fibrodysplasia ossificans progressiva

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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 ???

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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. - PowerPoint PPT Presentation

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Page 1: Fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressivaMyositis ossificans progressiva

Big toe malformationCongenital

Heterotopic enchondral ossificationLater

Ligaments

Fasciae

Striated MusclesWith predictable pattern

Autosomal dominantBone morphogenetic protein ???

Page 2: Fibrodysplasia ossificans progressiva

First ray shortening Delta shape proximal phalanx

I.P.fusion Hallux Valgus

Page 3: Fibrodysplasia ossificans progressiva

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 appendicularCranial to caudalProximal to distal

Dorsal to ventral

PainErythematicWarmthTenderness

Like infectionAfter several weeksSubside but Induration increases

Intermediate lesion12 weeksX Ray evident hard painless mass

Page 4: Fibrodysplasia ossificans progressiva

Advanced Subcutneous Ossification

Spontaneous fusion along post.elements & Lat. masses

Page 5: Fibrodysplasia ossificans progressiva

Short thumb metacarpal

Page 6: Fibrodysplasia ossificans progressiva
Page 7: Fibrodysplasia ossificans progressiva
Page 8: Fibrodysplasia ossificans progressiva

TreatmentSupportive measures

Avoiding trauma Padding

Nutrition

Research

Inhibition of Enchondral ossification & Angiogenic factors

BMP 4 inhibitors