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1.
Ophthalmology ; 130(8): 844-853, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37044159

RESUMEN

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.


Asunto(s)
Glaucoma , Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Niño , Humanos , Lactante , Estudios Retrospectivos , Vitrectomía , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/cirugía , Vítreo Primario Hiperplásico Persistente/complicaciones , Resultado del Tratamiento , Desprendimiento de Retina/cirugía , Glaucoma/cirugía , Complicaciones Posoperatorias/cirugía
2.
Am J Med Genet A ; 191(5): 1373-1377, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-36734406

RESUMEN

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.


Asunto(s)
Extracción de Catarata , Catarata , Microftalmía , Vítreo Primario Hiperplásico Persistente , Humanos , Catarata/diagnóstico , Catarata/genética , Catarata/congénito , Ojo , Microftalmía/diagnóstico , Microftalmía/genética , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/genética , Vítreo Primario Hiperplásico Persistente/cirugía
3.
Graefes Arch Clin Exp Ophthalmol ; 261(10): 2795-2804, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37219614

RESUMEN

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.


Asunto(s)
Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Humanos , Lactante , Estudios Retrospectivos , Vitrectomía , Agudeza Visual , Retina , Vítreo Primario Hiperplásico Persistente/complicaciones , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/cirugía , Pronóstico , Desprendimiento de Retina/cirugía
4.
Retina ; 43(3): 523-528, 2023 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-32516225

RESUMEN

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.


Asunto(s)
Enfermedades del Tejido Conjuntivo , Enfermedades Orbitales , Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Retinopatía de la Prematuridad , Recién Nacido , Humanos , Niño , Lactante , Vitrectomía , Vitreorretinopatías Exudativas Familiares/complicaciones , Retinopatía de la Prematuridad/diagnóstico , Retinopatía de la Prematuridad/cirugía , Retinopatía de la Prematuridad/complicaciones , Agudeza Visual , Estudios Retrospectivos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/cirugía , Enfermedades del Tejido Conjuntivo/complicaciones , Desprendimiento de Retina/cirugía , Estudios de Seguimiento , Resultado del Tratamiento
5.
BMC Ophthalmol ; 23(1): 267, 2023 Jun 13.
Artículo en Inglés | MEDLINE | ID: mdl-37312173

RESUMEN

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature (PFV), is a clinical entity that traditionally presents with leukocoria, microphthalmia, retinal dysplasia, or eyeball shrinkage which is associated with poor vision. However, there is a dearth of literature on cases of PHPV in adulthood or with asymptomatic occurrence. This report presents the clinical and pathological findings of a non-typical PHPV case and discuss the current knowledge for this condition. CASE PRESENTATION: A 68-year-old healthy male was referred to our outpatient department for evaluation of age-related cataract without other visual symptoms. Preoperative fundus examination occasionally detected an isolated stalk-like band extending to the posterior pole of the eye with normal central vitreous and retina. Other ocular examinations including b-mode ultrasonography, optical coherence tomography did not unveil any abnormalities, which caused diagnostic uncertainty. We referred to cataract surgery along with histopathological study, that revealed characteristics of PHPV including fibrous connective tissues mainly composed of fibrocyte proliferation and a very few capillary vessels. Thereafter, a definitive diagnosis of non-typical PHPV was established. CONCLUSION: Our case is unique due to it was not discovered until adulthood, presence with only age-related cataract, and accompanied with normal central vitreous and retina. Histopathological explorations lead to an accurate diagnosis of the condition. Those results broaden the phenotype spectrums of PHPV and further provide clinical clues for the cognition of the disease.


Asunto(s)
Extracción de Catarata , Catarata , Vítreo Primario Hiperplásico Persistente , Humanos , Masculino , Catarata/diagnóstico , Fondo de Ojo , Vítreo Primario Hiperplásico Persistente/diagnóstico , Retina , Anciano
6.
Int J Mol Sci ; 24(6)2023 Mar 19.
Artículo en Inglés | MEDLINE | ID: mdl-36982910

RESUMEN

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).


