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Central retinal artery occlusion in Crohn disease
Central retinal artery occlusion in Crohn disease Khalil Ghasemi Falavarjani, MD,a Mohammad Mehdi Parvaresh, MD,a Kourosh Shahraki, MD,a Shahbaz Nekoozadeh,a and Abdollah Amirfarhangi, MDb
Crohn disease is an inflammatory bowel disease frequently associated with extraintestinal manifestations. Ocular manifestations are uncommon but may cause significant morbidity, including blindness. We report the first case of a 9-year-old boy with biopsyproven Crohn disease who developed a cilioretinal artery–sparing central retinal artery occlusion. After 2 months of follow-up, the patient developed optic atrophy with no change in visual acuity.
negative; serum levels of anti-thrombin III, protein C, protein S, and activated protein C resistance were normal. No ocular therapy was offered, and the patient was advised to return for regular follow-up examination. At the most recent evaluation 2 months after presentation, visual acuity remained unchanged; however, the optic nerve was atrophic.
Case Report
Discussion
A
9-year-old boy was referred the retina clinic of the Rassoul Akram Hospital, Tehran, with a chief symptom of sudden, painless blurred vision in his left eye of 3 days’ duration. He had been diagnosed with Crohn disease at age 5, and several episodes of bowel disease reactivation had been controlled with the administration of systemic steroids. The disease had been quiescent and steroids discontinued several months before he presented at our clinic; however, a flare-up occurred 6 days before presentation and oral prednisone 1 mg/kg and sulfasalazine were started. There was no history of previous ocular disease. Ophthalmologic examination revealed best-corrected visual acuity of 20/20 in both eyes with a positive relative afferent pupillary defect in the left eye. The results of the anterior segment examination were normal, and the results of the Fundus examination of the right eye were normal; however, there was retinal whitening along the superior and inferior temporal arcades in the posterior pole with sparing of the fovea and much of the macula (Figure 1A). The findings were compatible with the diagnosis of central retinal artery occlusion (CRAO) with cilioretinal artery sparing. Fluorescein angiography showed delayed filling of the major temporal vessels and marked delay in the arteriovenous transit time in the left eye (Figure 1B). No abnormality was found on cardiovascular examination, including when echocardiography and Doppler carotid ultrasonography were used. Lupus anticoagulant antibody and antineutrophil cytoplasmic antibodies were Author affiliations: aEye Research Center, Rassoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran; bCardiology Department, Rassoul Akram Hospital, Tehran University of Medical Sciences Submitted December 24, 2011. Revision accepted March 20, 2012. Published online July 21, 2012. Correspondence: Khalil Ghasemi Falavarjani, MD, Eye Research Center, Rassoul Akram Hospital, Sattarkhan-Niayesh Street, Tehran 14455-364, Iran (email: drghasemi@yahoo. com). J AAPOS 2012;16:392-393. Copyright Ó 2012 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2012.03.004
392
The diagnosis of CRAO in a child suggests an underling systemic disease, usually from cardiovascular or inflammatory origin.1 The central retinal artery can be affected by many systemic inflammatory disorders, such as polyarteritis nodosa, Wegener granulomatosis, Churge Strauss syndrome, Behc¸et disease, sarcoidosis, and ulcerative colitis.1 The most common ocular manifestations of Crohn disease include uveitis, episcleritis, scleritis, and conjunctivitis; however, a few patients with central retinal vein occlusion, branch retinal artery occlusion, ischemic optic neuropathy, or retinal vasculitis have been reported.2-6 Saatci and colleagues2 reported a 38-year-old woman with Crohn disease and unilateral retinal vasculitis, branch retinal artery occlusion, and subsequent retinal neovascularization. Retinal neovascularization regressed after argon green laser panretinal photocoagulation in addition to oral steroid and salazopyrine. Ruby and Jampol4 reported 2 young women with Crohn disease, one with unilateral nonischemic central retinal vein occlusion and the other with unilateral retinal vasculitis with BRAO were treated with various doses of prednisolone and salazopyrine. Bonvin and colleagues3 reported a 44-year-old woman with ophthalmic artery occlusion who presented before enteric manifestations of Crohn disease. Heuer and colleagues6 reported a 24-year-old man with bilateral ischemic optic neuropathy, vitritis, and retinovascular sheathing associated with Crohn disease. To best of our knowledge, CRAO in association with Crohn disease was not previously reported. Our patient had CRAO without any sign of vascular involvement in other retinal vessels. Because all systemic examinations were within normal limits, we believe that the vascular occlusion may be attributed to the underlying Crohn disease; an alternative etiology is occlusive vasculitis caused by arterial wall granulomas.7,8 A close relationship between Crohn disease and optic nerve vasculitis has been previously reported.6 Inflammatory vascular occlusion may result from focal arteritis, disruption of the blood vessel walls, adherence of chronic inflammatory cells, and fibrin deposition.9 An embolic
Journal of AAPOS
Volume 16 Number 4 / August 2012
Falavarjani et al
393
FIG 1. Fundus photography (A) and fluorescein angiography (B) of a 9-year-old boy with cilioretinal artery–sparing central retinal artery occlusion associated with Crohn disease. Fluorescein angiography shows delayed filling of major vessels both superiorly and inferiorly at 40 seconds.
