Abstract
Purpose
Patients with mucopolysaccharidosis type IVA (MPS IVA) have many risk factors for myelopathy and paresis. These are spinal cord compression, bone stenosis, and soft tissue thickening with ligament laxity, deformity, odontoid hypoplasia, and atlantoaxial instability. Although most patients with MPS IVA appear generally healthy at birth, patients often show skeletal deformities within a few years. Surgical indications are difficult to determine. Historically, many physicians have used prophylactic decompression and fusion in young, asymptomatic MPS IVA patients to prevent cord compression. Although spinal cord decompression is usually required at the craniocervical junction in patients with MPS IVA, decompression may be required at other spinal cord levels as well. There is a risk of developing neurological damage during surgery. The most common causes are ischemia secondary to cardiac output deteriorated in the prone position or due to artery damage, and local trauma due to neck movements or traction while bringing the patient to the prone position. Neurophysiological monitoring is very important during surgery to reduce the risk of neurological damage in spinal cord surgery. In this case report, a case with loss of lower extremity neuromonitorization motor evoked potential (MEP) responses in the early period of surgery without any intervention to the craniocervical junction after prone positioning will be presented.
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References
Ransford AO, Crockard HA, Stevens JM, Modaghegh S (1996) Occipito-atlanto-axial fusion in Morquio-Brailsford syndrome. A ten-year experience. J Bone Joint Surg Br 78:307–313
Stevens JM, Kendall BE, Crockard HA, Ransford A (1991) The odontoid process in Morquio-Brailsford’s disease. The effects of occipitocervical fusion. J Bone Joint Surg Br 73:851–858. https://doi.org/10.1302/0301-620X.73B5.1910048
Charrow J, Alden TD, Breathnach CAR et al (2015) Diagnostic evaluation, monitoring, and perioperative management of spinal cord compression in patients with Morquio syndrome. Mol Genet Metab 114:11–18. https://doi.org/10.1016/j.ymgme.2014.10.010
Ofiram E, Lonstein JE, Skinner S, Perra JH (2006) The disappearing evoked potentials: a special problem of positioning patients with skeletal dysplasia: case report. Spine (Phila Pa 1976) 31:E464–470. https://doi.org/10.1097/01.brs.0000222122.37415.4d
Tong CKW, Chen JCH, Cochrane DD (2012) Spinal cord infarction remote from maximal compression in a patient with Morquio syndrome. J Neurosurg Pediatr 9:608–612. https://doi.org/10.3171/2012.2.PEDS11522
Borlot F, Arantes PR, Capel Cardoso AC, Kim CA (2014) Remote spinal cord injury in mucopolysaccharidosis type IVA after cervical decompression. Neurology 82:1382–1383. https://doi.org/10.1212/WNL.0000000000000341
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Sahin, M.C., Ipek, E.M., Zinnuroglu, M. et al. How did intraoperative neuromonitorization prevent tetraplegia?. Childs Nerv Syst 39, 1323–1328 (2023). https://doi.org/10.1007/s00381-022-05748-5
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DOI: https://doi.org/10.1007/s00381-022-05748-5