Asunto(s)
Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Humanos , Recién Nacido , Lactante , Preescolar , Niño , Estudios Retrospectivos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/cirugía , Vitrectomía , Resultado del Tratamiento , Complicaciones Posoperatorias
7.
BMC Ophthalmol ; 22(1): 308, 2022 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-35842607

RESUMEN

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) in a patient with tuberous sclerosis (TS) has been described in one previous case report in 1999. Otherwise, there is no literature around this potential association. We describe a case of an infant with TS and PHPV. CASE PRESENTATION: An 11-month old male was under investigation for leukocoria, microphthalmia and suspected PHPV after being seen in ophthalmology clinic. He presented to hospital with seizures and was diagnosed with TS on imaging. Imaging also showed the known microphthalmia and a mass associated with the lens. Subsequent paediatric ophthalmology review and examination under anaesthesia confirmed microphthalmia, PHPV and a retrolental mass which was thought to represent total retinal detachment or a retinal hamartoma within a retinal detachment. CONCLUSIONS: This is the second case report of PHPV in a patient with TS. The previous case report postulated that the atypical location of the retinal hamartoma was secondary to the abnormal globe development in PHPV.


Asunto(s)
Cristalino , Microftalmía , Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Esclerosis Tuberosa , Niño , Humanos , Lactante , Masculino , Vítreo Primario Hiperplásico Persistente/complicaciones , Vítreo Primario Hiperplásico Persistente/diagnóstico , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/diagnóstico
8.
Retina ; 40(3): 572-580, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30531421

RESUMEN

OBJECTIVE: This study aims to examine retinal vascular findings for affected eyes and contralateral eyes as well in typical cases of unilateral persistent fetal vasculature. METHODS: We retrospectively reviewed all patients evaluated at Chang Gung Memorial Hospital, Linkou, for unilateral persistent fetal vasculature between January 2008 and July 2017. All patients underwent fluorescein angiography (FA) examination under general anesthesia. FA was performed using RetCam 3 (Clarity Medical Systems, Inc, Pleasanton, CA). RESULTS: Ten patients (eight male and two female) were identified as having adequate clinical data for the final analysis. The mean age at diagnosis was 13.7 ± 17.2 months (range 1-58). The mean axial length was shorter in the affected eyes as compared to the fellow eyes (17.27 ± 2.8 vs. 20.2 ± 1.7 mm; P = 0.024). In the affected eyes, nine cases (90.0%) showed a concomitant retrolental stalk, avascular peripheral retina, regional capillary dropout, and absence of foveal avascular zone. Hyperfluorescent stalk was seen in seven cases (70.0%). Four eyes (40.0%) showed leaking vessels. Terminal supernumerary branching was seen in two cases (20.0%). Popcorn hyperfluorescence was noted in one case (10.0%). In the fellow eyes, peripheral avascular zone was noted in nine eyes (90.0%), of which six (60.0%) had peripheral zones greater than two-disk diameters. Seven eyes (70.0%) presented with regional capillary dropout and abnormal choroidal filling. Three eyes (30.0%) had abnormal vessel straightening. Aberrant circumferential vessels and leaking spots were seen in two eyes (20.0%). Regional dilation of disk vessels, peripheral vessel dilation, and terminal bulbing were noted in one eye (10.0%). The mean best-corrected visual acuity of the fellow eyes was 20/39 (0.29 in logarithm of the minimum angle of resolution). CONCLUSION: Retinal vascular abnormalities in the affected eyes and fundoscopically normal fellow eyes of unilateral persistent fetal vasculature patients were found in 100% and 90.0% of patients, respectively. Fellow eyes had some subtle abnormalities that were only revealed through FA. These unilateral persistent fetal vasculature cases were still bilaterally affected.


