[Fibrodysplasia ossificans progressiva or Munchmeyer disease apropos of 2 cases].
Fibrodysplasia ossificans progressiva or myositis ossificans progessiva or still Munchmeyer disease is a genetic ailment with dominant autosomic transmission. It includes a high rate of change and doesn't appear any race. The authors gave an account of two remarks about it on a causal and late discovery upon two young black Africans. The first observation is a nine year-old-boy, without any similar family previous history and who has been hospitalized in maxillo-facial milieu for a mandible osteitis staphiloccocus. It showed some muscular ossifications of paravertebral, cervical, dorsal and lumbar nature--from a spontaneous appearance and evolving progressively since the age of three months. The radiographic results displayed some specific bones disorders which enable to retain the diagnosis above. The second observation is the case of a twenty four-year-old-woman who has previously benefitted from a surgical exploration a non-inflammatory muscular tumefication at the right arm which occurred at the age of twenty one. Three years later, she took surgery for the restriction of the oral gap and a right hand side lumbar paravertebral tumefaction which was thus restricting the mobility of the rachis. The radiological results have found some specific bone disorder++ at the hands and the feet which enable to link the paravertebral ossifications to their fibrodysplasic origin. Munchmeyer disease remains a affection of easy radio-clinical diagnosis. The essential point is to precociously think of it before the specific osseous anomalies occurring at the level of the feet and the hands. The functional or even vital prognosis remains closely linked to both the important and the topography of conjunctivo-muscular ossifications.
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Service d'Orthopédie-Traumatologie CHU Le DANTEC. mhsy@ucad.refer.sn
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D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D009221:Myositis Ossificans / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000235:genetics; D011859:Radiography