Living with Fibrodysplasia Ossificans Progressiva: Radiological Images of a Patient with Extensive Heterotopic Ossification.

Fibrodysplasia ossificans progressiva (FOP) is an exceptionally rare genetic disorder characterized by the progressive formation of heterotopic bone in soft tissues. Here, we present the radiological findings of an 18-year-old female diagnosed with FOP who had severe spinal and right-upper-limb abnormalities. Her SF-36 scores suggested significant impairment in physical function, affecting work and other regular daily activities. Radiographic evaluation with X-rays and CT scans revealed scoliosis and total fusion of almost all levels of the spine, with only a few disc spaces spared. A large mass of heterotopic bone was observed, corresponding to the location of the paraspinal muscles in the lumbar region, branching upwards and fusing with the scapulae on both sides. On the right side, this exuberant heterotopic bone mass fused with the humerus, resulting in a fixed right shoulder, while the rest of the upper and lower limbs are spared and have a range of motion. Our report highlights the extensive ossification that can manifest in patients with FOP, resulting in restricted mobility and a poor quality of life. While there is no definite treatment that can reverse the effects of the disease, preventing injuries and minimizing iatrogenic harm is of critical importance in this patient as inflammation is known to play a key role in triggering heterotopic bone. Meanwhile, ongoing research into therapeutic strategies holds the key to unlocking a potential cure for FOP in the future.

Telescoping vascularized fibular graft: a new method for treatment of congenital tibial pseudarthrosis with severe shortening.

Vascularized fibular grafts have proved reliable in the treatment of congenital pseudarthrosis of the tibia with a high success rate. However, severe shortening cannot be primarily corrected by this technique and requires a second-stage lengthening procedure. Ilizarov's method allows correction of shortening and axial malalignment together with the non-union. However, in the dysplastic type with severe shortening, corticotomy of the affected bone may result in delayed consolidation or recurrence of disease. In addition, the large distraction distance (equal to the amount of shortening plus the resulting defect after excision of the pseudorthrosis site) requires prolonged frame application, which may not be tolerated by the patient. We present a new technique combining vascularized fibular graft and Ilizarov distraction that allows simultaneous correction of shortening while treating the non-union in a single-stage operation. This method avoids corticotomy in the congenitally affected bone and markedly shortens the time of frame application.