Ophthalmological Monitoring Research Articles

Optic Nerve Head Morphological Variation in Craniosynostosis: A Cohort Study

To evaluate optic nerve head morphology in children with craniosynostosis versus healthy controls. Single-centre, prospective cohort study METHODS: Handheld optical coherence tomography (OCT) was performed in 110 eyes of 58 children (aged 0-13 years) with craniosynostosis. Inclusion criteria were as follows: normal intracranial pressure (ICP) on invasive overnight monitoring, or clinically stable ICP. The latter was defined as stable VA within 1 logMAR line and no papilloedema on fundoscopy for at least four months following OCT, and normal/stable visual evoked potentials. Control data for 218 eyes of 218 children were obtained from a published normative dataset. The main outcome measures were disc width, cup width, rim width and retinal nerve fibre layer (RNFL) thickness (nasal and temporal). Outcome measures were compared using three-way linear mixed model regression analysis (FGFR 1/2-associated craniosynostosis, non-FGFR 1/2-associated craniosynostosis, and controls). Out of 63 eligible children with craniosynostosis, handheld OCT imaging was successful in 110 eyes of 58 children (92%). Of these, 22 (38%) were female. Median subject age at OCT examination was 53 months (range: 2 to 157; IQR: 39 to 73). Twelve children (21%) had FGFR1/2-associated syndromes (Crouzon, n=6; Apert, n=4; Pfeiffer, n=2). Control data were available for 218 eyes of 218 healthy children. 122 controls (56%) were female. Median control age at OCT examination was 20 months (range: 0 to 163; IQR: 6 to 59). When comparing optic nerve head morphology in craniosynostosis (n=58) versus controls (n=218), disc width was 6% greater (p=0.001), temporal cup width was 13% smaller (p=0.027), rim width was 16% greater (p<0.001) and temporal RNFL was 11% smaller (p=0.027). When comparing FGFR1/2-associated syndromes (Crouzon, Apert and Pfeiffer syndromes, n=12) to the rest of the craniosynostosis group (n=46), disc width was 10% smaller (p=0.014) and temporal cup width was 38% smaller (p=0.044). This cohort demonstrated morphological differences of the optic nerve head in craniosynostosis, most markedly in Crouzon, Apert and Pfeiffer syndromes. These findings could help improve ophthalmological monitoring and surgical decision-making in children with craniosynostosis. Further work on longitudinal optic nerve head changes in syndromic and non-syndromic craniosynostosis would be valuable.

Ocular Effects of MEK Inhibitor Therapy: Literature Review, Clinical Presentation, and Best Practices for Mitigation.

MEK signaling pathway targeting has emerged as a valuable addition to the options available for the treatment of advanced cancers including melanoma and non-small cell lung cancer. Ophthalmologic monitoring of patients taking part in clinical trials of MEK inhibitors has shown that while ocular effects are common, generally emerging during the first days to weeks of treatment, the majority are either asymptomatic or have minimal visual impact and are benign, resolving without intervention or the need to reduce or stop MEK inhibitor therapy. However rare cases of serious, potentially vision-threatening ocular toxicities have been reported during MEK inhibitor therapy. There is currently no recommendation for routine ophthalmologic screening or monitoring of patients with advanced cancer who are initiating MEK inhibitor therapy. However, baseline ophthalmologic examination may be useful for all patients initiating MEK inhibitor therapy to allow the differentiation of preexisting pathology versus the development of MEK inhibitor-associated retinopathy in the event of the emergence of symptomatic ocular events. Regular ophthalmologic examination may be appropriate for patients at increased risk for ocular events, such as patients with a history of ocular inflammation, infection, or underlying macular/retinal disease. All patients reporting visual disturbance should be referred for prompt ophthalmologic review to determine the potential seriousness of any underlying abnormalities and whether there is a need for treatment modification or specific intervention. Understanding the potential consequences of ocular toxicities is of particular importance in the context of decision-making for the continuation of potentially life-prolonging medications such as MEK inhibitors.

