Central retinal artery occlusion or branch retinal artery occlusion in the young associated with high lipoprotein (a) levels

INTRODUCTION: High plasma lipoprotein (a) concentration is an independent risk factor for atherosclerosis and thrombosis. PATIENTS: We present the cases of two young women, one with central retinal artery occlusion and the other with branch retinal artery occlusion. Case 1: A 32-year-old woman was seen in our clinic complaining of a sudden decrease in visual acuity to 1/50 with a central visual field defect in the right eye. The fundus examination revealed an occlusion of the central retinal artery, which was confirmed by fluorescein fundus angiography. Thorough systemic evaluation showed no signs of cardiovascular disease, no coagulation abnormalities, and no clinical or biological evidence of a systemic disorder. On the other hand, hypercholesterolemia and elevated levels of lipoprotein (a) were detected. Case 2: A 35-year-old woman consulted complaining of a visual field loss in the left eye without reduction of visual acuity. Fundus examination and angiography showed the occlusion of the temporal-superior branch artery. A detailed systemic work-up revealed a pseudo-bicuspid aortic valve and high lipoprotein (a) levels. CONCLUSION: These two cases emphasize the importance of a thorough systemic evaluation of young adults with a retinal artery occlusion. High plasma lipoprotein (a) concentration is a risk factor which should not be neglected.     

An unusual cause of central retinal artery occlusion: acquired immunodeficiency syndrome

PURPOSE: In patients with acquired immunodeficiency syndrome (AIDS), disturbances in the circulation of retinal vessels are mostly encountered at the microvascular level. Rarely observed large retinal vessel occlusions frequently affect retinal veins. METHODS: A 32-year-old woman was admitted to the authors' clinic with sudden loss of vision. Her clinical and ophthalmologic examinations and laboratory tests were carried out and the results were evaluated. RESULTS: The patient's history revealed a diagnosis of AIDS established 5 years ago. Her corrected visual acuity was limited to light perception in the right eye and 20/60 in the left eye. There was afferent pupillary defect in the right eye. Posterior segment examination demonstrated central retinal artery occlusion in the right eye and cotton-wool spots in the left eye. The clinical examination and laboratory test results did not reveal any comorbid disease state that can contribute to this presentation. CONCLUSIONS: As thrombi may develop in patients with human immunodeficiency virus infection, they should be closely followed up for the development of vasoocclusive disease.     

Spectral domain optical coherence tomography findings of acute branch retinal artery occlusion from calcific embolus

An 82-year-old female presented with sudden painless decrease in vision in the right eye after awakening. She could see the "superior half" of her vision from the right eye only. On examination, best-corrected vision was 20/300 in the right eye and 20/30 in the left eye. The fundus in the right eye revealed recent superotemporal branch retinal artery occlusion (BRAO) with calcified plaque at the disc. Spectral domain optical coherence tomography (OCT) (OTI Ophthalmic Technologies, Inc.), revealed hyperreflectivity and increased thickness of the inner retinal layers of the superior compared to the inferior retina. Imaging at the optic disc revealed the blocked artery containing a highly reflective material. The high reflectivity of the material and underlying optical shadowing could be characterized as calcific emboli.     

Central retinal artery occlusion in an ANCA negative Churg-Strauss syndrome patient

Purpose To describe a central retinal artery occlusion in a patient with antineutrophil cytoplasmic antibody (ANCA) negative Churg-Strauss syndrome. Methods Review of clinical and laboratory findings of a 44-year-old woman with ANCA negative Churg-Strauss syndrome that developed sudden vision loss in left eye. Results Left central retinal artery occlusion was diagnosed by retinal whitening, a cherry-red spot, and delayed arterial filling on fluorescein angiography. Perinuclear ANCA and cytoplasmic ANCA were negative. Conclusion Central retinal artery occlusion can occur in ANCA negative Churg-Strauss syndrome. Patients with this diagnosis should be considered for prophylactic high dose corticosteroid, regardless of their ANCA status.     

