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Year : 1999  |  Volume : 47  |  Issue : 3  |  Page : 195-197

Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome

Medical and Vision Research Foundation, Chennai, India

Correspondence Address:
J Biswas
Medical and Vision Research Foundation, Chennai
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Source of Support: None, Conflict of Interest: None

PMID: 10858779

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How to cite this article:
Biswas J, Raizada S, Gopal L, Kumarasamy N, Solomon S. Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome. Indian J Ophthalmol 1999;47:195-7

How to cite this URL:
Biswas J, Raizada S, Gopal L, Kumarasamy N, Solomon S. Bilateral frosted branch angiitis and cytomegalovirus retinitis in acquired immunodeficiency syndrome. Indian J Ophthalmol [serial online] 1999 [cited 2021 Sep 22];47:195-7. Available from: https://www.ijo.in/text.asp?1999/47/3/195/14914

This report describes a case of frosted branch angiitis associated with AIDS.

Frosted branch angiitis is a rare vasculitis usually seen in young children. The first case was reported from Japan by Ito and co-workers.[1] They described a case of a 6-year-old boy who presented with sudden bilateral visual loss and unusual thick sheathing of the retinal vessels. They termed this condition "frosted branch angiitis". According to initial reports, the mean age in the Japanese group of patients was 9 years, but this disease has been reported in an older age group (range 23-29 years) also.[2] We have reported a case of bilateral frosted branch angiitis in an 8-year-old Indian girl presumably caused by rubella virus.[3] All such patients reported were found to be otherwise healthy. However, there has been a report of 3 patients with Acquired Immunodeficiency Syndrome (AIDS) presenting with frosted branch angiitis associated with small patches of cytomegalovirus (CMV) retinitis.[4] We report here a case of frosted branch angiitis in a 39-year-old male with cytomegalovirus retinitis due to AIDS.

  Case Report Top

A 39-year-old heterosexual male with AIDS and a history of pulmonary tuberculosis, oropharyngeal candidiasis and pneumocystis carinii pneumonia was referred from an AIDS research centre in February 1997. He was detected HIV-positive in March 1995. He was asymptomatic, had 6/6, N6 vision and normal anterior segment in both eyes. Fundus examination revealed a solitary cottonwool spot in each eye that did not require any treatment. He was advised to continue anti-HIV therapy. Five months later, visual acuity remained normal but he had developed necrotising retinitis with yellow granular border and retinal hemorrhages typical of CMV infection on both nasal and temporal periphery of both eyes. In the right eye the retinitis patch was superonasal to disc along the superonasal vessels and in the left eye [Figure - 1] it was located in the nasal periphery. Both eyes had perivascular infiltrates predominantly involving the veins but also affecting arterioles close to the involved retinal veins, with an appearance typical of frosted branch angiitis. Laboratory evaluation revealed Hb 9.0 gm% and a total leucocyte count of 2400/mm3. The absolute CD4 helper cell count was 69/mm3 and CD8 suppressor cell count was 564/mm3, giving a helper-suppressor cell ratio of 0.12. His platelet count was 2,35,000/mm3. Viral load (HIV 1, RNA level) was 223,000 copies per cml.

Intravenous injection of gancyclovir 5mg/kg body weight /day was prescribed. This treatment continued for 14 days. The patient was reviewed after 3 weeks. At this stage, his vision deteriorated to 2/60 in the right eye and 6/60 in the left eye. Fundus examination revealed marked regression of CMV retinitis and angiitis in both eyes but subsequently the patient developed combined tractional and rhegmatogenous retinal detachment in the left eye [Figure: 2]. He underwent vitrectomy with silicone oil tamponade and barrier endolaser photocoagulation in the left eye. The retina was reattached in the early postoperative period. A week later, the right eye showed total retinal detachment and reappearance of frosted branch angiitis. Biweekly intravitreal injections of gancyclovir (400 mgm) were given. The patient was advised vitrectomy with silicone oil tamponade and endolaser in right eye. However his systemic condition deteriorated due to multiple systemic infections and the patient died in November 1997, 4 months after the diagnosis of CMV retinitis associated with frosted branch angiitis. The eyeballs were not available for histopathologic correlation.

  Discussion Top

The main features of frosted branch angiitis as originally reported by Ito and coworkers[1] are as follows: (i) bilateral acute visual disturbance in a healthy child; (ii) swollen retina with severe sheathing of the retinal vessels, resembling frosted branches of a tree, especially at the periphery; (iii) normal pattern on fundus fluorescein angiography in the initial stages, but leakage of the dye from vessels seen in later stages, the sheathed vessels showing no sign of occlusion or stasis; (iv) marked concentric constriction of visual fields during the acute stages of the disease which improves along with reduction of swelling and inflammation; (v) markedly abnormal ERG; and (vi) resolution of disease and visual acuity returning to normal with high doses of systemic corticosteroids and absence of recurrences.

After the initial report, other investigators have described cases of frosted branch angiitis.[4],[5] Frosted branch angiitis is essentially a severe form of vasculitis which affects the entire retina. No specific cause can be attributed for this disorder. Kestelyn et al[6] reported 5 cases of perivasculitis in a series of 29 HIV patients in Africa. None of these cases had frosted branch angiitis. Although both arteries and veins may be involved, the venules tend to be more commonly affected. Some consider it to be a severe case of diffuse perivasculitis.

