Orbital manifestations of inflammatory bowel disease - a systematic review

Extra-intestinal manifestations of inflammatory bowel disease (IBD) occur in 25-40% of patients; however, orbital involvement is rare and underreported. Only case reports and series have been documented in the literature with no consensus on diagnosis or management.

This systematic review aims to compile and analyse the published literature on orbital manifestations of IBD, focusing on clinical presentation, imaging, management and outcomes.

A systematic search of PubMed, Embase, and Web of Science was performed from database inception to April 2025. Data were collected and risk of bias was assessed using the JBI Critical Appraisal Checklist for Case Reports/Case Series.

41 studies reporting 48 patients were included. Mean age at orbital presentation was 31 years (range 2-63 years) with a female predominance (3:1). Orbital inflammation preceded IBD diagnosis in 19 patients. Crohn's disease was most frequently associated (n = 40). The most common clinical features were periorbital pain, swelling and ophthalmoplegia, with orbital myositis (n = 32) and dacryoadenitis (n = 15) being the predominant manifestations. 30 of 48 cases (63%) presented with bilateral disease, in contrast to the 8-20% bilateral rate typically reported for non-specific orbital inflammation (NSOI). Imaging most often demonstrated extraocular muscle enlargement. The majority of patients received systemic corticosteroids, often in combination with immunomodulators or biologics with most achieving remission. Recurrence occurred in 22 patients, and two experienced permanent vision loss.

Orbital IBD is a rare but clinically significant entity, and may precede intestinal symptoms, sometimes representing the first sign of underlying IBD. Clinicians should maintain vigilance for orbital involvement in IBD, and standardised prospective reporting is needed to better characterise these manifestations and optimise patient outcomes.