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“Black disc” tandem spinal stenosis in ochronotic arthropathy

Alkaptonuria (AKU) is a rare hereditary autosomal recessive disorder characterized by a defect in the gene for enzyme homogentisate 1,2-dioxygenase resulting in the metabolic disorder of phenylalanine and tyrosine. Tandem spinal stenosis refers to spinal canal diameter narrowing in at least 2 distinct regions of the spine, most commonly the lumbar and cervical regions. This entity can be an asymptomatic radiographic finding, or it can present with severe myelopathy and lower-extremity symptoms. On review of literature, in PubMed and Google Scholar, there were no cases of ochronotic tandem spinal stenosis. This is the first case to be reported in the world literature of ochronotic tandem spinal stenosis operated surgically. We report a rare case of a patient suffering from ochronosis with tandem spinal stenosis who was diagnosed during the late course of disease once the myelopathy had set in resulting in increased neck and back pain and associated with walking trouble and clumsiness in hands. A 60-year-old hypertensive farmer suffering from late-stage ochronotic arthropathy with tandem spinal stenosis with cervical spondylotic myelopathy with lumbar disc protrusion with radiculopathy was surgically treated with single-stage simultaneous decompression surgery. His early follow-up showed marked improvement. AKU is a rare metabolic genetic disorder that is caused by the deficiency of homogentisic acid oxidase, affecting multiple organ systems in the body. Detailed history can help diagnose it early and accurately. Ochronotic arthropathy can cause tandem spinal stenosis by ligamentum flavum thickening and disc protrusions, which if overlooked can lead to serious complications. Meticulous preoperative and intraoperative planning with expeditious implementation helps to achieve a successful outcome after a single simultaneous procedure.