Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments.

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Fibrodysplasia ossificans Progressiva (FOP)
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Presentation transcript:

Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments Fasciae Striated Muscles With predictable pattern Autosomal dominant Bone morphogenetic protein ???

First ray shorteningDelta shape proximal phalanx I.P.fusion Hallux Valgus

Usually around 5 yr. Shortly after birth Second decade Spontaneous or Blunt trauma Some nodules regress but usually progress to form mature bone Axial to appendicular Cranial to caudal Proximal to distal Dorsal to ventral Pain Erythematic Warmth Tenderness Like infection After several weeks Subside but Induration increases Intermediate lesion 12 weeks X Ray evident hard painless mass

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

Short thumb metacarpal

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