Central retinal vein occlusion combined with cilioretinal artery occlusion

A healthy 65-year-old man with sudden profound visual loss in his right eye presented with clinical signs of central retinal venous occlusion and retinal whitening, indicative of a cilioretinal arterial obstruction. He had been diagnosed with cilioretinal artery occlusion at a private ophthalmology clinic three days before being referred to our department. On fluorescein angiogram of the affected eye, the proximal portion of the retinal arteries filled with dye 27.3 seconds after injection, indicating a delay in retinal arterial filling. Moreover, the cilioretinal artery did not fill at that phase, but went on to fill 45.1 seconds after injection. Over 63.4 seconds after the filling of the retinal arteries, the laminar flow of the retinal venous vessels appeared. This was not until 90.7 seconds after injection. This patient was elderly, had no systemic diseases, and showed non-ischemic CRVO, prolonged retinal arterial filling on fluorescein angiography, and poor prognosis in visual acuity. His clinical course seemed to favor the pathogenetic hypothesis of a primary arterial affection.     

A case of central retinal vein occlusion accompanied by central retinal artery occlusion

A 81-year-old woman developed central retinal vein occlusion (CRVO) in her left eye subsequent to central retinal artery occlusion (CRAO). She noticed sudden visual loss in the left eye. At initial examination, her left visual acuity was 0.03, and only a small visual field was preserved at temporal area. The left fundus showed findings of mild non-ischemic CRVO. In addition white clouded retina was recognized at the left posterior pole which indicated CRAO. FAG showed remarkably prolonged arterial circulation, but no retinal capillary obliteration. Then retinal hemorrhage increased rapidly and her left eye developed hemorrhagic retinopathy. Two weeks after initial examination, FAG showed extensive retinal capillary obliteration. In this case it was supposed that central retinal artery occlusion due to arteriosclerosis produced ischemic capillaropathy and venous thrombosis, after which restoration of arterial circulation caused hemorrhagic retinopathy.     

Spontaneous central retinal artery occlusion in hemoglobin sickle cell disease

PURPOSE: To describe a case of spontaneous central retinal artery occlusion in a young man with hemoglobin sickle cell disease. METHOD: Case report. RESULTS: A 31-year-old African-American man with a history of hemoglobin sickle cell disease developed sudden painless loss of vision in the right eye. Medical history was remarkable for the recent history of a mild painful crisis, but no other systemic illness or contributing factors. Central retinal artery occlusion was diagnosed with retinal whitening, cherry red spot, and delayed arteriovenous transit on fluorescein angiography. Over the ensuing week, the patient had visual recovery to 20/60 in the absence of therapeutic intervention. CONCLUSION: Central retinal artery occlusion has been reported in sickle cell hemoglobinopathies (ie, SS, S-thal, sickle trait, and sickle cell), but the association with sickle cell disease is rare. Most reports have described additional contributing factors, such as trauma or concomitant systemic illness, to help account for the central retinal artery occlusion. The present case suggests that sickle cell disease alone is sufficient for the development of central retinal artery occlusion.     

Unilateral sequential papillophlebitis and central retinal artery occlusion in a young healthy patient

A 23-year-old girl presented to the clinic with metamorphopsia and photopsia in her left eye. After detailed ophthalmic examination, central retinal vein occlusion with optic disc edema was detected in that eye. Three days after diagnosis, the patient returned to our clinic with visual acuity decrease. Central retinal artery occlusion sparing cilioretinal artery was detected. All the laboratory tests were normal except for heterozygous methylenetetrahydrofolate reductase mutation (A1298C genotypes) and an indefinite Lyme disease seropositivity. Symptoms and visual disturbance recovered without any further treatment other than acetylsalicylic acid for prophylaxis.     

Correction-spontaneous central retinal artery occlusion in hemoglobin SC disease(1)

PURPOSE: To describe a case of spontaneous central retinal artery occlusion in a young man with hemoglobin sickle cell disease.METHOD: Case report.RESULTS: A 31-year-old African-American man with a history of hemoglobin SC (sickle C) disease developed sudden painless loss of vision in the right eye. Medical history was remarkable for the recent history of a mild sickle crisis, but no other systemic illness or contributing factors. Central retinal artery occlusion was diagnosed with retinal whitening, cherry red spot, and delayed arteriovenous transit on fluorescein angiography. Over the ensuing week, the patient had visual recovery to 20/60 in the absence of therapeutic intervention.CONCLUSION: Central retinal artery occlusion has been reported in sickle cell hemoglobinopathies (ie, SS, S-thal, sickle trait, and SC), but the association with double heterozygous SC disease is rare. Most reports have described additional contributing factors, such as trauma or concomitant systemic illness, to help account for the central retinal artery occlusion. The present case suggests that SC disease alone is sufficient for the development of central retinal artery occlusion.     

