Anterior Persistent Fetal Vasculature Research Articles

Profile of a large cohort of children with persistent fetal vasculature and their predictors of poor visual outcome in a tertiary eye hospital in Saudi Arabia.

To present the profile of a large cohort of children with persistent fetal vasculature (PFV) and identify their predictors of poor visual outcome in a tertiary eye hospital in Saudi Arabia. This was a single-center study. Medical records of children diagnosed with persistent fetal vasculature between January 1990 and January 2020 at King Khaled Eye Specialist Hospital (KKESH), Riyadh, were reviewed. Data were collected on PFV type, presenting features, management, and visual acuity. Poisson regression with robust error variance was used to identify the predictors of poor visual outcome. Data of 175 eyes of 170 children with PFV were analyzed. Most had unilateral disease (n = 165, 96.2%), and over half of them (57.1%) were males. The main presenting complaints were leukocoria (n = 126, 72.0%), followed by smaller eye (n = 36, 20.6%), poor vision (n = 15, 8.6%), and strabismus (n = 13, 7.4%). Anterior, posterior, and combined PFV were present in 30.9%, 10.9%, and 58.3% eyes, respectively. In eyes with measurable visual acuity (n = 124), BCVA at the last follow-up was ≥20/100, <20/100-20/400, CF/HM and LP/NLP in 11 (8.9%), 32 (25.8%), 43 (34.7%), and 38 (30.6%) eyes, respectively. In the multivariable poisson regression analysis, eyes with posterior PFV were approximately two times more likely to have a BCVA of CF or worse than those with anterior PFV (Adjusted incidence ratio: 1.82 (95%: 1.23, 2.70; P = 0.003). Compared with eyes with primary or secondary IOL implantation, aphakic eyes or those with no cataract surgery were significantly more likely to have BCVA of CF or worse (adjusted incidence ratio: 1.84 [95%: 1.18, 2.86], p = 0.007 and adjusted incidence ratio: 1.64 [95%: 1.08, 2.50], p = 0.020, respectively). The prognosis of PFV varies depending on the severity of the disease. Patients with posterior PFV had worse visual outcome than anterior or combined type. Whether primary or secondary, IOL implantation increases the probability of having BCVA better than CF.

Severe anterior persistent fetal vasculature: the role of anterior retinal elongation on prognosis.

This study aims to investigate surgical outcomes of eyes with severe anterior persistent fetal vasculature (PFV) and the role of associated anatomical anomalies on prognosis. This is a retrospective, comparative case series of 32 eyes of 31 patients who underwent vitreoretinal surgery for severe anterior PFV, defined as fibrovascular tissue totally covering the back of cataractous lens. Based on the degree of anterior retinal elongations, cases were classified as follows: group 1, eyes with well-developed pars plana and minor/no abnormalities (n = 11, 34%); group 2, eyes with partially developed pars plana and broad-based elongations (n = 9, 28%); and group 3, eyes with no visible pars plana and fibrovascular membrane having 360° continuity with peripheral retina (n = 12, 38%). Complications and functional and anatomical outcomes were investigated. The median surgical age was 2 (1-12) months. The median follow-up was 26 (6-120) months. Seventy-three percent in group 1 achieved finger counting or better vision with a single surgery and without any pupillary/retinal complication. Groups 2 and 3 required 2.1 ± 0.9 and 2.6 ± 1.2 surgeries on average. Pupillary obliteration and RD occurred in 33% and 22% in group 2 and 58% and 67% in group 3. Retina remained attached after silicone oil removal in 89% of group 2 and 25% of group 3. Phthisis developed in 50% in group 3. Peripheral retinal anomalies are common in severe anterior PFV and have a major impact on prognosis. Prognosis is favorable in cases with mild-to-moderate anomalies with appropriate management of possible retinal tears. In eyes with 360° retinal elongations, severe fibrous proliferation and eventual eye loss are common.

Outcomes of secondary sulcus intraocular lens implantation in unilateral anterior persistent fetal vasculature.

