Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
More Than Just Optic Disc Swelling Roomasa Channa, MD; Derek S. Welsbie, MD, PhD; Vivek R. Patel, MD
Figure 1. Fundus photograph centered over the optic nerve of the right eye.
A healthy woman in her 50s presents with sudden-onset, painless blurred vision in her right eye. She describes it as a black lace over her field of vision that she first noticed after waking 8 days prior to presentation. Owing to worsening vision, she was referred to us for further evaluation. She has no systemic symptoms and no past ocular or systemic health Quiz at problems. She does not take any medicajamaophthalmology.com tions. She is currently working and lives at home with her male partner and her pets. She is a nonsmoker and drinks alcohol socially. Her best-corrected visual acuity is 20/200 in the right eye and 20/20 in the left eye, with a right relative afferent pupil defect. The results of a slitlamp examination of the intraocular pressure, motility, and anterior segment in both eyes are unremarkable. Dilated examination findings of the left eye are normal. Results of a dilated examination of the right eye reveal a clear vitreous body, tortuous veins, and macula lutea, as shown in Figure 1.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Perform magnetic resonance imaging of the brain and orbits B. Use antibiotics C. Use steroids D. Order serologic tests
JAMA Ophthalmology November 2013 Volume 131, Number 11
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Grand Valley State University User on 06/10/2015
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Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Neuroretinitis from Bartonella henselae
What To Do Next D. Order serologic tests
Discussion Figure 1 shows a preretinal tuft of tissue over the optic nerve with a small portion of the nerve visible behind the tissue. The visible portion of the optic nerve is edematous with overlying nerve fiber layer edema with hemorrhage. The macula showed diffuse edema as depicted by the optical coherence tomographic scans in Figure 2. No macular exudates typical of neuroretinitis were visible at this time. However, the combination of optic disc edema with macular edema was suggestive of a form of neuroretinitis. The preretinal tuft of tissue is unusual but appeared similar to an inflammatory collection sometimes seen in cases of exuberant uveitis. An infectious or inflammatory process was strongly suspected. To narrow the differential diagnoses, we ordered testing for Bartonella, Lyme disease, syphilis, Toxocara, and Toxoplasma. We also ordered a computed tomographic scan of the chest to look for evidence of sarcoidosis or tuberculosis, but the results were normal. During a follow-up examination 4 days later, a classic macular star was visible, and the preretinal tuft of tissue had resolved. The Bartonella and Toxocara serologic test results were still pending at that time. Given the clinical appearance and relevant negative test results, we started her on doxycycline and prednisone. Two and a half weeks after the initial presentation, her serologic results were reported and confirmed our clinical diagnosis of Bartonella henselae. Our patient continued to show improvement in her visual symptoms, and by week 7, her vision was 20/30.
There are numerous ocular manifestations ofBartonella henselae, including optic neuropathy, neuroretinitis, vitritis, focal retinitis, and choroiditis. The preretinal tuft seen in our case, in addition to the findings of optic neuropathy and macular edema, makes the presentation of the disease unusual. Based on the findings of the Optic Neuritis Treatment Trial,1 we know that the presence of hemorrhages, poor vision, disc swelling, and the absence of pain are highly atypical features for a demyelinating disease. Hence, in this setting, it would be less useful to perform magnetic resonance imaging of the brain and orbits in order to look for a demyelinating disease. However, neuroimaging may help identify enhancement or thickening along the retrobulbar aspect of the optic nerve, as can be seen in infiltrative processes such as tuberculosis or sarcoidosis. Imaging could also be useful to identify a compressive lesion, but the relatively abrupt onset and clinical fundoscopic appearance argue against this mechanism. The appearance of the characteristic macular star is very helpful in reaching the diagnosis because relatively few ocular conditions lead to this stellate pattern of lipid deposition. However, it may not always be present at the time of initial presentation owing to extensive edema, and it does not develop in all cases. In the case series by Chi and colleagues,2 a macular star developed in 45% of 63 eyes that tested positive for cat-scratch disease associated with optic neuropathy. Other series3,4 reporting neuroretinitis to be the most common posterior segment complication of ocular cat-scratch disease have reported its occurrence in up to 64% to 71% of patients. The clinical constellation of retinal edema, disc edema, and painless loss of vision are suggestive of a diagnosis of neuroretinitis. The additional preretinal tuft seen in our case at initial presentation and its subsequent dissipation even before institution of antimicrobial therapy are likely due to the exuberant inflammatory response at the time of the initial examination, and, to the best of our knowledge, it has not been previously reported in connection with this entity.
A
B
Figure 2. Macular optical coherence tomographic (OCT) scan (A) with infrared fundus image (B) of the right eye showing macular edema.
ARTICLE INFORMATION
REFERENCES
Author Affiliation: Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.
1. Beck RW, Cleary PA, Anderson MM Jr, et al; The Optic Neuritis Study Group. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. N Engl J Med. 1992;326(9):581-588.
Corresponding Author: Derek S. Welsbie, MD, PhD, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287 ([email protected]). Conflict of Interest Disclosures: None reported.
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2. Chi SL, Stinnett S, Eggenberger E, et al. Clinical characteristics in 53 patients with cat scratch optic neuropathy. Ophthalmology. 2012;119(1):183-187.
3. Reed JB, Scales DK, Wong MT, Lattuada CP Jr, Dolan MJ, Schwab IR. Bartonella henselae neuroretinitis in cat scratch disease. Ophthalmology. 1998;105(3):459-466. 4. Ormerod LD, Dailey JP. Ocular manifestations of cat-scratch disease. Curr Opin Ophthalmol. 1999;10(3):209-216.
