BackgroundMutations in the CRB1 gene are associated with variable phenotypes of severe retinal dystrophies, and retinal dystrophies resulting from CRB1 mutations may be accompanied by specific fundus features such as coat’s like vasculopathy in retinitis pigmentosa patients. This is the first report of the occurrence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by a CRB1 mutation.Case presentationAn 18-year old Syrian female patient presented with bilateral gradual loss of vision since early childhood, with recent deterioration in her left eye. She appeared to have an asymmetric bilateral coat’s like vasculopathy which was more severe in the left eye. The diagnosis of Leber congenital amaurosis was suggested, and a genetic CRB1 sequencing for the patient and her two younger siblings, who also had severe vision loss, was done, upon which the diagnosis of Leber congenital amaurosis associated with exudative retinal detachment due to coat’s like vasculopathy was made. Treatment with panretinal photocoagulation was attempted in the worse left eye, but with no improvement. As the disease suddenly progressed in both eyes, pars plana vitrectomy with endolaser and silicone oil tamponade was performed in the better right eye which led to anatomical stabilization of the case without improvement in the visual acuity.ConclusionLeber congenital amaurosis is reported to be associated with multiple systemic and ocular findings, none of which is coat’s like vasculopathy. CRB1 gene mutations are associated with remarkable retinal findings in patients with retinitis pigmentosa and other fundus dystrophies. In this unique case we are reporting the incidence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by CRB1 gene mutation, and its management. CRB1 mutant patients should be followed up closely as sudden progression can have permanent poor outcomes and as early management is vital in such cases.

Visual symptoms often accompany pituitary diseases. Pituitary tumors may compress surrounding structures such as optic chiasm leading to visual field defects including bitemporal hemianopia and visual disturbance. They also may compress cranial nerves III, IV, and VI, leading to ocular motility abnormalities. Pituitary adenomas are the most common cause of chiasmal compression. Patients with nonsecreting tumors present initially with vision loss, and these tumors can reach large size without causing other symptoms; however, hormonally active tumors are detected before vision loss because of systemic symptoms. Acute hemorrhage or infarction of the pituitary tumor known as pituitary apoplexy causes diplopia, loss of vision, and visual field. Thus, the ophthalmologist's role is crucial in diagnosis and treatment of pituitary tumors. As visual loss may be the first sign of recurrence after treatment, it is essential to repeat visual field and visual acuity testing every 6-12 months.

Background Suprachoroidal Drug Delivery has emerged in recent years as a novel promising approach, which may help address the clinical unmet needs in the management of Retinal Vein Occlusion (RVO) associated Macular Edema (ME). In this study, we aim to evaluate the feasibility in regard of the potential efficacy and safety of suprachoroidal injection of Triamcinolone Acetonide (TA) using a microinjector as a mono-treatment of ME due to RVO. Methods This trial included 16 eyes of 16 patients with RVO associated ME presenting to the department of ophthalmology, Al Mouwasat university hospital, Syria. 4 mg of preserved TA was injected suprachoroidally 4 mm away from the inferotemporal limbus using a patient-customized microinjector. After injection, patients were followed after 1 week then monthly for 3 months. Primary outcome measures included the percentage of participants with best-corrected visual acuity (BCVA) gain≥15 letters and increased intraocular pressure (IOP) ≥ 20 mmHg in months 1,2, and 3, secondary measures included mean change from baseline BCVA, central subfield thickness (CST), and IOP through each of the follow-up points in addition to other measures. Results After injection, BCVA gain≥15 letters occurred in 68.7, 62.5, 50, 50% of patients at week 1 and through months 1,2 and 3 respectively, the mean BCVA improved significantly by 16.4, 16, 14.4, and 11.9 letters (p-value< 0.0005) at week 1 and months 1,2 and 3 respectively. This visual gain was associated with a significant reduction of CST by 290.94 ± 181.76 (week-1) (p-value< 0.0005), 274.31 ± 184.60 (month-1) (p-value< 0.0005), 183.50 ± 165.61 (month-2) (p-value = 0.006) and 137,75 ± 156.25 μm (month-3) (p-value = 0.038). We reported one case of increased IOP ≥ 20 mmHg in the first month that decreased in the second month. The mean change of IOP readings was not statistically significant, with an increase ranging from 0.75 mmHg after the first week (p-value = 0.09) and 0.5 mmHg after 3 months (p-value = 0.72). Conclusion This study suggests that suprachoroidal TA could be well tolerated and efficacious as a mono-treatment of RVO associated ME. Future clinical trials are required to confirm its longer-term safety and efficacy and to compare this efficacy with the other therapeutic options. Trial registration This study was retrospectively registered at clinicaltrials.gov (ID: NCT05038072) on 08/09/2021. This article was published as a preprint on 22/06/2022. https://doi.org/10.21203/rs.3.rs-1701105/v1.

