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This case report describes a diagnosis of combined hamartoma of the retina and retinal pigment epithelium (RPE) with filamentous RPE hyperplasia in a female child with a history of amblyopia, myopia, and exotropia of the affected eye.
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Hamartoma , Hiperplasia , Doenças Retinianas , Epitélio Pigmentado da Retina , Tomografia de Coerência Óptica , Humanos , Angiofluoresceinografia/métodos , Hamartoma/diagnóstico , Doenças Retinianas/diagnóstico , Epitélio Pigmentado da Retina/patologiaRESUMO
Purpose: Advancements in retinal imaging have augmented our understanding of the pathology and structure-function relationships of retinal disease. No single diagnostic test is sufficient; rather, diagnostic and management strategies increasingly involve the synthesis of multiple imaging modalities. Methods: This literature review and editorial offer practical clinical guidelines for how the retina specialist can use multimodal imaging to manage retinal conditions. Results: Various imaging modalities offer information on different aspects of retinal structure and function. For example, optical coherence tomography (OCT) and B-scan ultrasonography can provide insights into the microstructural anatomy; fluorescein angiography (FA), indocyanine green angiography (ICGA), and OCT angiography (OCTA) can reveal vascular integrity and perfusion status; and near-infrared reflectance and fundus autofluorescence (FAF) can characterize molecular components within tissues. Managing retinal vascular diseases often includes fundus photography, OCT, OCTA, and FA to evaluate for macular edema, retinal ischemia, and the secondary complications of neovascularization (NV). OCT and FAF play a key role in diagnosing and treating maculopathies. FA, OCTA, and ICGA can help identify macular NV, posterior uveitis, and choroidal venous insufficiency, which guides treatment strategies. Finally, OCT and B-scan ultrasonography can help with preoperative planning and prognostication in vitreoretinal surgical conditions. Conclusions: Today, the retina specialist has access to numerous retinal imaging modalities that can augment the clinical examination to help diagnose and manage retinal conditions. Understanding the capabilities and limitations of each modality is critical to maximizing its clinical utility.
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This report describes a unique case of a Coats-like presentation of familial exudative vitreoretinopathy in an 11-year-old girl. The patient was originally referred for evaluation of presumed Coats disease and presented with telangiectatic vessels, perivascular exudates, diffuse peripheral exudation, and intraretinal hemorrhages. Clinical and angiographical findings were consistent with familial exudative vitreoretinopathy, while genetic testing identified variants of uncertain significance in two associated genes, LRP5 and ZNF408. In silico analysis predicts the LRP5 variant to be pathogenic. Retinal vasculopathies often have phenotypic overlap, warranting angiographic examination of both eyes and genetic testing to uncover the correct diagnosis and guide proper treatment. [Ophthalmic Surg Lasers Imaging Retina 2024;55:462-466.].
