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Results for “MELORHEOSTOSIS”

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Vascular anomalies in association with osteodystrophies--a spectrum.

A wide spectrum of osseous abnormalities has been reported in association with vascular lesions. Enchondromas, bone hypertrophy, bone lysis, and sclerotic bone lesions have all been reported in association with hemangiomas, lymphangiomas, varicosities, and arteriovenous fistulae. These associations can be grouped into four major syndromes: Maffucci syndrome, mixed sclerosing bone dystrophy with angiodysplasia, congenital angiectatic hypertrophy (Klippel-Trenaunay-Weber syndrome), and massive osteolysis (Gorham syndrome).

Adult↗

Mixed sclerosing bone dystrophy. Report of a case and review of the literature.

A 63-year old woman was admitted because of hip pain. Radiographs showed multiple round and oval sclerotic lesions involving humeral heads, pelvis, vertebral bodies and both femoral bones. Diaphyseal periosteal proliferation was found in metatarsal bones. A diagnosis of mixed sclerosing bone dystrophy was made. We review clinical, epidemiological and radiological findings of this entity.

Aged↗

Molecular and radiological diagnosis of sclerosing bone dysplasias.

Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights.

Bone Density↗

Osteopoikilosis: report of a clinical case and review of the literature.

Osteopoikilosis is an uncommon benign sclerosing bone dysplasia. Herein we present a case of osteopoikilosis with characteristic roentgenographic findings and clinical findings of cervical myelopathy. The importance of the differential diagnosis in symptomatic cases of osteopoikilosis is emphasized with the review of the literature.

Bone Neoplasms↗

Case of the day.

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Adolescent↗

Cranio-facial and humeral melorrheostosis.

First described by LERY and JOANNY in 1992, melorrheostosis is an uncommon linear hyperostosis of unknown aetiology, which may be associated with soft tissures changes. Although all bones may be affected, cranio-facial involvement is very rare. Only six such cases have been found in the literature. This report describes a case of cranio-facial and left humeral melorrheostosis with symptomatic radial nerve involvement. Resection of the melorrheostotic bone was performed because of involvement of the radial nerve.

Adult↗