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Fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressiva. Myositis ossificans progressiva. Genetic. Big toe malformation. Congenital. Later. Heterotopic enchondral ossification. Ligaments. Fasciae. Striated Muscles. With predictable pattern. Mutation. Bone morphogenetic protein ???. Autosomal dominant.

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Fibrodysplasia ossificans progressiva

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  1. Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Genetic Big toe malformation Congenital Later Heterotopic enchondral ossification Ligaments Fasciae Striated Muscles With predictable pattern Mutation Bone morphogenetic protein ??? Autosomal dominant

  2. Clinical features Big toe anomalies First ray shortening Delta shape proximal phalanx I.P.fusion Hallux Valgus

  3. Heterotopic ectopic ossification Usually around5 yr. Shortly after birth Second decade Spontaneous or Blunt trauma Some nodules regress but usually progress to form mature bone Predictable pattern Axial to appendicular 12 weeks After several weeks Pain Erythematic Warmth Tenderness Cranial to caudal Subside but Induration increases Like infection X Ray evident hard painless mass Proximal to distal Intermediate lesion Dorsal to ventral

  4. Advanced Subcutneous Ossification Spontaneous fusion along post.elements & Lat. masses

  5. Short thumb metacarpal

  6. Extra articular ankylosis of diarthrodial joints Disability Hip Knee Spine Trauma Shoulder T.M joint New lesions Decubiti Third decade wheel- chair

  7. Trauma Dental care Venipuncture Bx. Surgery I.M Injection

  8. Treatment Supportive measures Avoiding trauma Padding Nutrition Research Inhibition of Enchondral ossification & Angiogenic factors BMP 4 inhibitors

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