New Insights in 9q21.13 Microdeletion Syndrome: Genotype–Phenotype Correlation of 28 Patients

The implementation of array comparative genomic hybridisation (array-CGH) allows us to describe new microdeletion/microduplication syndromes which were previously not identified. 9q21.13 microdeletion syndrome is a genetic condition due to the loss of a critical genomic region of approximately 750kb and includes several genes, such as RORB and TRPM6. Here, we report a case of a 7-year-old boy affected by 9q21.13 microdeletion syndrome. He presents with global developmental delay, intellectual disability, autistic behaviour, seizures and facial dysmorphism. Moreover, he has severe myopia, which was previously reported in only another patient with 9q21.13 deletion, and brain anomalies which were never described before in 9q21.13 microdeletion syndrome. We also collect 17 patients from a literature search and 10 cases from DECIPHER database with a total number of 28 patients (including our case). In order to better investigate the four candidate genes RORB, TRPM6, PCSK5, and PRUNE2 for neurological phenotype, we make, for the first time, a classification in four groups of all the collected 28 patients. This classification is based both on the genomic position of the deletions included in the 9q21.3 locus deleted in our patient and on the different involvement of the four-candidate gene. In this way, we compare the clinical problems, the radiological findings, and the dysmorphic features of each group and of all the 28 patients in our article. Moreover, we perform the genotype-phenotype correlation of the 28 patients to better define the syndromic spectrum of 9q21.13 microdeletion syndrome. Finally, we propose a baseline ophthalmological and neurological monitoring of this syndrome.

HIV Retinopathy and Immune Reconstitution Uveitis on CMV Retinitis: A Case Report

Aim: In HIV patients with CD4+count&lt;50cells/µL, after initiating HAART, close ophthalmological monitoring is mandatory, in order to watch for the development of IRU.&#x0D; Case Presentation: A 39-year-old patient with unknown medical history consulted for blurred vision and myodesopsia. The examination found bilateral microangiopathy with temporal retinal ischemia in the right eye.&#x0D; The patient was tested positive for HIV-1 with a CD4count of 43 cells/mm3. The diagnosis of HIV-microangiopathy was made, he received a prophylactic retinal argon-laser and was put on HAART.&#x0D; Following the HAART, he presented decreased VA in the right eye, with in examination, a slight inflammation of the AC, moderate hyalitis, and a diffuse and confluent temporal retinal necrosis.&#x0D; The diagnosis of IRU associated with CMV-retinitis was presumed.&#x0D; Discussion: The HAART increases the number of CD4-T-cells and restores immune responses against a wide variety of pathogens which reduces the incidence of opportunistic infections. However, in some patients, a dysregulated immune response after initiation of HAART leads to the immune reconstitution inflammatory disease, characterized by the paradoxical aggravation of a treated opportunistic infection or the revealing of a previously untreated subclinical infection in HIV-positive patients. The ocular manifestation of IRIS is called immune reconstitution uveitis (IRU).&#x0D; IRU usually develops in patients with inactive CMV retinitis, but it can rarely occur in eyes with active CMV retinitis and occurs within the first few weeks of starting HAART.&#x0D; Conclusion: The initiation of HAART requires regular general and ophthalmological monitoring in order to recognize early IRU which is often associated with active CMV retinitis.

Accelerated Progression of Diabetic Retinopathy in a Patient with Acquired Lipodystrophy Following Hematopoietic Stem Cell Transplantation.

To report a case of bilateral proliferative diabetic retinopathy and lipemia retinalis in a patient with acquired lipodystrophy. A retrospective case report. A 23-year-old woman with a diagnosis of acquired lipodystrophy was referred for ophthalmic evaluation. She had been diagnosed with acute lymphocytic leukemia at the age of 3 years and undergone hematopoietic stem cell transplantation. Best-corrected visual acuity was 20/20 in both eyes; however, funduscopy revealed bilateral diabetic retinopathy and lipemia retinalis, neither of which was apparent 2 years earlier. She had a glycated hemoglobin level of 9.6% and a triglyceride level of 7,394 mg/dL with creamy appearance of serum. Fluorescein angiography showed bilateral neovascularization. Accelerated progression of diabetic retinopathy in the present case appeared to be associated with the onset of lipemia retinalis. We encourage continued ophthalmologic monitoring of patients who underwent hematopoietic stem cell transplantation in childhood for possible accelerated course of diabetic retinopathy.

Screening for toxic retinopathy due to antimalarial drugs in Tunisia.

