Burnout syndrome in Spanish, Portuguese and Latin American ophthalmologists: a cross-sectional analysis.

PURPOSE: Despite the well-known consequences of burnout syndrome, there has been little research into this syndrome in the field of ophthalmology. Our objective is to determine the frequency of burnout syndrome between Spanish, Portugal and Latin-American ophthalmologists, as well as to analyse its relationship with different sociodemographic variables. METHODS: This is an observational cross-sectional descriptive study including ophthalmologists from Spain, Portugal and 15 Latin American countries. Burnout syndrome was evaluated by the Maslach Burnout Inventory-Human Services Survey. Burnout syndrome was defined as high emotional exhaustion, high depersonalization or low personal accomplishment. In addition, we recorded sociodemographic variables and aspects related to the type of work. A conditional backwards stepwise multivariate binomial logistic regression analysis was run to assess the variables associated with burnout syndrome, expressed as an odds ratio (OR). RESULTS: A total of 527 surveys were analysed, the majority from Spain (401; 76.1%), followed by Argentina (45; 8.5%), and Mexico (31; 5.9%). The number of ophthalmologists with burnout syndrome was 351 (66.6%). The results show the profile of a young ophthalmologist (age OR 0.985), carrying out a public activity (OR 2.118) and dedicated to general ophthalmology (OR 1.860). CONCLUSIONS: The frequency detected in this study confirms a steady increase in rates of burnout among ophthalmologists. As the consequences of this are well known, it is vital for health centre managers to develop a strategic plan to allow the causes to be understood better and to implement steps to improve the situation in each medical centre.

Peripheral Ulcerative Keratopathy following Systemic Treatment with Bortezomib: A Case Report.

A 60-year-old patient was diagnosed with multiple myeloma in November 2020, and started treatment in December 2020 with bortezomib, dexamethasone, and thalidomide. Four months later, he presented to the ophthalmology emergency department with inflammation and pain in the left eye. Examination of the anterior segment revealed severe, mixed anterior squamous blepharitis with significant bilateral palpebral inflammation, dysfunction of the meibomian glands, and several styes on both eyelids bilaterally. A peripheral ulcerative keratopathy was detected while examining the left eye cornea, with an inferior infiltrate and significant thinning, approximately 5 mm in length, at the limbal margin (Figure 1). Tear break-up time was shortened bilaterally. In addition to palpebral hygiene and oral treatment with doxycycline 100 mg every 24 hours, the patient was prescribed the following topical treatment without preservatives: artificial tears with lipid emulsion, netilmicin 0,3% 0,4 ml eye drops every 6 hours and dexamethasone 1 mg/ml every 8 hours.

Unusual clinical phenotype of Stargardt disease / Fenótipo clínico incomum da doença de Stargardt

ABSTRACT Mutations in the ABCA4 gene are a common cause of Stargardt disease; however, other retinal phenotypes have also been associated with mutations in this gene. We describe an observational case report of an unusual clinical phenotype of Stargardt disease. The ophthalmological examination included best corrected visual acuity, color and autofluorescence photography, fluorescein angiography, optical coherence tomography, and electrophysiology tests. Targeted next-generation sequencing of 99 genes associated with inherited retinal dystrophies was performed in the index patient. A 48-year-old woman presented with a best corrected visual acuity of 20/25 and 20/20. Fundoscopy revealed perifoveal yellow flecked-like lesions. Fluorescein angiography and fundus autofluorescence findings were consistent with pattern dystrophy. Pattern electroretinogram demonstrated bilateral decrease of p50 values. Genetic testing identified two heterozygous missense mutations, c.428C>T, p.(Pro143Leu) and c.3113C>T, p.(Ala.1038Val), in the ABCA4 gene. Based on our results, we believe that these particular mutations in the ABCA4 gene could be associated with a specific disease phenotype characterized by funduscopic appearance similar to pattern dystrophy. A detailed characterization of the retinal phenotype in patients carrying specific mutations in ABCA4 is crucial to understand disease expression and ensure optimal clinical care for patients with inherited retinal dystrophies.

