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Age / Sex : 59 / F
Chief complaint: pain in neck, left shoulder with radiating pain in left elbow and dorsum of wrist
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Two weeks later, you can see the final diagnosis with a brief discussion of this case. (Please submit only one answer)
Courtesy: Yeo Ju Kim, Inha University Hospital
Diagnosis: Melorheostosis
Discussion
Findings:
There is a wavy hyperostosis on the internal and external aspect of bone involving left lamina of C6 and C7 across the left facet joint of C6-7. This hyperostosis is protruded into central canal and the inlet portion of the left neural foramen of C6-7. It also reaches near the posterior surface of C6 and C7 vertebra bodies resulting in pressure erosion into the left posterior surface of C6,7 vertebral bodies. Consequently, the spinal cord is deviated toward right side with severe neural foraminal compromise at left C6-7
Differential Diagnosis:
tumoral calcinosis, tumoral calcium pyrophosphate dihydrate deposition disease (CPPD), ivory osteomas, heterotopic ossification (myositis ossificans), and parosteal and periosteal osteosarcoma
Diagnosis:
Melorheostosis involving left lamina of C6 and C7 across the left facet joint of C6-7
Discussion:
Melorheostosis (Leri disease) is a rare nonhereditary sclerosing mesenchymal dysplasia of bone. It is commonly present as wavy longitudinal bars of hyperostosis resembling molten wax flowing down one side of a candle (so-called flowing candle wax appearance). One or multiple adjacent bones are commonly involved, often in a sclerotomal distribution. Melorheostosis commonly affects limb bones and may extend across synovial joints. Lesions may occasionally be associated with skin lesions, vascular anomalies, and joint contractures, although they were not present in this case. Histologic findings include variable degrees of cortical thickening consisting of chondroid islands surrounded by mature lamellar and woven bone, as well as adjacent zones of fibrocartilage with irregular surface fibrillation. Soft tissue abnormalities consisting of osseous, chondroid, vascular, and fibrocartilaginous tissue have been reported in 76% of cases of melorheostosis.
Although melorheostosis is rarely occurred in the spine, the imaging features of this case, such as wavy hyperostosis with extension across the facet joint, are sufficient to make a diagnosis and to differentiate from other diseases. Although melorheostosis is rare in the axial skeleton, it should be a definite consideration in the differential diagnosis of unilateral or segmental lesions of cortical hyperostosis in the spine because accurate detection can prevent an unwarranted biopsy.
References:
1. Motimaya AM(1), Meyers SP. AJNR Am J Neuroradiol. 2006 Jun-Jul;27(6):1198-200.
2. Zeiller SC, Vaccaro AR, Wimberley DW, et al. Severe myelopathy resulting from melorheostosis of the cervicothoracic spine. A case report. J Bone Joint Surg Am 2005;87:2795–62
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