Toxic retinopathy due to antimalarial drugs is characterized by structural anomalies associated with severe, irreversible visual loss. The advantage of ophthalmologic monitoring is to detect these anomalies at an asymptomatic, preclinical stage, so that the recommended dose can be adjusted before the ophthalmologic manifestations appear. Cross-sectional study carried out in the ophthalmology department of Habib Bourguiba University Hospital, Sfax, between August 2016 and February 2018. All patients treated in the internal medicine department of Hedi Chaker University Hospital with synthetic antimalarial drugs for at least 1 year were included. A complete ophthalmologic examination and specialized retinal testing (fundus autofluorescence, 10-2 automated visual field and swept source OCT) were performed for all patients. Fifty-six patients treated with antimalarial drugs were analyzed. The main indication was systemic lupus erythematosus (80.3%). Fifty-three patients (94.64%) were treated with hydroxychloroquine, and 3 patients (5.4%) with chloroquine. Thirteen patients (23.2%) exhibited signs of retinal toxicity, with fundus autofluorescence alterations in 8% of cases, fundus anomalies in 12.5% of cases, 10-2 automated visual field defects in 16% of cases, and SS-OCT alterations in 23.2% of cases. We did not find a statistically significant association between retinal toxicity, weight, age, sex and renal insufficiency (p values of 0.8, 0.6, 0.66 and 0.7 respectively). Furthermore, the association between the cumulative dose and retinal toxicity was statistically significant (p=0.02). The prevalence of toxic retinopathy was identified as 5% at 5 years, 25% at 10 years and 70% at 20 years. A better understanding of the risk factors for retinal toxicity is necessary when prescribing synthetic antimalarial drugs. Screening should be systematic. It should be based on a combination of functional and anatomic tests. The frequency of screening depends on the associated risk factors.

Bilateral Central Serous Chorioretinopathy (CRSC) in Pregnancy Complicated with Retroplacental Hematoma: About A Case

Introduction: Central serous chorioretinopathy (CSCR) is associated with serous sensorineural retinal detachment in the macular region. The condition that affects the male gender more than their female counterpart results from focal leakage disturbance of retinal pigment epithelial cells and choroidal abnormalities. The corticosteroid is highlighted as one of the major factors that promote the occurrence of CSCR. Stress and pregnancy, especially in the third trimester, have also been reported by some authors. Central serous chorioretinopathy associated with pregnancy complicated by retroplacental hematoma is a rare event. Observation: We report the case of a pregnant woman at approximately 34 weeks of amenorrhea who presented a sudden and severe drop in visual acuity on a pregnancy complicated by retroplacental hematoma and delivered by cesarean section. The diagnosis was based on clinical arguments associated with photos of the retina made by the MII Ret Cam device (Made In India, Retinoscope Camera) highlighting the lifting of the macular region. Ophthalmological monitoring without medical or physical treatment was instituted. At two months of progression without treatment, bilateral visual acuity was 10/10. Conclusion: Central serous chorioretinopathy and pregnancy is not unusual; however, its bilateral and brutal form in a context of pregnancy complicated by retroplacental hematoma is rare. Despite this favorable evolution, monitoring remains necessary in order to prevent recurrences and complications.

Side effects of long-term treatment with chloroquine for rheumatoid arthritis - a review of the literature

Background: Chloroquine is a 4-aminoquinoline derivative. Initially the substance was used as an antimalarial drug, but now chloroquine is also used to treat connective tissue diseases. It is a widely used drug in rheumatological diseases, especially in rheumatoid arthritis and systemic lupus erythematosus. A number of side effects have been reported in the public literature among patients using this drug. This article reviews side effects during long-term treatment of patients with rheumatoid arthritis. Materials and methods: A review of the literature available in the PubMed database was performed, using the key words: "Chloroquin" ; "Chloroquin rheumatoid" ; "Chloroquin toxicity" ; "Chloroquine" ; "Chloroquine rheumatoid" ; "Chloroquine toxicity". Results: Chloroquine is a quinine derivative, originally used in the prevention and treatment of malaria. It is now being successfully used to treat patients with certain connective tissue diseases, including rheumatoid arthritis. A number of publications have described various side effects of this drug. Serious and major problems arise from the drug's toxicity with long-term use, such as in the treatment of rheumatoid arthritis. Long-term use of the drug results in retinopathy and cardiotoxicity (conduction disturbances, cardiomyopathy). The available literature also mentions neuromyotoxicity which is rarely reported by patients. Conclusion: Long-term use of chloroquine carries serious side effects. The main complications of long-term use of the drug affect the visual, cardiovascular and nervous systems. The use of chloroquine in patients with rheumatoid arthritis should emphasize the need for accurate dosing and periodic cardiovascular, ophthalmologic and neurologic monitoring.

