An atypical finding in an idiopathic unilateral frosted branch angiitis case: premacular hemorrhage

BACKGROUND: The authors describe a case of unilateral idiopathic acute frosted branch angiitis with premacular hemorrhage. CASE: A 35-year-old woman was referred because of acute vision loss in her left eye during the puerperal period. Her initial visual acuity was 20/20 OD and 20/200 OS. The left eye presented severe sheathing of retinal vessels inferiorly, heavy perivascular intraretinal hemorrhages, and premacular hemorrhage. There was no evidence of vascular leakage in fluorescein angiography. All of the laboratory workup was negative for frosted branch angiitis (FBA). During the follow-up period, FBA resolved spontaneously within a few days, but the amount of premacular hemorrhage increased. Vitrectomy with internal limiting membrane peeling was performed at the third month, resulting in 20/25 vision and no recurrence of the disease during the 13-month follow-up. Conclusion: This is an idiopathic case of acute FBA that exhibited spontaneous rapid regression of angiitis but was complicated by an unusual premacular hemorrhage.     

Frosted branch angiitis: a review

The purpose of this study is to present the first report of a case of primary frosted branch angiitis from the UK and to review the characteristics of this rare disease. Primary frosted branch angiitis causes characteristic florid translucent retinal perivascular sheathing of both arterioles and venules in association with variable uveitis, retinal oedema and visual loss, normally with good recovery. A total of 57 cases have been reported in the world literature. Atypical, typically focal frosted branch angiitis may also occur secondary to other causes of intraocular inflammation, especially cytomegalovirus retinitis. Primary frosted branch angiitis has a characteristic presentation but a variable course, typically affecting children or young adults. The disease is likely to represent a common immune pathway in response to multiple infective agents. The optimal treatment is unclear.     

Recurrent secondary frosted branch angiitis after toxoplasmosis vasculitis

PURPOSE: To describe a case of recurrent frosted branch angiitis after treatment of ocular toxoplasmosis. METHODS: In a 6-year-old boy, we found perivascular, creamy, patchy, retinal sheathing in both eyes without any focal necrotizing retinochoroiditis or scarring. IgM antibodies for toxoplasma gondii were also found. The patient was treated with antitoxoplasmosis medication and a systemic steroid. RESULTS: Several years after treatment of the toxoplasmosis, frosted branch angiitis occurred twice without any retinal scarring or serological evidence of toxoplasmosis. After systemic steroid therapy, the angiitis improved without further complications. CONCLUSIONS: Toxoplasmic retinal vasculitis should be considered as a cause of frosted branch angiitis.     

Frosted branch angiitis associated with cytomegalovirus retinitis.

We examined three patients with acquired immunodeficiency syndrome who had frosted branch angiitis associated with small patches of cytomegalovirus retinitis. Each patient had a low CD4-helper T-lymphocyte count and a T-lymphocyte helper-suppressor ratio of less than 0.1. Treatment with intravenous anticytomegalovirus antibiotics caused the vascular sheathing to resolve within two weeks in all three patients, but each patient continued to have a smoldering retinitis. Retinal biopsy in one of the patients demonstrated virions whose morphologic characteristics were consistent with cytomegalovirus on electron microscopy and the identity of which was confirmed by immunohistochemistry. Although frosted branch angiitis in otherwise healthy patients responds to corticosteroids, similar treatment with corticosteroids for frosted branch angiitis associated with cytomegalovirus retinitis in patients with AIDS does not seem to be indicated. Before corticosteroid treatment is started for a patient with the clinical signs and symptoms of frosted branch angiitis, careful medical examination of the patient is necessary.     

Unilaterale retinale Pseudoarteritis nach Contusio bulbi

This article reports an unusual case of unilateral frosted branch angiitis. Three major groups of this disease are known: secondary frosted branch angiitis due to viral infection or autoimmune diseases, frosted branch-like angiitis related to malignant diseases and the rare entity of idiopathic frosted branch angiitis. A 58-year-old patient presented with strictly unilateral sheathing and partly occluded retinal arteries, vitritis and macular edema and 4 months prior to presentation he suffered a contusion of the eye with traumatic cataract and vitreous hemorrhage followed by cataract extraction and vitrectomy. The general medical history revealed that the contralateral eye and biochemical analyses were unremarkable despite slightly elevated antinuclear antibody (ANA) levels. Under high dose steroid therapy the patient showed slow improvement in all clinical findings. This case does not fit into any of the known groups and can therefore be defined as pseudoarteritis.     

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 Associated with Epstein-Barr Virus Systemic Infection

Purpose: To describe the first case that the authors are aware of frosted branch angiitis associated with Epstein-Barr virus infection. Methods: Case report. Results: A 7-year-old boy presented with bilateral decreased visual acuity. Funduscopy showed a typical image of frosted branch angiitis. He was started on treatment with intravenous steroids and acyclovir. Serological testing was positive for anti-Epstein-Barr virus IgM antibodies. Anti-Epstein-Barr virus IgG antibodies tested positive later. During follow-up, the venous sheathing decreased. Three months later funduscopy showed no abnormalities. Conclusion: Epstein-Barr virus infection should be considered in patients presenting with the typical clinical manifestations of this syndrome.     

Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease

Behçet disease is an idiopathic, multisystem disorder characterized by recurrent episodes of orogenital ulceration and vasculitis of the veins and arteries of all calibers. Ocular involvement may affect the conjunctiva, sclera, uveal tract, vitreous, blood vessels, and retina. Many theories have pointed toward an autoimmune response behind its pathogenesis, which may be triggered by exposure to an infectious agent. Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology. Association between Behηet disease, Frosted branch angiitis, and neuroretinitis is not reported in literature. This uncommon combination reflects the varied systemic and ocular manifestations in Behηet disease, especially in patients who are not diagnosed and treated in time. We hereby report a case of bilateral frosted branch angiitis and neuroretinitis in a young male from Middle-east, suffering from Behçet disease.     

Frosted Branch Angiitis Secondary to Macular Choroidal Neovascularization in a Chinese Woman

Background This is perhaps the first report of frosted branch angiitis secondary to choroidal neovascularization (CNV) from China. We describe a case of frosted branch angiitis associated with macular CNV.Case A 19-year-old Chinese woman complained of metamorphopsia and blurred vision in the right eye on August 27, 2002.Observations Fluorescein angiography demonstrated CNV in the macula. Five months later, focal and atrophic choroidal scars were observed in the macular area. She complained of blurred vision again on April 14, 2003. Iridocyclitis and frosted branch angiitis were found in both fundi. Following treatment with systemic acycloguanosine and corticosteroids for 4 months, the fundi recovered almost completely.Conclusions A case of frosted branch angiitis secondary to macular choroidal neovascularization is reported in a Chinese woman. The relation between frosted branch angiitis and CNV remains to be further explored. Jpn J Ophthalmol 2005;49:228–230 © Japanese Ophthalmological Society 2005     

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.     

Frosted branch angiitis associated with rapidly progressive glomerulonephritis

Simultaneous occurrence of frosted branch angiitis and immune-mediated rapidly progressive glomerulonephritis is reported. The two diseases possibly share a common immune mechanism. Patients of frosted branch angiitis should undergo complete systemic evaluation including renal function tests even if the patient is systemically asymptomatic.     

Frosted branch angiitis in a child with HIV infection

PURPOSE: In adults with human immunodeficiency virus (HIV) infection, frosted branch angiitis is commonly associated with cytomegalovirus retinitis and responds to anti-cytomegalovirus therapy. We describe the first pediatric case of HIV-associated frosted branch angiitis. METHODS: Case report. RESULTS: A 7-year-old HIV-infected male with frosted branch angiitis was refractory to induction doses of intravenous ganciclovir and foscarnet over a 2-month period. Although cytomegalovirus antigenemia resolved, the angiitis only improved after subsequent treatment with systemic corticosteroids. CONCLUSION: Frosted branch angiitis in this patient was not attributed to cytomegalovirus. The pathogenesis of HIV-associated frosted branch angiitis may differ between children and adults.     

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.     

Acute idiopathic frosted branch angiitis.

This is a case of acute idiopathic frosted branch angiitis in a 4-year-old African American girl with history of sickle cell trait. She developed bilateral, subacute vision loss attributed to acute idiopathic frosted branch angiitis and was treated with systemic corticosteroids with a good recovery of vision. Acute idiopathic frosted branch angiitis is a rare disease, usually with a good prognosis. This is, to our knowledge, the 10th reported case in the United States.     

Unilateral frosted branch angiitis with vitreous haemorrhage

Idiopathic retinal frosted branch angiitis is a rare bilateral condition and presents with an acute reduction of vision. Unilateral affection is rare. We report an unusual case of unilateral frosted branch angiitis where the patient developed total vitreous haemorrhage.     

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.     

Frosted branch angiitis associated with Harada disease-like manifestations recurs 10 years later

PURPOSE: To describe a case with frosted branch angiitis occurring in association with manifestations of Harada disease. CASE: A 41-year-old man developed marked iridocyclitis, frosted branch angiitis and retinal white dots, together with serous detachment of the sensory retina in the posterior pole of both eyes. OBSERVATIONS: Fluorescein angiography demonstrated multiple retinal pigment epithelial leakage points in the early phase followed by late dye pooling in the serous detachment, consistent with the manifestations of Harada disease. The patient experienced recurrence of the same combination of manifestations 10 years later. On both occasions, he showed mononuclear cell increase and protein elevation in the cerebrospinal fluid on spinal tap. CONCLUSION: Fluorescein angiography and spinal tap supported the diagnosis of Harada disease in the patient. The present patient could be diagnosed as having either Harada disease with frosted branch-like response or frosted branch angiitis with serous retinal detachment as occurs in Harada disease.     

A case report of frosted branch angiitis associated with aseptic meningitis in a young boy

We report on an 11-year-old boy with frosted branch angiitis in both eyes associated with aseptic meningitis. The patient presented after a severe decrease in vision in both eyes and a 40°C fever. The examination of the fundus of the eyes revealed a diffuse edema of the retina and diffuse bilateral vascular sheathing. The complementary examinations revealed aseptic meningitis. Neither infectious etiologies nor systemic diseases could be confirmed and an inflammatory digestive disease was suspected because of recent diarrhea events responsible for substantial weight loss. A systemic corticotherapy slowly improved the vision in both eyes. After 1 year of medication, visual recovery was full with no sequelae on fundus examination.     

Behçet disease presenting with frosted branch angiitis

The authors present a case of Beh?et disease presenting with frosted branch angiitis. Frosted branch angiitis is a rare clinical finding and there are only two reported cases in the literature associated with Beh?et disease.