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Fibrodysplasia ossificans progressiva and associated osteochondroma of the coronoid process in a child
Authors:Chichareon V  Arpornmaeklong P  Donsakul N
Institution:Surgery Department, Medical School, Prince of Songkla University, Haad Yai, Thailand.
Abstract:The article reports the occurrence of osteochondroma in a fibrodysplasia ossificans progressiva patient. A 5-year-old boy presented with limited mouth opening and firm swelling of the right zygomatic complex area. The boy had bilateral hallux valgus of the great toes and heterotopic endochondral ossification of facial and neck regions. Associated osteochondroma of the coronoid process and aggressive heterotopic ossification of masticatory and neck muscles were found in response to traumatic injuries. Natural and clinical histories of fibrodysplasia ossificans progressiva were reviewed. An early diagnosis and avoidance of factors that aggravate ossification are key factors in reducing the expected degree of physical disabilities of patients. An early recognition of congenital skeletal deformities, early detection of abnormal ossification, and awareness of the disease by the involved physicians are important factors in the early diagnosis of the disease and in reducing any unnecessary trauma. Bone scintigrams and CT scans are effective noninvasive tools for an early detection of ossification and for monitoring the progression of the disease. Further investigation of its pathogenesis at a molecular level is important to understand better the nature of the disease and to develop an effective treatment protocol.
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