The clinical effectiveness and safety of prophylactic retinal interventions to reduce the risk of retinal detachment and subsequent vision loss in adults and children with Stickler syndrome: a systematic review.
The clinical effectiveness and safety of prophylactic retinal interventions to reduce the risk of retinal detachment and subsequent vision loss in adults and children with Stickler syndrome: a systematic review.
复制标题
预防性视网膜干预措施的临床有效性和安全性，以降低斯蒂克勒综合征成人和儿童视网膜脱离和随后视力丧失的风险：系统评价。
DOI：
--
复制
发表时间：
2011
影响因子：
3.6
通讯作者：
E. Kaltenthaler
中科院分区：
文献类型：
--
作者：
C. Carroll;D. Papaioannou;A. Rees;E. Kaltenthaler
背景
Stickler综合征，又称遗传性进行性关节眼病，是一种遗传性进行性胶原结缔组织疾病。症状包括近视、白内障、导致视网膜脱离的视网膜问题和可能的失明。这主要是在患有1型斯蒂克勒综合征的个体中出现的。它是儿童时期视网膜脱离最常见的遗传性病因。然而，没有就最佳做法达成共识，也没有关于对这一人群进行预防干预的现行指导方针。
目标
这项系统性综述的目的是评估初级预防性干预预防Stickler综合征患者未经治疗但未发生视网膜脱离的眼睛的临床有效性和安全性的证据。令人感兴趣的主要结果是预防后视网膜脱离。
数据源
系统检索了11个已发表和未发表的文献数据库，包括MEDLINE、MEDLINE in-Process和其他非索引引文、EMBASE、护理和相关卫生文献的累积索引和Cochrane图书馆。没有语言或日期的限制。检查所有纳入研究的参考文献，以寻找进一步的相关引用，并联系具有潜在相关数据的研究的作者。
评审方法
两位评价者对搜索检索到的所有引文的标题和摘要进行了双重筛选，以确定符合纳入标准的研究。两位评价者还独立摘录了所有纳入的研究，并进行了质量评估。进行了叙事合成。
结果
文献检索确定了1444条独特引文，其中四项研究符合纳入标准。这两项主要研究都是对患有1型斯蒂克勒综合征的人群进行的与对照组的回顾性队列研究。其中一项研究评估了360°冷冻治疗(n=204)和另一项焦点或环周激光治疗(n=22)。这两项研究都报告了接受预防性治疗的组和对照组之间每只眼的视网膜剥离率在统计学上的差异。然而，这两项研究都存在很高的偏见风险。对瓦格纳-斯蒂克勒患者的两项支持性研究的结果要么相对不一致，要么不可靠。没有研究报告任何与干预相关的重大或长期并发症。尽管证据不充分，但干预组，尤其是冷冻组的视网膜剥离率低于研究控制组的发病率，也低于没有接受预防措施的未经治疗的斯蒂克勒综合征人群的其他研究报告的发病率。
结论
对于1型斯蒂克勒综合征患者，只评估了360°冷冻治疗以及焦点和周向激光治疗，并且只对每个病例进行了一项回顾、对照、队列研究。这两项研究都报告了干预组和对照组之间的显著差异(主要是不治疗)，并且没有重大或长期的副作用或并发症。然而，这两项研究存在很高的偏差风险，因此任何一种干预的相对有效性都不确定。
下一步工作
服务重点是可靠地确定斯蒂克勒综合征的患病率，即有多少人患有1型或2型斯蒂克勒综合征，以及他们患视网膜脱离和随后失明的风险。在一项非随机的前瞻性队列比较研究中，符合条件的参与者在三个研究小组中的一个中接受治疗、跟踪和分析，不接受治疗、激光治疗或冷冻治疗，这可能会在预防与不预防和冷冻治疗与激光治疗的相对有效性方面提供比目前可用的数据更多的确定性。或者，需要继续跟踪和分析现有研究数据，并从相关样本人群中收集数据，以评估失明、视网膜脱离和预防的长期风险。
资金来源
这项研究由国家卫生研究所卫生技术评估方案资助。
BACKGROUND
Stickler syndrome, also known as hereditary progressive arthro-ophthalmopathy, is an inherited progressive disorder of the collagen connective tissues. Manifestations include short-sightedness, cataracts, retinal problems leading to retinal detachment and possible blindness. This is principally the case among individuals with type 1 Stickler Syndrome. It is the most commonly identified inherited cause of retinal detachment in childhood. However, there is no consensus regarding best practice and no current guidelines on prophylactic interventions for this population.
