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.