event from cardiovascular origin not detectable by routine echocardiography may also be considered a cause.1 The tendency for thrombosis in Crohn disease is well documented.4 References 1. Hayreh SS. Acute retinal arterial occlusive disorders. Prog Retin Eye Res 2011;30:359-94. 2. Saatci OA, Koc¸ak N, Durak I, Ergin MH. Unilateral retinal vasculitis, branch retinal artery occlusion and subsequent retinal neovascularization in Crohn’s disease. Int Ophthalmol 2001;24:89-92. 3. Bonvin ER, Dosso AA, Safran AB. Occlusion of the ophthalmic artery in Crohn disease. Klin Monbl Augenheilkd 1994;204:450-52.
Journal of AAPOS
4. Ruby AJ, Jampol LM. Crohn’s disease and retinal vascular disease. Am J Ophthalmol 1990;110:349-53. 5. Mintz R, Feller ER, Bahr RL, Shah SA. Ocular manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2004;10:135-9. 6. Heuer DK, Gager WE, Reeser FH. Ischemic optic neuropathy associated with Crohn’s disease. J Clin Neuro Ophthalmol 1982;2: 175-81. 7. Pounder RE. The pathogenesis of Crohn’s disease. J Gastroenterol 1994;29:11-5. 8. Sheehan-Dare RA, Goodfield MJ, Wilson PD, et al. Axillary artery occlusion as a presenting feature of Crohn’s disease. Postgrad Med J 1989;65:758-60. 9. Wakefield AJ, Sankey EA, Dhillon AP, et al. Granulomatous vasculitis in Crohn’s disease. Gastroenterology 1991;100:1279-87.
Crohn disease is an inflammatory bowel disease frequently associated with extraintestinal manifestations. Ocular manifestations are uncommon but may cause significant morbidity, including blindness. We report the first case of a 9-year-old boy with biopsyproven Crohn disease who developed a cilioretinal artery–sparing central retinal artery occlusion. After 2 months of follow-up, the patient developed optic atrophy with no change in visual acuity.
negative; serum levels of anti-thrombin III, protein C, protein S, and activated protein C resistance were normal. No ocular therapy was offered, and the patient was advised to return for regular follow-up examination. At the most recent evaluation 2 months after presentation, visual acuity remained unchanged; however, the optic nerve was atrophic.
Case Report
Discussion
A
9-year-old boy was referred the retina clinic of the Rassoul Akram Hospital, Tehran, with a chief symptom of sudden, painless blurred vision in his left eye of 3 days’ duration. He had been diagnosed with Crohn disease at age 5, and several episodes of bowel disease reactivation had been controlled with the administration of systemic steroids. The disease had been quiescent and steroids discontinued several months before he presented at our clinic; however, a flare-up occurred 6 days before presentation and oral prednisone 1 mg/kg and sulfasalazine were started. There was no history of previous ocular disease. Ophthalmologic examination revealed best-corrected visual acuity of 20/20 in both eyes with a positive relative afferent pupillary defect in the left eye. The results of the anterior segment examination were normal, and the results of the Fundus examination of the right eye were normal; however, there was retinal whitening along the superior and inferior temporal arcades in the posterior pole with sparing of the fovea and much of the macula (Figure 1A). The findings were compatible with the diagnosis of central retinal artery occlusion (CRAO) with cilioretinal artery sparing. Fluorescein angiography showed delayed filling of the major temporal vessels and marked delay in the arteriovenous transit time in the left eye (Figure 1B). No abnormality was found on cardiovascular examination, including when echocardiography and Doppler carotid ultrasonography were used. Lupus anticoagulant antibody and antineutrophil cytoplasmic antibodies were Author affiliations: aEye Research Center, Rassoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran; bCardiology Department, Rassoul Akram Hospital, Tehran University of Medical Sciences Submitted December 24, 2011. Revision accepted March 20, 2012. Published online July 21, 2012. Correspondence: Khalil Ghasemi Falavarjani, MD, Eye Research Center, Rassoul Akram Hospital, Sattarkhan-Niayesh Street, Tehran 14455-364, Iran (email: drghasemi@yahoo. com). J AAPOS 2012;16:392-393. Copyright Ó 2012 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/$36.00 http://dx.doi.org/10.1016/j.jaapos.2012.03.004
