Acute idiopathic frosted branch angiitis

Acute idiopathic frosted branch angiitis

Acute idiopathic frosted branch angiitis Mehryar Taban, MD,a Jonathan E. Sears, MD,a Eric Crouch, MD,b Andrew P. Schachat, MD,a and Elias I. Traboulsi...

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Acute idiopathic frosted branch angiitis Mehryar Taban, MD,a Jonathan E. Sears, MD,a Eric Crouch, MD,b Andrew P. Schachat, MD,a and Elias I. Traboulsi, MDa

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.


cute idiopathic frosted branch angiitis is a rare condition, first reported by Ito et al1 in 1976 in a Japanese boy. Since then, 56 other cases have been reported in the world literature, with the great majority (75%) in Japanese literature.2 Only nine cases have been reported from the United States. Herein, we describe the case of a 4-year-old African American girl, treated with systemic corticosteroids, with good recovery of vision.

Case Report A 4-year-old African American girl, with past medical history of sickle cell trait, was referred to Cole Eye Institute in Cleveland with bilateral vision loss over 1 day. Of note, she had mild upper respiratory infection symptoms a week prior to presentation that had since resolved. She could fix and follow targets poorly. Pupils were sluggish, without a relative afferent pupillary defect. The extraocular movements were full. Anterior segment examination was normal without iris neovascularization or iris nodules. Ophthalmoscopy revealed bilateral, diffuse, striking perivascular sheathing restricted primarily to the major retinal veins, serous macular detachments, and widespread retinal edema with few intraretinal hemorrhages (Figure 1). There was no evidence of vitritis, vitreous hemorrhage, or papillitis. A presumptive diagnosis of acute idiopathic frosted branch angiitis was made and prednisone (1 mg/ kg/day) treatment was initiated. An extensive evaluation failed to detect any underlying etiology. Tests included complete blood count and titers for cytomegalovirus, HIV, Epstein-Barr, herpes simplex, varicella zoster, toxo-

a Author Affiliations: aDepartment of Ophthalmology, Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio; bDepartment of Ophthalmology, Eastern Virginia Medical School, Norfolk, Virginia The authors have no conflict of interest to report. Submitted August 16, 2006. Revision accepted October 2, 2006. Published online January 26, 2007. Reprint requests: Elias I. Traboulsi, MD, i32, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland OH 44195 (email: [email protected]) J AAPOS 2007;11:286-287. Copyright © 2007 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2007/$35.00 ⫹ 0 doi:10.1016/j.jaapos.2006.10.010


plasmosis, and syphilis, as well as angiotensin-1-converting enzyme, erythrocyte sedimentation rate, chest x-ray, and brain magnetic resonance imaging. Prednisone was tapered over 4 weeks and the patient showed gradual improvement in vision acuity and retinal edema. At her 6-week follow-up visit, visual acuity was 20/30 in both eyes with trace subretinal fluid and absence of retinal perivascular sheathing.

Discussion Acute idiopathic frosted branch angiitis predominantly affects the young and healthy, with a reported age range of onset of 2 to 42 years.2 Seventy-five percent of cases are bilateral. There is significant subacute vision loss. The disease has a characteristic fundus appearance with widespread retinal vasculitis and variable degrees of retinal edema, intraretinal hemorrhages, papillitis, vitritis, and iritis. Fluorescein angiography is near normal in the early phase of the transit, but shows late vascular leakage. The electroretinogram and electro-oculogram are usually reduced and may remain subnormal despite visual recovery.2,3 As in the present case, most patients with acute idiopathic frosted branch angiitis have been treated with systemic corticosteroids with good, rapid visual recovery within weeks.2 There are, however, case reports of patients who recovered good vision acuity without systemic corticosteroids.4,5 Since the natural progression of the disease without systemic steroids is not well delineated and due to the possibility for permanent loss of vision secondary to macular scarring, prompt institution of corticosteroids is recommended. Due to a postulated viral underlying etiology, antivirals (eg, acyclovir) have also been used empirically, with unclear therapeutic contribution.2,6 Of note, our patient did not receive any antiviral therapy. By definition, the cause of acute idiopathic frosted branch angiitis is unknown. It has been postulated that it may be due to immune-complex deposition from hypersensitivity reaction to a variety of infectious agents.2,7,8 This is supported by its occurrence after a prodromal illness in 33% of cases, and its response to systemic corticosteroids.2 Moreover, there is one case of acute idiopathic frosted branch angiitis followed by immune-mediated rapidly progressive glomerulonephritis.9 It is important to exclude secondary causes of retinal vasculitis, such as viral retinitis (cytomegalovirus, HIV ), toxoplasmosis, syphilis, sarcoidosis, and lupus, among others.2,8,10 Masquerade conditions, including intraocular lymphoma or leukemia with retinal infiltrative phenomena, also need to be considered. Only then a presumptive diagnosis of acute idiopathic frosted

Journal of AAPOS

Volume 11 Number 3 / June 2007

Taban et al


FIG 1. Fundus photographs showing bilateral, diffuse perivascular sheathing and retinal edema with few intraretinal hemorrhages. (A) Right eye; (B) left eye.

branch angiitis can be made. Our case adds to the literature of this rare disease, illustrates its characteristic clinical features, and to our knowledge is only the second known case in an African American individual.4 References 1. Ito Y, Nakano M, Kyu N, Takeuchi M. Frosted branch angiitis in a child. Rinsho Ganka ( Jpn J Clin Ophthalmol) 1976;30:797-803. 2. Walker S, Iguchi A, Jones NP. Frosted branch angiitis: a review. Eye 2004;18:527-33. 3. Luo G, Yang P, Huang S, Jiang F, Wen F. A case report of frosted branch angiitis and its visual electrophysiology. Doc Ophthalmol 1998-99;97:135-42. 4. Vander JF, Masciulli L. Unilateral frosted branch angiitis. Am J Ophthalmol 1991;112:477-8.

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5. Hamed LM, Fang EN, Fanous MM, Mames R, Friedman S. Frosted branch angiitis: The role of systemic corticosteroids. J Pediatr Ophthalmol Strabismus 1992;29:312-3. 6. Narita K, Sato A. Systemic acyclovir was effective in a case of recurrent retinal angiitis. Rinsho Ganka ( Jpn J Clin Ophthalmol) 1990;44:739-43. 7. Okinami S, Nakamatsu T, Saito I. Four cases of frosted retinal angiitis. Nihon Ganka Kiyo (Folia Ophthalmol Jpn) 1994;45:314-8. 8. Kleiner RC. Frosted branch angiitis: Clinical syndrome or clinical sign? Retina 1997;17:370-1. 9. Gupta A, Narang S, Gupta V, Minz R, Sakhuja K. Frosted branch angiitis associated with rapidly progressive glomerulonephritis. Indian J Ophthalmol 2002;50:317-9. 10. Quillen DA, Stathopoulos NA, Blankenship GW, Ferriss JA. Lupus associated frosted branch periphlebitis and exudative maculopathy. Retina 1997;17:449-51.