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1.
Graefes Arch Clin Exp Ophthalmol ; 253(5): 739-43, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25592478

RESUMEN

PURPOSE: To report the postoperative outcomes of cataract surgery in microphthalmic eyes of infants. METHODS: This prospective observational study was carried out on 20 infants with microphthalmos with visually significant cataract. Microphthalmos was defined as axial length of the globe 16.50 mm or less. We excluded eyes with ocular trauma, inflammation, posterior hyperplastic primary vitreous or a tractional retinal detachment, aniridia, or chorioretinal coloboma. All the infants enrolled in this study underwent phacoaspiration with primary posterior capsulotomy, anterior vitrectomy, and peripheral iridectomy. Intraocular lens was not implanted in these children. Post-operative evaluation included refractive errors, irregularity of pupil, posterior synechiae, visual axis obscuration, and intraocular pressure. These children were followed up for a minimum of 12 months. RESULTS: We evaluated 37 eyes of 20 infants, of whom 17 infants had bilateral and three infants had unilateral cataract. The mean age of the children and the mean axial length at the time of surgery were 3.78 ± 2.25 months and 15.76 ± 0.56 mm respectively. The complications observed were irregularity of pupil in seven eyes (18.9 %), glaucoma in five eyes (13.5 %), posterior synechiae in two eyes (5.2 %), visual axis obscuration due to posterior capsule opacification (PCO) in two eyes (5.2 %) and phthisis in one eye (2.7 %). CONCLUSION: Infants achieved a favorable outcome after phacoaspiration with primary posterior capsulotomy with anterior vitrectomy. However, these children must be followed up to detect and treat postoperative complications such as visual axis obscuration, posterior synechiae, and glaucoma to achieve optimal outcome.


Asunto(s)
Catarata/congénito , Microftalmía/complicaciones , Facoemulsificación , Afaquia Poscatarata/etiología , Afaquia Poscatarata/fisiopatología , Longitud Axial del Ojo , Femenino , Humanos , Lactante , Iridectomía , Masculino , Facoemulsificación/métodos , Capsulotomía Posterior , Estudios Prospectivos , Retinoscopía , Agudeza Visual/fisiología , Vitrectomía
2.
Int Ophthalmol ; 35(1): 95-106, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25502985

RESUMEN

The purpose is to study the ability of an event-based analysis of retinal nerve fibre layer (RNFL) attenuation measured by Stratus(®) optical coherence tomography (OCT) and to detect progression across the spectrum of glaucoma. Adult glaucoma suspects, ocular hypertensives and glaucoma patients who had undergone baseline RNFL thickness measurement on Stratus OCT and reliable automated visual field examination by Humphrey's visual field analyser prior to March 2007 and had 5-year follow-up data were recruited. Progression on OCT was defined by two criteria: decrease in average RNFL thickness from baseline by at least 10 and 20 µ. Visual field progression was defined by the modified Hodapp-Parrish-Anderson criteria. Absolute and percentage change in RNFL thickness from baseline was compared in progressors and non-progressors on visual fields. Concordance between structural and functional progression was analysed. 318 eyes of 162 patients were analysed. 35 eyes (11 %) progressed by visual fields, 8 (2.5 %) progressed using the 20 µ loss criterion, while 30 eyes (9.4 %) progressed using the 10 µ loss criterion. In glaucoma suspects, mean absolute RNFL attenuation was 8.6 µ (12.1 % of baseline) in those who progressed to glaucoma by visual fields. OCT was more useful to detect progression in early glaucoma, but performed poorly in advanced glaucoma. The 10 µ criterion appears to be closer to visual field progression. However, the ability to detect progression varies considerably between functional and structural tools depending upon the severity of the disease.


Asunto(s)
Glaucoma/diagnóstico , Presión Intraocular/fisiología , Disco Óptico/patología , Células Ganglionares de la Retina/patología , Tomografía de Coherencia Óptica/métodos , Campos Visuales/fisiología , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Glaucoma/complicaciones , Glaucoma/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Fibras Nerviosas , Hipertensión Ocular/diagnóstico , Hipertensión Ocular/fisiopatología , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/etiología , Enfermedades del Nervio Óptico/fisiopatología , Estudios Prospectivos , Estudios Retrospectivos , Pruebas del Campo Visual
3.
J Glaucoma ; 33(3): 211-217, 2024 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-37671499

