OPHTHALMIC ARTERY OCCLUSION IN RELAPSING POLYCHONDRITIS Eric D. Weichel, MD, Andrew S. Eiseman, MD
Purpose: To describe ophthalmic artery occlusion in a patient with relapsing polychondritis. Methods: Observational case report Results: An acute episode of relapsing polychondritis caused ophthalmic artery occlusion with resultant visual acuity of no light perception. Conclusion: Ophthalmic artery occlusion can occur in patients with relapsing polychondritis. Patients with relapsing polychondritis need close follow-up to exclude severe vascular disease. RETINAL CASES & BRIEF REPORTS 3:18 –20, 2009
From the Ophthalmology Service and the Department of Surgery, Walter Reed Army Medical Center, Washington, DC.
prednisone (50 mg every 8 hours). Over a 24-hour period of oral prednisone administration, she developed severe left-sided periorbital pain, edema, proptosis, and bullae of the superior and inferior lids of the left eye. The patient denied any changes in baseline visual acuity. Initial computed tomography revealed proptosis and left periorbital inflammation with extension into the postseptal lateral and superior extraconal fat. There was no evidence of sinusitis. Treatment with intravenous methylprednisolone (125 mg every 6 hours) was started again; treatment continued with oral azathioprine (125 mg alternating daily with 150 mg) and then intramuscular methotrexate (25 mg once per week), intravenous vancomycin (1 g every 12 hours), intravenous clindamycin (900 mg every 8 hours), intravenous aztreonam (2 g every 8 hours), and oral ciprofloxacin (500 mg twice per day). Her condition was stable for 48 hours without changes in medical management when she developed no light perception visual acuity in the left eye. Ocular examination revealed a 4⫹ afferent pupillary defect. Magnetic resonance imaging of the brain and orbits with gadolinium revealed lateral preseptal and lateral extraconal postseptal enhancement in the left eye. The coronal sections also disclosed enhancement of the optic nerve extending from the lamina cribosa to the optic chiasm as well as dural enhancement. Laboratory testing revealed the following significant findings: erythrocyte sedimentation rate, 100 mm/h (normal range, 0 –20 mm/h); and C-reactive protein level, 15.97 mg/dL (normal range, 0.03–5.0 mg/dL). Dilated fundus examination of the left eye revealed a white retina, absent cherry-red spot, venous tortuosity and dilation with intraretinal hemorrhages in all quadrants, disk edema, and diffuse peripapillary hemorrhages (Fig. 1). Fluorescein angiography showed delayed choroidal filling with a delayed arm to central retinal artery time of 70 seconds and a generalized delay of arteriovenous transit time of 2:41 minutes with trace blood flow in the superonasal quadrant. The late-phase frames showed no flow or leakage in the macular region or optic disk (Fig. 2). Visual acuity remained no light perception at follow-up examinations for 1 year without any evidence of rubeosis iridis. Repeated magnetic resonance imaging of the orbit with gadolinium every 3
R
elapsing polychondritis is a life-threatening rare systemic connective tissue disease caused by antibodies to type II collagen. The diagnosis is usually made clinically using three or more of the following criteria established by McAdam et al1: recurrent chondritis of both auricles; nonerosive inflammatory polyarthritis; chondritis of the nasal cartilage; inflammation of ocular structures; chondritis of the respiratory tract involving laryngeal or tracheal cartilage; and cochlear or vestibular damage manifested by neurosensory hearing loss, tinnitus, or vertigo. We report a case of unilateral ophthalmic artery occlusion associated with optic neuritis during an episode of relapsing polychondritis. Case Report A 57-year-old woman with a 17-year history of relapsing polychondritis without any prior ocular involvement was treated with oral azathioprine (125 mg alternating daily with 150 mg). She developed left-sided auricular pain, and treatment with oral prednisone (60 mg every day) was started. She was admitted to the internal medicine service 19 days later with worsening left auricular pain. The internal medicine service discontinued treatment with oral prednisone and started treatment with intravenous methylprednisolone (125 mg every 6 hours). Over the next 72 hours, the patient’s condition improved, and treatment was switched to oral Reprint requests: Eric D. Weichel, MD, Ophthalmology Service and Department of Surgery, Walter Reed Army Medical Center, 6900 Georgia Avenue, Washington, DC 20307-5001; e-mail: [email protected]
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OPHTHALMIC ARTERY OCCLUSION IN RELAPSING POLYCHONDRITIS
months for 1 year demonstrated persistent and stable left optic nerve enhancement from the lamina cribosa to the optic chiasm. The left optic nerve head became atrophic with markedly narrowed and obliterated retinal vessels (Fig. 3). Follow-up examinations of the right eye revealed stable visual field loss in the inferonasal quadrant extending to within 10° of fixation with normal intraocular pressures.
Discussion
Fig. 1. Left eye at presentation showing absent cherry-red spot, dilated retinal veins, intraretinal hemorrhages, and optic disk edema.
