Bilateral Central Retinal Vein Occlusion Research Articles

Simultaneous Bilateral Central Retinal Vein Occlusion as the First Manifestation of Chronic Myeloid Leukemia

Simultaneous Bilateral Central Retinal Vein Occlusion as the First Manifestation of Chronic Myeloid Leukemia

English

Five case histories are presented. Waldenstrom's macroglobulinaemia caused bilateral central retinal vein occlusion, proptosis was the presenting feature of retro-orbital plasmacytoma in relapsed multiple myeloma, a red painful eye was due to neovascular glaucoma in primary polycythaemia, bilateral VIth nerve palsy caused convergent squint and diplopia in meningeal relapse of acute lymphoblastic leukaemia and lymphoma of the eyelid caused complete ptosis. Interdisciplinary management is described. Ophthalmological lesions in haematological disease should be promptly recognized and managed. Collaboration between ophthalmology and haematology departments may be effective for palliative management.

Bilateral Central Retinal Vein Occlusion in a Patient with Mantle Cell Lymphoma

Bilateral Central Retinal Vein Occlusion in a Patient with Mantle Cell Lymphoma

Fulminant Idiopathic Intracranial Hypertension and Venous Stasis Retinopathy Resulting in Severe Bilateral Visual Impairment

To report a complicated case of fulminant idiopathic intracranial hypertension and concomitant venous stasis retinopathy leading to postpapilledema optic atrophy. Case report. A 34-year-old morbidly obese woman with a history of idiopathic intracranial hypertension (IIH) presented with a 1-month history of bilateral vision loss, diplopia, and left eye pain after being lost to follow-up for 6 years. Fundus examination revealed florid papilledema with venous tortuosity bilaterally. Brain and orbit magnetic resonance imaging showed bilateral globe flattening, intraocular optic nerve swelling in both eyes, and no abnormality on magnetic resonance venography. After additional workup including lumbar puncture with an opening pressure of 55 cm H2O, a diagnosis of IIH was confirmed. Medical treatment with oral carbonic anhydrase inhibitors was initiated, followed by same-day bilateral optic nerve sheath decompression and ventriculoperitoneal shunt placement the following week. Fundus examination 2 months later revealed a persistent blood and thunder fundus suggestive of bilateral central retinal vein occlusions. Over the course of 6 months, both eyes displayed postpapilledema optic atrophy with light perception and hand motion vision in the right and left eyes, respectively. On Goldmann perimetry, the patient had vague limited isolated responses in both eyes to the largest target. Fulminant IIH can present with profoundly blinding complications recalcitrant to aggressive medical and surgical intervention. Central retinal vein occlusion is an uncommon blinding complication of IIH.

Bilateral Simultaneous Central Retinal Vein Occlusion in a Patient with Waldenstrom's Macroglobulinemia

Purpose: The authors report a case of bilateral simultaneous central retinal vein occlusion caused by Waldenstrom’s macroglobulinemia. Case summary: A 65-year-old man presented to our department complaining of decreased visual acuity for the duration of about 6 months. On his initial visit, best-corrected visual acuity was 0.02 in the right eye and 0.06 in the left eye. Based on the findings of a funduscopic examination, the patient had bilateral diffuse retinal hemorrhages, dilated tortuous veins, and macular edema. He had experienced recurrent spontaneous epistaxis 6 months previously and had undergone treatments such as intravitreal bevacizumab injection and intravitreal dexamethasone implantation at another hospital. Laboratory tests at that hospital showed anemia and hyperproteinemia, for which he was referred to our hemato-oncology department. Bone marrow biopsy was consistent with Waldenstrom’s macroglobulinemia/lymphoplasmacytoid lymphoma, and he was treated with systemic chemotherapy. One year after the systemic chemotherapy, his best-corrected visual acuity was 0.15 in the right eye and 0.6 in the left eye. Funduscopy showed decreased bilateral retinal hemorrhages and macular edema. Conclusions: When simultaneous bilateral central retinal vein occlusion occurs in a patient with no other underlying disease such as hypertension or diabetes, it might be a sign of serum hyperviscosity, and there should be a very high level of suspicion for presence or progression of systemic disease. If such a disease is properly and timely diagnosed, effective early systemic evaluation and therapy can be administered, and it is important to have initial general treatment as well as ophthalmic treatment. J Korean Ophthalmol Soc 2016;57(6):1012-1017

