Merih Onal, Munir Demir Bajin and Taner Yilmaz
Accepted Abstracts: Surgery Curr Res
Introduction: Fibrodysplasia Ossificans Progressiva (FOP) is a rare, severely disabling, autosomal dominant disease characterized by recurrent painful episodes of soft tissue swelling and the development of heterotopic ossification. The main target is the axial musculature, but eventually ectopic bone formation occurs in the ligaments, the fascia, the tendons and the joint capsules. Small soft tissue traumas and intramuscular injections exacerbate this extraskeletal bone formation. Case: We presented a 16-year-old male patient who has an osseous lesion beginning from the left ramus mandible, extending along the sternocleidomastoid muscle, vertebral region and deltoid. A restriction in temporomandibuler joint movement was visible. There is a conflict about any surgery for improvement of his mouth opening. Discussion: Fibrodysplasia ossificans progressiva is heterotopic bone formation leads to locking in the joints and makes the movement impossible. Immobilization is gradually increased. Unfortunately, no effective medical therapy is known. These patients may require some oral surgical and anesthetic procedures. It is essential to decrease trauma. The importance of the decision of the surgery has been stressed . Conclusion: Pathophysiology of this disease will be better understood as the issues such as the mechanism triggering the inflammatory system, the interaction between the immune system and progenitor cells and microenvironmental formations are clarified and new developments will occur in antenatal diagnosis and therapy in the future.
Merih Onal has completed her medical education at Hacettepe University Medical Faculty and she has completed her specialist training at Hacettepe University Medical Faculty Ear Nose Throat Department on 2014. She has more than 5 publications in various journals.
