Spindle Jordan, Tang Sunny X, Davies Brett, Wladis Edward J, Piozzi Elena, Pellegrini Marco, Lally Sara E, Shields Carol, Shinder Roman
*Department of Ophthalmology, SUNY Downstate Medical Center, Brooklyn, New York; †Department of Ophthalmology, The University of Colorado, Denver, Colorado; ‡Lions Eye Institute, Albany Medical College, Albany, New York; §A.O. Ospedale Niguarda Ca' Granda, Milan, Italy; ‖Eye Clinic, Department of Biomedical and Clinical Science, Luigi Sacco Hospital, University of Milan, Milan, Italy; and ¶Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, U.S.A.
Ophthalmic Plast Reconstr Surg. 2016 Jul-Aug;32(4):270-4. doi: 10.1097/IOP.0000000000000494.
Pediatric idiopathic orbital inflammation (IOI) is a rare entity with little known about the clinical presentation and natural history. The authors report the demographics, clinical presentations, radiographic and histopathologic characteristics, and treatment outcome of 30 children with IOI.
Retrospective chart review of 30 patients 18 years and younger diagnosed with IOI and statistical analysis using analysis of variance and Fisher's exact test. This study was reviewed and approved by the Institutional Review Board of SUNY Downstate Medical Center.
There were 9 males (30%) and 21 females (70%) with pediatric IOI who presented at a median age of 11 years (range 2-18 years). Primary IOI was found in 19 patients (63%) and recurrent IOI in 11 patients (37%). Overall, 26 patients (87%) had unilateral IOI while 4 patients (13%) had bilateral disease at presentation. There were 12 patients (40%) with systemic constitutional signs. The most common ophthalmic findings included periorbital edema (n = 20, 67%) and blepharoptosis (n = 17, 57%). All patients had orbital radiography with common findings of dacryoadenitis (n = 12, 40%), orbital mass (n = 12, 40%), or myositis (n=10, 33%). The presence of a radiographic orbital mass was significantly related to the clinical presence of blepharoptosis (p = 0.03). The most common treatment was oral glucocorticoids in 24 patients (80%). Over mean follow up of 19 months (range 6-64 months), females were more likely to display recurrent disease (p = 0.01).
Idiopathic orbital inflammation is an uncommon but important cause of acute orbital syndrome in children, manifesting as a bilateral condition in 13% and with constitutional symptoms in 40%. Posttreatment recurrence is found in 37% of cases.
儿童特发性眼眶炎症（IOI）是一种罕见疾病，其临床表现和自然病程鲜为人知。作者报告了30例IOI患儿的人口统计学特征、临床表现、影像学和组织病理学特征以及治疗结果。
对30例18岁及以下诊断为IOI的患者进行回顾性病历审查，并使用方差分析和Fisher精确检验进行统计分析。本研究经纽约州立大学下州医学中心机构审查委员会审查并批准。
30例儿童IOI患者中，男性9例（30%），女性21例（70%），中位年龄为11岁（范围2 - 18岁）。19例患者（63%）为原发性IOI，11例患者（37%）为复发性IOI。总体而言，26例患者（87%）初诊时为单侧IOI，4例患者（13%）为双侧病变。12例患者（40%）有全身症状体征。最常见的眼科表现包括眶周水肿（n = 20，67%）和上睑下垂（n = 17，57%）。所有患者均进行了眼眶影像学检查，常见表现为泪腺炎（n = 12，40%）、眶内肿块（n = 12，40%）或肌炎（n = 10，33%）。眼眶影像学肿块的存在与上睑下垂的临床表现显著相关（p = 0.03）。最常见的治疗方法是24例患者（80%）口服糖皮质激素。平均随访19个月（范围6 - 64个月），女性更易出现疾病复发（p = 0.01）。
特发性眼眶炎症是儿童急性眼眶综合征的一种少见但重要的病因，13%表现为双侧病变，40%伴有全身症状。37%的病例治疗后复发。
