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1.
Ophthalmology ; 128(5): 672-685, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33045315

RESUMEN

PURPOSE: To report the anatomic and functional outcomes of autologous retinal transplantation (ART). DESIGN: Multicenter, retrospective, interventional, consecutive case series. PARTICIPANTS: One hundred thirty eyes of 130 patients undergoing ART for the repair of primary and refractory macular holes (MHs), as well as combined MH-rhegmatogenous retinal detachment (MH-RRD), between January 2017 and December 2019. METHODS: All patients underwent pars plana vitrectomy and ART, with surgeon modification of intraoperative variables. A large array of preoperative, intraoperative, and postoperative data was collected. Two masked reviewers graded OCT images. Multivariate statistical analysis and subgroup analysis were performed. MAIN OUTCOME MEASURES: Macular hole closure rate, visual acuity (VA), external limiting membrane and ellipsoid zone (EZ) band integrity, and alignment of neurosensory layers (ANL) on OCT. RESULTS: One hundred thirty ART surgeries were performed by 33 vitreoretinal surgeons worldwide. Patient demographics were: mean age of 63 ± 6.3 years, 58% female, 41% White, 23% Black, 19% Asian, and 17% Latino. Preoperative VA was 1.37 ± 0.12 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, approximately 20/500), which improved significantly to 1.05 ± 0.09 logMAR (Snellen equivalent, approximately 20/225; P < 0.001) after surgery (mean follow-up, 8.6 ± 0.8 months). Autologous retinal transplantation was performed for primary MH repair in 27% of patients (n = 35), for refractory MH in 58% of patients (n = 76; mean number of previous surgeries, 1.6 ± 0.2), and for MH-RRD in 15% of patients (n = 19). Mean maximum MH diameter was 1470 ± 160 µm, mean minimum diameter was 840 ± 94 µm, and mean axial length was 24.6 ± 3.2 mm. Overall, 89% of MHs closed (78.5% complete; 10% small eccentric defect), with a 95% closure rate in MH-RRD (68.4% complete; 26.3% small eccentric defect). Visual acuity improved by at least 3 lines in 43% of eyes and by at least 5 lines in 29% of eyes. Reconstitution of the EZ (P = 0.02) and ANL (P = 0.01) on OCT were associated with better final VA. Five cases of ART graft dislocation (3.8%), 5 cases of postoperative retinal detachment (3.8%), and 1 case of endophthalmitis (0.77%) occurred. CONCLUSIONS: In this global experience, patients undergoing ART for large primary and refractory MHs and MH-RRDs achieved good anatomic and functional outcomes, with low complication rates despite complex surgical pathologic features.


Asunto(s)
Retina/trasplante , Desprendimiento de Retina/cirugía , Perforaciones de la Retina/cirugía , Anciano , Membrana Basal/fisiología , Femenino , Estudios de Seguimiento , Salud Global , Humanos , Masculino , Persona de Mediana Edad , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/fisiopatología , Perforaciones de la Retina/diagnóstico , Perforaciones de la Retina/fisiopatología , Estudios Retrospectivos , Tomografía de Coherencia Óptica , Trasplante Autólogo , Resultado del Tratamiento , Agudeza Visual/fisiología , Vitrectomía
2.
Mol Cell ; 49(1): 145-57, 2013 Jan 10.
Artículo en Inglés | MEDLINE | ID: mdl-23201126

RESUMEN

The production of pigment by melanocytes tans the skin and protects against skin cancers. UV-exposed keratinocytes secrete α-MSH, which then activates melanin formation in melanocytes by inducing the microphthalmia-associated transcription factor (MITF). We show that PPAR-γ coactivator (PGC)-1α and PGC-1ß are critical components of this melanogenic system in melanocytes. α-MSH signaling strongly induces PGC-1α expression and stabilizes both PGC-1α and PGC-1ß proteins. The PGC-1s in turn activate the MITF promoter, and their expression correlates strongly with that of MITF in human melanoma cell lines and biopsy specimens. Inhibition of PGC-1α and PGC-1ß blocks the α-MSH-mediated induction of MITF and melanogenic genes. Conversely, overexpression of PGC-1α induces pigment formation in cell culture and transgenic animals. Finally, polymorphism studies reveal expression quantitative trait loci in the PGC-1ß gene that correlate with tanning ability and protection from melanoma in humans. These data identify PGC-1 coactivators as regulators of human tanning.


