背景:纤维发育不良是一种正常骨逐渐被未成熟的纤维骨组织所取代的疾病,所有良性骨肿瘤的发病率不到7%。这种疾病的管理对于整形外科医生和神经外科医生来说是一个挑战。
目的:为了描述诊断,治疗性的,在波哥大圣何塞医院整形外科服务处看到的颅面纤维发育不良患者的结果方法,哥伦比亚。
方法:这是一项描述性和回顾性病例系列研究,研究对象为2010年1月1日至2023年7月31日期间在圣何塞医院整形外科治疗的诊断为单骨和多骨纤维发育不良的患者。
结果:所有(n=10)患者均患有单骨性颅面部纤维发育不良。单骨纤维发育不良患者受影响最大的骨骼是I区骨骼(n=10,100%),其次是II区骨骼(n=2,20%)。I区和II区受累的患者表现出与磷和耳鸣相关的搏动性头痛(n=8,80%),以及与受影响的脸颊水肿相关的闭塞过程中的疼痛(n=5,50%)。体格检查显示,患有眶壁受累(I区骨骼)的患者患有眼逆视(n=7,70%)。关于患者接受的治疗,90%(n=9)的患者接受手术管理作为主要治疗,眼眶切开术,replacement,和/或使用植骨重建眼眶的顶部和侧壁,钻孔,than成形术,纤毛悬吊是最常执行的程序(n=6,60%)。在患者中,20%(n=2)需要再次干预。
结论:FD是一种缓慢进展的良性纤维骨病,个性化,和多学科的诊断和治疗,以获得良好的临床和手术效果。治疗的主要支柱是手术作为预防措施,因为它是重要的,以避免未来的功能改变,根据发育不良的位置,会导致相邻结构改变的高风险。
BACKGROUND: Fibrous dysplasia is a disorder in which normal bone is gradually replaced by immature fibro-osseous tissue, with an incidence of less than 7% of all benign bone tumors. The management of this disease is a challenge for plastic surgeons and neurosurgeons.
OBJECTIVE: To describe the diagnostic, therapeutic, and outcome approach of patients with craniofacial fibrous dysplasia seen at the Plastic Surgery Service of the Hospital San José in Bogotá, Colombia.
METHODS: This is a descriptive and retrospective case series study of patients diagnosed with monostotic and polyostotic fibrous dysplasia treated at the Plastic Surgery Department of Hospital San José during the period from January 1, 2010, to July 31, 2023.
RESULTS: All (n=10) of the patients had monostotic craniofacial fibrous dysplasia. The most affected bones in patients with monostotic fibrous dysplasia were zone I bones (n=10, 100%), followed by zone II bones (n=2, 20%). Patients with zone I and II involvement manifested throbbing headaches associated with phosphenes and tinnitus (n=8, 80%) and pain during occlusion associated with edema in the affected cheek (n=5, 50%). Physical examination showed that patients with orbital wall involvement (zone I bone) had ocular dystopia (n=7, 70%).Regarding the treatment received by the patients, 90% (n=9) of the patients received surgical management as primary treatment, with orbitotomy, replacement, and/or remodeling of the roof and lateral wall of the orbit with bone graft, drilling, canthoplasty, ciliary suspension being the most frequently performed procedure (n=6, 60%). Of the patients, 20% (n=2) required reintervention.
CONCLUSIONS: FD is a slowly progressive benign fibro-osseous disease that requires a timely, individualized, and multidisciplinary diagnosis and treatment to obtain favorable clinical and surgical results.The mainstay of treatment is surgery as a preventive measure since it is important to avoid future functional alterations that, depending on the location of the dysplasia, would cause a high risk of alteration of adjacent structures.