392
The diagnosis of CRAO in a child suggests an underling systemic disease, usually from cardiovascular or inflammatory origin.1 The central retinal artery can be affected by many systemic inflammatory disorders, such as polyarteritis nodosa, Wegener granulomatosis, Churge Strauss syndrome, Behc¸et disease, sarcoidosis, and ulcerative colitis.1 The most common ocular manifestations of Crohn disease include uveitis, episcleritis, scleritis, and conjunctivitis; however, a few patients with central retinal vein occlusion, branch retinal artery occlusion, ischemic optic neuropathy, or retinal vasculitis have been reported.2-6 Saatci and colleagues2 reported a 38-year-old woman with Crohn disease and unilateral retinal vasculitis, branch retinal artery occlusion, and subsequent retinal neovascularization. Retinal neovascularization regressed after argon green laser panretinal photocoagulation in addition to oral steroid and salazopyrine. Ruby and Jampol4 reported 2 young women with Crohn disease, one with unilateral nonischemic central retinal vein occlusion and the other with unilateral retinal vasculitis with BRAO were treated with various doses of prednisolone and salazopyrine. Bonvin and colleagues3 reported a 44-year-old woman with ophthalmic artery occlusion who presented before enteric manifestations of Crohn disease. Heuer and colleagues6 reported a 24-year-old man with bilateral ischemic optic neuropathy, vitritis, and retinovascular sheathing associated with Crohn disease. To best of our knowledge, CRAO in association with Crohn disease was not previously reported. Our patient had CRAO without any sign of vascular involvement in other retinal vessels. Because all systemic examinations were within normal limits, we believe that the vascular occlusion may be attributed to the underlying Crohn disease; an alternative etiology is occlusive vasculitis caused by arterial wall granulomas.7,8 A close relationship between Crohn disease and optic nerve vasculitis has been previously reported.6 Inflammatory vascular occlusion may result from focal arteritis, disruption of the blood vessel walls, adherence of chronic inflammatory cells, and fibrin deposition.9 An embolic
Journal of AAPOS
Volume 16 Number 4 / August 2012
Falavarjani et al
393
FIG 1. Fundus photography (A) and fluorescein angiography (B) of a 9-year-old boy with cilioretinal artery–sparing central retinal artery occlusion associated with Crohn disease. Fluorescein angiography shows delayed filling of major vessels both superiorly and inferiorly at 40 seconds.
event from cardiovascular origin not detectable by routine echocardiography may also be considered a cause.1 The tendency for thrombosis in Crohn disease is well documented.4 References 1. Hayreh SS. Acute retinal arterial occlusive disorders. Prog Retin Eye Res 2011;30:359-94. 2. Saatci OA, Koc¸ak N, Durak I, Ergin MH. Unilateral retinal vasculitis, branch retinal artery occlusion and subsequent retinal neovascularization in Crohn’s disease. Int Ophthalmol 2001;24:89-92. 3. Bonvin ER, Dosso AA, Safran AB. Occlusion of the ophthalmic artery in Crohn disease. Klin Monbl Augenheilkd 1994;204:450-52.
Journal of AAPOS
4. Ruby AJ, Jampol LM. Crohn’s disease and retinal vascular disease. Am J Ophthalmol 1990;110:349-53. 5. Mintz R, Feller ER, Bahr RL, Shah SA. Ocular manifestations of inflammatory bowel disease. Inflamm Bowel Dis 2004;10:135-9. 6. Heuer DK, Gager WE, Reeser FH. Ischemic optic neuropathy associated with Crohn’s disease. J Clin Neuro Ophthalmol 1982;2: 175-81. 7. Pounder RE. The pathogenesis of Crohn’s disease. J Gastroenterol 1994;29:11-5. 8. Sheehan-Dare RA, Goodfield MJ, Wilson PD, et al. Axillary artery occlusion as a presenting feature of Crohn’s disease. Postgrad Med J 1989;65:758-60. 9. Wakefield AJ, Sankey EA, Dhillon AP, et al. Granulomatous vasculitis in Crohn’s disease. Gastroenterology 1991;100:1279-87.