Asunto(s)
Proteínas Portadoras/fisiología , Proteínas de Choque Térmico/fisiología , Melanoma/metabolismo , Factor de Transcripción Asociado a Microftalmía/metabolismo , Neoplasias Cutáneas/metabolismo , Bronceado/genética , Factores de Transcripción/fisiología , Animales , Proteínas Portadoras/genética , Proteínas Portadoras/metabolismo , Estudios de Casos y Controles , Línea Celular Tumoral , Expresión Génica , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Proteínas de Choque Térmico/genética , Proteínas de Choque Térmico/metabolismo , Humanos , Queratinocitos/metabolismo , Queratinocitos/efectos de la radiación , Melaninas/biosíntesis , Melanocitos/enzimología , Melanocitos/metabolismo , Melanoma/genética , Melanoma/patología , Ratones , Ratones Endogámicos C57BL , Factor de Transcripción Asociado a Microftalmía/genética , Monofenol Monooxigenasa/metabolismo , Coactivador 1-alfa del Receptor Activado por Proliferadores de Peroxisomas gamma , Polimorfismo de Nucleótido Simple , Regiones Promotoras Genéticas , Estabilidad Proteica , Proteínas de Unión al ARN , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/patología , Factores de Transcripción/genética , Factores de Transcripción/metabolismo , Activación Transcripcional , alfa-MSH/metabolismo , alfa-MSH/fisiología
3.
Retina ; 41(3): 480-486, 2021 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-32969983

RESUMEN

PURPOSE: To evaluate the retinal periphery in patients with idiopathic juxtafoveal telangiectasis or macular telangiectasis Type 2 (MacTel2), using widefield fluorescein angiography. METHODS: Single-center, retrospective, observational case series of 50 eyes of 50 patients with MacTel2 and 50 eyes of 50 age-matched controls. RESULTS: Thirty-seven eyes in the MacTel2 group (74%) showed peripheral capillary nonperfusion or dropout, compared with 37 eyes in the control group (74%, P = 1.0). Morphologically, the MacTel2 group trended toward having a higher proportion of pruning-type capillary dropout (44%) compared with controls (28%), but this was not statistically significant (P = 0.12). Patients with MacTel2 had a higher incidence of microaneurysms compared with controls (MacTel2 56%; controls 42%; P = 0.048), independent of age or systemic risk factors. There was no difference in the incidence of venous-venous shunts (MacTel2 10%; controls 10%; P = 1.0), arteriovenous shunts (MacTel2 14%; controls 18%; P = 0.60), venous tortuosity (MacTel2 60%; controls 66%; P = 0.58), or arterial tortuosity (MacTel2 54%; controls 68%; P = 0.20), which was mild in most cases. CONCLUSION: We note a high incidence of peripheral vascular and retinal findings in both patients with MacTel2 and age-matched controls, using widefield fluorescein angiography. Patients with MacTel2 had significantly more microaneurysms, independent of age or other systemic risk factors.


Asunto(s)
Angiografía con Fluoresceína/métodos , Mácula Lútea/irrigación sanguínea , Telangiectasia Retiniana/diagnóstico , Vasos Retinianos/diagnóstico por imagen , Anciano , Capilares/diagnóstico por imagen , Femenino , Humanos , Mácula Lútea/diagnóstico por imagen , Masculino , Estudios Retrospectivos , Tomografía de Coherencia Óptica
4.
Retina ; 41(6): 1242-1250, 2021 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-33079789

RESUMEN

PURPOSE: To evaluate the clinical course of patients with neovascular age-related macular degeneration (nAMD) after developing endophthalmitis during their treatment with intravitreal injections. METHODS: Multicenter, retrospective series. RESULTS: From April 2013 to October 2018, 196,598 intravitreal anti-vascular endothelial growth factor (VEGF) injections were performed, with 75 cases of endophthalmitis (incidence 0.0381%). There was no association between intravitreal anti-VEGF drug (P = 0.29), anesthetic method (P = 0.26), povidone concentration (P = 0.22), or any intraprocedure variable and endophthalmitis incidence. Seventy-two patients (96%) were treated with intravitreal tap and inject , while 3 underwent immediate pars plana vitrectomy. After endophthalmitis resolution, 17 patients (22.7%) were not re-treated for nAMD (in 10 cases due to inactive disease; follow-up, 115 ± 8.4 weeks). Patients required less frequent anti-VEGF injections after infection (7.4 ± 0.61 weeks vs. 11.5 ± 1.8 weeks; P = 0.004). Preinfection logarithm of the minimum angle of resolution visual acuity was 0.585 ± 0.053 (∼20/77). It worsened with endophthalmitis (1.67 ± 0.08, ∼20/935; P < 0.001) and again on postendophthalmitis treatment day 1 (1.94 ± 0.064; count fingers; P < 0.001), but improved after reinitiating nAMD therapy (1.02 ± 0.11; ∼20/209; P < 0.001). Better visual acuity on postendophthalmitis week 1 (P = 0.002) and reinitiation of nAMD treatment (P = 0.008) were associated with better final visual acuity, and streptococcal culture with worse visual acuity (P = 0.028). The postendophthalmitis treatment interval was associated with the anti-VEGF drug used (aflibercept = ranibizumab > bevacizumab; P < 0.001). CONCLUSION: Patients with nAMD required fewer injections after endophthalmitis, suggesting a biological change in disease activity. Neovascular age-related macular degeneration became quiescent in 13.3% of eyes. Most achieved better outcomes with anti-VEGF reinitiation.


