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AIM: To investigate whether the American Joint Committee on Cancer (AJCC) clinical category cT2b needs to be subclassified by the type and distribution of retinoblastoma (RB) seeding. METHODS: Multicentre, international registry-based data were collected from RB centres enrolled between January 2001 and December 2013. 1054 RB eyes with vitreous or subretinal seeds from 18 ophthalmic oncology centres, in 13 countries within six continents were analysed. Local treatment failure was defined as the use of secondary enucleation or external beam radiation therapy (EBRT) and was estimated with the Kaplan-Meier method. RESULTS: Clinical category cT2b included 1054 eyes. Median age at presentation was 16.0 months. Of these, 428 (40.6%) eyes were salvaged, and 430 (40.8%) were treated with primary and 196 (18.6%) with secondary enucleation. Of the 592 eyes that had complete data for globe salvage analysis, the distribution of seeds was focal in 143 (24.2%) and diffuse in 449 (75.8%). The 5-year Kaplan-Meier cumulative globe-salvage (without EBRT) was 78% and 49% for eyes with focal and diffuse RB seeding, respectively. Cox proportional hazards regression analysis confirmed a higher local treatment failure risk with diffuse seeds as compared with focal seeds (hazard rate: 2.8; p<0.001). There was insufficient evidence to prove or disprove an association between vitreous seed type and local treatment failure risk(p=0.06). CONCLUSION: This international, multicentre, registry-based analysis of RB eyes affirmed that eyes with diffuse intraocular distribution of RB seeds at diagnosis had a higher risk of local treatment failure when compared with focal seeds. Subclassification of AJCC RB category cT2b into focal vs diffuse seeds will improve prognostication for eye salvage.
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Neoplasias da Retina , Retinoblastoma , Humanos , Lactente , Retinoblastoma/diagnóstico , Retinoblastoma/radioterapia , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/radioterapia , Inoculação de Neoplasia , Corpo Vítreo , Falha de Tratamento , Estudos RetrospectivosRESUMO
PURPOSE: To evaluate presenting features, tumor size, and treatment methods for risk of metastatic death due to advanced intraocular retinoblastoma (RB). DESIGN: International, multicenter, registry-based retrospective case series. PARTICIPANTS: A total of 1841 patients with advanced RB. METHODS: Advanced RB was defined by 8th edition American Joint Committee on Cancer (AJCC) categories cT2 and cT3 and new AJCC-Ophthalmic Oncology Task Force (OOTF) Size Groups (1: < 50% of globe volume, 2: > 50% but < 2/3, 3: > 2/3, and 4: diffuse infiltrating RB). Treatments were primary enucleation, systemic chemotherapy with secondary enucleation, and systemic chemotherapy with eye salvage. MAIN OUTCOME MEASURES: Metastatic death. RESULTS: The 5-year Kaplan-Meier cumulative survival estimates by patient-level AJCC clinical subcategories were 98% for cT2a, 96% for cT2b, 88% for cT3a, 95% for cT3b, 92% for cT3c, 84% for cT3d, and 75% for cT3e RB. Survival estimates by treatment modality were 96% for primary enucleation, 89% for systemic chemotherapy and secondary enucleation, and 90% for systemic chemotherapy with eye salvage. Risk of metastatic mortality increased with increasing cT subcategory (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of metastatic mortality in categories cT3c (glaucoma, hazard ratio [HR], 4.9; P = 0.011), cT3d (intraocular hemorrhage, HR, 14.0; P < 0.001), and cT3e (orbital cellulitis, HR, 19.6; P < 0.001) than in category cT2a and with systemic chemotherapy with secondary enucleation (HR, 3.3; P < 0.001) and eye salvage (HR, 4.9; P < 0.001) than with primary enucleation. The 5-year Kaplan-Meier cumulative survival estimates by AJCC-OOTF Size Groups 1 to 4 were 99%, 96%, 94%, and 83%, respectively. Mortality from metastatic RB increased with increasing Size Group (P < 0.001). Cox proportional hazards regression analysis revealed that patients with Size Group 3 (HR, 10.0; P = 0.002) and 4 (HR, 41.1; P < 0.001) had a greater risk of metastatic mortality than Size Group 1. CONCLUSIONS: The AJCC-RB cT2 and cT3 subcategories and size-based AJCC-OOTF Groups 3 (> 2/3 globe volume) and 4 (diffuse infiltrating RB) provided a robust stratification of clinical risk for metastatic death in advanced intraocular RB. Primary enucleation offered the highest survival rates for patients with advanced intraocular RB.
