FROSTED BRANCH ANGIITIS IN AN OCTOGENARIAN WITH INFECTIVE ENDOCARDITIS Neharika Sharma, MBBS,* Sumu Simon, FRANZCO,* Graham Fraenkel, FRACS, FRANZCO,† Jagjit Gilhotra, FRANZCO*
Purpose: To present the difficulties in diagnosing frosted branch angiitis (FBA) in an elderly person, and to present the likely immunogenic association of FBA with alphahemolytic streptococci. Methods: Thorough review of the medical records of an 80-year-old white man diagnosed with FBA. Patient consent was obtained before the writing of this article. Results: Our patient presented with 1 week of decreasing vision and floaters in his right eye and 3 days of floaters in his left eye. Significant medical history included a prosthetic mitral valve. Notable features on examination were bilateral retinal vascular sheathing, with retinal edema and hemorrhage. Preliminary investigations—including a transoesophageal echocardiogram—did not reveal intraocular or systemic infection, autoimmune disease, or underlying malignancy. Idiopathic FBA was unlikely given that the majority of cases had been documented in middle-aged Japanese populations. Thus, we chose to treat our patient empirically for common causes of retinal vasculitis such as viral retinitis. Interestingly, 2 weeks after the diagnosis of FBA, our patient became febrile. He was subsequently diagnosed with infective endocarditis and commenced on intravenous vancomycin (penicillin allergy). Review of his transoesophageal echocardiogram found an oscillating lesion that had been initially missed. Therefore, it is likely that our patient had blood culture–negative endocarditis at the time of presentation. This was further supported by the regression of his ocular disease with intravenous vancomycin. Conclusion: We hypothesize that our patient developed FBA as an immunogenic response to Streptococcus viridans and Streptococcus oralis infection. Previous associations between streptococci and FBA have been made. Notably, beta-hemolytic streptococcal antigens are known to cross-react with retinal antigens. However, the implicated pathogens in our patient were alpha-hemolytic. Alpha-hemolytic streptococcal endophthalmitis has been linked to frosted branch response before, but it has never been associated with FBA through an immunogenic pathway. Therefore, it is important to note that idiopathic FBA can be associated with infective endocarditis and alpha-hemolytic streptococcal infections. Furthermore, FBA in elderly patients needs thorough evaluation to rule out systemic causes. RETINAL CASES & BRIEF REPORTS 9:47–50, 2015
idiopathic or associated with hematological malignancy (masquerade) or active intraocular infection or autoimmune disease (frosted branch response).1 Idiopathic FBA is thought to be secondary to hypersensitivity to an infectious precedent.1 To date, 75% of the reported 58 cases of idiopathic FBA occur in adolescent to middleaged Japanese populations.1 We present the first case of FBA in an elderly patient and the first to be associated with infective endocarditis.
From the *South Australian Institute of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia; and †Cataract and Laser Surgicentre, Adelaide, South Australia.
