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Transoral decompression and posterior stabilisation in Morquio's disease
J Ashraf1, H A Crockard, A O Ransford
1National Hospitals for Neurology and Neurosurgery, Queen Square, London.
Archives of Disease in Childhood
|November 1, 1991
Summary
A child with Morquio's disease experienced hemiparesis due to craniocervical junction compression. A novel combined surgical approach successfully treated this complex atlantoaxial subluxation.
Area of Science:
- Neurosurgery
- Pediatric Orthopedics
- Medical Genetics
Background:
- Morquio's disease can lead to craniocervical instability.
- Atlantoaxial subluxation is a severe complication causing spinal cord compression.
- Neurological deficits, like hemiparesis, necessitate urgent intervention.
Observation:
- A 3.5-year-old boy with Morquio's disease presented with persistent left hemiparesis post-trauma.
- Imaging revealed craniocervical junction compression from atlantoaxial subluxation and anterior soft tissue impingement.
- Transient unconsciousness was attributed to medullary concussion from hyperextension.
Findings:
- Conventional posterior occipitocervical fusion is often recommended for cervical myelopathy.
- The significant anterior compression in this case made the standard approach unsafe.
- A combined anterior transoral decompression and posterior fusion was performed.
Implications:
- This combined approach offers a viable solution for complex craniocervical pathologies.
- Early surgical intervention can prevent further neurological deterioration in patients with Morquio's disease.
- Managing atlantoaxial subluxation in rare genetic disorders requires tailored surgical strategies.