Klempous Joanna, Kuźniar Jolanta, Sztuka Aleksandra, Jaworska Marzena
Katedry i Kliniki Otolaryngologii Akademii Medycznej we Wrocławiu.
Wiad Lek. 2006;59(1-2):125-7.
Fibrous dysplasia (FD) is a benign but slowly progressive disorder of bone in which normal cancellous bone is replaced by immature woven bone and fibrous tissue. Fibrous dysplasia is a very uncommon disorder and the total number of cases is not known. It is usually diagnosed in children and young adults. Some patients have only one bone affected (monostotic), whereas other patients have numerous bones affected (polyostotic). Multiple affected bones are often found on one side of the body. The craniofacial involvement causes very difficult therapeutic problem due to localisation and uncontrolled proliferation followed by compression, both resulting in facial asymmetry, pain, cranial nerve deficiencies, alterations in hearing and loss of vision. Treatment of FD depends on the associated symptoms and may include a range of therapies from observation to complete surgical excision. We describe a rare case of polyostotic fibrous dysplasia with craniofacial localisation associated with the involvement of the humerus in a 65-year-old female patient. The first symptoms in the patient were noted 4 years ago. The diagnosis was based on clinical examination, X-ray, CT and histological examination.
骨纤维发育不良(FD)是一种良性但进展缓慢的骨疾病,其中正常的松质骨被不成熟的编织骨和纤维组织所取代。骨纤维发育不良是一种非常罕见的疾病,病例总数尚不清楚。它通常在儿童和年轻人中被诊断出来。一些患者只有一块骨头受累(单骨型),而其他患者有多处骨头受累(多骨型)。多处受累的骨头通常位于身体的一侧。颅面部受累由于其位置和不受控制的增殖随后导致压迫,会引发非常棘手的治疗问题,这两者都会导致面部不对称、疼痛、颅神经功能缺损、听力改变和视力丧失。骨纤维发育不良的治疗取决于相关症状,可能包括从观察到完全手术切除等一系列治疗方法。我们描述了一例罕见的多骨型骨纤维发育不良病例,该病例颅面部定位,同时累及一名65岁女性患者的肱骨。患者的最初症状在4年前被注意到。诊断基于临床检查、X线、CT和组织学检查。