ACUTE IDIOPATHIC MULTIFOCAL HEMORRHAGIC RETINAL VASCULITIS WITH TRANSIENT PROFOUND VISUAL LOSS Guri Bronner, MD, Sumit Shah, MD, Neelakshi Bhagat, MD, MPH, Marco Zarbin, MD, PHD
Background: Acute idiopathic multifocal hemorrhagic retinal vasculitis is a rare entity. The diagnosis is based on typical fundus findings following exclusion of other more common conditions with similar features. The authors present a case that outlines their approach to diagnosis and management of a patient with simultaneous bilateral presentation. Method: Retrospective case report. Results: This diagnosis often carries a poor prognosis. However, the reported patient did well, with an improvement in vision from light perception in the right eye and hand motions in the left eye at presentation to 20/60 in the right eye and 20/25 in the left eye at 24-month follow-up. Management included intravitreal ganciclovir, oral corticosteroids, panretinal photocoagulation in both eyes, and vitrectomy in one eye. Conclusion: Patients with acute idiopathic multifocal hemorrhagic choroiditis can exhibit delayed and substantial visual improvement despite initial profound visual loss. The authors discuss this case and review the current understanding of the diagnosis and management of this disease. RETINAL CASES & BRIEF REPORTS 2:178 –180, 2008
From the Institute of Ophthalmology and Visual Science, New Jersey Medical School, University of Medicine and Dentistry of New Jersey, Newark.
subsequently referred to us by an ophthalmologist who administered one dose of 650 mg acyclovir intravenously. At presentation, visual acuity was light perception in the right eye and hand motions in the left. She had moderate conjunctival injection and moderate nongranulomatous anterior uveitis bilaterally. Examination of the right posterior segment disclosed media clarity of 20/30 to 20/40 with mild vitritis. The retina was flat, and there were intraretinal hemorrhages along the temporal arcades as well as marked macular edema (Figures 1 and 2). Intense perivascular whitening and perivascular retinitis with foveal involvement was present. The cup to disk ratio was 0.1, and the optic nerve head was hyperemic. There was no peripheral retinitis. A fluorescein angiogram showed dye leakage from the optic nerve head, veins, and, in some areas, arterioles (Figures 3 and 4). Findings were similar in the left eye.
A
35-year-old woman presented to our emergency department on July 16, 2003, with a 4-day history of conjunctival injection followed by photophobia. During the 24 hours before presentation, she developed bilateral visual loss. The patient had no significant past medical history with the exception of recent flulike symptoms during the prior week, for which she was started on erythromycin 1 day earlier by her primary physician. The patient was evaluated and
Supported in part by grants from Research to Prevent Blindness, Inc., the New Jersey Lions Eye Research Foundation, and the Eye Institute of New Jersey. The authors have no proprietary interest in the contents of this article. Reprint requests: Marco Zarbin, MD, PhD, Institute of Ophthalmology and Visual Science, New Jersey Medical School, DOC Building, Suite 6100, 90 Bergen Street, Newark, NJ 07103.
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Fig. 1. Right eye at presentation. Note intraretinal hemorrhage, perivascular retinal whitening, and, in areas of retina without hemorrhage, periphlebitis.
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Fig. 3. Late phase fluorescein angiogram of right eye at presentation demonstrating papillitis, periarteritis, and periphlebitis.
Because of the possibility of viral retinitis, the patient underwent bilateral intravitreal ganciclovir injection (2 mg) and vitreous biopsy. The patient was admitted to the hospital and was treated with intravenous ganciclovir 350 mg every 12 hours, aspirin 325 mg per day, as well as topical prednisolone acetate 1% suspension and atropine 1% solution to both eyes every 6 hours and every 12 hours, respectively. Her condition progressed rapidly with vision diminishing to bare light perception in both eyes, increasing macular edema, and eventually bilateral inferior serous retinal detachments. On hospital day 3 oral prednisone (60 mg [1 mg/kg] PO QD) was added to the regimen. This dose was continued for 10 days followed by a rapid taper over 3 days. Ganciclovir was discontinued on the ninth hospital day due to negative polymerase chain reaction (PCR) results of the vitreous biopsy (see below).
