Persistent hyperplastic primary vitreous

持续性增生性原发性玻璃体
  • 文章类型: Journal Article
    OBJECTIVE: To study the complications and surgical outcomes of cataract surgery in patients of persistent fetal vasculature (PFV) with cataract.
    METHODS: In this prospective study, phacoaspiration with/without intraocular lens implantation (IOL) was done in 20 children (mean age 14.2 months) with unilateral cataract with anterior (n = 6) or combined (n = 14) PFV. The rentrolental vascularized membrane was cauterized and dissected circumferentially, followed by cauterization and resection of the PFV stalk. The outcome measures included fixation preference using the CSM (central, steady, maintained) method and intraoperative and postoperative complications in an 18-month follow-up. The difference in outcomes of anterior and combined PFV, as well as aphakic and pseudophakic eyes, was studied.
    RESULTS: CSM fixation was seen in 16 patients after 18 months. The intraocular lens was implanted in 16 eyes and 4 eyes with combined PFV were left aphakic. None of our patients had intraoperative bleeding. Visual axis obscuration was the major complication seen, requiring membranectomy in 8 children. Pupilloplasty was required with membranectomy in one eye. None of our patients developed glaucoma or retinal detachment.
    CONCLUSIONS: Timely surgical intervention and aggressive amblyopia therapy led to good visual results in our study. Poor prognosis was seen in combined PFV, aphakia, and microphthalmia.
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  • 文章类型: Journal Article
    To study the clinicopathological findings of Persistent Fetal Vasculature (PFV) in patients with congenital cataract and PFV.
    Six eyes with anterior or combined PFV with cataract underwent phacoaspiration with primary posterior capsulotomy with anterior vitrectomy with intraocular lens implantation followed by histopathological evaluation of the PFV stalk and membrane.
    Four and two patients had combined and anterior PFV respectively. There was no postoperative hyphema, vitreous haemorrhage, glaucoma or retinal detachment in six months. Haematoxylin and eosin staining showed inflammatory cells predominantly with extramedullary hematopoeisis and vascularisation.
    We recommend IOL implantation in PFV, with early and aggressive amblyopia therapy.
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  • 文章类型: Journal Article
    确定小儿患者双侧同期玻璃体视网膜手术的可行性和安全性。
    国际,多中心,介入,回顾性病例系列。
    来自全球24个中心的17岁或以下的患者,接受了立即序贯双侧玻璃体视网膜手术(ISBVS)-定义为玻璃体切除术,巩膜带扣,或使用玻璃体切割器进行晶状体切除术-在同一麻醉期间连续进行双眼。
    临床病史,手术细节和适应症,麻醉下的时间,并回顾了术中和术后眼科和全身不良事件。
    眼部和全身不良事件。
    本研究共纳入167例患者的172例ISBVS手术中的344例手术。该队列的平均年龄为1.3±2.6岁。ISBVS的非排他性适应症是快速进行性疾病(74.6%),全身发病率使儿童处于高麻醉风险(76.0%),和远离手术地点的住所(30.2%)。最常见的诊断是早产儿视网膜病变(ROP;72.7%[P<0.01];3期,4.8%;4A期,44.4%;4B阶段,22.4%;第五阶段,26.4%),家族性渗出性玻璃体视网膜病变(7.0%),虐待性头部外伤(4.1%),持续的胎儿脉管系统(3.5%),先天性白内障(1.7%),后囊混浊(1.7%),孔源性视网膜脱离(1.7%),先天性X连锁视网膜劈裂(1.2%),诺里病(2.3%),和病毒性视网膜炎(1.2%)。双眼的平均手术时间为143±59分钟。ROP分期越高,手术时间越长(P=0.02)。没有报告术中眼部并发症。在术后即刻,来自不同患者的2只眼显示单侧玻璃体出血(0.6%)。没有眼内炎的病例,脉络膜出血,或者发生了低血压。平均总麻醉时间为203±87分钟。没有麻醉相关死亡病例,恶性高热,过敏反应,或心脏事件。再插管1例(0.6%),氧饱和度延长1例(0.6%)。手术后平均随访103周,解剖成功率和全球抢救率为89.8%和98.0%,分别。
    本研究发现,对于具有双侧玻璃体视网膜病理特征的儿科患者,当反复全身麻醉是不可取的或不切实际的时,ISBVS是一种可行且安全的治疗模式。
    To determine the feasibility and safety of bilateral simultaneous vitreoretinal surgery in pediatric patients.
    International, multicenter, interventional, retrospective case series.
    Patients 17 years of age or younger from 24 centers worldwide who underwent immediate sequential bilateral vitreoretinal surgery (ISBVS)-defined as vitrectomy, scleral buckle, or lensectomy using the vitreous cutter-performed in both eyes sequentially during the same anesthesia session.
    Clinical history, surgical details and indications, time under anesthesia, and intraoperative and postoperative ophthalmic and systemic adverse events were reviewed.
    Ocular and systemic adverse events.
