目的:检查临床特征,诊断和治疗,中国人群眼淀粉样变性及预后。
方法:进行回顾性病例系列研究。收集37例眼淀粉样变性患者的临床资料,诊断和治疗,并对预后进行总结分析。
结果:37例患者包括12例男性和25例女性,年龄在22至75岁之间。平均年龄49岁。临床体征和症状包括37例患者(100%)的结膜肿块,29例(61.9%)患者的眶周不适或疼痛,18例患者(23.8%),眼球突出或眼球移位3例(14.3%),2例患者眼球运动受限(9.52%),1例患者视力下降(4.76%),复视1例(4.76%)。共有29例患者仅结膜受累,8例患者伴有眼眶和结膜受累。结膜受累患者的主要治疗方法是手术切除。31名患者病情稳定,4名患者进展或复发,2例患者失访。
结论:眼部淀粉样变性最常见表现为眼睑或结膜肿块或弥漫性增厚,也可表现为眼眶肿块。诊断主要依靠组织病理学检查。手术是主要的治疗方法,为了明确诊断,指导进一步的治疗,保留函数,并防止威胁视力的并发症。术后密切随访是必要的。
OBJECTIVE: To examine the clinical characteristics, diagnosis and treatment, and prognosis of ocular amyloidosis in a Chinese population.
METHODS: A retrospective case series study was conducted. The clinical data of 37 patients with ocular amyloidosis were collected and the clinical characteristics, diagnosis and treatment, and prognosis were summarized and analyzed.
RESULTS: The 37 patients included 12 males and 25 females ranging in age from 22 to 75 years, with median age of 49 years. The clinical signs and symptoms included a conjunctival mass in 37 patients (100%), periorbital discomfort or pain in 29 patients (61.9%), ptosis in 18 patients (23.8%), exophthalmos or eyeball displacement in 3 patients (14.3%), restricted eye movement in 2 patients (9.52%), vision loss in 1 patient (4.76%), and diplopia in 1 patient (4.76%). A total of 29 patients had only conjunctival involvement and 8 patients had concomitant orbital and conjunctival involvement. The main treatment for patients with conjunctival involvement was surgical resection. Thirty-one patients had stable disease, 4 patients progressed or relapsed, and 2 patients were lost to follow-up.
CONCLUSIONS: Ocular amyloidosis most commonly presents as an eyelid or conjunctival mass or diffuse thickening and can also present as an orbital mass. Diagnosis is mainly dependent on histopathological examination. Surgery is the main treatment and is done to confirm the diagnosis to guide further treatment, preserve function, and prevent complications that threaten visual acuity. Close postoperative follow-up is necessary.