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1.
Eur J Ophthalmol ; 34(1): NP18-NP21, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37424268

ABSTRACT

PURPOSE: Surgical removal of a vascularized pupillary membrane may be challenging with the risk of intraoperative bleeding and postoperative recurrence. We present a case of a 4-week-old who presented with anterior persistent fetal vasculature (PFV) and dense vascularized pupillary membrane in which the use of intracameral and intravitreal bevacizumab may have contributed to successful treatment. OBSERVATION: A 4-week-old-month-old otherwise healthy girl was referred to Boston Children's Hospital for evaluation of cataract. Ocular examination revealed right microcornea and vascularized pupillary membrane. The left eye exam was unremarkable. Only three weeks after surgical excision of the pupillary membrane and cataract extraction, recurrence of a vascular pupillary membrane was noted. Repeat membranectomy with pupilloplasty and use of intracameral bevacizumab was performed. The pupillary opening was further opened 5 months later, after repeat (intravitreal) bevacizumab, and the pupil has remained open and stable with >6 months' follow-up. CONCLUSION AND IMPORTANCE: This case suggests a role for bevacizumab in the management of PFV, however, a cause-and-effect relationship cannot be proven. Further prospective comparative studies are needed to confirm our findings.


Subject(s)
Cataract Extraction , Cataract , Eye Abnormalities , Persistent Hyperplastic Primary Vitreous , Child , Female , Humans , Infant, Newborn , Bevacizumab , Persistent Hyperplastic Primary Vitreous/surgery , Eye Abnormalities/diagnosis , Cataract/chemically induced , Cataract/diagnosis
2.
Am J Ophthalmol ; 260: 30-36, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37939986

ABSTRACT

PURPOSE: To report 5-year outcomes after surgery for cataract associated with persistent fetal vasculature (PFV). DESIGN: Clinical cohort study using pediatric cataract registry data collected annually from medical records. METHODS: This study included 64 children <13 years of age who were undergoing surgery for unilateral, nontraumatic cataract associated with PFV. Proportions with age-normal visual acuity (VA) and VA better than 20/200 at 5 years' follow-up were estimated. Cumulative incidences of complications and additional surgeries by 5 years were calculated. Outcomes were compared between eyes with unilateral PFV and eyes with unilateral non-PFV cataract from our registry. RESULTS: Forty-eight of 64 eyes were aphakic postoperatively (median age at surgery 2 months [range 1-13 months]) and 16 were pseudophakic (29 months [range 2-92 months]). Overall, 4 of 42 eyes (10% [95% confidence interval {CI} 3%-23%]) achieved age-normal VA. VA better than 20/200 was achieved in 17 (59% [95% CI 39%-76%]) unilateral aphakic PFV eyes and 44 (43% [95% CI 32%-54%]) unilateral non-PFV aphakic eyes (age-adjusted odds ratio = 1.90 [95% CI 0.81-4.50]; P = .14). The most common complication in aphakic PFV eyes was glaucoma-related adverse events (cumulative incidence 24% [95% CI 9%-37%]). There was no significant difference in glaucoma-related adverse events between PFV and non-PFV eyes in aphakic participants ≤1 year of age at lensectomy (age-adjusted hazard ratio = 1.20 [95% CI 0.54-2.64], P = .66). CONCLUSIONS: A wide range of visual outcomes for PFV cataract were observed with a 10% probability of achieving age-normal VA. There was an ongoing risk for the development of glaucoma-related adverse events in PFV eyes.


Subject(s)
Cataract Extraction , Cataract , Glaucoma , Persistent Hyperplastic Primary Vitreous , Child , Humans , Infant , Child, Preschool , Cataract Extraction/adverse effects , Cohort Studies , Follow-Up Studies , Visual Acuity , Cataract/complications , Persistent Hyperplastic Primary Vitreous/complications , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Glaucoma/surgery , Glaucoma/complications , Retrospective Studies , Postoperative Complications/surgery
3.
Graefes Arch Clin Exp Ophthalmol ; 261(10): 2795-2804, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37219614

ABSTRACT

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.


