Persistent Hyperplastic Primary Vitreous Research Articles

PATHOLOGY OF THE VITREOUS BODY IN CHILDREN. POSSIBILITIES OF INSTRUMENTAL AND LASER SURGERY

Despite the rather active use of YAG-laser surgery in adult patients, it is practically not used on children. At the same time, some children have a vitreous pathology what can be operate with laser surgery - changes in the vitreous body can be a manifestation of the congenital persistent fetal vasculature syndrome, retinopathy of prematurity, effects of the injuries, operations and uveitis. The opacites and synechia forming of the vitreous body reduce acuity and often adduce to the traction, which can lead to detachment of the retina, ciliary body, progress of eye subatrophy. The review presents the main pathological conditions of the vitreous body with syndromes, congenital and systemic diseases in children, the possibilities of instrumental and laser vitreal surgery in children. A literature search for the review was conducted using the Medline and RINC database.

Anterior–Posterior Persistent Fetal Vasculature With Multiple Stalks: Persistent Vasa Hyaloidea Propria

An 8-week-old boy referred for an abnormal pupil was found to have an atypical presentation of persistent fetal vasculature (PFV) with multiple vascular stalks. Examination under anesthesia with fluorescein angiography (FA) revealed 2 perfused persistent hyaloid vessels, one extending from the optic disc and another from the inferonasal retina. These vessels meet anteriorly to form a vascular network at the lens, which is the remnant of the tunica vasculosa lentis. Although the posterior portion of PFV typically presents as a single stalk attached at the optic disc, this case features an atypical presentation of 2 distinct vascular stalks, which may expand our understanding of ocular development and pathogenesis of PFV. We hypothesize that the aberrant additional stalk may represent failure of the vasa hyaloidea propria (tributaries of the hyaloid artery) to regress.

Unilateral Congenital Cataract: Clinical Profile and Presentation.

To study the clinical profile and presentation of children with unilateral cataract. In this hospital-based, observational, cross-sectional study, patients 15 years of age or younger who presented with unilateral cataract were recruited. Cases of cataract secondary to causes such as trauma or uveitis were excluded. Age at detection and presentation, distance from the treatment center, presenting complaints, cataract morphology, and biometry were noted for each case. A total of 76 patients were recruited. Most patients presented with complaints of leukocoria. Persistent fetal vasculature accounted for 27.6% of cases and was the most common identifiable cause of cataract in this study. Subsequently, patients were divided into two groups: no persistent fetal vasculature (control) and persistent fetal vasculature. A male predominance was noted in both groups. The mean age at detection was 27.58 ± 37.02 and 6.17 ± 8.42 months and the mean age at presentation was 55.613 ± 45.21 and 14.83 ± 17.75 months in the control and persistent fetal vasculature groups, respectively. In the persistent fetal vasculature group, a significant difference was noted in the axial length, keratometry, and corneal diameter between the affected and normal eyes (P = .027, .00176, and .0114, respectively). In the control group, this difference was observed only in keratometry readings (P = .0464). The mean distance traveled by patients to reach the treatment center was 211 km. Persistent fetal vasculature is an important and less identified cause of unilateral cataract. A significant delay is noted in the detection and presentation of unilateral cataract. [J Pediatr Ophthalmol Strabismus. 2018;55(2):107-112.].

MPS VII mice display reduced circulating IGF1 and disrupted cell cycle progression in the growth plate

To develop a nomogram based on age and disease type for sclerotomy placement in pars plana vitrectomy for infants and children with congenital and acquired vitreoretinopathies.A retrospective, single-center, single-surgeon comparative case series.A total of 171 eyes of 93 patients ranging in age from a postmenstrual age (PMA) of 34 weeks to a chronological age of 23 years with congenital and acquired vitreoretinopathies.The corneal white-to-white diameter and the distance between the ora serrata and surgical limbus (ora-limbus distance) in the nasal, temporal, inferior, and superior quadrants were measured externally in vivo under general anesthesia prior to planned pars plana vitrectomy or laser procedure. The average ora-limbus distance of the quadrants was calculated for each eye (mean ora-limbus distance); and mean ora-limbus distance was evaluated as a function of age and compared among age-matched subjects from control and disease groups. Results were used to create a nomogram for safe sclerotomy placement based on age and disease type.The main outcome measure was distance (millimeters) from the ora serrata to the surgical limbus; a secondary measurement was corneal white-to-white distance (millimeters).Among all subjects, the ora-limbus distance was greatest in the superior quadrant, followed by the temporal, inferior, and nasal quadrants. A positive logarithmic relationship between ora-limbus distance and age was demonstrated in all groups. Whereas age-adjusted mean ora-limbus distances were similar among the familial exudative vitreoretinopathy and control groups, growth trends differed in patients with coloboma, persistent fetal vasculature, Stickler syndrome and/or myopia, and retinopathy of prematurity (ROP). In ROP, when subjects <12 months of age were considered separately, a negative correlation was found between the ora-limbus distance and PMA, with infants of lesser gestational age exhibiting greater restriction in ora-limbus distance.Placement of sclerotomies for pars plana vitrectomy in children requires consideration of ocular development in congenital and acquired vitreoretinopathies; we present an age-based nomogram for sclerotomy placement in infants and children based on analyses of ora-limbus distance in several pediatric ocular conditions.