To evaluate the surgical results of sulcus intraocular lens (IOL) implantation in children with unilateral anterior persistent fetal vasculature (PFV) underwent primary vitrectomy combined with lensectomy and preservation of the peripheral anterior capsule. Twenty-two eyes of 22 children with unilateral anterior PFV who underwent sulcus secondary IOL implantation were analyzed. Main outcome measures were preoperative and postoperative visual acuity, and complications both intraoperatively and postoperatively. Review of 22 consecutive patients identified best-corrected visual acuity (BCVA) improvement from 1.37±0.84 to 0.73±0.57 logarithm of the minimal angle of resolution (logMAR) after IOL implantation (P<0.001) with a mean follow-up was 16.55±5.86mo. Average age at secondary IOL implantation was 41.05±15.41mo. Three eyes (13.64%) achieved BCVA of 0.3 logMAR at the final visit. Transient intraocular pressure rise (4 eyes; 18.18%), postoperative increased inflammation (3 eyes; 13.64%) and postoperative hypotony (2 eyes; 9.09%) were common complications. Properly preservation of the anterior lens capsule during the primary surgery facilitated secondary sulcus IOL implantation in pediatric patients with anterior PFV, with favorable postoperative visual outcomes and compatible percentage of complications.

Anterior elongation of the retina in persistent fetal vasculature: emphasis on retinal complications.

To identify the characteristics of peripheral retinal anomalies associated with persistent fetal vasculature (PFV) and evaluate the complications and outcomes. Retrospective, noncomparative case series of patients with PFV who underwent surgery. Type of PFV, presence of peripheral retinal anomalies, extent of the retrolental membrane, complications, and functional and anatomical results were evaluated. Twenty-nine eyes of 28 patients were enrolled in the study: 14 eyes (48.3%) with anterior PFV, 10 eyes (34.5%) with posterior PFV, and 5 eyes (17.2%) with combined PFV. The retina was found to elongate anteriorly as finger-like projections beyond the ora serrata, incorporating into retrolental fibrovascular tissue in 81.8% of the anterior PFV cases. Cases with more extensive retrolental tissue had a higher risk of retinal complications (p = 0.009) and anterior segment complications (p = 0.026) than those with localized disease. Leaving the peripheral part of the fibrovascular tissue in place led to complications by later contraction. A total of 35.7% of the anterior PFV cases had 20/200 or better vision at the final follow-up versus 6.7% of the cases with posterior involvement. Retinal attachment was achieved in 12 eyes (80%) with posterior involvement. Four eyes (13.7%) resulted in total retinal detachment at final follow-up. The high incidence of peripheral retinal anomalies that are found in anterior PFV patients and their relation to retinal complications warrant careful examination of the ora serrata-ciliary body area. Limbal approach may be preferred as a safer method in cases with an extensive fibrovascular membrane obscuring the view. Leaving the peripheral part of the fibrovascular tissue may result in severe postoperative complications, which should be avoided.

A clinicopathological study of persistent fetal vasculature.

Purpose:To study the clinicopathological findings of Persistent Fetal Vasculature (PFV) in patients with congenital cataract and PFV.Methods: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.Results: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.Conclusion:We recommend IOL implantation in PFV, with early and aggressive amblyopia therapy.

Persistent Fetal Vasculature With Elongated Ciliary Processes in Children

Outcomes and reoperation rates in infants with unilateral persistent fetal vasculature (PFV) with elongated or stretched ciliary processes have not been extensively studied and were excluded from the Infant Aphakia Treatment Study (IATS). The purpose of this study is to analyze the preoperative measurements, reoperation rates, and complications after surgery in unilateral anterior PFV with stretched processes. Retrospective case series. Inclusion criteria consisted of unilateral cataract surgery prior to 7months of age and anterior PFV with elongated ciliary processes. Eyes with posterior retinal involvement and less than 6months of follow-up were excluded. All patients underwent lensectomy, posterior capsulectomy, and vitrectomy. Eleven eyes of 11 patients were included. Patients had a mean age at surgery of 2.4 ± 1.4months. Average follow-up was 4.5 ± 3.7years. Globe axial lengths were 18.6 ± 1.9mm. Ten patients (91%) were initially left aphakic. Three patients (27%) later received a secondary intraocular lens (IOL), and 1 patient underwent an IOL exchange. Six out of 10 (60%) aphakic patients developed visual axis opacification. One aphakic patient required topical therapy for glaucoma. One additional patient developed neovascular glaucoma and retinal detachment. The eye was subsequently enucleated. Three patients underwent strabismus surgery. Two patients underwent pupilloplasty. Two patients (18.2%) had a final visual acuity better than 20/200. Eyes operated for PFV with elongated ciliary processes are unlikely to have a final visual acuity greater than 20/200 and many will need additional surgery. Postoperative visual axis opacification occurred in 60% and glaucoma developed in 18%.