A lumbar puncture was unremarkable including negative cerebrospinal fluid cytology, Gram stain, cultures, Venereal Disease Research Laboratory, and antigen screens for Haemophilus influenzae, Streptococcus pneumonia, Neisseria meningitidis, Group B Streptococcus, and Cryptococcus. Serology was negative for human immunodeficiency virus, rapid plasma reagin, fluorescent treponemal antibody, Toxoplasma IgG and IgM, Lyme, -HCG, and antinuclear antibodies. Erythrocyte sedimentation rate was 16 mm/hr. Angiotensin converting enzyme was 15 U/L. Cytomegalovirus (CMV) IgG was positive, but IgM was negative. Urinalysis was culture-negative and was within normal limits on repeat examination following initial presence of 4 RBC/HPF. Chest x-ray was normal. Purified protein derivative testing was negative. Gram and acid-fast bacilli stain of the vitreous biopsy
Fig. 2. Left eye at presentation. Less marked intraretinal hemorrhage and retinal whitening are present as are periarteritis and periphlebitis.
Fig. 4. Late phase fluorescein angiogram of left eye at presentation demonstrating findings similar to those of the right eye.
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specimen was negative. Biopsy also yielded negative PCR results for Epstein-Barr virus, varicella zoster virus, CMV, and herpes simplex virus. With this negative infectious disease workup, particularly the results from vitreous PCR for viral disease, other entities such as postviral periphlebitis and frosted branch angiitis seemed less likely. Since there was no clear demonstrable association with a virus and due to the characteristic appearance of the fundus, the patient was diagnosed with acute multifocal hemorrhagic retinal vasculitis. Subsequently, the patient developed retinal ischemia and retinal neovascularization bilaterally. She also developed a nonclearing vitreous hemorrhage on the right for which she underwent uncomplicated vitrectomy and panretinal photocoagulation. The left eye responded to panretinal photocoagulation alone. Her visual acuity improved and remained stable at 20/60 on the right and 20/25 on the left 24 months after presentation.
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alone3 can give the clinical picture of frosted branch vasculitis. Acute retinal necrosis can present with a similar picture and is often associated with herpesvirus infection.4 These conditions often are associated with positive laboratory findings, response to antiviral medication, and late retinal detachment. Negative PCR results in our patient ruled out these possibilities, and lack of response to antiviral medication was consistent with this conclusion. Patients with systemic lupus erythematosus or Crohn’s disease may present secondarily with frosted branch vasculitis, usually in the setting of established systemic disease.5,6 Ocular lymphoma can mimic a retinal vasculitis and should be considered in vitreous biopsy assessment.7 If the clinical course is unusual, such as poor response to steroids, heightened suspicion may prompt a retinal biopsy. Of note, our patient achieved a sustained response to steroid treatment. This case provides an unusual example of significant visual improvement following profound visual loss in idiopathic acute multifocal hemorrhagic retinal vasculitis.
Discussion Acute multifocal hemorrhagic retinal vasculitis is a rare entity. The diagnosis is based on typical fundus findings following exclusion of other more common conditions with similar features. Typical presentation involves retinal vasculitis with hemorrhages, vitritis, and anterior uveitis. Retinal infiltrates are predominantly located in the posterior pole. In one series, most patients developed neovascularization as a late sequela.1 Thus, this patient’s presentation and clinical course were characteristic of the condition. This otherwise healthy young woman did complain of a viral prodrome but no other associated systemic or ocular involvement. Steroids have been shown to be of possible benefit in this disease.1 Treatment with oral prednisone correlated with symptomatic improvement in this patient. The differential diagnosis for the described clinical picture includes the following entities. CMV retinitis2 and possibly human immunodeficiency virus infection
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References 1.
2.
3.
4.
5.
6.
7.
Blumenkranz MS, Kaplan HJ, Clarkson JG, et al. Acute multifocal hemorrhagic retinal vasculitis. Ophthalmology 1988; 95:1663–1672. Spaide RF, Vitale AT, Toth IR, Oliver JM. Frosted branch angiitis associated with cytomegalovirus retinitis. Am J Ophthalmol 1992;113:522–528. Fine HF, Smith JA, Murante BL, Nussenblatt RB, Robinson MR. Frosted branch angiitis in a child with HIV infection. Am J Ophthalmol 2001;131:394–396. Duker JS, Blumenkranz MS. Diagnosis and management of the acute retinal necrosis (ARN) syndrome. Surv Ophthalmol 1991;35:327–343. Quillen DA, Stathopoulos NA, Blankenship GW, Ferriss JA. Lupus associated frosted branch periphlebitis and exudative maculopathy. Retina 1997;17:449–451. Sykes SO, Horton JC. Steroid-responsive retinal vasculitis with a frosted branch appearance in Crohns disease. Retina 1997;17:451–454. Ridley ME, McDonald HR, Sternberg P Jr, Blumenkranz MS, Zarbin MA, Schachat AP. Retinal manifestations of ocular lymphoma (reticulum cell sarcoma). Ophthalmology 1992;99: 1153–1161.
