Orbital pseudotumor: distinct diagnostic features and management.
Orbital pseudotumor: distinct diagnostic features and management.
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DOI：
10.4103/0974-9233.53370
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发表时间：
2008-01
影响因子：
0.6
通讯作者：
Riley FC
中科院分区：
文献类型：
--
作者：
Chaudhry IA;Shamsi FA;Arat YO;Riley FC
概述被称为“特发性眼眶炎症综合征”（也称为眼眶假瘤）的疾病谱，重点是评估和管理这种疾病患者的具体诊断挑战。回顾相关文献并总结有关眼眶假瘤的流行病学、诊断、病理生理学和治疗的最新研究结果。眼眶假瘤是一种局限于眼眶内的良性眼眶内病变，但也可能发生眼眶外受累。它是与甲状腺性眼眶病和淋巴组织增生性疾病并列的第三大常见眼眶疾病，占眼眶突的 5-10%。临床上，眼眶假瘤可分为肌炎、泪腺炎、前部假瘤、顶端假瘤和弥漫性假瘤。患者可能会出现复视、结膜水肿、眼球突出或异常计算机断层扫描 (CT 扫描) 结果。患者还可能患有相关的视神经病变。诊断基于仔细的病史、超声检查 (U/S)、CT 扫描和磁共振成像 (MRI) 研究，这些研究也可以提供预后信息。治疗包括口服或静脉注射全身皮质类固醇。通过眼眶活检进行确认。除放射线外，细胞毒剂、免疫抑制剂、静脉注射免疫球蛋白、生物疗法、TNF-α抑制剂单克隆抗体和霉酚酸酯已被发现可用于治疗难治性眼眶假瘤。了解眼眶假瘤患者的临床特征，通过使用成像技术将其与其他眼眶过程区分开来并及时实施可用的治疗策略可能有助于预防这种情况引起的视力丧失和相关发病率。
To provide an overview of the spectrum of diseases known as ‘idiopathic orbital inflammatory syndrome’ also known as orbital pseudotumor, with emphasis on specific diagnostic challenges in the evaluation and management of patients with this disorder. Review of the relevant literature and summarize recent findings regarding the epidemiology, diagnosis, pathophysiology and treatment of orbital pseudotumor. Orbital pseudotumor is a benign intraorbital process confined to the orbit but extra orbital involvement can occur. It is among the 3rd most common orbital diseases along with thyroid orbitopathy and lymphoproliferative disorder and accounts for 5-10% of orbital processes. Clinically, orbital pseudotumor has been categorized as myositis, dacryoadenitis, anterior, apical and diffuse process. Patients may present with diplopia, conjunctival chemosis, proptosis or abnormal computed tomography scan (CT-scan) findings. Patients may also have associated optic neuropathy. Diagnosis is based on careful history, ultrasonography (U/S), CT-scan and magnetic resonance imaging (MRI) studies which may also provide prognostic information. Treatment consists of systemic corticosteroids in the form of oral or intravenous administration. Confirmation is made by orbital biopsy. In addition to radiation, cytotoxic agents, immunosuppressant, IV immunoglobulin, biological therapy, TNF-alpha inhibitor monoclonal antibody and Mycophenolate Moftil have been found to be useful in the management of refractory orbital pseudotumor. Understanding of the clinical features of patients with orbital pseudotumor, differentiating it from other orbital processes by use of imaging techniques and timely implementation of available treatment strategies may help prevent visual loss and associated morbidity from this condition.