目的:描述临床特征,后持续胎儿血管(PFV)的管理和结局,并建议一种管理算法。
方法:回顾性连续病例系列。
方法:2011年6月至2021年9月期间,所有诊断为后PFV并在法国罗斯柴尔德基金会医院接受治疗或随访的儿童。
方法:回顾性分析后端PFV的临床特征。我们在诊断时报告:年龄,性别,出现症状,眼内压(IOP),视力(VA)。根据眼前段的严重程度和受累与否,将患者分为四组。我们报告了所使用的玻璃体视网膜手术技术。
方法:解剖学结果,高眼压,BCVA,在每次随访时记录是否存在术后不良事件和额外的手术干预.
结果:共纳入96例患者。诊断时的中位年龄为8个月(平均18.9±30.9个月),平均随访时间为27±31.2个月。虽然PFV通常是一种孤立的疾病,8%的病例与全身性疾病相关.后PFV与62眼(64%)的前部受累有关。41只眼睛(42.7%)是小眼,并且更频繁地与严重的PFV相关[53%vs.25%;(p=0.01)]。85例患者(89%)进行了手术。其中,69(81%)是完全成功的,5(6%)是由于持续的限制性周边视网膜脱离(RD)部分成功,11例(13%)由于手术后持续的总RD而失败。24只眼出现术后不良事件,包括需要滴眼液治疗的高眼压(6.6%),IOL周围的继发性细胞增殖(7.7%),玻璃体内出血(6.6%),持续性牵引RD(9.9%)。对15例患者(16%)进行了第二次手术。在最后的随访中,VA可以用logMAR测量43名儿童(45%),是21只眼睛的光感知(22%),32只眼睛没有光感知或无法评估(33%)。
结论:在我们的案例系列中,大多数出现PFV并后部受累的患者接受了复杂的玻璃体视网膜手术.手术的目标各不相同,包括视网膜变平,玻璃体视网膜牵引减少,释放视觉轴和美学关注。我们提出了一种用于PFV的手术和医疗管理算法。
To describe clinical features, management, and outcomes of posterior persistent fetal vasculature (PFV) and suggest a management algorithm.
Retrospective, consecutive case series.
All children diagnosed with posterior PFV and treated or followed at the Rothschild Foundation Hospital in France between June 2011 and September 2021.
Retrospective analysis of the clinical characteristics of posterior PFV. We reported age, gender, presenting symptoms, intraocular pressure, and visual acuity (VA) at diagnosis. Patients were divided into 4 groups depending on the severity and involvement or not of anterior segment. We reported the vitreoretinal surgical techniques used.
Anatomic results, ocular hypertension, best-corrected distance visual acuity (BCDVA), presence of postoperative adverse events, and additional surgical interventions were recorded at each follow-up visit.
Ninety-six patients were included. The median age at diagnosis was 8 months (IQR = 12), mean 18.9 ± 30.9 months) with a mean follow-up of 27 ± 31.2 months. Although PFV is often an isolated disease, it was associated with a systemic disease in 8% of cases. There was anterior involvement in 62 (64%) of eyes. Forty-one eyes (42.7%) were microphthalmic and more frequently associated with severe PFV (53% vs. 25%; P = 0.01). Surgery was performed in 85 patients (89%). Of them, 69 (81%) had a total success, 5 (6%) had a partial success due to persistent limited peripheral retinal detachment (RD), and 11 (13%) had a failure due to persistent total RD after surgery. Postoperative adverse events occured in 38 eyes including ocular hypertension requiring eye drop medication (7.1%), secondary cell proliferation around the intraocular lens (8.2%), intravitreal hemorrhages (7.1%), and persistent tractional RD (10.6%). Second surgery was performed in 18 patients (21%). At last follow-up, VA could be measured in logarithm of the minimum angle of resolution in 43 children (45%), light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%).
In our case series, most patients presenting with posterior PFV received complex vitreoretinal surgery. Goals of the surgery vary and include retinal flattening, reduction of vitreoretinal traction, freeing of visual axis, and aesthetic concerns. We propose a surgical and medical management algorithm for PFV.
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