Asunto(s)
Inhibidores de la Angiogénesis/administración & dosificación , Endoftalmitis/etiología , Medición de Riesgo/métodos , Degeneración Macular Húmeda/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Inhibidores de la Angiogénesis/efectos adversos , Endoftalmitis/epidemiología , Femenino , Humanos , Incidencia , Inyecciones Intravítreas/efectos adversos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Tomografía de Coherencia Óptica/métodos , Estados Unidos/epidemiología , Factor A de Crecimiento Endotelial Vascular/antagonistas & inhibidores , Agudeza Visual , Degeneración Macular Húmeda/diagnóstico
5.
Graefes Arch Clin Exp Ophthalmol ; 258(12): 2629-2638, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-32910308

RESUMEN

BACKGROUND: The classification of macular hole closure patterns (MHCPs) currently relies on time domain OCT allowing only "open" and "closed" statuses or is based on inner foveal contour shape. Both classification types give no information on retinal layer reconstitution. Novel sophisticated surgical techniques lead to previously unknown MHCPs, outdating existing classifications and urging new ones. The purpose of the present study is to introduce a new classification allowing proper description of all MHCPs resulting from newer surgeries and based on the restoration of retinal layers. METHODS: Retrospective analysis of patients undergoing MH surgery with five different surgical techniques was performed. MHCPs were classified according to spectral domain optical coherence tomography (SD-OCT). Type 0: open MH (0A: flat margin, 0B: elevated, 0C: oedematous); type 1: closed MHs (1A: reconstitution all retinal layers; 1B interruption of the external layers; 1C interruption of internal layers); type 2: MH closed with autologous or heterologous filling tissue interrupting the normal foveal layered anatomy (2A: filling tissue through all layers; 2B reconstitution of normal inner retinal layers; 2C reconstitution of normal outer retinal layers; 2D H-shaped bridging of filling tissue). RESULTS: Closure rate was 95.2% (241/253). Surgical technique and vision correlated to closure pattern (p < 0.001). Type 1 MHCPs had the best post-operative visual acuity (VA) compared with type 2 and type 0 (p < 0.001). MHCPs 1A and 1C performed better than all others. MHCP at months 1 and 3 changed in 42/254 (16.5%) and remained stable in 212/254 (83.5%). Improvement in vision was higher in eyes with shifting closure pattern (0.57 ± 0.33 vs 0.51 ± 0.48 logMAR; p 0.021). CONCLUSION: MHCP classification based on retinal layer restoration properly comprises post-operative anatomic morphologies. MHCPs correlate the surgical technique and post-operative visual outcomes.


Asunto(s)
Perforaciones de la Retina , Membrana Basal , Fóvea Central , Humanos , Perforaciones de la Retina/diagnóstico , Perforaciones de la Retina/cirugía , Estudios Retrospectivos , Tomografía de Coherencia Óptica , Vitrectomía
6.
Nature ; 485(7398): 333-8, 2012 May 09.
Artículo en Inglés | MEDLINE | ID: mdl-22596155

RESUMEN

Peripartum cardiomyopathy (PPCM) is an often fatal disease that affects pregnant women who are near delivery, and it occurs more frequently in women with pre-eclampsia and/or multiple gestation. The aetiology of PPCM, and why it is associated with pre-eclampsia, remain unknown. Here we show that PPCM is associated with a systemic angiogenic imbalance, accentuated by pre-eclampsia. Mice that lack cardiac PGC-1α, a powerful regulator of angiogenesis, develop profound PPCM. Importantly, the PPCM is entirely rescued by pro-angiogenic therapies. In humans, the placenta in late gestation secretes VEGF inhibitors like soluble FLT1 (sFLT1), and this is accentuated by multiple gestation and pre-eclampsia. This anti-angiogenic environment is accompanied by subclinical cardiac dysfunction, the extent of which correlates with circulating levels of sFLT1. Exogenous sFLT1 alone caused diastolic dysfunction in wild-type mice, and profound systolic dysfunction in mice lacking cardiac PGC-1α. Finally, plasma samples from women with PPCM contained abnormally high levels of sFLT1. These data indicate that PPCM is mainly a vascular disease, caused by excess anti-angiogenic signalling in the peripartum period. The data also explain how late pregnancy poses a threat to cardiac homeostasis, and why pre-eclampsia and multiple gestation are important risk factors for the development of PPCM.


Asunto(s)
Cardiomiopatías/etiología , Cardiomiopatías/fisiopatología , Neovascularización Patológica/complicaciones , Neovascularización Patológica/fisiopatología , Complicaciones Cardiovasculares del Embarazo/etiología , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Animales , Bromocriptina/farmacología , Bromocriptina/uso terapéutico , Cardiomiopatías/sangre , Cardiomiopatías/tratamiento farmacológico , Modelos Animales de Enfermedad , Femenino , Corazón/efectos de los fármacos , Corazón/fisiopatología , Humanos , Estimación de Kaplan-Meier , Masculino , Ratones , Ratones Noqueados , Miocitos Cardíacos/efectos de los fármacos , Miocitos Cardíacos/metabolismo , Neovascularización Patológica/tratamiento farmacológico , Neovascularización Fisiológica/efectos de los fármacos , Neovascularización Fisiológica/fisiología , Coactivador 1-alfa del Receptor Activado por Proliferadores de Peroxisomas gamma , Preeclampsia/fisiopatología , Embarazo , Complicaciones Cardiovasculares del Embarazo/sangre , Complicaciones Cardiovasculares del Embarazo/tratamiento farmacológico , Transactivadores/deficiencia , Transactivadores/genética , Transactivadores/metabolismo , Factores de Transcripción , Factor A de Crecimiento Endotelial Vascular/farmacología , Factor A de Crecimiento Endotelial Vascular/uso terapéutico , Receptor 1 de Factores de Crecimiento Endotelial Vascular/sangre , Receptor 1 de Factores de Crecimiento Endotelial Vascular/genética , Receptor 1 de Factores de Crecimiento Endotelial Vascular/metabolismo , Receptor 1 de Factores de Crecimiento Endotelial Vascular/farmacología
9.
BMJ Open ; 11(3): e038565, 2021 03 17.
Artículo en Inglés | MEDLINE | ID: mdl-33737416

