Ang Alan, Poulson Arabella V, Goodburn Sandy F, Richards Allan J, Scott John D, Snead Martin P
Vitreoretinal Service, Addenbrooke's Hospital, Cambridge, United Kingdom.
Ophthalmology. 2008 Jan;115(1):164-8. doi: 10.1016/j.ophtha.2007.03.059. Epub 2007 Aug 2.
To report the prevalence of retinal detachment (RD) and results of prophylaxis against detachment from a giant retinal tear in a large cohort of patients with type 1 Stickler syndrome.
Retrospective study.
Two hundred four type 1 Stickler syndrome patients.
Pedigrees and individuals with type 1 Stickler syndrome were identified from the vitreous research clinic and divided into 3 groups. Group 1 consisted of patients who received no prophylaxis (control group). Group 2 consisted of patients who had bilateral 360 degrees prophylactic cryotherapy (study group). Group 3 consisted of patients referred with unilateral RD for surgical repair and who underwent prophylaxis in the fellow eye (mixed group).
Retinal status after prophylaxis, with failure of prophylaxis being defined as the development of RD or retinal tears needing further retinopexy.
Of 111 patients who had no prophylactic retinopexy (group 1; mean age, 49 years), 73% (81/111) suffered RD and 48% (53/111) were bilateral. Of 62 patients who had bilateral prophylactic cryotherapy (group 2; mean age, 21 years), 8% (5/62) suffered failure of prophylaxis. There were no cases of bilateral detachments. The mean follow-up period was 11.5 years. In 31 patients who had unilateral prophylactic cryotherapy to the fellow eye (group 3; mean age, 36 years), failure occurred in 10% (3/31) of cases with a mean follow-up of 15.5 years. The prevalence of failure of prophylaxis in treated patients was significantly less than prevalence of RD in untreated patients (chi2(1) = 119.2, P<0.001).
Prophylactic cryotherapy substantially reduces the risk of RD in type 1 Stickler syndrome and, in this series, eliminated the risk of bilateral detachments.
报告1型斯蒂克勒综合征（Stickler syndrome）患者队列中视网膜脱离（RD）的患病率以及针对巨大视网膜裂孔性视网膜脱离的预防结果。
回顾性研究。
204例1型斯蒂克勒综合征患者。
从玻璃体研究诊所中识别出1型斯蒂克勒综合征的家系和个体，并将其分为3组。第1组由未接受预防措施的患者组成（对照组）。第2组由接受双侧360度预防性冷冻疗法的患者组成（研究组）。第3组由因单侧视网膜脱离前来接受手术修复且对侧眼接受了预防措施的患者组成（混合组）。
预防后的视网膜状态，预防失败定义为发生视网膜脱离或出现需要进一步视网膜凝固术的视网膜裂孔。
在111例未接受预防性视网膜凝固术的患者中（第1组；平均年龄49岁），73%（81/111）发生了视网膜脱离，48%（53/111）为双侧性。在62例接受双侧预防性冷冻疗法的患者中（第2组；平均年龄21岁），8%（5/62）预防失败。无双侧视网膜脱离病例。平均随访期为11.5年。在31例对侧眼接受单侧预防性冷冻疗法的患者中（第3组；平均年龄36岁），10%（3/31）的病例出现预防失败，平均随访期为15.5年。接受治疗患者的预防失败患病率显著低于未治疗患者的视网膜脱离患病率（χ2(1)=119.2，P<0.001）。
预防性冷冻疗法可显著降低1型斯蒂克勒综合征患者发生视网膜脱离的风险，在本系列研究中，消除了双侧视网膜脱离的风险。
