Sahin Mustafa Caglar, Ipek Emine Metin, Zinnuroglu Murat, Borcek Alp Ozgun
Faculty of Medicine Department of Neurosurgery, Gazi University, 06560, Ankara, Turkey.
Faculty of Medicine Department of Physical Medicine and Rehabilitation, Gazi University, 06560, Ankara, Turkey.
Childs Nerv Syst. 2023 May;39(5):1323-1328. doi: 10.1007/s00381-022-05748-5. Epub 2022 Nov 18.
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.
IVA型黏多糖贮积症(MPS IVA)患者存在许多脊髓病和轻瘫的危险因素。这些因素包括脊髓受压、骨质狭窄、伴有韧带松弛、畸形、齿状突发育不全和寰枢椎不稳的软组织增厚。尽管大多数MPS IVA患者出生时总体看起来健康,但患者常在几年内出现骨骼畸形。手术指征难以确定。从历史上看,许多医生对年轻无症状的MPS IVA患者进行预防性减压和融合术以防止脊髓受压。尽管MPS IVA患者通常需要在颅颈交界处进行脊髓减压,但在其他脊髓节段也可能需要减压。手术期间有发生神经损伤的风险。最常见的原因是俯卧位时心输出量恶化继发的缺血或动脉损伤,以及在将患者置于俯卧位时颈部运动或牵引导致的局部创伤。在脊髓手术中,神经生理监测对于降低神经损伤风险非常重要。在本病例报告中,将呈现一例在俯卧位后未对颅颈交界处进行任何干预的手术早期出现下肢神经监测运动诱发电位(MEP)反应消失的病例。