RESUMEN

PRCIS: Trans-corneal transillumination (TI) aided transscleral cyclophotocoagulation (TSCPC) for intraocular pressure control of refractory pediatric glaucoma was found to be effective at 1 year. PURPOSE: To study 1-year outcomes of diode laser TSCPC with trans-corneal TI using a novel low-cost torchlight method in refractory pediatric glaucoma. METHODS: This prospective interventional study included children with refractory glaucoma who underwent TSCPC with the Oculight laser system (IRIS Medical Instruments) with trans-corneal TI (TSCPC-TI) using a novel low-cost torchlight method. Children completing a minimum 1-year follow-up were analyzed. They were compared with a historical control group of children who underwent TSCPC without TI (TSCPC-No-TI) at the 1-year follow-up period. We analyzed the mean laser energy delivered, post-laser intraocular pressure reduction, number of antiglaucoma medications (AGM), the requirement of retreatment and complications of the procedure in both groups. RESULTS: Forty-two eyes of 35 patients comprised the TSCPC-TI group and were compared with 31 eyes of 21 patients in the TSCPC-No-TI group. The TSCPC-TI group required lower energy than the TSCPC-No-TI group (24.7±7.8 J vs. 47.2±10.9 J, P <0.0001). Mean topical antiglaucoma drug requirement decreased from 2.9±0.08 before treatment to 0.66±0.8 at the end of 1 year in TSCPC-TI and from 2.6±1.0 before treatment to 0.6±0.77 in the TSCPC-No-TI groups, respectively ( P =0.15). There was a significantly reduced dependence of oral acetazolamide in the TSCPC-TI group at 1-year follow-up [5.7% vs. 61.9%, respectively ( P <0.001%)]. No adverse event of hypotony or choroidal detachment was noted in any group. CONCLUSIONS: TSCPC aided by TI with a low-cost torchlight for pediatric refractory glaucoma was found to be effective at 1 year in reducing intraocular pressure and the burden of medication.


Asunto(s)
Glaucoma , Presión Intraocular , Niño , Humanos , Coagulación con Láser/métodos , Láseres de Semiconductores/uso terapéutico , Estudios Prospectivos , Transiluminación/efectos adversos , Glaucoma/diagnóstico , Glaucoma/cirugía , Glaucoma/etiología , Cuerpo Ciliar/cirugía , Resultado del Tratamiento , Estudios Retrospectivos
4.
Am J Ophthalmol Case Rep ; 33: 101962, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38192606

RESUMEN

Purpose: To describe a novel technique for repair of rhegmatogenous retinal detachment in an eye with a previous non-valved glaucoma drainage device, the Aurolab Aqueous Drainage Implant (AADI). Observations: A 5-year-old child with bilateral primary congenital glaucoma presented with an inferior retinal detachment (RD) in the left eye. The left eye had a history of multiple surgical interventions including combined trabeculotomy and trabeculectomy done twice, AADI implantation and subsequently phacoaspiration with IOL implantation, 18 months prior to presentation. The left eye retinal detachment was managed by scleral buckling technique using the plate of the AADI as a buckling element without its explantation. Conclusions: AND IMPORTANCE: Management of retinal detachment in eyes with a pre-existing glaucoma drainage device (GDD) is uniquely challenging. Explantation of the GDD would likely result in intractable glaucoma post-operatively, requiring another surgery. Use of the trimmed plate of the GDD itself as the buckling element helped in settling the RD and preserving intraocular pressure control.

5.
Eur J Ophthalmol ; : 11206721241253305, 2024 May 06.
Artículo en Inglés | MEDLINE | ID: mdl-38710194

RESUMEN

PURPOSE: To study the outcomes of diode laser transscleral cyclophotocoagulation (TSCPC) with trans-corneal transillumination using a novel low-cost torchlight method in refractory glaucoma. METHODS AND ANALYSIS: This prospective interventional study included patients with refractory glaucoma who underwent TSCPC with trans-corneal transillumination (TSCPC-TI) using a novel low-cost torchlight method. Patients completing a minimum 6-month follow-up were analyzed. They were compared to a historical control group of patients who underwent TSCPC without transillumination (TSCPC-No TI) at 6-month follow-up period. We analyzed the mean laser energy delivered, post-laser intraocular pressure (IOP) reduction, number of antiglaucoma medications (AGM), the requirement of retreatment and complications of the procedure in both groups. RESULTS: 32 eyes of 29 patients comprised the TSCPC-TI group and were compared with 39 eyes of 37 patients in the TSCPC-No TI group. The TSCPC-TI group required lower energy than the TSCPC-No TI group (46.15 ± 22.8 Vs 80.65 ± 56.1 J p < 0.001). At 6-month follow-up, the TSCPC-TI group required lesser AGM for IOP control (2.33 ± 1.02 vs 3.02 ± 1.32 p = 0.01). There was a significantly reduced dependence of oral acetazolamide in the TSCPC-TI group at 6 months follow-up (15.6% vs 41% p = 0.03%). The success and response rates were 71.8% Vs 23.1%; p < 0.0001 and 87.5% Vs 51.2%; p = 0.001 significantly high in the TSCPC-TI group. The TSCPC-No TI group had a significantly high failure rate (12.5% Vs 48.2% p = 0.001). Hypotony (n = 1) and phthisis (n = 2) were noted TSCPC-No TI group. CONCLUSIONS: TSCPC with transillumination with a low-cost torchlight resulted in a more efficient and effective cycloablation than TSCPC without transillumination.