Subject(s)
Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Humans , Infant , Retrospective Studies , Vitrectomy , Visual Acuity , Retina , Persistent Hyperplastic Primary Vitreous/complications , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Prognosis , Retinal Detachment/surgery
4.
Ophthalmology ; 130(8): 844-853, 2023 08.
Article in English | MEDLINE | ID: mdl-37044159

ABSTRACT

PURPOSE: To describe clinical features, management, and outcomes of posterior persistent fetal vasculature (PFV) and suggest a management algorithm. DESIGN: Retrospective, consecutive case series. PARTICIPANTS: All children diagnosed with posterior PFV and treated or followed at the Rothschild Foundation Hospital in France between June 2011 and September 2021. METHODS: Retrospective analysis of the clinical characteristics of posterior PFV. We reported age, gender, presenting symptoms, intraocular pressure, and visual acuity (VA) at diagnosis. Patients were divided into 4 groups depending on the severity and involvement or not of anterior segment. We reported the vitreoretinal surgical techniques used. MAIN OUTCOME MEASURES: Anatomic results, ocular hypertension, best-corrected distance visual acuity (BCDVA), presence of postoperative adverse events, and additional surgical interventions were recorded at each follow-up visit. RESULTS: Ninety-six patients were included. The median age at diagnosis was 8 months (IQR = 12), 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. There was anterior involvement in 62 (64%) of eyes. Forty-one eyes (42.7%) were microphthalmic and more frequently associated with severe PFV (53% vs. 25%; P = 0.01). Surgery was performed in 85 patients (89%). Of them, 69 (81%) had a total success, 5 (6%) had a partial success due to persistent limited peripheral retinal detachment (RD), and 11 (13%) had a failure due to persistent total RD after surgery. Postoperative adverse events occured in 38 eyes including ocular hypertension requiring eye drop medication (7.1%), secondary cell proliferation around the intraocular lens (8.2%), intravitreal hemorrhages (7.1%), and persistent tractional RD (10.6%). Second surgery was performed in 18 patients (21%). At last follow-up, VA could be measured in logarithm of the minimum angle of resolution in 43 children (45%), light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%). CONCLUSIONS: In our case series, most patients presenting with posterior PFV received complex vitreoretinal surgery. Goals of the surgery vary and include retinal flattening, reduction of vitreoretinal traction, freeing of visual axis, and aesthetic concerns. We propose a surgical and medical management algorithm for PFV. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.


Subject(s)
Glaucoma , Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Child , Humans , Infant , Retrospective Studies , Vitrectomy , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Persistent Hyperplastic Primary Vitreous/complications , Treatment Outcome , Retinal Detachment/surgery , Glaucoma/surgery , Postoperative Complications/surgery
5.
Int J Mol Sci ; 24(6)2023 Mar 19.
Article in English | MEDLINE | ID: mdl-36982910

ABSTRACT

This study aims to investigate the outcomes and risk factors associated with poor vision (vision less than counting fingers, 2.0 logMAR, Snellen vision 20/2000) in patients with posterior or combined persistent fetal vasculature (PFV), with or without surgery. We retrospectively reviewed the medical records of patients who were diagnosed with PFV from January 2008 to April 2021. We included 51 eyes of 44 patients who presented with PFV, of which 38 eyes underwent surgical correction (pars plicata/plana vitrectomy, with or without lensectomy, and intraocular lens implantation) at the median age of 6.0 months (range: 0.7 to 82.0). The mean follow-up was 68.8 months ± 38.0 months. The axial length change in the eyes undergoing surgery was significantly higher than the eyes without surgery (p = 0.025). Initial anterior chamber collapse and retinal detachment were associated with poor vision (p = 0.006 and p = 0.002, respectively). In addition, 37% of eyes with posterior or combined PFV had vision better than counting fingers. Surgery for eyes with PFV could result in better eye growth. Visual outcomes remained poor and were associated with the level of macular abnormality. Initial anterior chamber collapse and retinal detachment at presentation were the risk factors for poor visual outcomes. Vitrectomy for selected PFV eyes is valuable and associated with a better cosmetic outcome (better eye growth).


