Congenital stromal corneal dystrophy in a Spanish family: Clinical, genetic and histological analysis.

PURPOSE: To present the clinical, genetic, and histopathological features of the ninth family affected by congenital stromal corneal dystrophy (CSCD) to date. METHODS: Twelve cases of a Spanish family affected by CSCD were analyzed regarding history, visual acuity (VA, decimal scale), an ophthalmologic exam and specular microscopy. Five eyes were treated by deep anterior lamellar keratoplasty (DALK), and thirteen eyes by penetrating keratoplasty (PK). In the two last generations, a genetic study was performed. RESULTS: Most of the patients affected were born with opaque corneas except for three, whose corneas were clear at birth. Biomicroscopy showed a whitish diffuse stromal opacity with an unaltered epithelium, causing poor VA (from hand motions to 0.4). Patients treated with PK presented mean postoperative VA of 0.19±0.20 over a follow-up time of 235.3±101.4months with 38% recurrences. Patients who underwent DALK experienced VA improvement to 0.17±0.11 over a follow-up time of 10.8±2.6months without signs of recurrence. In the latter, the big bubble technique was not achieved, so a manual technique was performed. The genetic study showed heterozygosis for a 1-bp deletion at nucleotide 962 in exon 8 of the decorin gene. CONCLUSIONS: CSCD is a rare entity, which should be treated by DALK whenever possible, obtaining better results than PK. Close monitoring of children of affected individuals is important, because CSCD can progress during the early years of life.

[Detection of anti-SARS-CoV-2 antibodies in tears: Ocular surface immunity to COVID-19]. / Detección de anticuerpos anti-SARS-CoV-2 en lágrimas: inmunidad de la superficie ocular frente a COVID-19.

Purpose: To evaluate the presence of SARS-CoV-2 specific IgA and IgG antibodies in tears of unvaccinated and anti-COVID-19 vaccinated subjects with previous history of SARS-CoV-2 infection. To compare results in tears with those in saliva and serum and correlate with clinical data and vaccination regimens. Methods: Cross-sectional study including subjects with a previous history of SARS-CoV-2 infection, both unvaccinated and vaccinated against COVID-19. Three samples were collected: tears, saliva and serum. IgA and IgG antibodies against S-1 protein of SARS-CoV-2 were analyzed with a semi-quantitative ELISA. Results: Thirty subjects, mean age 36.4 ± 10, males 13/30 (43.3%) with history of mild SARS-CoV-2 infection were included. 13/30 (43.3%) subjects had received a 2-dose regimen and 13/30 (43.3%) a 3-dose regimen of anti-COVID-19 vaccine, 4/30 (13.3%) subjects were unvaccinated. All the participants with full anti-COVID-19 vaccination (2-or 3-doses) presented detectable anti-S1 specific IgA in all 3 biofluids, tears, saliva and serum. Among unvaccinated subjects, specific IgA was detected in 3/4 subjects in tears and saliva, whereas IgG was not detected. Considering IgA and IgG antibodies titers, no differences were observed between the 2- and 3-dose vaccination regimen. Conclusions: SARS-CoV-2-specific IgA and IgG antibodies were detected in tears after mild COVID-19, highlighting the role of the ocular surface as a first line of defense against infection. Most naturally infected unvaccinated individuals exhibit long-term specific IgA in tears and saliva. Hybrid immunization (natural infection plus vaccination) appears to enhance mucosal and systemic IgG responses. However, no differences were observed between the 2- and 3-dose vaccination schedule.

Detection of anti-SARS-CoV-2 antibodies in tears: Ocular surface immunity to COVID-19.

PURPOSE: To evaluate the presence of SARS-COV-2 specific IgA and IgG antibodies in tears of unvaccinated and anti-COVID-19 vaccinated subjects with previous history of SARS-COV-2 infection. To compare results in tears with those in saliva and serum and correlate with clinical data and vaccination regimens. METHODS: Cross-sectional study including subjects with a previous history of SARS-CoV-2 infection, both unvaccinated and vaccinated against COVID-19. Three samples were collected: tears, saliva and serum. IgA and IgG antibodies against S-1 protein of SARS-CoV-2 were analyzed with a semi-quantitative ELISA. RESULTS: 30 subjects, mean age 36.4 ±â€¯10, males 13/30 (43.3%) with history of mild SARS-CoV-2 infection were included. 13/30 (43.3%) subjects had received a 2-dose regimen and 13/30 (43.3%) a 3-dose regimen of anti-COVID-19 vaccine, 4/30 (13.3%) subjects were unvaccinated. All the participants with full anti-COVID-19 vaccination (2-or 3-doses) presented detectable anti-S1 specific IgA in all three biofluids, tears, saliva and serum. Among unvaccinated subjects, specific IgA was detected in 3/4 subjects in tears and saliva, whereas IgG was not detected. Considering IgA and IgG antibodies titers, no differences were observed between the 2- and 3-dose vaccination regimen. CONCLUSIONS: SARS-CoV-2-specific IgA and IgG antibodies were detected in tears after mild COVID-19, highlighting the role of the ocular surface as a first line of defense against infection. Most naturally infected unvaccinated individuals exhibit long-term specific IgA in tears and saliva. Hybrid immunization (natural infection plus vaccination) appears to enhance mucosal and systemic IgG responses. However, no differences were observed between the 2- and 3-dose vaccination schedule.

