Dig Dis Sci (2006) 51:1847–1849DOI 10.1007/s10620-006-9188-z

Central Retinal Vein Thrombosis in a Patientwith Ulcerative ColitisAlan L. Buchman · Anna Marie Babbo ·Richard G. Gieser

Received: 31 August 2005 / Accepted: 18 December 2005 / Published online: 9 September 2006C© Springer Science+Business Media, Inc. 2006

Keywords Ulcerative colitis . Thrombosis . Retina . Eye

Introduction

Both Crohn’s disease and ulcerative colitis are associ-ated with ocular disease, including anterior episcleritis anduveitis. However, central vein occlusion has been a rarely re-ported complication of Crohn’s disease (four cases) [1–4] orulcerative colitis (four cases) [5–8]. We report the fifth caseof central vein occlusion in a patient with ulcerative coli-tis. In addition, six cases of retinal vasculitis have also beendescribed in association with inflammatory bowel disease(IBD), although there was no evidence of venous occlusionin these patients [3, 9–13].

Case report

The patient is a 37-year-old female immigrant from Ethiopiawith a history of left-sided ulcerative colitis for diagnosedat an outside hospital 6 months previous to coming to

A. L. Buchman (�) · A. M. BabboDivision of Gastroenterology,Feinberg School of Medicine, Northwestern University,676 North St. Clair Street, Suite 1400, Chicago,Illinois 60611, USAe-mail: a-buchman@northwestern.edu

R. G. GieserWheaton Eye Clinic,Wheaton, Illinois, USA

Northwestern. She had symptoms of rectal bleeding and ab-dominal pain for 7 years. At the time of her original diagnosisshe was treated with intravenous corticosteroids followed byoral corticosteroids and mesalamine enemas, although neverachieved complete clinical remission. Infliximab (5 mg/kg)was used as a single dose with no clinical effect. At the timeof presentation to Northwestern, she had lost 36 lb and expe-rienced three or four bowel movements daily, all with a smallamount of bright red blood. Energy level was self-describedas poor. She was taking prendisone, 20 mg/day, but had dis-continued the enemas. Her medical history was notable fora history of endometriosis, for which she had required threelaparascopies and a hemorrhoidectomy. There was no his-tory of clotting disorder or thrombosis. The patient gave ahistory of bilateral eye erythema at the time of her origi-nal diagnosis of ulcerative colitis. No definitive diagnosiswas made and it resolved. She did not smoke and used nononsteroidal anti-inflammatory agents. There was no familyhistory of IBD or thromboses.

On physical examination, the patient’s height was 62 in.and her weight was 91 lb. HEENT examination revealedmoist mucous membranes and no apthous ulcers. Lungswere clear. A grade 3/4 holosystolic heart murmur was aus-cultated. The abdomen was soft and nondistended; mildtenderness was present in the left lower quadrant. Bowelsounds were evident. Extremity examination was unremark-able except for some mild skeletal muscle loss. Colonoscopyshowed moderately severe ulcerations involving the rectumand sigmoid colon. Corticosteroids were continued and oralmesalamine (4.8 g/day) and mesalamine enemas (b.i.d.) wereprescribed. Because of continued symptoms, the patient in-creased her prednisone to 40 mg/day, without noticeable ef-fect. She was therefore admitted to Northwestern MemorialHospital for intravenous corticosteroid therapy. Anti-CD3antibody (visulizimab) was administered per protocol after

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Figs. 1–3 Fig. 1a. Optic nervepallor with narrowed arteriolesand veins and subretinalhemorrhage (right eye). Fig. 1b.Normal optic nerve and vessels(left eye). Fig. 2. Subretinalperipapillary hemorrhage (thickarrows) and engorged retinalveins (thin arrows). Fig. 3.

she failed to achieve clinical remission with intravenous cor-ticosteroids in 7 days. A few days later the patient developedmild blurred vision in her left eye but did not complain aboutit. Three weeks later she was seen by an opthalmologist andretinal examination showed evidence of central retinal veinocclusion (Fig. 1a). Rectal bleeding discontinued and shegained 10 lb, although abdominal and rectal pain continuedat 4 weeks. At this time, vision was 20/25 in the right eyeand 20/20 in the left eye. Narrowed arterioles and peripap-illary subretinal hemorrhage were observed in the right eye,although the veins were no longer engorged (Fig. 2). Twoweeks later, vision was 20/20 bilaterally, although mild en-gorgement of veins was observed in the right eye along withintraretinal hemorrhages in the nerve fiber layer superior andinferior to the optic nerve; the left eye was normal (Fig. 1b).Serum protein S, protein C, antithrombin III, anti-cardiolipinIgM and IgG, homocystein, and fibrinogen concentrationswere all normal, as was the platelet count. Factor VIII wasminimally decreased at 48% activity (normal: 50%–150%).Factor V Leiden mutation, prothrombin gene G20210A mu-tation, and methylenetetrahydrofolate reductase (MTHFR)gene mutation were not present.

Flexible sigmoidoscopy showed only mild erythema andshallow ulcerations in the rectum. Mesalamine enemas werereinstituted. Abdominal discomfort continued and the patientwas referred back to her gynecologist for management ofendometriosis.

Discussion

Coagulation abnormalities in patients with Crohn’s diseaseand ulcerative colitis related to increased concentrations offactor V, factor VIII, and fibrinogen or decreased concen-trations of antithrombin III have been described [14]. IBDis also associated with deep venous thrombosis and pul-monary emboli, even in the face of normal coagulation pa-rameters [15]. However, the retinal vein is an unusual site forthrombosis.

Retinal vein occlusion typically manifests in blurred vi-sion and, therefore, should be considered in the differentialdiagnosis of a patient with IBD who develops new-onsetunilateral blurred vision in the absence of scleral injection.However, because there are limited treatment descriptions,no definitive therapy can be recommended at this time. Inour case, these symptoms resolved spontaneously, althoughclinical improvement corresponded to improvement in theclinical symptoms of her ulcerative colitis. We also can-not exclude the possibility of a contribution from the anti-CD3 therapy, although the mechanism for such an effectwould be unclear. One previous patient was treated success-fully with high-dose oral corticosteroids [8], and another wastreated with panretinal photocoagulation [2]. Treatment wasnot described in the other six reported cases. It cannot bedetermined from these case reports whether the resolutionof blurred vision simply mirrored the clinical response to

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treatment for the underlying IBD. Affected individualsshould undergo a complete thrombotic evaluation. Althoughanticoagulation has not been described, it may be considered,especially if coagulation abnormalties are identified. Giventhe risk of recurrent ulceration and hemorrhage in ulcerativecolitis or Crohn’s disease, anticoagulation is probably rela-tively contraindicated but may be indicated in patients witha coagulation abnorma.

References

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