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.

Persistent hyaloid artery with posterior polar cataract in a young male: A rare presentation

Persistent fetal vasculature (PFV) is a rare congenital developmental malformation of the eye which results from a failure of the fetal hyaloid artery to involute. PFV is usually characterized by microphthalmia, a shallow anterior chamber, elongated ciliary processes, a posterior subcapsular cataract, and a fibrovascular stalk that extends from the optic disc to the lens. It is a rare and sporadic disease with an overall prevalence of 0.064%. We report a case of a young male who presented with decreased vision in the right eye. On examination, it was diagnosed with complete persistent hyaloid artery. The patient was advised regular follow-up.

Retinal detachments in the pediatric population.

In this review, we present a concise summary of the more commonly seen types of retinal detachments (RDs) that one can encounter in pediatric patients. A spectrum of diseases from rhegmatogenous RD in Stickler syndrome, Marfan syndrome, and choroidal coloboma to exudative RD in Coats disease, to tractional RD in persistent fetal vasculature, and combined RDs in familial exudative vitreoretinopathy are described with the management pearls for each.

Outcomes of small gauge pars plicata vitrectomy for patients with persistent fetal vasculature: a report of 105 cases.

To evaluate the surgical outcomes in eyes with persistent fetal vasculatures (PFV) managed by small gauge pars plicata vitrectomy. Consecutive patients with PFV treated by small gauge pars plicata vitrectomy at Beijing Tongren Eye Center between January 2010 and January 2013 were retrospectively reviewed. A total of 118 eyes of 105 patients with PFV were included and undergone small gauge pars plicata vitrectomy, of which 84 (71.2%) eyes had lensectomy and 16 (13.6%) eyes had lens aspiration and immediate intraocular lens implantation. The percentage of sutured scleral incision of 23 gauge vitrectomy (71.7%, 33/46) was higher than that of the 25 gauge vitrectomy (18.1%, 13/72). At last follow-up, visual acuity remained stable in 34 eyes (28.8%) and improved in 84 eyes (71.2%). Age at surgery (less than 2y), anterior type of PFV, and immediate IOL implantation were associated with postoperative improved visual acuity. Sixty five (55.1%) eyes had retinal detachment preoperatively, among which 33 (50.8%, 33/65) eyes had retinal reattachment or partial retinal reattachment. The results suggest that cases with PFV have a potential for developing good visual acuity after small gauge pars plicata vitrectomy with favorable anatomic outcomes and acceptable rate of serious surgical complications.

Myosin-X knockout is semi-lethal and demonstrates that myosin-X functions in neural tube closure, pigmentation, hyaloid vasculature regression, and filopodia formation

Myosin-X (Myo10) is an unconventional myosin best known for its striking localization to the tips of filopodia. Despite the broad expression of Myo10 in vertebrate tissues, its functions at the organismal level remain largely unknown. We report here the generation of KO-first (Myo10tm1a/tm1a), floxed (Myo10tm1c/tm1c), and KO mice (Myo10tm1d/tm1d). Complete knockout of Myo10 is semi-lethal, with over half of homozygous KO embryos exhibiting exencephaly, a severe defect in neural tube closure. All Myo10 KO mice that survive birth exhibit a white belly spot, all have persistent fetal vasculature in the eye, and ~50% have webbed digits. Myo10 KO mice that survive birth can breed and produce litters of KO embryos, demonstrating that Myo10 is not absolutely essential for mitosis, meiosis, adult survival, or fertility. KO-first mice and an independent spontaneous deletion (Myo10m1J/m1J) exhibit the same core phenotypes. During retinal angiogenesis, KO mice exhibit a ~50% decrease in endothelial filopodia, demonstrating that Myo10 is required to form normal numbers of filopodia in vivo. The Myo10 mice generated here demonstrate that Myo10 has important functions in mammalian development and provide key tools for defining the functions of Myo10 in vivo.