Hypertensive and HLA-B27 negative uveitis: Disease course and complications.

PURPOSE: To perform a retrospective analysis on patients with HLA-B27 negative hypertensive acute anterior uveitis. Aqueous humor samples were obtained on which a polymerase chain reaction (PCR) test was performed. The patients were then classified into 3 groups depending on whether they were positive for cytomegalovirus (CMV) or herpesvirus (HSV-VZV) or negative for both. MATERIAL AND METHODS: Different variables were collected in successive visits (baseline, 3, 6, and 12 months). The variables were age, sex, visual acuity, intraocular pressure (IOP), cells in the anterior chamber, retro-keratic precipitates, hypotensive treatment, glaucoma or retina surgery, corneal transplantation, and central thickness of the retinal nerve fiber layer. RESULTS: The sample was 36 patients, with a mean age of 59.78 ± 15.26 years. The mean baseline IOP value was 40 ± 10.42 mmHg in the CMV group compared to 23.8 ± 10.4 mmHg in the HSV-VZV, and 22.65 ± 9.9 mmHg in the negative group. The baseline frequency of retro-keratic precipitates, hypotensive treatment, glaucoma surgery, and corneal transplantation was higher in CMV positives. At one year, the loss of retinal nerve fiber layer and glaucoma surgery was greater in the negative group. In the 3 groups, there was a direct and positive correlation between IOP and inflammation in the anterior chamber. Being 0.94 (P = .05) for the positive for CMV, 0.24 (P = .75) in that of HSV-VZV, and 0.98 (P = .17) in the negative group. CONCLUSIONS: HLA-B27 negative hypertensive acute anterior uveitis with CMV positive has a more aggressive initial presentation. However, after one year, the glaucomatous damage is less than in the negative group. In hypertensive acute anterior uveitis, when inflammation in anterior chamber is controlled then IOP is also controlled.

Myopia progression and axial elongation in Spanish children: Efficacy of atropine 0.01% eye-drops.

PURPOSE: To assess myopia progression in Spanish children and whether treatment with low-dose atropine eye drops delays myopia progression and axial elongation. METHODS: 339 eyes of 339 Caucasian patients with myopia, aged 5 to 11 years, were examined. Participants were randomized to a treatment arm, receiving one atropine (0.01%) eye drop/day for two, and an untreated control arm. At the baseline and 2-year follow-up visits, we recorded: spherical equivalent (SE), axial length (AL), mean keratometry (Mean-K) and anterior chamber depth (ACD). We also examined the rate of children with higher myopia progression (change in SE >1 D/2 years) and identified risk factors for progression. RESULTS: In 339 eyes of the 339 children (age=7.61; SD 1.70; range 5-11 years), the mean baseline SE was-2.15 (SD 0.62) D, and AL was 24.24 (SD 0.79) mm. After 2 years, higher increases occurred in all variables except ACD in the untreated group vs. the atropine group, respectively: SE (-0.51 (SD 0.39) D vs. -0.76 (SD 0.37) D, P<0.001), AL (0.20 (SD 0.20) mm vs. 0.37 (SD 0.27) mm, P<0.001) and Mean-K (0.01 (0.28) D vs. 0.09 (0.32) D, P=0.018). Myopia progression was reduced by 32% in the treatment group. There were more progressors >1D/2y in the control group: 62/168 (36.9%) vs. 35/171 (20.5%) (P<0.001). Atropine was identified as a protective factor against myopia progression (B=1.12; 95% CI= 0.98-1.27; P=<0.001). CONCLUSION: Spanish children showed a low rate of myopia progression. Atropine 0.01% showed a significant effect in slowing the progression of both refractive error and axial elongation.

Hypertensive and HLA-B27 negative uveitis: Disease course and complications. / Evolución y complicaciones en pacientes con uveítis hipertensivas HLA-B27 negativas.