RESUMEN

OBJECTIVE: To identify factors that contribute to missed cataract surgery follow-up visits, with an emphasis on socioeconomic and demographic factors. METHODS: In this retrospective cohort study, patients who underwent cataract extraction by phacoemulsification at Massachusetts Eye and Ear between 1 January and 31 December 2014 were reviewed. Second eye cases, remote and international patients, patients with foreign insurance and combined cataract cases were excluded. RESULTS: A total of 1931 cases were reviewed and 1089 cases, corresponding to 3267 scheduled postoperative visits, were included. Of these visits, 157 (4.8%) were missed. Three (0.3%) postoperative day 1, 40 (3.7%) postoperative week 1 and 114 (10.5%) postoperative month 1 visits were missed. Age<30 years (adjusted OR (aOR)=8.2, 95% CI 1.9 to 35.2) and ≥90 years (aOR=5.7, 95% CI 2.0 to 15.6) compared with patients aged 70-79 years, estimated travel time of >2 hours (aOR=3.2, 95% CI 1.4 to 7.4), smokers (aOR=2.7, 95% CI 1.6 to 4.8) and complications identified up to the postoperative visit (aOR=1.4, 95% CI 1.0 to 2.1) predicted a higher rate of missed visits. Ocular comorbidities (aOR=0.7, 95% CI 0.5 to 1.0) and previous visit best-corrected visual acuity (BCVA) of 20/50-20/80 (aOR=0.4, 95% CI 0.3 to 0.7) and 20/90-20/200 (aOR=0.4, 95% CI 0.2 to 0.9), compared with BCVA at the previous visit of 20/40 or better, predicted a lower rate of missed visits. Gender, race/ethnicity, language, education, income, insurance, alcohol use and season of the year were not associated with missed visits. CONCLUSIONS: Medical factors and demographic characteristics, including patient age and distance from the hospital, are associated with missed follow-up visits in cataract surgery. Additional studies are needed to identify disparities in cataract postoperative care that are population-specific. This information can contribute to the implementation of policies and interventions for addressing them.


Asunto(s)
Extracción de Catarata , Catarata , Lentes Intraoculares , Facoemulsificación , Adulto , Anciano , Anciano de 80 o más Años , Catarata/epidemiología , Estudios de Seguimiento , Disparidades en Atención de Salud , Humanos , Massachusetts , Atención Perioperativa , Estudios Retrospectivos , Estados Unidos/epidemiología
10.
Clin Lab Med ; 40(2): 189-204, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32439068

RESUMEN

Inherited retinal diseases (IRDs) represent a diverse array of conditions characterized by dysfunction or loss of 1 or more retinal cell types. Next-generation sequencing has enabled rapid and relatively inexpensive genotyping, with more than 250 genes identified as responsible for IRDs. This expansion in molecular diagnostic accuracy, in combination with the retina's relative accessibility and immune privilege, has fostered the development of precision therapies to treat these myriad conditions. Novel techniques are being used in early trials. Precision molecular therapies for IRDs hold great promise as diagnostic and treatment strategies continue to expand.


Asunto(s)
Terapia Genética , Genómica , Medicina de Precisión , Enfermedades de la Retina , Sistemas CRISPR-Cas/genética , Dependovirus/genética , Edición Génica , Secuenciación de Nucleótidos de Alto Rendimiento , Técnicas de Diagnóstico Molecular , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/genética , Enfermedades de la Retina/terapia , Análisis de Secuencia de ADN
11.
Ophthalmic Surg Lasers Imaging Retina ; 50(4): 215-220, 2019 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-30998242

RESUMEN

BACKGROUND AND OBJECTIVE: To quantify vessel tortuosity among infants with retinopathy of prematurity (ROP). PATIENTS AND METHODS: This was a retrospective study including 61 RetCam images from 33 infants. The laser treatment (LT) group included 17 infants who underwent laser for ROP. The no-treatment (NT) group included 16 infants. The modified ROPtool was used to calculate mean vessel tortuosity (MVT) and highest vessel tortuosity (HVT) for the participants and for the standard plus disease photograph from the Early Treatment for Retinopathy of Prematurity (ETROP) study. RESULTS: The median MVT was 1.226 versus 1.056 for the LT and NT groups, respectively (P < .001). The median HVT was 1.346 versus 1.088 (P < .001). An MVT of 1.124 was 96.7% sensitive and 100% specific for identifying infants with treatment-requiring ROP. Both MVT and HVT cutoff values correctly captured plus disease in the standard ETROP trial photograph. CONCLUSION: The modified ROPtool can be used to identify infants who have treatment-requiring ROP with a high level of sensitivity and specificity. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:215-220.].


