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BMJ Case Rep. 2010; 2010: bcr10.2009.2394.
Published online 2010 April 5. doi:  10.1136/bcr.10.2009.2394
PMCID: PMC3047488
Unusual association of diseases/symptoms

Concurrent macular oedema and optic neuritis in Crohn’s disease with varied response to steroids

Abstract

A patient with a 10-year history of Crohn’s disease presented to our ophthalmology service with symptoms of acute anterior uveitis. Previous episodes had been treated successfully with topical steroids.

However, on this occasion after treatment with topical steroids, the patient developed macular oedema with concurrent optic neuritis in the affected eye.

Furthermore, optic neuritis persisted despite oral steroid treatment contrary to expectations based on current literature. After 6 months of treatment, optic disc oedema had markedly improved but was replaced by sectoral disc pallor.

Background

Crohn’s disease is associated with several inflammatory ophthalmic complications, which occur in 4–10% of patients.1,2 Apart from well-established associations such as uveitis and episcleritis, rare case reports exist describing optic disc swelling and macular oedema in Crohn’s disease.3,4 Treatment of intestinal inflammation, either medically or surgically, usually aids resolution of ophthalmic complications.5 We report for the first time in our knowledge a case of stable Crohn’s disease in which macular oedema and optic neuritis occur simultaneously.

Some investigators have concluded an immediate improvement in visual acuity and neurological symptoms after systemic steroid treatment to optic neuritis in Crohn’s disease. Our case disputes this; thus, further work is required to establish factors contributing to poor steroid response in the treatment of optic neuritis associated with Crohn’s disease.

Case presentation

A 67-year-old woman with a 10-year history of Crohn’s disease presented to eye casualty with complaints of blurred vision, pain and photophobia in her right eye. She denied any gastrointestinal symptoms of abdominal pain or diarrhoea. She had a history of episodic anterior uveitis on four previous occasions, which responded well each time to topical steroids. Uveitis was believed to be associated with Crohn’s disease and the patient remained compliant on azathioprine and mesalazine. The patient had never received treatment with monoclonal antibodies against tumour-necrosis factor alpha, such as infliximab.

Examination at presentation revealed best corrected visual acuity (BCVA) of 6/9 in the affected right eye (6/9 in the left eye). The patient’s pupils were equal and reactive to light and accommodation. Eye movements were full and complete and visual fields were normal. Colour vision was normal in both eyes (17/17 Ishihara plates bilaterally).

Examination of the dilated fundus revealed macular oedema in the affected eye with an optic disc of normal appearance.

A diagnosis of acute anterior uveitis with macular oedema associated with Crohn’s disease was made and the patient was started on topical dexamethasone 0.1% ophthalmic suspension hourly for 48 h then tapering over 4 weeks.

At review 2 weeks later, the patient reported a marked deterioration in her vision in the affected eye. Examination revealed worsening of her visual acuity to 6/18 and this was attributed to opacification of the posterior lens capsule in the pseudophakic eye, which received treatment in the form of YAG-laser capsulotomy.

However, soon afterwards, her visual acuity deteriorated further to 6/36 and a relative afferent pupillary defect was noted. Colour vision was also markedly diminished (1/17 Ishihara plates) in the affected eye.

Stereoscopic slit lamp examination of the dilated fundus revealed macular oedema with severe swelling of the optic disc (figure 1).

Figure 1
Fundus photograph. Notice the marked optic disc swelling with vascular congestion.

Despite a paucity of gastrointestinal symptoms, blood tests were requested. These revealed normal white cell count (6.7×109/L) and C-reactive protein levels (<5 mg/L).

Investigations

Optical coherence tomography was used to image the posterior segment, which revealed marked cystoid macular oedema (figure 2).

Figure 2
Optical coherence tomography section of the affected retina revealing cystoid macular oedema.

Differential diagnosis

A diagnosis of concurrent macular oedema and optic neuritis in association with Crohn’s disease was made. Although the patient failed to report any gastrointestinal symptoms at the time, it was judged likely that ocular disease was an extraintestinal manifestation of well-established Crohn’s disease.

