Humanistic and Economic Burden of Blindness Associated with Retinal Disorders in a Brazilian Sample: A Cross-Sectional Study.

INTRODUCTION: Vision impairment and blindness have been significantly associated with high medical care expenditures, decrease in health utility, and loss or reduction of productivity. The objective of this study was to assess the humanistic and economic burden of blindness in a Brazilian sample from a societal perspective. METHODS: Cross-sectional, observational, and multicenter study enrolling individuals with blindness (defined as the best corrected visual acuity less than 6/60 in the better-seeing eye) caused by retinal disorders. Data collection was performed between December 2012 and December 2014 through face-to-face interview using a structured questionnaire and three standardized patient-reported outcomes instruments. Direct costs were estimated by multiplying the amount of resources used (12-month recall period) by the corresponding unit cost. Productivity losses were measured using the human capital method. All data were collected in Brazilian real (BRL) and converted to United States dollar (USD), using the exchange rate of 1 USD = 3.0415 BRL (May 7, 2015). RESULTS: A total of 146 subjects from 17 research sites were included with a mean age of 68 (SD = 14.8) years and equal gender distribution. Blindness negatively affected both general and vision-specific health-related quality of life. One-half of patients presented some level of anxiety and depression; of these, about 50% with moderate or severe symptoms. Around one-third of subjects (34.2%) reported at least one fall in the previous 12 months due to vision impairment; of these subjects, 14% reported fractures. Emergency room visits and hospitalization were reported by around 25% and 5% of subjects, respectively. The short-term costs (annual costs) of severe vision impairment or blindness for the studied subjects was USD 128,389.09 (USD 879.37 per person). Total medical direct costs summed USD 116,182.00 (USD 795.77 per person), 61.7% of which was due to outpatient visits (with physicians and other healthcare professionals). The long-term costs (lifetime productivity loss) totalized USD 1,962,599.50 (USD 13,442.47 per person). CONCLUSION: This study demonstrated that blindness imposes both humanistic and economic burden for individuals and for Brazilian society. It also pointed out that there is room to improve blindness management, especially for the poorest people, including health education for individuals, availability of services, and reduction of barriers to patients' access to healthcare assistance. This was a good starting point; however, further research is needed.

Combined Femtosecond Laser-Assisted Cataract Surgery and Small-Gauge Pars Plana Vitrectomy Using Different Devices: A New Trend for Vitreoretinal Surgery?

BACKGROUND AND OBJECTIVE: To report the efficacy, safety, and benefits of femtosecond laser-assisted cataract surgery (FLACS) combined with sutureless 23-gauge pars plana vitrectomy (PPV). PATIENTS AND METHODS: This multicenter, retrospective study evaluated patient records and videos of 43 cases with retinal pathologies and cataract who underwent the combined procedure. RESULTS: In 44.2% and 55.8% of cases, respectively, the LenSx Laser (femtosecond machine; Alcon, Fort Worth, TX) and the Constellation (vitreous cutter; Alcon, Fort Worth, TX), and the Victus (femtosecond machine; Bausch + Lomb, Rochester, NY) and Stellaris PC (vitreous cutter; Bausch + Lomb, Rochester, NY) were used. No complications developed during capsulorrhexis, even without a red fundus reflex, retrobulbar block, or scleral indentation. Foldable intraocular lenses remained stable in the capsular bag during the vitreoretinal surgeries and postoperative visits. The mean times of femtosecond phacoemulsification, vitreoretinal surgery, and total surgery were 22.9 minutes ± 4.7 minutes, 43.1 minutes ± 9.8 minutes, and 65.3 minutes ± 8.6 minutes, respectively. CONCLUSION: This emerging technology is safe and offers several potential benefits for the success of the combined procedure. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:374-379.].

Kabuki Syndrome: a case report with severe ocular abnormalities / Síndrome de Kabuki: relato de caso com alterações oculares severas

Kabuki syndrome is a rare congenital anomaly, characterized by five fundamental features, the "Pentad of Niikawa": dysmorphic facies, skeletal anomalies, dermatoglyphic abnormalities, mild to moderate mental retardation and postnatal growth deficiency. Patients present characteristic external ocular features, nonetheless they may also present significant ocular abnormalities. We report a case of a brazilian child diagnosed with Kabuki syndrome, addressing the clinical features observed, with emphasis on the ocular manifestations. This case highlights the existence of this syndrome and all of its complexity. The identification of preventable causes of loss of vision underlines the value of detailed ophthalmologic examination of Kabuki syndrome patients.

A síndrome de Kabuki é uma anomalia congênita rara, caracterizada por cinco aspectos fundamentais a "Pêntade de Niikawa": face dismórfica, anomalias esqueléticas, alterações dermatoglíficas, retardo mental leve a moderado e retardo de crescimento pós-natal. Os pacientes apresentam aspectos oculares externos característicos. Além disso, também podem apresentar anormalidades oculares significativas. Reportamos um caso de uma criança brasileira diagnosticada com síndrome de Kabuki, relatando os aspectos clínicos observados, com ênfase nas manifestações oculares. Esse caso chama a atenção para a existência dessa síndrome e toda sua complexidade. A identificação de causas evitáveis de perda de visão reforça a importância do exame oftalmológico detalhado de pacientes com síndrome de Kabuki.

Macular hole secondary to X-linked juvenile retinoschisis.

The authors describe a rare case of full-thickness macular hole formation in a 10-year-old boy with X-linked juvenile retinoschisis.

Intravitreal bevacizumab in choroidal neovascularization secondary to Grönblad-Strandberg syndrome: case report.