Asunto(s)
Terapia por Láser/métodos , Vasos Retinianos/patología , Retinopatía de la Prematuridad/diagnóstico , Diagnóstico por Computador , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Valor Predictivo de las Pruebas , Curva ROC , Reproducibilidad de los Resultados , Retinopatía de la Prematuridad/cirugía , Estudios Retrospectivos
12.
Am J Ophthalmol ; 199: 94-100, 2019 03.
Artículo en Inglés | MEDLINE | ID: mdl-30339795

RESUMEN

PURPOSE: To ascertain the incidence of unexpected management changes at the postoperative week 1 visit in asymptomatic patients who have had an uncomplicated cataract surgery and a routine postoperative day 1 examination. DESIGN: Retrospective observational study. METHODS: A retrospective chart review was conducted of all cases of cataract extraction by phacoemulsification with intraocular lens insertion performed by the Comprehensive Ophthalmology Service at Massachusetts Eye and Ear between January 1, 2014 and December 31, 2014. The preoperative consultation, operative report, and postoperative day 1 and week 1 (postoperative days 5-14) visits were reviewed. Cases with intraoperative complications, as well as clinical findings at postoperative day 1 requiring close follow-up, were excluded. The main outcome measure was incidence of unexpected management changes at the postoperative week 1 visit after cataract surgery, defined as an unanticipated change in postoperative drops, additional procedures, or urgent referral to a specialty service. RESULTS: Overall, 1938 surgical cases of 1471 patients were reviewed, and 1510 cases (77.9%) underwent uncomplicated phacoemulsification with intraocular lens implantation with a routine postoperative day 1 examination. Of these 1510 cases, 238 (15.8%) reported symptoms at the postoperative week 1 visit, including flashes, floaters, redness, pain, or decreased vision, which warranted an examination. In total, 1272 cases were asymptomatic, and only 11 of these cases (0.9%) had an unexpected management change at postoperative week 1. Eight of 11 patients were asymptomatic steroid responders requiring alteration of their postoperative drops. Two of these patients had an intraocular pressure >30 mm Hg. CONCLUSIONS: Unexpected management changes at the postoperative week 1 timepoint after cataract surgery are rare in asymptomatic patients who have had uncomplicated cataract surgery and a routine postoperative day 1 examination. Limited data are available to outline an optimal postoperative regimen after cataract surgery. The results of this study suggest that postoperative week 1 examinations could potentially be performed on an as-needed basis in the appropriate subgroup of patients after cataract surgery.


Asunto(s)
Implantación de Lentes Intraoculares/métodos , Atención Perioperativa/métodos , Facoemulsificación/métodos , Complicaciones Posoperatorias/epidemiología , Anciano , Antiinflamatorios no Esteroideos/uso terapéutico , Antihipertensivos/uso terapéutico , Femenino , Humanos , Incidencia , Inflamación/tratamiento farmacológico , Inflamación/etiología , Presión Intraocular/efectos de los fármacos , Masculino , Persona de Mediana Edad , Hipertensión Ocular/tratamiento farmacológico , Hipertensión Ocular/etiología , Complicaciones Posoperatorias/tratamiento farmacológico , Periodo Posoperatorio , Estudios Retrospectivos
13.
Ophthalmol Retina ; 3(1): 77-82, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30935660

RESUMEN

PURPOSE: To evaluate the natural history of congenital X-linked retinoschisis (CXLRS) and to assess disease stability or progression over time. DESIGN: Retrospective case series at a single-center, tertiary care, pediatric retina practice. PARTICIPANTS: One hundred two eyes of 51 patients with CXLRS. METHODS: The clinical examinations, fundus photographs, and OCT images of all patients with CXLRS were assessed. Eyes that initially demonstrated combined retinoschisis-retinal detachments and those with large, centrally overhanging schisis cavities were excluded from the analysis (n = 49) because they underwent surgery, which precluded observation of the natural disease course. MAIN OUTCOME MEASURES: Stability or conversion of CXLRS phenotype over time. RESULTS: Fifty-three eyes met inclusion criteria for observation of natural disease history over time. At the time of diagnosis, 7.5% of eyes showed type 1 disease (n = 4), 17% showed type 2 disease (n = 9), 66% showed type 3 disease (n = 35), and 9.5% showed type 4 disease (n = 5). Mean length of follow-up was 7.4 years. A total of 7.5% of eyes demonstrated a combined retinoschisis-retinal detachment requiring surgery (n = 4), whereas 1.8% demonstrated a large, centrally overhanging schisis cavity requiring surgery (n = 1). Overall, 83% of eyes (n = 44) remained the same type without conversion or development of a complication requiring surgery. The remaining 17% of eyes (n = 9) experienced some type of change from their baseline diagnosis, with 7.5% (n = 4) converting between phenotypes and 9.5% (n = 5) demonstrating a complication requiring surgery; 3.75% of eyes (n = 2) converted from type 2 to 3 and 7.5% of eyes (n = 4) converted from type 3 to a combined retinoschisis-retinal detachment with mean time to conversion of 4.07 years. CONCLUSIONS: This longitudinal study conveyed the natural history of CXLRS. Congenital X-linked retinoschisis displayed long-term stability in 83% of eyes with conversion or progression of the disease to a more severe phenotype in the remaining cases. Type 3 CXLRS was a risk factor for the development of a combined retinoschisis-retinal detachment and may benefit from closer follow-up.