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Neoplasias da Retina , Retinoblastoma , Enucleação Ocular , Humanos , Lactente , Sistema de Registros , Neoplasias da Retina/tratamento farmacológico , Neoplasias da Retina/patologia , Retinoblastoma/tratamento farmacológico , Retinoblastoma/patologia , Estudos RetrospectivosRESUMO
PURPOSE: To determine the value of clinical features for advanced intraocular retinoblastoma as defined by the eighth edition of the American Joint Committee on Cancer (AJCC) cT3 category and AJCC Ophthalmic Oncology Task Force (OOTF) Size Groups to predict the high-risk pathologic features. DESIGN: International, multicenter, registry-based retrospective case series. PARTICIPANTS: Eighteen ophthalmic oncology centers from 13 countries over 6 continents shared evaluations of 942 eyes enucleated as primary treatment for AJCC cT3 and, for comparison, cT2 retinoblastoma. METHODS: International, multicenter, registry-based data were pooled from patients enrolled between 2001 and 2013. High-risk pathologic features were defined as AJCC categories pT3 and pT4. In addition, AJCC OOTF Size Groups were defined as follows: (1) less than half, (2) more than half but less than two thirds, (3) more than two thirds of globe volume involved, and (4) diffuse infiltrating retinoblastoma. MAIN OUTCOME MEASURES: Statistical risk of high-risk pathologic features corresponding to AJCC cT3 subcategories and AJCC OOTF Size Groups. RESULTS: Of 942 retinoblastoma eyes treated by primary enucleation, 282 (30%) showed high-risk pathologic features. Both cT subcategories and AJCC OOTF Size Groups (P < 0.001 for both) were associated with high-risk pathologic features. On logistic regression analysis, cT3c (iris neovascularization with glaucoma), cT3d (intraocular hemorrhage), and cT3e (aseptic orbital cellulitis) were predictive factors for high-risk pathologic features when compared with cT2a with an odds ratio of 2.3 (P = 0.002), 2.5 (P = 0.002), and 3.3 (P = 0.019), respectively. Size Group 3 (more than two-thirds globe volume) and 4 (diffuse infiltrative retinoblastoma) were the best predictive factors with an odds ratio of 3.3 and 4.1 (P < 0.001 for both), respectively, for high-risk pathologic features when compared with Size Groups 1 (i.e., < 50% of globe volume). CONCLUSIONS: The AJCC retinoblastoma staging clinical cT3c-e subcategories (glaucoma, intraocular hemorrhage, and aseptic orbital cellulitis, respectively) as well as the AJCC OOTF Size Groups 3 (tumor more than two thirds of globe volume) and 4 (diffuse infiltrative retinoblastoma) both allowed stratification of clinical risk factors that can be used to predict the presence of high-risk pathologic features and thus facilitate treatment decisions.
