Activated protein C resistance in young adults with central retinal vein occlusion.

BACKGROUND: Central retinal vein occlusion is a disease that is most common in old people. While the disease in old people often is associated with atherosclerosis, hypertension, diabetes, or glaucoma, this is much less evident in young people. However, a new defect in the anticoagulant system has recently been discovered, activated protein C resistance. This hereditary defect may well be associated with central retinal vein occlusion, and so this factor was analysed in patients younger than 50 years with a history of central retinal vein occlusion. METHODS: Blood samples were obtained from 31 patients younger than 50 years with a history of central retinal vein occlusion, and analysed for activated protein C resistance with standard clinical laboratory methods. RESULTS: In this material 26% of all the patients and 36% of the patients younger than 45 years were resistant to activated protein C. The normal incidence of activated protein C resistance is 2-7%. CONCLUSION: Activated protein C resistance seems to be the most common known cause of central retinal vein occlusion in young people.     

Activated protein C resistance in patients with central retinal vein occlusion

AIM/BACKGROUND—A new defect in the anticoagulant system has recently been discovered—activated protein C resistance. The frequency of this disorder has been shown to be increased in young patients (<50 years of age) with central retinal vein occlusion. This study was carried out to determine if there was any overrepresentation of activated protein C resistance in patients >50 years of age with central retinal vein occlusion.
METHODS—Blood samples were obtained from 83 patients >50 years of age and with a history of central retinal vein occlusion. The blood samples were analysed for activated protein C resistance with standard clinical laboratory methods.
RESULTS—In this material 11% of the patients were resistant to activated protein C. The normal incidence of activated protein C resistance in the same geographical area is 10-11%.
CONCLUSION—Activated protein C resistance does not seem to be a cause of central retinal vein occlusion in people older than 50 years.

     

Cilioretinal artery occlusion in young adults with central retinal vein occlusion

Ten patients, all younger than 50 years of age, had a temporal cilioretinal artery occlusion associated with a nonischemic central retinal vein occlusion. On fluorescein angiography, the cilioretinal artery eventually filled in all but one eye. The cilioretinal artery showed pulsations on fluorescein angiography in five eyes. The central retinal vein occlusion eventually resolved and the fundus assumed a normal appearance in all nine of the followed cases. Eight of nine eyes that underwent follow-up examination had final visual acuity of 20/30 or better. The occlusion of the central retinal vein produces an elevation of intraluminal capillary pressure because the central retinal artery continues to pump blood into the retina. Because the perfusion pressure of the cilioretinal artery is lower than the central retinal artery, it becomes relatively occluded. The prognosis for these patients is generally good unless the entire parafoveal capillary net is affected by the cilioretinal artery that is occluded.     

Central retinal vein occlusion in young people

In a study performed on 20 subjects with central retinal vein occlusion (CRVO) aged 40 years or less we found the ischemic form in 20%. Disc edema was a common finding at the onset, while macular edema was less frequently seen. Systemic or ocular disorders that could be related with the development of the CRVO were often found; a patient was affected with myasthenia gravis and another with Sturge-Weber syndrome: these two diseases were not previously reported in association with CRVO. In only two of the 11 patients followed-up the visual acuity improved. The visual prognosis in CRVO of young people is often poor; the more frequent cause of the reduced visual acuity is chronic cystoid macular edema.     

Central retinal vein occlusion in young adults

Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease. CRVO can be seen in young adults, and although it is occasionally associated with a systemic disease, in the majority of cases it occurs in an otherwise healthy patient with no known systemic disease or ocular problem. Inflammation of the central retinal vein has been proposed as a cause of the occlusion in young adults and for that reason it has been called papillophlebitis. The appearance of unilateral optic disc edema, dilatation, and tortuosity of the major retinal veins with a variable amount of retinal hemorrhage in young, healthy adults with complaints of blurred vision or photopsias has been called, in addition to papillophlebitis, benign retinal vasculitis, optic disc vasculitis, nonischemic CRVO, big blind spot syndrome, and presumed phlebitis of the optic disc. An approach to the diagnostic evaluation of the young adult with CRVO is presented. Although most eyes recover vision to better than 20/40, about one-fifth have significant visual loss, and many suffer ocular sequelae. Many treatment modalities have been tried for this entity, but no conclusive evidence exists that any treatment alters its natural history.     