Bilateral simultaneous central retinal vein occlusion secondary to trauma

Retinal vein occlusion refers to the closure of the central retinal vein that drains the retina or to that of one of its branches. Central retinal vein occlusion (CRVO) is caused by age related changes in the retinal vessels. Bilateral CRVO is a relatively rare event. Only less than 10% cases are bilateral, simultaneous bilateral CRVO is very rare. We are presenting a case report regarding a middle aged patient with history of trauma who presented with bilateral CRVO in the depatment of ophthalmology, BIRDEM General Hospital, Dhaka. After detailed clinical work up and invesigation a diagnosis of bilateral CRVO secondary to trauma was made. DOI: http://dx.doi.org/10.3329/bmj.v43i1.21377 Bangladesh Med J. 2014 January; 43 (1): 33-35

Bilateral Central Retinal Vein Occlusion with Hyperhomo cysteinaemia, treated early with anti VEGF

Bilateral Central Retinal Vein Occlusion with Hyperhomo cysteinaemia, treated early with anti VEGF

Bilateral central retinal artery occlusion and vein occlusion complicated by severe choroidopathy in systemic lupus erythematosus

Severe retinal vascular occlusions resulting in blindness is a rare occurrence in patients with systemic lupus erythematosus (SLE). Herein, we report a case of a 33-year-old female who developed combined central retinal artery occlusion, retinal vein occlusion, and choroidopathy and rapidly became completely blind in both eyes within a week. The electroretinogram revealed a severely attenuated a-wave and b-wave, indicating a profound dysfunction of both choroidal and retinal circulation, respectively. The current case demonstrates objectively the functional impact of severe choroidopathy in SLE for the first time. Patients with unilateral blindness due to combined retinal/choroidal vascular obstructions should be monitored carefully to ensure adequate anticoagulant therapy in an attempt to guard the vision in the fellow eye.

SEQUENTIAL BILATERAL CENTRAL RETINAL VEIN OCCLUSIONS IN A CYSTIC FIBROSIS PATIENT WITH HYPERHOMOCYSTEINEMIA AND HYPERGAMMA-GLOBULINEMIA

To report a case of sequential bilateral central retinal vein occlusions in a cystic fibrosis patient with hyperhomocysteinemia and hypergamma-globulinemia over 6 years of follow up. Observational case report of one patient. A 31 year-old male with a history of cystic fibrosis presented with a central retinal vein occlusion (CRVO) in his left eye, followed by a CRVO in his right eye 4 years later. His medical workup was significant for elevated levels of homocysteine and gamma-globulins, which coincided with initiation of intravenous immunoglobulin (IVIG) proceeding his second CRVO. We describe a case of sequential bilateral central retinal vein occlusions in a cystic fibrosis patient with hyperhomocysteinemia and hypergamma-globulinemia over 6 years of follow up and discuss the important role of these risk factors in retinal venous occlusive disease.

Immunogammopathy maculopathy associated with Waldenström macroglobulinemia is refractory to conventional interventions for macular edema.

To describe immunogammopathy maculopathy in the setting of Waldenström macroglobulinemia and its lack of response to ocular treatment. Case report of a 65-year-old male patient who presented with a 4-month history of progressive vision loss in the left eye. Initial ophthalmic examination showed bilateral central retinal vein occlusion with macular edema, including serous macular detachment in the left eye. Serum protein electrophoresis revealed elevated IgM levels, leading to a diagnosis of Waldenström macroglobulinemia. The patient underwent plasmapheresis and systemic chemotherapy with improvement of hyperviscosity retinopathy. However, the macular detachment and edema in the left eye persisted despite repeated intravitreal injections of bevacizumab. Panretinal photocoagulation and intravitreal corticosteroid treatment were also unsuccessful in reversing the maculopathy. A diagnosis of immunogammopathy maculopathy was made, and partial improvement was seen over time after the treatment of Waldenström macroglobulinemia. In the setting of systemic immunogammopathy, conventional treatments for macular edema, including intravitreal anti-vascular endothelial growth factor agents and corticosteroids, may be ineffective. Ocular involvement responds to systemic chemotherapy and plasmapheresis directed against the underlying hematologic malignancy, although this response may be slow and incomplete.