6.
Indian J Ophthalmol ; 2024 May 20.
Artículo en Inglés | MEDLINE | ID: mdl-38770604

RESUMEN

PURPOSE: To study the morphological and histological characteristics of the fibrous capsule formed around a novel biodegradable glaucoma drainage device (GDD) implant made of oxidized regenerated cellulose (ORC) after 1 and 3 months of its implantation. METHODS: It was a prospective, interventional, preclinical experimental study using New Zealand white rabbits. All animals underwent GDD implantation with a novel biodegradable device. The device's body was made of ORC and attached to a silicone tube, connecting the body to the anterior chamber. Histopathology (hematoxylin and eosin/Masson's trichrome staining) and immunohistochemistry (Alpha-Smooth Muscle Actin expression) characteristics of the bleb formed around the novel device were noted at 1 and 3 months. RESULTS: One month post implantation, the biodegradable material produced a significant foreign body type of reaction evidenced by the exuberant infiltration by macrophages, lymphocytes, and multinucleated giant cells. The granulomatous response subsided by 3 months with disorganized collagen deposition on Masson's trichrome staining. The silicone tube was an internal control, and histopathology demonstrated well-organized collagen deposition around it at 3 months. Immunohistochemistry for α-smooth muscle actin also demonstrated more myofibroblast transformation at the site of the tube than the biodegradable implant. CONCLUSIONS: Our results indicate that the tissue response around a biodegradable GDD was different from the response to conventional devices. A diffuse, loose vascularized mesh was observed to develop, which may be more compatible with sustained IOP control over a longer period, in contrast to the usual thick and dense capsule formed around the non-biodegradable devices.

7.
J AAPOS ; 28(2): 103863, 2024 04.
Artículo en Inglés | MEDLINE | ID: mdl-38458600

RESUMEN

PURPOSE: To investigate the correlation between swept-source anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) in congenital corneal opacity (CCO). METHODS: All children with unilateral or bilateral congenital corneal opacities who underwent examination under anesthesia (EUA) and anterior segment optical coherence tomography (AS-OCT) imaging from January 1, 2022, to December 31, 2022, were included. Main outcome measures were corneal and anterior segment evaluation and correlation of UBM and AS-OCT findings. RESULTS: A total of 22 eyes of 15 patients were imaged using both technologies. The age at first EUA ranged from 11 days to 4 years. Different phenotypes were classified based on the clinical examination, UBM, and AS-OCT findings. Fourteen eyes were diagnosed with Peters anomaly, congenital corneal staphyloma was observed in 4 eyes, 2 eyes had coloboma, 1 eye had peripheral sclerocornea, and 1 eye was diagnosed with congenital primary aphakia. AS-OCT and UBM findings were closely correlated in 18 of 22 eyes (82%) but AS-OCT failed to provide detailed information in 4 eyes (18%) where UBM revealed more details. CONCLUSIONS: Although AS-OCT offers valuable preliminary data for initial assessment and counseling, it may not consistently provide precise assessments in all cases. Therefore, UBM should be considered for definitive evaluation.


Asunto(s)
Opacidad de la Córnea , Microscopía Acústica , Niño , Humanos , Recién Nacido , Microscopía Acústica/métodos , Tomografía de Coherencia Óptica/métodos , Opacidad de la Córnea/diagnóstico por imagen , Segmento Anterior del Ojo/diagnóstico por imagen , Córnea/diagnóstico por imagen
8.
Cornea ; 43(8): 1015-1021, 2024 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-38692674

RESUMEN

PURPOSE: The purpose of this study is to report ocular cicatricial pemphigoid (OCP) occurring in young patients. Relevant literature is also reviewed. METHODS: Medical records of patients aged 30 years or younger who were treated for OCP between August 2021 and May 2023 at a tertiary care eye institute were reviewed. The most common differential diagnoses of cicatrizing conjunctivitis, such as Stevens-Johnson syndrome sequelae, chemical injury, chronic topical/systemic drug use, autoimmune connective tissue disorders, or allergic eye diseases, were ruled out based on clinical history, examination, and the Cicatrizing Conjunctivitis Score described by Shanbhag et al. The diagnosis of OCP was confirmed by positive direct immunofluorescence of oral mucosal and/or conjunctival biopsy in a majority of the patients. RESULTS: Seven patients fulfilled the criteria for a diagnosis of OCP. The mean age at presentation was 21.86 ± 5.25 years (13-28 years). Some of the patients presented with relatively atypical features for OCP such as corneal immune ring infiltrate and bilateral corneal perforation. Six patients exhibited systemic mucosal lesions, and the direct immunofluorescence yield was 85.71%. All patients required aggressive immunosuppressive treatment. CONCLUSIONS: OCP is classically described as a rare disease that occurs in old age. This case series highlights the importance of a higher index of suspicion for diagnosing OCP at a younger age. Early administration of immunosuppressive agents can potentially control severe ocular surface inflammation and its sequelae.