Activated protein C resistance and anticoagulant proteins in young adults with central retinal vein occlusion

BACKGROUND: Central retinal vein occlusion is a disease that is most common in old people, and often associated with atherosclerosis, hypertension, diabetes or glaucoma. Since these diseases are much less evident in young people, we wanted to investigate the prevalence of disorders in the most common anticoagulant proteins in a group of young patients with central retinal vein occlusion. METHODS: 37 consecutive patients younger than 50 years and with a history of central retinal vein occlusion, were analysed for deficiencies of natural inhibitors of coagulation (protein C, S, and antithrombin III), plasminogen, resistance to activated protein C, and the presence of anticardiolipin or lupus anticoagulants. RESULTS: Anticoagulant protein deficiencies were found in 4 patients (11%) and activated protein C resistance in 7 patients (19%). Anticardiolipin or lupus anticoagulants were not found in the patients. CONCLUSION: Activated protein C resistance and anticoagulant protein deficiencies seem to be important factors to the etiology to central retinal vein occlusion in young patients.     

Central retinal vein occlusion in a young adult

This report describes a case of central retinal vein occlusion (CRVO) which was classified as papillophlebitis in a young female adult. In this age group, CRVO is relatively rare and tends to be mild in both its short-term and long-term visual consequences. The patient in this case showed a concurrent episode of bilateral intra-ocular pressure (IOP) elevation and the presence of cilio-retinal arteries in the affected eye. Despite extremely poor vision at presentation and the poor prognostic sign of cilio-retinal arteries, the patient made an excellent visual recovery with only mild paracentral field damage. (Clin Exp Optom 1995; 78: 2: 60–64)     

Multifocal electroretinograms in patients with retinal vein occlusion

PURPOSE: To investigate waveform changes of multifocal electroretinograms(mERG) in patients with retinal vein occlusion(RVO). METHODS: Nine eyes of 8 patients with RVO and 29 eyes of 29 normal subjects were examined using mERG. An array of 103 hexagonal elements was displayed on a monitor. mERG latencies(ms) and response densities (nV/deg2) were measured for center area(Ct) and for each of four quadrant areas. The peak and troughs were named N1, P1 and N2, consecutively. RESULTS: In pathological quadrants, although the response densities were abnormal in only one eye, latencies of the N2 and P1 were prolonged in 7 eyes and in 2 eyes, respectively(over 1 SD-2 SD). The latencies were significantly prolonged compared with those of normal eyes(p < or = 0.03, U-test). On the other hand, in the central area, although the latencies were abnormal in 3 eyes, the response densities were reduced in 6 eyes(over 1 SD-2 SD). The response densities were significantly reduced compared with those of normal eyes(p < or = 0.024, U-test). CONCLUSIONS: Both peak latencies in pathological quadrants and response densities in the central retinal area can be sensitive indicators of retinal dysfunction caused by RVO. The mERG system is useful for detecting local retinal dysfunction in patients with RVO.     

Baseline clinical features predict visual outcome in young patients with central retinal vein occlusion

Graefe's Archive for Clinical and Experimental Ophthalmology - To evaluate prognostic factors in young patients with central retinal vein occlusion (CRVO). Retrospective case series. CRVO...     