Subject(s)
Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Humans , Infant, Newborn , Infant , Child, Preschool , Child , Retrospective Studies , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Vitrectomy , Treatment Outcome , Postoperative Complications
6.
Am J Med Genet A ; 191(5): 1373-1377, 2023 05.
Article in English | MEDLINE | ID: mdl-36734406

ABSTRACT

Persistent fetal vasculature (PFV) is a rare malformative ocular disorder resulting from the failure of the hyaloid vasculature to regress. The severity of the visual impairment is depending on the underlying eye defects, ranging from discreet hyaloid remnants to severe ocular anomalies. Although PFV is generally unilateral, sporadic and idiopathic, a genetic cause has been described in some individuals, especially those presenting with a bilateral and/or syndromic form of PFV. The genes occasionally described in PFV are most often responsible for a wide spectrum of ocular phenotypes such as ATOH7 or NDP, a gene also known to be involved in Norrie disease, a X-linked vitreoretinopathy with extra-ocular features. We describe here a patient with an ocular phenotype consisting in non-syndromic bilateral PFV with cataract and microphthalmia, in whom a recurrent heterozygous de novo MIP disease-causing variant was detected after using a dedicated 119-ocular genes panel approach. Defects in the MIP gene are classically associated with dominant non-syndromic congenital cataract without other ocular malformative features. Thus, this case highlights the value of exploring individuals with PFV, even those with non-syndromic forms. It also broadens the phenotypic spectrum of the MIP gene, adding new insights into the gene networks underlying PFV pathophysiology, that remains unclear.


Subject(s)
Cataract Extraction , Cataract , Microphthalmos , Persistent Hyperplastic Primary Vitreous , Humans , Cataract/diagnosis , Cataract/genetics , Cataract/congenital , Eye , Microphthalmos/diagnosis , Microphthalmos/genetics , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/genetics , Persistent Hyperplastic Primary Vitreous/surgery
7.
Retina ; 43(3): 523-528, 2023 03 01.
Article in English | MEDLINE | ID: mdl-32516225

ABSTRACT

PURPOSE: To report viscodelamination of localized retrolental plaques during lens-sparing vitrectomy in eyes with pediatric tractional vitreoretinopathy. METHODS: Viscodelamination of retrolental plaques was performed during 23-gauge lens-sparing vitrectomy in 11 eyes from 11 children with a median age (range) of 12 (4-58) months. There were five eyes with Stage 4 familial exudative vitreoretinopathy, one eye with Stage 4B retinopathy of prematurity, and five eyes with anterior-posterior persistent fetal vasculature syndrome. Retrolental plaques were separated from the posterior lens capsule by the tension of viscoelastic injection in the Berger's space. RESULTS: In 10 of 11 eyes, retrolental plaques were removed from posterior surface of lens without capsular tearing. During the 12-month to 18-month follow-up period, lens clarity along the visual axis was retained in 5 eyes with familial exudative vitreoretinopathy, one eye with retinopathy of prematurity, and 2 of 5 eyes with persistent fetal vasculature. Atraumatic viscodelamination was associated with retrolental plaques that did not incorporate into the posterior lens capsule. Retinal dragging was reversed in all 11 eyes. CONCLUSION: Viscodelamination may be effective for the dissection of retrolental plaques related to pediatric vitreoretinopathy in selected cases.


Subject(s)
Connective Tissue Diseases , Orbital Diseases , Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Retinopathy of Prematurity , Infant, Newborn , Humans , Child , Infant , Vitrectomy , Familial Exudative Vitreoretinopathies/complications , Retinopathy of Prematurity/diagnosis , Retinopathy of Prematurity/surgery , Retinopathy of Prematurity/complications , Visual Acuity , Retrospective Studies , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Connective Tissue Diseases/complications , Retinal Detachment/surgery , Follow-Up Studies , Treatment Outcome
8.
Eye (Lond) ; 37(7): 1371-1376, 2023 05.
Article in English | MEDLINE | ID: mdl-35739244