Central retinal vein occlusion with depot medroxyprogesterone acetate use – an extremely rare complication

A 35-year old woman developed bilateral central retinal vein occlusion secondary to 3 years of DMPA use. The results of the investigations for inherited/acquired thrombophilia, diabetes and connective tissue disorders were normal. She was treated by a private ophthalmologist with aspirin, 100 mg daily. She made a complete recovery from bilateral central retinal vein occlusion. She was referred by a general practitioner to our gynaecology outpatient department clinic for decision on non-hormonal, contraceptive choices and she underwent laparoscopic sterilization procedure.

Chronic myeloid leukemia presenting with bilateral central retinal vein occlusion and massive retinal infiltrates

Visual impairment as the initial symptom of leukemia, especially chronic myeloid leukemia, is rare, occurring in approximately 3% of all leukemias in children. We describe the case of a 14-year-old boy with vision loss attributable to bilateral central retinal vein occlusion and massive retinal infiltrates. Bone marrow biopsy demonstrated the BCR-ABL translocation, and the patient was diagnosed with chronic myeloid leukemia. A major cytogenetic remission was achieved 6 months after initiation of therapy.

Bilateral central retinal vein occlusion in non-Hodgkin lymphoma and

The purpose of this study is to report bilateral central retinal vein occlusion in a patient with non-Hodgkin lymphoma and the effects of intravitreal bevacizumab injection. This is a case report of a patient seen and treated in the ophthalmology department of Ondokuz Mayis University Medical Faculty. Bilateral central retinal vein occlusion was observed in the patient because of hyperviscosity due to non-Hodgkin lymphoma which resulted in decreased visual acuity and increased macular thickness. Bilateral intravitreal bevacizumab injection increased best corrected visual acuity increased and reduced intraretinal, subretinal edema markedly. A hyperviscosity syndrome or inflammatory condition should be suspected when a patient, less than 50 years, has bilateral and simultaneous central retinal vein occlusion. Intravitreal bevacizumab injection is as an effective treatment for intraretinal and subretinal edema in central retinal vein occlusion secondary to non-Hodgkin lymphoma. J. Exp. Clin. Med., 2012; 29:80-82

Bilatéralisation à distance d’une occlusion de la veine centrale de la rétine associée à un syndrome des anti-phospholipides primaire

PurposeCase report of a young female patient presenting with bilateral sequential central retinal vein occlusion associated with primary antiphospholipid syndrome (APS), which rapidly improved with systemic anticoagulation. Case reportA complete ophthalmologic examination and hematological evaluation were performed on an 18-year-old female patient presenting with unilateral decreased visual acuity in the left eye, secondary to a central retinal vein occlusion. Three years later, she experienced a central retinal vein occlusion of the right eye. Hematologic evaluation revealed primary antiphospholipid syndrome. The patient was treated with systemic anticoagulation. ResultsOne month after the diagnosis and treatment of the central retinal vein occlusion in the left eye, the patient discontinued anticoagulant therapy, unfortunately resulting in severe visual loss. Three years later, upon recurrence in the fellow eye, systemic anticoagulation was reinstituted. Over 12 months follow-up, upon complete ophthalmologic examination, the patient showed normalization of all retinal signs. ConclusionIn the case of young patients with bilateral central retinal vein occlusions, hematologic evaluation should be performed so as to detect APS. Prompt, effective treatment is necessary to prevent further retinal damage. Recurrent thrombotic events may jeopardize anatomic and functional prognosis.

Bilateral central retinal vein occlusion in a patient with disseminated metastatic renal cell carcinoma treated with sorafenib.

The aim of the article is to report a case of a patient with disseminated metastatic renal cell carcinoma on long-term treatment with sorafenib who had developed bilateral central retinal vein occlusion over a period of 6 months. A 73-year-old man with decreased visual acuity in the right eye and central retinal vein occlusion in the left eye 6 months previously. The patient had been on long-term treatment with the antiangiogenic drug, sorafenib for disseminated metastatic renal cell carcinoma-a highly vascularized tumor. The physical examination and fluorescein angiography showed a thrombus in the central retinal vein in the right eye and evidence of a past thrombus in the central retinal vein in the left eye. Numerous avascular zones in the retinas of both eyes were seen on angiography. The patient was prescribed anticoagulant medication and laser photocoagulation of the retina in both eyes and transscleral cryoapplication in the right retina were performed. The treatment resulted in stabilizing the visual acuity (best-corrected visual acuity of the right eye was 0.01, best-corrected visual acuity of the left eye was 0.06) and decreasing the number and extent of avascular zones in the retinas of both eyes. No reduction of the chronic macular edema was achieved in the right eye. The patient with disseminated metastatic renal cell carcinoma on long-term sorafenib treatment should undergo periodic ophthalmologic examinations to exclude any potential vascular complications associated with cancer (vein occlusion associated with disseminated metastatic disease, tumor embolus, tumor thrombus) or with treatment (antiangiogenic drugs, steroids).