Functional and anatomical outcomes following surgical management of persistent fetal vasculature: a single-center experience of 44 cases.

To investigate the functional and anatomical outcomes following surgical management of persistent fetal vasculature (PFV). Single-center, retrospective, interventional consecutive case series of 41 patients (44 eyes) who underwent vitrectomy with or without lensectomy through a limbal or pars plana/plicata approach with diagnosis of anterior, posterior, or combined anterior and posterior segment PFV were included. The median age at the time of surgery was 3months (range, 1-36months), and the mean follow-up period was 37.2 ± 38.1months (range, 12-164months). Of the 44 eyes, 5 (11%) had clinical and ultrasonographic findings of anterior PFV, 5 (11%) had posterior PFV, and 34 (78%) had components of both anterior and posterior disease. At the last visit, 38 eyes (87%) were aphakic, 5 eyes (11%) were phakic, and 1 eye (2%) was pseudophakic. The mean Snellen equivalent VA at the final visit for the 14 (32%) eyes with measurable VA was 20/283. Nine (20%) eyes had final VA of only light perception or no light perception. The remaining 21 (48%) eyes behaved consistently with form vision, but the patients were not able to report VA. Most of the eyes achieved at least form vision, with acceptable postoperative complication rates. Functional and anatomical outcomes are not strictly dependent on axial length, and microphthalmic eyes with mild posterior segment involvement have the potential for good visual results. Outcomes after surgery for posterior PFV associated with tractional retinal detachment are limited. Anatomical success does not always equate to functional improvement.

Congenital cataract surgery without intraocular lens implantation in persistent fetal vasculature syndrome: Long-term clinical and functional results

To evaluate long-term clinical and functional outcomes after congenital cataract surgery in persistent fetal vasculature (PFV). Department of Ophthalmology, Goethe-University, Frankfurt, Germany. Retrospective comparative case series. The records of eyes with congenital cataract associated or not associated with PFV that had cataract surgery without intraocular lens (IOL) implantation within the first 18months of life were reviewed. Long-term visual outcomes and clinical parameters, including aphakic glaucoma, posterior capsule opacification (PCO), vitreous and anterior segment hemorrhage, retinal detachment (RD), and fibrin reaction, were evaluated. Congenital cataract was associated with PFV in 19 eyes (19 children) and not associated in 69 eyes (40 children). Sixteen patients had isolated anterior PFV; 3 eyes were classified as combined anterior and posterior PFV. The mean follow-up was 53.21months. Postoperative visual acuity improvement was recorded in 68.4% of PFV-associated cataract eyes with a corrected visual acuity up to 20/100. Long-term visual outcomes in PFV-associated cataract eyes were significantly worse than in the 11 unilateral cataract eyes without additional PFV (P=.0067). The presence of PFV was strongly associated with a long-term increased risk for postoperative hemorrhage (P=.0001) and RD (P=.009) after primary cataract surgery and secondary IOL implantation. The prevalence of aphakic glaucoma, PCO, and fibrinous reaction was similar between PFV cataract eyes and controls. In most cases of PFV-associated congenital cataract, postoperative visual acuity improvement is possible. However, congenital cataract eyes associated with PFV have a long-term increased risk for postoperative hemorrhage and RD. None of the authors has a financial or proprietary interest in any material or method mentioned.

