Tay Eugene, Gibson Andrew, Chaudhary Neeraj, Olver Jane
Oculoplastic and Orbital Service, Western Eye Hospital, London, UK.
Orbit. 2008;27(6):458-61. doi: 10.1080/01676830802352436.
To report a case of idiopathic orbital inflammation with extensive extraorbital involvement presenting as 6th nerve palsy.
The patient presented with right periorbital pain, headache, and diplopia. Orthoptic examination revealed 6th nerve palsy. Investigations, including imaging (CT, MRI, MRA, chest x-ray), lumbar puncture, and screening blood tests (autoimmune disease and TB), were normal. He was lost to follow-up but presented again with no perception of light in the right eye with almost complete ophthalmoplegia and 3 mm of proptosis four months later.
Repeat imaging showed a poorly defined mass involving the extraocular muscles in the posterior right orbit extending into the cavernous sinus and infratemporal fossa. Histopathologic examination showed a fibro-inflammatory infiltration of lymphocytes, plasma cells, and histiocytes, with widespread collagen deposition. No granulomas, vasculitis, or necrosis were seen. A tuberculosis screen (Mantoux) and lumbar puncture were negative. Idiopathic orbital inflammation (IOI) was diagnosed, and 60 mg of oral prednisolone and 300 mg of isoniazid were commenced. Fourteen months later, the patient remained blind, but ocular motility had returned to normal and no proptosis was evident.
IOI can present in an atypical fashion and early disease may be undetectable radiologically.
报告一例表现为第六脑神经麻痹且伴有广泛眶外受累的特发性眼眶炎症病例。
患者出现右侧眶周疼痛、头痛及复视。眼肌检查显示第六脑神经麻痹。包括影像学检查（CT、MRI、MRA、胸部X线）、腰椎穿刺及筛查血液检查（自身免疫性疾病和结核病）在内的各项检查均正常。患者失访，但四个月后再次就诊时右眼无光感，几乎完全性眼肌麻痹，眼球突出3毫米。
重复影像学检查显示右眼眶后部有一个边界不清的肿块，累及眼外肌，延伸至海绵窦和颞下窝。组织病理学检查显示淋巴细胞、浆细胞和组织细胞的纤维炎性浸润，伴有广泛的胶原沉积。未见肉芽肿、血管炎或坏死。结核菌素试验（曼托试验）和腰椎穿刺结果均为阴性。诊断为特发性眼眶炎症（IOI），开始给予60毫克口服泼尼松龙和300毫克异烟肼治疗。14个月后，患者仍失明，但眼球运动已恢复正常，且无明显眼球突出。
IOI可能以非典型方式呈现，早期疾病在影像学上可能无法检测到。