JAMA Ophthalmology November 2013 Volume 131, Number 11
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Grand Valley State University User on 06/10/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
More Than Just Optic Disc Swelling Roomasa Channa, MD; Derek S. Welsbie, MD, PhD; Vivek R. Patel, MD
Figure 1. Fundus photograph centered over the optic nerve of the right eye.
A healthy woman in her 50s presents with sudden-onset, painless blurred vision in her right eye. She describes it as a black lace over her field of vision that she first noticed after waking 8 days prior to presentation. Owing to worsening vision, she was referred to us for further evaluation. She has no systemic symptoms and no past ocular or systemic health Quiz at problems. She does not take any medicajamaophthalmology.com tions. She is currently working and lives at home with her male partner and her pets. She is a nonsmoker and drinks alcohol socially. Her best-corrected visual acuity is 20/200 in the right eye and 20/20 in the left eye, with a right relative afferent pupil defect. The results of a slitlamp examination of the intraocular pressure, motility, and anterior segment in both eyes are unremarkable. Dilated examination findings of the left eye are normal. Results of a dilated examination of the right eye reveal a clear vitreous body, tortuous veins, and macula lutea, as shown in Figure 1.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Perform magnetic resonance imaging of the brain and orbits B. Use antibiotics C. Use steroids D. Order serologic tests
JAMA Ophthalmology November 2013 Volume 131, Number 11
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Grand Valley State University User on 06/10/2015
1477
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Neuroretinitis from Bartonella henselae
What To Do Next D. Order serologic tests
Discussion Figure 1 shows a preretinal tuft of tissue over the optic nerve with a small portion of the nerve visible behind the tissue. The visible portion of the optic nerve is edematous with overlying nerve fiber layer edema with hemorrhage. The macula showed diffuse edema as depicted by the optical coherence tomographic scans in Figure 2. No macular exudates typical of neuroretinitis were visible at this time. However, the combination of optic disc edema with macular edema was suggestive of a form of neuroretinitis. The preretinal tuft of tissue is unusual but appeared similar to an inflammatory collection sometimes seen in cases of exuberant uveitis. An infectious or inflammatory process was strongly suspected. To narrow the differential diagnoses, we ordered testing for Bartonella, Lyme disease, syphilis, Toxocara, and Toxoplasma. We also ordered a computed tomographic scan of the chest to look for evidence of sarcoidosis or tuberculosis, but the results were normal. During a follow-up examination 4 days later, a classic macular star was visible, and the preretinal tuft of tissue had resolved. The Bartonella and Toxocara serologic test results were still pending at that time. Given the clinical appearance and relevant negative test results, we started her on doxycycline and prednisone. Two and a half weeks after the initial presentation, her serologic results were reported and confirmed our clinical diagnosis of Bartonella henselae. Our patient continued to show improvement in her visual symptoms, and by week 7, her vision was 20/30.
There are numerous ocular manifestations ofBartonella henselae, including optic neuropathy, neuroretinitis, vitritis, focal retinitis, and choroiditis. The preretinal tuft seen in our case, in addition to the findings of optic neuropathy and macular edema, makes the presentation of the disease unusual. Based on the findings of the Optic Neuritis Treatment Trial,1 we know that the presence of hemorrhages, poor vision, disc swelling, and the absence of pain are highly atypical features for a demyelinating disease. Hence, in this setting, it would be less useful to perform magnetic resonance imaging of the brain and orbits in order to look for a demyelinating disease. However, neuroimaging may help identify enhancement or thickening along the retrobulbar aspect of the optic nerve, as can be seen in infiltrative processes such as tuberculosis or sarcoidosis. Imaging could also be useful to identify a compressive lesion, but the relatively abrupt onset and clinical fundoscopic appearance argue against this mechanism. The appearance of the characteristic macular star is very helpful in reaching the diagnosis because relatively few ocular conditions lead to this stellate pattern of lipid deposition. However, it may not always be present at the time of initial presentation owing to extensive edema, and it does not develop in all cases. In the case series by Chi and colleagues,2 a macular star developed in 45% of 63 eyes that tested positive for cat-scratch disease associated with optic neuropathy. Other series3,4 reporting neuroretinitis to be the most common posterior segment complication of ocular cat-scratch disease have reported its occurrence in up to 64% to 71% of patients. The clinical constellation of retinal edema, disc edema, and painless loss of vision are suggestive of a diagnosis of neuroretinitis. The additional preretinal tuft seen in our case at initial presentation and its subsequent dissipation even before institution of antimicrobial therapy are likely due to the exuberant inflammatory response at the time of the initial examination, and, to the best of our knowledge, it has not been previously reported in connection with this entity.
A
B
Figure 2. Macular optical coherence tomographic (OCT) scan (A) with infrared fundus image (B) of the right eye showing macular edema.
ARTICLE INFORMATION
REFERENCES
Author Affiliation: Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.
1. Beck RW, Cleary PA, Anderson MM Jr, et al; The Optic Neuritis Study Group. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. N Engl J Med. 1992;326(9):581-588.
Corresponding Author: Derek S. Welsbie, MD, PhD, Wilmer Eye Institute, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287 ([email protected]). Conflict of Interest Disclosures: None reported.
1478
2. Chi SL, Stinnett S, Eggenberger E, et al. Clinical characteristics in 53 patients with cat scratch optic neuropathy. Ophthalmology. 2012;119(1):183-187.
3. Reed JB, Scales DK, Wong MT, Lattuada CP Jr, Dolan MJ, Schwab IR. Bartonella henselae neuroretinitis in cat scratch disease. Ophthalmology. 1998;105(3):459-466. 4. Ormerod LD, Dailey JP. Ocular manifestations of cat-scratch disease. Curr Opin Ophthalmol. 1999;10(3):209-216.
JAMA Ophthalmology November 2013 Volume 131, Number 11
Copyright 2013 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a Grand Valley State University User on 06/10/2015
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