Knowledge of sickle cell patients and attitudes and practices relating to the ophthalmological monitoring at the University Hospital of Bouaké (Côte d’Ivoire)

Introduction: Sickle cell disease is a potentially blinding haemoglobinopathy. The aim of this study was to assess the impact of the knowledge, attitudes and practices of sickle cell patients relating to the disease and its ocular complications on their adherence to ophthalmological monitoring of sickle cell disease. Material and methods: Cross-sectional study by systematic recruitment of sickle cell patients received for any reason, in the outpatient department of the Bouaké University Hospital, during the study period. Results: Out of 50 sickle cell patients aged from 8 to 71 years (average of 28.12) the male accounted for 52%. Pupils and students were predominant (60%) and the patients from urban areas represented 92% (n=46). The patients with a high level and secondary level of education represented respectively 42.00% (n=21) and 40.00%. The AS forms and the SC forms were the most represented with respectively 34% (n=17) and 42% (n=21). Those who did not know if sickle cell disease could cause ocular damages represented 52.00% (n=26) and among the 21 (42%) who said they knew, those who did not know that the disease could be blinding by its ocular involvement were 15 accounting for (71.43%). The sickle cell sufferers who had never consulted an ophthalmologist as part of the follow-up of the disease represented 74.00% (n=37) and among them, 33 (89.19%) affirmed that the monitoring consultation had never been prescribed to them. Conclusion: Poor health education of the sickle cell patients is an obstacle to their adherence to the ophthalmological monitoring and the prevention of blindness due to sickle cell disease.

Патология центральной нервной системы у детей с ретинопатией недоношенных

Purpose. To evaluate neurosonographic symptoms of preterm infants as a risk criterion for the development of retinopathy of prematurity. Material and Methods. 579 premature babies with risk of retinopathy of prematurity (ROP) (weight up to 2000 g, gestational age 34–35 weeks) were prospectively examined. Ophthalmic monitoring, neurosonographic examination was carried out. The data were statistically processed, the classical Pearson χ² criterion was calculated. Differences were considered significant at p&lt;0.05. Results. ROP developed in 172 cases (29.7%), 57 of them (33%) had a severe type of the disease (ROP type I). In 83 children neurosonographic symptoms of the head of the caudate nucleus ischemia were revealed. Intraventricular hemorrhage of the third stage was formed in 106 children (18.3%); periventricular leukomalacia was detected in 103 cases (17.7%). The incidence of structural hypoxic lesions of the central nervous system was higher in children with ROP. Severe form of ROP, which required surgical intervention, in 54.3% of cases developed in patient with intraventricular hemorrhages of the 3rd stage and in 50.8% of cases in patients with periventricular leukomalacia. There was a significant difference in the incidence of intraventricular hemorrhages of the 3rd stage (df=1; χ²=41.58, p&lt;0.0001) and periventricular leukomalacia (df=1; χ²=41.49, p&lt;0.0001) in children with ROP and without it. There was a significant difference in the incidence of intraventricular hemorrhages of the 3rd stage (df=1; χ² =51.09; p&lt;0.0001) and periventricular leukomalacia (df=1; χ² =25.31; p&lt;0.0001) in children with ROP I and II types. Significant differences in the prevalnece of neurosonographic signs of head ischemia of the caudate nucleus in the first month of life in the group with ROP and the group without ROP were revealed (df=1; χ² =84.14; p&lt;0.0001). Ischemia of caudate nucleus heads was more often detected in children with severe ROP than in children with ROP with spontaneous regression (df=1; χ² =7.79; p=0.053). Specificity (77.4%), accuracy (76.7%), predictive value of the positive result (34.9%) of the diagnostic criterion «Ischemia of caudate nucleus» is higher than similar indices for intraventricular hemorrhage of the third stage and periventricular leukomalacia, p&lt;0.001. The sensitivity of intraventricular hemorrhage of the third stage as a diagnostic criterion differs insignificantly (p=0.04). Conclusion. Neurosonographic symptoms are an early prognostic criterion for the development and severe course of retinopathy of prematurity. Key words: premature infants; retinopathy of prematurity; ophthalmological monitoring; neurosonography; intraventricular hemorrhage; periventricular leukomalacia; ischemia of the head of the caudate nucleus

