Axial melorheostosis: A rare presentation

Abstract

Melorheostosis is a rare sclerosing bone dysplasia that most commonly affects the lower extremity long bones in a sclerotomal distribution. Melorheostosis of the spine is a particularly rare manifestation of this disease. In the appendicular skeleton, melorheostosis has a pathognomonic imaging appearance of flowing hyperostosis resembling melted candle wax flowing down the margins of a candlestick. In the spine, it can have a variety of imaging manifestations from unilateral focal sclerotic lesions resembling enostoses, to more bulky and deformative hyperostosis that span and fuse multiple adjacent spinal segments. This combination of nonaggressive radiologic features makes melorheostosis a particularly important diagnosis for radiologists to understand so that they may spare their patients unnecessary biopsy. Here we present the clinical features and computed tomography findings in a 33-year-old male with spinal melorheostosis involving the first and second cervical vertebrae.

Spinal Melorheostosis Associated with Intradural Fibrous Band and Extensive Lipomatosis Causing Thoracic Cord Tethering and Myelomalacia: A Unique Case Highlighting Importance of MRI in Management

“…Our case showed clear demarcation between involved and normal bone at all the levels supporting sclerotomal distribution of hyperostosis. 1 Most of the reported cases are incidentally detected 2 3 4 5 6 or presenting with vague back pain. 7 8 Cases with myelopathic symptoms secondary to the spinal cord or nerve root compression by hyperostotic bone in melorheostosis have been reported.…”

Bisphosphonate Therapy in the Management of Symptomatic Melorheostosis of Tibia

“…It presents with lesions mostly in the diaphysis of the long bone commonly involving the lower extremity sometimes associated with hyperpigmented sclerosis in overlying skin [ 6 ]. They may present in different forms as monostotic, polyostotic, or monomelic forms [ 7 ].…”