    A total of 344 surgeries from 172 ISBVS procedures in 167 patients were included in the study. The mean age of the cohort was 1.3±2.6 years. Nonexclusive indications for ISBVS were rapidly progressive disease (74.6%), systemic morbidity placing the child at high anesthesia risk (76.0%), and residence remote from surgery location (30.2%). The most common diagnoses were retinopathy of prematurity (ROP; 72.7% [P < 0.01]; stage 3, 4.8%; stage 4A, 44.4%; stage 4B, 22.4%; stage 5, 26.4%), familial exudative vitreoretinopathy (7.0%), abusive head trauma (4.1%), persistent fetal vasculature (3.5%), congenital cataract (1.7%), posterior capsular opacification (1.7%), rhegmatogenous retinal detachment (1.7%), congenital X-linked retinoschisis (1.2%), Norrie disease (2.3%), and viral retinitis (1.2%). Mean surgical time was 143±59 minutes for both eyes. Higher ROP stage correlated with longer surgical time (P = 0.02). There were no reported intraoperative ocular complications. During the immediate postoperative period, 2 eyes from different patients demonstrated unilateral vitreous hemorrhage (0.6%). No cases of endophthalmitis, choroidal hemorrhage, or hypotony occurred. Mean total anesthesia time was 203±87 minutes. There were no cases of anesthesia-related death, malignant hyperthermia, anaphylaxis, or cardiac event. There was 1 case of reintubation (0.6%) and 1 case of prolonged oxygen desaturation (0.6%). Mean follow-up after surgery was 103 weeks, and anatomic success and globe salvage rates were 89.8% and 98.0%, respectively.
    This study found ISBVS to be a feasible and safe treatment paradigm for pediatric patients with bilateral vitreoretinal pathologic features when repeated general anesthesia is undesirable or impractical.
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  • 文章类型: Comparative Study
    BACKGROUND: The Infant Aphakia Treatment Study is a randomized trial that compares the treatment of unilateral congenital cataract with primary intraocular lens (IOL) implantation versus aphakic contact lens (CLs). The purpose of this study was to compare the outcomes for infants with lens opacity associated with persistent fetal vasculature (PFV) to those without.
    METHODS: Retrospective subgroup analysis of grating visual acuity at 1 year of age and adverse events up to 1 year after surgery in eyes identified intraoperatively as having evidence of mild PFV from the IATS.
    RESULTS: Of 83 infants, 18 (22%: 11 CL, 7 IOL) had PFV. Median logMAR visual acuity was 0.88 for patients with PFV and 0.80 for patients without PFV (P = 0.46). One or more adverse events up to 1 year after surgery occurred in 12 infants (67%) with PFV and 30 infants (46%) without PFV (P = 0.18). The incidence of adverse events was significantly greater in patients with PFV compared with patients without PFV in the CL group (55% vs 20%, P = 0.049) but not in the IOL group (86% vs 71%, P = 0.65), possibly because all children receiving IOLs had greater rates of adverse events when compared with aphakic children (73% vs 29%, P < 0.001).
    CONCLUSIONS: Aphakic infants with mild PFV treated with CL had a greater incidence of adverse events after lensectomy compared with children with other forms of unilateral congenital cataract; nevertheless, similar visual outcomes at 1 year after surgery were obtained.
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  • 文章类型: Editorial
    暂无摘要。
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  • DOI:
    文章类型: English Abstract
    OBJECTIVE: To study the pathological characteristics of retrolental membranes (RLMs) secondary to persistent hyperplastic primary vitreous (PHPV), and to discuss the possible pathogenesis of PHPV.
    METHODS: Experimental study. Six RLMs obtained from six patients with PHPV during vitrectomy were examined by light microscopy (HE & PAS staining). All of them were observed with proliferating cell nuclear antigen (PCNA) immunostaining, together with collagen I, factor VIII related antigen, smooth muscle actin (SMA), epithelial membrane antigen (EMA), neuron specific enolase (NSE) and glial fibrillary acidic protein (GFAP) staining. Apoptosis were detected by terminal deoxynucleotidyl transferase-mediated deoxyuridine 5-triphosphate nick-end labeling (TUNEL).
    RESULTS: Light microscopy showed that the RLM was a dense connective tissue with numerous inflammatory cells including mast cells and lymphocytes. PAS staining showed that RLMs contained a larger amount of polysaccharides. Histopathology and immunohistochemistry showed that there were vascular channels, smooth muscle cells, nervous cells and epithelial cells scattered in RLMs. Collagen I was the main component of RLMs. PCNA-positive nuclei were widely found in RLMs. TUNEL-positive nuclei were also found in all RLMs, as well as in the posterior subcapsular epithelial cells of lens.
    CONCLUSIONS: The cell types of RLMs secondary to PHPV are similar to those of the primary vitreous. It is possible that the mechanism of the progression of RLMs is the over-development and incomplete regression of the retrolental vascular system. Inflammation may play an important role in the regression of RLMs.
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  • DOI:
    文章类型: Comparative Study
    In children with persistent hyperplastic primary vitreous body (PHPVB) concurrent with unilateral congenial cataracts (UCC), combined ultrasound study used to examine blood supply to and hemodynamics of the hyaloid system--a fibrovascular band. To determine the prognosis of a surgical intervention in the mode of color and energy Doppler mapping in the persistent band, retrolenticular membrane, the authors studied whether they had functioning vessels and linear blood flow velocity in different degrees of PHPVB. Based on the results of combined ultrasound study, the authors defined indications for and contraindications to surgical treatment of UCC concurrent with PHPVB in order to prevent hemorrhagic complications (hyphema, hemophthalmos, hyaloid vascular bleeding), as well as the optimal time of surgical treatment.
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