Asunto(s)
Penfigoide Benigno de la Membrana Mucosa , Humanos , Penfigoide Benigno de la Membrana Mucosa/diagnóstico , Penfigoide Benigno de la Membrana Mucosa/tratamiento farmacológico , Masculino , Adulto , Adolescente , Femenino , Adulto Joven , Estudios Retrospectivos , Inmunosupresores/uso terapéutico , Técnica del Anticuerpo Fluorescente Directa , Diagnóstico Diferencial , Conjuntiva/patología , Biopsia , Mucosa Bucal/patología
9.
Taiwan J Ophthalmol ; 13(4): 425-433, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38249512

RESUMEN

The basic pathophysiology of all childhood glaucoma results from impaired outflow through the trabecular meshwork. Anterior Segment Dysgeneses (ASD) are a group of nonacquired anomalies associated with secondary developmental glaucoma, characterized by impaired development of the structures of the anterior segment. Many genes impact the development of the anterior segment. The cause of the development of the abnormalities is thought to be multifactorial. Molecular research has helped our understanding of the molecular basis of ASD and the developmental mechanisms underlying these conditions. Identifying the genetic changes underlying ASD has gradually led to the recognition that some of these conditions may be parts of a disease spectrum rather than isolated anomalies. The characterization of the underlying genetic abnormalities responsible for glaucoma is the first step toward developing diagnostic and screening tests, which could identify individuals at risk for disease before irreversible optic nerve damage occurs. It is also crucial for genetic counseling and risk stratification of later pregnancies. It also aids prenatal testing by various methods allowing for effective genetic counseling. This review summarizes various ocular and systemic conditions that result in secondary developmental glaucoma and provide an overview of the phenotypes, the diagnosis and principles of management of the various disorders.

10.
J AAPOS ; 27(4): 203.e1-203.e7, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37442537

RESUMEN

PURPOSE: To compare visual field indices in normal children and adults with similar retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT). METHODS: This cross-sectional study included 59 eyes of 59 normal children 6-18 years of age compared to normal adults. The children underwent visual field testing on the Humphrey Field Analyzer (HFA) and RNFL thickness measurement on OCT. Normal adults who had undergone OCT and visual field testing were retrieved from the OCT database. The mean deviation (MD) in each child was compared with the MD in RNFL thickness-matched eyes of normal adults. Children 6-11 years and 12-18 years were analyzed separately. RESULTS: Overall, the MD of children was significantly less than that of the normal adults despite having the same RNFL thickness (-2.42 ± 1.42 dB vs -1.61 ± 1.47 dB [P = 0.006]). When stratified by age, the difference in MD of children <12 years was significantly less than normal adults (-2.72 ± 1.5dB vs -1.53 ± 1.2 dB [P = 0.003) while in children >12 years, the difference did not reach statistical significance (-2.18 ± 1.2 vs -1.51 ± 1.32 [P = 0.12]). CONCLUSIONS: In our study cohort, children <12 years had lower visual field indices than adults. Their visual fields may appear worse than they would for an adult with the same RNFL thickness. Normal children >12 years of age seem to have a retinal sensitivity comparable to the adult normative database. It is worthwhile to consider the development of a separate pediatric normative database for the visual field assessment of children <12 years of age.


Asunto(s)
Fibras Nerviosas , Campos Visuales , Adulto , Humanos , Niño , Estudios Transversales , Retina , Pruebas del Campo Visual/métodos , Tomografía de Coherencia Óptica/métodos
11.
Br J Ophthalmol ; 107(2): 227-233, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34526297

RESUMEN

OBJECTIVE: To compare CYP1B1 and MYOC variants in a cohort of neonatal-onset (NO) and infantile-onset (IO) primary congenital glaucoma (PCG). METHODS: This prospective observational study included 43 infants with PCG (14 NO and 29 IO) presenting between January 2017 and January 2019 with a minimum 1-year follow-up. CYP1B1 and MYOC genes were screened using Sanger sequencing with in-silico analysis of the variants using Polymorphism Phenotyping v.2 and Protein Variation Effect Analyser platforms. Allelic frequency was estimated using Genome Aggregation Database (gnomAd). Disease presentation and outcome were correlated to the genetic variants in both groups. RESULTS: Babies with CYP1B1 mutations had more severe disease at presentation and worse outcomes. Six of 14 (42.8%) NO glaucoma and 5 of 29 (17.2%) IO harboured CYP1B1 mutations. Five of six babies in the NO group and three of five in the IO group harboured the variant c.1169G>A, [p.R390H]. They required more surgeries and had a poorer outcome. On in-silico analysis c.1169G>A, [p.R390H] scored very likely pathogenic. Two patients in the IO group who had the c.1294C>G, [p.L432V] variant had a good outcome. Five of 14 NO-PCG and 8 of 29 IO-PCG harboured the variant c.227G>A, [p.R76K] in the MYOC gene, which was scored benign by in-silico analysis, and was also found in 2 of 15 normal controls. CONCLUSIONS: Patients with CYP1B1 pathogenic variants had a poorer outcome than those without. We found more NO PCG babies with CYP1B1 mutations compared with IO PCG. This may be one of the reasons for NO PCG having a poorer prognosis compared with IO PCG.