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Vitreorretinopatias Exsudativas Familiares , Angiofluoresceinografia , Proteína-5 Relacionada a Receptor de Lipoproteína de Baixa Densidade , Humanos , Feminino , Vitreorretinopatias Exsudativas Familiares/diagnóstico , Criança , Proteína-5 Relacionada a Receptor de Lipoproteína de Baixa Densidade/genética , Angiofluoresceinografia/métodos , Telangiectasia Retiniana/diagnóstico , Telangiectasia Retiniana/genética , Tomografia de Coerência Óptica/métodos , Fundo de Olho , Mutação , Oftalmopatias Hereditárias/diagnóstico , Oftalmopatias Hereditárias/genética , Linhagem , Proteínas de Ligação a DNA , Fatores de TranscriçãoRESUMO
PURPOSE: To report the clinical presentation and outcomes in patients who underwent surgery for proliferative sickle cell retinopathy (PSCR). DESIGN: Retrospective, consecutive case series. SUBJECTS: All patients who underwent vitreoretinal surgery for complications secondary to PSCR between January 1, 2014, and December 31, 2021, at a university referral center. METHODS: Retrospective consecutive case series. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), single operation anatomic success rate. RESULTS: The study included 65 eyes of 61 patients. Disease distribution included 24 (44.4%) eyes with hemoglobin SC disease, 14 (25.9%) with hemoglobin SS disease, 13 (24.1%) with sickle cell trait, and 3 (5.6%) with sickle cell-ß thalassemia. Preoperative transfusion was not performed in any study patients. Regional anesthesia with monitored anesthesia care (RA-MAC) was utilized in 58 (89.2%) eyes and general anesthesia in 7 (10.8%). In eyes that underwent surgery for retinal detachment (RD; N = 52) the rate of single operation anatomic success was 72.4% with combined scleral buckling/pars plana vitrectomy (SB/PPV; N = 29) compared with 47.8% with PPV alone (N = 23; P = 0.07). Mean BCVA at the last follow-up examination was 1.27 (20/372) in the SB/PPV group and 1.05 (20/226) in the PPV group (P = 0.48). In all SB cases, an encircling band was utilized and there were no known cases of anterior segment ischemia. All eyes that had surgery for vitreous hemorrhage (N = 13) underwent PPV with endolaser and mean BCVA improved from 1.67 (20/944) preoperatively to 0.45 (20/56) at last follow-up examination (P < 0.001). Mean preoperative BCVA, indication for surgery, single operation success rate, and mean BCVA at last follow-up examination did not differ based on sickle cell disease type (P > 0.05). CONCLUSIONS: In patients with RD, SB/PPV achieved slightly higher rates of single operation anatomic success compared with PPV alone. Visual acuity outcomes were similar in the 2 groups. The majority of patients received RA-MAC anesthesia and preoperative transfusions were not performed. There were no cases of postoperative anterior segment ischemia. Hemoglobin SC disease was the most common disease type in the current study and surgical outcomes did not differ between sickle cell disease types. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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Anemia Falciforme , Acuidade Visual , Cirurgia Vitreorretiniana , Humanos , Masculino , Estudos Retrospectivos , Feminino , Anemia Falciforme/complicações , Adulto , Cirurgia Vitreorretiniana/métodos , Pessoa de Meia-Idade , Seguimentos , Resultado do Tratamento , Adulto Jovem , Doenças Retinianas/etiologia , Doenças Retinianas/diagnóstico , Doenças Retinianas/cirurgia , Adolescente , Idoso , Vitrectomia/métodosRESUMO
A 37-week-old girl underwent ophthalmic examination. Born at 32 weeks, the infant weighed 680 grams and received high-flow nasal cannula for respiratory distress of the newborn. Dilated fundus examination of the right eye revealed an atypical chorioretinal coloboma; the left eye revealed hyperpigmentary changes in the macula. Fluorescein angiography of both eyes showed retinal vascularization to zone II. Genetic testing revealed a heterozygous variant of uncertain significance in the catenin Alpha 1 (CTNNA1) gene. CTNNA1 gene abnormalities have been implicated as causes of familial exudative vitreoretinopathy (FEVR). It is important to recognize possible simultaneous retinopathy of prematurity and FEVR. [Ophthalmic Surg Lasers Imaging Retina 2024;55:285-288.].
