Posterior Persistent Fetal Vasculature Research Articles

OCT angiography findings in posterior persistent fetal vasculature

Aims/Purpose: To report optical coherence tomography angiography findings in a patient presenting unilateral posterior persistent fetal vasculature (PVF).Methods: We report the case of a 19‐year‐old female patient with longstanding visual impairment of the left eye (LE). Patient was assessed with multimodal imaging including color fundus photography, swept source optical coherence tomography (SS‐OCT) and SS‐OCT angiography (SS‐OCTA) of the macula and the optic disc.Results: The patient had a history of unilateral visual impairment of unknown aetiology and consulted for a routine examination. Best corrected visual acuity was 10/10 in the right eye (RE) and limited to counting fingers in the LE. Ophthalmological examination of the LE revealed a mild exophoria, a quiet anterior segment, a vestigial capsular bag and an inferonasal whitish spot at the posterior lens capsule corresponding to Mittendorf's dot. Dilated fundus examination of the LE showed a lack of foveal reflex, retinal vessel contraction associated to the presence of a fibrovascular stalk extending from the OD to the crystalline lens via the vitreous humour, corresponding to fibrovascular contraction of the persistent and hyperplastic Cloquet's canal. No associated retinal detachment was noted. The clinical diagnosis of posterior PVF was confirmed. Macular SS‐OCT revealed retinal thinning with absence of foveal pit. Optic disc SS‐OCT highlighted a hyporeflective tubular structure overlying a Bergmeister'papilla. Cross‐sectional SS‐OCTA detected a flow signal at the level of Bergmeister's papilla, but no confirmed flow signal was identified at the level of the fibrovascular stalk.Conclusions: PVF is a rare condition characterized by fetal remnant from developmental vascular system defects, clinically recognized in its three forms (anterior, posterior and combined). OCT‐angiography is a useful non‐invasive tool to confirm of persistent hyaloid artery patency and assess risk of vascular complications. Contraction of the fibrovascular mass is prone to tractional retinal detachment and repeated episodes of intravitreal or intracapsular haemorrhage. Monitoring is therefore recommended even in adulthood.

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.

Lens-Sparing Surgery for Retrolental Stalk in Persistent Fetal Vasculature

To describe a new surgical technique and outcomes of lens-sparing vitrectomy and retrolental stalk dissection in posterior persistent fetal vasculature (PFV). Retrospective interventional case series. RESULTS: Among the 21 included eyes, 8 (38%) had no macular involvement and 4 (19%) presented with microphthalmia. Median age at the first surgery was 8 months (range: 1-113 months). Surgical success was obtained in 71.4% of cases (15 of 21). In the remaining cases, the lens was removed because of capsular rupture in 2 cases (9.5%) and a large capsular opacity after stalk removal or an adherent stalk impossible to dissect in 4 cases (19.1%). In the capsular bag, IOL implantation was accomplished for all but 1 eye. None of the eyes developed retinal detachment or required glaucoma surgery. Endophthalmitis occurred in 1 eye. Secondary lens aspiration was needed in 3 eyes after a mean interval of 10.7 months following initial surgery. At last follow-up, half of the eyes remained phakic. Lens-sparing vitrectomy is a useful approach to addressing the retrolental stalk in selected cases of persistent fetal vasculature syndrome. By delaying or avoiding lens extraction, this approach allows preservation of accommodation, reduction of the risk of aphakia, glaucoma, and development of secondary lens reproliferation.

Management and Outcomes of Posterior Persistent Fetal Vasculature

To describe the 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 at diagnosis: age, gender, presenting symptoms, intraocular pressure (IOP), visual acuity (VA). Patients were divided in four group depending on severity and involvement or not of the anterior segment. We reported the vitreoretinal surgical techniques used. Anatomical results, ocular hypertension, BCVA, presence of post-operative adverse events and additional surgical interventions were recorded at each follow-up visit. A total of 96 patients were included. Median age at diagnosis was 8 months (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. Posterior PFV was associated with anterior involvement in 62 eyes (64%). Forty-one eyes (42.7%) were microphthalmic and were more frequently associated with severe PFV [53% vs. 25%; (p=0.01)]. Surgery was performed in 85 patients (89%). Of them, 69 (81%) were a total success, 5 (6%) were a partial success due to a persistent limited peripheral retinal detachment (RD), and 11 (13%) were a failure due to persistent total RD after surgery. Twenty-four eyes presented post-operative adverse events including ocular hypertension requiring eye drop medication (6.6%), secondary cell proliferation around the IOL (7.7%), intravitreal hemorrhages (6.6%), persistent tractional RD (9.9%). A second surgery was performed in 15 patients (16%). At last follow-up, VA could be measured in logMAR in 43 children (45%), was light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%). In our case-series, most of patients presenting PFV with posterior involvement received complex vitreoretinal surgery. Goals of the surgery varies, and include retinal flattening, reduction of vitreoretinal traction, freeing of the visual axis and aesthetic concerns. We proposed a surgical and medical management algorithm for PFV.