Outcomes of pediatric cataract surgery in anterior persistent fetal vasculature

To report the intraoperative performance and postoperative outcomes in eyes with anterior persistent fetal vasculature (PFV). Iladevi Cataract & IOL Research Centre, India, and Filatov Institute Odessa, Ukraine. Prospective interventional case series. Eyes with anterior PFV had surgery using a standardized surgical technique. Plaque peeling was used for small plaques and partial excision for larger plaques. In eyes in which the entire lens converted into a fibrovascular tissue, extensive capsulectomy with anterior 2-port limbal vitrectomy was performed. Microphthalmic eyes had no intraocular lens implantation. This study comprised 33 eyes. The mean age at surgery was 6.30 months ± 5.16 (SD). Microcornea was observed in 10 eyes (30.3%). Within the morphology of cataract, 10 eyes (30.3%) had the lens converted into fibrovascular mass, of which 4 had associated prominent ciliary process. Anterior continuous curvilinear capsulorhexis (CCC) and manual posterior CCC were performed in 23 eyes (69.7%) and 3 eyes (9.1%), respectively. Intraoperatively, posterior capsule plaque was seen in 20 eyes (60.6%). In 31 eyes (93.9%), 2-port limbal anterior vitrectomy was performed and in 2eyes (6.1%), pars plana vitrectomy was performed. Intraocular lens implantation was performed in 16 eyes (48.5%); 17 eyes (51.5%) were left aphakic. Visual axis obscuration was observed in 6 eyes (18.2%). At the 3-year follow-up, visual acuity remained stable in 11 eyes (33.3%) and improved in 22 eyes (66.6%). The results suggest that good visual outcomes can be obtained in PFV eyes after surgical intervention, with an acceptable rate of serious postoperative complications. No author has a financial or proprietary interest in any material or method mentioned.

Visual acuity outcomes with and without surgery in patients with persistent fetal vasculature

Purpose To investigate visual acuity outcomes in patients with persistent fetal vasculature (PFV) left untreated or treated with vitreoretinal surgical techniques and to investigate clinical features associated with prognosis. Design Retrospective, noncomparative case series. Participants All patients with PFV examined at the Bascom Palmer Eye Institute from January 1, 1983 through December 31, 1998. Intervention All patients in the study had unilateral PFV. Of 42 PFV patients identified, 30 patients underwent vitreoretinal surgery. Indications for surgery included media opacity (e.g., cataract), vitreoretinal traction, and retinal detachment. Main outcome measures Final best postoperative visual acuity, prognostic ocular clinical features, and surgical complications. Results In the surgical group of patients, median age at diagnosis was 8 weeks, and median length of follow-up was 32 months, with all patients having at least 1 year of follow-up. Two patients had clinical and echographic findings consistent with anterior PFV, 2 patients had strictly posterior PFV, and the remaining 26 patients had components of both anterior and posterior PFV. Fourteen eyes (47%) achieved a final visual acuity of 20/400 or better at last follow-up. Risk factors for a poor visual acuity outcome (<20/400) included microphthalmia (28% of patients with microphthalmia versus 67% of patients with normal axial length achieved a final vision of 20/400 or better; P = 0.061) and preoperative retinal detachment or retinal or optic nerve abnormalities, or both, such as hypoplasia, folds, or indistinct macula with hypopigmentation (25% of patients with any of these anomalies versus 61% of patients without these findings achieved a final vision of 20/400 or better; P = 0.072). After surgery, retinal detachment developed in three eyes, chronic hypotony in two other eyes, and neovascular glaucoma in one eye. In the nonsurgical group there were 6 male and 6 female patients. Two patients with posterior PFV had minimal disease and were not considered surgical candidates, whereas 10 patients with combined anterior and posterior PFV had advanced pathologic features, and it was believed that surgery would not offer significant visual improvement; median age at diagnosis was 9.5 months, and median length of follow-up was 36 months, with all patients having at least 1 year of follow-up. At last follow-up, 3 eyes (25%) had a final visual acuity of 20/400 or better. During follow-up, retinal detachment developed in 2 eyes and chronic hypotony in an additional 2 eyes. Conclusions The current study indicates that approximately 50% of patients undergoing surgery for PFV will achieve useful vision. Visual acuity outcomes in patients with PFV are correlated with the nature and extent of ocular risk factors. Some patients may not be candidates for surgery because of either minimal changes or advanced disease that limit the potential of visual improvement.