Prevalence of retinopathy in newly diagnosed type 2 diabetes mellitus patients and its association with microalbuminuria and HbA1c: A cross sectional study from Rajasthan

Background: Diabetes mellitus is a major public health problem with multiple medical complications. Microvascular complications of diabetes including diabetic retinopathy, depend on the duration and severity of hyperglycemia. There is a paucity of data about the relationship among diabetic retinopathy, microalbuminuria and HbA1c in newly diagnosed type 2 diabetes mellitus (T2DM) patients. The study was conducted on the prevalence of diabetic retinopathy and its association with microalbuminuria and HbA1c among newly diagnosed T2DM patients. Material and method: It was a hospital-based, cross-sectional study conducted at a tertiary care hospital in Rajasthan. After obtaining written informed consent, data were collected from 150 newly diagnosed T2DM patients. Data were analysed using SPSS 20.0. Categorical variables were presented as proportion and continuous variables were presented as mean (SD). Chi square test and one way ANOVA tests were used for bivariate analysis. Results: The mean (SD) age of patients was 50.43 (12.73) years. About 52.7% (n=79) patients were male. About 30% (n=45) of newly diagnosed T2DM patients had microalbuminuria. Thirty patients (20%) had diabetic retinopathy (DR). About 8% (n=12) participants had mild DR, 10.7% (n=16) had moderate to severe DR and 1.3 (n=2) % had proliferative DR. Microalbuminuria was found significantly associated with HbA1c (P&lt;0.01). Diabetic retinopathy and its severity were also found significantly associated with HbA1c (P&lt;0.01). Conclusion: In newly diagnosed T2DM patients, HbA1c and microalbuminuria are associated with the presence of retinopathy. If follow up studies support these results, periodic ophthalmologic monitoring may be beneficial for those newly diagnosed with T2DM.

Effects of systemic targeted immunosuppressive therapy on ocular surface.

The purpose of this review is to give an overview of the corneal manifestations of targeted systemic immunotherapies and provide guidelines for management when applicable. The advent of newer systemic immunosuppressive therapy has resulted in the need for more awareness of potential ocular side effects. Side effects can range from vortex keratopathy as seen with the tyrosine kinase inhibitors, to epithelial microcysts as reported in the use of cytarabine and belantamab mafodotin, spontaneous corneal perforations have been reported with programmed death 1 inhibitors, while eyelid cicatrization has been reported epidermal growth factor inhibitors. Several immunomodulatory therapies result in conjunctivitis which tends to respond to topical lubrication and corticosteroid treatment. Most manifestations listed in the review are limited to the anterior segment; however, some may lead to retinal and optic nerve changes which can be permanently damaging. Ocular surface and corneal changes secondary to systemic immunosuppression can affect main components of the ocular surface. Although most adverse effects are reversible, few changes can be permanent and therefore close ophthalmologic monitoring is necessary.

OPEN-ANGLE GLAUCOMATOUS OPTIC NEUROPATHY IS RELATED TO DIPS RATHER THAN INCREASES IN THE MEAN ARTERIAL PRESSURE OVER 24-H