A rare association of blepharophimosis–ptosis–epicanthus inversus syndrome with unilateral posterior persistent fetal vasculature

Blepharophimosis–ptosis–epicanthus inversus syndrome (BPES) is a rare genetic syndrome characterized by dysmorphism of ocular adnexa. We report a rare presentation of BPES and posterior persistent fetal vasculature (PFV) in a 10-year-old male child. A thorough evaluation showed all the classical features of BPES, calcified cataract in the left eye, and an echogenic mass arising from the optic disc with blood flow characteristic consistent with PFV. The coexistence of BPES and posterior PFV has never been reported in the literature before, and their co-occurrence poses a unique surgical challenge.

Surgical outcomes of posterior persistent fetal vasculature syndrome: cases with tent-shaped and closed funnel-shaped retinal detachment.

To determine the role of vitreoretinal surgery (VRS) for two different forms of posterior persistent fetal vasculature syndrome (PFVS); with tent-shaped tractional retinal detachment (TRD) and closed funnel-shaped TRD. Retrospective, single surgeon, consecutive case series of 52 eyes of 44 patients with posterior PFVS who underwent VRS. Cases were divided into "tent-shaped TRD" and "funnel-shaped TRD" groups based on the preoperative TRD configuration. Associated anomalies, functional and anatomical outcomes were evaluated. The cosmetic appearance was defined as poor if there was phthisis bulbi, gross buphthalmos, or corneal opacification; acceptable if there was apparent leukocoria; and excellent if none were noted at the last follow-up. Thirty eyes of 29 patients presented with tent-shaped TRD; 70% of which obtained counting fingers or better vision and 90% showed significant reversal of tenting achieving retinal reattachment. The cosmetic appearance was excellent in 87%. Two eyes (7%) became phthisic. Twenty-two eyes of 15 patients presented with funnel-shaped TRD and leukocoria; 45% achieved LP vision and 70% of patients with bilateral pathology had LP in at least one eye. The cosmetic appearance was acceptable to excellent in 73%. Three eyes (14%) became phthisic, one (5%) of which required enucleation. The median follow-up time was 16 (6-71) months. VRS often provides functional vision and anatomy in posterior PFVS with tent-shaped TRD morphology. In the funnel-shaped TRD morphology, where no treatment has historically been recommended, surgery may be considered with an aim of restoring light perception and globe preservation, particularly in bilateral cases.

Posterior persistent fetal vasculature associated with tractional retinal detachment

Posterior persistent fetal vasculature associated with tractional retinal detachment

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.

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.

Visual outcome in early vitrectomy for posterior persistent fetal vasculature associated with traction retinal detachment.

This study investigates the anatomical and visual outcome of vitrectomy for the treatment of unilateral posterior persistent fetal vasculature with associated traction retinal detachment in very young patients. A retrospective case series study from 1998 through 2010 of 11 eyes in 11 patients who underwent pars plana vitrectomy with or without lensectomy for unilateral posterior persistent fetal vasculature with traction retinal detachment affecting the macula. Ten of the 11 patients (91%) received surgical intervention when 13 months old or younger (average age, 4 months). Postoperatively, 6 of 10 eyes (60%) had 20/800 or better vision, 2 of which had 20/60 vision. All 10 patients in this group had their retinas reattached postoperatively with significant reversal of retinal dragging. One of the 11 patients (9%) received surgical intervention for posterior persistent fetal vasculature at 33 months of age. This patient had persistent traction retinal detachment and a postoperative visual acuity of hand motion. Postoperative glaucoma was detected in 4 patients (36%). This study suggests that early intervention in patients with unilateral posterior persistent fetal vasculature with associated macula affecting traction retinal detachment may provide a better visual and anatomical outcome when vitrectomy and retinal reattachment are performed at a very early age.

