Department of Oral, Teeth and Jaw Radiology, University of Ankara, Faculty of Dentistry, Ankara, Turkey.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2012 Mar;113(3):404-9. doi: 10.1016/j.tripleo.2011.03.011. Epub 2011 Jun 12.
Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary connective tissue disease characterized by the progressive ectopic ossification of ligaments, tendons, and facial and skeletal muscles throughout life. Symptoms begin in childhood as localized soft tissue swellings. Immobility and articular dysfunction appear with involvement of the spine and proximal extremities. The temporomandibular joint (TMJ) is a critical component involved in the maxillofacial region, resulting in severe limitation of masticatory function, although TMJ involvement is rare. We report a case of FOP presenting as severely limited TMJ movements owing to ectopic calcification of the left coronoid process. In addition to the clinical examination, panoramic radiography and cone-beam computed tomography images were obtained. The case is presented and the clinical and imaging findings, differential diagnosis, and treatment modalities are discussed.
进行性骨化性纤维发育不良(FOP)是一种罕见的遗传性结缔组织疾病,其特征是韧带、肌腱以及面部和骨骼肌肉在整个生命周期中进行性异位骨化。症状始于儿童时期的局部软组织肿胀。随着脊柱和四肢近端的受累,出现活动受限和关节功能障碍。颞下颌关节(TMJ)是颌面部的一个重要组成部分,尽管 TMJ 受累很少见,但会导致咀嚼功能严重受限。我们报告了一例 FOP 病例,其表现为由于左侧喙突的异位钙化导致 TMJ 运动严重受限。除了临床检查外,还获得了全景放射摄影和锥形束计算机断层扫描图像。本文介绍了该病例,并讨论了其临床和影像学表现、鉴别诊断和治疗方法。