继发于巨细胞动脉炎的全眼肌麻痹、视神经鞘膜炎和视网膜中央动脉阻塞1例(英文)

一位67岁男性患者出现右边颞侧头痛和右眼视力突发性无痛性丧失,出现这些症状时还伴有全部眼肌麻痹和视盘水肿。ESR和C-反应蛋白升高。核磁共振检查证实有视神经鞘膜炎的特征。右颞侧动脉的活组织切片检查正常。眼外肌运动随着全身激素的应用而好转。不幸的是此后患者右眼发展为中央视网膜动脉阻塞。全眼肌麻痹是巨细胞动脉炎的一种罕见表现,为了得到满意的治疗结果并且阻止对侧眼盲,疾病开始时使用皮质类固醇激素是必要的。     

Combined optic neuropathy and central retinal artery occlusion in presumed ocular tuberculosis without detectable systemic infection

Purpose: To report a rare case of combined optic neuropathy and central retinal artery occlusion in presumed ocular tuberculosis without systemic infection. Case Report: A young man presented with sudden onset of decreased vision in his left eye with combined optic disc swelling and central retinal artery occlusion in a background of vasculitic changes of the same eye. There were no signs or symptoms of active systemic tuberculosis infection. Chest X-ray and computed tomography thorax findings were normal and sputum cultures for tuberculosis were negative. The Mantoux and QuantiFERON-TB Gold tests were strongly positive. No tuberculosis polymerase chain reaction testing was done. Anti-tuberculosis therapy was initiated, based on the strong clinical evidence. Conclusion: The ocular findings improved remarkably with the anti-tuberculosis treatment, although the left eye vision remained poor. A high index of suspicion is required to diagnose ocular tuberculosis when all other systemic investigations are negative, especially in this part of the world where tuberculosis is endemic.     

Bilateral ophthalmic artery occlusion in rhino-orbito-cerebral mucormycosis

PURPOSE: To report a case of bilateral ophthalmic artery occlusion in rhino-orbito-cerebral mucormycosis. METHODS: Reviewed clinical charts, photographs, and fluorescein angiography RESULTS: An 89-year-old man with poorly controlled diabetes developed sudden bilateral ptosis, complete ophthalmoplegia of the right eye, and superior rectus palsy of the left eye. Brain and orbit magnetic resonance imaging showed midbrain infarction and mild diffuse sinusitis. On the 2nd day of hospitalization, sudden visual loss and light reflex loss developed. There were retinal whitening, absence of retinal arterial filling, and a total lack of choroidal perfusion on fluorescein angiography of the right eye. The left eye showed a cherry red spot in the retina and the absence of retinal arterial filling and partial choroidal perfusion on fluorescein angiography. On rhinologic examination, mucormyosis was noticed. Despite treatment, visual acuity and light reflex did not recover and he died 4 days after admission. CONCLUSIONS: Bilateral ophthalmic artery occlusion can occur in rhino-orbital-cerebral mucormycosis.     

Central retinal artery occlusion in a patient homozygous for factor V Leiden

PURPOSE: To report an association between central retinal artery occlusion and homozygosity for factor V Leiden. METHOD: Case report. RESULTS: A 48-year-old woman with sudden loss of visual acuity in her right eye caused by a central retinal artery occlusion showed abnormal resistance to activated protein C and was homozygous for the factor V Leiden gene. CONCLUSION: Activated protein C resistance may be a risk factor for central retinal artery occlusion in young adults.     

Combined central retinal artery and vein occlusion in Churg-Strauss syndrome: case report

PURPOSE: To describe a rare case of Churg-Strauss syndrome presenting with severe visual loss due to a combined central retinal vein and artery occlusion. METHODS: A 42-year old man with a medical history of asthma and blood hypereosinophilia developed a sudden loss of vision in his right eye. We describe the clinical features and evolution of the case after treatment. RESULTS: A combined occlusion of the central retinal artery and central retinal vein was diagnosed by the funduscopic appearance of retinal whitening, macular cherry-red spot, papilloedema, retinal haemorrhages in all four quadrants and dilated and tortuous veins. The diagnosis was confirmed by a fluorescein angiogram showing absence of retinal filling and normal choroidal filling. Churg-Strauss syndrome was diagnosed based on the necessary presence of four of six criteria for the disease proposed by the American College of Rheumatology. Corticosteroid therapy was initiated. However, during the following year when tapering off the daily dosage, the patient experienced two relapses, with pulmonary symptoms and hypereosinophilia, and the corticosteroid dosage had to be augmented. The patient presented with neovascular glaucoma 7 weeks after the vascular occlusion and experienced no visual improvement. CONCLUSION: Combined central retinal artery and vein occlusion can occur in Churg-Strauss syndrome. We suggest that regional vasculitis may be the pathological mechanism underlying the vascular occlusions observed in our case. The condition carries a very poor prognosis for vision, due to the resulting retinal ischaemia, and a poor general prognosis due to the late stage of the systemic disease. Corticosteroids should be instigated promptly in order to prevent further systemic or ocular vasculitis.     