Objective: Mean arterial pressure (MAP) drives ocular perfusion. Excessive 24-h MAP variability relates to glaucoma, however, whether this is due to dips or increases in the blood pressure (BP) is undocumented. We investigated the association of open-angle glaucoma (OAG) in relation to the five largest MAP dips/increases over 24-h, henceforth called dips/blips. Design and method: In the Maracaibo Aging Study (MAS), 93 participants aged 40y or older (women, 87.1%; mean age, 61.9y) underwent baseline ophthalmological and 24-h ambulatory BP monitoring assessments. OAG was the presence of optic nerve damage and visual field defects. Statistical methods included logistic regression and the generalized R2 statistic. For replication, 48 OAG cases at the Leuven Glaucoma Clinic were matched with 48 controls recruited from Flemish population. Results: In MAS, 26 had OAG. OAG compared to non-OAG participants experienced longer and deeper dips (116.5 vs. 102.7 minutes; to 60.3 vs. 66.6 mmHg; 21.0 vs. 18.0 mmHg absolute or 0.79 vs. 0.81 relative dips compared to the preceding reading). The adjusted odds ratios associated with dip measures ranged from 2.25 (95% confidence interval [CI], 1.31–4.85; P = 0.009) to 3.39 (95% CI, 1.36 8.46; P = 0.008). On top of covariables and 24 MAP level/variability, the dip measures increased the model performance (P values below 0.025). Blips did not associate with OAG. The case-control study replicated the MAS observations. Conclusions: Dips rather than increases in the 24-h MAP level were associated with increased risk for OAG. An ophthalmological examination combined with 24-h BP monitoring might be precautious steps required in normotensive and hypertensive patients at risk of OAG.

Open-Angle Glaucomatous Optic Neuropathy Is Related to Dips Rather Than Increases in the Mean Arterial Pressure Over 24-H.

Mean arterial pressure (MAP) drives ocular perfusion. Excessive 24-h MAP variability relates to glaucoma, however, whether this is due to dips or increases in the blood pressure (BP) is undocumented. We investigated the association of open-angle glaucoma (OAG) in relation to the 5 largest MAP dips/increases over 24-h, henceforth called dips/blips. In the Maracaibo Aging Study (MAS), 93 participants aged ≥40 y (women, 87.1%; mean age, 61.9 y) underwent baseline ophthalmological and 24-h ambulatory BP monitoring assessments. OAG was the presence of optic nerve damage and visual field defects. Statistical methods included logistic regression and the generalized R2 statistic. For replication, 48 OAG cases at the Leuven Glaucoma Clinic were matched with 48 controls recruited from Flemish population. In the MAS, 26 participants had OAG. OAG compared to non-OAG participants experienced longer and deeper dips (116.5 vs. 102.7 minutes; to 60.3 vs. 66.6 mm Hg; -21.0 vs. -18.0 mm Hg absolute or 0.79 vs. 0.81 relative dip compared to the preceding reading). The adjusted odds ratios associated with dip measures ranged from 2.25 (95% confidence interval [CI], 1.31-4.85; P = 0.009) to 3.39 (95% CI, 1.36-8.46; P = 0.008). On top of covariables and 24-MAP level/variability, the dip measures increased the model performance (P ≤ 0.025). Blips did not associate with OAG. The case-control study replicated the MAS observations. Dips rather than increases in the 24-h MAP level were associated with increased risk for OAG. An ophthalmological examination combined with 24-h BP monitoring might be precautious steps required in normotensive and hypertensive patients at risk of OAG.

Первая доклиническая ангиоспастическая фаза ретинопатии недоношенных

Purpose. To evaluate the role of the first preclinical phase in the pathogenesis of retinopathy of prematurity (ROP). Material and methods. 642 premature babies were examined. The gestational age at the time of birth was up to 30 weeks and the weight was up to 1500 g. Ophthalmological monitoring was carried out from 28–30 weeks of postconceptional gestational age. Indirect ophthalmoscopy, digital calibration of retinal vessels, and studies on a wide-field retinal pediatric camera were performed. Dopplerography data of the blood flow in the brain vessels and in the ocular artery were analyzed in 28 children (56 eyes). The partial pressure of oxygen (pO2) and carbon dioxide (pCO2) in capillary blood was studied using Radiometer ABL800 FLEX gas analyzer (normal levels of pO2 are 40–60 mmHg, pCO2 is 35–45 mmHg). Results. It was revealed that the lower the degree of maturity of the child’s body was, the more often retinal arteriospasm was detected. In children with a postconceptional age of 25–27 weeks, arteriospasm was detected in 82% of cases, at 28 – 29 weeks – in 67%, 30 – 32 weeks – in 54% of cases. The degree of angiospasm correlates with a high index of blood flow resistance in the brain vessels and the ocular artery. The predominance of a high index of blood flow resistance in more premature infants with low body weight was revealed.The index of resistance of brain vessels 0.8 and higher, according to dopplerography, is a critical indicator of the threat of ROP development in a premature baby. Conclusion. During retinopathy of prematurity, it is important to detect the first preclinical, antispasmodic phase of ROP development, which lasts about one month and until now practically does not attract the attention of ophthalmologists. In this phase, the pathogenesis of pathological angiogenesis is formed. Further study of the preclinical phase will radically revise the strategy and tactics of diagnosis and treatment of RP. Key words: retinopathy of prematurity, first preclinical angiospastic ROP phase, active ROP phase, scarring ROP phase, autoregulation of retinal vessels, hypoxic and hypercapnic drives.