Asunto(s)
Coroides/irrigación sanguínea , Angiografía con Fluoresceína/métodos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vasos Retinianos/patología , Preescolar , Femenino , Fondo de Ojo , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodos
9.
BMC Ophthalmol ; 20(1): 290, 2020 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-32677902

RESUMEN

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is a congenital form of vitreous dysplasia that can be categorized into anterior, posterior, and mixed types according to the affected location within the eye. Definitive diagnoses of PHPV are usually made based on B-mode ultrasound, optical coherence tomography (OCT), and Doppler ultrasound findings. In this report, we discuss the case of a 7-year-old boy in whom a definitive diagnosis of atypical anterior PHPV was possible based on intraoperative observations, pathological findings, and the results of ophthalmic examination. CASE PRESENTATION: A 7-year-old boy presented with leukocoria and acute glaucoma in his right eye. Imaging suggested characteristics of mixed PHPV. Surgical treatment and pathological examination were performed due to the presence of acute glaucoma and abnormal lens morphology. Typical signs of posterior PHPV (e.g., eyeball shrinkage, the presence of vascular membranes connected to the optic disc, etc.) were not observed. However, there were abundant fibrous vascular membranes around the lens. Pathological examination revealed fibrocyte proliferation in the lens and capsular tissue. Intraoperative findings were used in conjunction with the results of pathological and ophthalmological examinations to make the final diagnosis of anterior PHPV. CONCLUSION: The course and characteristics of PHPV can be unpredictable, and it is often the case that a clear diagnosis cannot be obtained based on clinical characteristics and typical imaging examinations alone. Further surgical treatment and pathological examination may aid in establishing a final diagnosis. In addition to treating the complications of PHPV (e.g., glaucoma), surgery may improve eye appearance and restore visual function to some degree.


Asunto(s)
Glaucoma , Cristalino , Disco Óptico , Vítreo Primario Hiperplásico Persistente , Niño , Humanos , Masculino , Vítreo Primario Hiperplásico Persistente/diagnóstico , Cuerpo Vítreo
10.
Exp Eye Res ; 181: 271-276, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30817926

RESUMEN

To identify imaging characteristics of mouse persistent hyperplastic primary vitreous (PHPV) by Spectralis Optical Coherence Tomography (OCT), as well as to assess and compare the sensitivity and precision of OCT with color photography (CP) and Fundus Fluorescein Angiography (FFA) imaging in detecting mouse PHPV. Notch4-/- C57BL/6J mice (224 eyes) aged from 3 months to 7 months were examined in this study. CP, FFA and OCT imaging were utilized to examine vitreous cavity and retina of mouse eyes. Horizontal and radial OCT scan volume was centered on the optic nerve head. Hematoxylin and eosin (H&E) staining was performed to validate PHPV. For color photography and FFA imaging, retrolental irregular fibrovascular membrane-like tissues were found in 33 eyes with/without blood vessels in vitreous cavity. Among them, 31 eyes were visualized with lateral and oblique linear hyperreflective opacities in vitreous cavity using Spectralis OCT. Position of PHPV in posterior segment of eyes was also measured via OCT. Mouse PHPV was validated by H&E staining. Typical hyperreflective opacities in vitreous cavity were detected in PHPV mouse using Spectralis OCT. Spectralis OCT imaging can effectively detect mouse PHPV as color photography and FFA.


Asunto(s)
Vítreo Primario Hiperplásico Persistente/diagnóstico , Tomografía de Coherencia Óptica/métodos , Cuerpo Vítreo/patología , Animales , Modelos Animales de Enfermedad , Progresión de la Enfermedad , Estudios de Factibilidad , Angiografía con Fluoresceína/métodos , Fondo de Ojo , Ratones , Ratones Endogámicos C57BL , Disco Óptico/patología , Índice de Severidad de la Enfermedad
11.
BMC Ophthalmol ; 19(1): 141, 2019 Jul 04.
Artículo en Inglés | MEDLINE | ID: mdl-31272412

RESUMEN

BACKGROUND: A persistent hyaloid artery is a rare fetal remnant. Several complications such as amblyopia, vitreous hemorrhage, and retinal detachment have been reported. Here, we present a case of vitreous hemorrhage with a persistent hyaloid artery. CASE PRESENTATION: A healthy 16-year-old male presented with blurred vision in his left eye. Vitreous hemorrhage occurred and absorbed spontaneously. Slit-lamp examination demonstrated a Mittendorf's dot and fundus examination revealed a persistent hyaloid artery. Optical coherence tomography (OCT) showed a Bergmeister's papilla. The blood flow of the persistent hyaloid artery via the Bergmeister's papilla was found by OCT angiography. CONCLUSION: The persistent hyaloid artery should be considered as a cause of spontaneous vitreous hemorrhage of young healthy patient. The OCT angiography will be a useful noninvasive approach to confirm the patency of the persistent hyaloid artery.