RESUMO Mutações no gene ABCA4 são causa comum da doença de Stargardt, mas outros fenótipos da retina também foram associados a mutações nesse gene. Apresentamos um relato de caso observacional de um fenótipo clínico incomum da doença de Stargardt. O exame oftalmológico incluiu a acuidade visual com melhor correção, fotografia em cores e com autofluorescência, angiofluoresceinografia, tomografia de coerência óptica e testes de eletrofisiologia. Na paciente em questão, realizou-se o sequenciamento de próxima geração de 99 genes associados a distrofias retinais hereditárias. Tratava-se de uma mulher de 48 anos com melhor acuidade visual corrigida de 20/25 e 20/20. A fundoscopia revelou lesões puntiformes amarelas perifoveais. Os resultados da angiofluoresceinografia e da autofluorescência do fundo de olho foram consistentes com distrofia em padrão. A eletrorretinografia por padrões mostrou diminuição bilateral dos valores de p50. Os testes genéticos revelaram duas mutações missense heterozigóticas, c.428C>T, p. (Pro143Leu) e c.3113C>T, p. (Ala.1038Val), no gene ABCA4. Nossos resultados nos fazem pensar que essas mutações específicas em ABCA4 talvez possam estar associadas a um fenótipo específico da doença, caracterizado por uma aparência fundoscópica semelhante à da distrofia em padrão. Uma caracterização detalhada do fenótipo da retina em pacientes portadores de mutações específicas em ABCA4 é crucial para compreender a expressão da doença e para garantir o tratamento clínico ideal para pacientes com distrofias retinais hereditárias.

Unusual clinical phenotype of Stargardt disease.

Mutations in the ABCA4 gene are a common cause of Stargardt disease; however, other retinal phenotypes have also been associated with mutations in this gene. We describe an observational case report of an unusual clinical phenotype of Stargardt disease. The ophthalmological examination included best corrected visual acuity, color and autofluorescence photography, fluorescein angiography, optical coherence tomography, and electrophysiology tests. Targeted next-generation sequencing of 99 genes associated with inherited retinal dystrophies was performed in the index patient. A 48-year-old woman presented with a best corrected visual acuity of 20/25 and 20/20. Fundoscopy revealed perifoveal yellow flecked-like lesions. Fluorescein angiography and fundus autofluorescence findings were consistent with pattern dystrophy. Pattern electroretinogram demonstrated bilateral decrease of p50 values. Genetic testing identified two heterozygous missense mutations, c.428C>T, p.(Pro143Leu) and c.3113C>T, p.(Ala.1038Val), in the ABCA4 gene. Based on our results, we believe that these particular mutations in the ABCA4 gene could be associated with a specific disease phenotype characterized by funduscopic appearance similar to pattern dystrophy. A detailed characterization of the retinal phenotype in patients carrying specific mutations in ABCA4 is crucial to understand disease expression and ensure optimal clinical care for patients with inherited retinal dystrophies.

Sclerouvectomy plus intraoperative ophthalmic brachytherapy for iris-ciliary body melanomas.

