Standalone cranio-cervical decompression is feasible in children with mucopolysaccharidosis type I, IVA and VI

In patients with Mucopolysaccharidosis (MPS), glycosaminoglycans deposits in the dura mater and supporting ligaments cause spinal cord compression and consecutive myelopathy, predominantly of the cranio-cervical junction. Disease characteristics of cranio-cervical stenosis (CCS) in MPS patients differ profoundly from other hereditary and degenerative forms.Due to high peri-procedural morbidity and mortality, MPS patients pose a substantial challenge to the unexperienced medical care provider. As literature remains scarce, we present our experience with a large cohort of MPS patients treated for CCS without atlanto-occiptal instrumentation.

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