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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 310-312

Bilateral occlusive retinal vasculitis in ulcerative colitis


Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission26-Jun-2020
Date of Acceptance12-Dec-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Mohit Dogra
Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2099_20

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  Abstract 


Inflammatory bowel disease is a chronic disorder of the gastrointestinal tract that is frequently associated with extra-intestinal manifestations. Ocular manifestations generally include episcleritis, scleritis, and anterior uveitis. Vision-threatening complications like retinal vascular occlusions and retinal vasculitis have been anecdotally reported in these patients. We present a case of ulcerative colitis who presented with bilateral occlusive retinal vasculitis and retinal neovascularization, 10 years after being diagnosed with the disease. She was successfully managed with oral immunosuppressive agents and scatter laser photocoagulation. This case highlights the importance of ophthalmic examination in patients with ulcerative colitis, which is considered to be infrequently associated with ocular manifestations.

Keywords: Crohn's disease, extra-intestinal manifestation, inflammatory bowel disease, retinal vasculitis, ulcerative colitis


How to cite this article:
Chandra K, Singh SR, Singh R, Dogra MR, Dogra M. Bilateral occlusive retinal vasculitis in ulcerative colitis. Indian J Ophthalmol Case Rep 2021;1:310-2

How to cite this URL:
Chandra K, Singh SR, Singh R, Dogra MR, Dogra M. Bilateral occlusive retinal vasculitis in ulcerative colitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jun 14];1:310-2. Available from: https://www.ijoreports.in/text.asp?2021/1/2/310/312357



Ulcerative colitis (UC) and Crohn's disease (CD) are two major forms of inflammatory bowel disease (IBD). Though IBD primarily involves the gastrointestinal tract, extra-intestinal manifestations (EIMs) involving the joints, skin, hepatobiliary system, lungs, kidneys, and the eye are seen in 6%–40% patients.[1],[2] Ophthalmic manifestations are seen in up to 13% of patients.[2],[3] The majority of these are mild and include episcleritis, scleritis, and anterior uveitis.[1],[2],[3] Vision-threatening posterior uveitic entities like inflammatory retinal vascular occlusions and retinal vasculitis have been anecdotally reported in patients with CD.[4],[5] EIM including ocular involvement are less commonly seen in patients with UC as compared to patients with CD. We report a case of bilateral occlusive retinal vasculitis in a lady with biopsy-proven UC, with the disease in remission for many years.


  Case Report Top


A 45-year-old Asian Indian female presented with sudden painless and progressive diminution of vision in her right eye (OD) for a month. Her corrected visual acuity (CVA) was 20/60 in OD and 20/30 in the left eye (OS). Slit-lamp examination revealed 2+ cells and flare in the anterior chamber, pigment on the anterior lens surface, normal pupillary reactions, and intraocular pressures in both eyes (OU). Posterior segment examination showed vitreous cell, multiple dot-blot retinal hemorrhages, and extensive peripheral retinal vasculitis with perivascular sheathing in OU. The affliction of the retinal arterioles was more than that of the venules. In addition, OD had a subhyaloid hemorrhage inferotemporal to the fovea [Figure 1]a and [Figure 1]b along with a central macular thickness of 310 microns and presence of cystoid macular edema (CME) in OD with a normal scan of OS on optical coherence tomography (OCT) [Figure 1]c and [Figure 1]d. A clinical diagnosis of bilateral occlusive retinal vasculitis (RV) was made and fundus fluorescein angiography (FFA) was done. FFA showed 3600 peripheral capillary non-perfusion with abrupt pruning of retinal arterioles and venules and diffuse vascular leakage in the late phase in OU. OD also had a pettaloid-pattern of leakage in the fovea, whereas OS had a focus of retinal neovascularization [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f, [Figure 2]g, [Figure 2]h. OU underwent scatter laser photocoagulation of the ischemic peripheral retina on the same day.
Figure 1: (a and b) Fundus picture of the right and left eye showing multiple dot-blot haemorrhages in both eyes with a subhyaloid hemorrhage inferior to the fovea in the right eye. (c and d) Cross-sectional optical coherence tomography (OCT) scan highlighting cystoid macular edema (CME) in the right eye and a normal foveal contour in the left eye

