Visual prognosis of posterior and combined persistent fetal vasculature.

INTRODUCTION: The persistent fetal vasculature refers to congenital anomalies of the globe resulting from the abnormal persistence of the hyaloid vascular system. It can present as anterior, posterior, or combined form. The aim of this study was to report the visual outcomes of posterior and combined forms of persistent fetal vasculature. METHODS: This retrospective, single-center study included every patient referred to our outpatient clinic with a posterior or combined form of persistent fetal vasculature. The primary endpoint was the visual acuity of the impaired eye, or of the best eye if bilateral, at the end of follow-up. RESULTS: In total, 18 eyes of 14 patients (10 males) were included. The combined form was the most prevalent (12 of 18 eyes), and 4 of 14 patients had bilateral impairment. The range of assessed visual acuity was from 20/2000 to 20/25. The best visual acuity in patients having undergone a surgical procedure was 20/63 (cataract extraction = 3, combined phacovitrectomy = 1). In patients who had been treated for amblyopia with patching, without surgery, the best visual acuity measured was 20/100 (5 patients). Among patients who had neither surgery nor patching therapy, there was one 63-year-old patient with a 20/25 visual acuity; the other ones had a low visual acuity of less than 20/200. All included eyes presented with nystagmus, amblyopia, and/or strabismus at the end of follow-up. CONCLUSION: The posterior and combined forms of persistent fetal vasculature are of poor visual prognosis. The severe or occulting presentations require surgery to obtain the same visual outcomes as the moderate forms treated for amblyopia with patching therapy.

Regression of fetal vasculature and visual improvement in nonsurgical persistent hyperplastic primary vitreous: a case report.

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is a rare congenital developmental ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature. Here we report a case of nonsurgical unilateral anterior PHPV that was managed by amblyopia treatment and resulted in an improvement of visual acuity and regression of the fetal vasculature. CASE PRESENTATION: A three-year-old girl was diagnosed with unilateral anterior PHPV in the left eye, manifested with posterior pole cataract, posterior capsule opacification, tunica vasculosa lentis, and a floating hyaloid artery connected to the retrolental mass. The plaque was not large enough to fill the pupil, and conservative management along with amblyopia treatment was conducted. Nineteen months later, the visual acuity in the affected eye improved from 20/100 to 20/50 with correction, and the fetal vasculature regressed gradually and finally into a nonperfusion ghost vessel. CONCLUSIONS: In PHPV-affected children, regression of the fetal vasculature may be observed, and conservative management and amblyopia treatment may be helpful for visual improvement.

Visual and Anatomical Outcomes With Vitrectomy in Posterior or Combined Persistent Fetal Vasculature in an Asian Population.

BACKGROUND AND OBJECTIVE: To investigate clinical features and surgical outcomes of vitrectomy in posterior or combined persistent fetal vasculature (PFV) in an Asian pediatric population. PATIENTS AND METHODS: This study was a retrospective, noncomparative, interventional case series relating the surgical outcome of PFV. Eyes that underwent pars plicata vitrectomy and/or lensectomy for posterior or combined PFV between 2006 and 2015 were included. The main outcome measures were the anatomic and functional results as well as the complications after the vitrectomy with or without lensectomy. RESULTS: A total of 25 eyes of 18 patients younger than 8 years of age were included in the study. The mean age of the patients receiving first pars plicata vitrectomy and/or lensectomy was 15.2 months ± 21.7 months (range: 1 month to 83 months). Postoperatively, successful anatomic correction in the posterior segment was observed in 20 eyes (80%). In addition, 19 of the 25 eyes (76%) had visual acuity (VA) better than 20/4000, and the mean logMAR VA of these 19 eyes was 1.74 (range: 0.48 to 2.30). The mean change of axial length of the eyes receiving surgery was 0.7 mm ± 1.4 mm (range: -1.0 mm to 2.4 mm; P = .18). None of the patients ended up with phthisis or glaucoma. CONCLUSIONS: This study suggests that vitrectomy and/or lensectomy in patients with posterior or combined PFV with macular involvement may result in an acceptable anatomical outcome; however, the functional outcome remained poor despite surgical intervention in these patients. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:377-384.].

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

PURPOSE: To investigate the functional and anatomical outcomes following surgical management of persistent fetal vasculature (PFV). METHODS: 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. RESULTS: The median age at the time of surgery was 3 months (range, 1-36 months), and the mean follow-up period was 37.2 ± 38.1 months (range, 12-164 months). 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. CONCLUSIONS: 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.

Different gene knockout/transgenic mouse models manifesting persistent fetal vasculature: Are integrins to blame for this pathological condition?

