SCLEROTIC OSTEODYSPLASIAS

Abstract

Sclerotic osteodysplasias represent a group of relatively rare diseases; a common feature of them is the diffuse or local bone sclerosis. The reasons for their development are congenital abnormalities of endochondral and intramembranous bone formation either the hereditary nature or arising from spontaneous mutations. The main diagnostic problems arise when the differential diagnostic sclerotic dysplasias from various symptomatic osteopathies entailing bone sclerosis is needed. The aim of this research is the information about the group of rare congenital disorders characterized by diffuse and local osteosclerosis, and analysis of the literature on the diagnosis and differential diagnosis from other diseases and states, accompanied by compaction of bone tissue. A search of publications in English and Russian in electronic databases PUBMED, PUBMED CENTRAL, GOOGLE SHOLAR and eLIBRARY is performed. Keyword: sclerotic osteodisplasias, hyperostosis, osteopetrosis, osteopoikilosis, pyknodisostosis, melorheostosis, osteopathia striata, progressive diaphiseal dysplasia, diaphiseal sclerosis, enostosis and their Russian analogs for the period from 1953 to 2015. This clinical and radiological signs of osteopetrosis, osteopathia striata. osteopoikilosis, enostosis, piknodisostosis, progressive diaphyseal dysplasia, generalized hyperostosis cortical generalisata, melorheostosis and other forms of sclerotic osteodysplasias are presented. The main attention is paid to clinical and radiological diagnosis and differential diagnosis from osteoblastic metastases, primary benign and malignant bone tumors, chronic osteomyelitis and other diseases accompanied by local or diffuse osteosclerosis.