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Glaucoma , Celulite Orbitária , Neoplasias da Retina , Retinoblastoma , Glaucoma/patologia , Hemorragia , Humanos , Estadiamento de Neoplasias , Neoplasias da Retina/patologia , Retinoblastoma/patologia , Estudos RetrospectivosRESUMO
OBJECTIVES: To audit the structural and functional outcomes of surgery for acute tractional retinal detachment due to retinopathy or prematurity between 2004 and 2014 in Oxford UK. METHODS: Consecutive operations were identified from a surgical log. Clinical data including demography, perioperative data, and retinal outcomes were extracted into a spreadsheet and compared against two international data sets referenced in the method section. Nonparametric tests (Fisher's exact, and the Mann-Whitney U-tests) were used for statistical analysis with a p-value < 0.05 considered significant. RESULTS: Twenty-nine eyes of 19 babies underwent surgery. The mean age (SD) at final follow-up was 6.4 (3.7) years of age and comparable to the reference data sets. The mean birth weight and gestational age of babies matched the ETROP data set referenced in the method section. Anatomical success was obtained in 16/29 (55.2%) of eyes and more likely with stage 4 ROP than stage 5 disease (p < 0.05). Thirteen of 29 eyes (44.8%) obtained form vision post-operatively. All instances of macular retinal reattachment during follow up were verified with post-operative OCT. CONCLUSIONS: Surgery for stage 5 ROP is not worthwhile. For stage 4 ROP it yielded better visual outcomes than ETROP but registration for visual impairment was not prevented. Innovation such as endoscopic vitrectomy could yield better outcomes. Earlier detection of vitreoretinal fibrosis could result in timelier referral. A formally funded national service is needed to ring-fence resource to avoid delays in access to surgery, which has a narrow surgical window.
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Descolamento Retiniano , Retinopatia da Prematuridade , Criança , Seguimentos , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , Retinopatia da Prematuridade/complicações , Retinopatia da Prematuridade/diagnóstico , Retinopatia da Prematuridade/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Acuidade Visual , Vitrectomia/métodosRESUMO
PURPOSE: To compare metastasis-related mortality, local treatment failure, and globe salvage after retinoblastoma in countries with different national income levels. DESIGN: International, multicenter, registry-based retrospective case series. PARTICIPANTS: Two thousand one hundred ninety patients, 18 ophthalmic oncology centers, and 13 countries on 6 continents. METHODS: Multicenter registry-based data were pooled from retinoblastoma patients enrolled between January 2001 and December 2013. Adequate data to allow American Joint Committee on Cancer staging, eighth edition, and analysis for the main outcome measures were available for 2085 patients. Each country was classified by national income level, as defined by the 2017 United Nations World Population Prospects, and included high-income countries (HICs), upper middle-income countries (UMICs), and lower middle-income countries (LMICs). Patient survival was estimated with the Kaplan-Meier method. Logistic and Cox proportional hazards regression models were used to determine associations between national income and treatment outcomes. MAIN OUTCOME MEASURES: Metastasis-related mortality and local treatment failure (defined as use of secondary enucleation or external beam radiation therapy). RESULTS: Most (60%) study patients resided in UMICs and LMICs. The global median age at diagnosis was 17.0 months and higher in UMICs (20.0 months) and LMICs (20.0 months) than HICs (14.0 months; P < 0.001). Patients in UMICs and LMICs reported higher rates of disease-specific metastasis-related mortality and local treatment failure. As compared with HICs, metastasis-related mortality was 10.3-fold higher for UMICs and 9.3-fold higher for LMICs, and the risk for local treatment failure was 2.2-fold and 1.6-fold higher, respectively (all P < 0.001). CONCLUSIONS: This international, multicenter, registry-based analysis of retinoblastoma management revealed that lower national income levels were associated with significantly higher rates of metastasis-related mortality, local treatment failure, and lower globe salvage.
