16 year old male presents with extensive heterotropic ossification over the neck, back & shoulders and decreased chest movements. He gives history of progressive immobility since the age of 3 years. Which of the following statement about his affecting condition is not true ?
FIBRODYSPLASIA OSSIFICANS PROGRESSIVA : This rare condition, formerly known as myositis ossificans progressiva, is characterized by widespread ossification of the connective tissue of muscle, mainly in the trunk. It stas in early childhood with episodes of fever and soft-tissue inflammation around the shoulders and trunk. As this subsides the tissues harden and plaques of ossification extend throughout the affected areas. In the worst cases movements are restricted and the patient is severely disabled. Associated anomalies are shoening of the big toe and thumb. And the life expectancy of the children will be reduced. REF:Apley&;s system of ohopaedics- 9th edn - pg no 174.
They have a near normal life expectancy
They are predisposed to Pneumonia
They have sho hallux
Increased expression of BMP4 gene is seen
Important complications in orthopaedics