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Spine challenges in mucopolysaccharidosis
Marco Crostelli1, Osvaldo Mazza2, Massimo Mariani2
1Spine Surgery Unit, Surgery and Transplantations Department, Ospedale Pediatrico Bambino Gesù, Rome, Italy. marco.crostelli@opbg.net.
International Orthopaedics
|September 16, 2018
Summary
Mucopolysaccharidosis (MPS) spine disease management involves bracing for kyphosis and surgery for severe instability. Surgical outcomes show good fusion, but pediatric instrumentation remains a challenge.
Area of Science:
- Rare inherited metabolic diseases
- Lysosomal storage disorders
- Skeletal system pathology
Background:
- Mucopolysaccharidoses (MPS) cause lysosomal storage of mucopolysaccharides, affecting the spine.
- Spinal manifestations include thoracolumbar kyphosis and atlantoaxial subluxation, leading to neurological complications.
- Current medical treatments have limited impact on spinal disease progression.
Purpose of the Study:
- To present the experience in treating spine pathology in patients with Mucopolysaccharidosis (MPS).
- To evaluate the effectiveness of bracing and surgical interventions for spinal deformities in MPS.
Main Methods:
- Treatment of cervical instability involved surgical decompression and stabilization.
- Thoracic lumbar kyphosis was managed with bracing or surgery, including vertebrectomy and lateral costo-transverse approach.
- Evaluation included clinical follow-up and CT scans for fusion and correction.
Main Results:
- Surgical patients experienced no major complications, with CT scans showing complete fusion and maintained correction.
- A case of cervical instability in an MPS1 patient utilized adult rigid instrumentation with successful long-term follow-up.
- A thoracic lumbar kyphosis case treated with vertebrectomy achieved fusion despite suboptimal cage positioning due to anatomical constraints.
Conclusions:
- Bracing is effective for moderate thoracic lumbar kyphosis, yielding good medium-term results.
- Surgical treatment is effective for severe cervical and thoracic lumbar spinal deformities in MPS.
- A significant challenge in pediatric MPS spinal surgery is the lack of appropriately sized instrumentation.
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