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Braquiterapia , Enucleação Ocular , Renda/estatística & dados numéricos , Neoplasias da Retina/economia , Neoplasias da Retina/terapia , Retinoblastoma/economia , Retinoblastoma/terapia , Pré-Escolar , Bases de Dados Factuais , Feminino , Saúde Global , Humanos , Lactente , Masculino , Oncologia , Sistema de Registros , Neoplasias da Retina/mortalidade , Retinoblastoma/mortalidade , Estudos Retrospectivos , Terapia de Salvação , Falha de Tratamento , Resultado do TratamentoRESUMO
PURPOSE: To evaluate the ability of the American Joint Committee on Cancer (AJCC) 8th edition to predict local tumor control and globe salvage for children with retinoblastoma (RB). DESIGN: International, multicenter, registry-based retrospective case series. PARTICIPANTS: A total of 2854 eyes of 2097 patients from 18 ophthalmic oncology centers from 13 countries over 6 continents. METHODS: International, multicenter, registry-based data were pooled from patients enrolled between January 2001 and December 2013. All RB eyes with adequate records to allow tumor staging by the AJCC 8th edition criteria and follow-up to ascertain treatment outcomes were included. MAIN OUTCOME MEASURES: Globe-salvage rates were estimated by AJCC clinical (cTNMH) categories and tumor laterality. Local treatment failure was defined as use of enucleation or external beam radiation therapy (EBRT), with or without plaque brachytherapy or intra-arterial chemotherapy (IAC). RESULTS: Unilateral RB occurred in 1340 eyes (47%). Among the 2854 eyes, tumor categories were cT1 to cT4 in 696 eyes (24%), 1334 eyes (47%), 802 eyes (28%), and 22 eyes (1%), respectively. Of these, 1275 eyes (45%) were salvaged, and 1179 eyes (41%) and 400 eyes (14%) underwent primary and secondary enucleation, respectively. The 2- and 5-year Kaplan-Meier cumulative globe-salvage rates without the use of EBRT by cTNMH categories were 97% and 96% for category cT1a tumors, 94% and 88% for cT1b tumors, 68% and 60% for cT2a tumors, 66% and 57% for cT2b tumors, and 32% and 25% for cT3 tumors, respectively. Risk of local treatment failure increased with increasing cT category (P < 0.001). Cox proportional hazards regression analysis confirmed a higher risk of local treatment failure in categories cT1b (hazard ratio [HR], 3.5; P = 0.004), cT2a (HR, 15.1; P < 0.001), cT2b (HR, 16.4; P < 0.001), and cT3 (HR, 45.0; P < 0.001) compared with category cT1a. Use of plaque brachytherapy and IAC improved local tumor control in categories cT1a (P = 0.031) and cT1b (P < 0.001). CONCLUSIONS: Multicenter, international, internet-based data sharing validated the 8th edition AJCC RB staging to predict globe-salvage in a large, heterogeneous, real-world patient population with RB.
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Braquiterapia , Enucleação Ocular , Radioterapia Assistida por Computador , Neoplasias da Retina/terapia , Retinoblastoma/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Internacionalidade , Estimativa de Kaplan-Meier , Masculino , Oncologia , Estadiamento de Neoplasias , Sistema de Registros , Neoplasias da Retina/patologia , Neoplasias da Retina/radioterapia , Neoplasias da Retina/cirurgia , Retinoblastoma/patologia , Retinoblastoma/radioterapia , Retinoblastoma/cirurgia , Estudos Retrospectivos , Sociedades Médicas , Taxa de Sobrevida , Resultado do Tratamento , Estados Unidos/epidemiologia , Adulto JovemRESUMO
A 38-year-old man with a traumatic full-thickness macular hole (FTMH) presented to our eye casualty department with a sudden deterioration of his right eye vision to hand movements over the past one week. The suspected traumatic FTMH was present since he was 13 years old from a direct impact of a golf ball in his right eye and his best-corrected visual acuity (BCVA) has always remained at 1/60 Snellen vision. On examination, he had a very large FTMH measuring 1635 µm with central foveal retinal detachment. Pars plana vitrectomy combined with large inverted internal limiting membrane (ILM) peel flap, 5000 Cs silicone oil tamponade, and autologous platelets implantation was performed. Follow-up visits revealed that the FTMH was closed under silicone oil. The silicone oil was removed six months after the surgery and the FTMH remained close with the retina remaining attached. His BCVA was restored to his previous baseline level of 1/60 Snellen vision. With the advent of multiple techniques to repair FTMH such as the ILM flaps, we have combined this technique with older proven techniques such as silicone oil tamponade and autologous platelets implantation to close the giant traumatic FTMH. This case study demonstrates that combining techniques can help close a FMTH that is otherwise deemed impossible in the past.