Persistent fetal vasculature: ocular features, management of cataract and outcomes

To describe ocular features, management of cataract and functional outcomes in patients with persistent fetal vasculature. Retrospective, descriptive case series of patients with persistent fetal vasculature. Data were recorded from the Congenital Cataract Section of Federal University of São Paulo, Brazil from 2001 to 2012. All patients were evaluated for sex, age at diagnosis, systemic findings, laterality, age at surgery, and initial and final follow-up visual acuities. Follow-up and complications after cataract surgery were recorded. Ultrasound was performed in all cases and ocular eco-Doppler was performed in most. The study comprised 53 eyes from 46 patients. Age at diagnosis ranged from 5 days of life to 10 years-old (mean 22.7 months). Twenty-seven patients were male (58.7%). Persistent fetal vasculature was bilateral in 7 patients (15.2%). Forty-two eyes (79.2%) had combined (anterior and posterior forms) PFV presentation, 5 eyes (9.4%) had only anterior persistent fetal vasculature presentation and 6 eyes (11.3%) had posterior persistent fetal vasculature presentation. Thirty-eight eyes (71.7%) were submitted to cataract surgery. Lensectomy combined with anterior vitrectomy was performed in 18 eyes (47.4%). Phacoaspiration with intraocular lens implantation was performed in 15 eyes (39.5%), and without lens implantation in 5 eyes (13.2%). Mean follow-up after surgery was 44 months. Postoperative complications were posterior synechiae (3 cases), retinal detachment (2 cases), phthisis (3 cases), posterior capsular opacification (8 cases), inflammatory pupillary membrane (5 cases), glaucoma (4 cases), intraocular lens implantation displacement (1 case) and vitreous hemorrhage (2 cases). Complications were identified in 19 (50%) of the 38 operated eyes. Visual acuity improved after cataract surgery in 83% of the eyes. Patients with persistent fetal vasculature have variable clinical presentation. There is an association of persistent fetal vasculature with congenital cataract. Severe complications are related to cataract surgery in patients with persistent fetal vasculature, but 83% of the operated eyes improved visual acuity.

Therapeutic effects of vitrectomy for persistent fetal vasculature

Objective To observe the effects of vitrectomy for persistent fetal vasculature (PFV).Methods The clinical data of 11 patients (14 eyes) with PFV, including posterior PFV (one eye) and combined PFV (13 eyes), were retrospectively analyzed. Vitrectomy was performed for all patients,lensectomy was performed for seven eyes, retinotomy was performed for five eyes, retinotomy and scleral buckling was performed for one eye. Intraoperative and postoperative complications were observed. The follow-up period ranged from eight to 74 months, with the mean of 44 months. The visual acuity (VA) and intraocular pressure before and after surgery were comparatively analyzed. Results There were no intraoperative complications in all the patients. The surgery was performed successfully in 12 eyes (86.0 %)and failed in two eyes (14. 0%). The postoperative VA of five eyes (35.7%) increased, four eyes (28. 6%)didn't change and five eyes (35.7%) decreased. The postoperative intraocular pressure of two eyes (14. 3%) increased, 11 eyes (78. 6%) didn't change and one eye (7. 1%) decreased. There was one eye with persistent hypotension and one eye with recurrent retinal detachment after surgery. Conclusion Vitrectomy can prevent complications and improve visual acuity in some PFV patients. Key words: Persistent hyperplastic primary vitreous/surgery; Vitrectomy

Bilateral persistent fetal vasculature: A study of 11 cases

To describe the clinical and radiologic features of patients with bilateral persistent fetal vasculature (PFV). All patients diagnosed with symmetric, bilateral PFV over a 3.5-year period were included in this representative, observational case series. Only patients with more or less symmetrical disease in both eyes were included. Examination under anesthesia was performed in all but 1 case. B-scan ultrasonography of the eye was performed in all cases. A computed tomographic or magnetic resonance imaging scan was done if the ocular ultrasonography was inconclusive. A systemic workup was performed to rule out associated systemic anomalies. The study group included 11 patients. Of the 22 eyes studied, 20 (91%) showed features of both the anterior and the posterior forms of PFV. Two children tested positive for IgM rubella antibodies. Ten eyes (45.5%) had a horizontal corneal diameter of less than 10 mm. Fifteen eyes (68.2%) had cataract at presentation. Intraocular pressure was >21 mm Hg in 17 eyes (77.3%). Microcornea and cataract were common findings. Bilateral PFV cases were more commonly associated with combined anterior and posterior PFV. Bilateral PFV, although infrequent, should be considered in the differential diagnosis of bilateral leukocoria.