F
rosted branch angiitis (FBA) is characterized by retinal perivascular sheathing that resembles frosted branches of a tree, varying degrees of uveitis, and retinal edema and hemorrhage.1 Frosted branch angiitis can be
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Case Report An 80-year-old white man presented with 1 week of decreasing vision and floaters in his right eye and 3 days of floaters in his left eye. He had uncomplicated cataract surgery 3 weeks before in his right eye and 2 weeks before in his left eye. Best-corrected visual acuity at postoperative Day 1 was 6/6 in the right eye and 6/7.5 in the left eye. Preoperative and postoperative examinations were unremarkable. At presentation, his best-corrected visual acuity was 1/60 in the right eye and 6/18−1 in the left eye. Examination revealed mild vitritis and anterior chamber inflammation bilaterally, as well as extensive bilateral perivascular sheathing, retinal edema, and retinal hemorrhages (Figure 1). His right disk was edematous, but no afferent pupillary defect was noted. Vitreous hemorrhage obscured the view of his right macula. His left superior macula was thickened. Optical computed tomography showed bilateral intraretinal fluid. Fluoroscein angiography revealed bilateral venous and arteriolar sheathing (Figure 1). His history included diet-controlled diabetes mellitus, hypertension, congestive cardiac failure, mitral valve replacement (2005), and a curative cystectomy for transitional cell carcinoma (2001). His regular medications were 3 mg of warfarin daily, 240 mg of diltiazem daily, and 250 mg of digoxin daily. Preliminary investigations did not reveal an underlying cause, with no evidence of hematological malignancy on full blood examination, normal inflammatory markers and vasculitic screen, negative blood cultures and viral serologies (including HIV), normal chest x-ray, and a normal MRI of the brain and orbits. He had a transoesophageal echocardiogram that was reported as negative for vegetations. His age and prosthetic valve meant that we had a high index of suspicion for an infective etiology, despite no definitive investigation results. He underwent right vitreous and anterior chamber taps, which were tested for herpes simplex virus, varicella zoster virus, and cytomegalovirus polymerase chain reactions; gram staining; microscopy, culture, and sensitivities; and flow cytometry. He was treated empirically for bilateral viral retinitis with a dose of intravitreal ganciclovir 0.1 mL of 2 mg and intravenous acyclovir TDS. He was also commenced on topical Prednefrin Forte (prednisolone acetate 1% + phenylephrine hydrochloride 0.12%) hourly and atropine 1% TDS, as well as oral prednisone 1 mg/kg daily. Despite 7 days of treatment, his right visual acuity further deteriorated to finger counting (1 m), thus we began to consider atypical fungal infections and endogenous endophthalmitis. He was commenced on 200 mg of oral voriconazole daily and underwent a right vitrectomy with intravitreal injections of 10 mg of amphotericin, 2.2 mg of ceftazidime, and 1 mg of vancomycin. After vitrectomy, his right visual acuity improved to 6/24. Incidentally, 2 days later, his left vision worsened from 6/18 to 6/60 secondary to a vitreous hemorrhage. This was attributed to uncontrolled hypertension and an International Normalized Ratio of 4.6, rather than progression of ocular disease. All systemic antiinfective medications were ceased on Day 12 after his vitreous biopsy returned negative. Topical and oral steroids were not altered. Two days later, he became febrile and was found to have an elevated white cell count of 29.1 · 109 per None of the authors have any financial/conflicting interests to disclose. Reprint requests: Neharika Sharma, MBBS, Level 5 Eye Outpatients, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia; e-mail: [email protected]
liter and a raised C-reactive protein at 24 mg/L. Repeat blood cultures grew Streptococcus viridans and Streptococcus oralis. Review of his original transoesophageal echocardiogram revealed a 1.6-mm oscillating vegetation on his prosthetic valve that had initially been missed (Figure 2). In light of his streptococci-positive blood cultures and valvular lesion, our patient was diagnosed with infective endocarditis and commenced on 1 g of intravenous vancomycin daily (penicillin allergy).2 The infectious disease team believed that our patient had blood culture–negative endocarditis at presentation because the valvular lesion had been present throughout the course of his FBA.2 This hypothesis correlated well with the clinical picture because there was regression of ocular disease with the commencement of vancomycin. On discharge, 1 month later, best-corrected visual acuity was 6/12 bilaterally. Two months after discharge, he presented with a rubeotic right eye secondary to retinal ischemia. He was treated with a dose of intracameral bevacizumab, four doses of intravitreal bevacizumab, and panretinal photocoagulation in his right eye. He subsequently had prophylactic panretinal photocoagulation in his left eye because of extensive angiographic capillary dropout. Eight months after presentation, his best-corrected visual acuity was 6/24 in the right eye and 6/9 in the left eye.
