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Retinal arterial macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS), also known as Familial Retinal Arterial Macroaneurysms (FRAM) syndrome, is a very rare multisystem disorder. Here, we present a case series comprising ophthalmologic and systemic evaluation of patients homozygous for RAMSVPS syndrome causative IGFBP7 variant. New clinical details on 22 previously published and 8 previously unpublished patients are described. Age at first presentation ranged from 1 to 34 years. The classical feature of macroaneurysms and vascular beading involving the retinal arteries was universal. Follow up extending up to 14 years after initial diagnosis revealed recurrent episodes of bleeding and leakage from macroaneurysms in 55% and 59% of patients, respectively. The majority of patients who underwent echocardiography (18/23) showed evidence of heart involvement, most characteristically pulmonary (valvular or supravalvular) stenosis, often requiring surgical correction (12/18). Four patients died in the course of the study from complications of pulmonary stenosis, cerebral hemorrhage, and cardiac complications. Liver involvement (usually cirrhosis) was observed in eight patients. Cerebral vascular involvement was observed in one patient, and stroke was observed in two. We conclude that RAMSVPS is a recognizable syndrome characterized by a high burden of ocular and systemic morbidity, and risk of premature death. Recommendations are proposed for early detection and management of these complications. [ABSTRACT FROM AUTHOR]

Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities. [ABSTRACT FROM AUTHOR]

Objective and Design: A retrospective review study was designed to describe five cases of optic disc melanocytomas with tumor-related visual impairment. Participants: Five patients with optic disc melanocytoma presented with visual complaints to a tertiary eye hospital in Saudi Arabia. Materials and Methods: Demographic and clinical data were analyzed, including the results of ocular examination, lesion laterality, best-corrected Snellen visual acuity, pupillary reflex, visual field testing, color fundus photography, fundus fluorescein angiography, and ophthalmic ultrasound. Results: Visual dysfunction secondary to optic disc melanocytoma was identified. Case 1 had macular star edema with mild tumor enlargement, Case 2 had optic atrophy, Case 3 had juxtapapillary choroidal neovascular membrane with macular involvement, Case 4 had optic disc swelling with an enlarged blind spot, and Case 5 had a large altitudinal visual field defect. Conclusion: Although melanocytomas of the optic disc tend to have a benign behavior with slow evolution and stable vision, they may adversely affect visual function through a variety of mechanisms. [ABSTRACT FROM AUTHOR]

Background: Neovascular glaucoma (NVG) is a severe form of secondary glaucoma caused by the growth of newvessels over the trabecular meshwork. The principal causes are associated with retinal ischemia. Ablative treatmentof the retina can prevent, halt, and even reverse the growth of new vessels on the iris and angle. It is an essential partof the management in most cases. Aims: To determine the causes of NVG among Saudi patients, presented at the King Khaled Eye Specialist Hospital. Methods: A retrospective review of 337 Saudi patients with NVG was obtained. All cases were reviewed for theevidence and causes of the disease, and their basic demographic information. A subset of 100 diabetic patients withPDR was further studied in greater detail for clinical findings and treatment history. Results: The most common primary etiologic associations for NVG included diabetic retinopathy (DR) (56.06%), retinalvenous obstruction (26.40%), and chronic retinal detachment (03.56%). A history of diabetes mellitus was reported in65.04%, systemic arterial hypertension was noted in 61.00%, and evidence of renal impairment was documented in22.00%. Vision was markedly reduced in most eyes with NVG (median: hand motion). The median best visual acuity inthe fellow eye was 20/160. Among the 100 cases, with DR as a cause of NVG, 43 patients had bilateral neovascularizationof the iris (NVI) and 72 had bilateral PDR. Sixty-one patients had no previous laser treatment before the diagnosis of NVG. Among these, who received treatment, the median number of total laser spots was 1,003. Conclusions: Diabetes is a major cause of NVG presented to this tertiary eye care center in the Kingdom of Saudi Arabia followed by retinal venous obstruction. Close monitoring and full pan-retinal photocoagulation (PRP) were absent in most of the diabetic cases. It is important to recognize that the "unaffected" fellow eye, particularly in diabetic patients, may require fairly urgent treatment as well. [ABSTRACT FROM AUTHOR]