ABSTRACT

BACKGROUND/OBJECTIVES: 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. SUBJECTS/METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Subject(s)
Iris Diseases , Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Retinal Diseases , Humans , Persistent Hyperplastic Primary Vitreous/complications , Persistent Hyperplastic Primary Vitreous/surgery , Retinal Detachment/surgery , Retrospective Studies , Treatment Outcome , Vitrectomy
9.
J AAPOS ; 26(6): 296.e1-296.e5, 2022 12.
Article in English | MEDLINE | ID: mdl-36265751

ABSTRACT

BACKGROUND: Persistent fetal vasculature (PFV) is a congenital anomaly caused by failure of the ocular vasculature to regress. We report the visual and anatomic outcomes in patients who underwent surgery for PFV between 7-48 months of age. METHODS: The medical records of patients who underwent lensectomy and anterior or core vitrectomy for unilateral PFV, with or without intraocular lens implantation, were reviewed retrospectively. Inclusion criteria were surgery between 7-48 months of age and at least 12 months of follow-up. Patients with severe posterior segment involvement were excluded. The primary outcome was the final visual acuity using age-appropriate tests converted to logMAR. Secondary outcomes included the rate of adverse events and number of subsequent intraocular procedures. RESULTS: A total of 20 patients with a mean age at surgery of 19.3 ± 10.5 months and mean follow-up of 73.7 ± 46.7 months were included. Sixteen patients had delayed surgery due to late presentation; the remaining 4 were managed initially with refractive correction and occlusion. Eight patients (40%) achieved a final visual acuity better than 1.0 logMAR. Four eyes had adverse events, including one retinal detachment. No patient developed glaucoma. Four eyes underwent subsequent procedures. CONCLUSIONS: In our study cohort, surgery for unilateral PFV between 7-48 months of age achieved functional visual acuity in over one-third of patients. This is comparable to results achieved with surgery before 7 months of age, but with fewer adverse events. Differences in disease severity could account for later PFV presentation and surgery as well as outcomes.


Subject(s)
Persistent Hyperplastic Primary Vitreous , Postoperative Complications , Humans , Infant , Retrospective Studies , Postoperative Complications/surgery , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Vitrectomy , Visual Acuity , Follow-Up Studies
10.
Turk J Ophthalmol ; 51(6): 407-411, 2021 Dec 28.
Article in English | MEDLINE | ID: mdl-34963272

ABSTRACT

Persistent fetal vasculature (PFV) syndrome is characterized by abnormal regression of the fetal hyaloid system and may occur in various forms. In this report, two atypical cases associated with posterior capsular defect and ectopic lens material located along Cloquet's canal are discussed. Ultrasonography of these patients presenting with bilateral total cataracts revealed a hyaloidal stalk extending from the optic nerve head to the retrolental area. During lensectomy, it was observed that lens particles were moving anteriorly from the central mid-vitreous to the aspiration port and that the posterior capsule was developmentally defective. There was no pathological vascular remnant, rather the lens material partially filled Cloquet's canal through the opening in the posterior capsule and created a pseudo-stalk appearance on the preoperative ultrasonography. We aim to discuss possible mechanisms underlying these cases, which may help to improve our understanding of the PFV spectrum.


Subject(s)
Cataract Extraction , Cataract , Persistent Hyperplastic Primary Vitreous , Cataract/diagnosis , Humans , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Vitreous Body
12.
BMJ Case Rep ; 14(2)2021 Feb 05.
Article in English | MEDLINE | ID: mdl-33547123

ABSTRACT

A 6-year-old systemically healthy child presented with visual acuity of 1/60, N18 oculusdextrus (OD), and 6/18, N6 oculus sinister (OS). Slit-lamp biomicroscopy revealed suspicious bilateral inferotemporal pigmented ciliary body (CB) tumour, protruding posterior capsule and temporal posterior subcapsular cataract oculus uterque. Anterior segment optical coherence tomography, ultrasonography, ultrasonic biomicroscopy and Scheimpflug imaging revealed protruding posterior capsule and cortex abutting but not arising from CB suggestive of peripheral pigmented posterior lenticonus with hypermetropia (axial length 20.27 mm OD and 19.97 mm OS). Aberrometry revealed high internal aberrations and low Dysfunctional Lens Index (DLI). Lens aspiration with intraocular lens implantation in the bag OD and contact lens correction OS were undertaken. The child had a postoperative visual gain of 3/60, N18 with improved aberrometric profile OD, and was advised amblyopia therapy. Rarely posterior lenticonus can mimic a CB mass. Multi-modal ocular imaging can aid in its diagnosis and management. DLI may serve as a useful indicator of surgery in such cases.


