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Delayed Onset Post-Operative Neurologic Deficit in a Patient With Mucopolysaccharidosis type VI: A Case Report
Christopher Lindsay1, Joshua Holt1, Stuart Weinstein1
1Department of Orthopedics and Rehabilitation, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA.
The Iowa Orthopaedic Journal
|January 5, 2023
Summary
Mucopolysaccharidosis type VI patients undergoing spine surgery face risks of delayed neurological issues. Prompt surgical intervention for spinal cord compression led to significant recovery in a young patient.
Area of Science:
- Biochemistry
- Genetics
- Orthopedics
Background:
- Mucopolysaccharidoses (MPS) are lysosomal storage disorders due to glycosaminoglycan (GAG) accumulation.
- MPS type VI (Maroteaux-Lamy syndrome) involves a deficiency in N-acetylgalactosamine-4-sulfatase.
- GAG deposition causes thoracolumbar kyphosis, ligamentous incompetence, and trunk instability.
Observation:
- A 14-year-old girl with MPS VI had severe progressive lumbar kyphosis corrected surgically.
- Postoperatively, she experienced delayed onset of severe lower extremity weakness.
- Urgent laminectomies and ligamentum flavum resection were performed due to spinal canal compression.
Findings:
- The patient achieved near-complete neurologic recovery within one year of the decompression surgery.
- This case highlights a rare instance of late-onset neurological compromise after spinal deformity correction in MPS.
Implications:
- Patients with MPS are at high risk for neurological compromise from both the disease and surgical correction.
- Spinal surgeons must consider pre-operative tissue hypertrophy and GAG deposition.
- Early recognition and surgical decompression are crucial for managing delayed neurological deficits in MPS patients.