Asunto(s)
Angiografía con Fluoresceína/métodos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vasos Retinianos/diagnóstico por imagen , Tomografía de Coherencia Óptica/métodos , Agudeza Visual , Adolescente , Fondo de Ojo , Humanos , Masculino , Vítreo Primario Hiperplásico Persistente/complicaciones , Hemorragia Vítrea/diagnóstico , Hemorragia Vítrea/etiología
12.
BMC Ophthalmol ; 19(1): 150, 2019 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-31311513

RESUMEN

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.


Asunto(s)
Disco Óptico/anomalías , Nervio Óptico/anomalías , Vítreo Primario Hiperplásico Persistente , Desprendimiento de Retina , Preescolar , Femenino , Humanos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/cirugía , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/cirugía , Resultado del Tratamiento , Vitrectomía/métodos
13.
BMC Ophthalmol ; 19(1): 161, 2019 Jul 26.
Artículo en Inglés | MEDLINE | ID: mdl-31349817

RESUMEN

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.


Asunto(s)
Tratamiento Conservador/métodos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Recuperación de la Función , Agudeza Visual/fisiología , Cuerpo Vítreo/anomalías , Preescolar , Progresión de la Enfermedad , Anteojos , Femenino , Estudios de Seguimiento , Humanos , Vítreo Primario Hiperplásico Persistente/fisiopatología , Vítreo Primario Hiperplásico Persistente/terapia , Privación Sensorial , Cuerpo Vítreo/diagnóstico por imagen
14.
Int Ophthalmol ; 39(3): 579-587, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29476279

RESUMEN

PURPOSE: Persistent fetal vasculature (PFV) is a unique ocular disorder usually presenting early in life. The unregressed embryonal hyaloid vasculature poses a risk of severe ocular complications leading to decreased visual acuity. Surgery is the mainstay of therapy in complicated cases. We describe the clinical presentation and surgical treatment of PFV managed at our center from 2012 to 2015. METHODS: The study is a case series comprised eight patients who were diagnosed with complicated severe PFV. All were managed with a tailored surgical approach. The clinical characteristics, medical and surgical treatment, and follow-up findings of each case are described. RESULTS: There were six males and two females. Surgical intervention involved anterior or posterior vitrectomy, lens extraction, and intraocular lens implantation. Hyaloid stalk removal with release of ciliary traction was variably utilized in selected cases. Endodiathermy controlled intraocular bleeding, and intraocular scissors proved helpful in anterior PFV for disinserting the ciliary process from an abnormally thickened posterior lens capsule. Visual outcomes differed in each case, depending on multiple clinical factors. CONCLUSION: Severe complex PFV presents a therapeutic challenge. A tailored surgical approach with meticulous postoperative management is essential for visual rehabilitation.


Asunto(s)
Vítreo Primario Hiperplásico Persistente/cirugía , Agudeza Visual , Vitrectomía/métodos , Cuerpo Vítreo/irrigación sanguínea , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Vítreo Primario Hiperplásico Persistente/diagnóstico , Resultado del Tratamiento , Cuerpo Vítreo/anomalías , Cuerpo Vítreo/cirugía
15.
Graefes Arch Clin Exp Ophthalmol ; 256(3): 495-501, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29299742

RESUMEN

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.


Asunto(s)
Vítreo Primario Hiperplásico Persistente/cirugía , Agudeza Visual , Vitrectomía , Cuerpo Vítreo/diagnóstico por imagen , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/fisiopatología , Periodo Posoperatorio , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Cuerpo Vítreo/fisiopatología
16.
BMC Ophthalmol ; 18(1): 216, 2018 Aug 29.
Artículo en Inglés | MEDLINE | ID: mdl-30157795

