Surgical Management in Cervical Spondylotic Myelopathy Due to Alkaptonuria

Sandal, Evren; Middlebrooks, Erik H.; Şenoğlu, Mehmet

doi:10.1016/j.wneu.2018.02.148

Cited by 6 publications

(1 citation statement)

References 14 publications

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“…Few articles to date have described the surgical management of patients with ochronotic cervical myelopathy. One case report described a patient with ochronotic cervical myelopathy caused by pannus formation at the atlantoaxial joint, which was treated surgically with decompression and posterior occipitocervical fixation with lateral mass screws [ 13 ]. Another case report described a patient with compression caused by thickened ligamentum flavum; this patient was treated surgically by modified cervical laminoplasty to relieve the compression [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Cervical Myelopathy Due to Ochronosis: An Intraoperative Suspicion

et al. 2020

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Patient: Female, 55-year-old Final Diagnosis: Alkaptonuria Symptoms: Neck pain Medication:— Clinical Procedure: — Specialty: Neurosurgery • Orthopedics and Traumatology Objective: Rare disease Background: Alkaptonuria (AKU) is a rare metabolic disease caused by a deficiency in homogentisic acid oxidase, which leads to the accumulation of homogentisic acid dark pigments in tissues such as bones, ligaments, and ten-dons. Long-term duration of this condition, termed ochronosis, can result in degenerative arthropathy involving the spine and large joints. Case Report: This report describes a 55-year-old Jordanian woman presenting with chronic neck and lower back pain. History, physical examination, and radiological imaging indicated cervical myelopathy and lumbar spine degeneration. Two-level anterior cervical discectomy and fusion was performed successfully. Intra-operatively, the cervical discs were observed to be black, suggesting a diagnosis of alkaptonuria, which was later confirmed by genetic testing. A detailed history and physical examination revealed the absence of classical features of AKU. Conclusions: Intraoperative detection of black disc material suggests the need for further tests to diagnose AKU, especially in indolent patients who did not have classical clinical features. Surgical management may improve outcomes in patients with cervical myelopathy due to ochronosis.

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Section: Discussionmentioning

confidence: 99%