Friday, October 26, 2012

Fibrodysplasia Ossificans Progressiva

1. Bilateral Hallux Valgus - with malformed first metatarsal and phalanges.






2. Medial cortical thickening with formation of Pseudo-exostosis ( Calcification of Gracilis / Semitendinosus tendons).

3. Shortened first metacarpals

4. Usually the hetrotopic ossification begins in the soft tissues of neck, even after trivial trauma.

5. FNAC is contraindicated as it can result in permanent deforming ossification.

6. F.O.P is an AD disease with complete penetrance,, but with variable expressivity.

7. Can be diagnosed by the HALLUX VALGUS. Genetic testing is available.

8. Small vertebral bodies, enlarged pedicles, small spinal canal may be seen.














9. Starts by 2-5yrs, Death by 3rd decade by recurrent pulmonary infections due to prevention of expansion of thoracic cage (Impairing Respiration).

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