Asunto(s)
Glaucoma , Humanos , Lactante , Recién Nacido , Citocromo P-450 CYP1B1/genética , Análisis Mutacional de ADN , Frecuencia de los Genes , Glaucoma/genética , Glaucoma/congénito , Mutación , Linaje , Estudios Prospectivos
12.
Indian J Ophthalmol ; 70(2): 662-664, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-35086259

RESUMEN

Animal models are useful in glaucoma research to study tissue response to wound healing. Smaller animals such as rats offer additional advantages in terms of availability of detection antibodies and microarrays with cheaper maintenance costs. In this study, we describe a glaucoma filtering surgery (GFS) model in adult Sprague-Dawley rats by performing a sclerostomy using a 26-G needle and additionally placing a silicone tube (27 G) connecting the anterior chamber to the subconjunctival space to maintain a patent fistula for the flow of aqueous humor, thus providing a more definitive bleb. This technique will be useful in identifying and modifying newer targets in the wound healing process in order to improve surgical outcomes following GFS.


Asunto(s)
Cirugía Filtrante , Glaucoma , Esclerostomía , Animales , Conjuntiva , Cirugía Filtrante/métodos , Glaucoma/cirugía , Humanos , Presión Intraocular , Ratas , Ratas Sprague-Dawley
13.
J AAPOS ; 26(6): 298.e1-298.e5, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36183996

RESUMEN

PURPOSE: To describe the presentation and treatment outcomes of a cohort of children with primary aphakia (PA). METHODS: Clinical photographs and ultrasound biomicroscopy (UBM) images of children presenting with sclerocornea and undetermined anterior segment dysgenesis between July 2017 and December 2020 were reviewed retrospectively. Children who had no crystalline lens visible on UBM were included. RESULTS: A total of 124 UBM images were captured for 124 children with cloudy corneas. Twelve children were identified with congenital primary aphakia: 5 had bilateral buphthalmos, 2 had buphthalmos in one eye and microphthalmos in the other, and 5 had bilateral sclerocornea-microphthalmia complex. All patients had a peculiar silvery-blue corneal appearance, with fine vascularization on the corneal surface. The overall corneal thickness was 409.1 ± 8.7 µm. The intraocular pressure (IOP) in eyes with glaucoma was 24.5 ± 7.3 mm Hg; in microphthalmic eyes, 11.4 ± 3.4 mm Hg (P <0.001). The raised IOP was treated with limited trans-scleral cyclophotocoagulation under transillumination and topical antiglaucoma medications. Children with glaucoma gained ambulatory vision with spectacles. CONCLUSIONS: Congenital primary aphakia has a characteristic clinical appearance and may present as buphthalmos or microphthalmos, depending on the extent of dysgenesis. Incisional surgery may result in phthisis because of ciliary body dysgenesis and unpredictable aqueous production.


Asunto(s)
Afaquia , Glaucoma , Hidroftalmía , Microftalmía , Niño , Humanos , Microftalmía/diagnóstico , Estudios Retrospectivos , Afaquia/diagnóstico , Presión Intraocular , Glaucoma/congénito
14.
Rom J Ophthalmol ; 66(2): 125-131, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35935082

RESUMEN

Aim: To report the cases of five children with unilateral advanced glaucoma in isolated congenital ectropion uveae (CEU) with ipsilateral ptosis and myopia. Methods: This is an ambispective observational case series. After diagnosing one patient with CEU and glaucoma, consecutive patients presenting with unilateral ptosis, congenital iris anomaly, and glaucoma between 2014 to 2020, and had completed a minimum one-year postoperative follow-up, were analyzed. Results: Of the 1421 newly registered pediatric glaucoma patients in the period under review, five children were diagnosed with CEU. All patients presented with gradual painless diminution of vision in the left eye in early adolescence. The left eye of all patients had peculiar clinical features: mild congenital ptosis, high iris insertion, crypt-less smooth iris surface, congenital ectropion uveae, pigments over anterior lens capsule, high myopia, advanced glaucomatous optic disc cupping, and very high intraocular pressure (IOP), which was > 45 mmHg in all cases. The right eye showed signs of angle dysgenesis with mild anterior iris insertion and numerous fine iris processes. Antiglaucoma medications and angle surgery failed to control the IOP, and all children required glaucoma filtration surgery, resulting in reasonable IOP control. Despite the older age, postoperative strict amblyopia treatment resulted in significant improvement in vision. Conclusions: Although ectropion uveae and ptosis have been present since birth, unilaterality, and the asymptomatic nature of the disease led to the late presentation with irreversible damage. Early surgical management and amblyopia therapy are the cornerstones of management. Abbreviations: CEU = Congenital ectropion uvea, CIES = Congenital Iris Ectropion Syndrome, ASD = Anterior segment dysgenesis syndrome, BCVA = Best-corrected visual acuity, IOP = Intraocular pressure.


