Idiopathic apical orbital inflammation: clinical features and outcomes

Idiopathic apical orbital inflammation (AOI) is used to describe inflammation involving the posterior third of the orbit where no specific cause can be identified. It is a rare entity in which the presentation can vary widely. We aim to describe a case series of patients with idiopathic AOI and their clinicoradiological features and visual outcomes.

Retrospective multi-centre case series involving patients with idiopathic AOI.

Seven patients were included. The mean age was 50.3 ± 18.4 years (range: 31-84 years). The left eye was involved in the majority of patients (4, 57.1%), with the most common presenting clinical symptoms and signs being limitation of extraocular movements (6, 85.7%), periorbital pain (4, 57.1%), and blurred vision (4, 57.1%). Optic neuropathy was present in 3 (42.9%) patients. Three patients (42.9%) underwent an orbital biopsy, which were all consistent with nonspecific inflammation. Medical treatment was used for all patients in the form of oral steroids (5, 71.4%) or nonsteroidal anti-inflammatories (1, 14.3%). Two (28.6%) patients had additional immunosuppression. At last follow-up, 2 (28.6%) patients had achieved complete resolution of all of their symptoms, 2 (28.6%) patients had significant clinical improvement with mild residual disease, 2 (28.6%) had stable disease, and 1 (28.6%) was lost to follow-up.

Idiopathic AOI is a rare condition that can present with a wide variety of symptoms and be potentially blinding. Patients responded well to oral corticosteroids, but biopsy and additional immunosuppressive agents should be considered in those with recurrent, poorly responsive, or atypical disease.