Subject(s)
Corneal Wavefront Aberration/diagnostic imaging , Lens, Crystalline/abnormalities , Persistent Hyperplastic Primary Vitreous/diagnostic imaging , Persistent Hyperplastic Primary Vitreous/surgery , Child , Corneal Wavefront Aberration/surgery , Diagnosis, Differential , Humans , Visual Acuity
14.
J Cataract Refract Surg ; 46(4): 497-502, 2020 04.
Article in English | MEDLINE | ID: mdl-32271292

ABSTRACT

Anterior and posterior capsulotomies were performed in 12 eyes of 12 patients (age range 3 months to 6 years) with congenital cataracts and primary persistent hyperplastic primary vitreous (PHPV) syndrome using a femtosecond laser. The procedure was performed in 8 eyes with PHPV severity level 1 and 4 eyes with severity level 2 (Sudovsky classification). Surgeries were performed at the Kaluga branch of the S. Fyodorov Eye Microsurgery Federal State Institution, Russia. Postoperative follow-up was between 8 months and 36 months. The use of a low-energy femtosecond laser-assisted posterior capsulotomy in this pediatric population provided safe and predictable results with a reduced number of intraocular manipulations, and reduced the risk for complications.


Subject(s)
Anterior Capsule of the Lens/surgery , Lens Implantation, Intraocular , Persistent Hyperplastic Primary Vitreous/complications , Phacoemulsification , Posterior Capsulotomy/methods , Cataract/congenital , Child , Child, Preschool , Electroretinography , Female , Humans , Infant , Intraocular Pressure/physiology , Male , Microscopy, Acoustic , Operative Time , Ophthalmoscopy , Persistent Hyperplastic Primary Vitreous/surgery , Pseudophakia/physiopathology , Visual Acuity/physiology
15.
Eur J Ophthalmol ; 30(2): 284-288, 2020 Mar.
Article in English | MEDLINE | ID: mdl-30712383

ABSTRACT

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.


Subject(s)
Persistent Hyperplastic Primary Vitreous/physiopathology , Visual Acuity/physiology , Adolescent , Cataract Extraction , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Middle Aged , Persistent Hyperplastic Primary Vitreous/surgery , Prognosis , Retrospective Studies , Vitrectomy , Young Adult
17.
J AAPOS ; 23(6): 327.e1-327.e5, 2019 12.
Article in English | MEDLINE | ID: mdl-31629823

ABSTRACT

BACKGROUND: Persistent fetal vasculature (PFV) is a spectrum of congenital anomalies caused by complete or partial failure of the ocular fetal vasculature to regress. We report the visual and anatomic outcomes in a large cohort of patients who underwent early surgery for PFV. METHODS: We retrospectively reviewed the medical records of patients who underwent lensectomy and anterior or core vitrectomy for unilateral PFV without primary intraocular lens implantation through limbal or pars plana/plicata approach. Inclusion criteria were surgery prior to 7 months of age, with at least 12 months of follow-up. Eyes with severe posterior segment involvement and retinal detachment deemed beyond repair were excluded. RESULTS: A total of 58 patients met inclusion criteria. Mean age at surgery was 2.1 ± 1.5 months. Mean follow-up was 6.7 ± 4.2 years. At final follow-up, 19 eyes (33%) had visual acuity better than 1.0 logMAR. Thirty-three eyes (57%) developed 1 or more postoperative adverse events: glaucoma in 21 (36%) and retinal detachment in 11 (19%), 8 of which occurred in eyes that had pars plana or pars plicata incisions (P = 0.002). In patients with limbal incisions, 17 of 40 (43%) achieved a visual acuity better than 1.0 logMAR, compared with 2 of 18 patients (11%) with a pars plana/pars plicata incision (P = 0.03). CONCLUSIONS: In our study cohort, early surgery for PFV achieved functional visual acuity in about one-third of patients. Limbal approach to surgery may result in better visual acuity and anatomic results.