PURPOSE: To perform a retrospective analysis on patients with HLA-B27 negative hypertensive acute anterior uveitis. Aqueous humor samples were obtained on which a polymerase chain reaction (PCR) test was performed. The patients were then classified into 3 groups depending on whether they were positive for cytomegalovirus (CMV) or herpesvirus (HSV-VZV) or negative for both. MATERIAL AND METHODS: Different variables were collected in successive visits (baseline, 3, 6, and 12 months). The variables were age, sex, visual acuity, intraocular pressure (IOP), cells in the anterior chamber, retro-keratic precipitates, hypotensive treatment, glaucoma or retina surgery, corneal transplantation, and central thickness of the retinal nerve fiber layer. RESULTS: The sample was 36 patients, with a mean age of 59.78±15.26 years. The mean baseline IOP value was 40±10.42mmHg in the CMV group compared to 23.8±10.4mmHg in the HSV-VZV, and 22.65±9.9mmHg in the negative group. The baseline frequency of retro-keratic precipitates, hypotensive treatment, glaucoma surgery, and corneal transplantation was higher in CMV positives. At one year, the loss of retinal nerve fiber layer and glaucoma surgery was greater in the negative group. In the 3 groups, there was a direct and positive correlation between IOP and inflammation in the anterior chamber. Being 0.94 (P=.05) for the positive for CMV, 0.24 (P=.75) in that of HSV-VZV, and 0.98 (P=.17) in the negative group. CONCLUSIONS: HLA-B27 negative hypertensive acute anterior uveitis with CMV positive has a more aggressive initial presentation. However, after one year, the glaucomatous damage is less than in the negative group. In hypertensive acute anterior uveitis, when inflammation in anterior chamber is controlled then IOP is also controlled.

Management of corneal neurotrophic ulcers with Cacicol®-RGTA (ReGeneraTing Agent): a case series. / Manejo de las úlceras corneales neurotróficas con Cacicol®- ReGeneraTing Agent: serie de casos.

PURPOSE: Neurotrophic corneal ulcers are difficult to treat, and the conventional treatment often results in failure. A new matrix regenerating agent ("ReGeneraTing Agents"), Cacicol® (Laboratoires Théa), has demonstrated good results over the last few years. Therefore, the aim of this study was to evaluate the response to Cacicol® in a series of cases with neurotrophic corneal ulcers. METHODS: Retrospective case series looking at 11 patients with corneal ulcers unresponsive to conventional therapy that underwent treatment with Cacicol®. One cycle included 1 drop every two days for 5 days. RESULTS: The range of conventional therapy prior to Cacicol® was 0-91 days. On introducing Cacicol® 82% (9/11) of the cases were cured, and 18% (2/11) failed, requiring an amniotic membrane transplant or penetrating keratoplasty. The healing only required one cycle of Cacicol® in 67% (6/9) of the patients. More than one cycle of Cacicol® was needed in 45% (5/11) patients. One corneal bacterial ulcer responded favourably and one case related to Acanthamoeba did not respond. Most of the patients improved or maintained their visual acuity. CONCLUSION: Cacicol® was a useful therapy in a high number of difficult neurotrophic corneal ulcers, including corneal infections. Some cases may require more than one cycle of Cacicol® or used as first-line treatment in order to achieve the desired result.

Vitreous haemorrhage a rare manifestation of retinal astrocytic hamartoma: a paediatric case report. / Hemorragia vítrea una rara manifestación del hamartoma astrocítico retiniano: a propósito de un caso pediátrico.

INTRODUCTION: Retinal astrocytic hamartoma is generally an asymptomatic benign tumour that may or may not be associated with the tuberous sclerosis complex. Haemorrhage is a rare presentation. CASE REPORT: The case concerns a 12-year-old patient with "a black spot" vision in the upper temporal hemifield of the right eye, who referred a similar episode 2 years ago. The anterior pole was normal in the slit lamp. A mass of translucent white-yellow peri-papillary appearance and vitreous peri-papillary haemorrhage was observed in funduscopy. The autofluorescence, fluorescence angiography, and optical coherence tomography characteristics were all compatible with retinal astrocytic hamartoma. Complementary studies (serology and X-rays) and the complete clinical examination rule out associated systemic involvement. The patient was followed-up closely until the vitreous haemorrhage was reabsorbed. CONCLUSION: Vitreous haemorrhage is a rare complication of Retinal astrocytic hamartoma and funduscopic exploration is difficult. Systemic involvement should be ruled out.

Comparison of corneal biomechanical properties of patients with dry eye secondary to Sjögren's syndrome and healthy subjects.

