[Fibrodysplasia ossificans progressiva. about one case]

ABSTRACT

Myositis ossificans progressiva is a rare autosomal dominant progressive disease of connective tissue. It consists on a heterotopic osteogenesis frequently associated to characteristic congenital malformations. We report a case of a 12-year-old girl which presented a heterotopic bone formation leading gradually to ankylosis. The diagnosis was suggested when we have discovered bone bridges between the rib cage and the superior limbs. Congenital malformations, most commonly of big toes and thumbs, are important criteria to draw on the diagnosis earlier and to avoid unnecessary biopsy or resection which may trigger off a flare of the disease. Therapeutic measures are essentially preventive and the prognosis is poor