Subject(s)
Eye Abnormalities/surgery , Forecasting , Persistent Hyperplastic Primary Vitreous/complications , Visual Acuity/physiology , Vitrectomy/methods , Vitreous Body/abnormalities , Child , Child, Preschool , Eye Abnormalities/etiology , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Persistent Hyperplastic Primary Vitreous/surgery , Postoperative Period , Retrospective Studies , Treatment Outcome , Vitreous Body/blood supply
18.
BMC Ophthalmol ; 19(1): 150, 2019 Jul 16.
Article in English | MEDLINE | ID: mdl-31311513

ABSTRACT

BACKGROUND: Morning glory syndrome is a relatively rare congenital optic disc anomaly that is often difficult to identify when associated with additional congenital ocular anomalies. This case report describes the diagnosis, treatment, and postoperative follow-up care of a young girl with morning glory syndrome accompanied by persistent fetal vasculature, retinal fold, and retinal detachment. Here, we also give a brief review of the relevant literature. CASE PRESENTATION: A 5-year-old girl was referred to our clinic for a complaint of decreased vision for 6 months in the right eye. The best corrected visual acuity was hand motion in her right eye and 0.8 in her left eye. A fundus examination indicated vitreous opacities and scattered hemorrhages, as well as striped folds in the temporal retina of the affected eye. B-ultrasound and magnetic resonance imaging scans suggested that it could be a congenital dysplasia of the right eye. Pars plana vitrectomy was performed in the right eye. Morning glory syndrome associated with persistent fetal vasculature was confirmed in subsequent follow-up observation according to the fundus appearance, optical coherence tomography, and fundus fluorescein angiography imaging. CONCLUSIONS: The patient was diagnosed as morning glory syndrome associated with persistent fetal vasculature and retinal fold. The morning glory disc with the presence of retinal folds did not seem quite typical and that made the diagnosis difficult. This report stresses the importance of considering concurrent morning glory syndrome and persistent fetal vasculature. Vitrectomy may be beneficial in the management of the morning glory syndrome and persistent fetal vasculature if accompanied by retinal detachment in similar cases.


Subject(s)
Optic Disk/abnormalities , Optic Nerve/abnormalities , Persistent Hyperplastic Primary Vitreous , Retinal Detachment , Child, Preschool , Female , Humans , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Retinal Detachment/diagnosis , Retinal Detachment/surgery , Treatment Outcome , Vitrectomy/methods
19.
Ophthalmic Surg Lasers Imaging Retina ; 50(6): 377-384, 2019 06 01.
Article in English | MEDLINE | ID: mdl-31233155

ABSTRACT

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.].


Subject(s)
Persistent Hyperplastic Primary Vitreous/surgery , Visual Acuity/physiology , Vitrectomy/statistics & numerical data , Asian People , Child, Preschool , Female , Humans , Infant , Male , Persistent Hyperplastic Primary Vitreous/physiopathology , Postoperative Complications , Retrospective Studies , Vitrectomy/methods
20.
Eye (Lond) ; 33(6): 938-947, 2019 06.
Article in English | MEDLINE | ID: mdl-30728490

ABSTRACT

PURPOSE: To identify the characteristics of peripheral retinal anomalies associated with persistent fetal vasculature (PFV) and evaluate the complications and outcomes. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Subject(s)
Persistent Hyperplastic Primary Vitreous/complications , Retina/diagnostic imaging , Retinal Diseases/etiology , Retinal Vessels/diagnostic imaging , Visual Acuity , Vitrectomy/methods , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Persistent Hyperplastic Primary Vitreous/diagnosis , Persistent Hyperplastic Primary Vitreous/surgery , Retinal Diseases/diagnosis , Retinal Diseases/surgery , Retrospective Studies , Time Factors , Ultrasonography
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