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BACKGROUND: To determine the occurrence of macular edema (ME) in vitreoretinal lymphoma (VRL). METHODS: Retrospective analysis of 17 patients (31 eyes) with VRL. A review of the literature was done as well. RESULTS: Nine patients (15 eyes) had fluorescein angiography and/or optical coherence tomography at presentation. In the ME group (six eyes of four patients), three patients (five eyes) had prior chemotherapy and radiation. Excluding eyes with radiation retinopathy (three eyes), rate of ME was 25% (3/12). When two unirradiated fellow eyes of eyes with radiation retinopathy were also excluded, ME rate was 10% (1/10). Excluding the eyes with intraocular surgery, the rate of ME was 0%. In the group without ME (nine eyes of six patients), one patient (one eye) was treated with chemotherapy and radiation and three patients (five eyes) with chemotherapy. Review of the literature showed that the ME was found between 2 and 60% of cases, but most of the cases with ME had prior interventions. CONCLUSIONS: Macular edema in VRL is not uncommon but usually related to prior interventions. Macular edema as an initial presentation of VRL is rare.
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PURPOSE: To evaluate the anatomical and functional results obtained with pars plana vitrectomy (PPV) plus autologous platelet concentrate (APC) as a treatment for macular detachment associated with optic disc pit (ODP). METHODS: We performed a prospective interventional study of 19 eyes of 19 consecutive patients with posterior macular detachment due to ODP. All patients underwent PPV, posterior hyaloid peeling, fluid-air exchange, injection of 0.05 mL of APC over the ODP and 15% perfluoropropane (C3F8) endotamponade. Postoperative measures included face-up positioning for 2 hours and then avoidance of the face-up position during the ensuing 10 days. All patients underwent complete ophthalmologic examination and optical coherence tomography preoperatively at 1 month, 3 months, 6 months, 9 months, and 12 months postoperatively and then annually. Outcome measures were best corrected visual acuity (BCVA) by logMAR, improvement of quality of vision, macular attachment, and resolution of intraretinal schisis-like separation. RESULTS: Preoperatively, the median BCVA was 0.70 (range: 0.30-1.70) and all patients showed improved visual acuity after surgery; BCVA was 0.22 (range: 0.07-0.52) at 12 months follow-up. All patients showed complete reabsorption of intraretinal fluid (median time: 3.5 months [range: 2-8 months]) and macular attachment at the end of follow-up (median: 60 months [range: 12-144 months]), with stable or improved visual acuity. No reoperations were needed and no major adverse events were recorded. CONCLUSION: For macular detachment associated with ODP, the combination of PPV, posterior hyaloid peeling, APC, and C3F8 tamponade is a highly effective alternative technique with stable anatomical and functional results.
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BACKGROUND: Hypotony maculopathy (HM) changes may persist, and visual acuity remains poor, despite normalization of intraocular pressure (IOP). The aim of this study was to evaluate the visual and anatomical results of pars plana vitrectomy (PPV), internal limiting membrane (ILM) peeling, and 20% SF6 gas tamponade in five myopic patients with HM. METHODS: This retrospective interventional study was conducted at the Barraquer Center of Ophthalmology, a tertiary care center in Barcelona, Spain, and included five eyes from five consecutive patients (aged 55.4±13.1 years) with HM caused by different conditions. All the patients were treated with 23-gauge PPV, ILM peeling, and 20% SF6 gas tamponade. Preoperative and postoperative evaluation was performed using anterior and posterior biomicroscopy and best corrected visual acuity (BCVA) by logMAR charts. RESULTS: Before surgery, median spherical equivalent was -13.1 (range -7, -19) diopters of myopia. Preoperatively, four cases presented IOP <6.5 mmHg for 3 (range 2-8) weeks. In three of these four cases, IOP >6.5 mmHg was achieved over 16 (range 16-28) weeks, without resolution of HM; increased IOP was not achieved in the remaining case treated 2 weeks after diagnosis of HM. One case presented IOP >6.5 mmHg with HM for 28 weeks before surgery. Preoperative BCVA was 0.7 (range 0.26-2.3) logMAR, and 0.6 (range 0.3-0.7) logMAR and 0.5 (range 0.2-1) logMAR, respectively, at 4 and 12 months after surgery. There was no statistically significant difference between preoperative and postoperative BCVA. Hyper-pigmentation lines in the macular area were observed in three cases with hypotony. These lines progressed after surgery despite resolution of the retinal folds in the three cases, and BCVA decreased in parallel in two of these cases. CONCLUSION: PPV with ILM peeling followed by gas tamponade is a good alternative for the treatment of HM in myopic patients. However, persistent choroidal folds may compromise BCVA. We therefore recommend initiating treatment as early as possible.
