CAMURATI ENYELMANN سندرم اسكلتي

CAMURATI ENGELMANN SYNDROME

Camurati Engelmann Syndrome is a skeletal developmental disease which is inheriteds by a dominant Autosomal gene. In this syndrome , diaphysis of long bones become wide and fusiform and these is periosteal ,and endosteal new bone formation without metaphyseal and epiphyseal involvment. The reported case is a young adult 14 years old. The chief complain are upper and lower limbs pain, muscular weakness and fatique while walking there was not mental retardation, and muscular atrophy on examination. X-Ray of tibia and femure showed increase bone density and fusiform shape tibia. Lab investigation showed increases Alp. Considering patient,s history, clinical examination and para clinical investigation the diagnosis was comurati Engelmann or progressive diaphyseal dysplasia.