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Corioide , Coloboma , Angiofluoresceinografia , Retinopatia da Prematuridade , Humanos , Feminino , Coloboma/diagnóstico , Coloboma/genética , Retinopatia da Prematuridade/diagnóstico , Corioide/anormalidades , Angiofluoresceinografia/métodos , Recém-Nascido Prematuro , Recém-Nascido , Fundo de Olho , Idade Gestacional , Vitreorretinopatias Exsudativas Familiares/diagnósticoRESUMO
BACKGROUND: Choroidal neovascularization (CNV) is a rare complication of choroideremia that occurs secondary to relative atrophy of the retinal pigment epithelium and eventual rupture of Bruch's membrane. The ideal management of CNV in choroideremia is unclear. MATERIALS AND METHODS: Case report. OBSERVATIONS: A 14-year-old male with no known ocular history presented to the eye emergency department complaining of a central scotoma in the right eye for 4 days. He had no past medical history and family history was unremarkable for known ocular disease. Visual acuity was 20/70 in the right eye and 20/30 in the left eye. Posterior segment exam revealed chorioretinal atrophy extending from the outer macula to the midperiphery in both eyes. There was CNV with associated subretinal hemorrhage in the right eye. Optical coherence tomography demonstrated the presence of CNV with subretinal fluid in the right eye and parafoveal outer retinal atrophy in both eyes. Genetic testing revealed a hemizygous exon 2 deletion on the CHM gene, pathogenic for choroideremia. The patient received a total of 3 injections 4 weeks apart followed by 1 injection 6 weeks later with resolution of the subretinal hemorrhage and reduction in CNV size with improvement in visual acuity to 20/20 at last follow-up exam. CONCLUSIONS AND IMPORTANCE: Choroidal neovascularization is a rare cause of central vision loss in patients with choroideremia. In this report, we demonstrate a good functional and anatomic response to intravitreal bevacizumab in a 14-year-old patient with undiagnosed choroideremia who presented with CNV-induced central vision loss.
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Neovascularização de Coroide , Coroideremia , Masculino , Humanos , Adolescente , Inibidores da Angiogênese/uso terapêutico , Coroideremia/complicações , Coroideremia/diagnóstico , Coroideremia/genética , Injeções Intravítreas , Bevacizumab/uso terapêutico , Neovascularização de Coroide/diagnóstico , Neovascularização de Coroide/tratamento farmacológico , Neovascularização de Coroide/etiologia , Transtornos da Visão , Hemorragia Retiniana/diagnóstico , Hemorragia Retiniana/etiologia , Tomografia de Coerência Óptica , Atrofia/complicações , AngiofluoresceinografiaRESUMO
Purpose: To evaluate the visual outcomes with unexplained vision loss during or after silicone oil (SO) tamponade. Methods: This multicenter retrospective case series comprised patients with unexplained vision loss associated with SO tamponade or its removal. Eyes with other clear secondary identifiable causes of vision loss were excluded. Results: Twenty-nine eyes of 28 patients (64% male) were identified. The mean age was 50 ± 13 years (range, 13-78 years). The mean duration of SO tamponade was 148 ± 38 days. Eighteen eyes (62%) developed unexplained vision loss while under SO; 11 (38%) had vision loss after SO removal. The most common optical coherence tomography (OCT) finding was ganglion cell layer (GCL) thinning (55%). Eyes with vision loss after SO removal had a mean logMAR best-corrected visual acuity (BCVA) of 0.6 ± 0.7 (Snellen 20/85) before SO tamponade and 1.2 ± 0.4 (20/340) before SO removal. By the last follow-up after SO removal, the BCVA had improved to 1.1 ± 0.4 (20/235). In eyes with vision loss after SO removal, the BCVA before SO removal was 0.7 ± 0.7 (20/104), which deteriorated to 1.4 ± 0.4 (20/458) 1 month after SO removal. By the last follow-up, the BCVA had improved to 1.0 ± 0.5 (20/219). Conclusions: Unexplained vision loss can occur during SO tamponade or after SO removal. Vision loss was associated with 1000-centistoke and 5000-centistoke oil and occurred in macula-off and macula-on retinal detachments. The duration of tamponade was 3 months or longer in the majority of eyes. Most eyes had GCL thinning on OCT. Gradual visual recovery can occur yet is often incomplete.