Treatment

After consultation with gastroenterology colleagues, high-dose systemic steroids (oral prednisolone 80 mg daily) were commenced.

Outcome and follow-up

At 2-week follow up, visual acuity had improved to 6/18 in the affected eye and consequently the dose of prednisolone was tapered.

Two weeks thereafter, BCVA was maintained at 6/12 with near complete resolution of macular oedema (figure 3). Colour vision also recovered (15/17 Ishihara plates). However, despite one month of steroids, optic neuritis persisted—a response that contradicts accepted thinking. Prior to stopping steroids, congestion and oedema of the optic disc had grossly improved only to be replaced with sectoral pallor (figure 4).

Figure 3
Optical coherence tomography section taken 4 weeks after commencement of high-dose systemic prednisolone showing near-complete resolution.
Figure 4
Fundus photograph showing sectoral pallor of the optic disc after resolution of swelling and vascular congestion.

Due to the observed improvement in visual acuity and optic disc appearance, it was felt highly likely optic neuritis was associated with Crohn’s disease. Alternative aetiologies, such as multiple sclerosis-associated optic neuritis or disc swelling secondary to local or systemic bacterial or viral infection, were judged unlikely due to a paucity of symptoms and normal inflammatory markers.

Discussion

Macular oedema represents the final common pathway of many intraocular and systemic insults,6 and has been associated with inflammatory bowel disease, although the cause of posterior segment manifestations of Crohn’s disease are poorly understood.7

Some investigators have concluded an immediate improvement in visual acuity and neurological symptoms after steroid treatment to optic neuritis in Crohn’s disease.8 Although this rule holds true for the majority of patients, our case presents a rare treatment failure. Further work is required to establish factors contributing to poor steroid response in the treatment of optic neuritis associated with Crohn’s disease.

Learning points

  • Optic neuritis and macular oedema are recognised as rare posterior segment manifestations of Crohn’s disease.
  • We present, for the first time in our knowledge, a case of stable Crohn’s disease in which macular oedema and optic neuritis are seen to occur simultaneously.
  • Steroids are the mainstay of treatment in Crohn’s optic neuritis. Nevertheless, our case is rare due to the patient’s poor response to high-dose systemic steroids.
  • The factors causing posterior segment involvement in Crohn’s disease are poorly understood, as are factors contributing to the onset, progression and treatment response of these manifestations.
  • Further work is required to establish the pathogenesis of posterior segment manifestations of inflammatory bowel disease, as well as the place of steroids in the treatment of Crohn’s optic neuritis.

Footnotes

Competing interests: None.

Patient consent: Patient/guardian consent was obtained for publication.

REFERENCES

1. Yilmaz S, Aydemir E, Maden A, et al. The prevalence of ocular involvement in patients with inflammatory bowel disease. Int J Colorectal Dis 2007; 22: 1027–30 [PubMed]
2. Igarashi H, Yanagawa C, Igarashi S. Crohn’s disease and central retinal vein occlusion. Indian J Ophthalmol 1996; 44: 97–9 [PubMed]
3. Özdemir Y, Cosar B, Önder F, et al. Ocular complications in inflammatory bowel disease. Ann Ophthalmol 2000; 32: 245–8
4. Walker JC, Selva D, Pietris G, et al. Optic disc swelling in Crohn’s disease. Aust N Z J Ophthalmol 1998; 26: 329–32 [PubMed]
5. Ghanchi FD, Rembacken BJ. Inflammatory bowel disease and the eye. Surv Ophthalmol 2003; 48: 663–76 [PubMed]
6. Tranos PG, Wickremasinghe SS, Stangos NT, et al. Macular edema. Surv Ophthalmol 2004; 49: 470–90 [PubMed]
7. Sedwick LA, Klingele TG, Burde RM, et al. Optic neuritis in inflammatory bowel disease. J Clin Neuroophthalmol 1984; 4: 3–6 [PubMed]
8. Han SH, Lee OY, Yang SY, et al. A case of optic neuritis associated with Crohn’s disease. Korean J Gastroenterol 2006; 48: 42–5 [PubMed]

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