Osseous and Adipocytic Differentiations in the Intraocular Lens and Vitreous

To analyze 3 unusual mesenchymal transformations within the eye: adipose or osseous metaplasia of the lens and adipose tissue in the vitreous cavity. Observational case series. Reevaluation of clinicopathologic diagnoses and histopathologic findings in sections stained with hematoxylin-eosin, periodic acid-Schiff (PAS) reaction, and Masson trichrome method. The 3 cases of mesenchymal transformation occurred in microphthalmic eyes with persistent hyperplastic primary vitreous (more recently termed persistent fetal vasculature). In 1 case there was total lens replacement with lamellar bone; in another, total replacement of the crystalline lens by adipose tissue; and in a third, an anomalous pocket of adipose tissue in the central vitreous. Multifocal remnants of the lens capsule were seen in the osseous case but were absent from the adipocytic cases. The vitreous adipose tissue was surrounded by an elaborate capillary plexus with an empty, collapsed PAS-positive lens capsule in the pupillary region. Anterior pigmented neuroectodermal disorganization, dysgenesis of angle structures, and a hypoplastic or disorganized iris were also observed in the 3 cases. After review of the literature, it appears that lenticular osseous replacement occurs more often than adipocytic. In addition to vascularization of the lens through a capsular dehiscence, other causes are explored, including direct epithelial-mesenchymal transformations of the lens epithelium or, less likely, of the disorganized adjacent neuroectoderm. The focus of vitreous adipose tissue may represent a transformed luxated lens extruded from its capsule, which was left behind in the pupillary zone.

Diagnosis Please Certificates of Recognition Awarded to Three Individuals and to International and North American Radiology Resident Groups.

HomeRadiologyVol. 285, No. 2 PreviousNext CommunicationsFrom the EditorDiagnosis Please Certificates of Recognition Awarded to Three Individuals and to International and North American Radiology Resident GroupsHerbert Y. KresselHerbert Y. KresselHerbert Y. KresselPublished Online:Oct 18 2017https://doi.org/10.1148/radiol.2017171839MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Article HistoryPublished online: Oct 18 2017Published in print: Oct 2017 FiguresReferencesRelatedDetailsRecommended Articles Diagnosis Please Certificates of Recognition Awarded to Five Individuals and to International and North American Radiology Resident GroupsRadiology2020Volume: 297Issue: 2pp. 247-250Looking AheadRadiology: Artificial Intelligence2019Volume: 1Issue: 6Congratulations to the 2018 Editorial FellowsRadiology2018Volume: 289Issue: 1pp. 1-2Congratulations to the 2018 Editorial FellowsRadioGraphics2018Volume: 38Issue: 5pp. 1287-1288Diagnosis Please Certificates of Recognition Awarded to Five Individuals and to International and North American Radiology Resident GroupsRadiology2016Volume: 281Issue: 2pp. 329-332See More RSNA Education Exhibits Engaging Medical Students in Interventional Radiology: In the Era of the New Integrated IR ResidencyDigital Posters2018Occupational Radiation Exposure of the Eye in Neurovascular Interventional PhysicianDigital Posters2018Can You Hear Me Now? Implementation of Telemedicine (TM) in an Academic Interventional Radiology (IR) Practice during the COVID-19 PandemicDigital Posters2020 RSNA Case Collection Persistent fetal vasculature (PFV)RSNA Case Collection2020"Cold" Sentinel Lymph Node in Cutaneous Melanoma RSNA Case Collection2020Primary pancreatic lymphoma RSNA Case Collection2020 Vol. 285, No. 2 Metrics Altmetric Score PDF download

The status and progress of morning glory syndrome

Morning glory syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function. Key words: optic nerve disease/congenital; retinal detachment/treatment; photocoagulation; vitrectomy; association; treatment; review

Outcomes of cataract surgery in children with persistent hyperplastic primary vitreous.

