Sy M H, Diouf A, Diallo B K, Dansokho A V, Ndiaye A, Tall A, Badiane M, Sèye S I
Service d'Orthopédie-Traumatologie CHU Le DANTEC.
Dakar Med. 1999;44(1):126-30.
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.
进行性骨化性纤维发育不良或进行性骨化性肌炎或仍称为蒙克迈尔病是一种常染色体显性遗传的基因疾病。其发病率变化很大，无种族差异。作者报告了关于两名年轻非洲黑人患者的两个病例，涉及病因及较晚确诊情况。第一个病例是一名9岁男孩，其家族中无类似病史，因下颌骨葡萄球菌性骨炎入住颌面科。自3个月大时起，他就出现了一些椎旁、颈部、背部和腰部的肌肉骨化，且呈自发出现并逐渐进展。影像学结果显示出一些特定的骨骼病变，从而得以确诊上述疾病。第二个病例是一名24岁女性，她曾因21岁时右臂出现的非炎性肌肉肿胀接受过手术探查。三年后，她因口腔间隙受限及右侧腰部椎旁肿胀接受手术，而后者限制了脊柱的活动。影像学结果在手部和足部发现了一些特定的骨骼病变，这使得椎旁骨化与其纤维发育不良的起源相关联。蒙克迈尔病仍是一种易于进行放射临床诊断的疾病。关键在于在手部和足部出现特定骨骼异常之前尽早考虑到该病。功能甚至生命预后与结膜-肌肉骨化的范围和部位密切相关。