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BACKGROUND AND OBJECTIVE: To describe the multimodal imaging and histopathological features of patients with dragged optic disc vessels (DODV). PATIENTS AND METHODS: This is a retrospective, observational analysis using multimodal imaging of eyes with DODV in patients with retinal vascular diseases including familial exudative vitreoretinopathy, inflammatory disease, and others. In addition, two additional enucleated eyes with DODV underwent histopathological analysis. RESULTS: Of the 13 patients, eight were girls and five were boys. Mean age was 5 years (ranging from 4 months to 10 years old). Of the 15 eyes, 12 (80%) demonstrated temporal dragging and three (20%) had nasal dragging. Retinal vascular abnormalities were present in 11 of the fellow eyes. Multimodal imaging demonstrated features of DODV including direction of traction, outer retinal thickening, increased flow, and other features. Only two (13.3%) eyes required surgical intervention. Mean follow-up was 14 (range 3 to 30 months) months. At last follow-up all eyes remained stable. Last visual acuity ranged from 20/100 to counting fingers. As well, two enucleated globes of adults with retinopathy of prematurity underwent histopathologic evaluation, showing optic nerve fibers that extended from the optic nerve into the DODV. CONCLUSIONS: DODV is a sign of various late-stage retinal vascular diseases, associated with poor visual function. Multimodal imaging and histopathology can assist in understanding the disease pathology. [Ophthalmic Surg Lasers Imaging Retina 2023;54:634-642.].
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Disco Óptico , Doenças Retinianas , Doenças Vasculares , Masculino , Adulto , Feminino , Recém-Nascido , Humanos , Pré-Escolar , Disco Óptico/patologia , Doenças Retinianas/patologia , Retina/patologia , Imagem Multimodal , Doenças Vasculares/patologia , Estudos RetrospectivosRESUMO
BACKGROUND AND OBJECTIVE: Retinal detachments (RDs) are a complication of X-linked retinoschisis (XLRS) with a poor prognosis. This study aims to report outcomes of XLRS-RD repair in pediatric patients. MATERIALS AND METHODS: The study is a retrospective analysis of pediatric patients undergoing vitreoretinal surgery for XLRS-RDs from 2000 to 2022. RESULTS: Nine patients (11 eyes) met inclusion criteria. All patients were boys, with a mean age of 8 years. Most RDs arose inferiorly (64%). Seven (64%) detachments were macula-involving. Seven (64%) detachments were repaired with combined scleral buckling and vitrectomy, two (18%) detachments were repaired with vitrectomy alone, one (9%) detachment underwent a primary scleral buckling procedure, and one (9%) asymptomatic detachment with a pigment demarcation line was observed. Silicone oil was used in eight of nine (89%) eyes undergoing vitrectomy. Final visual acuity was 20/200 or better in eight (73%) eyes. One eye that underwent surgical intervention remained unattached at follow-up. CONCLUSION: Surgical repair in patients with XLRSRDs was associated with successful reattachment, although visual prognosis varied. [Ophthalmic Surg Lasers Imaging Retina 2023;54:574-579.].
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Macula Lutea , Descolamento Retiniano , Retinosquise , Masculino , Humanos , Criança , Feminino , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , Retinosquise/diagnóstico , Retinosquise/etiologia , Retinosquise/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Recurvamento da Esclera/métodos , Vitrectomia/métodosRESUMO
We report the case of a 28-year-old man with X-linked retinoschisis (XLRS) and type I diabetes mellitus. The patient had bilateral foveoschisis with a tractional retinal fold in the right eye. Optical coherence tomography (OCT) revealed hyperreflective material within the inner nuclear and outer plexiform layers, photoreceptor atrophy, and retinal pigment epithelium irregularities in both eyes. Fluorescein angiography showed hyperfluorescent foveal spots corresponding to the hyperreflective material observed on OCT. This is a unique presentation of XLRS, with concurrent foveoschisis and photoreceptor atrophy in both eyes. The hyperreflective material on OCT serves as a distinctive feature of XLRS. [Ophthalmic Surg Lasers Imaging Retina 2023;54:603-606.].