Discussion Kleiner divided FBA into 3 categories—masquerade, frosted branch response, and idiopathic.1 Masquerade refers to a frosted branch-like picture associated with hematological malignancies, where vascular sheathing is secondary to malignant infiltration of vessel walls rather than an inflammatory process.1 Frosted branch response is when the trademark clinical appearance occurs secondary to active intraocular infection or autoimmune disease.1 The term idiopathic FBA was used to describe a group of otherwise young and healthy patients who presented with a similar cluster of signs without an identifiable cause.1 Since then, there has been much debate as to whether idiopathic FBA is a distinct syndrome or a clinical sign of posterior uveitis.1,3,4 Although our patient presented with the clinical features of FBA, he proved to be a diagnostic challenge, as negative results for active infection, autoimmune disease, and hematological malignancy, suggested that he had idiopathic FBA.1 However, to date, the oldest documented case of idiopathic FBA was in a 42-year-old Korean, and the average age of presentation in whites is during the third decade of life.1 Therefore, given our patient’s age and the rare nature of this condition, we believed that idiopathic FBA was unlikely and proceeded to empirically treat more common causes of retinal vasculitis in elderly patients.3–5 Interestingly, 2 weeks after his diagnosis of FBA, our patient fulfilled the diagnostic criteria for infective endocarditis. It is likely that he had blood
FBA ASSOCIATED WITH INFECTIVE ENDOCARDITIS
49
Fig. 1. Color fundus photographs and fundus fluorescein angiograms (FFA) from presentation and Day 4 of admission. A. Right fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema, vitreous hemorrhage obscuring view of macula. B. Left fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema. C. Right fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema, vitreous hemorrhage obscuring view of macula. D. Left fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema. E. Right FFA at presentation: perivenular leakage and capillary dropout, no vascular occlusion. F. Left FFA at presentation: perivenular leakage and capillary dropout, no vascular occlusion. G. Right fundus photograph at Day 4: less marked arteriolar and venous sheathing, clearing vitreous hemorrhage, ongoing retinal hemorrhage and edema. H. Left fundus photograph at Day 4: less marked arteriolar and venous sheathing, ongoing retinal hemorrhage and edema. I. Right FFA at Day 4: less extensive leakage, capillary dropout still present. J. Left FFA at Day 4: less extensive leakage, capillary dropout still present.
culture–negative endocarditis at presentation. We note that blood cultures can remain negative in 2% to 7% of patients with infective endocarditis, excluding those with previous antibiotic treatment and improper culturing technique.2 In light of this, we hypothesized that FBA was secondary to a poststreptococcal immunogenic response.6–10 Uveitis, retinitis, and even a case of FBA have been reported poststreptococcal infection. All of these reported cases of poststreptococcal ocular inflammation occurred secondary to initial
infection with beta-hemolytic streptococcal strains, as was suggested by the type of preceding streptococcal infection (e.g., streptococcal pharyngitis) and by the use of a high antistreptolysin-O titer as diagnostic evidence.8–10 Therefore, pathogenesis was thought to be secondary to an autoimmune process triggered by the cross-reactivity of Group A streptococcal M-proteins and retinal S-antigens.7–10 A distinguishing feature of our case was that this seemed to be the first immunogenic response secondary to alpha-hemolytic streptococci.
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Fig. 2. Freeze frame of transoesophageal echocardiogram depicting the oscillating mass seen on the prosthetic mitral valve.
The only association in the literature between alpha-hemolytic streptococci and FBA was that of a Streptococcus mitis endophthalmitis causing a frosted branch response. However, this was due to active ocular infection not an immunogenic response.1,6 The cardinal teaching points of our case are that FBA in elderly patients requires thorough evaluation to rule out systemic causes and that FBA can occur secondary to an immunogenic response to alphahemolytic streptococcal infections. Furthermore, the advanced age of our patient, plus a new immunogenic association, adds weight to the theory that FBA is in fact a clinical sign of posterior uveitis, rather than a distinct clinical syndrome. Key words: blood culture–negative endocarditis, elderly, frosted branch angiitis, retinal vasculitis, poststreptococcal. Acknowledgment The authors thank Alan Hoare1 for his technical assistance with taking and editing the displayed clinical photographs and fundus fluorescein angiograms.