Angioid streaks are commonly related to Grönblad-Strandberg syndrome, that often shows dermatological characteristics, such as pseudoxanthoma elasticum. Choroidal neovascularization is uncommon, often occurring after ocular trauma. This is a case report of the successful use of intravitreal bevacizumab for the treatment of choroidal neovascularization secondary to Grönblad-Strandberg syndrome.

Intravitreal bevacizumab in choroidal neovascularization secondary to Grõnblad-Strandberg syndrome: case report / Uso de bevacizumab intravítreo em neovascularização coroidiana secundária a síndrome de Grõnblad-Strandberg: relato de caso

Angioid streaks are commonly related to Grönblad-Strandberg syndrome, that often shows dermatological characteristics, such as pseudoxanthoma elasticum. Choroidal neovascularization is uncommon, often occurring after ocular trauma. This is a case report of the successful use of intravitreal bevacizumab for the treatment of choroidal neovascularization secondary to Grönblad-Strandberg syndrome.

As estrias angióides estão comumente relacionadas à síndrome de Grönblad-Strandberg, que se caracteriza ainda pela presença de alterações dermatológicas caracterizadas pelo pseudoxantoma elástico. A presença de neovascularização coroidiana nesses pacientes é incomum, ocorrendo geralmente após trauma ocular. Relatamos um caso de tratamento bem sucedido de neovascularização coroidiana secundária à síndrome de Grönblad-Strandberg com bevacizumab.

Photodynamic therapy for bilateral and simultaneous choroidal neovascularization in stargardt disease.

BACKGROUND: Stargardt disease may lead to chronic destructive changes to the retinal pigment epithelium and secondarily cause a lesion in the Bruch membrane, predisposing to subsequent subretinal choroidal neovascularization (CNV). METHODS: The authors report an angiographically documented, bilateral, and simultaneous case of CNV in Stargardt disease that was successfully treated with photodynamic therapy (PDT). RESULTS: A 53-year-old man with unequivocal Stargardt disease presented with CNV in both eyes simultaneously. Best-corrected visual acuity was 20/30 in the right eye and 20/40 in the left eye. PDT was performed in both eyes and vision improved to 20/25 in the right eye and 20/30 in the left eye. The lesions recurred 3 months later when vision had decreased to 20/40 in the right eye and 20/60 in the left eye. Retreatment with PDT was performed in both eyes and vision improved to 20/30 in the right eye and 20/50 in the left eye. No further recurrence was observed for 4 months. CONCLUSION: Considering the positive short-term outcome in this case as well as the limited data available, PDT may be considered in cases of CNV in Stargardt disease.

[Fundus changes evaluation in degenerative myopia]. / Avaliação das alterações fundoscópicas na miopia degenerativa.

PURPOSE: To evaluate the presence of fundus changes in degenerative myopia. METHODS: Forty patients with refractive error of at least -6.00 diopters were selected for ophthalmologic examination followed by posterior pole photograph and echobiometry. RESULTS: We studied 57 eyes of 37 patients with refractive error ranging from -6.25 to -28.50 diopters, with mean -14.05, and axial length ranging from 26.06 to 32.86 mm, with mean 28.01. We found a temporal crescent in 36.5% and a peripapillary one in 20% of the eyes. Choroidal vessels were seen in 35% of the eyes. Posterior pole changes were as follow: posterior staphyloma in 10.5%, Fuchs' spots in 3.5% and lacquer cracks in 1.5%. Peripheral retinal examination revealed paving stone chorioretinal atrophy in 17.5%, white without pressure in 10.5%, lattice degeneration in 5%, retinal tears in 3.5% and retinoschisis in 1.5% of the examined eyes. CONCLUSIONS: Fundus changes that lead to decreased vision are common in patients with degenerative myopia. Peripheral retinal examination is very important in these patients due to the increased risk of retinal detachment.

Avaliação das alterações fundoscópicas na miopia degenerativa / Fundus changes evaluation in degenerative myopia

OBJETIVO: Avaliar a presença de alterações fundoscópicas em pacientes portadores de miopia degenerativa. MÉTODOS: Quarenta pacientes com erro refrativo de pelo menos -6,00 dioptrias foram selecionados para exame oftalmológico, complementado por retinografia do pólo posterior e ecobiometria. RESULTADOS: Foram estudados 57 olhos de 37 pacientes com erro refrativo de -6,25 a -28,50 dioptrias, com média de -14,05, e comprimento axial de 26,06 a 32,86 mm, com média de 28,01. Encontramos crescente temporal em 36,5 por cento e circunferencial em 21 por cento dos olhos. Vasos da coróide foram visualizados em 35 por cento dos olhos. As alterações do pólo posterior foram as seguintes: estafiloma posterior em 10,5 por cento, mancha de Fuchs em 3,5 por cento e "lacquer cracks" em 1,5 por cento. O exame da periferia retiniana evidenciou atrofia corio-retiniana tipo pavimentosa em 17,5 por cento, branco sem pressão em 10,5 por cento, degeneração "lattice" em 5 por cento, ruptura retiniana em 3,5 por cento e retinosquise em 1,5 por cento dos olhos examinados. CONCLUSÕES: Alterações fundoscópicas que levam à baixa visual são freqüentes em pacientes com miopia degenerativa. O exame da periferia retiniana é muito importante nestes pacientes devido ao risco aumentado de descolamento de retina.

Coroidopatia lúpica: relato de 2 casos / Lupus choroidopathy: report of 2 cases

Descrevemos 2 casos de coroidopatia lúpica, uma manifestação ocular incomum do lúpus eritematoso sistêmico, caracterizada por elevação serosa do epitélio pigmentário retiniano e/ou retina sensorial e moteamento do epitélio pigmentário.