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Diabetes Mellitus Tipo 1 , Retinosquise , Masculino , Humanos , Adulto , Retinosquise/diagnóstico , Retinosquise/etiologia , Retinosquise/patologia , Diabetes Mellitus Tipo 1/patologia , Retina/patologia , Fóvea Central/patologia , Tomografia de Coerência Óptica/métodos , Atrofia/patologiaRESUMO
BACKGROUND AND OBJECTIVE: Inducing a posterior vitreous detachment (PVD) may be challenging, especially in pediatric patients with firm vitreo-retinal adherence. This case report will present an alternative method of PVD induction using adjunctive perfluorooctane (PFO). MATERIALS AND METHODS: An 11-year-old boy underwent scleral buckle placement and 23-gauge pars plana vitrectomy for macula-off retinal detachment. Triamcinolone acetonide was used for vitreous staining. A flexible loop was used to peel the hyaloid membrane from attached retina nasal to the optic nerve. A small hole was created in the posterior hyaloid, and a small PFO bubble was injected under the detached vitreous to further dissect it from the retina and propagate the vitreous detachment anteriorly. The vitrector was used to lift and complete the PVD peripherally. RESULTS: PVD induction was obtained with no iatrogenic retinal breaks or postoperative complications. CONCLUSION: PVD induction using a stepwise PFO approach is a safe and effective alternative to conventional techniques. [Ophthalmic Surg Lasers Imaging Retina 2023;54:543-546.].
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Fluorocarbonos , Descolamento do Vítreo , Masculino , Humanos , Criança , Dissecação , Retina , Fluorocarbonos/efeitos adversosRESUMO
OBJECTIVE: To report the management and outcomes of vitreous hemorrhage in pediatric patients with X-linked retinoschisis (XLRS). MATERIALS AND METHODS: Retrospective case series of pediatric patients with XLRS with vitreous hemorrhage between January 2000 and January 2022 at the Bascom Palmer Eye Institute. RESULTS: Nine patients (12 eyes) met inclusion criteria. The average age at presentation was 5.75 years and mean follow-up time was 6 years. All eyes (58.3%) that underwent fluorescein angiography exhibited peripheral capillary dropout. Six of 12 eyes (50%) were observed without intervention. Four of 12 eyes (33.3%) underwent intravitreal injection of bevacizumab and 2 (16.6%) underwent vitreoretinal surgery. Seven of 12 eyes (58.3%) had documented recurrence of vitreous hemorrhage during follow-up. All retinas were attached and had clear media at final follow-up. CONCLUSION: Fluorescein angiography is a helpful tool to evaluate the vascular phenotype in XLRS. Favorable anatomic outcomes were noted in this cohort, but recurrence of vitreous hemorrhage was common. [Ophthalmic Surg Lasers Imaging Retina 2023;54:513-518.].
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Retinosquise , Criança , Humanos , Bevacizumab , Retina , Retinosquise/diagnóstico , Estudos Retrospectivos , Hemorragia Vítrea/diagnóstico , Hemorragia Vítrea/etiologiaRESUMO
PURPOSE: To report the surgical approaches and outcomes in patients undergoing surgery for retinal detachment associated with retinal dialysis. DESIGN: Retrospective, consecutive case series. SUBJECTS: All patients who underwent surgery for retinal detachment secondary to retinal dialysis between January 1, 2012, and January 1, 2022. METHODS: Retrospective consecutive case series. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), single-operation success rate. RESULTS: The study cohort included 60 eyes of 58 patients with a mean age of 26.4 (standard deviation, 13.0) years. Males comprised 49 (84.5%) patients. Known trauma occurred in 35 (61.4%) cases. Initial surgical management included scleral buckling (SB) in 49 (81.7%) eyes and combined SB and pars plana vitrectomy (PPV) in 11 (18.3%) eyes. Preoperative BCVA correlated with BCVA at last follow-up visit (r = 0.66; P < 0.001). At last visit, the SB group had a mean logarithm of the minimum angle of resolution BCVA of 0.36 (20/46) and a single-operation success rate of 76.9% at 6 months, whereas the SB/PPV group had a mean logarithm of the minimum angle of resolution BCVA of 1.08 (20/238) and single-operation success rate of 77.8% (P = 0.04 and P = 0.96, respectively). Six eyes in the SB/PPV group received silicone oil tamponade. In eyes with at least 1 year of follow-up, 4 (14.8%) in the SB group and 6 (100%) in the SB/PPV group developed a visually significant cataract requiring cataract surgery (P < 0.001). CONCLUSIONS: Retinal detachment associated with retinal dialysis is typically associated with trauma and more often occurs in young male patients. The current study confirms that SB without PPV is an effective initial treatment strategy for most patients with retinal dialysis and has a low rate of cataract formation. