Abstract
The mucopolysaccharidoses (MPS) are genetic lysosomal storage diseases. Peripheral bone dysplasia and spinal involvement are the predominant orthopedic damage. The risk of spinal cord compression due to stenosis of the craniocervical junction is well known in these patients, whereas the thoracolumbar kyphosis is often well tolerated over a long period of time. Thus, signs of spinal cord compression at this level occur later and more insidiously. The authors describe three cases of patients with thoracolumbar kyphosis who suffered from postoperative spinal cord compression in the absence of direct surgical trauma. Analysis of these cases and review of the literature helped identify causal factors resulting in spinal cord ischemia. The risk of perioperative spinal cord complications related to a thoracolumbar kyphosis must be discussed with patients with MPS and their families prior to any surgery, including extra-spinal procedures.
Competing interests: None declared
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Communicated by: Maurizio Scarpa, M.D, Ph.D
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Thoracolumbar kyphosis in patients suffering from mucopolysaccharidoses is raising the risk of perioperative medullary complications, even in extra-spinal surgery.
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All the authors of this chapter declare that there are no conflicts of interest.
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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (5). Informed consent was obtained from all patients and their legal representative when necessary for being included in the study.
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Nicolas Pauchard has reviewed all the patients.
Jean-Luc Jouve, Christophe Garin, and Pierre Journeau performed the surgical procedure for the patients and followed them.
Nicolas Pauchard, Pierre Journeau and Pierre Lascombes have written and reviewed the article.
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Pauchard, N., Garin, C., Jouve, J.L., Lascombes, P., Journeau, P. (2014). Perioperative Medullary Complications in Spinal and Extra-Spinal Surgery in Mucopolysaccharidosis: A Case Series of Three Patients. In: Zschocke, J., Gibson, K., Brown, G., Morava, E., Peters, V. (eds) JIMD Reports Volume 16. JIMD Reports, vol 16. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2014_325
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DOI: https://doi.org/10.1007/8904_2014_325
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