Asunto(s)
Predicción , Retina/patología , Retinosquisis/congénito , Tomografía de Coherencia Óptica/métodos , Agudeza Visual , Adolescente , Adulto , Niño , Progresión de la Enfermedad , Electrorretinografía , Proteínas del Ojo/genética , Proteínas del Ojo/metabolismo , Femenino , Estudios de Seguimiento , Humanos , Masculino , Mutación , Linaje , Fenotipo , Retinosquisis/diagnóstico , Retinosquisis/genética , Estudios Retrospectivos , Adulto Joven
14.
Ophthalmic Surg Lasers Imaging Retina ; 50(5): e171-e175, 2019 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-31100172

RESUMEN

Optic nerve aplasia (ONA) is a rare phenomenon characterized by absence of the optic nerve, retinal blood vessels, and retinal ganglion cells. The authors report a case of optic nerve aplasia in association with congenital cytomegalovirus (CMV) infection, conveyed through multimodal imaging including color fundus photography, fluorescein angiography, and B-scan ultrasonography. Furthermore, the authors review the common ocular findings and previously reported cases of ONA in congenital CMV infection. Screening and counseling can be performed to advise parents about the potential risks and severity of this rare clinical manifestation. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e171-e175.].


Asunto(s)
Anomalías Múltiples , Córnea/diagnóstico por imagen , Enfermedades de la Córnea/etiología , Infecciones por Citomegalovirus/complicaciones , Citomegalovirus , Enfermedades del Nervio Óptico/etiología , Nervio Óptico/diagnóstico por imagen , Enfermedades de la Córnea/diagnóstico , Infecciones por Citomegalovirus/congénito , Infecciones por Citomegalovirus/virología , Femenino , Angiografía con Fluoresceína , Fondo de Ojo , Humanos , Lactante , Enfermedades del Nervio Óptico/diagnóstico , Ultrasonografía
15.
Ophthalmol Retina ; 3(7): 597-606, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31277801

RESUMEN

PURPOSE: To evaluate the macular microvasculature in patients with familial exudative vitreoretinopathy (FEVR) using OCT angiography (OCTA) and to assess for peripheral vascular changes using widefield fluorescein angiography (WFA). DESIGN: Multicenter, retrospective, comparative, observational case series. PARTICIPANTS: We identified 411 patients with FEVR, examined between September 2014 and June 2018. Fifty-seven patients with FEVR and 60 healthy controls had OCTA images of sufficient quality for analysis. METHODS: Custom software was used to assess for layer-specific, quantitative changes in vascular density and morphologic features on OCTA by way of vessel density (VD), skeletal density (SD), fractal dimension (FD), vessel diameter index (VDI), and foveal avascular zone (FAZ). Widefield fluorescein angiography images were reviewed for peripheral vascular changes including capillary dropout, late-phase angiographic posterior and peripheral vascular leakage (LAPPEL), vascular dragging, venous-venous shunts, and arteriovenous shunts. MAIN OUTCOME MEASURES: Macular microvascular parameters on OCTA and peripheral angiographic findings on WFA. RESULTS: OCT angiography analysis of 117 patients (187 eyes; 92 FEVR patients and 95 control participants) demonstrated significantly reduced VD, SD, and FD and greater VDI in patients with FEVR compared with controls in the nonsegmented retina, superficial retinal layer (SRL), and deep retinal layer (DRL). The FAZ was larger compared with that in control eyes in the DRL (P < 0.0001), but not the SRL (P = 0.52). Subanalysis by FEVR stage showed the same microvascular changes compared with controls for all parameters. Widefield fluorescein angiography analysis of 95 eyes (53 patients) with FEVR demonstrated capillary nonperfusion in all eyes: 47 eyes (49.5%) showed LAPPEL, 32 eyes (33.7%) showed vascular dragging, 30 eyes (31.6%) had venous-venous shunts, and 33 eyes (34.7%) had arteriovenous shunts. Decreasing macular VD on OCTA correlated with increasing peripheral capillary nonperfusion on WFA. Decreasing fractal dimension on OCTA correlated with increasing LAPPEL severity on WFA. CONCLUSIONS: Patients with FEVR demonstrated abnormalities in the macular microvasculature and capillary network, in addition to the peripheral retina. The macular microvascular parameters on OCTA may serve as biomarkers of changes in the retinal periphery on WFA.


