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Fibrodysplasia Ossificans Progressiva (Signs and Symptoms (Jaw fixation,…
Fibrodysplasia Ossificans Progressiva
Anatomy
Muscle, ligaments, tendons, etc. replaced by bone
Commonly affects c-spine, neck and shoulders
Spreads throughout thorax and limbs
Signs and Symptoms
Jaw fixation
Malnourishment
Abnormal big toes (hallux valgus)
Joint immobility
Hearing loss
Alopecia
Respiratory failure
Radiographic Appearance
X-ray and CT are most useful
Demonstrate abnormal heterotopic ossification
Allows for 3D reconstructed models
Helps to determine progression and severity of disease
Treatment and Prognosis
No definitive treatment exists
Corticosteroids can help reduce inflammation but wont stop the progression of disease
Mis-diagnosis and delayed diagnoses are extremely common
Most patients don't live past 45 years old