Steroid Responsive Idiopathic Superior Orbital Fissure Syndrome: A Case Series

Superior orbital fissure syndrome (SOFS) is a rare condition characterized by ophthalmoplegia, proptosis, ptosis, and dilation and fixation of the pupil. We present a case series with clinical and radiological findings of patients with idiopathic SOFS. All five patients had a subacute onset of lateralized painful ophthalmoplegia. All patients were female, and 3 patients were aged >50 years. Diabetes was the common (n=3) comorbidity and 4 patients sought medical help in the second/third week after symptom onset. The most common presenting symptom was drooped eyelid followed by mild-to-moderate headache. The ophthalmic division of the trigeminal nerve was involved in 3 patients. The most common radiologic abnormality was visible soft tissue in superior orbital fissure extending anteriorly, which showed faint enhancement. Patients were managed with corticosteroids, with complete recovery seen within 8 weeks. One patient had disease relapse after 6 months with the same side affected but with a larger granulomatous lesion, who responded well with a second course of corticosteroids. In conclusion, most cases of SOFS are idiopathic or inflammatory and respond well to corticosteroids. The treatable causes need to be ruled out before considering neoplastic etiology. The early recognition of symptoms and prompt treatment may result in better outcomes.