BACKGROUND: Partial lamellar sclerouvectomy was designed more than 30 years ago by Jerry and Carol Shields, in order to remove melanomas that involved the ciliary body and/or the choroid, while leaving intact the outer portion of the sclera and the overlying sensory retina [1]. METHODS: We present two cases of iris-ciliary body melanoma with different size. The performed surgery was a partial lamellar sclerouvectomy plus iridectomy, complemented by intraoperative juxtalimbar ruthenium-106 brachytherapy. RESULTS: Both cases achieved anatomical success without developing chronic ocular hypotony in the postoperative period, an infrequent but possible complication [2]. Even one of them reached a best-corrected visual acuity of 0.7 after silicone oil removal and intravitreal implant of dexamethasone because of secondary macular edema. The other remains stable with counting fingers vision. In this case, the poor functional outcome might be influenced by larger size and longer evolution of the tumor, as well as the uncontrolled arterial hypertension and older age of the patient. DISCUSSION: Intraoperative ophthalmic brachytherapy may have a beneficial effect not only over neoplastic activity [2], but also over early choroidal detachments. Its pro-fibrotic/hemostatic stimulus should be further examined by comparative studies with larger cohorts, either prospectively or retrospectively. Other protective factors could be the utilization of equine pericardial grafts for covering scleral defects as well as leaving high-density silicone oil. Another key aspect is to maintain low blood pressure levels during these procedures in order to avoid intraocular hemorrhages [3], especially when the patient exceeds the sixth decade, so the anaesthesiologist will also play a decisive role in the operating room. We prefer to anticipate the likely development of lens opacification after this operation [4], performing cataract surgery at the beginning and using a Cionni capsular tension ring due to a probable zonular instability. Pars plana vitrectomy would also remove peripheral vitreoretinal tractions and seal the retina preventing a detachment. CONCLUSION: As can be observed during the surgical video, a wide range of complications could occur during a partial lamellar sclerouvectomy. Tackle them ahead of time is crucial to achieve anatomical and functional success.

Auditoría inmunitaria de COVID-19 en el servicio de Oftalmología de un hospital de tercer nivel antes del desconfinamiento / Immune audit of COVID-19 at the Department of Ophthalmology of a tertiary hospital before deconfinement

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Improved Tear Film Stability in Patients with Dry Eye After Hyaluronic Acid and Galactoxyloglucan Use.

PURPOSE: Artificial tears only provide transient relief for dry eye. To the best of our knowledge, this is the first study to objectively compare treatment with artificial tears with Keratograph 5M, which allows accurate and objective investigation of dry eye and artificial tear treatment. We aimed to evaluate whether a preservative-free combination of 0.4% hyaluronic acid and 0.2% galactoxyloglucan can improve dry eye using the new topographer, Keratograph 5M. PATIENTS AND METHODS: This prospective longitudinal, single-arm interventional cohort study was performed at a tertiary referral center and involved 20 patients with dry eye (40 eyes). Preservative-free artificial tears were administered every 3 h. The participants underwent clinical and instrumental evaluations at baseline, 15, 30, 60, 90 and 120 min after instillation and 1 week and 1 month after treatment. Baseline values were considered as the controls. All patients were assessed with Keratograph 5M for non-invasive Keratograph first break-up time and non-invasive Keratograph average break-up time, conjunctival hyperemia, and tear meniscus height. Ocular surface staining with fluorescein was evaluated using the slit-lamp and fluorescein break-up time examinations, and the Ocular Surface Disease Index score was recorded for each patient. RESULTS: The signs and symptoms improved after 1 month of preservative-free 0.4% hyaluronic acid and 0.2% galactoxyloglucan treatment. There was a significant increase in the non-invasive Keratograph first break-up time and non-invasive Keratograph average break-up time at 15, 30, 60, and 90 min, and 1 week and 1 month (P < 0.05) and a decrease in hyperemia, corneal staining, and Ocular Surface Disease Index scores after 1 week and 1 month (P < 0.05). No treatment-related adverse event was observed. CONCLUSION: A combination of 0.4% hyaluronic acid and 0.2% galactoxyloglucan artificial tears seems effective for treating dry eye. Keratograph 5M can objectively detect these changes during the follow-up period.

Posterior microphthalmos, retinitis pigmentosa, and foveoschisis caused by a mutation in the MFRP gene: a familial study.