Persistent fetal vasculature (PFV) occurs as a result of a failure of fetal vasculature to undergo normal programmed involution. During development, before the formation of retinal vessels, the lens and the inner retina are nourished by the hyaloid vasculature. Hyaloid vessels extend from the optic nerve and run through the vitreous to encapsulate the lens. As fetal retinal vessels develop, hyaloid vasculature naturally regresses. Failure of regression of the hyaloid artery has been shown to lead to severe congenital pathologies. Studies on childhood blindness and visual impairment in the United States have shown that PFV accounts for 4.8% of total blindness. Although PFV is a serious developmental disease affecting the normal visual development pathway, the exact regulatory mechanism responsible for the regression of the hyaloid artery is still unknown. In this review, we have summarized the cellular defects associated with different knockout models that manifest features of persistent fetal vasculature. Based on similar cellular defects observed in different knockouts (KO)s such as altered migration, increased proliferation and decreased apoptosis and, the known role of integrins in the regulation of these cellular behaviors, we propose here that integrins may play a significant role in the pathophysiology of persistent fetal vasculature disease.

Papila de Bergmeister / Bergmeister’s papilla

No disponible

Canal de Cloquet o conducto de Stilling y espacio de Martegiani / Cloquet's canal or Stilling's duct and Martegiani's space

No disponible

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

PURPOSE: 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. METHODS: 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. RESULTS: 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%). CONCLUSION: 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.

Alterations of the tunica vasculosa lentis in the rat model of retinopathy of prematurity.

PURPOSE: To study the relationship between retinal and tunica vasculosa lentis (TVL) disease in retinopathy of prematurity (ROP). Although the clinical hallmark of ROP is abnormal retinal blood vessels, the vessels of the anterior segment, including the TVL, are also altered. METHODS: ROP was induced in Long-Evans pigmented and Sprague Dawley albino rats; room-air-reared (RAR) rats served as controls. Then, fluorescein angiographic images of the TVL and retinal vessels were serially obtained with a scanning laser ophthalmoscope near the height of retinal vascular disease, ~20 days of age, and again at 30 and 64 days of age. Additionally, electroretinograms (ERGs) were obtained prior to the first imaging session. The TVL images were analyzed for percent coverage of the posterior lens. The tortuosity of the retinal arterioles was determined using Retinal Image multiScale Analysis (Gelman et al. in Invest Ophthalmol Vis Sci 46:4734-4738, 2005). RESULTS: In the youngest ROP rats, the TVL was dense, while in RAR rats, it was relatively sparse. By 30 days, the TVL in RAR rats had almost fully regressed, while in ROP rats, it was still pronounced. By the final test age, the TVL had completely regressed in both ROP and RAR rats. In parallel, the tortuous retinal arterioles in ROP rats resolved with increasing age. ERG components indicating postreceptoral dysfunction, the b-wave, and oscillatory potentials were attenuated in ROP rats. CONCLUSIONS: These findings underscore the retinal vascular abnormalities and, for the first time, show abnormal anterior segment vasculature in the rat model of ROP. There is delayed regression of the TVL in the rat model of ROP. This demonstrates that ROP is a disease of the whole eye.

The deletion of Math5 disrupts retinal blood vessel and glial development in mice.

Retinal vascular development is a complex process that is not yet fully understood. The majority of research in this area has focused on astrocytes and the template they form in the inner retina, which precedes endothelial cells in the mouse retina. In humans and dogs, however, astrocyte migration follows behind development of blood vessels, suggesting that other cell types may guide this process. One such cell type is the ganglion cell, which differentiates before blood vessel formation and lies adjacent to the primary retinal vascular plexus. The present study investigated the potential role played by ganglion cells in vascular development using Math5(-/-) mice. It has previously been reported that Math5 regulates the differentiation of ganglion cells and Math5(-/-) mice have a 95% reduction in these cells. The development of blood vessels and glia was investigated using Griffonia simplicifolia isolectin B4 labeling and GFAP immunohistochemistry, respectively. JB-4 analysis demonstrated that the hyaloid vessels arose from choriovitreal vessels adjacent to the optic nerve area. As previously reported, Math5(-/-) mice had a rudimentary optic nerve. The primary retinal vessels did not develop post-natally in the Math5(-/-) mice, however, branches of the hyaloid vasculature eventually dove into the retina and formed the inner retinal capillary networks. An astrocyte template only formed in some areas of the Math5(-/-) retina. In addition, GFAP(+) Müller cells were seen throughout the retina that had long processes wrapped around the hyaloid vessels. Transmission electron microscopy confirmed Müller cell abnormalities and revealed disruptions in the inner limiting membrane. The present data demonstrates that the loss of ganglion cells in the Math5(-/-) mice is associated with a lack of retinal vascular development.