Background: Intravitreal administration of therapeutic agents for the management of Retinal vein occlusion (RVO) associated Macular Edema (ME) is still hindered by adverse events. Delivery of therapeutics into the Suprachoroidal space provides a novel promising approach. we aim to study the efficacy and safety of suprachoroidal injection of Triamcinolone Acetonide (TA) using a microinjector as a mono-treatment of ME due to RVO. Methods: This trial included 16 eyes of 16 patients with RVO associated ME presenting to the department of ophthalmology, Al Mouwasat university hospital, Syria. 4 mg of preserved TA was injected suprachoroidally 4 mm away from the inferotemporal limbus using a patient-customized microinjector. After injection, patients were followed after 1 week then monthly for 3 months. Primary outcome measures included the percentage of participants with best-corrected visual acuity (BCVA) gain≥ 15 letters and increased intraocular pressure (IOP)≥ 20 mmHg in months 1,2, and 3, secondary measures included mean change from baseline BCVA, central subfield thickness (CST), and IOP through each of the follow-up points in addition to other measures. Results: After injection, BCVA gain≥15 letters occurred in 68.7%, 62.5%, 50%, 50% of patients at week 1 and through months 1,2 and 3 respectively, the mean BCVA improved significantly by 16.4, 16, 14.4, and 11.9 letters (p-value<0.0005) at week 1 and months 1,2 and 3 respectively. This visual gain was associated with significant reduction of CST by 290.94±181.76 (week-1) (p-value<0.0005), 274.31±184.60 (month-1) (p-value<0.0005), 183.50±165.61 (month-2) (p-value=0.006) and 137,75±156.25 microns (month-3) (p-value=0.038). We reported one case of increased IOP ≥ 20 mmHg in the first month that decreased in the second month. The mean change of IOP readings was not statistically significant, with an increase ranging from 0.75 mmHg after the first week (p-value=0.09) and 0.5 mmHg after 3 months (p-value=0.72). Conclusion: This study suggests that suprachoroidal TA could be well tolerated and efficacious as a mono-treatment of RVO associated ME. Future clinical trials are required to confirm its longer-term safety and efficacy and to compare this efficacy with the other therapeutic options. Trial registration: This study was retrospectively registered at clinicaltrials.gov (ID: NCT05038072) on 08/09/2021. This article was published as preprint on 22/06/2022. https://doi.org/10.21203/rs.3.rs-1701105/v1

Background: Intravitreal administration of therapeutic agents for the management of Retinal vein occlusion (RVO) associated Macular Edema (ME) is still hindered by adverse events. Delivery of therapeutics into the Suprachoroidal space provides a novel promising approach. we aim to study of the efficacy and safety of suprachoroidal injection of Triamcinolone Acetonide (TA) using a microinjector as a mono treatment of ME due to RVO.Methods: This trial included 16 eyes of 16 patients with RVO associated ME presenting to the department of ophthalmology, Almouwasat university hospital, Syria. 4 mg of preserved TA was injected suprachoroidally 4 mm away from the inferotemporal limbus using a patient-customized microinjector. After injection, patients were followed after 1 week then monthly for 3 months.Primary outcome measures included the percentage of participants with best-corrected visual acuity (BCVA) gain≥ 15 letters and increased intraocular pressure (IOP)≥ 20 mmHg in months 1,2, and 3, secondary measures included mean change from baseline BCVA, central subfield thickness (CST), and IOP through each of the follow-up points in addition to other measures.Results: After injection, BCVA gain≥15 letters occurred in 68.7%, 62.5%, 50%, 50% of patients at week 1 and through months 1,2 and 3 respectively, the mean BCVA improved significantly by 16.4, 16, 14.4, and 11.9 letters (p-value<0.0005) at week 1 and months 1,2 and 3 respectively. This visual gain was associated with significant reduction of CST by 290.94±181.76 (week-1) (p-value<0.0005), 274.31±184.60 (month-1) (p-value<0.0005), 183.50±165.61 (month-2) (p-value=0.006) and 137,75±156.25 microns (month-3) (p-value=0.038). We reported one case of increased IOP ≥ 20 mmHg in the first month that decreased in the second month. The mean change of IOP readings was not statistically significant, with an increase ranging from 0.75 mmHg after the first week (p-value=0.09) and 0.5 mmHg after 3 months (p-value=0.72).Conclusion: This study suggests that suprachoroidal TA could be well tolerated and efficacious as a mono-treatment of RVO associated ME. Future clinical trials are required to confirm its longer-term safety and efficacy and to compare this efficacy with the other therapeutic options.Trial registration: This study was retrospectively registered at clinicaltrials.gov (ID: NCT05038072) on September 8, 2021.