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Figure 2: Fluorescein angiography (FA) at presentation showing extensive peripheral capillary non-perfusion areas in both eyes with abrupt cut-off of retinal arterioles and venules, macular neovascularization in the left eye and diffuse vascular leakage in the late phase (right eye a-d, left eye e-h)

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A differential diagnosis of tubercular (TB) RV, connective tissue disease-related RV and syphilitic RV was made. Mantoux, quantiferon TB (QFT) gold, TPHA, HIV serology and computed tomography (CT) of the chest, ANA and anti-ds DNA were within normal limits and the patient denied any history of fever, skin rash, arthralgia or weight loss. A detailed history revealed that the patient was a diagnosed case of UC, which was biopsy-proven 10 years ago. She had received oral steroids and mesalazine for two years at that time. She had been off treatment for the past eight years. She had no bowel-related symptoms and her systemic evaluation was unremarkable. Fecal calprotectin levels (15 micrograms/mg), C-reactive protein, and erythrocyte sedimentation rate were not elevated and a diagnostic colonoscopy did not reveal any active areas of ulceration or hemorrhage. She was started on 60 mg oral prednisolone and 100 mg oral azathioprine along with topical betamethasone and atropine drops for the control of ocular inflammation, under guidance of the treating gastoentrologist. Over the following six weeks, her CVA improved to 20/30 in the OD , whereas OS improved to 20/20 with quiescence of anterior chamber inflammation in OU. The subhyaloid hemorrhage in OD had reduced and OU had absence of vitreous cells along with 3600 peripheral pigmented laser scars [Figure 3]a and [Figure 3]b and normal foveal contour with resolution of CME on OCT [Fig. 3c and d]. Oral and topical steroids were tapered and azathioprine was increased to 150 mg. At nine months follow-up, she had a CVA of 20/20, absence of ocular inflammation, no clinical or FFA evidence of RV, and had no exacerbation of UC during this period.
Figure 3: Ultrawide-field color photographs of the right eye (a) and left eye (b) at 6 weeks follow-up, showing 3600 peripheral laser scars in both eyes with reduction in the temporal subhyaloid hemorrhage. Cross-sectional OCT scans showing normal foveal contour with resolution of CME and posterior vitreous detachment in the right eye (c) and normal foveal contour in the left eye (d)

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  Discussion Top


IBD is a chronic immune-mediated disorder of the gastrointestinal tract that is characterized by recurrent acute attacks. CD and UC are the most common types of IBD differing in their pathophysiology and clinical features. EIM may be seen in one-third of these patients, with ocular involvement seen in 0.3%–13% cases.[1],[2] Patients with CD have a greater chance (3.5%–6.8%) of developing ocular manifestations compared to those with UC (1.6%–5.4%); however, Indian patients with UC and CD have shown equal propensity to cause ocular involvement.[1],[2],[3],[6]

Common ocular manifestations in Indian patients with IBD include episcleritis/scleritis (3%-8%) and non-granulomatous anterior uveitis (2%–6%).[3],[6] Retinal vascular involvement in patients with CD has been reported previously. Retinal vascular involvement may be in the form of vascular occlusions (venous or arterial) or RV.[3],[4],[5] Duker et al. reported a 26 year female with biopsy-proven CD who went on to develop bilateral obliterative retinal arteritis and phlebitis that was managed with high dose systemic steroids and cyclophosphamide.[4] Saatci et al. reported a 38 year female with CD who developed bilateral occlusive retinal arteritis with left optic disc pallor and was treated with oral steroids and laser photocoagulation.[5] RV accounts for 8.68% of total uveitis and 34.51% of posterior uveitis cases in North India.[7] Kawali et al. reported a single case in large series of RV patients who developed UC 16 months after presenting as RV. No details of the nature of RV (occlusive or exudative) and outcome of this patient were however provided.[8] There is just one other case report of RV developing in a patient of UC, and even in that there is no mention about the nature, management and outome.[9] Our patient was a biopsy-proven case of UC who developed bilateral occlusive RV 10 years after the initial diagnosis. UC was under remission for eight years at the time of ocular involvement. She responded well to systemic immunosuppressive therapy (IST) combined with scatter laser photocoagulation.