Hemorragia vítrea asociada a arteria hialoidea persistente / Vitreous haemorrhage associated with persistent hyaloid artery

Introducción: La hemorragia vítrea secundaria a una arteria hialoidea persistente (AHP) íntegra es una anomalía infrecuente. Caso clínico: Una mujer de 52 años presentó disminución de visión en su ojo ambliope. La biomicroscopia reveló una AHP rellena de sangre que se insertaba en la cristaloides posterior. En la oftalmoscopia se objetivó un hemovítreo leve. La tomografía de coherencia óptica (TCO) permitió analizar la estructura tubular de la AHP mostrando un interior hiporreflectivo. Sin embargo, el eco-doppler no detectó la presencia de flujo sanguíneo activo en su interior. Conclusiones: La realización de pruebas de imagen no invasivas que incluyen la TCO y el eco-doppler pueden ser útiles en el estudio de una hemorragia asociada a una AHP(AU)

Introduction: Haemorrhage from an entire persistent hyaloid artery (PHA) is an uncommon anomaly. Case report: A 52 year old female presented with blurred vision in her amblyopic eye. Biomicroscopy revealed a blood filled PHA which was inserted in the posterior crystalloid. Ophthalmoscopy showed a mild vitreous haemorrhage. A tubular structure showing a hypo-reflexivity inside the PHA was able to be analysed using optical coherence tomography (OCT). However, Doppler ultrasound did not detect the presence of active blood flow within it. Conclusions: The performance of non-invasive imaging tests such as the OCT and Doppler ultrasound may be useful in the study of vitreous haemorrhage associated with PHA(AU)

A developmental defect in astrocytes inhibits programmed regression of the hyaloid vasculature in the mammalian eye.

Previously we reported the novel observation that astrocytes ensheath the persistent hyaloid artery, both in the Nuc1 spontaneous mutant rat, and in human PFV (persistent fetal vasculature) disease (Developmental Dynamics 234:36-47, 2005). We now show that astrocytes isolated from both the optic nerve and retina of Nuc1 rats migrate faster than wild type astrocytes. Aquaporin 4 (AQP4), the major water channel in astrocytes, has been shown to be important in astrocyte migration. We demonstrate that AQP4 expression is elevated in the astrocytes in PFV conditions, and we hypothesize that this causes the cells to migrate abnormally into the vitreous where they ensheath the hyaloid artery. This abnormal association of astrocytes with the hyaloid artery may impede the normal macrophage-mediated remodeling and regression of the hyaloid system.

Visual and anatomic outcomes with or without surgery in persistent fetal vasculature.

PURPOSE: To determine visual and anatomic outcomes for patients with persistent fetal vasculature (PFV) observed or treated with surgery. DESIGN: Retrospective interventional consecutive case series. PARTICIPANTS: A total of 165 eyes of 150 patients diagnosed with PFV between January 1, 1983, and December 31, 2006, at the Bascom Palmer Eye Institute. INTERVENTION: Patients with media opacity, progressive glaucoma, or retinal detachment who were deemed to have visual potential underwent lensectomy, 3-port vitrectomy, or both, through a limbal or pars plicata/plana approach. MAIN OUTCOME MEASURES: Vision, postsurgical retinal attachment, lens status, need for eye removal, and rate of complications. RESULTS: Of the 81 eyes that underwent surgical repair, 70 had at least 6 months of follow-up (median 47 months) and 49 (70.0%) had form vision, defined as finger counting, fix-and-follow, or better. Twenty (95.2%) of 21 eyes without form vision had a significant posterior component of PFV (P < 0.001). Forty eyes had limbal incisions, and 30 eyes had pars plicata incisions. The choice of surgical approach did not have a statistically significant effect on final visual acuity or rate of complications (P=0.43). Postoperative events occurred in 28 eyes (40.0%). Retinal attachment was achieved in 54 eyes (77.1%), and 61 eyes (87.1%) were left aphakic. Eighty-four eyes were not offered surgery, of which 78 eyes (92.9%) had a posterior component of PFV. The median age at diagnosis was greater compared with the surgical group (197 vs. 67.5 days, P=0.00545). Fifty-eight eyes (69.0%) lacked form vision, and 39 eyes (46.4%) had no light perception. Posterior manifestations of PFV, bilaterality, and microphthalmia were associated with poorer visual outcomes (P < 0.001, 0.041, and 0.002, respectively). CONCLUSIONS: The majority of patients receiving surgical intervention for PFV achieved form vision. Posterior disease, microphthalmia, glaucoma, and amblyopia limited visual acuity outcomes even after aggressive intervention. The choice of limbal versus pars plicata approach produced similar visual and anatomic outcomes without a significant difference in rate of complications. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome.

PURPOSE: The purpose of this study was to review our surgical experience with patients with bilateral combined anterior and posterior persistent fetal vasculature syndrome (PFVS). METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Combined ultrasound study in the prediction of the results of surgical treatment for persistent hyperplastic primary vitreous body in children].

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.