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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.
Background:
Mucopolysaccharidoses (MPS) are lysosomal storage disorders characterized by abnormal deposition of glycosaminoglycans (GAGs) in tissues. In type VI MPS, otherwise known as Maroteaux-Lamy syndrome, the defect is in the enzyme N-acetylgalactosamine-4-sulfatase. Thoracolumbar kyphosis results from GAG deposition, leading to incompetence of posterior ligamentous structures as well as poor trunk control. Though neurologic symptoms from canal compression due to deformity and hypertrophy of tissues have been described, occasionally requiring surgical decompression, there has not been a prior report of late onset of symptoms in a previously neurologically intact patient following surgery to correct spine deformity.
Methods:
The case reviewed is a 14 year old girl with mucopolysaccharidosis type VI underwent anterior release and posterior instrumentation for correction of severe progressive lumbar kyphosis. Postoperatively she developed delayed onset of profound lower extremity weakness and underwent urgent wide laminectomies and resection of thickened ligamentum flavum. At 1 year follow-up, she had near complete neurologic recovery.
Conclusion:
Patients with mucopolysacchari-doses are at significant risk for neurologic compromise both as part of the natural history of the disease, and as a risk of deformity correction. The surgeon must consider the pathologic thickening of tissues surrounding the spinal cord when planning surgery. Level of Evidence: IV.
Insights
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.