To evaluate postoperative outcomes of cataract surgery in children with persistent hyperplastic primary vitreous (PHPV) and factors affecting outcomes. We analyzed 29 eyes of 28 children with PHPV presenting at a tertiary care center in northern India. All eyes underwent phacoaspiration followed by primary posterior capsulotomy with or without cauterization of persistent fetal vasculature under general anesthesia. Hydrophobic intraocular lens was implanted in selective cases. Postoperative outcomes such as clarity of visual axis, need for secondary surgical procedures, and complications were noted. Out of a total of 28 children (16 male and 12 female), 27 were unilateral while 1 patient had bilateral involvement. Mean age at surgery was 25.14 months, ranging from 2 months to 144 months. Mean axial length of the globe at surgery was 19.66 ± 2.28 mm. Cataract surgery with intraocular lens implantation was done in 22 eyes whereas 7 were left aphakic. The most common intraoperative complication noted was intraoperative bleed in 11 eyes (37.9%) resulting in postoperative hyphema in 9 eyes (31%) and vitreous hemorrhage in 8 eyes (27.5%). Visual axis opacification was seen in 12 eyes and all required membranectomy. Three eyes developed glaucoma while retinal detachment was noted in 2 eyes and 1 of them became phthisical. Favorable outcome was more often achieved in anterior PHPV. Surgical outcomes in eyes with PHPV undergoing cataract surgery are limited by intraoperative and postoperative complications such as hyphema, vitreous hemorrhage, recurrent visual axis opacification, glaucoma, and retinal detachment.

Tetrahydrobiopterin (BH4) deficiency is associated with augmented inflammation and microvascular degeneration in the retina

BackgroundTetrahydrobiopterin (BH4) is an essential cofactor in multiple metabolic processes and plays an essential role in maintaining the inflammatory and neurovascular homeostasis. In this study, we have investigated the deleterious effects of BH4 deficiency on retinal vasculature during development.Methodshph-1 mice, which display deficiency in BH4 synthesis, were used to characterize the inflammatory effects and the integrity of retinal microvasculature. BH4 levels in retinas from hph-1 and wild type (WT) mice were measured by LC-MS/MS. Retinal microvascular area and microglial cells number were quantified in hph-1 and WT mice at different ages. Retinal expression of pro-inflammatory, anti-angiogenic, and neuronal-derived factors was analyzed by qPCR. BH4 supplementation was evaluated in vitro, ex-vivo, and in vivo models.ResultsOur findings demonstrated that BH4 levels in the retina from hph-1 mice were significantly lower by ~ 90% at all ages analyzed compared to WT mice. Juvenile hph-1 mice showed iris atrophy, persistent fetal vasculature, significant increase in the number of microglial cells (p < 0.01), as well as a marked degeneration of the retinal microvasculature. Retinal microvascular alterations in juvenile hph-1 mice were associated with a decreased expression in Norrin (0.2-fold) and its receptor Frizzled-4 (FZD4; 0.51-fold), as well as with an augmented expression of pro-inflammatory factors such as IL-6 (3.2-fold), NRLP-3 (4.4-fold), IL-1β (8.6-fold), and the anti-angiogenic factor thrombospondin-1 (TSP-1; 17.5-fold). We found that TSP-1 derived from activated microglial cells is a factor responsible of inducing microvascular degeneration, but BH4 supplementation markedly prevented hyperoxia-induced microglial activation in vitro and microvascular injury in an ex-vivo model of microvascular angiogenesis and an in vivo model of oxygen-induced retinopathy (OIR).ConclusionOur findings reveal that BH4 is a key cofactor in regulating the expression of inflammatory and anti-angiogenic factors that play an important function in the maintenance of retinal microvasculature.

Surgical treatment of Persistent Fetal Vasculature (PFV)

PurposePersistent fetal vasculature (PFV), or persistent hyperplastic primary vitreous (PHPV), is a congenital ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature (associated with cataract and glaucoma). Our aim is to overview primary surgical approaches in treatment of PFV with anterior disease based on literature review and two consecutive cases.MethodsReview of published cohort studies and case reports was performed with PubMed Search engine (keywords: PFV, PHPV, lensectomy, vitrectomy, anterior disease). Two consecutive cases of unilateral PFV with anterior disease treated with lensectomy and vitrectomy were analyzed regarding surgery timing and surgical steps.ResultsLiterature review showed limited follow‐up (mean 22–76 months) in cohort studies, as well as variation of timing, surgical approaches, and correction of aphakia. During surgery protection of corneal endothelium, removal of capsular remnants and endodiathermy of the hyaloid artery are critical.ConclusionsTiming of primary surgery in PFV and proper surgical intervention are crucial for the best visual outcome possible. Postoperative period has to include thorough monitoring of intraocular pressure and amblyopia treatment.

Bullous X linked retinoschisis: clinical features and prognosis

Background/AimsA subset of patients with X linked retinoschisis (XLRS) have bullous schisis cavities in the peripheral retina. This study describes the characteristics and prognosis of the bullous form of XLRS.MethodsA...

The analysis of posterior segment findings in term and premature infants using RetCam images.