PURPOSE: The goal of this study is to determine whether any difference in corneal biomechanical properties exists between Sjögren's syndrome dry eye patients and healthy subjects. METHODS: Thirty-one patients diagnosed with Sjögren's syndrome and associated dry eye manifestations and 44 healthy individuals were included in the study. Ultrasonic pachymetry (UP) was used to measure central corneal thickness (CCT). Corneal biomechanical parameters were obtained using ocular response analyzer (ORA). The main parameters assessed were corneal hysteresis (CH), corneal resistance factor (CRF), Goldmann correlated intraocular pressure (IOPg) and corneal compensated IOP (IOPcc). A Student's t-test for independent groups was performed to compare the mean of these variables between both groups. RESULTS: Mean CH values in Sjögren's syndrome and healthy subject eyes were 10.1mmHg and 11.18mmHg respectively, representing a statistically significant difference (P=0.003). No other variable measured differed between cases and controls (P>0.05). Mean CRF values were 9.51mmHg and 10.37mmHg respectively, and mean CCT measured by UP in cases and controls was 527.41µm and 552.51µm respectively. CONCLUSIONS: Sjögren's syndrome can influence corneal biomechanical properties, specifically CH. ORA measurements should be considered of interest in the evaluation of Sjögren syndrome subjects.

The role of tocilizumab in the treatment of inflammatory diseases of the eye and orbit: A useful alternative.

PURPOSE: With uveitis being one of the leading causes of blindness worldwide, biological therapies have arisen as an option for the treatment of refractory cases based on good results shown in clinical practice. The goal of this study is to provide a systematic review of current knowledge of the role and possible uses of tocilizumab in the field of ophthalmology. MATERIALS AND METHODS: We performed a search for records reporting the use of tocilizumab for various diseases in MEDLINE (PubMed and OVID). We conducted an analysis of several individual studies and their reported individual patient data (82 eyes of 45 patients) published from 2011 to 2017. CONCLUSIONS: Tocilizumab may prove to be an effective choice for the treatment of a variety of ocular conditions such as refractory uveitis, inflammatory macular edema, vitreo-retinal tumors and thyroid orbitopathy, leading to control of the inflammation in these patients. Further studies need to be conducted to establish its safety and efficacy.

Confocal biomicroscopy in four patients with polychromatic corneal dystrophy. / Biomicroscopia confocal en 4 pacientes con distrofia corneal policromática.

INTRODUCTION: Polychromatic corneal dystrophy is an unusual pre-descemet dystrophy, about which there are very few publications. The findings are presented in a case series of four patients with polychromatic corneal dystrophy, using a slit lamp, specular biomicroscopy, and confocal microcospy. CLINICAL CASES: Four women, between 36 and 72 year-old, with the diagnosis of polychromatic corneal dystrophy in routine reviews. None reported visual symptoms or ocular history of interest. Anterior biomicroscopy showed multiple and small multicoloured brilliant opacities in the posterior area of the corneal stroma, with normal epithelium and anterior stroma. The opacities were bilateral and distributed throughout the entire cornea. Direct family members were examined, but none of them showed opacities. In the specular biomicroscopy, a normal endothelium, with pre-descemet hypereflective particles, was observed. With confocal microscopy, there were no abnormalities in epithelium, Bowman layer, or sub-basal nervous plexus. In two cases, the anterior stroma showed hyper-reflective keratocytes and with small hypereflective particles among them. In the middle stroma, hyper-reflective keratocytes were seen in the four cases, two of them showed tiny hypereflective particles, and in the other two there were abnormal keratocytes with prominent cytoplasmic processes. Posterior stroma in the four cases showed a lot of hypereflective keratocytes and hypereflective particles of different sizes. These particles prevented examining the endothelium. CONCLUSIONS: Polychromatic corneal dystrophy has typical signs that allow it to be diagnosed and characterised. Although the biomicroscopy image only seems to show alterations in the posterior stroma, confocal microscopy shows that the dystrophy affects the entire corneal stroma.

Fibrin glue conjunctival autograft for primary pterygium: Overall outcomes and outcomes in expert versus trainee ophthalmologists.

INTRODUCTION: To determine outcomes of conjunctival autograft attached with fibrin glue (FG) for primary pterygium, and compare these outcomes in expert versus closely supervised trainee ophthalmologists. METHODS: This was a retrospective, comparative, non-randomized, interventional study. Patients were recruited among those with primary nasal pterygium undergoing FG conjunctival autograft. Surgery was performed by expert (136 eyes) or closely supervised trainee (128 eyes) ophthalmologists. Mean follow-up was 7.82±8.23months. Main outcome measures were recurrence rate, reoperation rate and complications. RESULTS: The study sample comprised 264 eyes of 225 patients. Participants were of mean age 47.09±12.89years; 46.7% were male, 28.4% Caucasian and 70.5% Hispanic. Recurrence was recorded in 6.4%: 5.9% in the expert group and 7% in the trainee group (P=0.704) and reoperation in 1.9%: 0.7% and 3.1% (P=0.202), respectively. Both groups showed similar rates of complications such as transient graft edema, graft dehiscence, hematoma or ocular hypertension. Reoperation was slightly more frequent in patients younger than 40years (P=0.064). CONCLUSIONS: Good outcomes were observed for FG conjunctival autografting in primary pterygium surgery, with no differences recorded between supervised trainee and expert surgeons. Our findings suggest the need to supervise pterygium surgeries during training.