Background: To characterize the phenotype and genotype of a rare syndrome associating posterior microphthalmos (PM), retinitis pigmentosa (RP), and foveoschisis in a consanguineous Spanish family. Methods: The study involved five family members, consisting of three siblings and their parents. All members underwent comprehensive eye examinations for best corrected visual acuity, axial length and refractive error, electroretinography (ERG), fundus photography, retinal fluorescein angiography (FA), and optical coherence tomography (OCT). Clinical exome sequencing of more than 6,000 clinically relevant genes (SureSelect Focused Exome, Agilent) was performed using the Illumina HiSeq 3000 system. Candidate variants were validated and segregated by Sanger sequencing. Results: The affected siblings had bilateral shortening of the posterior ocular segment and normal anterior segment dimensions. The fundoscopy, ERG, and FA results were compatible with RP. Macular OCT analysis revealed schisis of the outer retinal layer. Our data analysis pipeline identified a homozygous frameshift mutation in exon 5 of the membrane frizzled-related protein (MFRP) gene (c.498delC; p.Asn167Thrfs*25). Conclusion: Our study confirmed the association of PM with RP as an autosomal recessive syndrome. Although this has previously been described, it seems that there are some constant (i.e., PM and RP) and some variable features (i.e., optic nerve drusen and foveoschisis). The MFRP mutation has also been detected in other studies associating PM with RP. Analysis of a larger series of cases at the clinical and genetic levels would certainly help us to better understand the phenotype-genotype correlations of this syndrome.

Renal function and choroidal thickness using swept-source optical coherence tomography in diabetic patients.

AIM: To assess the relationship between choroidal thickness and renal function in diabetic patients. METHODS: Cross-sectional retrospective clinical study of 42 eyes of 21 ocular treatment-naïve diabetic patients. Demographic data included: age, sex, type and course of diabetes. Ocular data included: severity of diabetic retinopathy; retinal thickness at the central macular region, as well as choroidal thickness at the central and paracentral quadrants, using automatically generated maps by swept-source optical coherence tomography; presence of cystic macular edema; and ocular axial length (AXL). Lab-test parameters included: glycated hemoglobin (HbA1c), albuminuria, albumin/creatinine ratio in urine, and glomerular filtration rate. RESULTS: A significant negative correlation was mainly observed between several choroidal thicknesses, age (P<0.020) and ocular AXL (P<0.030). On the contrary, a significant positive correlation was found between all choroidal thicknesses, HbA1c (P<0.035) and albuminuria (P<0.040). CONCLUSION: Choroidal thickness can represent an additional tool to help clinicians predicting the renal status in ocular treatment-naïve diabetic patients.

FOVEAL HYPOPLASIA: DIAGNOSIS USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To describe the clinical presentations and image findings of a patient with foveal hypoplasia. METHODS: Observational case report of one patient. The clinical presentation of the patient was documented by fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. RESULTS: A 55-year-old healthy white female with bilateral foveal hypoplasia without nystagmus was presented. There were no signs suggestive of ocular albinism. Absence of foveal depression was noted on both clinical examination and optical coherence tomography. Lack of foveal avascular zone was confirmed on fluorescein angiography and optical coherence tomography angiography. CONCLUSION: To the best of our knowledge this is the first report of findings in foveal hypoplasia examined by angio-optical coherence tomography. Optical coherence tomography angiography is an easy, rapid, and noninvasive tool that allows imaging of the retinal microvasculature without intravenous dye injection.

Extreme Surgical Maneuvers in Fungal Endophthalmitis.

