Bilateral phakic cystoid macular edema associated with Crohn's disease.

ABSTRACT

I report an association between Crohn's disease and bilateral phakic cystoid macular edema. A 51-year-old man with a diagnosis of Crohn's disease was referred for evaluation of decreased vision bilaterally. He had bilateral phakic cystoid macular edema, with a vision of 20/40 in the right eye and 20/60 in the left eye. Over a 2-year period, the patient was treated with nonsteroidal agents, as well as steroids, systemically, topically, and periocularly. Each recurrence of the Crohn's disease was associated with a worsening of the bilateral phakic cystoid macular edema. During the treatment period, the patient did not have clinical evidence of intraocular inflammation. Bilateral phakic cystoid macular edema may be associated with Crohn's disease, despite the lack of clinical evidence of intraocular inflammation.

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CROHN'S DISEASE is a disorder of unknown cause usually affecting young adults. The prevalence and incidence rates range from 9 to 50 cases per 100,000 and 0.8 to 5.0 cases per 100,000, respectively. (1)

With Crohn's disease, lymphocytic infiltration and granulomatous inflammation involve the full thickness of the intestinal wall. It is chronic and relapsing and can be manifested by extraintestinal complications, including arthritis, psoriasis, and mild to severe ocular disease. Ocular complications occur in 4% to 10% of patients with Crohn's disease. (2,3) Posterior segment manifestations include central serous retinopathy, uveitis, choroiditis, exudative retinal detachment, ischemic optic neuropathy, neuroretinitis, and vasculitis. Macular edema has been associated with Crohn's disease only in patients with choroiditis. (1,4) Bilateral phakic cystoid macular edema (CME) without choroiditis or clinically apparent ocular inflammation, to date, has not been associated with Crohn's disease.

CASE REPORT

A 51-year-old man with a diagnosis of Crohn's disease (pathologic diagnosis confirmed by intestinal biopsy) was referred for evaluation of bilateral decreased vision occur-ring over a 2-month period. The decrease in vision was concurrent with the episode of gastrointestinal symptoms of Crohn's disease. At presentation, visual acuities were 20/40 in the right eye and 20/60 in the left eye. Anterior segment examination showed no inflammation and was unremarkable. Posterior segment examination revealed bilateral CME, with no evidence of vasculitis, choroiditis, retinitis, vitritis, or vitreomacular traction (findings on ultrasonography were normal). Fluorescein angiography revealed bilateral CME (Figs 1 and 2). Over a 2-year period, the patient was treated with nonsteroidal agents and with steroids, systemically, topically, and periocularly. Each recurrence of the Crohn's disease was associated with a worsening of the bilateral phakic CME despite the use of systemic prednisone to control the gastrointestinal sym ptoms. The Crohn's disease has been in remission for the past year. However, the OME is persistent and not responsive to multiple medications given systemically, periocularly, and topically. His final visual acuity 3 years after initial diagnosis remains 20/40 in the right eye and 20/60 in the left eye.

DISCUSSION

Ocular complications of inflammatory bowel disease may coincide with gastrointestinal exacerbations or, in fact, may not parallel the severity of the colitis. The cause of posterior segment manifestations of Crohn's disease is not known. It may be an ocular hypersensitivity reaction as a complication of the granulomatous colitis (autoimmune mechanism). (1)

Cystoid macular edema is a nonspecific response to a multitude of ocular conditions. Any ocular condition inducing retinal traction, intraocular inflammation, or vascular compromise may induce CME. Induced conditions would include retinal vascular disease, inflammatory disease, tumors, toxic reactions (eg, to epinephrine, nicotinic acid), surgical trauma, hereditary disorders, and other causes.

Cystoid macular edema has been reported to be associated with Crohn's disease only in patients who had active choroiditis or clinically apparent uveitis. This case shows that bilateral phakic CME may be associated with Crohn's disease despite the lack of clinical evidence of intraocular inflammation. Other causes of CME in this patient were ruled out, including autosomal dominant CME (of which there was no family history). In conclusion, bilateral phakic CME without clinically apparent ocular inflammation should be added to the list of extraintestinal manifestations associated with Crohn's disease.

References

(1.) Knox DL, Schachat AP, Mustonen E: Primary, secondary and coincidental ocular complications of Crohn's disease. Ophthalmology 1984; 91:163-192

(2.) Greenstein AJ, Janowitz HD, Sachar DB: The extraintestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients. Medicine 1976; 55:401-412

(3.) Hopkins DJ, Horan E, Burton IL, et al: Ocular disorders in a series of 322 patients with Crohn's disease. Br J Ophthalmol 1974; 58:732-737

(4.) Ernst BB, Lowder CY, Meisler DM, et al: Posterior segment manifestations of inflammatory bowel disease. Ophthalmology 1991; 98:1272-1280

RELATED ARTICLE: KEY POINTS

* Ocular complications occur in 4% to 10% of patients with Crohn's disease.

* Bilateral phakic cystoid macular edema (OME) without choroiditis or clinically apparent ocular intlammation to date has not been associated with Crohns disease.

* Each occurrence of Crohn's disease was associated with a worsening of bilateral phakic CME, despite the use of systemic prednisone to control the gastrointestinal systems.

* This case illustrates that bilateral phakic OME may be associated with Crohn's disease despite the lack of clinical evidence of intraocular inflammation.

From the Department of Ophthalmology and Vitreoretinal Surgery, University of Alabama at Birmingham School of Medicine.

Supported by a departmental grant from the Research to Prevent Blindness.

Reprint requests to John 0. Mason III, MD, University of Alabama at Birmingham School of Medicine, Department of Ophthalmology and Vitreoretinal Surgery, 700 5 18th St, Suite 505, Birmingham, AL 35233.