Asunto(s)
Capilares/patología , Vitreorretinopatías Exudativas Familiares/fisiopatología , Mácula Lútea/irrigación sanguínea , Vasos Retinianos/patología , Adolescente , Adulto , Capilares/diagnóstico por imagen , Niño , Preescolar , Vitreorretinopatías Exudativas Familiares/diagnóstico por imagen , Femenino , Angiografía con Fluoresceína , Humanos , Masculino , Persona de Mediana Edad , Vasos Retinianos/diagnóstico por imagen , Estudios Retrospectivos , Tomografía de Coherencia Óptica , Agudeza Visual , Adulto Joven
16.
J Surg Educ ; 76(1): 150-157, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30131282

RESUMEN

OBJECTIVES: To evaluate the impact of a comprehensive cataract surgery curriculum on the incidence of intraoperative complications. DESIGN: We retrospectively compared the total number of cataract surgeries that the residents performed in all of the teaching sites, and the incidences of intraoperative complications (anterior capsule tear, posterior capsule rent, vitreous loss, anterior vitrectomy, zonular dialysis, iris trauma, hemorrhage, dropped lens fragment, corneal wound burn, incorrect intraocular lens) for the surgeries performed at Massachusetts Eye & Ear by residents in the pre-intervention group (residents graduating in 2004 and 2005), before the implementation of a surgical curriculum, and the residents in the post-intervention group (residents graduating in 2014 and 2015). SETTING: Ophthalmology residency program at a major academic institution. PARTICIPANTS: Residents graduating in 2004, 2005, 2014, and 2015. RESULTS: We reviewed 4373 charts. 2086 of those surgeries were performed at Massachusetts Eye & Ear. The incidence of posterior capsule rent/vitreous loss/anterior vitrectomy was lower in the post-intervention group (1.4% versus 7.7%, p < 0.0001). Other complications were also lower in the post-intervention group. CONCLUSIONS: Implementation of a comprehensive cataract surgery curriculum focusing on pre-operative, intra-operative and post-operative interventions, with an emphasis on patient outcomes resulted in a decrease in the rate of intraoperative complications.


Asunto(s)
Extracción de Catarata/educación , Competencia Clínica , Curriculum , Internado y Residencia , Complicaciones Intraoperatorias/epidemiología , Complicaciones Intraoperatorias/prevención & control , Oftalmología/educación , Humanos , Incidencia , Massachusetts , Estudios Retrospectivos
17.
Ophthalmol Retina ; 3(3): 258-269, 2019 03.
Artículo en Inglés | MEDLINE | ID: mdl-31014704

RESUMEN

PURPOSE: To report the ocular and adnexal injuries sustained by patients with Thomas A. Swift's electric rifles (TASER; TASER International, Scottsdale, AZ), review the literature, and discuss the management of this complex trauma. DESIGN: Multicenter, retrospective case series and literature review. PARTICIPANTS: Seventeen eyes of 16 patients (5 eyes of 5 patients treated at 3 institutions, and 12 eyes of 11 previously reported cases). METHODS: The clinical data of 17 eyes were pooled. Spearman's correlation coefficient was used to assess the association between the extent of TASER injury and patient outcomes. MAIN OUTCOME MEASURES: Extent of TASER injury (zone of injury, penetrating vs. perforating) and association with patient outcomes (visual acuity [VA] and retinal detachment [RD]). RESULTS: In our cohort, 4 patients were transported by law enforcement and 1 was transferred from a community hospital. Four patients were taken to the operating room for TASER removal and globe repair; 1 patient underwent removal in the emergency room. Of 17 pooled cases, 12 (71%) involved open-globe injury. Of these, there was a high rate of zone 3 injuries (100%; n = 12) and a high incidence of RD (73%; 8 of 11, eviscerated eye excluded). Among patients with closed-globe injury (n = 5), 1 patient demonstrated exudative RD and 1 patient demonstrated retinal dialysis with RD. Of 10 patients with RD, 1 (10%) achieved resolution with monitoring (exudative RD); 1 (10%) underwent cryopexy and pneumatic retinopexy; 3 (30%) underwent vitrectomy, and 5 (50%) with poor prognosis did not undergo vitreoretinal surgery. In the 3 patients who underwent vitrectomy, all 3 (100%) demonstrated redetachment resulting from proliferative vitreoretinopathy and required additional surgery. Visual acuity on presentation was significantly correlated with final VA (ρ = 0.783; P = 0.02). Men (94%) were more likely than women (6%) to sustain TASER trauma. Median age was 26 years. There was a 50% rate of loss to follow-up. CONCLUSIONS: Thomas A. Swift's electric rifle injuries to the eyes or ocular adnexa represent complex trauma. Zone 3 injuries are common. The visual prognosis is guarded, and eyes may require multiple surgeries to preserve vision. Patients are at high risk for loss to follow-up by way of incarceration.


