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X-linked Hypophosphatemic Rickets Resulting in Severe Spinal Stenosis and Myelopathy: A Case Report 2014

Category General Spine Joel R. Thomas, MD
Amy L. Kotsenas, MD
Felix Diehn, MD
John Wald, MD
Purpose Enthesopathy related to x-linked hypophosphatemic rickets is a rare cause of spinal stenosis and can result in spinal cord compression with consequent myelopathy. Only a few case reports exist in the literature. We present the case of a 67 year old woman who presented to our institution with a known history of x-linked dominant hypophosphatemic rickets and symptoms of mild myelopathy. Materials & Methods After obtaining IRB approval, pertinent patient history and imaging studies were reviewed on our institution’s electronic medical record and PACS. Results Imaging of the patient's spine revealed exuberant ligamentum flavum calcification and facet hypertrophy on computed tomography at multiple thoracic segments resulting in severe spinal canal narrowing. Magnetic resonance imaging demonstrated distal spinal cord compression with intramedullary signal abnormality on T2-weighted images. After neurosurgical consultation, decompressive laminectomies at the level of cord compression were planned. Picture1.tif Conclusion Lessons to be learned: Enthesopathy related to x-linked hypophosphatemic rickets can result in severe spinal stenosis and cause cord compression with neurologic sequelae. Characteristic imaging findings can include thickening and calcification of the ligamentum flavum and hypertrophic changes of the facets as illustrated in this case. References 1. Burnstein MI, Lawson JP, Kottamasu SR, et. al. The Enthesopathic Changes of Hypophosphatemic Osteomalacia in Adults: Radiologic Findings. AJR 1989; 153:785-790. 2. Dunlop DJ, Stirling AJ. Thoracic spinal cord compression caused by hypophosphataemic rickets: a case report and review of the world literature. Eur Spine J. 1996;5(4):272-4. 3. Kawaguchi A, Miyamoto K, Wakahara K, et. al. Katsuji Shimizu Surgical treatment of multiple spinal canal stenoses associated with vitamin D-resistant rickets. J Clin Neuro. 2009;16(5): 717-719.