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.     

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.     

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.     

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.     

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.     

眼压在视网膜静脉阻塞中的意义

目的:探讨眼压在视网膜静脉阻塞中的意义。方法:应用TX-F眼压计对23例23眼,视网膜静脉阻塞(retinal vein occlusion,RVO)患者的眼压进行测量。对照组与病例组年龄和性别相配,例数相等。结果:18例RVO阻塞眼眼压较对侧眼有不同程度降低。RVO阻塞眼眼压显著低于对侧眼及正常对照眼(P<0.01)。结论:RVO可引起患者眼压降低。     

Long-term follow-up of occlusion of the central retinal vein in young adults

We did a long-term follow-up study of 42 patients aged 40 years and younger who had occlusion of the central retinal vein in order to learn its course and determine the frequency of related systemic disorders. Two groups of venous occlusion (complete and incomplete) were delineated by initial ocular findings. Final visual prognosis could not be predicted by the severity of the venous occlusion at the time of diagnosis. The presence of anomalous disk vessels closely correlated with a favorable prognosis in patients who had complete occlusion of the central retinal vein. None of the patients with incomplete central vein occlusion developed neovascular glucoma; three (14%) of the 21 patients with complete venous occlusion developed neovascular glucoma, which resulted in enucleation. Significant associated systemic maladies included cardiovascular disease and diabetes mellitus. An apparent correlation exists between occlusion of the central retinal vein and early death.     

眼压和轴长在视网膜中央静脉阻塞中的意义

目的　探讨眼压和轴长在视网膜中央静脉阻塞中的意义。方法　应用TX- 10眼压计对4 6例(46只眼) CRVO患者的眼压进行测量。并使用A型超声仪对其中16例CRVO眼轴进行测量,对照组与病例组年龄和性别相配,例数相等。结果　36例CRVO阻塞眼眼压较对侧眼有不同程度降低。CRVO阻塞眼眼压显著低于对侧眼及正常对照眼(P <0 .0 1)。CRVO阻塞眼轴长显著短于对侧眼及正常对照眼(P <0 .0 1)。结论　CRVO可引起眼压降低,短眼轴是CRVO的危险因素。     

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 young adults (papillophlebitis).

We performed a retrospective study of 103 cases of central retinal vein occlusion (CRVO) in young, nondiabetic adults that were followed for at least six months. Of these patients, 64% were men and 36% were women. While visual acuity was usually good, 33 eyes (32%) had a final visual acuity of 20/200 or worse, including 6 eyes (6%) with a final visual acuity of no light perception. Ocular complications included chronic cystoid macular edema, macular pigmentary changes (37%), sheathing of retinal vessels (22%), venous collaterals of the disc (33%), macular hole formation (1%), neovascularization of the disc (1%), retina (1%), and iris (19%), neovascular glaucoma (8%), and vitreous hemorrhage (7%).     

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.     

Intraocular pressure abnormalities associated with central and hemicentral retinal vein occlusion

OBJECTIVE: To evaluate the prevalence of ocular hypertension (OHT) and glaucoma in patients with central retinal vein occlusion (CRVO) and hemi-CRVO (HCRVO) and of the fall in intraocular pressure (IOP) secondary to CRVO/HCRVO. DESIGN: Nonrandomized comparative case series. PARTICIPANTS AND METHODS: We investigated 674 consecutive patients who were initially seen with unilateral CRVO (n = 548) and HCRVO (n = 126) at their onset, with a normal fellow eye. The fellow uninvolved eye in each patient acted as a control. Central retinal vein occlusion and HCRVO were categorized into nonischemic and ischemic. At all visits, patients had a detailed ocular history, as well as a thorough bilateral ocular evaluation, including IOP recording with a Goldmann applanation tonometer; when the diagnosis of OHT or glaucoma was initially uncertain, the 24-hour diurnal IOP was recorded. The observed prevalence rates of OHT and glaucoma among patients with CRVO and HCRVO were compared with those in the general population. MAIN OUTCOME MEASURES: The prevalence of OHT and glaucoma, and of ocular hypotension secondary to CRVO/HCRVO. RESULTS: The overall prevalence of glaucoma was 9.9% and of OHT 16.2%. The prevalence of glaucoma/OHT was found to be significantly (P<0.0001) higher in patients with CRVO and HCRVO than in the general population. There was no significant difference in the proportion of patients with glaucoma/OHT among the various types of CRVO/HCRVO (P = 0.156). Forty-eight percent of all patients had lower IOP (>/==" BORDER="0">2 mmHg) in the CRVO/HCRVO eye than in the fellow (uninvolved) eye at their initial evaluation. The prevalence of ocular hypotension was significantly (P<0.0001) higher in patients with glaucoma/OHT not on ocular hypotensive therapy than in patients without glaucoma. Among the patients without glaucoma, the prevalence of ocular hypotension differed significantly among the various types of CRVO/HCRVO (P = 0.007). CONCLUSIONS: Central retinal vein occlusion and HCRVO have a significant association with glaucoma and OHT and with a subsequent fall in IOP in the involved eye. Few patients with CRVO/HCRVO have high IOP in the involved eye, although many of them do have it in the fellow uninvolved eye. It is important to exclude glaucoma/OHT in the fellow eye of any patient with CRVO/HCRVO; if present, elevated IOP should be treated to reduce the risk of that eye developing (1) CRVO/HCRVO and (2) glaucomatous damage. There may be no benefit to prescribing IOP-lowering drops for involved eyes whose IOP is already normal.     

