巩膜扣带手术和玻璃体切割手术治疗不同分期家族性渗出性玻璃体视网膜病变合并孔源性视网膜脱离的疗效观察

Clinical observation of sclera buckle and vitrectomy treating different stages of familial exudative vitreoretinopathy associated rhegmatogenous retinal detachment

目的观察巩膜扣带手术（SBP）和玻璃体切割手术（PPV）治疗不同分期家族性渗出性玻璃体视网膜病变（FEVR）合并孔源性视网膜脱离（RRD）的疗效。方法临床确诊为FEVR合并RRD的19例患者20只眼纳入研究。其中，FEVR分期3A期7只眼，4A期4只眼，4B期6只眼，5期3只眼。增生性玻璃体视网膜病变（PVR）B级5只眼，c1级2只眼，C2级3只眼，C3级7只眼，D1级3只眼。所有患眼均存在与视网膜脱离相关的视网膜裂孔。根据FEVR分期和PVR分级、视网膜裂孔位置、视网膜脱离范围选择SBP或PPV。20只眼中，行SBP（SBP组）、PPV（PPV组）各10只眼。SBP组、PPV组患眼平均最小分辨角对数（10gMAR）最佳矫正视力（BCVA）分别为0．60±0．32、1．81±0．53。SBP组患眼手术中冷冻视网膜裂孔及变性带，巩膜穿刺排放视网膜下液。PPV组患眼手术中彻底清除玻璃体并剥除视网膜前膜，硅油填充。手术后平均随访时间（20．20±7．25）个月。观察两组患眼最终视网膜复位情况、BCVA及不同手术方式的手术次数。结果两组患眼最终视网膜复位率均为100．0％。SBP组、PPV组患眼平均logMARBCVA分别为0．34±0．32、1．42±0．64。与手术前平均logMARBCVA比较，SBP组患眼logMARBCVA差异有统计学意义（t=2．932，P=0．017）；PPV组患眼logMARBCVA差异无统计学意义（t=1．812，P=0．103）。SBP组、PPV组患眼平均手术次数分别为（1．10±0．32）、（2．20±0．42）次。两组患眼平均手术次数比较，差异有统计学意义（t=6．588，P=0．000）。结论PVRC3级以下，FEVR分期4A期以下者，SBP视网膜复位率高，手术次数少，视力恢复较好。PVRC3级及以上，FEVR分期4B期及以上，PPV视网膜复位率高，但手术次数多，视力预后较差。

Objective To observe the outcome of seleral buckle and vitrectomy for familial exudative vitreoretinopathy （FEVR） associated rhegmatogenous retinal detachment （RRD） with different stages. Methods Twenty eyes in 19 patients were included in this study. All the eyes were staged according to the staging system of FEVR. There are 7 eyes at stage 3A, 4 eyes at stage 4A, 6 eyes at stage 4B, and 3 eyes at stage 5. According to classification of retinal detachment （RD） with proliferative vitreoretinopathy （PVR）, PVR B was in 5 eyes, PVR C1 in 2 eyes, PVR C2 in 3 eyes, PVR C3 in 7 eyes, PVR D1 in 3eyes. Retinal holes responsible for the RD could be found in every case. Scleral buckle or vitrectomy were chosen according to FEVR staging, PVR classification, location of retinal breaks, extent of RD. Ten eyes （stage 3A in 7 eyes, stage 4A in 3 eyes;PVR B in 5 eyes, PVR C1 in 2 eyes, PVR C2 in 3 eyes） were undergone scleral buckle, the mean preoperative minimum resolution angle in logarithmic （logMAR） best corrected visual acuity （BCVA） is 0.60±0.32. Ten eyes （stage 4A in 1 eyes,stage 4B in 6 eyes,stage 5 in 3 eyes; PVR C2 in 1 eyes,PVR C3 in 6 eyes,PVR D1 in 3 eyes） were undergone vitrectomy, the mean preoperative logMAR BCVA is 1.81±0.53. The mean follow up was（20.20±7.25） months, range 3 to 30 months. Surgical outcome were estimated by the average number of operation, reattachment of retina and BCVA. Results Final retinal attachment was obtained in 100% of all 20 eyes. The mean postoperative logMAR BCVA of scleral buckle group （0.34 ± 0.32） is improved than preoperative BCVA, the difference wan statistically significant （t= 2.932, P = 0.017）. The mean postoperative log MAR ECVA of vitrectomy group （1.42±0.64） is not changed compare with preoperative BCVA （t=1. 812,P=0. 103）. The mean number of operation of seleral buckle group （1.10±0.32） is less than vitrectomy group＇s （2.20±0.42） ,the difference wan statistically significant （t=6. 588, P=0. 000）. Conclusions Among the patients whose FEVR staging is less than 4A and PVR classification is less than C3, epiretinal membranes or subretinal membranes appears mild, and scleral buckle can achieve high success rate with less number of operations, and the BCVA is improved in most of the cases. For the patients whose FEVR staging is more than 4B and PVR classification is more than C3, proliferative vitreoretinopathy seems to be serious, retina can be effectively reattached via vitrectomy, however, the number of operations required is multiple, and the BCVA is probably unimproved after operation.