Rheumatology Department, Great Ormond Street Hospital, Great Ormond Street, Bloomsbury, London WC1N 3JH, UK.
Rheumatology (Oxford). 2012 Jun;51(6):1070-5. doi: 10.1093/rheumatology/ker432. Epub 2012 Jan 31.
To describe the clinical, laboratory, histopathological presentations and final diagnoses for children presenting to a tertiary paediatric rheumatology service with an inflammatory lesion of the orbit.
This was a retrospective descriptive case series of children with an inflammatory lesion of the orbit presenting to a single paediatric rheumatology service between January 1999 and July 2010.
Ten patients, median age 11.5 (range 3.1-16.2) years at referral to the paediatric rheumatology department were identified; median duration of symptoms at referral was 9 (0.75-17) months. Imaging was performed in 9/10 cases: orbital MRI (n = 4), orbital CT scan (n = 1), both MRI and CT scan (n = 4). All 10 patients had an orbital biopsy; 2 patients had repeat biopsies. The final diagnoses were granulomatosis with polyangiitis (Wegener's) (n = 5; ANCA positive n = 4, ANCA negative n = 1), idiopathic orbital inflammation (n = 3), atypical mycobacterial infection (n = 1) and sarcoidosis (n = 1).
Inflammatory mass lesion of the orbit is an unusual presentation in children. The differential diagnosis is wide and may evolve over time. Orbital biopsy and screening for systemic features is essential before treatment with CSs or other immunosuppressants to exclude malignancy, infection, vascular lesions, autoimmune conditions or other causes of orbital inflammation that can be associated with serious systemic manifestations.
描述在一家三级儿科风湿病服务机构就诊的伴有眼眶炎性病变的儿童的临床、实验室、组织病理学表现和最终诊断。
这是一项回顾性描述性病例系列研究，纳入了 1999 年 1 月至 2010 年 7 月期间在一家儿科风湿病服务机构就诊的伴有眼眶炎性病变的儿童。
共确定了 10 例患者，转诊至儿科风湿病科时的中位年龄为 11.5 岁（范围 3.1-16.2 岁）；转诊时症状的中位持续时间为 9 个月（0.75-17 个月）。9/10 例患者进行了影像学检查：眼眶 MRI（n=4）、眼眶 CT 扫描（n=1）、MRI 和 CT 扫描均进行（n=4）。10 例患者均进行了眼眶活检；2 例患者进行了重复活检。最终诊断为肉芽肿伴多血管炎（韦格纳氏）（n=5；ANCA 阳性 n=4，ANCA 阴性 n=1）、特发性眼眶炎症（n=3）、非典型分枝杆菌感染（n=1）和结节病（n=1）。
儿童眼眶炎性肿块病变是一种不常见的表现。鉴别诊断范围广泛，且可能随时间演变。在使用 CS 或其他免疫抑制剂治疗之前，进行眼眶活检和全身特征筛查至关重要，以排除恶性肿瘤、感染、血管病变、自身免疫性疾病或其他可能与严重全身表现相关的眼眶炎症原因。