Asunto(s)
Ambliopía , Ectropión , Glaucoma , Enfermedades del Iris , Miopía , Adolescente , Niño , Ectropión/congénito , Ectropión/diagnóstico , Glaucoma/complicaciones , Glaucoma/diagnóstico , Humanos , Presión Intraocular , Síndrome
15.
Am J Ophthalmol ; 239: 54-65, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-35085548

RESUMEN

PURPOSE: To report underlying genetic variants of recently described distinct phenotype of newborn glaucoma: neonatal-onset congenital ectropion uveae (NO-CEU). DESIGN: Prospective cohort study. METHODS: Setting: tertiary care teaching institute. SUBJECTS: Thirteen children with clinical diagnosis of NO-CEU who had completed 1-year follow-up after glaucoma surgery and had undergone clinical exome sequencing (CES) by selective capture and sequencing of the protein-coding regions of the genes including 19 candidate genes for NO-CEU were assessed. The same criteria were applied for evaluating pathogenicity of variants to all the candidate genes. OUTCOME MEASURES: primary-genetic variants found on CES keeping in view the clinical indication of congenital glaucoma; secondary-corneal clarity and intraocular pressure (IOP) at baseline and 1-year follow-up, interventions required to control IOP, and postoperative visual acuity. The genetic variants were correlated with the outcome. RESULTS: All 13 patients diagnosed with NO-CEU had onset of glaucoma at birth and severe bilateral disease. Twelve of 13 (92.3%) patients harbored CYP1B1 variants. Nine of these 12 patients (83.3%) were homozygous for [c.1169G>A(p.Arg390His)] in exon-3 of CYP1B, with 5 common homozygous single-nucleotide polymorphisms flanking the pathogenic variant. They had intractable glaucoma and required multiple surgeries. Six patients had persistent corneal opacities, necessitating optical iridectomies. Three patients were compound heterozygous for CYP1B1 variants, showing [c.1169G>A(p.Arg390His)] along with [c.1103G>A(p.Arg368His)], [c.1103G>A (p.Arg368His)] along with [c.1403_1429dup(p.Arg468_Ser476dup)], and [(c.1063C>T(p.Arg355Ter)] along with [c.1325del(p.Pro442GlnfsTer15)]. These patients had better visual outcomes. CONCLUSIONS: NO-CEU appears to be a phenotypic marker for specific CYP1B1 genotypes, one of which is [c.1169G>A(p.Arg390His)] in our study population. Phenotype recognition is helpful to characterize the underlying genetic variants.


Asunto(s)
Ectropión , Glaucoma , Hidroftalmía , Citocromo P-450 CYP1B1/genética , Análisis Mutacional de ADN , Ectropión/congénito , Ectropión/genética , Glaucoma/diagnóstico , Glaucoma/genética , Glaucoma/cirugía , Humanos , Hidroftalmía/diagnóstico , Hidroftalmía/genética , Hidroftalmía/cirugía , Recién Nacido , Presión Intraocular , Mutación , Estudios Prospectivos
16.
Br J Ophthalmol ; 106(3): 368-375, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-33268344

RESUMEN

BACKGROUND: Neonatal-onset glaucoma (NOG) is a severe form of childhood glaucoma and is not always due to primary congenital glaucoma (PCG). Due to advances in neonatal care, the incidence of NOG is rising, but it remains an under-reported entity. The objective of the paper was to study the clinical profiles, surgical and visual outcomes of NOG at least 1 year following early surgery. METHODS: Prospective interventional cohort study at a tertiary care referral centre. Babies with NOG, who presented between January 2013 and December 2017, had a history suggestive of disease onset within 1 month of birth, and underwent surgery by 3 months of age, were prospectively enrolled. Those who completed a 1-year follow-up after surgery were analysed. RESULTS: 94 eyes of 53 babies were analysed. 35 (66%) had PCG. Neonatal congenital ectropion uveae, congenital rubella syndrome, Peter's anomaly and Sturge-Weber syndrome comprised the non-PCG group. The mean age at presentation and surgery was 24.8±21.9, and 36.7±29.9 days. Additional glaucoma surgery was required in 43 of the 94 eyes (45.7%). PCG had significantly better outcomes than other glaucomas at all time points. 28.3% of eyes had good vision (LogMar (0-0.5)), 34.7% had moderate visual impairment (LogMar 0.7-1.0) and 16% were blind (LogMar <1.62) . CONCLUSION: Our study shows that NOG does not always have a dismal prognosis. A small but significant proportion could have other underlying conditions than PCG. Timely surgery and rigorous amblyopia therapy resulted in good outcomes in terms of intraocular pressure control and vision in this cohort.


