Branch Retinal Arteriolar Occlusions Research Articles

Retinal diffusion restrictions in acute branch retinal arteriolar occlusion

This study sought to investigate the occurrence of retinal diffusion restrictions (RDR) in branch retinal arteriolar occlusion (BRAO) using standard brain diffusion-weighted imaging (DWI). Two radiologists assessed DWI MRI scans of BRAO patients for RDR in a retrospective cohort study. Inter- and intrarater reliability were calculated using Kappa statistics. Detection rates of RDR were compared among MRI scans with varying field strength, sequence type and onset-to-DWI time intervals. 85 BRAO patients (63.1 ± 16.5 years) and 89 DWI scans were evaluated. Overall sensitivity of RDR in BRAO was 46.1% with visually correlating low ADC signal in 56.1% of cases. Localization of RDR matched distribution of fundoscopic retinal edema in 85% of patients. Inter- and intra-rater agreement for RDR in BRAO was κinter = 0.64 (95% CI 0.48–0.80) and κintra = 0.87 (95% CI 0.76–0.96), respectively. RDR detection rate tended to be higher for 3T, when compared to 1.5T MRI scans (53.7% vs. 34.3%%; p = 0.07). RDR were identified within 24 h up to 2 weeks after onset of visual impairment. RDR in BRAO can be observed by means of standard stroke DWI in a substantial proportion of cases, although sensitivity and interrater reliability were lower than previously reported for complete central retinal artery occlusion.

A Novel Quantitative Assessment Method of Disease Activity in Susac’s Syndrome Based on Ultra-Wide Field Imaging

ABSTRACT Purpose Susac’s syndrome (ScS) is a rare, potentially life-threatening auto-immune disease. Ophthalmic imaging can depict characteristic branch retinal arteriolar occlusions (BRAO) and arterial wall hyperfluorescences that form one of the three diagnostic pillars of this condition. We aim to demonstrate that ophthalmological ultrawide-field (UWF) imaging allows for a qualitative and quantitative assessment in ScS, with application in diagnostics, monitoring of treatment response, and titration of therapy. Methods In seven ScS patients (♀:♂ = 2:5), with a median age of 36 years, range 18 to 57 years, serial ultrawide-field fluoresceine angiography (UWF-FA) studies were performed, with adjunctive wide-field optical coherence tomography angiography analyses (WF-OCTA) in five patients. Mean follow-up was 12.5 months, range 1 to 46 months. Results In all seven patients, BRAO and arterial wall hyperfluorescences were present in UWF-FA and calculated as a quantitative score of disease activity in fluorescein angiography (DA-FA) during follow-up visits. Treatment response was accessible in follow-up imaging as partial reperfusion of retinal vessels, resolution of arterial wall hyperfluorescences and consequently, in reduction of DA-FA score. While qualitative analysis of WF-OCTA provided further information about retinal micro-perfusion, quantitative analysis did not demonstrate a vectored treatment response as it was accessible in FA. Conclusion DA-FA score, as a comprehensive disease activity parameter in ScS has potential to facilitate optimal communication between subspecialties and thereby treatment success.

Association of Acute Macular Neuroretinopathy or Paracentral Acute Middle Maculopathy with Sickle Cell Disease

To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Retrospective case series. Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n= 2) or PAMM (n= 2). Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed. Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.

Retinal and optic disc vasculitis in Susac's syndrome

Abstract Case report The case is presented of 42 year-old woman with no significant medical history, with severe headaches, nausea and vomiting, hearing loss, and alteration of mental status with disorientation and confusion. Ophthalmic examination showed optic disc hyperaemia in right eye, and focal areas of arteriolar occlusion in both eyes. Audiometry demonstrated bilateral neurosensory hypoacusis. Magnetic Resonance Imaging showed multiple small round hyperintense lesions located in the splenium, corpus callosum, basal ganglia, and white matter. Discussion Besides branch retinal arteriolar occlusion, the angiopathy in Susac's syndrome may affect the optic disc vessels.

Diverse Clinical Signs of Ocular Involvement in Cat Scratch Disease.

Objectives:To describe ocular manifestations, diagnosis, and treatment of cat scratch disease.Materials and Methods:Clinical records of patients with ocular cat scratch disease were reviewed.Results:Thirteen eyes of 10 patients (7 female, 3 male) with a mean age of 26.9±18.5 years were included. Nine patients had a history of cat contact and had systemic symptoms associated with cat scratch disease 2-90 days prior to the ocular symptoms. Ocular signs were: neuroretinitis in 4 eyes (associated with serous retinal detachment in the inferior quadrant in 1 eye), optic neuropathy in 2 eyes (1 papillitis and optic disc infiltration, 1 optic neuritis), retinal infiltrates in 6 eyes, retinochoroiditis in 1 eye, branch retinal arteriolar occlusion in 3 eyes, and endophthalmitis in 1 eye. Visual acuities at presentation were 1.0 in 7 eyes, 0.3 in 1 eye, ≤0.1 in 4 eyes, and light perception in 1 eye. Bartonella henselae immunoglobulin (Ig) M and/or IgG were positive in all patients. Systemic antibiotic therapy was administered in all patients. Systemic corticosteroid treatment (15-40 mg/day) was added to the therapy in 4 patients, following 5 days of intravenous pulse methylprednisolone in 2 patients. Treatment was ongoing for 1 patient and the mean treatment duration of the other 9 patients was 47±14.5 days. Visual acuities at final visit were 1.0 in 9 eyes, 0.8 in 1 eye, 0.4 in 1 eye, and no light perception in 1 eye.Conclusion:Cat scratch disease may present with different ocular signs and should be considered in the differential diagnosis in patients with such presentations.