References 1. Walker S, Iguchi A, Jones NP. Frosted branch angiitis: a review. Eye (Lond) 2004;18:527–533. 2. Raoult D, Sexton DJ. Culture negative endocarditis. Up To Date. 2012. 3. Kleiner RC. Frosted branch angiitis: clinical syndrome or clinical sign. Retina 1997;17:370–371. 4. Davis JL. Diagnostic dilemmas in retinitis and endophthalmitis. Eye (Lond) 2012;26:194–201. 5. Silva RA, Albini TA, Flynn HW. It still Happens Today: bilateral frosted Branch angiitis in cytomegalovirus retinitis. Retin Physician 2010 [cited Jan 2014]; feature article 8: 5 pages. Available at: http://www.retinalphysician.com/articleviewer. aspx?articleID=104854. 6. Chen E, Ho AC, Garg SJ, et al. Streptococcus mitis endophthalmitis presenting as frosted branch angiitis after intravitreal pegaptanib sodium injection. Ophthalmic Surg Lasers Imaging 2009;40:192–194. 7. Lerner MP, Donoso LA, Nordquist RE, Cunningham MW. Immunological mimicry between retinal S-antigens and Group A streptococcal M proteins. Autoimmunity 1995;22:95–106. 8. Han J, Lee SC, Song WK. Recurrent bilateral retinal vasculitis as a manifestation of post-streptococcal uveitis syndrome. Korean J Ophthalmol 2012;26:309–311. 9. Reddy UP, Albini TA, Banta JT. Post-streptococcal vasculitis. Ocul Immunol Inflamm 2008;16:35–36. 10. Kono H, Ikewaki J, Kimoto K, et al. Frosted branch angiitis associated with streptococcal infection: optical coherence tomography as a follow-up tool. Acta Ophthalmol 2009;87:909–911.
Purpose: To present the difficulties in diagnosing frosted branch angiitis (FBA) in an elderly person, and to present the likely immunogenic association of FBA with alphahemolytic streptococci. Methods: Thorough review of the medical records of an 80-year-old white man diagnosed with FBA. Patient consent was obtained before the writing of this article. Results: Our patient presented with 1 week of decreasing vision and floaters in his right eye and 3 days of floaters in his left eye. Significant medical history included a prosthetic mitral valve. Notable features on examination were bilateral retinal vascular sheathing, with retinal edema and hemorrhage. Preliminary investigations—including a transoesophageal echocardiogram—did not reveal intraocular or systemic infection, autoimmune disease, or underlying malignancy. Idiopathic FBA was unlikely given that the majority of cases had been documented in middle-aged Japanese populations. Thus, we chose to treat our patient empirically for common causes of retinal vasculitis such as viral retinitis. Interestingly, 2 weeks after the diagnosis of FBA, our patient became febrile. He was subsequently diagnosed with infective endocarditis and commenced on intravenous vancomycin (penicillin allergy). Review of his transoesophageal echocardiogram found an oscillating lesion that had been initially missed. Therefore, it is likely that our patient had blood culture–negative endocarditis at the time of presentation. This was further supported by the regression of his ocular disease with intravenous vancomycin. Conclusion: We hypothesize that our patient developed FBA as an immunogenic response to Streptococcus viridans and Streptococcus oralis infection. Previous associations between streptococci and FBA have been made. Notably, beta-hemolytic streptococcal antigens are known to cross-react with retinal antigens. However, the implicated pathogens in our patient were alpha-hemolytic. Alpha-hemolytic streptococcal endophthalmitis has been linked to frosted branch response before, but it has never been associated with FBA through an immunogenic pathway. Therefore, it is important to note that idiopathic FBA can be associated with infective endocarditis and alpha-hemolytic streptococcal infections. Furthermore, FBA in elderly patients needs thorough evaluation to rule out systemic causes. RETINAL CASES & BRIEF REPORTS 9:47–50, 2015
idiopathic or associated with hematological malignancy (masquerade) or active intraocular infection or autoimmune disease (frosted branch response).1 Idiopathic FBA is thought to be secondary to hypersensitivity to an infectious precedent.1 To date, 75% of the reported 58 cases of idiopathic FBA occur in adolescent to middleaged Japanese populations.1 We present the first case of FBA in an elderly patient and the first to be associated with infective endocarditis.