Hyperhomocysteinemia in central retinal vein occlusion in young adults

PURPOSE: Several investigators have tried to assess the role of hyperhomocysteinemia and the 677C-T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene as risk factors in retinal vein occlusion with contrasting results. Aim of the study is to investigate the correlation between increased homocysteine plasma level and the homozygosity for the 677C-T mutation in the gene MTHFR in patients aged under 50 years affected by central retinal vein occlusion (CRVO). METHODS: Through a prospective, case-control study, 31 patients under 50 years of age and diagnosed with CRVO were compared with two control groups. The first control group (GROUP I) included 31 subjects matched for age, sex, laboratory tests and the main risk factors for atherosclerosis. The second control group (GROUP II) consisted of 31 volunteers matched only for age and sex. RESULTS: The mean homocysteine plasma level was 10.60 micromol/l in patients, 10.39 micromol/l in GROUP I and 9.34 micromol/l in GROUP II. There was no statistically significant difference between mean homocysteine plasma level in cases and in GROUP I. Mean homocysteine plasma level was lower in GROUP II than in patients, and the difference was statistically significant. Homozygosity for the 677C-T mutation in the MTHFR was found in four patients (12.9%), in five controls in GROUP I (16.1%) and in four controls in GROUP II (12.9%). CONCLUSION: Our results support first of all the hypothesis that the homocysteine plasma level is not a primary and independent risk factor for central retinal vein occlusion, but is more likely a marker of atherosclerosis and the consequence of other well-established risk factors. Second, the importance of the design of the study is highlighted, since the obtained results differed on the basis of the considered control group. This feature could contribute to explain the contradictory results previously reported in the literature.     

Hyperhomocysteinemia in central retinal vein occlusion in young adults

Purpose. Several investigators have tried to assess the role of hyperhomocysteinemia and the 677C-T mutation in the methylenetetrahydrofolate reductase (MTHFR) gene as risk factors in retinal vein occlusion with contrasting results. Aim of the study is to investigate the correlation between increased homocysteine plasma level and the homozygosity for the 677C-T mutation in the gene MTHFR in patients aged under 50 years affected by central retinal vein occlusion (CRVO). Methods. Through a prospective, case-control study, 31 patients under 50 years of age and diagnosed with CRVO were compared with two control groups. The first control group (GROUP I) included 31 subjects matched for age, sex, laboratory tests and the main risk factors for atherosclerosis. The second control group (GROUP II) consisted of 31 volunteers matched only for age and sex. Results. The mean homocysteine plasma level was 10.60µmol/l in patients, 10.39µmol/l in GROUP I and 9.34µmol/l in GROUP II. There was no statistically significant difference between mean homocysteine plasma level in cases and in GROUP I. Mean homocysteine plasma level was lower in GROUP II than in patients, and the difference was statistically significant. Homozygosity for the 677C-T mutation in the MTHFR was found in four patients (12.9%), in five controls in GROUP I (16.1%) and in four controls in GROUP II (12.9%). Conclusion. Our results support first of all the hypothesis that the homocysteine plasma level is not a primary and independent risk factor for central retinal vein occlusion, but is more likely a marker of atherosclerosis and the consequence of other well-established risk factors. Second, the importance of the design of the study is highlighted, since the obtained results differed on the basis of the considered control group. This feature could contribute to explain the contradictory results previously reported in the literature.     

Diurnal intraocular pressure in young adults with central retinal vein occlusion

In the older population, there is a well-known relationship between central retinal vein occlusion (CRVO) and glaucoma and ocular hypertension. In young adults, CRVO is a rare occurrence, the cause of which is not well understood. Seven patients under the age of 36 years with CRVO and no associated systemic disease underwent modified diurnal intraocular pressure (IOP) measurements (8:00 AM to 11:00 PM). Abnormal IOPs were found in the affected and/or the unaffected fellow eyes. To the authors' knowledge, this is the first report implicating abnormal IOP as an etiologic factor in the development of CRVO in young adults. Only with diurnal IOP measurements were the elevated swings and peak IOPs detected. This finding suggests that abnormal IOP may be an important factor in the development of CRVO in young adults.     

Choroidal thickening in retinal vein occlusion patients with serous retinal detachment

Graefe's Archive for Clinical and Experimental Ophthalmology - To evaluate choroid thickness and macular retinal metrics in treatment naïve retinal vein occlusion (RVO) patients with...     

