Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
Bilateral Neuroretinitis and Retinal Hemorrhages in an Immunocompetent Patient Nadim Rayess, MD; Ehsan Rahimy, MD; James P. Dunn, MD
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B
Figure 1. Baseline montage color fundus photographs. A, Right eye demonstrates retinal whitening superior to the optic nerve adjacent to several pigmented chorioretinal scars. B, Left eye demonstrates a preretinal gliotic lesion with a macular star.
A previously healthy woman in her early 20s presented with bilateral decreased vision of 2 weeks’ duration in the left eye and 5 days’ duration in the right eye. She denied any medication use and had no history of ocular surgery or known ophthalmic disorders. Bestcorrected visual acuity was counting fingers at 1 ft OD and 20/300 OS. There was no afferent pupillary deQuiz at fect. Slitlamp examination revealed fine keratic precipijamaophthalmology.com tates bilaterally, with 2+ anterior chamber cells in her right eye and 0.5+ cells in her left eye. Dilated fundus examination of the right eye demonstrated a creamy white lesion emanating superiorly from the optic nerve associated with areas of intraretinal hemorrhage and extensive perivascular sheathing along the vascular arcades into the midperiphery (Figure 1A). Ophthalmoscopic examination of the left eye showed a peripapillary preretinal gliotic lesion exerting mild traction on the macula with fine retinal striae and a macular star configuration of exudates (Figure 1B). Spectral-domain optical coherence tomography revealed significant cystoid macular edema with associated subretinal fluid in the right eye and hard exudates without evidence of cystoid macular edema in the left eye.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Diagnostic pars plana vitrectomy B. Neuroimaging of brain and orbits C. Anterior chamber paracentesis for polymerase chain reaction D. Blood cultures
JAMA Ophthalmology October 2014 Volume 132, Number 10
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/30/2015
1243
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
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B
Figure 2. Six-week follow-up color fundus photographs. A, Right eye demonstrates a consolidating white lesion with the development of a macular star. B, Left eye shows a stable preretinal gliotic lesion.
Diagnosis Bilateral toxoplasmosis neuroretinitis
What To Do Next C. Anterior chamber paracentesis for polymerase chain reaction
Discussion Given the patient’s presentation of bilateral neuroretinitis, we highly suspected viral neuroretinitis or toxoplasmosis neuroretinitis. A diagnostic anterior chamber paracentesis was performed and she was treated empirically with bilateral intravitreal injections of clindamycin phosphate, foscarnet sodium, and ganciclovir sodium to cover for toxoplasmosis and herpetic viral retinitis. She began treatment with systemic pyrimethamine (100-mg loading dose followed by 50 mg/d thereafter), sulfadiazine (4 g/d), and folinic acid (25 mg/d), topical hourly prednisolone acetate, and twice-daily atropine sulfate eyedrops. Subsequent magnetic resonance imaging findings were unremarkable for intracranial or intraorbital pathology. Polymerase chain reaction results of aqueous humor were positive for Toxoplasma gondii and negative for cytomegalovirus, herpes simplex virus types 1 and 2, and varicella-zoster virus. Serological workup revealed positive T gondii IgG titers but was otherwise negative for an infectious or autoimmune etiology. After the third day an oral prednisone taper beginning at 60 mg/d was initiated. It is not uncommon for individuals with acquired toxoplasmic retinochoroiditis to present without systemic symptoms such as myalgias and chills.1 Vascular involvement, noted as perivascular sheathing in our case, is often seen in toxoplasmosis retinochoroiditis.2 Patients with neuroretinitis typically present with moderate to severe vision loss with associated optic nerve head edema.3 Gilbert4 described 2 patients with isolated toxoplasmic neuroretinitis, of whom
1244
one had optic nerve neovascularization and the other had optic nerve edema associated with a macular star. Fish et al5 described 5 patients with isolated unilateral toxoplasmic neuroretinitis who presented with optic nerve edema and a macular star. Bilateral toxoplasmosis retinochoroiditis is extremely rare in immunocompetent patients. Perrotta et al3 described a 6-year-old boy with bilateral neuroretinitis due to acquired toxoplasmosis. In contrast, CochereauMassin et al6 reported bilateral involvement in 8 of 45 patients (18%) with AIDS and ocular toxoplasmosis. Our patient was negative for human immunodeficiency virus and was not taking any immunosuppressive medications. She did live in Brazil throughout her childhood, and southern Brazil has a higher prevalence of the more virulent type I strain of T gondii.7 Clinicians should be aware that bilateral peripapillary involvement may be an early manifestation of toxoplasmic neuroretinitis, with subsequent formation of the characteristic macular star and potential for substantial visual acuity improvement with appropriate therapy.