Catastrophic antiphospholipid antibody syndrome and cocaine abuse associated with bilateral retinal vascular occlusions.

To describe a case of bilateral central retinal vein occlusion and central retinal artery occlusion associated with antiphospholipid antibody syndrome and cocaine abuse. A single case report of a 44-year-old woman with a history of cocaine abuse, vasculitis, and 3 spontaneous abortions who developed painless complete loss of vision in both eyes concurrently with progressive motor and sensory polyneuropathy. The patient underwent an extensive laboratory, radiographic, ophthalmologic, and pathologic workup. Diagnosis of catastrophic antiphospholipid antibody syndrome was made based on established clinical and laboratory criteria, including a positive Russell's viper venom test, history of three spontaneous abortions and evidence of microvascular and macrovascular venous occlusions, and multiorgan failure. Urine toxicology screen was positive for cocaine metabolites. Her clinical course was remarkable for progressive mononeuritis multiplex and quadriplegia. She had no light perception on presentation, and fundus examination revealed extensive preretinal, intraretinal, and subretinal hemorrhages. Fluorescein angiography revealed complete occlusion of central retinal arteriolar and venular flow and retinal hemorrhages in both eyes. Bilateral central retinal vein occlusion and central retinal artery occlusion have been rarely reported in the literature and are often associated with underlying thrombotic risk factors, such as antiphospholipid antibody syndrome. Workup for underlying hypercoagulability and/or cocaine use should be considered in atypical and bilateral cases of central vein or artery occlusion. In this case, bilateral central retinal artery and vein occlusions developed as manifestations of catastrophic antiphospholipid antibody syndrome, which has not been previously reported.

Bilateral Central Retinal Vein Occlusion in Patient with Disseminated Intravascular Coagulation

Bilateral Central Retinal Vein Occlusion in Patient with Disseminated Intravascular Coagulation

Bilateral Central Retinal Vein Occlusion Caused By Malignant Hypertension in a Young Patient

Central retinal vein occlusion (CRVO) occurring simultaneously in both eyes is a rare condition, in such cases usually associated with severe systemic disease. Overall, only 10% of all CRVO patients are younger than 40 years. A relatively young patient reported with simultaneous bilateral CRVO associated with chronic kidney disease and malignant hypertension. A case of a 35-year-old male patient presented with the complaint of decreased vision in both eyes. Ophthalmic examination revealed bilateral CRVO. Chronic kidney disease leading to malignant hypertension and hyperviscosity was diagnosed as the underlying cause. Clinical support and medical therapy effectively controlled the hypertension, leading to improvement of both the systemic and ocular changes. To the best of our knowledge, this is the first case report of simultaneous bilateral CRVO in a young patient associated with chronic renal failure and malignant hypertension. Primary care providers, either ophthalmologists or physicians, need to consider both common and atypical risk factors for retinal vein occlusion, in order to prevent further ocular morbidity and systemic complications.

CENTRAL RETINAL VEIN OCCLUSION IN A YOUNG CHINESE POPULATION

The purpose of this study was to assess the risk factors for central retinal vein occlusion and associated morbidity and mortality in a Chinese population. The participants included patients with central retinal vein occlusion 40 years old and younger. Predisposing factors, mortality, and systemic complications were examined in this group. Unilateral (n = 19) and bilateral (n = 3) central retinal vein occlusions were identified in a total of 22 patients (25 eyes), with a mean follow-up time of 37 months. Hypercholesterolemia (65%), hypertriglyceridemia (64%), and hyperhomocysteinemia (42%) were all identified as risk factors. Three patients (14%) developed stroke and 1 (5%) developed transient ischemic attacks during follow-up. Renal failure and pulmonary hypertension resulted in the death of two patients. The mean initial and final visual acuities (+ or - standard deviation) were 20/400 (+ or - 20/250) and 20/500 (+ or - 20/320), respectively, and treatments did not result in vision improvement (P = 0.57). The poor visual prognosis was likely due to macular edema in 7 eyes (28%), optic atrophy in 4 eyes (16%), and secondary glaucoma in 3 eyes (12%). Morbidity and mortality are high in young Chinese patients with central retinal vein occlusion who may have associated serious complications including stroke, blindness, and death. Central retinal vein occlusion may serve as an initial clinical presentation of serious systemic diseases.

