Melorheostosis: Two atypical cases

Abstract

Melorheostosis is an uncommon mesenchymal dysplasia that rarely affects the axial skeleton. We describe two atypical cases of melorheostosis with classical imaging findings – the first one involving the cervico-dorsal spine with encroachment of left vertebral artery canal causing attenuation of the left vertebral artery and the second one of mixed sclerosing bony dysplasia (monomelic involvement coexisting with osteopoikilosis).

A Unique Case of Melorheostosis Presenting with Two Radiologically Distinct Lesions in the Shoulder

“…The diagnosis of melorheostosis is usually established through radiological findings: X-rays, computed tomography, magnetic resonance imaging, and bone scans; specific features have been described in every modality; and these findings gave a solid foundation for diagnosis in many studies [ 1 , 8 , 11 , 13 , 16 , 17 , 19 , 21 – 25 ]. A biopsy was carried out in cases of sinister or suspicious lesions and in many incidences as part of the surgical intervention [ 5 , 7 – 10 , 12 , 18 , 20 , 26 , 27 ], but this was not mandatory for every case.…”

Dripping Wax Bone Disease – Melorheostosis – A Rare Case Scenario

“…Skin changes may be seen in about 17% of cases and that may include hyperpigmentation of overlying skin and scleroderma [ 4 ]. Association of this condition is report with other conditions including neurofibromatosis, tuberous sclerosis, linear scleroderma, tricho-dento-osseous syndrome, rheumatoid arthritis, hypophosphatemic rickets, vascular malformations, and hemangioma [ 12 ] none of this features were present in our patient. Campbell (1968) described in his review of 14 cases arthrogryposis and scleroderma as the most common misdiagnosis Buschke-Ollendorff syndrome, another rare disease can include melorheostosis.…”

“…The onset of this rare disease is insidious, the first symptom is usually pain because of the sub periosteal bone formation and in our patient pain was the only complaint. The disease may extend along the affected cortex in a proximal to distal pattern, resembling the pathognomonic “dripping candle wax” appearance on an X-ray [ 12 ]. Soft-tissue abnormalities are common, and skin lesions resemble scleroderma [ 3 ].…”

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

“…7 8 Cases with myelopathic symptoms secondary to the spinal cord or nerve root compression by hyperostotic bone in melorheostosis have been reported. 9 10 11 12 13 14 …”