Çakan Mustafa, Aktay-Ayaz Nuray, Karadağ Şerife Gül, Keskindemirci Gonca
Clinics of Pediatric Rheumatology, Kanuni Sultan Süleyman Research and Training Hospital, Istanbul, Turkey.
Clinics of Pediatrics, Kanuni Sultan Süleyman Research and Training Hospital, Istanbul, Turkey.
Turk J Pediatr. 2018;60(5):593-597. doi: 10.24953/turkjped.2018.05.021.
Çakan M, Aktay-Ayaz N, Karadağ ŞG, Keskindemirci G. An extreme entity in differential diagnosis of musculoskeletal involvement-fibrodysplasia ossificans progressiva: a case based review. Turk J Pediatr 2018; 60: 593-597. Fibrodysplasia ossificans progressiva is one of the most devastating disorder of mankind characterized by progressive heterotopic ossification. Apart from hallux valgus, other symptoms start to develop in the first decade of life. The initial symptoms are tumefactive lesions on the back that gives an impression of benign or malignant tumoral lesion. It may cause restricted motion of the neck and shoulders and magnetic resonance imaging of the lesions may be reported as myositis or myofasciitis and these children may be referred to rheumatologists. Currently there is no definitive treatment of the disease but the most important issue in these patients is primum non nocere, because any invasive procedure could potentially trigger a flare and heterotopic calcification. Herein, we present a young case of fibrodysplasia ossificans progressiva to remind the typical signs and symptoms of the disease to all clinicians caring for children.
恰坎·M、阿克泰-阿亚兹·N、卡拉达格·ŞG、凯斯金德米尔西·G。肌肉骨骼受累鉴别诊断中的一种极端情况——进行性骨化性纤维发育不良：基于病例的综述。《土耳其儿科学杂志》2018年；60:593 - 597。进行性骨化性纤维发育不良是人类最具破坏性的疾病之一，其特征为进行性异位骨化。除拇外翻外，其他症状在生命的第一个十年开始出现。最初的症状是背部的肿胀性病变，给人一种良性或恶性肿瘤性病变的印象。它可能导致颈部和肩部活动受限，病变的磁共振成像可能报告为肌炎或肌筋膜炎，这些儿童可能会被转诊给风湿病学家。目前该疾病尚无确切的治疗方法，但这些患者中最重要的问题是“首要的是不伤害”，因为任何侵入性操作都可能引发病情加重和异位钙化。在此，我们展示一例进行性骨化性纤维发育不良的年轻病例，以提醒所有照顾儿童的临床医生该疾病的典型症状和体征。
