Cheung Ning, Selva Dinesh, McNab Alan A
Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
Ophthalmology. 2007 Aug;114(8):1569-73. doi: 10.1016/j.ophtha.2006.10.056. Epub 2007 Mar 6.
To report 3 patients with adult-onset orbital Langerhans cell histiocytosis (LCH).
Retrospective interventional case reports.
Three adult patients with orbital LCH.
Case reports from 2 different clinics and review of the scientific literature.
Clinical course and radiologic and histologic findings.
Three healthy patients, aged 20 to 36 (mean, 27) years, presented with biopsy-proven unilateral orbital LCH. The initial presenting complaints were mainly orbital pain and headache with or without orbital swelling and minimal effect on ocular movements. There was radiologic evidence of disease centered on the greater wing of sphenoid bone in all cases. Biopsy via lateral canthotomy was performed with LCH confirmed based on histologic and immunohistochemical findings. There was no evidence of disease elsewhere on systemic investigations. One patient had curettage and intralesional corticosteroids, 1 patient had a craniotomy for more complete resection of the lesion, and the third patient was referred for radiotherapy. All patients achieved complete resolution of symptoms and signs. None of the patients demonstrated any evidence of distant or recurrent disease at follow-up.
Orbital LCH in adults is a rare, usually unifocal, and relatively benign disease most commonly affecting the greater wing of sphenoid bone, where there is still active bone marrow in young adults. There is no consensus on cause or therapy, but our reported cases show that several treatment modalities are effective.
报告3例成人眼眶朗格汉斯细胞组织细胞增多症（LCH）患者。
回顾性介入病例报告。
3例成人眼眶LCH患者。
来自2个不同诊所的病例报告及科学文献回顾。
临床病程以及放射学和组织学检查结果。
3例健康患者，年龄20至36岁（平均27岁），经活检证实为单侧眼眶LCH。最初的主诉主要是眼眶疼痛和头痛，伴有或不伴有眼眶肿胀，对眼球运动影响极小。所有病例的放射学检查均显示病变以蝶骨大翼为中心。通过外眦切开术进行活检，根据组织学和免疫组化结果确诊为LCH。全身检查未发现其他部位有病变。1例患者接受了刮除术和病灶内注射皮质类固醇，1例患者接受了开颅手术以更彻底地切除病变，第3例患者接受了放射治疗。所有患者的症状和体征均完全缓解。随访期间，所有患者均未出现远处或复发性疾病的迹象。
成人眼眶LCH是一种罕见的、通常为单灶性且相对良性的疾病，最常累及蝶骨大翼，该部位在年轻人中仍有活跃的骨髓。目前对于病因和治疗尚无共识，但我们报告的病例表明，几种治疗方式均有效。
