior spinal fusion C7-T4none
4 F 98 112 113 C7-T2
1. Laminectomy C7-T2 + Posterior spinal fusion C6-T2
2. Anterior corpectomy, anterior and posterior fusion
C6-T2 and repair of pseudarthrosis
PseudarthrosisDeveloped T/L kyphosis
with T11-T12 disc protrusion which needed
Anterior decompression + fusion
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.
References
Blaw ME, and Langer LO. Spinal cord compression in Morquio-Brailsford's disease. J Pediatr. 1969;74:593-600.
Hughes DG, Chadderton RD, Cowie RA, et al. MRI of the brain and craniocervical junction in Morquio's disease. Neuroradiology. 1997;39:381-385.
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. 1987;5:317-323.
Nelson J, and Thomas PS. Clinical findings in 12 patients with MPS IV A (Morquio's disease). Further evidence for heterogeneity. Part
III: Odontoid dysplasia. Clin Genet. 1988;33:126-130.