Ophthalmodynamometric assessment of the central retinal vein collapse pressure in eyes with retinal vein stasis or occlusion

PURPOSE: Using a new Goldmann contact lens associated ophthalmodynamometric device, it was the purpose of the present study to determine the central retinal vein collapse pressure in eyes with retinal vein occlusions or retinal venous stasis. METHODS: The prospective clinical non-interventional comparative study included 19 patients with central retinal vein occlusion ( n=8), branch retinal vein occlusion (n=4), or retinal venous stasis (n=7) and 42 subjects of a control group. With topical anesthesia, a Goldmann contact lens fitted with a pressure sensor was put onto the cornea. Pressure was exerted on the globe by pressing the contact lens, and the pressure value at the time when the central retinal vein started pulsating was noted. RESULTS: Central retinal vein collapse pressure measured 103.6+/-25.4 arbitrary units (AU) in eyes with central retinal vein occlusion what was significantly higher than in the eyes with retinal venous stasis (58.1+/-37.5 AU; p=0.02) and the eyes with branch retinal vein occlusion (43.8+/-25.5 AU; p=0.004). In the latter two groups, the measurements of the central retinal vein collapse pressure were significantly (p<0.001) higher than the measurements in the eyes of the control group (4.2+/-7.8 AU). CONCLUSION: As measured by a new ophthalmodynamometer with direct biomicroscopic visualization of the central retinal vessels during examination, central retinal vein collapse pressure is significantly higher in eyes with central retinal vein occlusion, followed by eyes with branch retinal vein occlusion, eyes with retinal venous stasis and, finally, normal eyes. These findings may have diagnostic and therapeutic implications.     

Familial central retinal vein occlusion

AIM: To report four cases of central retinal vein occlusion (CRVO) in a French family. PATIENTS AND METHODS: Ophthalmological examination and medical work-up of seven members of the family. RESULTS: There were four cases of CRVO in two consecutive generations. Three of them had CRVO in both eyes. Arterial hypertension was present in two, associated to glaucoma in one. Medical work-up did not reveal additional risk factors. CONCLUSIONS: We report a case of familial clustering of CRVO. Our cases combined to other cases reported in the literature provide arguments for the existence in some subjects of a genetic predisposition of CRVO. Additional case series are however needed to confirm this hypothesis.     

Cilioretinal artery occlusion with central retinal vein occlusion.

BACKGROUND: Combined cilioretinal artery and retinal vein occlusions are infrequently documented retinal vascular disorders of speculative origin. Occlusion of the cilioretinal artery is believed to result from either mechanical compression of the artery as a result of an increase in venous pressure or from a reduction in perfusion pressure in both the cilioretinal and retinal arteries. The ophthalmoscopic and angiographic features of this condition are reviewed. CASE REPORTS: Two cases of cilioretinal artery occlusion after central retinal vein occlusion are presented, one of which evolved to the development of iris neovascularization. DISCUSSION: The incidence of cilioretinal artery occlusions due to central retinal vein occlusions is infrequently reported in the literature. Excluding those with chronic cystoid macular edema, most patients have a favorable visual outcome. It is possible that the incidence of combined cilioretinal artery and central retinal vein occlusions is grossly underestimated.     

Electroretinography in central retinal vein occlusion

In 149 eyes with central retinal vein occlusion (CRVO), we prospectively investigated the role of routine, clinical electroretinography (ERG) in differentiating ischemic (60 eyes) from nonischemic CRVO (89 eyes). Single-flash photopic and scotopic ERGs were recorded. Data for the amplitudes and implicit times of a- and b-waves and for the b-/a-wave amplitude ratio were analyzed in detail. The study revealed that the best ERG parameter (for both photopic and scotopic ERG) for differentiating ischemic from nonischemic CRVO was a subnormal b-wave amplitude (reduced to 60% or by 1 SD from the normal mean value, or 64%–69% of that in the fellow normal eye), with a sensitivity of 80%–90% and a specificity of 70%–80%. ERG findings were correlated with the relative afferent pupillary defect (RAPD). An RAPD of 0.7 log units showed a sensitivity of 88% and a specificity of 90% in differentiating ischemic from nonischemic CRVO. ERG and RAPD findings showed a good correlation. The combined ERG and RAPD tests could differentiate 97%–100% of ischemic from nonischemic CRVO cases, with a specificity of about 70%.Presented in part at the Annual Meeting of the Association for Research in Vision and Ophthalmology, Sarasota, Florida, USA, 2 May 1988Supported by grant EY-1151 from the National Institutes of Health, and in part by unrestricted grants from Research to Prevent Blindness, Inc., and from Alcon Research Institute     

Epidemiology of retinal vein occlusion and its association with glaucoma and increased intraocular pressure

A population-based study found an overall incidence rate of symptomatic retinal vein occlusion (RVO) in a 4-year period to be 2.14 per 1,000 in the 40 years and over age group. When cases found among glaucoma clinic patients were separated from the remainder of the population there was marked difference in the incidence rate of RVO in the same time period (1.85 and 17.3 per 1,000, respectively). The rate of RVO increased significantly (p less than 0.001) with age in the general population from 0.93 per 1,000 among persons under 64 years of age to 5.36 per 1,000 among persons over 65. The increase in the rate of RVO by age was less dramatic in the glaucoma clinic population. The two populations also differed in the frequency of the occlusion type: the ratio of the rate of branch vein occlusion to central vein occlusion was 3.2:1 in the general population, but equally distributed in the glaucoma clinic population. Persons with increased intraocular pressure and/or glaucoma were found to have a higher prevalence of RVO than persons with no history of elevated intraocular pressure.