Orbital apex syndrome secondary to granulomatosis with polyangiitis

Abstract

Orbital apex syndrome (OAS) is an optic nerve dysfunction with palsy of the third, fourth and sixth cranial nerves and ophthalmic division of the fifth cranial nerve. Causes can be infective, inflammatory, traumatic, neoplastic or vasculitic. We describe the first case in British literature and second worldwide of OAS presenting as granulomatosis with polyangiitis (GPA). A 38-year-old patient presented with left periorbital swelling and pain. An examination revealed left eye proptosis, tenderness in the ophthalmic distribution of the trigeminal nerve, unilateral ophthalmoplegia and reduced visual acuity. Initial treatment included intravenous antibiotics, steroids and nasal decongestants.Imaging demonstrated sinusitis and a suspected abscess from the infratemporal fossa to the orbital apex. However, sinus surgery showed granulation tissue without pus. The biopsy result was highly suggestive of GPA. A subsequent vasculitic screen was cytoplasmic-antineutrophil cytoplasmic antibody positive.This case highlights an unusual presentation of OAS secondary to GPA, as initial features suggested an infective cause.

Granulomatosis with Polyangiitis Presenting with Bilateral Orbital Apex Syndrome

“…Steroid pulse therapy followed by oral medication of prednisolone and cyclophosphamide resulted in an improvement in diplopia, facial palsy, meningeal thickening, and orbital abnormalities. Siddiqui et al [ 8 ] also reported a case of GPA presenting with OAS who achieved good recovery. Laboratory data showed an elevation in inflammatory markers.…”

“…Among these, orbital involvement is the most frequent manifestation, occurring in 22–45% of cases with ocular manifestations [ 3 , 4 , 5 ]. As a rare ocular manifestation, orbital apex syndrome (OAS) associated with GPA has been demonstrated in several reports [ 6 , 7 , 8 , 9 ]. OAS is characterized by optic neuropathy and total ophthalmoplegia caused by the damage of II, III, IV, V1, and VI cranial nerves due to the lesion of the orbital apex.…”

Bilateral Orbital Apex Syndrome Due to Granulomatosis with Polyangiitis

“…In most of these cases, OAS was caused by contiguous sinus or pachymeningeal infiltration. [3][4][5][6][7][8] OAS without contiguous sinus spread has been reported, but occurred in a patient with a pre-existing confirmed diagnosis of GPA. 9 This case highlights several key points.…”