Purpose: Novel spectral-domain optical coherence tomography (SD-OCT) findings in posterior microphthalmos (PM) include cystoid changes in the papillomacular fold (PMF). These changes may be misdiagnosed as cystoid macular edema (CME) and may trigger unnecessary treatment including intravitreal injections. We report a case that underscores this scenario aiming to increase awareness of this entity among ophthalmologists. Method: A case report. Results: A 25-year-old male presented with a history of clear lens extraction in both eyes as a refractive procedure. Postoperatively, he was diagnosed with CME and received five intravitreal bevacizumab injections in each eye as well as oral diamox for persistent cystic spaces noted on OCT. He was referred to our institution with the diagnosis of non-responding CME. A complete ophthalmic evaluation, including ultrasonography and SD-OCT, confirmed the diagnosis of PM and PMF with cystic cavities in the PMF. A fluorescein angiogram disclosed absence of macular leakage or optic nerve head staining indicating that the cystic cavities seen on SD-OCT are not due to postoperative CME but are rather the intrinsic cystic changes commonly seen in eyes with PM and a PMF. These cystic cavities were incorrectly thought to represent postoperative CME, which triggered the unnecessary treatment and lack of response. Conclusion: Cystic-like cavities are a component of the PMF in PM eyes and may be misdiagnosed as CME, especially in a postoperative setting. This case underscores the importance of understanding the intrinsic features of the PMF in eyes with PM and may help increase awareness among ophthalmologists of such a potentially confusing scenario. [ABSTRACT FROM AUTHOR]

Purpose: This work aims to assess the value of intravitreal triamcinolone acetonide (IVTA) as an adjunctive therapy in advanced Coats disease with exudative retinal detachment (ERD)., Methods: A retrospective review was conducted of patients with Coats disease stage 3 or higher who received IVTA to decrease subretinal fluid (SRF), facilitate retinal ablative therapy, and avoid surgical drainage. Primary outcomes were SRF resolution and avoidance of surgical SRF drainage., Results: Seventeen eyes of 17 patients (mean, [SD] age, 3.9 [3.4] years) met the inclusion criteria. ERD configuration was bullous in 7 and shallow in 10 eyes. Following a single IVTA injection, ablative therapy was achieved after a mean (SD) of 2.1 (3.0) weeks. Complete SRF resolution was observed in 13 eyes (76.4%) after a mean of 1.3 IVTA injections and a mean of 2 (SD, 1.27) laser sessions, and none of these eyes required SRF drainage up to last follow-up (mean [SD], 50.5 [26.24] months). In 4 eyes with bullous ERD at presentation, SRF persisted ( P = .015) despite additional measures including surgical drainage. Final visual acuity ranged from 20/100 to no light perception. Cataract developed in 12 of the 17 eyes (70.5%). None developed an increase in intraocular pressure at final follow-up., Conclusions: IVTA injection can be a helpful adjunctive modality to address SRF in advanced Coats disease. It may obviate the need to surgically drain SRF to effectively treat the condition, particularly when the ERD is not highly bullous., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2021.)

Bilateral optic disc swelling in children is a relatively uncommon sign with a wide array of differential diagnoses. We describe a boy with bilateral optic disc swelling referred as a case of papilledema. However, upon careful ocular fundus examination, detection of discrete retinal nerve fiber layer and pigment epithelial changes at a distance from the optic discs raised the probability of malignant hypertension. This was confirmed by a simple blood pressure measurement. Judicious medical control of the hypertension and a full systemic evaluation led to successful surgical treatment of the inciting pheochromocytoma. A thorough history and meticulous clinical examination are irreplaceable, powerful diagnostic tools that can correctly direct the plan of management, thus preventing unnecessary investigations and delays or potentially serious complications.