1/10. Increased procollagen alpha1(I) mRNA expression by dermal fibroblasts in melorheostosis.We report a patient with melorheostosis in whom increased procollagen alpha1(I) mRNA expression and alpha1(I), alpha2(I) and alpha1(III) collagen secretion were observed in dermal fibroblasts obtained from a skin biopsy overlying the involved bone. The patient was a 53-year-old man with melorheostosis lesions over the left knee joint. Multiple pigmented macules were present on the medial aspect of the lower left leg. hyperpigmentation of the basal keratinocytes, thick-walled vessels in the reticular dermis, and proliferation of normal-appearing collagen around the hair follicles were observed histologically.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
2/10. Painful lumbosacral melorheostosis treated by fusion.STUDY DESIGN: A case report of low back pain associated with a diagnosis of melorheostosis of the lumbosacral spine. OBJECTIVE: To describe a rare presentation of melorheostosis and subsequent successful surgical treatment. SUMMARY OF BACKGROUND DATA: melorheostosis is a rare condition and spinal pain has not been described in association with the condition. methods: A patient with disabling low back pain and suspected melorheostosis of the lumbosacral spine responded favorably to diagnostic facet joint blocks. Treatment was lumbosacral fusion and biopsy of the abnormal bone. The densely sclerotic bone presented technical difficulties requiring modification of surgical technique. RESULTS: Dramatic pain and disability reduction occurred following lumbosacral fusion. Histologic examination was consistent with melorheostosis. CONCLUSION: melorheostosis rarely causes severe low back pain that can respond favorably to fusion surgery.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
3/10. Ilizarov treatment for equinoplanovalgus foot deformity caused by melorheostosis.melorheostosis often is disabling because of progressive contracture of the joint and soft tissue involved. Operative intervention often is hazardous and patients also have recurrences. A 14-year, 2-month-old boy, who presented with a recurrent equinoplanovalgus deformity of the right foot caused by melorheostosis, was treated successfully using the ilizarov technique, including distraction osteotomy in the calcaneus. The patient had a painless, plantigrade, and functional foot at age 18 years.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
4/10. melorheostosis of the tenth and eleventh thoracic vertebrae crossing the facet joint: a rare cause of back pain.melorheostosis affecting the axial skeleton is a rare condition. We present a case affecting a single thoracic zygoapophyseal (facet) joint that proved to be a diagnostic challenge. CT, MRI and radionuclide imaging with surgical and histopathology findings are discussed.- - - - - - - - - - ranking = 5keywords = joint (Clic here for more details about this article) |
5/10. Soft tissue contractures from melorheostosis involving the upper extremity.melorheostosis is a rare disorder characterized by a linear hyperostosis of cortical bone, joint pain, stiffness, deformity, and limited motion. In the hand, the disease is often disabling because of progressive contractures of the joints and soft tissues involved. Two cases of melorheostosis involving the radial side of the hand and upper extremity are reported. Splinting or surgical release did not result in permanent correction of the soft tissue contractures. Retraining patients to use their unaffected hand should be considered early in the course of the disease.- - - - - - - - - - ranking = 2keywords = joint (Clic here for more details about this article) |
6/10. melorheostosis of Leri: report of a case in a young African.melorheostosis of Leri is a non-familial condition of hyperostosis of the cortical bone that usually presents unilaterally in long bones of the upper and lower limbs, but may also present in vertebra, ribs, skull and jaw. The incidence of this disease is quite rare, only about 300 cases have been reported worldwide. We present a case, which may be the first documented case in sub-Saharan africa. CLINICAL PRESENTATION: S.K. is a 14 year old male student who presented to the hospital with an 18 month history of persistent pain in the joints of the right upper limb and a limb length discrepancy since birth which has worsened with growth. Examination revealed generalised hypoplasia of the right upper limb with shortening of the limb and atrophy of the muscles, also hypoplasia and contracture of the thumb was observed. The radiographs of the limb showed multiple areas of dense hyperostosis and scleroderma, which showed a linear distribution along the radial half of the bones. CONCLUSION: In children presentation of melorheostosis, is more likely be as limb length discrepancy, deformity or joint contractures which may be seen before radiographic evidence of any bony changes. Improvement in imaging techniques will therefore result in early diagnosis and greater success with conservative management. Also the increased frequency of tumours necessitates long-term follow up. Keywords: melorheostosis, scleroderma.- - - - - - - - - - ranking = 2keywords = joint (Clic here for more details about this article) |
7/10. melorheostosis in a pediatric patient.melorheostosis is a nonhereditary and uncommon condition that can affect both adults and children. It can appear on radiographs as increased sclerosis on bones of the upper and lower extremities and may mimic other bony conditions such as osteopoikilosis, osteopetrosis, arthrogryposis multiplex congenita, and osteopathia striata. The sclerotic appearance can differ greatly between adults and children. The skin and subcutaneous tissues may be affected by fibrosis, resulting in contractures of joints and limbs that lead to deformities and limb-length discrepancies. This article reviews the literature on melorheostosis and describes a case in a 10-year-old boy.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
8/10. melorheostosis--an unusual cause of amputation.A 24-year-old female developed, in infancy, progressive right upper and lower limb muscle and soft tissue contractures and had a diagnosis of melorheostosis made on X-ray and pathological specimens. At the age of 11 years she began to have pain in the right hip and lower limb and this later became the dominant feature. She ultimately required amputation through the right hip joint and prosthetic fitting. She now has independent mobility with her prosthesis and has had no recurrence of pain. Her right arm remains flexed, shortened and contracted, but some hand function is retained. A review of the medical literature is discussed.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
9/10. melorheostosis on three-phase bone scintigraphy. Case report.melorheostosis is a benign sclerosing bone dysplasia with a very unusual and characteristic roentgenographic appearance. Its scintigraphic appearance also is characteristic, with asymmetric cortical activity that may cross joints to involve contiguous bones. The authors report the appearance of melorheostosis on angiogram and blood pool phases of three-phase bone scintigraphy.- - - - - - - - - - ranking = 1keywords = joint (Clic here for more details about this article) |
10/10. Soft tissue knee contracture of the knee due to melorheostosis, treated by total knee arthroplasty.melorheostosis is a rare condition which can cause soft tissue joint contractures. We present a case of melorheostosis causing disabling knee joint contracture, treated successfully by total knee arthroplasty.- - - - - - - - - - ranking = 2keywords = joint (Clic here for more details about this article) |
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