Eye Institute, Medical College of Wisconsin, Milwaukee, Wisconsin.
Optom Vis Sci. 2021 Apr 1;98(4):409-417. doi: 10.1097/OPX.0000000000001667.
Idiopathic sclerosing orbital inflammation (ISOI) is characterized by insidious, chronic, progressive inflammation and fibrosis that damage ocular structures and produce a mass effect. This case highlights the challenges in diagnosis and management of ISOI, as well as the associated ocular morbidities, including potential vision loss.
The purpose of this study was to provide education regarding a rare condition that exhibits variable presentation and has an unpredictable success rate with regard to treatment paradigm. Improved therapeutic options are promising. Ultimately, early detection and management are key and may allow for better visual outcome.
A 46-year-old woman presented with complaints of chronic right-sided facial headaches and eye pain and gradual right globe prominence over the previous 6 months. Worsening vision and decreased right peripheral visual field were also noted. Upon examination, an afferent pupillary defect and florid disc edema were evident. Imaging studies revealed an orbital and extraorbital infiltrative mass involving the right orbital apex, inferior orbital fissure, pterygopalatine fossa, and cavernous sinus. Right anterior orbitotomy with biopsy revealed fragments of fibroconnective and adipose tissue with sclerosis and chronic focal inflammation, consistent with ISOI. Treatment included intravenous methylprednisone, followed by oral prednisone, beginning at 60 mg/d with a slow taper thereafter. Signs and symptoms improved dramatically and eventually resolved. Vision significantly improved, and the afferent pupillary defect resolved. The patient remained asymptomatic at 3-month follow-up.
Idiopathic sclerosing orbital inflammation is difficult to diagnose and manage. No large studies exist because of the rare nature of the disease. Slowly progressive, nonspecific signs and symptoms may delay recognition and treatment. Orbital imaging and histopathologic analysis are critical for definitive diagnosis. Conventional treatment with corticosteroids is not uniformly successful, but newer combined therapy options can improve outcomes. Early identification and treatment are key to management and ultimate preservation of function and vision.
特发性硬化性眼眶炎症（ISOI）的特征是隐匿性、慢性、进行性炎症和纤维化，会损害眼结构并产生肿块效应。本病例突出了 ISOI 的诊断和管理挑战，以及相关的眼部病变，包括潜在的视力丧失。
本研究的目的是提供有关一种罕见疾病的教育，该疾病表现形式多样，治疗方案的成功率不可预测。改进的治疗选择有希望。最终，早期发现和管理是关键，可能会获得更好的视觉结果。
一名 46 岁女性因慢性右侧面部头痛和眼痛以及过去 6 个月内右侧眼球逐渐突出而就诊。还注意到视力下降和右侧周边视野减少。检查时，存在传入性瞳孔缺损和明显的视盘水肿。影像学研究显示眼眶和眶外浸润性肿块累及右侧眶尖、下眶裂、翼腭窝和海绵窦。右侧前眶切开活检显示纤维连接组织和脂肪组织碎片，伴有硬化和慢性局灶性炎症，符合 ISOI。治疗包括静脉注射甲基强的松龙，随后口服强的松，起始剂量为 60mg/d，然后缓慢减量。症状和体征显著改善，最终消退。视力显著提高，传入性瞳孔缺损消退。患者在 3 个月随访时无症状。
特发性硬化性眼眶炎症难以诊断和管理。由于疾病的罕见性质，没有大型研究。进展缓慢、非特异性的症状和体征可能会延迟识别和治疗。眼眶成像和组织病理学分析对明确诊断至关重要。常规皮质类固醇治疗并非普遍有效，但新型联合治疗选择可以改善预后。早期识别和治疗是管理和最终保持功能和视力的关键。