Background: Acute idiopathic multifocal hemorrhagic retinal vasculitis is a rare entity. The diagnosis is based on typical fundus findings following exclusion of other more common conditions with similar features. The authors present a case that outlines their approach to diagnosis and management of a patient with simultaneous bilateral presentation. Method: Retrospective case report. Results: This diagnosis often carries a poor prognosis. However, the reported patient did well, with an improvement in vision from light perception in the right eye and hand motions in the left eye at presentation to 20/60 in the right eye and 20/25 in the left eye at 24-month follow-up. Management included intravitreal ganciclovir, oral corticosteroids, panretinal photocoagulation in both eyes, and vitrectomy in one eye. Conclusion: Patients with acute idiopathic multifocal hemorrhagic choroiditis can exhibit delayed and substantial visual improvement despite initial profound visual loss. The authors discuss this case and review the current understanding of the diagnosis and management of this disease. RETINAL CASES & BRIEF REPORTS 2:178 –180, 2008
From the Institute of Ophthalmology and Visual Science, New Jersey Medical School, University of Medicine and Dentistry of New Jersey, Newark.
subsequently referred to us by an ophthalmologist who administered one dose of 650 mg acyclovir intravenously. At presentation, visual acuity was light perception in the right eye and hand motions in the left. She had moderate conjunctival injection and moderate nongranulomatous anterior uveitis bilaterally. Examination of the right posterior segment disclosed media clarity of 20/30 to 20/40 with mild vitritis. The retina was flat, and there were intraretinal hemorrhages along the temporal arcades as well as marked macular edema (Figures 1 and 2). Intense perivascular whitening and perivascular retinitis with foveal involvement was present. The cup to disk ratio was 0.1, and the optic nerve head was hyperemic. There was no peripheral retinitis. A fluorescein angiogram showed dye leakage from the optic nerve head, veins, and, in some areas, arterioles (Figures 3 and 4). Findings were similar in the left eye.
A
35-year-old woman presented to our emergency department on July 16, 2003, with a 4-day history of conjunctival injection followed by photophobia. During the 24 hours before presentation, she developed bilateral visual loss. The patient had no significant past medical history with the exception of recent flulike symptoms during the prior week, for which she was started on erythromycin 1 day earlier by her primary physician. The patient was evaluated and
Supported in part by grants from Research to Prevent Blindness, Inc., the New Jersey Lions Eye Research Foundation, and the Eye Institute of New Jersey. The authors have no proprietary interest in the contents of this article. Reprint requests: Marco Zarbin, MD, PhD, Institute of Ophthalmology and Visual Science, New Jersey Medical School, DOC Building, Suite 6100, 90 Bergen Street, Newark, NJ 07103.
178
ACUTE IDIOPATHIC MULTIFOCAL VASCULITIS
Fig. 1. Right eye at presentation. Note intraretinal hemorrhage, perivascular retinal whitening, and, in areas of retina without hemorrhage, periphlebitis.
179
Fig. 3. Late phase fluorescein angiogram of right eye at presentation demonstrating papillitis, periarteritis, and periphlebitis.
Because of the possibility of viral retinitis, the patient underwent bilateral intravitreal ganciclovir injection (2 mg) and vitreous biopsy. The patient was admitted to the hospital and was treated with intravenous ganciclovir 350 mg every 12 hours, aspirin 325 mg per day, as well as topical prednisolone acetate 1% suspension and atropine 1% solution to both eyes every 6 hours and every 12 hours, respectively. Her condition progressed rapidly with vision diminishing to bare light perception in both eyes, increasing macular edema, and eventually bilateral inferior serous retinal detachments. On hospital day 3 oral prednisone (60 mg [1 mg/kg] PO QD) was added to the regimen. This dose was continued for 10 days followed by a rapid taper over 3 days. Ganciclovir was discontinued on the ninth hospital day due to negative polymerase chain reaction (PCR) results of the vitreous biopsy (see below).
A lumbar puncture was unremarkable including negative cerebrospinal fluid cytology, Gram stain, cultures, Venereal Disease Research Laboratory, and antigen screens for Haemophilus influenzae, Streptococcus pneumonia, Neisseria meningitidis, Group B Streptococcus, and Cryptococcus. Serology was negative for human immunodeficiency virus, rapid plasma reagin, fluorescent treponemal antibody, Toxoplasma IgG and IgM, Lyme, -HCG, and antinuclear antibodies. Erythrocyte sedimentation rate was 16 mm/hr. Angiotensin converting enzyme was 15 U/L. Cytomegalovirus (CMV) IgG was positive, but IgM was negative. Urinalysis was culture-negative and was within normal limits on repeat examination following initial presence of 4 RBC/HPF. Chest x-ray was normal. Purified protein derivative testing was negative. Gram and acid-fast bacilli stain of the vitreous biopsy
Fig. 2. Left eye at presentation. Less marked intraretinal hemorrhage and retinal whitening are present as are periarteritis and periphlebitis.