Multidisciplinary care of patients with Myotonic Dystrophy type 1 (DM1) in South Aquitaine

DM1 is characterized by a multisystemic involvement. Our objective was to determine the proportion of adequate follow-up for each affected organ in DM1 patients based on the recently published American and Spanish recommendations. To this end, we conducted a descriptive cross-sectional survey by phone in adult, genetically proven DM1 patients followed in the two French neuromuscular centers of Bayonne and Hendaye located in South Aquitaine, France. The questionnaire selected the most stringent criteria of the two international recommendations for each item of follow-up. Seventy-three patients were included, 55% of which were women (mean age of 48 years) with an average number of 467 CTG repeats. The proportion of patients receiving clinical follow-up in accordance with the recommendations was 90% in cardiology, 60% in neurology, 68% in ophthalmology, 53% in physiotherapy, 23% in pneumology, and 12% in rehabilitation. The high rate of neurological, cardiological, and ophthalmological monitoring might be explained by a locally dense medical demography whereas low rate of respiratory follow up and rehabilitation may reflect an incomplete knowledge of both the disease and the questionnaire. These results should be carefully interpretated as cognitive status may influence such a declarative study. Our study nevertheless disclosed important disparities according to the recommended multidisciplinary follow-up criteria in this French cohort of adult DM1 patients. These results highlight the major role of a multidisciplinary care and monitoring in DM1.

Improved Motor Function With Risdiplam in Infants With Type 1 SMA

Improved Motor Function With Risdiplam in Infants With Type 1 SMA

Ocular Implications in Patients with Sleep Apnea

Sleep apnea syndrome (SAS) is a condition characterized by recurrent episodes of total or partial collapse of the upper respiratory tract associated with daytime drowsiness that cannot be explained by other factors. SAS is a pathology that can cause ophthalmological damage both directly through the pathophysiological mechanism characteristic of the disease on the ocular system, and indirectly by promoting the development of other pathologies (cardiovascular, metabolic), which are a risk factor for ocular morbidity in the absence of sleep apnea syndrome. The aim of this paper is to highlight the ocular symptoms determined by sleep apnea syndrome (SAS), by analyzing literature over the past 20 years. Method: A mini-review that collected data from Pub Med Central, ResearchGate, GoogleScholar, DovePress, ScienceDirect, Elsevier, related to the ocular implications given by sleep apnea syndrome, with or without continuous positive airway pressure (CPAP) treatment. The study included articles that identified a number of eye conditions associated with sleep apnea, such as: dry eye syndrome and impaired ocular surface, glaucoma, non-arteritic anterior ischemic optic neuropathy, floppy eyelid syndrome, keratoconus, central serous chorioretinopathy, central vein occlusion, corneal neovascularization, and age-related macular degeneration. Sleep apnea syndrome is a pathology that can cause the onset or worsening of varying degrees of severity eye diseases by its pathophysiological mechanism, with a different impact on the quality of the individual’s life. On one hand, the purpose of this review is to identify studies in literature that associate sleep apnea syndrome with eye alterations; on the other hand, to inform the Romanian medical staff in different fields of the patients’ guidance diagnosed with SAS to an ophthalmology clinic since early and mild symptoms, so that these patients benefit from an ophthalmological approach and monitoring, in an attempt to diagnose and treat eye diseases in time and prevent their worsening.

