Two cases of frosted branch angiitis with central retinal vein occlusion.

BACKGROUND: Frosted branch angiitis usually occurs in children, and has a good prognosis. We report two cases of unilateral frosted branch angiitis in adults. Both had poor visual outcomes because of associated central retinal vein occlusion and neovascular glaucoma. CASES: Case 1 was a 36-year-old woman. Almost all retinal veins and some retinal arteries showed vasculitis in her right eye, and veins were slightly dilated and sheathed. Case 2 was a 23-year-old woman. Angle hypopyon was observed in her left eye. Retinal veins were dilated, meandering, and sheathed. Retinal hemorrhages were also observed. In both cases, after systemic steroid therapy the retinal vasculitis gradually decreased, but central retinal vein occlusions gradually developed. Despite systemic administration of urokinase and panretinal photocoagulation, neovascular glaucoma developed, and visual acuity diminished in both cases. CONCLUSIONS: Two cases of frosted branch angiitis complicated by retinal vein occlusion are reported. Careful observation of retinal blood flow is necessary in frosted branch angiitis in adults.     

Retinal periphlebitis resembling frosted branch angiitis with nonperfused central retinal vein occlusion

PURPOSE: To report the unusual association between severe retinal periphlebitis resembling frosted branch angiitis and nonperfused central retinal vein occlusion (CRVO). METHODS: Observational case reports. RESULTS: Patient 1 was a 28-year-old man who presented with extensive sheathing involving all retinal veins in one eye followed by nonperfused CRVO. Twenty-seven months after initial presentation, he developed perfused CRVO in the other eye followed by periphlebitis that progressed into nonperfused CRVO. Patient 2 was a 47-year-old man who presented with unilateral severe retinal periphlebitis associated with nonperfused CRVO. Despite systemic administration of corticosteroid therapy, rubeosis iridis developed in both patients and neovascular glaucoma developed in Patient 1 despite full panretinal photocoagulation. Extensive systemic workup and coagulation studies were unremarkable except for the presence of antiphospholipid antibodies in both patients and elevated plasma homocysteine level in Patient 2. CONCLUSIONS: Severe retinal periphlebitis complicated by nonperfused CRVO is associated with poor visual outcome despite appropriate medical and surgical treatment.     

Recurrent unilateral frosted branch arthritis

In the left eye of a 27-year-old man we found perivascular creamy sheathing of retinal veins with retinal hemorrhages and, on fluorescein angiography, delayed filling of veins with late leakage. Dramatic recovery of visual acuity and healing of retinal lesions followed intravenous corticosteroid therapy. However, the condition recurred several times within a few months. Fluorescein angiography showed delayed filling of arteries and veins and arteriovenous anastomoses with a widespread capillary nonperfusion area. Eventually, neovascular glaucoma resulted. It is suggested that frosted branch angiitis is related to vascular occlusion. Systemic corticosteroid therapy seems to affect the course of this disease.     

Neovascular glaucoma associated with cilioretinal artery occlusion combined with perfused central retinal vein occlusion

BACKGROUND: Cilioretinal artery occlusion rarely results in neovascular glaucoma, especially in cases of extensive cilioretinal infarction and combined retinal vascular occlusion. CASE: A 62-year-old man with diabetes mellitus and essential hypertension showed a visual acuity of counting fingers, retinal whitening temporal to the optic disc with mild dilation and tortuosity of the retinal veins, and retinal hemorrhages in four quadrants of his right eye. Fluorescein angiography demonstrated a delayed filling of the central retinal vein and cilioretinal artery. OBSERVATIONS: Two months later, neovascular glaucoma developed and retinal ablation was performed using an argon laser. Trabeculectomy was also performed due to the intractability of the glaucoma, and central artery occlusion was found. On magnetic resonance angiography, the right distal common carotid artery was irregularly narrowed and the right ophthalmic artery was almost entirely occluded. CONCLUSIONS: In cases of cilioretinal artery occlusion and perfused central retinal vein occlusion with multiple risk factors, close follow-up is advised.     

A case of frosted branch angiitis.

We report a case of frosted branch angiitis in a 16-year-old-girl. She noted a sudden and severe visual disturbance in both eyes, without other systemic symptoms. Diffuse retinal oedema and unusual sheathing of retinal veins were characteristic in both fundi. Fluorescein angiography showed no occlusion of the sheathed retinal veins, but some paravenous extravasation of dye was found in the late phase. With high doses of systemic corticosteroids her fundi and visual acuity improved greatly, though the vessels continued to show severe narrowing. At three months the pattern visually evoked cortical potentials were found to be normal, while flash electroretinograms were absent. No systemic abnormality has been found to explain the aetiology of this condition.     

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.     

Central retinal vein occlusion during remission of ulcerative colitis.