OBJECTIVES
The aim of this systematic review was to assess the evidence for the clinical effectiveness and safety of primary prophylactic interventions for the prevention of retinal detachment in previously untreated eyes without retinal detachment in patients with Stickler syndrome. The primary outcome of interest was retinal detachment post prophylaxis.
DATA SOURCES
A systematic search was made of 11 databases of published and unpublished literature, which included MEDLINE, MEDLINE In-Process & Other Non-Indexed Citations, EMBASE, the Cumulative Index to Nursing and Allied Health Literature and The Cochrane Library. There was no restriction by language or date. The references of all included studies were checked for further relevant citations and authors of studies with potentially relevant data were also contacted.
REVIEW METHODS
Two reviewers double-screened all titles and abstracts of the citations retrieved by the search to identify studies that satisfied the inclusion criteria. Both reviewers also independently extracted and quality assessed all included studies. A narrative synthesis was performed.
RESULTS
The literature search identified 1444 unique citations, of which four studies satisfied the inclusion criteria. The two principal studies were both retrospective cohort studies with control groups in populations with type 1 Stickler syndrome. One study evaluated 360° cryotherapy (n = 204) and the other focal or circumferential laser treatment (n = 22). Both studies reported a statistically significant difference in the rate of retinal detachment per eye between the groups receiving prophylaxis and the controls. However, both studies were subject to a high risk of bias. The results of the two supporting studies of Wagner-Stickler patients were either relatively inconsistent or unreliable. No study reported any major or long-term complications associated with the interventions. Despite the weaknesses of the evidence, the rate of retinal detachment in the intervention groups, especially the cryotherapy group, was lower than the rate either experienced in the study control groups or reported in other studies of untreated Stickler syndrome populations not exposed to prophylaxis.
CONCLUSIONS
Only 360° cryotherapy and focal and circumferential laser treatment have been evaluated for the type 1 Stickler syndrome population, and then only by a single retrospective, controlled, cohort study in each case. Both of these studies report a significant difference between intervention and control groups (principally no treatment) and no major or long-term side effects or complications. However, there is a high risk of bias within these two studies, so the relative effectiveness of either intervention is uncertain.
FUTURE WORK
A service priority is to determine reliably the prevalence of Stickler syndrome, i.e. how many individuals have type 1 or type 2 Stickler syndrome, and their risk of retinal detachment and subsequent blindness. A non-randomised, prospective cohort comparison study, in which eligible participants are treated, followed-up and analysed in one of three study arms, for no treatment, laser therapy or cryotherapy, would potentially offer further certainty in terms of the relative efficacy of both prophylaxis versus no prophylaxis and cryotherapy versus laser therapy than is possible with the currently available data. Alternatively, continued follow-up and analysis of existing study data, and data collection from relevant sample populations, are required to assess the long-term risks of blindness, retinal detachment and prophylaxis.
FUNDING
This study was funded by the National Institute for Health Research Health Technology Assessment programme.
增殖性镰状细胞性视网膜病的饲养血管光凝的随机临床试验。
影响因子：
13.7
作者：
Condon,P;Jampol,LM;Farber,MD;Rabb,M;Serjeant,G
通讯作者：
Serjeant,G
影响因子：
4.2
作者：
Donoso, LA;Edwards, AO;Meyer, D
通讯作者：
Meyer, D