Fig. 2. Top right, Red-free photograph. Top left, First sign of arterial filling at 70 seconds. Bottom right, Completion of the arteriovenous transit phase at 4:31 minutes. Bottom left, Late-phase frame at 16:03 minutes demonstrating only trace blood flow in the superonasal quadrant without perfusion of the optic disk.
Isaak et al,2 in a series of 112 patients with relapsing polychondritis, found that 21% of patients presented with ophthalmic disease and 57% developed ocular manifestations at follow-up. They described episcleritis (39%) as the most common finding. Other common manifestations included scleritis (14%), keratitis sicca (10%), lid edema (9%), iritis (9%), retinopathy (9%), and optic nerve involvement (14%).Within this study, Isaak et al described one case of central retinal vein occlusion and one case of branch retinal vein occlusion. Hemry et al3 was the first to describe central retinal artery occlusion in a
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autoimmune response to collagen. This resulted in ophthalmic artery occlusion with closure of the central retinal artery and vein as well as the choroidal circulation. This hypothesis is supported by Richards5 who found an association between a combined central retinal artery and vein occlusion and inflammation of the retrobulbar portion of the optic nerve. Our case demonstrates the need for close evaluation of patients with relapsing polychondritis and optic nerve inflammation to rule out the possibility of severe retinal vascular disease as well. Key words: ophthalmic artery occlusion, relapsing polychondritis, vasculitis. References Fig. 3. Pale optic nerve head at the 1-year follow-up secondary to optic disk infarction.
patient with relapsing polychondritis. In a review of ocular manifestations of relapsing polychondritis, Magargal et al4 cited five articles describing optic neuritis and ischemic optic neuropathy. To our knowledge, the current case demonstrates the first case of ophthalmic artery occlusion in a patient with relapsing polychondritis. We believe that this represents severe vasculitis presumably due to an
1.
2.
3.
4. 5.
McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55:193– 215. Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986;93: 681–689. Hemry DA, Moss AJ, Jacox RF. Relapsing polychondritis, a “floppy” mitral valve, and migratory polytendonitis. Ann Intern Med 1972;77:576–580. Magargal LE, Donoso LA, Goldberg RE, et al. Ocular manifestations of relapsing polychondritis. Retina 1981;1:96–99. Richards RD. Simultaneous occlusion of the central retinal artery and vein. Trans Am Ophthalmol Soc 1979;77:191–209.
Purpose: To describe ophthalmic artery occlusion in a patient with relapsing polychondritis. Methods: Observational case report Results: An acute episode of relapsing polychondritis caused ophthalmic artery occlusion with resultant visual acuity of no light perception. Conclusion: Ophthalmic artery occlusion can occur in patients with relapsing polychondritis. Patients with relapsing polychondritis need close follow-up to exclude severe vascular disease. RETINAL CASES & BRIEF REPORTS 3:18 –20, 2009
From the Ophthalmology Service and the Department of Surgery, Walter Reed Army Medical Center, Washington, DC.
prednisone (50 mg every 8 hours). Over a 24-hour period of oral prednisone administration, she developed severe left-sided periorbital pain, edema, proptosis, and bullae of the superior and inferior lids of the left eye. The patient denied any changes in baseline visual acuity. Initial computed tomography revealed proptosis and left periorbital inflammation with extension into the postseptal lateral and superior extraconal fat. There was no evidence of sinusitis. Treatment with intravenous methylprednisolone (125 mg every 6 hours) was started again; treatment continued with oral azathioprine (125 mg alternating daily with 150 mg) and then intramuscular methotrexate (25 mg once per week), intravenous vancomycin (1 g every 12 hours), intravenous clindamycin (900 mg every 8 hours), intravenous aztreonam (2 g every 8 hours), and oral ciprofloxacin (500 mg twice per day). Her condition was stable for 48 hours without changes in medical management when she developed no light perception visual acuity in the left eye. Ocular examination revealed a 4⫹ afferent pupillary defect. Magnetic resonance imaging of the brain and orbits with gadolinium revealed lateral preseptal and lateral extraconal postseptal enhancement in the left eye. The coronal sections also disclosed enhancement of the optic nerve extending from the lamina cribosa to the optic chiasm as well as dural enhancement. Laboratory testing revealed the following significant findings: erythrocyte sedimentation rate, 100 mm/h (normal range, 0 –20 mm/h); and C-reactive protein level, 15.97 mg/dL (normal range, 0.03–5.0 mg/dL). Dilated fundus examination of the left eye revealed a white retina, absent cherry-red spot, venous tortuosity and dilation with intraretinal hemorrhages in all quadrants, disk edema, and diffuse peripapillary hemorrhages (Fig. 1). Fluorescein angiography showed delayed choroidal filling with a delayed arm to central retinal artery time of 70 seconds and a generalized delay of arteriovenous transit time of 2:41 minutes with trace blood flow in the superonasal quadrant. The late-phase frames showed no flow or leakage in the macular region or optic disk (Fig. 2). Visual acuity remained no light perception at follow-up examinations for 1 year without any evidence of rubeosis iridis. Repeated magnetic resonance imaging of the orbit with gadolinium every 3
