Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva

Fibrodysplasia ossificans progressiva (FOP) is the severest disease of ossification in the human. It forms an exoskeleton gradually. This process is started for a nodule that ossifies as days goes by. The initial pathway cannot be stopped unless early immunosuppress management is started very early after the aggression has been received. These aggressions may vary from contusions, viral illnesses, surgery, and other minor trauma.Because of this process, the FOP patient gradually worsens and decreases its autonomy as described from ability to walk, dress, or even to eat and breathe.

An 11-year-old female comes to evaluation for multiple ossifications, which worsen her autonomy and the ability to walk. The last ossification keeps the knee bent at 90° by a bony bridge from the femur to the tibia. To try to walk, it is necessary to flex hip, and ankle, with tilting of the pelvis, with pain and loss of autonomy. Due to the characteristic ossification pattern, FOP is diagnosed and the possibility of surgery to prolong autonomic gait is suggested. The procedure is performed under immunosuppression, and the knee is extended to a position that allows the patient to walk again freely. The steroid management is continued the convenient time to avoid exacerbation of the disease at a distance, and suspend it definitively afterward.

The FOP is adding an exoskeleton and its episodes of exacerbation end in new ossifications that deteriorate the quality of life. Once the ossifications have appeared, it is not possible to stop or reverse the process. Performing an osteotomy to prolong gait is a risky procedure. However, the present study shows that final ossification is expected at the point of resection of the extracted bone and suggests that immunosuppressing the patient could prevent distant exacerbations. The risk-benefit ratio of the procedure may be favorable if it is performed at a quiescent stage of the disease.