Melorheostosis involving the cervical and upper thoracic spine: radiographic, CT, and MR imaging findings. (1/5)
Melorheostosis, an uncommon mesenchymal dysplasia, rarely affects the axial skeleton. We describe the imaging findings of melorheostosis involving the cervical and upper thoracic spine. Radiographs and CT showed unilateral well-marginated undulating zones of cortical hyperostosis involving multiple vertebrae that were contiguous with a coalescent ossified right paravertebral mass. MR imaging showed zones of signal intensity void on all pulse sequences without contrast enhancement. Conservative management was elected because of lack of interval clinical and imaging changes for 8 years. (+info)Case report: severe melorheostosis involving the ipsilateral extremities. (2/5)
(+info)Atypical form of melorheostosis improved by pamidronate. (3/5)
BACKGROUND: Melorheostosis is a rare chronic bone disease of unknown etiology that often affects a single limb. AIM: Report a new case of melorheostosis of the ribs improved by pamidronate infusions CASE: A 36-year-old man without any medical history was admitted for a history of one month painful tumefaction on the 7th left rib. The diagnosis of melorheostosis of the rib and the tibia was made. Patient was treated by pamidronate infusions with useful and satisfactory outcome. (+info)Bone scintigraphy elucidates different metabolic stages of melorheostosis. (4/5)
Melorheostosis is a rare benign non-hereditary sclerosing dysplasia involving the bone, often in a sclerotomal distribution. we report the case of a 27 years old lady with painful swelling of the left hand and forearm lasting for almost 15 years. The patient experienced aggravation of symptoms and limitation of motion during the past two months. Radiographic assessment revealed hyperostosis involving the left 3(rd) and 4(th) metacarpal bones and corresponding digits as well as the left ulna and distal humerus, with no soft tissue ossification. Angiographic and blood pool images of bone scintigraphy showed increased activity of mid-metacarpal region, corresponding to the sclerotom C-8. Delayed static views showed increased radiotracer uptake of the left 4(th) metacarpal bone and the corresponding digit as well as the left ulna and humerus, but no abnormal osteoblastic activity of the 3(rd) left metacarpal and digit. Histopathologic assessment confirmed the diagnosis of Melorheostosis. The case confirms that even in the same sclerotomal distribution, the multiple foci of involvement can present in different metabolic stages. In fact, the disease does not progress uniformly and different lesions can be seen in dissimilar stages of activity. Hence, metabolic imaging can be important to unmask which of the radiographically detected bony lesions are metabolically active and have the potential to be the source of current patient's symptoms and which of them are old, metabolically inactive and silent lesions, which are not clinically relevant to the patient's complaints. (+info)Melorheostosis in children. Clinical features and natural history. (5/5)
Experience in the management of fourteen children with melorheostosis has been reviewed. The principal and presenting clinical features were unilateral soft-tissue contractures associated with inequality of limb length. In contrast to the disease in adults, pain occurred infrequently and was never intense. The average interval between the discovery of the clinical features and the correct diagnosis was six years. The distinctive radiographic feature in the child was an endosteal pattern of hyperostosis marked by streakiness of the long bones and spotting of the small. This differs from the usual subperiosteal or extracortical pattern of hyperostosis seen in adults. The surgical treatment of the contractures proved difficult and recurrence of the deformity was the rule. Distal ischaemia occurred when the chronically contracted and flexed joint was rapidly extended. (+info)Melorheostosis is a very rare, progressive bone disorder characterized by the thickening and hardening of the bones' outer covering (periosteum). The name "melorheostosis" means "melting bones," which describes the appearance of the long bones on X-rays. It resembles dripping candle wax flowing down the shafts of the bones.
The condition typically affects one side of the body, often involving the legs and arms, but can also affect the skull, spine, and ribs. The symptoms can vary widely, depending on the location and extent of bone involvement. They may include bone pain, deformities, limited mobility, joint stiffness, and skin changes over the affected bones.
The exact cause of melorheostosis is unknown, but it is not a hereditary condition. It is thought to be related to abnormal blood vessel formation during fetal development, leading to improper bone growth and development. There is no known cure for melorheostosis, but various treatments can help manage symptoms and improve quality of life. These may include pain management, physical therapy, surgery, and other supportive measures.