To analyze posterior segment findings in term and premature infants using the RetCam image database. RetCam images taken of infants born between January 2012 and December 2015 were reviewed. Group 1 included infants with posterior segment findings other than retinopathy of prematurity (ROP). Group 2 included infants with mild-to-severe ROP. The baseline characteristics, anterior segment findings, and percentage of infants who received treatment were compared among the 2 groups. In total, 331 out of 3440 infants (9.6%) were included. The major diagnoses in group 1 (n=75) were retinal hemorrhages in 26, optic nerve pathologies in 14, findings associated with a metabolic disease in 6, ocular tumors in 5, persistent hyperplastic primary vitreous in 4, and familial exudative vitreoretinopathy in 4 cases. The mean birth weight (g) (2481.9±700.5 in group 1 vs 1090.5±330.9 in group 2), gestational age (weeks) (35.9±3.4 in group 1 vs 28.2±2.4 in group 2), and postmenstrual age at initial examination (35.9±3.4 in group 1 vs 28.2±2.4 in group 2) were significantly different among the 2 groups (p<0.001). Thirteen out of 75 cases in group 1 and 124 out of 256 ROP cases received therapy (p<0.001). Anterior segment pathologies were found in 13.3% of group 1 versus 7.8% of group 2 infants (p=0.216). A considerable number of infants suffered from posterior segment disorders other than ROP. The majority of these infants were term babies. Routine fundus screening may be recommended in all newborns to diagnose all posterior segment pathologies other than ROP.

Measurements, reoperation rates, and outcomes in persistent fetal vasculature with elongated ciliary processes

Measurements, reoperation rates, and outcomes in persistent fetal vasculature with elongated ciliary processes

LONG-TERM RESULTS OF TOTAL RETINECTOMY IN CASES WITH ADVANCED PROLIFERATIVE VITREORETINOPATHY.

To evaluate the long-term results of total retinectomy in patients with rhegmatogenous retinal detachment and severe proliferative vitreoretinopathy. Seven eyes of 7 patients had pars plana vitrectomy, with or without pars plana lensectomy, and total retinectomy, with or without silicone oil tamponade, for the management of rhegmatogenous retinal detachment and proliferative vitreoretinopathy (Grade D1-3) after previous complex vitreoretinal surgery procedures. Visual acuity, intraocular pressure, details of previous operations, and development of complications were evaluated retrospectively. Five eyes had a history of penetrating eye trauma, one had persistent fetal vasculature, and one had Coats disease. Patients were all male, with a mean age of 15 ± 11 years (range, 2-31). The mean follow-up was 49.7 ± 50.2 months (range, 12-132). In 3 eyes (42%), enucleation and evisceration were avoided over a mean 100 ± 30.2 month follow-up. All 7 eyes had corneal opacity and band keratopathy after a mean 4.4 ± 3.2 months. Four eyes had phthisis bulbi after a mean 7 ± 4.2 months. No patients developed rubeosis iridis related to the total retinectomy. Total retinectomy may be a last option to preserve the eyeball in cases with massive fibrosis and rhegmatogenous retinal detachment, despite previous complex surgical treatments.

The Paediatric Cataract Register (PECARE): an overview of operated childhood cataract in Sweden and Denmark.

To report basic epidemiological data concerning surgically treated childhood cataract in Sweden and Denmark. Data were derived from the Paediatric Cataract Register (PECARE), a binational, web-based surgical register representing Sweden and Denmark. All children operated before 8years of age between 1 January 2007 and 31 December 2013 were included. Age-specific prevalence per 100000 population was calculated. A total 574 operations in 213 boys (51.7%) and 199 girls (48.3%), altogether 412 children, were registered, the vast majority (n=395/412; 95.9%) being individuals with congenital/infantile cataract. Of these 412, a total of 294 (147 boys and 147 girls) were Swedish and 118 (66 boys and 52 girls) were Danish. The age-specific prevalence of operated cataract in Sweden was 31/100 000 and in Denmark 28/100 000. In 454 of 574 eyes (79.1%), the cataract was dense. Altogether, 266 of 574 (46.3%) were operated during the first year of life, 193 during the first 12weeks representing 33.6% of all operations. A primary intraocular lens (IOL) implantation was done in altogether 411 of 574 eyes (71,6%). In total, 210 unilateral cataract operations (210/574; 36.6%) were performed. Persistent fetal vasculature (PFV) was present in 64 of 193 (33.1%) of those with a congenital unilateral cataract. In 84 individuals (84/395; 21.3%) with congenital or infantile cataract, a coexisting disorder was found. The age-specific binational prevalence of operated congenital/infantile cataract in Sweden and Denmark is 30/100000. About half of the operations are performed within the first year of life, one-third within the first 3months. In our study population, a primary IOL was implanted in the majority of cases.