Patel Sheel R, Rosenberg Jamie B, Barmettler Anne
Department of Ophthalmology, Montefiore Medical Center, Albert Einstein College of Medicine, 3332 Rochambeau Avenue, 3rd Floor, Bronx, NY, USA, 10467.
Cochrane Database Syst Rev. 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2.
Orbital lymphangiomas are a subset of localized vascular and lymphatic malformations, which most commonly occur in the head and neck region. Orbital lymphangiomas typically present in the first decade of life with signs of ptosis, proptosis, restriction of ocular motility, compressive optic neuropathy, and disfigurement. Therefore, early and effective treatment is crucial to preserving vision. Due to proximity to vital structures, such as the globe, optic nerve, and extraocular muscles, treatment for these lesions is complicated and includes a large array of approaches including observation, sclerotherapy, systemic therapy, and surgical excision. Of these options, there is no clear gold standard of treatment.
To assess the evidence supporting medical and surgical interventions for the reduction/treatment of orbital lymphangiomas in children and young adults.
We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Trials Register) (2018, Issue 5); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We did not use any date or language restrictions in the electronic search for trials. We last searched the electronic databases on 22 May 2018.
We planned to include randomized controlled trials (RCTs) comparing at least two of the following interventions with each other for the treatment of orbital lymphangiomas: observation; sildenafil therapy; sirolimus therapy; sclerotherapy; surgery (partial or complete resection). We planned to include trials that enrolled children and adults up to 32 years of age, based on a prior clinical trial protocol. There were no restrictions regarding location or demographic factors.
Two review authors independently screened the titles, abstracts, and full articles to assess their suitability for inclusion in this review. No risk of bias or data extraction was performed because we did not find any trials for inclusion. If there had been RCTs, two authors would have assessed the risk of bias and abstracted data independently with discrepancies being settled by consensus or consultation with a third review author.
There were no RCTs that compared any two of the mentioned interventions (medical or surgical) for treating orbital lymphangiomas in children and young adults.
AUTHORS' CONCLUSIONS: Currently, there are no published RCTs of orbital lymphangioma treatments. Without these types of studies, conclusions cannot be drawn regarding the effectiveness of the medical and surgical treatment options for patients with orbital lymphangiomas. The presence of only case reports and case series on orbital lymphangiomas makes it clear that RCTs are needed to address the differences between these options and help guide treatment plans. Such trials would ideally compare outcomes between individuals randomized to one of the following treatment options: observation, sclerotherapy, systemic sirolimus therapy, systemic sildenafil therapy, and surgical excision.
眼眶淋巴管瘤是局限性血管和淋巴管畸形的一种类型，最常见于头颈部区域。眼眶淋巴管瘤通常在生命的第一个十年出现，伴有上睑下垂、眼球突出、眼球运动受限、压迫性视神经病变和容貌毁损等症状。因此，早期有效治疗对于保护视力至关重要。由于靠近眼球、视神经和眼外肌等重要结构，这些病变的治疗较为复杂，包括观察、硬化治疗、全身治疗和手术切除等多种方法。在这些选择中，没有明确的治疗金标准。
评估支持对儿童和青年眼眶淋巴管瘤进行减少/治疗的医学和手术干预的证据。
我们检索了Cochrane对照试验中心注册库（CENTRAL）（其中包含Cochrane眼科和视力试验注册库）（2018年第5期）；Ovid MEDLINE；Embase.com；PubMed；拉丁美洲和加勒比健康科学文献数据库（LILACS）；ClinicalTrials.gov和世界卫生组织（WHO）国际临床试验注册平台（ICTRP）。在电子检索试验时，我们没有使用任何日期或语言限制。我们最后一次检索电子数据库是在2018年5月22日。
我们计划纳入随机对照试验（RCT），比较以下至少两种治疗眼眶淋巴管瘤的干预措施：观察；西地那非治疗；西罗莫司治疗；硬化治疗；手术（部分或完全切除）。根据先前的临床试验方案，我们计划纳入招募儿童和32岁以下成年人的试验。对地点或人口统计学因素没有限制。
两位综述作者独立筛选标题、摘要和全文，以评估其是否适合纳入本综述。由于未找到任何纳入试验，因此未进行偏倚风险评估或数据提取。如果有RCT，两位作者将独立评估偏倚风险并提取数据，如有分歧将通过协商或与第三位综述作者协商解决。
没有RCT比较上述任何两种治疗儿童和青年眼眶淋巴管瘤的干预措施（医学或手术）。
目前，尚无已发表的眼眶淋巴管瘤治疗的RCT。没有这类研究，就无法得出关于眼眶淋巴管瘤患者医学和手术治疗方案有效性的结论。仅存在眼眶淋巴管瘤的病例报告和病例系列，这清楚表明需要RCT来解决这些方案之间的差异并帮助指导治疗计划。这类试验理想情况下应比较随机分配到以下治疗方案之一的个体之间的结果：观察、硬化治疗、全身西罗莫司治疗、全身西地那非治疗和手术切除。