From the *South Australian Institute of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia; and †Cataract and Laser Surgicentre, Adelaide, South Australia.
F
rosted branch angiitis (FBA) is characterized by retinal perivascular sheathing that resembles frosted branches of a tree, varying degrees of uveitis, and retinal edema and hemorrhage.1 Frosted branch angiitis can be
47
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RETINAL CASES & BRIEF REPORTS´ 2015 VOLUME 9 NUMBER 1
Case Report An 80-year-old white man presented with 1 week of decreasing vision and floaters in his right eye and 3 days of floaters in his left eye. He had uncomplicated cataract surgery 3 weeks before in his right eye and 2 weeks before in his left eye. Best-corrected visual acuity at postoperative Day 1 was 6/6 in the right eye and 6/7.5 in the left eye. Preoperative and postoperative examinations were unremarkable. At presentation, his best-corrected visual acuity was 1/60 in the right eye and 6/18−1 in the left eye. Examination revealed mild vitritis and anterior chamber inflammation bilaterally, as well as extensive bilateral perivascular sheathing, retinal edema, and retinal hemorrhages (Figure 1). His right disk was edematous, but no afferent pupillary defect was noted. Vitreous hemorrhage obscured the view of his right macula. His left superior macula was thickened. Optical computed tomography showed bilateral intraretinal fluid. Fluoroscein angiography revealed bilateral venous and arteriolar sheathing (Figure 1). His history included diet-controlled diabetes mellitus, hypertension, congestive cardiac failure, mitral valve replacement (2005), and a curative cystectomy for transitional cell carcinoma (2001). His regular medications were 3 mg of warfarin daily, 240 mg of diltiazem daily, and 250 mg of digoxin daily. Preliminary investigations did not reveal an underlying cause, with no evidence of hematological malignancy on full blood examination, normal inflammatory markers and vasculitic screen, negative blood cultures and viral serologies (including HIV), normal chest x-ray, and a normal MRI of the brain and orbits. He had a transoesophageal echocardiogram that was reported as negative for vegetations. His age and prosthetic valve meant that we had a high index of suspicion for an infective etiology, despite no definitive investigation results. He underwent right vitreous and anterior chamber taps, which were tested for herpes simplex virus, varicella zoster virus, and cytomegalovirus polymerase chain reactions; gram staining; microscopy, culture, and sensitivities; and flow cytometry. He was treated empirically for bilateral viral retinitis with a dose of intravitreal ganciclovir 0.1 mL of 2 mg and intravenous acyclovir TDS. He was also commenced on topical Prednefrin Forte (prednisolone acetate 1% + phenylephrine hydrochloride 0.12%) hourly and atropine 1% TDS, as well as oral prednisone 1 mg/kg daily. Despite 7 days of treatment, his right visual acuity further deteriorated to finger counting (1 m), thus we began to consider atypical fungal infections and endogenous endophthalmitis. He was commenced on 200 mg of oral voriconazole daily and underwent a right vitrectomy with intravitreal injections of 10 mg of amphotericin, 2.2 mg of ceftazidime, and 1 mg of vancomycin. After vitrectomy, his right visual acuity improved to 6/24. Incidentally, 2 days later, his left vision worsened from 6/18 to 6/60 secondary to a vitreous hemorrhage. This was attributed to uncontrolled hypertension and an International Normalized Ratio of 4.6, rather than progression of ocular disease. All systemic antiinfective medications were ceased on Day 12 after his vitreous biopsy returned negative. Topical and oral steroids were not altered. Two days later, he became febrile and was found to have an elevated white cell count of 29.1 · 109 per None of the authors have any financial/conflicting interests to disclose. Reprint requests: Neharika Sharma, MBBS, Level 5 Eye Outpatients, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia; e-mail: [email protected]