PURPOSE: To present the different evolution of 2 cases of endophthalmitis caused by Fusarium solani, an aggressive filamentous fungus, depending on the medical and surgical treatment performed. METHODS: We present 2 cases of endophthalmitis caused by Fusarium solani. Topical, intrastromal, intravitreal, and systemic antifungal treatment (natamycin, voriconazole, amphotericin B) failed in both cases. Corneal perforation took place in one of them, being unsuccessfully treated with cyanoacrylate and several amniotic membrane transplants. It became necessary to perform a hot penetrating keratoplasty (PK) in both patients. The lenses were removed, and the microbiological analysis showed their colonization by Fusarium solani. In one of the cases, a second PK and a more aggressive pars plana vitrectomy (PPV) were performed after corneal recurrence detected by confocal microscopy, as well as the following therapeutic intra- and postoperative maneuvers: anterior chamber washing with povidone-iodine 5% for 1 min; iridectomy of the infiltrated regions; aspiration of the fungal colonies with vitrector; several air/fluid/amphotericin/voriconazole exchanges during PPV; endodiathermy and endophotocoagulation of the chorioretinitis foci; and intrascleral angle injections of voriconazole and amphotericin. RESULTS: These were the only cases of endophthalmitis caused by Fusarium attended to at our hospital during the last 10 years. In the case in which PPV was performed without those maneuvers, endophthalmitis rapidly recurred in a more aggressive way, so finally it became necessary to eviscerate the globe. On the other hand, in the patient who underwent PPV with the specific surgical maneuvers and postoperative procedures described above, we could preserve the eye and even a vision of hand motion without an intraocular lens. CONCLUSIONS: The main objectives of these surgical procedures are to control the fungal infection and to preserve the ocular globe. It is essential to eliminate all ocular structures (iris, lens, vitreous, etc.) affected by this strain of fungus in order to reduce the risk of recurrence. When indicated, early surgery with the appropriate maneuvers detailed above may make an evisceration unnecessary and even recover some visual acuity.

Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Patient with Gorlin Syndrome: Spontaneous Partial Resolution of Traction Caused by Epiretinal Membrane.

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS). Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS. Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period. Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.

Surgical Approaches for Vitreomacular Tractions.

PURPOSE: The aim is to describe the main characteristics of different approaches in vitreomacular traction surgery. Setting/Venue: The video (see www.karger.com/doi/10.1159/ 000442579) about vitreomacular traction surgery was created at the Department of Ophthalmology, Virgin Macarena University Hospital, Seville, Spain. METHODS: We present the surgical release of vitreomacular tractions in three different pathologies: (1) idiopathic epimacular membrane; (2) proliferative diabetic retinopathy with long-term hemovitreous, and (3) Coats' disease. RESULTS: Although functional success is less common than anatomical success, we will never be able to improve vision without restoring retinal anatomy. CONCLUSIONS: Vitreomacular tractions are perfectly well known by ophthalmologists. However, the method used to release them must be the least aggressive possible in order to avoid retinal tears or macular holes with subsequent visual loss.

Delayed Boston Keratoprosthesis Exchange due to a Preceding Vitreoretinal Surgery with Intraoperative Choroidal Detachment.

PURPOSE: The aim is to describe the main characteristics of an anterior/posterior segment surgery and how to resolve intraoperative complications. Setting/Venue: The anterior and posterior segment surgical video was created at the Department of Ophthalmology, Virgin Macarena University Hospital, Seville, Spain. METHODS: We present the case of a male with Stevens-Johnson syndrome and severe limbal deficiency who needed a Boston type 1 keratoprosthesis, reaching a visual acuity of 0.4 (0.05 before surgery). In the course of follow-up, he developed corneal melting with perforation, immune vitritis, and a large epimacular membrane. We decided to perform a 23-gauge vitrectomy associated with keratoprosthesis exchange. As a consequence of inappropriate anesthesia, the patient woke up during the surgery, provoking a retinal tear besides a choroidal detachment. These damages needed endolaser photocoagulation as well as silicone oil tamponade, forcing us to postpone the exchange. An intravitreal dexamethasone implant was also injected. Two months later, the silicone oil was removed, and the Boston keratoprosthesis was replaced by a new type 1 model with a titanium back plate, which likely improves biocompatibility and retention and may reduce complications such as retroprosthetic membranes and stromal corneal melts. RESULTS: Good anatomical results were achieved, and visual acuity slightly improved to 0.2. CONCLUSIONS: Combined anterior and posterior segment surgery represents a great challenge that can improve not only visual acuity but also quality of life in patients with severe diseases such as Stevens-Johnson syndrome.