RESUMEN

BACKGROUND: Falciform retinal detachment (FRD) usually causes pronounced retinal wrinkles, and the prognosis of visual function is poor. In this present study, we report a rare case of FRD in which optical coherence tomography (OCT) findings revealed a relatively good visual function. CASE PRESENTATION: This study involved a 22-year-old female who had previously been diagnosed with FRD at 2 years of age, and who presented with microphthalmus in both eyes with pronounced retinal folds from the optic disc to the inferior-temporal side. Based on the clinical findings, we diagnosed it as persistent fetal vasculature (PFV). We found the visual function in her left eye to be relatively poor, yet from 6 to 22 years of age, the corrected visual acuity in that eye remained at 0.08. Although a nystagmus was present, Goldman perimetry showed a relatively wider visual field than expected. Optical coherence tomography (OCT) findings revealed that the retinal layer structure near the FRD was relatively well maintained, except for the temporal peripheral region. CONCLUSIONS: Our findings reveal that OCT examination can be considered useful for predicting the visual function in cases of FRD.


Asunto(s)
Vítreo Primario Hiperplásico Persistente/complicaciones , Retina/diagnóstico por imagen , Desprendimiento de Retina/diagnóstico , Tomografía de Coherencia Óptica/métodos , Femenino , Humanos , Vítreo Primario Hiperplásico Persistente/diagnóstico , Desprendimiento de Retina/etiología , Adulto Joven
18.
Zhonghua Yan Ke Za Zhi ; 52(5): 362-5, 2016 May.
Artículo en Zh | MEDLINE | ID: mdl-27220710

RESUMEN

OBJECTIVE: To observe the manifestations of RetCam Ⅱ and color Doppler imaging (CDI) in a retrospective case series of persistent hyperplastic primary vitreous (PHPV). METHODS: Retrospective study. The medical records of 9 eyes/9 patients with PHPV went through RetCamⅡ and CDI from 2009 to 2014. RESULTS: There were 6 young boys and 3 young girl in this study, age from 2 months to 5 years. All the patients were born at full term. 9 eyes had complication (cataract). The manifestations of RetCam Ⅱ: There were pale in optic disc. There were white fibre rod connected with optic disc, then prolonged to vitreous cavity, connected with posterior lens capsule. CDI showed arterial blood stream signal in band-shaped echogenic structure within vitreous cavity, prolonged to lens from the optic disc, or showed funnel-shaped echogenic mass at the posterior surface of lens and anterior of vitreous body, adhered to ciliary body, lens and the optic disc. CONCLUSIONS: PHPV is congenital ocular anomalies because of a failure of primary vitreous and the hyaloids vascular system to regress. It manifests as unilateral and boys. We diagnosis PHPV by RetCamⅡ and CDI.


Asunto(s)
Técnicas de Diagnóstico Oftalmológico , Vítreo Primario Hiperplásico Persistente/diagnóstico , Ultrasonografía Doppler en Color , Catarata/etiología , Preescolar , Cuerpo Ciliar , Femenino , Humanos , Lactante , Cristalino , Masculino , Disco Óptico , Vítreo Primario Hiperplásico Persistente/complicaciones , Estudios Retrospectivos , Cuerpo Vítreo
20.
Vet Ophthalmol ; 18(6): 510-4, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25623165

RESUMEN

A 3-month wild rabbit was presented for examination of ocular opacities in the left eye. A complete bilateral ocular examination including slit-lamp examination, indirect ophthalmoscopy, tonometry, and ultrasound biomicroscopy was performed. Biomicroscopy of the lens of the left eye showed a retrolental fibrovascular membrane causing leukocoria. The opacity prevented biomicroscopy of the vitreous and funduscopy OS. No other disorder was present in either eye. Ultrasound examination did not show any difference between the right and left eye. Histopathological examination showed a 50-µm thick, preretinal, retrolental, nonpigmented, fibrovascular tissue. Posterior synechiae were present, but no other lesion of the posterior segment was found in this eye. These ocular abnormalities are consistent with a persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous (PHTVL/PHPV), similar to those described in other species.


Asunto(s)
Vítreo Primario Hiperplásico Persistente/veterinaria , Conejos/anomalías , Cuerpo Vítreo/anomalías , Animales , Femenino , Vítreo Primario Hiperplásico Persistente/diagnóstico , Vítreo Primario Hiperplásico Persistente/patología , Cuerpo Vítreo/patología
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