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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Catarata , Descolamento Retiniano , Perfurações Retinianas , Humanos , Masculino , Adulto , Feminino , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , Perfurações Retinianas/diagnóstico , Perfurações Retinianas/etiologia , Perfurações Retinianas/cirurgia , Estudos Retrospectivos , Acuidade Visual , Vitrectomia , Catarata/complicações , Catarata/diagnósticoRESUMO
Purpose: To evaluate the outcomes and complications in patients with retained lens fragments (RLF) after cataract surgery, comparing those who received pars plana vitrectomy (PPV) on the same day, within 1 week, or later than 1 week after cataract surgery. Patients and Methods: Retrospective case series of all patients who underwent PPV for RLF at Bascom Palmer Eye Institute between January 1, 2012 and September 30, 2020. Individuals with less than 3 months of follow-up, chronic uveitis, congenital cataract, previtrectomy retinal detachment (RD), and severe trauma were excluded. All analyses for categorical and binary variables used chi-squared tests. Analyses for continuous variables were performed using multivariate analyses of covariance, adjusting for differences in baseline visual acuity before cataract surgery. Results: The study included 246 eyes of 246 patients. The timing distribution included the following: 140 (57%) eyes underwent same-day PPV, 33 (13%) eyes underwent same-week PPV, and 73 (30%) eyes underwent PPV after 1 week (up to 90 days). When all eyes were included in analysis, there were no statistically significant differences in mean best-corrected visual acuity (BCVA) between groups at last follow-up examination (P = 0.07). When only eyes without known pre-existing ocular disease (N = 157) were included in analysis, there were no differences in mean BCVA between groups at all postoperative timepoints (P > 0.05). The rate of RD did not differ between groups when eyes with and without pre-existing ocular disease were analyzed (P > 0.05). Conclusion: In the current study, there were no statistically significant differences in postoperative BCVA or rates of RD at last follow-up examination in eyes undergoing PPV for RLF on the same day, within 1 week, or later than 1 week after cataract surgery.
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PURPOSE: To report on the feasibility of 27-gauge (G) vitrectomy for pediatric patients. METHODS: This study is an international, multicenter, retrospective, interventional case series. Participants were patients 17 years or younger who underwent 27-G vitrectomy for various indications. RESULTS: The records of 56 eyes from 47 patients were reviewed. Mean age was 5.7 ± 5.2 years. Diagnoses included retinopathy of prematurity (Stages 3 with vitreous hemorrhage, 4A, 4B, and 5), Terson's syndrome, traumatic macular hole, posterior capsular opacification, endophthalmitis, and others. Instruments used were the 27-G infusion, 27-G vitreous cutter, 27-G light pipe, and 27-G internal limiting membrane forceps. Instrument bending was noted in one (1.8%) case. There were no cases with intraoperative complications, infusion issues, or postoperative endophthalmitis. There were 67/145 (46%) sclerotomies that required suturing, of which most (51/145) were sutured out of precaution. There were four cases (7.1%) that required conversion to a larger gauge and three cases (5.3%) that developed postoperative hypotony. Mean visual acuity improved from logarithm of the minimum angle of resolution 1.32 (20/420) to 0.72 (20/105), after a mean follow-up of 125.1 days (P = 0.01). Anatomic success was achieved in 96.4% of eyes after a single surgery. CONCLUSION: Twenty-seven-gauge vitrectomy was safe and feasible in selected pediatric vitreoretinopathies. Further studies are warranted to examine indications and outcomes.