R
elapsing polychondritis is a life-threatening rare systemic connective tissue disease caused by antibodies to type II collagen. The diagnosis is usually made clinically using three or more of the following criteria established by McAdam et al1: recurrent chondritis of both auricles; nonerosive inflammatory polyarthritis; chondritis of the nasal cartilage; inflammation of ocular structures; chondritis of the respiratory tract involving laryngeal or tracheal cartilage; and cochlear or vestibular damage manifested by neurosensory hearing loss, tinnitus, or vertigo. We report a case of unilateral ophthalmic artery occlusion associated with optic neuritis during an episode of relapsing polychondritis. Case Report A 57-year-old woman with a 17-year history of relapsing polychondritis without any prior ocular involvement was treated with oral azathioprine (125 mg alternating daily with 150 mg). She developed left-sided auricular pain, and treatment with oral prednisone (60 mg every day) was started. She was admitted to the internal medicine service 19 days later with worsening left auricular pain. The internal medicine service discontinued treatment with oral prednisone and started treatment with intravenous methylprednisolone (125 mg every 6 hours). Over the next 72 hours, the patient’s condition improved, and treatment was switched to oral Reprint requests: Eric D. Weichel, MD, Ophthalmology Service and Department of Surgery, Walter Reed Army Medical Center, 6900 Georgia Avenue, Washington, DC 20307-5001; e-mail: [email protected]
18
19
OPHTHALMIC ARTERY OCCLUSION IN RELAPSING POLYCHONDRITIS
months for 1 year demonstrated persistent and stable left optic nerve enhancement from the lamina cribosa to the optic chiasm. The left optic nerve head became atrophic with markedly narrowed and obliterated retinal vessels (Fig. 3). Follow-up examinations of the right eye revealed stable visual field loss in the inferonasal quadrant extending to within 10° of fixation with normal intraocular pressures.
Discussion
Fig. 1. Left eye at presentation showing absent cherry-red spot, dilated retinal veins, intraretinal hemorrhages, and optic disk edema.
Fig. 2. Top right, Red-free photograph. Top left, First sign of arterial filling at 70 seconds. Bottom right, Completion of the arteriovenous transit phase at 4:31 minutes. Bottom left, Late-phase frame at 16:03 minutes demonstrating only trace blood flow in the superonasal quadrant without perfusion of the optic disk.
Isaak et al,2 in a series of 112 patients with relapsing polychondritis, found that 21% of patients presented with ophthalmic disease and 57% developed ocular manifestations at follow-up. They described episcleritis (39%) as the most common finding. Other common manifestations included scleritis (14%), keratitis sicca (10%), lid edema (9%), iritis (9%), retinopathy (9%), and optic nerve involvement (14%).Within this study, Isaak et al described one case of central retinal vein occlusion and one case of branch retinal vein occlusion. Hemry et al3 was the first to describe central retinal artery occlusion in a
20
RETINAL CASES & BRIEF REPORTSℜ
●
2009
●
VOLUME 3
●
NUMBER 1
autoimmune response to collagen. This resulted in ophthalmic artery occlusion with closure of the central retinal artery and vein as well as the choroidal circulation. This hypothesis is supported by Richards5 who found an association between a combined central retinal artery and vein occlusion and inflammation of the retrobulbar portion of the optic nerve. Our case demonstrates the need for close evaluation of patients with relapsing polychondritis and optic nerve inflammation to rule out the possibility of severe retinal vascular disease as well. Key words: ophthalmic artery occlusion, relapsing polychondritis, vasculitis. References Fig. 3. Pale optic nerve head at the 1-year follow-up secondary to optic disk infarction.
patient with relapsing polychondritis. In a review of ocular manifestations of relapsing polychondritis, Magargal et al4 cited five articles describing optic neuritis and ischemic optic neuropathy. To our knowledge, the current case demonstrates the first case of ophthalmic artery occlusion in a patient with relapsing polychondritis. We believe that this represents severe vasculitis presumably due to an
1.
2.
3.
4. 5.
McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55:193– 215. Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing polychondritis. Ophthalmology 1986;93: 681–689. Hemry DA, Moss AJ, Jacox RF. Relapsing polychondritis, a “floppy” mitral valve, and migratory polytendonitis. Ann Intern Med 1972;77:576–580. Magargal LE, Donoso LA, Goldberg RE, et al. Ocular manifestations of relapsing polychondritis. Retina 1981;1:96–99. Richards RD. Simultaneous occlusion of the central retinal artery and vein. Trans Am Ophthalmol Soc 1979;77:191–209.
