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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.
The authors describe a case of melorheostosis localised in the hand. Melorheostosis is an unusual affection. The first signs appear in some cases at birth, but the disease is often diagnosed during the fourth decade. Clinical symptoms are pain, ankylosis, the deformation provoked by the lesion of soft tissues and especially limitation of motion. Radiographs permit the diagnosis: opacities parallel to the diaphysis with a "monomelic" topography. The natural history of the condition is characterized by the possibility of extension of the disease. Operative treatment is rarely indicated.
Melorheostosis is a rare bone disease of unknown etiology. It is characterized by tense and erythematous skin, contractures and shortening of the affected limbs. Radiographs reveal hyperostosis in long bones resembling melting wax, and spotty or patchy endostic deposits in short bones. An 11-year-old girl showed, since birth, contractural deformities in the right hand without bone abnormalities. At the age of 12 months, she complained of soft tissue tightness in the right buttock. At 3 years, limitation of flexion and extension of the right knee was evident. Radiographs revealed findings typical of melorheostosis.
The association of linear scleroderma with melorheostosis is very rare. Until now, only a few cases have been reported in the English literature. We describe a case of melorheostosis of a rib associated with overlying skin changes.
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.
Summary. Melorheostosis is a sclerosing disease commonly affecting the bone, rarely soft tissue. Etiology is unknown. It causes uncharacteristic symptoms such as pain, joint stiffness, soft tissue contractures, and limb deformities. Standard radiology reveals the diagnosis. Until now there is no standard therapy. Melorheostosis is a rare bone dysplasia and important as a differential diagnosis for tumorous changes in the limb or in painful joints.
INTRODUCTION: Melorheostosis is a rare bony dysplasia and often recognised just sporadically by chance. CASE REPORT: We present a case of a 15 year old girl who presented a melorheostosis of the left foot. After birth there was recognized a shortening and deformity of the 2nd toe on the left foot. Furthermore she had an interphalangeal hallux valgus that displaced the 2nd toe increasingly. Thus in the last years there were more and more difficulties to wear normal shoes. Conservative therapy was not successful. We performed a lengthening extending osteotomy of the 2nd toe (a modified Weil osteotomy) and an Akin osteotomy of the interphalangeal hallux valgus. Since the surgical procedure the patient is out of any complaints. DISCUSSION: We demonstrate the radiologic and histologic findings and discuss the relevant literature and possible etiology.
Melorheostosis is a rare monostotic or polyostotic bone disease of unknown etiology. It is characterized by a slowly progressing linear hyperostosis, fibrosis of the skin and subcutaneous tissues, contractures of joints and aching pain. The lower extremities are involved much more often than the upper extremities. The distribution of the lesion usually follows sclerotomes. Bone scanning complements standard radiologic studies and permits evaluation of the extent and activity of the disease. Here we report two cases of melorheostosis. Conservative treatment was disappointing. Thus we recommend the excision of the exostosis.
Melorheostosis is a rare bone disorder, usually affecting the long bones and adjacent soft tissue. It was originally described by Leri and Joanny in 1922, after its classic x-ray features of flowing hyperostosis resembling dripping candle wax. There have been fewer than 10 reported cases of craniofacial involvement, and in most instances these have also involved the appendicular skeleton. The authors report a case of melorheostosis with isolated craniofacial involvement, describe the clinical course and radiologic and histologic features, and review the pertinent literature.
A case of melorheostosis that was first detected by bone scan during the staging evaluation of a patient with metastatic lung carcinoma is reported. The unique pattern of skeletal involvement of melorheostosis allowed its presumptive diagnosis on bone scan, with subsequent radiographic confirmation. A review of this disorder is presented.
The multiphase bone scan features of melorheostosis are presented. Early increased tracer delivery demonstrated by blood pool imaging supports the concept of ongoing accelerated bony metabolism as a cause of tracer uptake within the osteosclerotic lesions of melorheostosis. There is excellent correlation between the scintigraphic and radiographic distribution of these lesions.
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.
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.
A case of melorheostosis in a 57-year-old Japanese woman is presented. This case is noteworthy because of its unusual radiographic features and local findings and its unusually long follow-up period of more than 29 years. Melorheostosis progresses very slowly over a long period of time, and can show bizarre features, simulating malignant tumor.
Melorheostosis is a benign, rare, congenital disorder of hyperostosis of one or more bones. Associated soft tissue abnormalities are often observed. A case of melorheostosis with minimal change nephrotic syndrome, mesenteric fibromatosis and capillary haemangiomas is reported. A congenital mesenchymal defect occurring early in embryonic life is suggested.
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.
The authors present a review of the literature and the diagnostic difficulties in a case of melorheostosis in a 6 year old boy. Melorheostosis is characterized by longitudinal foci of subcutaneous sclerosis, limitation of motion in the joints and swelling of the knee and ankle joints. Radiologically numerous foci of hyperostosis similar to wax flowing down a burning candle have been noted.
Traditional conservative and surgical methods of treatment of melorheostosis, such as manipulations, plaster casts, soft-tissue releases, capsulotomies, and osteotomies cause a high recurrence rate. In a 12-year-old girl, flexion contracture of a knee with limb inequality caused by melorheostosis was corrected successfully with the Ilizarov distraction apparatus.