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Endoftalmite , Degeneração Retiniana , Cirurgia Vitreorretiniana , Recém-Nascido , Humanos , Criança , Lactente , Pré-Escolar , Vitrectomia , Estudos Retrospectivos , Resultado do Tratamento , Hemorragia Vítrea/cirurgia , Endoftalmite/etiologia , Endoftalmite/cirurgia , Retina , Complicações Pós-Operatórias/cirurgia , Degeneração Retiniana/cirurgiaRESUMO
Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disorder caused by mutations in the 7-dehydrocholesterol reductase (DHCR7) gene, located on chromosomal region 11q13. This results in reduced cholesterol and increased 7-dehydrocholesterol (7DHC) levels. Accumulation of 7DHC in patients with SLOS can affect multiple organs and display a broad phenotypic expression. Ophthalmic abnormalities related to SLOS are variable but the most common is blepharoptosis. Over 50% of these patients present with self-injurious behavior, such as head banging, which can result in ocular complications and blindness. We report the first case of peripheral avascularity of the retina in a patient with SLOS. Physicians should be aware of the potential ocular complications associated with SLOS and confounding factors, such as prematurity, given that referral is usually delayed due to the lack of awareness of these potentially blinding associations. This case highlights the importance of early referral and continuous ophthalmologic follow-up in preventing further deterioration of visual development and complications that can lead to blindness.
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Oftalmologia , Síndrome de Smith-Lemli-Opitz , Humanos , Síndrome de Smith-Lemli-Opitz/diagnóstico , Síndrome de Smith-Lemli-Opitz/genética , Síndrome de Smith-Lemli-Opitz/metabolismo , Seguimentos , Colesterol/metabolismo , CegueiraRESUMO
PURPOSE: To compare the types and dosages of anti-vascular endothelial growth factors (VEGFs) to ascertain whether specific dosages or types of injection were associated with retreatment in clinical practice in the United States. DESIGN: Multicenter, retrospective, consecutive series. PARTICIPANTS: Patients with retinopathy of prematurity (ROP) treated with anti-VEGF injections from 2007 to 2021. METHODS: Sixteen sites from the United States participated. Data collected included demographics, birth characteristics, examination findings, type and dose of anti-VEGF treatment, retreatment rates, and time to retreatment. Comparisons of retreatment rates between bevacizumab and ranibizumab intravitreal injections were made. MAIN OUTCOME MEASURES: Relative rate of retreatment between varying types of anti-VEGF therapy, including bevacizumab and ranibizumab, and the various dosages used for each. RESULTS: Data from 873 eyes of 661 patients (61% male and 39% female) were collected. After exclusion of 40 eyes treated with laser before anti-VEGF injection and 266 eyes re-treated with laser at or beyond 8 weeks after the initial anti-VEGF treatment, 567 eyes of 307 patients (63% male and 37% female) remained and were included in the primary analysis. There was no difference between the no retreatment and retreatment groups in terms of birthweight, gestational age, age at first injection, ROP stages, or number of involved clock hours. The retreatment group had a larger percentage of aggressive ROP (34% vs. 18%, P < 0.001) and greater percentage of zone 1 ROP (49 vs. 34%, P = 0.001) than the no retreatment group. Ranibizumab use was associated with a higher rate of retreatment than bevacizumab use (58% vs. 37%, P < 0.001), whereas the rate of retreatment was not associated with a specific dose of ranibizumab (R2 = 0.67, P = 0.32). Meanwhile, lower doses of bevacizumab were associated with higher rates of retreatment compared with the higher doses (R2 = 0.84, P = 0.01). There was a dose-specific trend with higher doses trending toward lower retreatments for bevacizumab. CONCLUSIONS: In a multicenter study of ROP patients initially treated with anti-VEGF therapy, ranibizumab and lower-dose bevacizumab use were associated with an increased rate of retreatment when compared with higher-dose bevacizumab. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.