ОФТАЛЬМОЛОГИЧЕСКАЯ ТОКСИЧНОСТЬ АНТИ-GD2 ИММУНОТЕРАПИИ (ДИНУТУКСИМАБ БЕТА) У ПАЦИЕНТА С НЕЙРОБЛАСТОМОЙ ГРУППЫ ВЫСОКОГО РИСКА: ОПИСАНИЕ КЛИНИЧЕСКОГО НАБЛЮДЕНИЯ И ОБЗОР ЛИТЕРАТУРЫ

Neuroblastoma (NB) is the most common extracranial solid embryonic tumor in children. The age of patient, the prevalence of the tumor process and the molecular genetic profile formed the basis of risk-adapted therapy protocols. High-risk patients receive complex treatment, including various combinations of chemotherapy drugs, surgical removal of the tumor, radiation and radioisotope therapy. Since the end of the 90s of the last century, immunotherapy has been introduced into clinical practice as a post-consolidation phase with the aim of affecting the minimal residual tumour mass. GD2-targeted drugs are used as a therapeutic agent for immunotherapy. An important aspect is the study of the early and late toxicity of this therapeutic method. The main side effects include fever, neuropathic pain, allergic reactions, and capillary leak syndrome. The article presents the analysis of literature data on toxic effects arising from immunotherapy (dinutuximab beta), as well and a description of clinical case report of rare specific pathology – ophthalmic complications, which include mydriasis and accommodation disorder up to paresis. The pathogenesis of these disorders is described in the literature as a parasympathetic deficiency, realized through exposure to anti-GD2 drugs, and is leveled after the end of treatment. Given the reversible nature of the disorders, the occurrence of ophthalmic complications does not require the abolition of immunotherapy, however, careful ophthalmological monitoring of the dynamics of pathological manifestations and timely symptomatic correction of the revealed changes are necessary. This information is very important not only for pediatric oncologists/hematologists, but also for ophthalmologists and pediatricians.

Результаты офтальмологического мониторинга пациентов с гипертонической болезнью и ишемической болезнью сердца

Aim: to study ophthalmological clinical functional parameters in patients with hypertension and coronary heart disease (CHD) with coronary artery stenosis. Patients and Methods: 30 patients (58 eyes) with hypertension and CHD with coronary artery stenosis (group A) and 30 healthy controls (60 eyes, group B) were examined. Best-corrected visual acuity (BCVA), macular sensitivity (MS) assessed by MAIA microperimetry, central macular thickness (CMT), foveal avascular zone (FAZ) area, vessel density of superficial capillary plexus (SCP VD) and deep capillary plexus (DCP VD) and choriocapillaris measured by optical coherence tomography angiography (OCTA) were evaluated. Results: FAZ area was 0.42±0.03 mm2 in group A and 0.28±0.03 mm2 in group B (p&lt;0.05). SCP VD was 11.4±3.1% in group A and 25.4±2.5% in group B (p&lt;0.05). DCP VD was 22.3±1.1% in group A and 39.2±3.5% in group B (p&lt;0.05). Choriocapillaris VD was 51.2±1.1% in group A and 63.1±1.2% in group B (p&lt;0.05). Correlations between ocular parameters and ejection fraction (EF) were revealed (BCVA: r=0.61, p&lt;0.05; MS: r=0.68, p&lt;0.05; CMT: r=-0.72, p&lt;0.05; FAZ: r=-0.73, p&lt;0.05; SCP VD: r=0.82, p&lt;0.05; DCP VD: r=0.81, p&lt;0.05; choriocapillaris VD: r=0.76, p&lt;0.05). Conclusions: in patients with hypertension and CHD with coronary artery stenosis, significant (p&lt;0.05) reduction in BCVA, MS, SCP VD, DCP VD, and choriocapillaris VD and increase in CMT and FAZ area were detected compared to healthy individuals. Additionally, strong correlations between ocular parameters (FAZ, SCP VD, DCP VD, and choriocapillaris VD) and EF (p&lt;0.05) were identified. OCTA may be recommended to monitor fundus vessels in hypertension and CHD as an effective noninvasive valuable diagnostic tool to verify hypertension and CHD. Keywords: hypertension, coronary heart disease, coronary artery stenosis, hypertensive retinopathy, foveal avascular zone, vessel density, optical coherence tomography angiography. For citation: Moshetova L.K., Vorobyeva I.V., Dgebuadze A. et al. Ophthalmological monitoring in patients with hypertension and coronary heart disease. Russian Journal of Clinical Ophthalmology. 2021;21(2):51–57. DOI: 10.32364/2311-7729-2021-21-2-51-57.