Stone Man Syndrome / Fibrodysplasia Ossificans Progressiva (FOP)

Fibrodysplasia ossificans progressive (previously known as myositis ossificans progressiva) also termed as stone man syndrome (1) is a extremely rare disorder of connective tissue. The disorder is characterized by deformed big toe bilaterally at birth which is the hallmark of the disorder associated with progressive heterotopic ossification of tendon, ligaments, fascia, and skeletal muscle occuring in predictable anatomic pattern . frequently the first sites of heterotopic bone formation occur within fibroproliferative lesion on the upper back and neck.(2) First flare up usually occurs before 10 years of life. Bone growth occurs from top to downwards typically involved in first dorsal, axial, cranial, and proximal region of the body. It doesn’t necessarily occurs in the order due to injury caused flare ups. Diaphragm, tongue, smooth muscle,myocardium, extraoccular muscle are spared.(3). Injury caused incorrectly expression of enzyme for bone repair by injured tissue and muscle which causes lymphocytic recruitment and excessive formation of BMP 4 which results in bone formation.(4) The disease is extremely rare only a few case have been reported in India even Globally. We came across a typical case of FOP in 3.5 year old female child with extensive involvement . extensive involvement at this age is a very rare presentation.