Central retinal vein occlusion in a young patient with seropositive syphilis

Central retinal vein occlusion (CRVO) is a common retinal vasculopathy typically affecting adults in the fifth to seventh decade of life. Systemic disease, particularly hypertension, is often a contributing factor in this sight-threatening condition. CRVO in young adults, however, is an uncommon occurrence with relatively few reported cases in the ophthalmic literature. Two studies performed on young adults (less than 40 years of age) presenting with CRVO revealed that, in most cases, there was not a strong correlation with hypertension or other systemic diseases. In more severe cases, namely those with poor visual outcome from the ischemic type of CRVO, there was a strong correlation with cardiovascular disease and diabetes mellitus. Systemic inflammatory conditions represent a small contributing factor in patients presenting with CRVO. This paper reports on a 21-year-old female with non-ischemic CRVO who was serologically positive for syphilis.     

Cardiovascular risk assessment in patients with retinal vein occlusion

AIM: Patients with retinal vein occlusions (RVO) are at increased risk of cardiovascular disease (CVD). The risk of future CVD was determined using the Framingham algorithm and this risk estimate was used to guide decisions about preventative treatment for CVD in RVO patients. METHODS: 107 unselected RVO patients were studied. After excluding 18 patients because of age, missing data, or pre-existing cardiovascular disease, the calculated coronary heart disease risks (cCHDR) and calculated cardiovascular disease risks (cCVDR) were calculated on the 89 remaining and compared with both the standardised risk and the published incidence of CHD in England by t test or chi(2) test. RESULTS: The mean 10 year cCVDR was significantly higher than the Framingham standardised risk for all RVOs (20.6% (1.2%) v 15.7% (1.1%), p = 0.009) and female RVOs (17.8% (1.2%) v 12.7% (1.0%), p = 0.022) in particular. The 10 year cCHDR, compared to the actual incidence of CHD in England between the ages of 30 and 74 years, was > 15% in twice as many males than expected (62% v 28%, p <0.0001). This rose to almost six times when cCHDRs greater than 30% were compared (17% v 3%, p = 0.002). There was a fourfold increase in the proportion of female RVO patients with a cCHDR above 15% (40% v 9%, p <0.0001) and at a cCHDR of 30% and above (10% v 0%, p = 0.004). There were also significant differences in the cCHDR between central and branch RVO (both sexes). The branch form of RVO (BRVO) having higher cCHDRs because of systolic hypertension (164.1 (21.6) mm Hg v 149.5 (23.5) mm Hg, p = 0.003) and age (61.7 (8.3) years v 56.7 (10.6) years, p = 0.017). CONCLUSIONS: RVO is the presenting complaint in a group of patients at increased risk of CVD and is in agreement with the long term follow up data demonstrating an increased mortality from CVD in patients with RVO. The Framingham algorithm can accurately determine the cCHDR (or cCVDR) to assist the clinician in deciding who to treat in accordance with the Joint British Societies' guidelines, with particular regard to hypertension, lipid lowering, and the use of aspirin therapy.     

Activated protein C resistance and lipoprotein (a) in young adults with branch retinal vein occlusion

We investigated the effects of activated protein C resistance (APCR), Factor V Leiden (FVL) mutation, and high lipoprotein (a) levels in 32 young patients with branch retinal vein occlusion (BRVO) vs 30 controls. No difference between patients with BRVO and controls was found with regard to APCR, FVL mutation, or lipoprotein (a) levels. These factors do not seem important in the etiology of BRVO. The authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or provider of services discussed in this article. The authors also do not discuss the use of off-label products, which includes unlabeled, unapproved, or investigative products or devices.     

Severe ischemic process in a young man with central retinal vein occlusion

A 32-year-old man with central retinal vein occlusion followed by severe and rapidly progressing neo-vascular glaucoma is presented. This case was characterized by a severe ischemic process, in which the rubeosis iridis was followed by almost total atrophy of the iris within a short period of time. The onset of the central retinal vein occlusion was associated with mild dehydration and stress polycythemia following strenuous physical activity.