In the series reported by Spaide and coworkers[4] all the patients had low CD4-helper T-lymphocyte count and T-lymphocyte helper-suppressor ratio of less than 0.1. Several other series have also reported that CMV retinitis is associated with low CD4 count and the mean count among patients with CMV retinitis is generally less than 50 cells/cu mm.[5] In our case the CD4 count was 69/cu mm and CD4/CD8 ratio was 0.1. The patient had areas of typical CMV retinitis as well. Treatment with gancyclovir 5mg/kg body weight/day intravenously resulted in rapid resolution of the perivascular infiltrate. The use of systemic steroid in such cases is controversial. Spaide and coworkers[4] in their study of 3 cases recommended avoidance of corticosteroids. All their cases showed rapid clearing of the inflammatory sheathing around the retinal veins with specific antiviral treatment alone. They felt that corticosteroids were not specifically indicated in immuno-compromised patients, as steroids suppress the immune system further without inhibiting CMV replication. However, Geier and coworkers[7] reported a case of frosted branch angiitis in a patient with cytomegalovirus retinitis they treated with gancyclovir (10 mg/kg body weight per day) and oral fluocortolone (100 mg/day). There was prompt reduction in vascular sheathing, CMV retinitis resolved and vision improved from 20/200 to 20/30 after 2 weeks of therapy. This particular patient was kept on gancyclovir maintenance therapy and was followed up for 9 months. No relapse was noted. In our case we did not start steroids as the patient was suffering from severe immuno-suppression and had already developed pulmonary tuberculosis and oropharyngeal candidiasis which could have been aggravated by the use of systemic steroid.

It appears that the clinical picture of "frosted branch angiitis" can be caused by direct infection by several infectious agents or due to immunologic response.[1] It is obvious in our case that the vascular sheathing was caused directly by CMV in view of the resolution with gancyclovir and reappearance of frosted branch angiitis with reactivation of CMV retinitis. The resolution of frosted branch angiitis was also accompanied by concomitant resolution of retinitis. In contrast to our case, the cases of "frosted branch angiitis" reported in healthy individuals responded to steroids, pointing to an immunological origin for the clinical appearance.[8]

It has been reported that despite the impressive initial treatment response, active retinitis recurs in virtually all patients within 3 - 4 weeks after anti-CMV therapy is stopped. Despite maintenance therapy, relapse has been reported in 18 - 50% of patients, though the time to relapse is much longer in patients on maintenance therapy than in untreated patients. Patients who relapse generally respond to a second course of induction therapy.[8] The resistance of CMV to foscarnet and gancyclovir has not been well studied. Retinal detachment is a known complication of anti-CMV therapy and has been reported to occur in up to 29% of patients. Anti-CMV therapy probably contributes to the development of retinal detachment by inhibiting the scar formation.[9] Some investigators suggested that patients with lesions involving more than 50% of the retina are at a greater risk of retinal detachment than are those with lesions involving less than 50% of the retina.[8]

This case of "frosted branch angiitis" with CMV retinitis in a patient with AIDS further proves that "frosted branch angiitis" is only a "descriptive fundus finding" and not a disease entity by itself and efforts must be made to identify a possible etiology.

  References Top

Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted branch angiitis in a child. Jpn J Ophthalmol 1976;30:797-803.  Back to cited text no. 1
Kleiner R, Kaplan HJ, Shakin JL, Yannuzzi LA, Crosswell HH, McLean WC. Acute frosted retinal periphlebitis. Am J Ophthalmol 1988;106:27.  Back to cited text no. 2
Biswas J, Fogla R, Madhavan HN. Bilateral frosted branch angiitis in an 8-year-old Indian girl. Retina 1996;16:444-45.  Back to cited text no. 3
Spaide RF, Vitale AT, Toth IR, Oliver JM. Frosted branch angiitis associated with cytomegalovirus retinitis. Am J Ophthalmol 1992;113:522-28.  Back to cited text no. 4
Kuppermann BD, Petty JG, Richman DD, Matthews C, Fullerston SC, Rickman LE, et al. Correlation between CD4+ counts and prevalence of cytomegalovirus retinitis and human immunodeficiency virus-related non-infectious retinal vasculopathy in patients with acquired immunodeficiency syndrome. Am J Ophthalmol 1993;575-82.  Back to cited text no. 5
Kestelyn P, Lepage P, Van de Perre P. Perivasculitis of the retinal vessels as an important sign in children with AIDS related complex. Am J Ophthalmol 1985;100:614-15.  Back to cited text no. 6
Geier SA, Nasemann J, Klauss V, Kronawitter U, Goebel F. Frosted branch angiitis in a patient with acquired immunodeficiency syndrome. Am J Ophthalmol 1992;113:203-5.  Back to cited text no. 7
Jabs DA, Enger C, Bartlett JG. Cytomegalovirus retinitis and acquired immunodeficiency syndrome. Arch Ophthalmol 1989;107:75.  Back to cited text no. 8
Freeman WR, Henderly DE, Wan WL, Causey D, Trousdale M, Green RL, Rao NA. Prevalence, pathophysiology and treatment of rhegmatogenous retinal detachment in treated cytomegalovirus retinitis. Am J Ophthalmol 1987;103:527-36.  Back to cited text no. 9


  [Figure - 1], [Figure - 2]


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