BACKGROUND: Retinal vascular disease is a rare complication of ulcerative colitis. CASE: We report a patient who developed unilateral nonischemic central retinal vein occlusion (CRVO) (papillophlebitis) without any other retinal vascular disease during remission of ulcerative colitis. OBSERVATIONS: The best-corrected visual acuities were 1.5 OD and 0.7 OS. Dilated and tortuous retinal veins and retinal bleeding were seen in the left eye. Macular edema and leakage from the papilla and the retinal veins of the left eye were evident on fluorescein angiography. After increased dosage of systemic prednisolone was prescribed, the retinal vascular changes resulting from CRVO (papillophlebitis) in the left eye gradually abated. CONCLUSIONS: Retinal vascular diseases should be monitored during both remission and activation of intestinal symptoms of ulcerative colitis.     

A new case of acute idiopathic frosted branch angiitis in Europe

CASE REPORT: A 20-year-old male with no history of any systemic or eye disease developed loss of visual acuity in both eyes. White exudates surrounding the retinal veins from the posterior pole to the periphery, retinal edema and hemorrhages in both eyes were evident on ophthalmoscopy. Fluorescein angiography showed leakage of the dye from the veins and extensive staining of the vein walls. A diagnosis of acute frosted branch angiitis was made. Systemic examination revealed axillary, submandibular and inguinal lymphadenopathies. VCA IgM antibody for Epstein-Barr virus was negative and IgG positive. Biopsy was taken of an axillary lymph node; a non-specific inflammatory reaction was found on anatomopathologic study. The patient was started on 90 mg prednisolone daily. After two weeks retinal vasculitis had improved and the lymphadenopathies soon resolved. Small, hard exudates were present in the posterior pole during absorption of the retinal edema and resolution of the vascular inflammation. Systemic prednisolone were reduced progressively and definitively withdrawn two months later. The patient's visual acuity was 20/25 in both eyes. No fibrotic scar tissue or atrophic lesions were noted in either fundus. CONCLUSIONS: We report a new case of acute frosted branch angiitis with an onset and favorable clinical course similar to previous reports. We found the additional presence of lymphadenopathies but have been unable to establish a possible causal agent. To our knowledge, apart from a recent case of frosted branch angiitis-like response in Greece, the present case is the first reported in western Europe.     

Neovascular glaucoma as a complication of retinal vasculitis in Crohn disease

PURPOSE: To report a case of neovascular glaucoma as a complication of retinal vasculitis in Crohn disease. METHODS: Case report with fluorescein angiogram. RESULTS: A 62-year-old man with biopsy-proven Crohn disease presented with bilateral uveitis, bilateral iris new vessels, and neovascular glaucoma in the left eye. Fluorescein angiography revealed signs of retinal vasculitis and capillary nonperfusion in both eyes. CONCLUSION: Crohn disease may be associated with retinal vasculitis and, thus, neovascular glaucoma. A satisfactory result can be achieved by using corticosteroids to control the retinal vascular inflammation, by applying panretinal photocoagulation and by controlling the increased intraocular pressure surgically.     

Central retinal vein occlusion in a patient treated with antiandrogenic drug

Central retinal vein occlusion (CRVO) is usually seen in older adults and is often associated with systemic vascular disease, this is much less evident in young people. A case report of a 28-year-old woman presented a central retinal vein occlusion in her left eye. This young woman was treated with antiandrogenic drug. Investigations revealed an abnormality of the hemostatic system. The central retinal vein occlusion was resolved and the fundus assumed a normal appearance one-month after the primary episode. Retinal vascular occlusions with antiandrogenic drug may be due to three mechanisms: increased platelet cell aggregation, alteration of fibrinolytic system and vascular endothelium hyperplasia.     

视网膜光凝术治疗视网膜静脉阻塞所致玻璃体积血的临床体会

目的:应用视网膜光凝术治疗视网膜静脉阻塞(RVO)所致的玻璃体积血,观察其保存视力、阻止病变发展为增殖性视网膜病变的临床效果。方法:选择RVO致玻璃体积血患者15例15眼,根据出血量多少决定光凝治疗时机,逐步完成全部阻塞区的激光治疗;术后观察视力、出血吸收及新生血管性青光眼消退等情况。结果:患者15例完成光凝时间历时1wk~6mo,光凝次数1~8次。14例玻璃体积血全部吸收,视力稳定或提高,达到临床治愈;1例发生反复出血,未愈;2例新生血管性青光眼消退。结论:对于适当病例,视网膜光凝术可有效治疗RVO所致玻璃体积血,并使已发生的继发性青光眼消退。     

Bartonella henselae infection associated with neuroretinitis, central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss

PURPOSE: To report a case of Bartonella henselae infection. DESIGN: Observational case report. METHODS: Review of the clinical, laboratory, photographic, and angiographic records of a patient with cat scratch disease associated with central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss. RESULTS: A 21-year-old man had no light perception in the left eye secondary to concurrent central retinal artery and vein occlusion believed to have resulted from infection with Bartonella henselae. Forty days later, he developed neovascular glaucoma in the left eye. CONCLUSION: Ocular complications associated with Bartonella henselae infection may include central retinal artery and vein occlusion, neovascular glaucoma, and severe vision loss.     