Asunto(s)
Traumatismos por Electricidad/cirugía , Lesiones Oculares Penetrantes/cirugía , Armas de Fuego , Retina/lesiones , Adolescente , Adulto , Cuerpos Extraños en el Ojo/cirugía , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Vitrectomía , Cirugía Vitreorretiniana , Adulto Joven
18.
J Cataract Refract Surg ; 44(12): 1469-1477, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30391157

RESUMEN

PURPOSE: To assess the outcomes of resident-performed cataract surgeries with iris challenges and to compare these outcomes with similar surgeries performed by attending surgeons. SETTING: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA. DESIGN: Retrospective chart review. METHODS: All cases of cataract extraction by phacoemulsification with intraocular lens implantation, performed by comprehensive ophthalmologists between January 1 and December 31, 2014, were reviewed. Cases with preoperative or intraoperative miosis, iris prolapse, and intraoperative floppy iris syndrome, were included for analysis. Visual outcomes and the rate of perioperative adverse events were compared between resident and attending surgeon cases. Factors predicting adverse events were also assessed. RESULTS: In total, 1931 eye cases of 1434 patients were reviewed, and 65 resident cases and 168 attending surgeon cases were included. The mean logarithm of the minimum angle of resolution corrected distance visual acuity was better in the resident group 1 month after surgery (0.051 ± 0.10 [SD] versus 0.132 ± 0.30, P = .03); however, the difference was eliminated when controlling for macular disease. The mean operative time was 43.8 ± 26.5 minutes and 30.9 ± 12.6 minutes for cases performed by resident surgeons and attending surgeons, respectively (P  .0001). Residents utilized supplemental pharmacologic dilation or retraction more frequently than attending surgeons (98% versus 87% of cases, P = .008). The overall rate of adverse events was no different between residents and attending surgeons (P = 0.16). Dense nuclear sclerosis predicted adverse events in cataract cases with iris challenges (adjusted odds ratio, 1.86; 95% confidence interval, 1.17-2.94; P = .001). CONCLUSION: Although requiring longer operative times and more surgical manipulation, residents who performed cataract surgeries with iris challenges achieved outcomes comparable to those performed by attending surgeons, and residents should be given the opportunity to operate on these eyes.


Asunto(s)
Educación de Postgrado en Medicina/métodos , Internado y Residencia , Iris/cirugía , Implantación de Lentes Intraoculares/educación , Oftalmología/educación , Atención Perioperativa/educación , Facoemulsificación/educación , Anciano , Anciano de 80 o más Años , Competencia Clínica , Femenino , Humanos , Masculino
19.
Am J Ophthalmol Case Rep ; 10: 77-80, 2018 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-29780921

RESUMEN

PURPOSE: To report the clinical presentation, multimodal imaging, and management of a patient with metastatic melanoma who presented with cystoid macular edema (CME). OBSERVATIONS: We report a case of a 71-year-old Caucasian male with metastatic melanoma who presented with bilateral cystoid macular edema after being on treatment with a programmed T cell death ligand 1 inhibitor, MPDL3280, for 1 year. Multimodal imaging techniques, including color fundus photographs, autofluorescence, spectral domain optical coherence tomography (OCT), fluorescein angiography (Spectralis, Heidelberg, Germany), and spectral-domain OCT angiography (Zeiss; California, USA) were performed to evaluate the etiology of his CME and to monitor his response to treatment. Clinical examination and multimodal imaging revealed 1 + chronic vitreous cells, an epiretinal membrane, and mild macular edema in both eyes. Fundus autofluorescence showed paravenous hypoautofluorescence in the right eye and scattered hypoautofluorescent spots in the left eye. Optical coherence tomography angiography (OCTA) revealed mild drop out of superficial vessels in the peri-foveal region bilaterally. These findings were concerning for melanoma-associated retinopathy, drug-related uveitis, or activation of a previous chronic autoimmune process. The patient was started on prednisone 30 mg oral daily and ketorolac tromethamine 0.5% 1 drop four times daily. He was then treated with bilateral sustained-release dexamethasone intravitreal implants (Ozurdex). He had complete resolution of CME, and was tapered off of oral steroids within 6 weeks. CONCLUSIONS AND IMPORTANCE: Melanoma-associated retinopathy can be accompanied by CME, which presents a diagnostic and therapeutic dilemma in cases where a new drug has been recently initiated. By treating the condition locally, the ophthalmologist may be able to taper systemic immunosuppression more quickly.

20.
J Glaucoma ; 26(6): e180-e186, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-28221332

RESUMEN

PURPOSE: To evaluate the clinical characteristics of a patient with primary open-angle glaucoma in whom macular retinoschisis resolved completely after trabeculectomy consistently lowered intraocular pressure (IOP). METHODS: A single case report. RESULTS: We report a case of retinoschisis involving the macula in a patient with primary open-angle glaucoma in the absence of myopic maculopathy, optic nerve anomaly, or x-linked retinoschisis. The patient's glaucoma was associated with progressive visual field loss in the setting of IOP fluctuations related to posture. A trabeculectomy reduced IOP and posture-related IOP fluctuations with subsequent resolution of macular retinoschisis. In the 1-year postoperative period following trabeculectomy, the patient has remained without retinoschisis and visual fields have been stable. CONCLUSIONS: Improved IOP control resulting in resolution of retinoschisis may distinguish retinoschisis associated with glaucoma from other forms of retinoschisis.


Asunto(s)
Glaucoma de Ángulo Abierto/cirugía , Retinosquisis/cirugía , Trabeculectomía , Humanos , Presión Intraocular , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
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