EARLY VITRECTOMY EFFECTIVE FOR BILATERAL COMBINED ANTERIOR AND POSTERIOR PERSISTENT FETAL VASCULATURE SYNDROME

The purpose of this study was to review our surgical experience with patients with bilateral combined anterior and posterior persistent fetal vasculature syndrome (PFVS). We retrospectively reviewed the charts of all patients seen in our tertiary care pediatric retinal practice from 1988 to 2008 with a potential diagnosis of bilateral PFVS with posterior involvement. Clinical diagnosis required the presence of either bilateral persistent hyaloidal stalk tissue with retinal involvement or bilateral dense retrolental fibrovascular plaques (usually with no posterior view preoperatively) without a family history or genetic testing consistent with Norrie disease or familial exudative vitreoretinopathy. Chart review showed 22 vitrectomized patients with clinical findings consistent with bilateral PFVS with posterior involvement who did not have a family history or genetic testing consistent with Norrie disease or familial exudative vitreoretinopathy. All 22 of these patients with posterior retinal involvement also had anterior findings and thus can be classified as combined anterior and posterior PFVS. Of the 13 patients with visual acuity follow-up data, 9 patients (69%) maintained at least light perception vision in at least 1 eye at last follow-up. Of the 28 operated eyes in 16 patients with follow-up data, 3 eyes (11%) were phthisical at last follow-up. Children with bilateral PFVS with posterior retinal involvement have a dismal visual prognosis if left unoperated. In this relatively large series of a rare condition, we find that vitrectomy with or without lensectomy is beneficial in bilateral combined anterior and posterior PFVS in two regards: maintenance or restoration of vision and avoidance of phthisis bulbi.

Intraoperative performance and postoperative outcomes of cataract surgery in infant eyes with microphthalmos

To report the intraoperative performance and postoperative outcomes in microphthalmic eyes of infants younger than 1 year having bilateral cataract surgery. Iladevi Cataract & IOL Research Centre, Ahmedabad, India. This prospective observational study excluded eyes with ocular trauma, inflammation, posterior persistent fetal vasculature causing stretching of the ciliary processes or tractional retinal detachment, aniridia, or chorioretinal coloboma. Intraoperative observations included the rate of peripheral extension of the anterior capsule tear, intraoperative iris prolapse/trauma, and posterior capsule tear. All eyes were left aphakic. Postoperative observations included visual outcome, posterior synechias, visual axis obscuration (VAO), and glaucoma. The study evaluated 42 eyes of 21 infants. The mean age at surgery was 4.0 months +/- 2.6 (SD) and the mean follow-up, 25.6 +/-11.3 months. Surgery was performed using the limbal approach in 30 eyes (71.4%) and by pars plicata lensectomy in 12 eyes (28.6%). In the limbal group, 3 eyes (10.0%) had an incomplete anterior capsulorhexis, 2 eyes (6.7%) had iris trauma, and 2 eyes (6.7%) had peripheral extension of the posterior capsulectomy edge. At the final follow-up, visual acuity remained stable in 4 eyes (9.5%) and was improved in 38 eyes (90.5%). The complications were posterior synechias in 15 eyes (35.7%), glaucoma in 13 eyes (30.9%), and VAO in 7 eyes (16.7%). The results suggest that good visual outcomes can be obtained in microphthalmic patients with bilateral congenital cataract after early surgical intervention, with an acceptable rate of serious postoperative complications.

Lens-sparing vitrectomy in predominantly posterior persistent fetal vasculature syndrome in eyes with nonaxial lens opacification.

To determine indications and document anatomic and functional outcomes for lens-sparing vitreous surgery in eyes with predominantly posterior persistent fetal vasculature syndrome and nonaxial lens opacification. Retrospective interventional clinical series. The authors performed lens-sparing vitreous surgery on nine eyes of infants with predominantly posterior persistent fetal vasculature syndrome and nonaxial lens opacification without removing the crystalline lens. Indications for surgery included primarily posterior complications of persistent fetal vasculature syndrome (localized tractional retinal detachment), a centrally clear lenticular axis, and frequently strabismus. Postoperatively, seven of nine eyes showed no evidence of increasing lens opacification with a median follow-up period of 16.8 months (range, 5-29 months). The first eye operated on developed a rapidly progressive cataract and prompted modification of the surgical technique. One additional eye also required cataract extraction for progressive lenticular opacification 23 months after surgery. No eyes developed postoperative retinal detachment, and all eyes maintained vision at the final follow-up. Of the seven eyes with strabismus, five improved after vitreous surgery alone, and two required additional strabismus surgery. This series shows that, in selected cases of persistent fetal vasculature syndrome that are predominantly posterior in nature with nonaxial lens opacification, lens-sparing vitrectomy can be of benefit.

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.