Fig. 4. Late phase fluorescein angiogram of left eye at presentation demonstrating findings similar to those of the right eye.
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specimen was negative. Biopsy also yielded negative PCR results for Epstein-Barr virus, varicella zoster virus, CMV, and herpes simplex virus. With this negative infectious disease workup, particularly the results from vitreous PCR for viral disease, other entities such as postviral periphlebitis and frosted branch angiitis seemed less likely. Since there was no clear demonstrable association with a virus and due to the characteristic appearance of the fundus, the patient was diagnosed with acute multifocal hemorrhagic retinal vasculitis. Subsequently, the patient developed retinal ischemia and retinal neovascularization bilaterally. She also developed a nonclearing vitreous hemorrhage on the right for which she underwent uncomplicated vitrectomy and panretinal photocoagulation. The left eye responded to panretinal photocoagulation alone. Her visual acuity improved and remained stable at 20/60 on the right and 20/25 on the left 24 months after presentation.
2008
●
VOLUME 2
●
NUMBER 2
alone3 can give the clinical picture of frosted branch vasculitis. Acute retinal necrosis can present with a similar picture and is often associated with herpesvirus infection.4 These conditions often are associated with positive laboratory findings, response to antiviral medication, and late retinal detachment. Negative PCR results in our patient ruled out these possibilities, and lack of response to antiviral medication was consistent with this conclusion. Patients with systemic lupus erythematosus or Crohn’s disease may present secondarily with frosted branch vasculitis, usually in the setting of established systemic disease.5,6 Ocular lymphoma can mimic a retinal vasculitis and should be considered in vitreous biopsy assessment.7 If the clinical course is unusual, such as poor response to steroids, heightened suspicion may prompt a retinal biopsy. Of note, our patient achieved a sustained response to steroid treatment. This case provides an unusual example of significant visual improvement following profound visual loss in idiopathic acute multifocal hemorrhagic retinal vasculitis.
Discussion Acute multifocal hemorrhagic retinal vasculitis is a rare entity. The diagnosis is based on typical fundus findings following exclusion of other more common conditions with similar features. Typical presentation involves retinal vasculitis with hemorrhages, vitritis, and anterior uveitis. Retinal infiltrates are predominantly located in the posterior pole. In one series, most patients developed neovascularization as a late sequela.1 Thus, this patient’s presentation and clinical course were characteristic of the condition. This otherwise healthy young woman did complain of a viral prodrome but no other associated systemic or ocular involvement. Steroids have been shown to be of possible benefit in this disease.1 Treatment with oral prednisone correlated with symptomatic improvement in this patient. The differential diagnosis for the described clinical picture includes the following entities. CMV retinitis2 and possibly human immunodeficiency virus infection
●
References 1.
2.
3.
4.
5.
6.
7.
Blumenkranz MS, Kaplan HJ, Clarkson JG, et al. Acute multifocal hemorrhagic retinal vasculitis. Ophthalmology 1988; 95:1663–1672. Spaide RF, Vitale AT, Toth IR, Oliver JM. Frosted branch angiitis associated with cytomegalovirus retinitis. Am J Ophthalmol 1992;113:522–528. Fine HF, Smith JA, Murante BL, Nussenblatt RB, Robinson MR. Frosted branch angiitis in a child with HIV infection. Am J Ophthalmol 2001;131:394–396. Duker JS, Blumenkranz MS. Diagnosis and management of the acute retinal necrosis (ARN) syndrome. Surv Ophthalmol 1991;35:327–343. Quillen DA, Stathopoulos NA, Blankenship GW, Ferriss JA. Lupus associated frosted branch periphlebitis and exudative maculopathy. Retina 1997;17:449–451. Sykes SO, Horton JC. Steroid-responsive retinal vasculitis with a frosted branch appearance in Crohns disease. Retina 1997;17:451–454. Ridley ME, McDonald HR, Sternberg P Jr, Blumenkranz MS, Zarbin MA, Schachat AP. Retinal manifestations of ocular lymphoma (reticulum cell sarcoma). Ophthalmology 1992;99: 1153–1161.
![[Retinal vasculitis with multifocal chorioretinitis].](/assets/img/hold.png)