Patient Outcome At 6-week follow-up, best-corrected visual acuity had improved to 20/50 OU. Ophthalmoscopic examination of the right eye showed consolidation of the white lesion with development of a macular star pattern following resolution of cystoid macular edema and subretinal fluid (Figure 2A), clearing of the intraretinal hemorrhages, and significant improvement of the perivascular sheathing. The left eye showed a stable preretinal gliotic lesion with mild macular traction and a macular star as before (Figure 2B). She maintained pyrimethamine, sulfadiazine, folinic acid, and oral prednisone taper for 2 months, while the topical corticosteroids and cycloplegic eyedrops were discontinued once her anterior chamber reaction resolved.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Retina Service, Wills Eye Hospital, Mid Atlantic Retina, Philadelphia, Pennsylvania.
1. Holland GN. Ocular toxoplasmosis. Am J Ophthalmol. 2003;136(6):973-988.
Corresponding Author: James P. Dunn, MD, Wills Eye Hospital, 840 Walnut St, Ste 1020, Philadelphia, PA 19107 ([email protected]).
2. Theodossiadis P, Kokolakis S, Ladas I, Kollia AC, Chatzoulis D, Theodossiadis G. Retinal vascular involvement in acute toxoplasmic retinochoroiditis. Int Ophthalmol. 1995;19(1):19-24.
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
3. Perrotta S, Nobili B, Grassia C, Sebastiani A, Parmeggiani F, Costagliola C. Bilateral neuroretinitis in a 6-year-old boy with acquired toxoplasmosis. Arch Ophthalmol. 2003;121(10):1493-1496.
4. Gilbert HD. Unusual presentation of acute ocular toxoplasmosis. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1980;215(1):53-58. 5. Fish RH, Hoskins JC, Kline LB. Toxoplasmosis neuroretinitis. Ophthalmology. 1993;100(8):1177-1182. 6. Cochereau-Massin I, LeHoang P, Lautier-Frau M, et al. Ocular toxoplasmosis in human immunodeficiency virus-infected patients. Am J Ophthalmol. 1992;114(2):130-135. 7. Sibley LD, Boothroyd JC. Virulent strains of Toxoplasma gondii comprise a single clonal lineage. Nature. 1992;359(6390):82-85.
JAMA Ophthalmology October 2014 Volume 132, Number 10
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/30/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
Bilateral Neuroretinitis and Retinal Hemorrhages in an Immunocompetent Patient Nadim Rayess, MD; Ehsan Rahimy, MD; James P. Dunn, MD
A
B
Figure 1. Baseline montage color fundus photographs. A, Right eye demonstrates retinal whitening superior to the optic nerve adjacent to several pigmented chorioretinal scars. B, Left eye demonstrates a preretinal gliotic lesion with a macular star.
A previously healthy woman in her early 20s presented with bilateral decreased vision of 2 weeks’ duration in the left eye and 5 days’ duration in the right eye. She denied any medication use and had no history of ocular surgery or known ophthalmic disorders. Bestcorrected visual acuity was counting fingers at 1 ft OD and 20/300 OS. There was no afferent pupillary deQuiz at fect. Slitlamp examination revealed fine keratic precipijamaophthalmology.com tates bilaterally, with 2+ anterior chamber cells in her right eye and 0.5+ cells in her left eye. Dilated fundus examination of the right eye demonstrated a creamy white lesion emanating superiorly from the optic nerve associated with areas of intraretinal hemorrhage and extensive perivascular sheathing along the vascular arcades into the midperiphery (Figure 1A). Ophthalmoscopic examination of the left eye showed a peripapillary preretinal gliotic lesion exerting mild traction on the macula with fine retinal striae and a macular star configuration of exudates (Figure 1B). Spectral-domain optical coherence tomography revealed significant cystoid macular edema with associated subretinal fluid in the right eye and hard exudates without evidence of cystoid macular edema in the left eye.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Diagnostic pars plana vitrectomy B. Neuroimaging of brain and orbits C. Anterior chamber paracentesis for polymerase chain reaction D. Blood cultures
JAMA Ophthalmology October 2014 Volume 132, Number 10
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/30/2015
1243
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
A
B
Figure 2. Six-week follow-up color fundus photographs. A, Right eye demonstrates a consolidating white lesion with the development of a macular star. B, Left eye shows a stable preretinal gliotic lesion.
