Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation...

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

Transcript of Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation...

Page 1: Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments.

Fibrodysplasia ossificans progressivaFibrodysplasia ossificans progressiva

Myositis ossificans progressivaMyositis ossificans progressiva

Big toe malformationCongenital

Heterotopic enchondral ossificationLater

Ligaments

Fasciae

Striated Muscles

With predictable pattern

Autosomal dominantBone morphogenetic protein ???

Page 2: Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments.

First ray shortening Delta shape proximal phalanx

I.P.fusion Hallux Valgus

Page 3: Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments.

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 caudalProximal to distal

Dorsal to ventral

PainErythematicWarmthTenderness

Like infectionAfter several weeksSubside but Induration increases

Intermediate lesion

12 weeksX Ray evident hard painless mass

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Advanced Subcutneous Ossification

Spontaneous fusion along post.elements & Lat. masses

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Short thumb metacarpal

Page 6: Fibrodysplasia ossificans progressiva Myositis ossificans progressiva Big toe malformation Congenital Heterotopic enchondral ossificationLater Ligaments.
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TreatmentTreatment

Supportive measuresAvoiding trauma

Padding

Nutrition

Research

Inhibition of Enchondral ossification & Angiogenic factors

BMP 4 inhibitors