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Ranibizumab , Retinopatia da Prematuridade , Recém-Nascido , Humanos , Masculino , Feminino , Bevacizumab/uso terapêutico , Ranibizumab/uso terapêutico , Inibidores da Angiogênese/uso terapêutico , Retinopatia da Prematuridade/tratamento farmacológico , Retinopatia da Prematuridade/diagnóstico , Injeções Intravítreas , Estudos Retrospectivos , Fator A de Crescimento do Endotélio Vascular , Idade GestacionalRESUMO
Objective: The purpose of the current study is to report outcomes of suprachoroidal hemorrhage (SCH) after anterior segment surgery at a single institution, and to identify clinical features associated with visual prognosis. Methods and Analysis: Retrospective consecutive case series of patients with SCH occurring after anterior segment surgery. Results: The study includes 112 eyes of 112 patients between 2014 and 2020. There were 76 cases of non-appositional SCH versus 36 cases of appositional SCH. The mean presenting visual acuity for patients with non-appositional versus appositional SCH was 2.03 logMAR (SD 0.78) versus 2.39 logMAR (SD 0.43), respectively. Visual acuity outcomes generally remained poor at last follow-up: 64 (58%) patients had a visual acuity (VA) of ≤ 20/200, including 19 (17%) with light perception (LP), and 11 (10%) with no light perception (NLP). Regarding management of non-appositional versus appositional SCH, observation was selected in 46 (61%) vs 12 (33%), delayed drainage in 14 (18%) vs 15 (42%), delayed pars plana vitrectomy in 16 (21%) vs 13 (36%), and VA at last follow-up was 1.2 versus 1.86 logMAR (p=0.002). In patients that were observed, both appositional SCH (p=0.01) and duration of apposition (p=0.04) were correlated with worse outcome. Conclusion: Appositional SCH was associated with poorer visual outcomes compared to non-appositional SCH. Observation remains a reasonable management strategy for non-appositional SCH.
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Importance: Literature and anecdotal evidence suggest a relationship between male sex and retinopathy of prematurity (ROP). It is not known whether a difference, if present, is sex-related pathophysiologic predisposition or sex difference in meeting ROP screening criteria. Objective: To evaluate the association of sex with the development of treatment-warranted ROP. Data Sources: PubMed, Embase, and Web of Science databases were searched from 2000 to 2022. The search strategy used keywords including retinopathy of prematurity or ROP or retrolental fibroplasia and treatment or anti-VEGF or bevacizumab or ranibizumab or aflibercept or conbercept or laser or cryotherapy and gender or sex or male or female and medical subject headings terms. Study Selection: All studies reporting on treatment with anti-vascular endothelial growth factor, laser photocoagulation, and/or cryotherapy for ROP were identified. Studies reporting sex distribution in the treatment group were included in the meta-analysis. Exclusion criteria included case reports, case series of fewer than 10 treated patients, systematic reviews, conference abstracts, letters to the editor, animal studies, and non-English records. Data Extraction and Synthesis: Two reviewers independently screened and extracted the data following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The proportions of treated male and female infants were combined using random-effects meta-analysis. Main Outcomes and Measures: Numbers and percentages of male and female infants treated for ROP. Results: Of 11â¯368 identified studies, 316 met inclusion criteria, yielding a total of 31â¯026 treated patients. A higher percentage of male infants were treated for ROP (55% [95% CI, 0.54%-0.55%]), with low heterogeneity between studies (I2 = 34%; P < .001). Thirty-eight studies reported sex distribution in the screened population (170â¯053 patients; 92â¯612 [53%] male vs 77â¯441 [47%] female). There was no significant difference in the odds of receiving treatment between screened male and female infants (pooled odds ratio, 1.04 [95% CI, 0.91-1.18]; P = .67). Conclusions and Relevance: More male infants are treated for ROP than female infants. This could be due to a known relative pathophysiological fragility of preterm male infants in addition to a difference in ROP screening rates, with more male infants meeting the criteria than female infants. These findings have implications for future studies and may prompt more careful clinical monitoring of male neonates.