Orbital Apex Syndrome as the Presenting Feature of Large Vessel Vasculitis

Abstract

Orbital apex syndromes are commonly due to metastases and orbital pseudotumour. We present a patient in whom an orbital apex syndrome was the initial manifestation of a large vessel vasculitis, which became apparent when she subsequently developed an aortic dissection. A 73–year–old lady presented with headaches for three days and high inflammatory markers (ESR 49, CRP 83). Scans showed extensive left sided sinusitis and she was given antibiotics. Despite antibiotics she developed a complete left external ophthalmoplegia, left visual loss, pyrexia and rising inflammatory markers. Steroids were added with clinical improvement and resolving inflammatory markers within ten days (CRP 18, ESR 10). Attempts at steroid withdrawal lead to recurrence of headache. She was discharged on Prednisolone and made a complete recovery with normal inflammatory markers. This was tapered to a maintainance dose of 2.5 mg. After ten months steroids were inadvertently stopped. She developed chest pain and was seen locally on a few occasions. Initial tests were negative for cardiac and respiratory causes, but in retrospect showed a small pleural effusion and rising inflammatory markers over this period (CRP 53 and rising). Two months after stopping steroids worsening chest pain let to a CT scan showing aortic dissection and pleural effusions (ESR 98, CRP 391). She was treated surgically. Aortic histology was consistent with aortitis, but it was not possible to exclude the changes being secondary to the dissection. High dose steroids were restarted with prompt normalisation of inflammatory markers and she has remained well on a small dose of prednisolone. Giant cell arteritis is a recognised cause of aortic dissection and can rarely mimic an orbital apex syndrome. Although the inflammatory changes in the aorta of our patient were not diagnostic, after exclusion of other causes of large vessel vasculitis, we suggest that the orbital apex syndrome, pleural effuions and subsequent aortic dissection were most likely an unusual presentation of giant cell arteritis.1–5