Central retinal vein occlusion after liver transplantation

Editor, Central retinal vein occlusion (CRVO) is associated with various systemic abnormalities (McGrath et al. 1978). In organ recipients, however, CRVO has been reported only after heart or heart–lung transplantation (Keller & Linn 1990; Allinson et al. 1993). To our knowledge, this is the first report of CRVO in liver transplant (LT) recipients. From 1995 to 2006, 3656 organ transplantations were performed at Asan Medical Centre (1290 liver, 786 bone marrow, 1445 kidney and 135 heart transplants). Of these patients, 1205 were examined by the Department of Ophthalmology. The medical records of these 1205 patients were reviewed retrospectively. CRVO was diagnosed in five patients, all LT recipients (Table 1). Interestingly, one patient developed sequential CRVO in both eyes following two LTs (1, 2). Fundus photographs of both eyes of patient 4 at first presentation. Features of venous tortuosity, macular thickening and intraretinal haemorrhage were observed in all four quadrants of the fundus of the right eye. There were no specific focal lesions, except small atrophic areas nasal to the macula, in the left eye. (A) Fundus photographs of the same patient at second presentation. There was a new onset of venous tortuosity, cotton-wool spots, retinal haemorrhages and macular oedema with ischaemia in the left eye. (B) Fundus photographs and Fluorescein angrogram (FA) of both eyes 6 months later. Severe ischaemia and non-perfusion were observed in the left eye. CRVO developed 3–40 months (mean 15 months) after LT. Haematological examination showed alterations in blood coagulability secondary to hepatic failure, normalization of absolute platelet count, INR and activated partial thromboplastin time. Increases in factor VIII and activated protein C were observed in the patient with bilateral CRVO (patient 4). A mild increase in factor VIII (195%, reference value 60–120%) was also observed in patient 5. Among the six affected eyes of these five patients, four developed non-perfused CRVO and two developed perfused CRVO, with one of the latter converted to non-perfused CRVO during follow-up. Despite aggressive treatment, including laser photocoagulation, vitrectomy and glaucoma surgery, final visual acuity equal to or worse than 20/200 occurred in three of the six eyes. CRVO has been found to be a complication of heart and lung transplantations (Keller & Linn 1990; Allinson et al. 1993), with cardiopulmonary bypass during heart–lung transplantation associated with factors predisposing to CRVO development. Pulmonary thromboembolism and/or intracardiac thrombus formation have been reported during LT or immediately after graft reperfusion (Gologorsky et al. 2001; Lerner et al. 2005). Among the factors inherent to LT that may be related to these major intraoperative complications are excessive activation of the coagulation system secondary to injury to a large capillary bed, venous stasis during clamping of the portal vein and inferior vena cava, activators released from the grafted liver and massive blood transfusion (Gologorsky et al. 2001). It is unclear, however, why CRVO only developed several months to years after transplantation and exclusively in LT recipients, although CRVO may have been caused by hypertension and/or hyperlipidaemia secondary to the large amount and duration of immunosuppressive therapy administered to LT recipients. Although the absolute levels of serum lipids, including cholesterol, before LT were within reference values in four of our five patients, these serum lipid levels increased markedly after LT. These patients had also received more combination treatments, including prophylaxis for opportunistic infections, and large quantities of transfused blood during the immediate postoperative period. This may be especially applicable to the patient with bilateral CRVO, who was also the only patient treated with interferon α-2b because of chronic hepatitis C. In addition, LT may induce hypercoagulability or relative hypercoagulability, especially because these patients had experienced decreases in platelets and coagulation factors caused by liver failure. The patient with bilateral CRVO was thrombophilic, as evidenced by elevated factor VIII and activated protein C. Factor VIII tested immediately after the onset of CRVO in patient 5 increased slightly. Although her fibrinogen (345 mg/dl) and antithrombin III (96%) levels at the onset of CRVO were within normal limits, they were very low before LT (104 mg/dl and 9%, respectively). CRVO could occur during follow-up in LT recipients. It may be the main cause of morbidity of patients who showed general recovery after successful LT, warranting familiarity with this complication. It is important to determine the risk factors for the development of CRVO in LT recipients, as well as possible preventative measures.