Dig Dis Sci (2006) 51:1847–1849DOI 10.1007/s10620-006-9188-z Central Retinal Vein Thrombosis in a Patient with Ulcerative Colitis Alan L. Buchman · Anna Marie Babbo · Richard G. Gieser Received: 31 August 2005 / Accepted: 18 December 2005 / Published online: 9 September 2006 C Springer Science+Business Media, Inc. 2006 Keywords Ulcerative colitis . Thrombosis . Retina . Eye Introduction Both Crohn’s disease and ulcerative colitis are associated with ocular disease, including anterior episcleritis and uveitis. However, central vein occlusion has been a rarely reported complication of Crohn’s disease (four cases) [1–4] or ulcerative colitis (four cases) [5–8]. We report the fifth case of central vein occlusion in a patient with ulcerative colitis. In addition, six cases of retinal vasculitis have also been described in association with inflammatory bowel disease (IBD), although there was no evidence of venous occlusion in these patients [3, 9–13]. Case report The patient is a 37-year-old female immigrant from Ethiopia with a history of left-sided ulcerative colitis for diagnosed at an outside hospital 6 months previous to coming to A. L. Buchman () · A. M. Babbo Division of Gastroenterology, Feinberg School of Medicine, Northwestern University, 676 North St. Clair Street, Suite 1400, Chicago, Illinois 60611, USA e-mail:
[email protected] R. G. Gieser Wheaton Eye Clinic, Wheaton, Illinois, USA Northwestern. She had symptoms of rectal bleeding and abdominal pain for 7 years. At the time of her original diagnosis she was treated with intravenous corticosteroids followed by oral corticosteroids and mesalamine enemas, although never achieved complete clinical remission. Infliximab (5 mg/kg) was used as a single dose with no clinical effect. At the time of presentation to Northwestern, she had lost 36 lb and experienced three or four bowel movements daily, all with a small amount of bright red blood. Energy level was self-described as poor. She was taking prendisone, 20 mg/day, but had discontinued the enemas. Her medical history was notable for a history of endometriosis, for which she had required three laparascopies and a hemorrhoidectomy. There was no history of clotting disorder or thrombosis. The patient gave a history of bilateral eye erythema at the time of her original diagnosis of ulcerative colitis. No definitive diagnosis was made and it resolved. She did not smoke and used no nonsteroidal anti-inflammatory agents. There was no family history of IBD or thromboses. On physical examination, the patient’s height was 62 in. and her weight was 91 lb. HEENT examination revealed moist mucous membranes and no apthous ulcers. Lungs were clear. A grade 3/4 holosystolic heart murmur was auscultated. The abdomen was soft and nondistended; mild tenderness was present in the left lower quadrant. Bowel sounds were evident. Extremity examination was unremarkable except for some mild skeletal muscle loss. Colonoscopy showed moderately severe ulcerations involving the rectum and sigmoid colon. Corticosteroids were continued and oral mesalamine (4.8 g/day) and mesalamine enemas (b.i.d.) were prescribed. Because of continued symptoms, the patient increased her prednisone to 40 mg/day, without noticeable effect. She was therefore admitted to Northwestern Memorial Hospital for intravenous corticosteroid therapy. Anti-CD3 antibody (visulizimab) was administered per protocol after Springer 1848 Dig Dis Sci (2006) 51:1847–1849 Figs. 1–3 Fig. 1a. Optic nerve pallor with narrowed arterioles and veins and subretinal hemorrhage (right eye). Fig. 1b. Normal optic nerve and vessels (left eye). Fig. 2. Subretinal peripapillary hemorrhage (thick arrows) and engorged retinal veins (thin arrows). Fig. 3. she failed to achieve clinical remission with intravenous corticosteroids in 7 days. A few days later the patient developed mild blurred vision in her left eye but did not complain about it. Three weeks later she was seen by an opthalmologist and retinal examination showed evidence of central retinal vein occlusion (Fig. 1a). Rectal bleeding discontinued and she gained 10 lb, although abdominal and rectal pain continued at 4 weeks. At this time, vision was 20/25 in the right eye and 20/20 in the left eye. Narrowed arterioles and peripapillary subretinal hemorrhage were observed in the right eye, although the veins were no longer engorged (Fig. 2). Two weeks later, vision was 20/20 bilaterally, although mild engorgement of veins was observed in the right eye along with intraretinal hemorrhages in the nerve fiber layer superior and inferior to the optic nerve; the left eye was normal (Fig. 1b). Serum protein S, protein C, antithrombin III, anti-cardiolipin IgM and IgG, homocystein, and fibrinogen concentrations were all normal, as was the platelet count. Factor VIII was minimally decreased at 48% activity (normal: 50%–150%). Factor V Leiden mutation, prothrombin gene G20210A mutation, and methylenetetrahydrofolate reductase (MTHFR) gene mutation were not present. Flexible sigmoidoscopy showed only mild erythema and shallow ulcerations in the rectum. Mesalamine enemas were reinstituted. Abdominal discomfort continued and the patient was referred back to her gynecologist for management of endometriosis. Springer Discussion Coagulation abnormalities in patients with Crohn’s disease and ulcerative colitis related to increased concentrations of factor V, factor VIII, and fibrinogen or decreased concentrations of antithrombin III have been described [14]. IBD is also associated with deep venous thrombosis and pulmonary emboli, even in the face of normal coagulation parameters [15]. However, the retinal vein is an unusual site for thrombosis. Retinal vein occlusion typically manifests in blurred vision and, therefore, should be considered in the differential diagnosis of a patient with IBD who develops new-onset unilateral blurred vision in the absence of scleral injection. However, because there are limited treatment descriptions, no definitive therapy can be recommended at this time. In our case, these symptoms resolved spontaneously, although clinical improvement corresponded to improvement in the clinical symptoms of her ulcerative colitis. We also cannot exclude the possibility of a contribution from the antiCD3 therapy, although the mechanism for such an effect would be unclear. One previous patient was treated successfully with high-dose oral corticosteroids [8], and another was treated with panretinal photocoagulation [2]. Treatment was not described in the other six reported cases. It cannot be determined from these case reports whether the resolution of blurred vision simply mirrored the clinical response to Dig Dis Sci (2006) 51:1847–1849 treatment for the underlying IBD. Affected individuals should undergo a complete thrombotic evaluation. Although anticoagulation has not been described, it may be considered, especially if coagulation abnormalties are identified. Given the risk of recurrent ulceration and hemorrhage in ulcerative colitis or Crohn’s disease, anticoagulation is probably relatively contraindicated but may be indicated in patients with a coagulation abnorma. References 1. Igarashi H, Yanagawa C, Igarashi S (1996) Crohn’s disease and central retinal vein occlusion. Indian J Ophthal 44:97–99 2. Larsson J, Hansson-Lundblad C (2000) Central retinal vein occlusion in two patients with inflammatory bowel disease. Retina 20:681–682 3. Ruby AJ, Jampol LM (1990) Crohn’s disease and retinal vascular disease. Am J Opthalmol 110:349–353 4. Puli SR, Benage DD (2003) Retinal vein thrombosis after infliximab (Remicaid) treatment for Crohn’s disease [letter]. Am J Gastroenterol 98:939–940 5. Von Eicken J, Inhoffen W, Schneider U (2003) Zentralvenenthrombose bei einem jungen Patienten. Opthalmologe 100:740–742 1849 6. 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