liter and a raised C-reactive protein at 24 mg/L. Repeat blood cultures grew Streptococcus viridans and Streptococcus oralis. Review of his original transoesophageal echocardiogram revealed a 1.6-mm oscillating vegetation on his prosthetic valve that had initially been missed (Figure 2). In light of his streptococci-positive blood cultures and valvular lesion, our patient was diagnosed with infective endocarditis and commenced on 1 g of intravenous vancomycin daily (penicillin allergy).2 The infectious disease team believed that our patient had blood culture–negative endocarditis at presentation because the valvular lesion had been present throughout the course of his FBA.2 This hypothesis correlated well with the clinical picture because there was regression of ocular disease with the commencement of vancomycin. On discharge, 1 month later, best-corrected visual acuity was 6/12 bilaterally. Two months after discharge, he presented with a rubeotic right eye secondary to retinal ischemia. He was treated with a dose of intracameral bevacizumab, four doses of intravitreal bevacizumab, and panretinal photocoagulation in his right eye. He subsequently had prophylactic panretinal photocoagulation in his left eye because of extensive angiographic capillary dropout. Eight months after presentation, his best-corrected visual acuity was 6/24 in the right eye and 6/9 in the left eye.
Discussion Kleiner divided FBA into 3 categories—masquerade, frosted branch response, and idiopathic.1 Masquerade refers to a frosted branch-like picture associated with hematological malignancies, where vascular sheathing is secondary to malignant infiltration of vessel walls rather than an inflammatory process.1 Frosted branch response is when the trademark clinical appearance occurs secondary to active intraocular infection or autoimmune disease.1 The term idiopathic FBA was used to describe a group of otherwise young and healthy patients who presented with a similar cluster of signs without an identifiable cause.1 Since then, there has been much debate as to whether idiopathic FBA is a distinct syndrome or a clinical sign of posterior uveitis.1,3,4 Although our patient presented with the clinical features of FBA, he proved to be a diagnostic challenge, as negative results for active infection, autoimmune disease, and hematological malignancy, suggested that he had idiopathic FBA.1 However, to date, the oldest documented case of idiopathic FBA was in a 42-year-old Korean, and the average age of presentation in whites is during the third decade of life.1 Therefore, given our patient’s age and the rare nature of this condition, we believed that idiopathic FBA was unlikely and proceeded to empirically treat more common causes of retinal vasculitis in elderly patients.3–5 Interestingly, 2 weeks after his diagnosis of FBA, our patient fulfilled the diagnostic criteria for infective endocarditis. It is likely that he had blood
FBA ASSOCIATED WITH INFECTIVE ENDOCARDITIS
49
Fig. 1. Color fundus photographs and fundus fluorescein angiograms (FFA) from presentation and Day 4 of admission. A. Right fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema, vitreous hemorrhage obscuring view of macula. B. Left fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema. C. Right fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema, vitreous hemorrhage obscuring view of macula. D. Left fundus photograph at presentation: arteriolar and venous sheathing, retinal hemorrhage and edema. E. Right FFA at presentation: perivenular leakage and capillary dropout, no vascular occlusion. F. Left FFA at presentation: perivenular leakage and capillary dropout, no vascular occlusion. G. Right fundus photograph at Day 4: less marked arteriolar and venous sheathing, clearing vitreous hemorrhage, ongoing retinal hemorrhage and edema. H. Left fundus photograph at Day 4: less marked arteriolar and venous sheathing, ongoing retinal hemorrhage and edema. I. Right FFA at Day 4: less extensive leakage, capillary dropout still present. J. Left FFA at Day 4: less extensive leakage, capillary dropout still present.