This case has various unique aspects. Firstly, all previous cases of RV have either been reported in patients with CD or have insufficient data in patients of UC.[3],[4],[5],[8],[9] Such a symmetrical bilateral occlusive RV with retinal neovascularization has not been reported in a proven case of UC till date. Secondly, ocular manifestations (except anterior uveitis) are a harbinger of increased disease activity in patients with UC.[1],[2],[3] In our case, UC was in long term remission and the patient was asymptomatic at the time of detection of RV. A normal colonoscopy and fecal calprotectin levels confirmed the absence of disease activity. Rarely, EIM may herald the onset of exacerbation of gastrointestinal symptoms in IBD.[1],[2] It is possible that in our patient, worsening of UC was prevented by the timely initiation of IST for managing RV. However, we acknowledge that the association of occlusive RV with UC may be coincidental and that there is no conclusive way to prove the same. Thirdly, TB is the commonest cause of occlusive RV in an endemic country like India.[7] TB was ruled out based on the negative Mantoux and QFT gold and a normal CT chest. The extensive occlusive RV was hypothesized to be due to alteration of the gut microbiome by UC which might have led to posterior uveitis in our patient.


  Conclusion Top


Both CD and UC should be considered in the differential diagnosis of occlusive RV with the prompt institution of systemic IST and laser photocoagulation. Ophthalmologists and gastroenterologists should be aware that patients with IBD may develop sight-threatening occlusive retinal vasculitis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Manser CN, Borovicka J, Seibold F, Vavricka SR, Lakatos PL, Fried M, et al.; Investigators of the Swiss Inflammatory Bowel Disease Cohort Study. Risk factors for complications in patients with ulcerative colitis. United European Gastroenterol J 2016;4:281-7.  Back to cited text no. 1
    
2.
Karmiris K, Avgerinos A, Tavernaraki A, Zeglinas C, Karatzas P, Koukouratos T, et al. Prevalence and characteristics of extra-intestinal manifestations in a large cohort of greek patients with inflammatory bowel disease. J Crohns Colitis 2016;10:429-36.  Back to cited text no. 2
    
3.
Troncoso LL, Biancardi AL, de Moraes HV Jr, Zaltman C. Ophthalmic manifestations in patients with inflammatory bowel disease: A review. World J Gastroenterol 2017;23:5836-48.  Back to cited text no. 3
    
4.
Duker JS, Brown GC, Brooks L. Retinal vasculitis in Crohn's disease. Am J Ophthalmol 1987;103:664-8.  Back to cited text no. 4
    
5.
Saatci OA, Koçak 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.  Back to cited text no. 5
    
6.
Bandyopadhyay D, Bandyopadhyay S, Ghosh P, De A, Bhattacharya A, Dhali GK, et al. Extraintestinal manifestations in inflammatory bowel disease: Prevalence and predictors in Indian patients. Indian J Gastroenterol 2015;34:387-94.  Back to cited text no. 6
    
7.
Dogra M, Singh R, Agarwal A, Sharma A, Singh SR, Gautam N, et al. Epidemiology of uveitis in a tertiary-care referral institute in North India. Ocul Immunol Inflamm 2017;25(sup1):S46-53.  Back to cited text no. 7
    
8.
Kawali A, Bavaharan B, Sanjay S, Mohan A, Mahendradas P, Shetty B. A long-term follow-up of retinal vasculitis-Do they develop systemic disease? Ocul Immunol Inflamm 2020;28:1181-6.  Back to cited text no. 8
    
9.
Felekis T, Katsanos K, Kitsanou M, Trakos N, Theopistos V, Christodoulou D, et al. Spectrum and frequency of ophthalmologic manifestations in patients with inflammatory bowel disease: A prospective single-center study. Inflamm Bowel Dis 2009;15:29-34.  Back to cited text no. 9
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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