Diagnosis Bilateral toxoplasmosis neuroretinitis
What To Do Next C. Anterior chamber paracentesis for polymerase chain reaction
Discussion Given the patient’s presentation of bilateral neuroretinitis, we highly suspected viral neuroretinitis or toxoplasmosis neuroretinitis. A diagnostic anterior chamber paracentesis was performed and she was treated empirically with bilateral intravitreal injections of clindamycin phosphate, foscarnet sodium, and ganciclovir sodium to cover for toxoplasmosis and herpetic viral retinitis. She began treatment with systemic pyrimethamine (100-mg loading dose followed by 50 mg/d thereafter), sulfadiazine (4 g/d), and folinic acid (25 mg/d), topical hourly prednisolone acetate, and twice-daily atropine sulfate eyedrops. Subsequent magnetic resonance imaging findings were unremarkable for intracranial or intraorbital pathology. Polymerase chain reaction results of aqueous humor were positive for Toxoplasma gondii and negative for cytomegalovirus, herpes simplex virus types 1 and 2, and varicella-zoster virus. Serological workup revealed positive T gondii IgG titers but was otherwise negative for an infectious or autoimmune etiology. After the third day an oral prednisone taper beginning at 60 mg/d was initiated. It is not uncommon for individuals with acquired toxoplasmic retinochoroiditis to present without systemic symptoms such as myalgias and chills.1 Vascular involvement, noted as perivascular sheathing in our case, is often seen in toxoplasmosis retinochoroiditis.2 Patients with neuroretinitis typically present with moderate to severe vision loss with associated optic nerve head edema.3 Gilbert4 described 2 patients with isolated toxoplasmic neuroretinitis, of whom
1244
one had optic nerve neovascularization and the other had optic nerve edema associated with a macular star. Fish et al5 described 5 patients with isolated unilateral toxoplasmic neuroretinitis who presented with optic nerve edema and a macular star. Bilateral toxoplasmosis retinochoroiditis is extremely rare in immunocompetent patients. Perrotta et al3 described a 6-year-old boy with bilateral neuroretinitis due to acquired toxoplasmosis. In contrast, CochereauMassin et al6 reported bilateral involvement in 8 of 45 patients (18%) with AIDS and ocular toxoplasmosis. Our patient was negative for human immunodeficiency virus and was not taking any immunosuppressive medications. She did live in Brazil throughout her childhood, and southern Brazil has a higher prevalence of the more virulent type I strain of T gondii.7 Clinicians should be aware that bilateral peripapillary involvement may be an early manifestation of toxoplasmic neuroretinitis, with subsequent formation of the characteristic macular star and potential for substantial visual acuity improvement with appropriate therapy.
Patient Outcome At 6-week follow-up, best-corrected visual acuity had improved to 20/50 OU. Ophthalmoscopic examination of the right eye showed consolidation of the white lesion with development of a macular star pattern following resolution of cystoid macular edema and subretinal fluid (Figure 2A), clearing of the intraretinal hemorrhages, and significant improvement of the perivascular sheathing. The left eye showed a stable preretinal gliotic lesion with mild macular traction and a macular star as before (Figure 2B). She maintained pyrimethamine, sulfadiazine, folinic acid, and oral prednisone taper for 2 months, while the topical corticosteroids and cycloplegic eyedrops were discontinued once her anterior chamber reaction resolved.
ARTICLE INFORMATION
REFERENCES
Author Affiliations: Retina Service, Wills Eye Hospital, Mid Atlantic Retina, Philadelphia, Pennsylvania.
1. Holland GN. Ocular toxoplasmosis. Am J Ophthalmol. 2003;136(6):973-988.
Corresponding Author: James P. Dunn, MD, Wills Eye Hospital, 840 Walnut St, Ste 1020, Philadelphia, PA 19107 ([email protected]).
2. Theodossiadis P, Kokolakis S, Ladas I, Kollia AC, Chatzoulis D, Theodossiadis G. Retinal vascular involvement in acute toxoplasmic retinochoroiditis. Int Ophthalmol. 1995;19(1):19-24.
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
3. Perrotta S, Nobili B, Grassia C, Sebastiani A, Parmeggiani F, Costagliola C. Bilateral neuroretinitis in a 6-year-old boy with acquired toxoplasmosis. Arch Ophthalmol. 2003;121(10):1493-1496.
4. Gilbert HD. Unusual presentation of acute ocular toxoplasmosis. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1980;215(1):53-58. 5. Fish RH, Hoskins JC, Kline LB. Toxoplasmosis neuroretinitis. Ophthalmology. 1993;100(8):1177-1182. 6. Cochereau-Massin I, LeHoang P, Lautier-Frau M, et al. Ocular toxoplasmosis in human immunodeficiency virus-infected patients. Am J Ophthalmol. 1992;114(2):130-135. 7. Sibley LD, Boothroyd JC. Virulent strains of Toxoplasma gondii comprise a single clonal lineage. Nature. 1992;359(6390):82-85.
JAMA Ophthalmology October 2014 Volume 132, Number 10
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/30/2015
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