FUNDUS CHANGES IN BRANCH RETINAL ARTERIOLAR OCCLUSION.

To investigate systematically various fundus changes in branch retinal arteriolar occlusion (BRAO) and their natural history. The study comprised a cohort of 123 consecutive patients (135 eyes) with BRAO. Ophthalmic evaluation at initial and follow-up visits included recording visual acuity, visual fields, and detailed anterior segment and fundus examinations, and at initial visit fluorescein fundus angiography. Probability estimates of retinal infarct still present were 89% 1 week after BRAO onset, 69% after 2 weeks, 67% after 3 weeks, 34% after 1 month, and 13% after 3 months. Optic disk pallor in the involved region developed in 21% within 1 month from onset, in 42% by 2 months, and in 65% by 3 months. Retinal arteriolar attenuation developed in 19% within 1 month from onset, and in 28% by 6 months. Arteriolar sheathing developed in 19% within 1 month and 25% within 12 months. Arteriolar emboli were found in 58%; 65% of those were at initial visit, in BRAO seen within 1 week of onset. Most common cause of BRAO is embolism from the heart or carotid arteries; emboli usually get impacted at the arteriolar bifurcation. Migration and disappearance of retinal emboli is a common finding. Evolution of the retinal and optic disk changes is described.

Combined bilateral retinal arteriolar and retinal vein occlusion: An unusual presentation of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a rare autoimmune disease associated with multi-organ damage mediated by tissue-binding autoantibodies and immune complexes. [1] Common ocular manifestations of SLE include nonspecific conjunctivitis, anterior uveitis, or iridocyclitis, which rarely threaten vision. In contrast, retinal vasculitis and optic neuritis are serious manifestations. [2] Branch retinal arteriolar occlusion (BRAO) and branch retinal vein occlusion (BRVO) in patients younger than 30 years of age is rarely caused by atheromatous diseases. Other diverse etiologies are more likely to be inflammatory and infectious conditions. Here, we have report a case of a 17-year-old woman with SLE and hypocomplementenemia, who presented with sudden loss of vision. A rare presentation of SLE is found as combined BRAO and BRVO in both the eyes (OU). Despite advances in the understanding of pathophysiology of SLE, its complications remains enigmatic and, in some instances, it may be multifactorial. Early accurate diagnosis and aggressive immunosuppression is recommended, which helps in the management and preservation of the vision.

Multiple branch retinal arteriolar occlusions associated with smallpox vaccination

We describe a rare combination of multiple multiple branch retinal arteriolar occlusions (BRAO) and encephalopathy, which developed in an adult male 10 days after smallpox vaccination. A 53-year-old physician presented with sudden temporal visual field loss in the left eye. Ten days earlier he had received a smallpox vaccination. Hyperbaric oxygen was administered because of suspected BRAO. Examination on day 3 revealed a decrease in visual acuity and loss of visual field. Fundoscopy of the left eye disclosed a retinal infarct in the inferior macula and multiple cotton wool spots over the entire posterior pole. Fluorescein angiography showed delayed arterial filling and multiple non-perfused areas. Magnetic resonance imaging of the brain disclosed white matter focal lesions. Neurological examination was normal. Audiological examination revealed mild hearing loss caused by an old phonic trauma. A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. At the follow-up visit 3 months later, visual acuity in the left eye improved, fundoscopy showed resolution of the cotton wool spots and temporal disc pallor, and the visual field was substantially restored.

Microangiopathy of the brain, retina, and ear: improvement without immunosuppressive therapy.

We describe a pregnant young woman with branch retinal arteriolar occlusions, encephalopathy, and hearing loss in whom we demonstrated a patent foramen ovale. She improved while receiving anticoagulants and no immunosuppressive therapy. The microangiopathic syndrome of retinopathy, encephalopathy, and deafness may be due to a disturbance of coagulation and/or microembolism.

Rubeosis iridis associated with branch retinal arteriolar occlusions.

A case is reported of neovascularization of the iris and anterior chamber angle in both eyes of a patient with bilateral occlusion of the temporal branches of the central retinal arteries. Although the etiology of the arteriolar occlusions remains uncertain, blockage secondary to embolization from a mural thrombus in the left ventricle seems most likely. The maintenance of normal intraocular pressure and outflow facility in both eyes, despite rubeosis iridis of at least seven months' duration, is noted. The documentation of this relatively benign course should encourage a careful search for asymptomatic neovascularization of the anterior segment in all patients with any degree of occlusive retinal vascular disease.