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The use of antibody to vascular endothelial growth factor to treat neovascular glaucoma yields good anatomic results in most cases. However, this type of glaucoma can cause angle closure with decompensation of intraocular pressure secondary to fibrovascular tissue contraction in the anterior chamber. Our surgical technique treats the cause by removing the anterior chamber fibrous complex after administration of antibody to vascular endothelial growth factor, thus restoring the chamber angle.
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Câmara Anterior/cirurgia , Glaucoma Neovascular/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Inibidores da Angiogênese/administração & dosagem , Câmara Anterior/efeitos dos fármacos , Anticorpos Monoclonais Humanizados/administração & dosagem , Bevacizumab , Fibrose/cirurgia , Gonioscopia , Humanos , Pressão Intraocular , Iris/patologia , Tomografia de Coerência Óptica , Tonometria Ocular , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Viscossuplementos/administração & dosagem , VitrectomiaRESUMO
PURPOSE: To evaluate the results of surgery with pars plana vitrectomy (PPV), endodiathermy of sclerotomy site fibrovascularization and adjuvant photocoagulation of the peripheral retina to the pars plana as treatment of recurrent vitreous hemorrhage secondary to PPV for proliferative diabetic retinopathy (PDR). METHODS: Tertiary care center, Barraquer Center of Ophthalmology, Barcelona, Spain. Prospective interventional study of 14 eyes of 14 consecutive patients aged 20 years to 47 years with recurrent vitreous hemorrhage because of sclerotomy site neovascularization secondary to PPV for PDR. All patients were treated by cataract, or transparent lens, extraction with intraocular lens implantation plus 20-gauge PPV of the vitreous base, including incarcerated vitreous of the sclerotomy site, together with endodiathermy of sclerotomy site fibrovascular tissue and adjuvant photocoagulation to the pars plana. Preoperative evaluation was performed by 20-MHz, high-resolution, anterior-segment ultrasonography of the neovascularized sclerotomy sites, pre- and postoperative visual acuity (logarithm of the minimum angle of resolution), and postoperative rebleeding rates. RESULTS: Recurrent vitreous hemorrhage appeared 8 ± 4 months after the initial PPV for PDR. Ten (71%) patients had isolated vascularization of a single sclerotomy site and 4 (29%) had vascularization of 2 sclerotomy sites. Nine of the 18 vascularized sites (50%) corresponded to optical fiber sclerotomies, 7 (39%) to vitreotome, and 2 (11%) to infusion sclerotomies. All the neovascularized sclerotomies presented sentinel vessels in the anterior segment. No patient showed postoperative recurrent vitreous hemorrhage during follow-up of 23 ± 10 months; all showed improved visual acuity, from initial 2.23 (±1.13 standard deviation) to final 0.23 (±0.09 standard deviation). CONCLUSION: According to our study, the technique of choice for the treatment of recurrent vitreous hemorrhage secondary to PPV for PDR is endodiathermy of sclerotomy site fibrovascularization and adjuvant photocoagulation of the peripheral retina to the pars plana. This technique treats the etiology of the problem, improves final best-corrected visual acuity, and, most importantly, helps prevent recurrent bleeding.