Asunto(s)
Glaucoma , Trabeculectomía , Estudios de Cohortes , Estudios de Seguimiento , Glaucoma/congénito , Glaucoma/epidemiología , Glaucoma/cirugía , Humanos , Lactante , Recién Nacido , Presión Intraocular , Estudios Prospectivos , Estudios Retrospectivos , Resultado del Tratamiento
17.
Ophthalmol Glaucoma ; 5(4): 428-435, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-34954410

RESUMEN

PURPOSE: To study the frequency of intrauterine rubella infection in a cohort of neonatal-onset glaucoma (NOG) and its effect on the treatment outcomes. DESIGN: Prospective cohort study. PARTICIPANTS: Infants with NOG presenting to the pediatric glaucoma service at a tertiary care center in northern India at Chandigarh between January 1, and September 30, 2018, with a minimum postoperative follow-up of 1 year were included in this study. METHODS: Rubella immunoglobulin-M (IgM) antibodies were tested in all patients. Surgery for intraocular pressure (IOP) control comprised combined trabeculotomy with trabeculectomy or goniotomy. Presenting features and treatment outcomes at 1 year were compared among infants with and without rubella-IgM antibodies. RESULTS: Of 27 eligible infants, 7 (25.9%) were rubella-IgM positive, and all had bilateral glaucoma. One eye in a rubella-positive baby was an unsightly blind eye and required a prosthesis. A total of 13 eyes of 7 patients were compared with 34 eyes of 20 rubella-negative patients. There was no significant difference in mean age and IOP at presentation. Rubella-positive infants had significantly more opaque corneas (P < 0.001), shorter eyes (P < 0.001), and smaller corneal diameters (P = 0.007) at presentation. Two patients in the rubella group had bilateral cataracts, 3 patients had heart disease, and 1 patient had sensorineural deafness. Salt-and-pepper retinopathy was noted in both rubella-positive infants where fundus evaluation was possible. Despite comparable IOP control (P = 0.51), the corneal clarity remained significantly worse in the rubella-positive patients (P = 0.02). CONCLUSIONS: Twenty-five percent of those with newborn glaucoma had underlying intrauterine rubella infection, thus making them susceptible for development of congenital rubella syndrome (CRS). Neonatal-onset glaucoma is an important component of CRS, which may present without buphthalmos and persistent corneal clouding despite good IOP control.


Asunto(s)
Glaucoma , Hidroftalmía , Síndrome de Rubéola Congénita , Niño , Glaucoma/cirugía , Humanos , Inmunoglobulina M , Lactante , Recién Nacido , Estudios Prospectivos , Estudios Retrospectivos , Síndrome de Rubéola Congénita/complicaciones , Síndrome de Rubéola Congénita/diagnóstico
18.
Eur J Ophthalmol ; 31(1): 234-239, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31587568

RESUMEN

AIM: To study the treatment outcomes and efficacy of pars plana vitrectomy-hyaloidotomy-zonulectomy-iridotomy in malignant glaucoma. METHOD: A retrospective case series study of patients treated for malignant glaucoma between 2005 and 2017. The treatment included medical management as first-line in the form of cycloplegic with anti-glaucoma medications (AGM) or pars plana vitrectomy-hyaloidotomy-zonulectomy-iridotomy as second-line therapy. The resolution was defined as 'deepening of central anterior chamber' with intraocular pressure of ⩽ 21 mm Hg (minimum on two consecutive visits 1 week apart) with or without topical anti-glaucoma medications in the absence (complete success) or presence (qualified success) of systemic medications. RESULTS: Twenty-three eyes of 22 patients were included. At the time of diagnosis of malignant glaucoma preceding surgeries were cataract surgery (13 eyes) and trabeculectomy (4 eyes). One eye each had phacotrabeculectomy, Yag capsulotomy and optical keratoplasty. Eighteen eyes were pseudophakic and two eyes were phakic. At presentation, mean intraocular pressure was 26.25 ± 14.78 mmHg which was decreased to 15.90 ± 8.12 mmHg (p < 0.0001) at final follow-up (median follow-up was 15.50 months). Fifteen (75%) eyes had complete success, 3 (15%) eyes had qualified success and 2 (10%) eyes had treatment failure. Of 15 eyes that had achieved complete success, 3 eyes had undergone medical management and 12 eyes undergone second-line procedure. There was no significant difference in visual acuity or number of anti-glaucoma medication at presentation and final follow-up (p > 0.05). CONCLUSIONS: Though malignant glaucoma is highly refractory to treatment, appropriate management in the form of medical or vitrectomy-hyaloidotomy-zonulectomy-iridotomy could achieve 90% success in this series.