Essential thrombocythemia: a rare case of central retinal artery occlusion

Essential thrombocythemia is a myeloproliferative disorder with an increased amount of abnormal platelets, causing both hemorrhagic and thrombotic pathology. Some of its systemic complications include deep vein thrombosis, pulmonary emboli, myocardial infarcts, and renal vessel thrombosis among others. We present the rare case of a woman suffering from essential thrombocythemia with the sudden loss of vision in her right eye, caused by central retinal artery occlusion. A possible connection between these two disorders is discussed.     

Branch retinal vein occlusion in the right eye and retinal hemorrhage in the left in a patient with classical Tsutsugamushi disease

PURPOSE: To report branch retinal vein occlusion and retinal hemorrhages associated with tsutsugamushi disease. METHODS: Case report of a 60-year-old woman who complained of fever, chills, headache, lymphadenopathy, and blurred vision in the right eye following an insect bite to the lower right forehead. RESULTS: Serological findings showed elevated titers for the strains of Rickettsia tsutsugamushi. Ophthalmologic examination disclosed bilateral conjunctival injection, flame-shaped hemorrhage in her right fundus, and scattered hemorrhage in her left fundus. Fluorescein angiography demonstrated dye leakage and dilation of capillaries. CONCLUSIONS: Branch retinal vein occlusion associated with classical tsutsugamushi disease, as demonstrated in our patient, may be rare.     

Branch retinal artery occlusion after thyroid artery interventional embolization

PURPOSE: To report a case of branch retinal artery occlusion after thyroid artery interventional embolization. METHODS: A 33-year-old man with hyperthyroidism complained of visual loss and scotoma in the left eye after thyroid artery interventional embolization. He underwent a full ophthalmologic examination, including fluorescein angiography. RESULTS: Visual acuity was 20/25, with inferior and superior scotomas present in the left eye. Fluorescein angiography of the left eye revealed delayed filling of a superotemporal branch retinal artery and nonfilling of an inferotemporal branch retinal artery. CONCLUSION: A small, but definite risk of retinal artery occlusion after thyroid artery interventional embolization should be considered.     

Central retinal artery occlusion after radial optic neurotomy in a patient with central retinal vein occlusion

PURPOSE: To describe a patient with a central retinal vein occlusion (CRVO) who developed central retinal artery occlusion after radial optic neurotomy. DESIGN: Interventional case report. METHODS: A 70-year-old woman with CRVO underwent a radial optic neurotomy on her right eye. Her preoperative visual acuity in the affected eye was 20/400. RESULTS: Radial optic neurotomy was performed after phacoemulsification and aspiration for a cataract with intraocular lens implantation. At the insertion of a CRVO knife, pulsating bleeding occurred from the cup of the optic disk; the bleeding was stopped within 2 minutes by elevating the intraocular pressure to 80 mm Hg. On the following day, the patient noticed that she had lost light perception. Fluorescein angiography showed a marked delay of arterial filling, indicating a central retinal artery occlusion. Retinal circulation returned to normal 2 months later; however, her vision was still no light perception. CONCLUSIONS: Ophthalmologists should be aware that severe complications such as central retinal artery occlusion can be associated with radial optic neurotomy, which is an unproven surgical procedure with a questionable pathophysiologic mechanism.     

Branch retinal artery occlusion and carotid artery stenosis

A patient who presented with a branch retinal artery occlusion (BRAO) of the left eye was discovered to have severe carotid artery stenosis bilaterally. A case report and a discussion of the optometrist's role in diagnosis and management of retinal artery occlusion and carotid stenosis are presented.     

Protein S deficiency and bilateral branch retinal artery occlusion.

A macular branch retinal artery occlusion developed in the right eye of a 25-year-old woman when she was 38 weeks pregnant. She subsequently presented 5 days postpartum with a branch retinal artery occlusion in her left eye. Although her initial work-up did not reveal a source for her occlusions, subsequent studies have documented a deficiency of protein S.