A case of frosted-branch retinal angiitis in a child.

We report the case of a patient with frosted-branch angiitis of the retina in a 3-year-old girl. She had acute visual disturbances OU, and we observed iritis, retinal edema, and retinal angiitis. Retinal angiitis showed the same condition as previously described, the so-called acute frosted retinal periphlebitis accompanied with vascular sheathing. Her eyes improved rapidly after administration of an oral corticosteroid, and during the ensuing four months, she had no recurrences.     

Ahmed青光眼阀植入联合视网膜光凝或冷凝治疗新生血管性青光眼

目的:探讨Ahmed青光眼阀植入联合视网膜光凝或冷凝术对新生血管性青光眼治疗的临床效果。方法:选取4a来住院的新生血管性青光眼56例58眼,其中视网膜中央静脉阻塞25例25眼,糖尿病性视网膜病变21例23眼,视网膜血管炎5例5眼,颈动脉狭窄4例4眼,视网膜分支静脉阻塞1例1眼,平均年龄50.6岁,均行Ahmed青光眼阀植入联合视网膜光凝或冷凝治疗,其中47眼于手术前后行全视网膜光凝,11眼于手术中联合周边视网膜冷凝术,平均随访18.0±6.2mo,观察手术前后视力和眼压的变化以及术后并发症的情况,结果进行统计学分析。结果:视力:术后43眼有不同程度地提高,15眼无变化,无视力下降者。眼压:随访6mo时眼压由术前49.56±8.25mmHg降至17.86±5.25mmHg,总成功率为85%;随访12mo时,眼压由术前50.25±7.18mmHg降至18.80±6.81mmHg,总成功率为78%;24mo以上随访19眼,眼压由术前51.05±8.10mmHg降至20.12±7.01mmHg,总成功率为74%,手术前后眼压比较差异有非常显著性。并发症主要有前房出血、前房延缓形成、引流管内口堵塞、引流盘纤维包裹,经术后处理均得到了恢复。结论:Ahmed青光眼阀植入联合视网膜光凝或冷凝是治疗新生血管性青光眼安全有效的方法之一。     

Infrequency of retinal neovascularization following central retinal vein occlusion.

Forty-eight patients with the clinical diagnosis of central retinal vein occlusion and ten surgically enulceated eyes with central retinal vein occlusion documented by histopathologic examination were studied and reviewed. Retinal neovascularization developed in only one of the ten eyes. Rubeosis iridis occurred in 14% (7) of the clinical cases and in all of the histopathologic cases. Ten eyes with neovascular glaucoma, enucleated following central retinal vein occlusion, showed absence of retinal endothelial cells. The absence of retinal capillary endothelial cells is significant in explaining the rare occurrence of retinal neovascularization following central retinal vein occlusion. However, the small number of cases may reflect a biased result. Further studies of more cases over a longer period are required to reach the final conclusion.     

Unilateral frosted branch angiitis

We examined two patients with monocular frosted branch angiitis. The patients were young and healthy; they rapidly developed severe visual loss with thick, white sheathing of the retinal veins and responded promptly to systemic corticosteroids. The fluorescein angiograms showed late leakage from the retinal veins, without evidence of stasis or occlusion. Frosted branch angiitis can be either a unilateral or a bilateral condition. We believe the potential for visual loss and the prompt response to systemic corticosteroids make early, accurate diagnosis and institution of therapy desirable.     

Frosted Branch Angiitis as Ocular Manifestation of Beh?et's Disease: Unusual Case Report and Literature Review

We report an unusual case of unilateral frosted branch angiitis associated with Behçet''s disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behçet''s disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behçet''s disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.     

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.     

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.     

Oclusión mixta de arteria y vena central de la retina; primera manifestación de lupus en paciente pediátrico

Combined central retinal artery and vein occlusion is uncommon in adults and even more so in young people. The main origins are vasculitis and thromboembolic disorders. The prognosis is poor due to irreversible visual loss and the development of neovascular glaucoma (NVG).A 14 year-old male arrived at the clinic complaining of sudden and painless visual loss in the left eye. Best corrected visual acuity was light perception with clinical and fluoro-angiographic findings characteristic of combined central retinal artery and vein occlusion in his left eye. The findings in the systemic and laboratory studies led to a diagnosis of systemic lupus erythematosus (SLE) and antiphospholipid syndrome. The patient received treatment with steroids and oral anticoagulant. Seven days later, due to prolonged coagulation time, he presented with a vitreous haemorrhage. He was then treated with intravitreal ranibizumab in order to prevent NVG. Afterwards, vitrectomy and retinal endophotocoagulation were performed.To the best of our knowledge, this is the first presentation of a male, paediatric with SLE and combined occlusion to be published in the literature, and is also the first case treated with antiangiogenic agents that has not developed NVG at 12 months of follow-up.