Asunto(s)
Glaucoma/cirugía , Iridectomía , Ligamentos/cirugía , Vitrectomía , Cuerpo Vítreo/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Extracción de Catarata , Femenino , Glaucoma/fisiopatología , Humanos , Presión Intraocular/fisiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tonometría Ocular , Trabeculectomía , Resultado del Tratamiento , Agudeza Visual
19.
Indian J Ophthalmol ; 69(7): 1928-1932, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-34146058

RESUMEN

PURPOSE: Coronavirus Disease 2019 (COVID-19) pandemic has negatively impacted medical professionals in all fields of medicine and surgery in their academic, clinical and surgical training. The impact of surgical training has been described as 'severe' by most ophthalmology residents worldwide due to their duties in COVID-19 wards, disruption of outpatient and camp services. METHODS: Ophthalmic surgery demands utmost accuracy and meticulousness. Fine motor proficiencies, stereoscopic skills and hand-eye coordination required can only be achieved by practice. So, a multileveled structured wet-lab teaching schedule was prepared for the residents and implemented to bridge this gap between theory and practice at our tertiary care institute. A semester-wise training schedule was made with the proper distribution of wet-lab and simulator training. Surgeries like phacoemulsification, scleral buckling, pars plana lensectomy and vitrectomy, trabeculectomy and intravitreal injections were practised by the residents on the goat eyes. Simulator training was provided for phacoemulsification and vitrectomy to increase the hand-eye coordination of the residents. RESULTS: Residents noticed improvement in their surgical skills and ambidexterity post wet-lab and simulator training . It also increased their confidence and provided essential surgical skills required to be used in the operation theater later. CONCLUSION: It is imperative that wet-lab training be included in the residency training programme in this COVID-19 era.


Asunto(s)
COVID-19 , Internado y Residencia , Oftalmología , Competencia Clínica , Educación de Postgrado en Medicina , Humanos , India/epidemiología , Oftalmología/educación , SARS-CoV-2 , Atención Terciaria de Salud
20.
Am J Ophthalmol ; 223: 83-90, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-33045217

RESUMEN

PURPOSE: To describe neonatal-onset congenital ectropion uveae (N-CEU) as a distinct clinical entity of newborn glaucoma (NG) and to study its significance toward the severity and outcome of NG. DESIGN: Prospective clinical cohort study. METHODS: The study took place at a tertiary care postgraduate teaching institute. It included consecutive patients with NG who presented between July 1, 2016 and September 30, 2017, with a minimum postoperative follow-up of 1 year. Infants with any ocular anomaly apart from CEU were excluded. Patients with N-CEU were compared with those with neonatal-onset primary congenital glaucoma (N-PCG). All infants underwent goniotomy or trabeculotomy, with trabeculectomy depending on corneal clarity. Clinical features at presentation and outcome 1 year after surgery were defined as good or satisfactory if intraocular pressure was ≤16.0 mm Hg under anesthesia without or with topical medications, respectively, and poor if the infant required additional surgery. RESULTS: Twenty eyes of 10 patients with N-CEU were compared with 16 eyes of 9 patients with N-PCG. Infants with N-CEU had significantly worse corneal clarity (mean grade 2.0 ± 0.7 vs 1.4 ± 0.8; P = .026) and poorer outcomes compared with those with N-PCG. Seven of 16 (43.7%) eyes with N-PCG had a cornea clear enough at presentation for a goniotomy compared with only 2 of the 20 (10%) eyes with N-CEU (P = .026). Thirteen of 16 (81.2%) eyes with N-PCG had a good or satisfactory outcome compared with 6 of 20 (30%) eyes with N-CEU (P = .001). CONCLUSIONS: N-CEU appears to be distinct from the unilateral CEU in older patients described in the literature and may be considered a poorer prognosis phenotype of neonatal-onset glaucoma.


Asunto(s)
Ectropión/congénito , Hidroftalmía/diagnóstico , Enfermedades del Recién Nacido/diagnóstico , Ectropión/epidemiología , Ectropión/fisiopatología , Femenino , Estudios de Seguimiento , Gonioscopía , Humanos , Hidroftalmía/epidemiología , Hidroftalmía/fisiopatología , Incidencia , Lactante , Recién Nacido , Enfermedades del Recién Nacido/epidemiología , Enfermedades del Recién Nacido/fisiopatología , Presión Intraocular/fisiología , Masculino , Fenotipo , Estudios Prospectivos , Tonometría Ocular , Trabeculectomía
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