Wald S L, Schmidek H H
Neurosurgery. 1984 Jan;14(1):83-8. doi: 10.1227/00006123-198401000-00020.
Spinal cord compression with resultant myelopathy is a frequent occurrence in patients with mucopolysaccharidoses. Etiological factors include developmental abnormalities of the cervical spine and infiltration of the dura mater by the accumulated products of mucopolyssacharide metabolism. Compression at the thoracolumbar junction is rare, but was found in a child with the characteristic physical and biochemical stigmata of the Maroteaux-Lamy syndrome (mucopolysaccharidosis VI). An anterolateral approach to remove the compressing bony elements resulted in symptomatic improvement. Careful radiological evaluation is required so that all surgical options can be considered. Patients with metabolic storage diseases and the capacity for normal intellectual function warrant aggressive surgical care to optimize neurological function.
黏多糖贮积症患者常发生脊髓受压并导致脊髓病。病因包括颈椎发育异常以及黏多糖代谢产物在硬脊膜的浸润。胸腰段交界处受压罕见，但在一名具有马罗-拉米综合征（黏多糖贮积症VI型）典型身体和生化特征的儿童中发现。采用前外侧入路切除压迫性骨结构后症状改善。需要进行仔细的影像学评估，以便考虑所有手术方案。具有正常智力功能的代谢性贮积病患者应积极接受手术治疗以优化神经功能。