culture–negative endocarditis at presentation. We note that blood cultures can remain negative in 2% to 7% of patients with infective endocarditis, excluding those with previous antibiotic treatment and improper culturing technique.2 In light of this, we hypothesized that FBA was secondary to a poststreptococcal immunogenic response.6–10 Uveitis, retinitis, and even a case of FBA have been reported poststreptococcal infection. All of these reported cases of poststreptococcal ocular inflammation occurred secondary to initial
infection with beta-hemolytic streptococcal strains, as was suggested by the type of preceding streptococcal infection (e.g., streptococcal pharyngitis) and by the use of a high antistreptolysin-O titer as diagnostic evidence.8–10 Therefore, pathogenesis was thought to be secondary to an autoimmune process triggered by the cross-reactivity of Group A streptococcal M-proteins and retinal S-antigens.7–10 A distinguishing feature of our case was that this seemed to be the first immunogenic response secondary to alpha-hemolytic streptococci.
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RETINAL CASES & BRIEF REPORTS´ 2015 VOLUME 9 NUMBER 1
Fig. 2. Freeze frame of transoesophageal echocardiogram depicting the oscillating mass seen on the prosthetic mitral valve.
The only association in the literature between alpha-hemolytic streptococci and FBA was that of a Streptococcus mitis endophthalmitis causing a frosted branch response. However, this was due to active ocular infection not an immunogenic response.1,6 The cardinal teaching points of our case are that FBA in elderly patients requires thorough evaluation to rule out systemic causes and that FBA can occur secondary to an immunogenic response to alphahemolytic streptococcal infections. Furthermore, the advanced age of our patient, plus a new immunogenic association, adds weight to the theory that FBA is in fact a clinical sign of posterior uveitis, rather than a distinct clinical syndrome. Key words: blood culture–negative endocarditis, elderly, frosted branch angiitis, retinal vasculitis, poststreptococcal. Acknowledgment The authors thank Alan Hoare1 for his technical assistance with taking and editing the displayed clinical photographs and fundus fluorescein angiograms.
References 1. Walker S, Iguchi A, Jones NP. Frosted branch angiitis: a review. Eye (Lond) 2004;18:527–533. 2. Raoult D, Sexton DJ. Culture negative endocarditis. Up To Date. 2012. 3. Kleiner RC. Frosted branch angiitis: clinical syndrome or clinical sign. Retina 1997;17:370–371. 4. Davis JL. Diagnostic dilemmas in retinitis and endophthalmitis. Eye (Lond) 2012;26:194–201. 5. Silva RA, Albini TA, Flynn HW. It still Happens Today: bilateral frosted Branch angiitis in cytomegalovirus retinitis. Retin Physician 2010 [cited Jan 2014]; feature article 8: 5 pages. Available at: http://www.retinalphysician.com/articleviewer. aspx?articleID=104854. 6. Chen E, Ho AC, Garg SJ, et al. Streptococcus mitis endophthalmitis presenting as frosted branch angiitis after intravitreal pegaptanib sodium injection. Ophthalmic Surg Lasers Imaging 2009;40:192–194. 7. Lerner MP, Donoso LA, Nordquist RE, Cunningham MW. Immunological mimicry between retinal S-antigens and Group A streptococcal M proteins. Autoimmunity 1995;22:95–106. 8. Han J, Lee SC, Song WK. Recurrent bilateral retinal vasculitis as a manifestation of post-streptococcal uveitis syndrome. Korean J Ophthalmol 2012;26:309–311. 9. Reddy UP, Albini TA, Banta JT. Post-streptococcal vasculitis. Ocul Immunol Inflamm 2008;16:35–36. 10. Kono H, Ikewaki J, Kimoto K, et al. Frosted branch angiitis associated with streptococcal infection: optical coherence tomography as a follow-up tool. Acta Ophthalmol 2009;87:909–911.
