Acute orbital inflammatory syndrome secondary to an ANCA-positive small-vessel vasculitis: a case report and review of the literature.
A 61-year-old man presents with sequential painful bilateral proptosis within 36 h and orbital compartment syndrome resulting in complete loss of vision bilaterally. Sequential urgent lateral canthotomy and cantholysis were performed to reverse the compartment syndrome. Orbital imaging showed non-specific orbital inflammation. Biopsies showed necrotizing inflammation and bloodwork was positive for c-ANCA. The patient was therefore treated with prednisone and cyclophosphomide and showed good recovery of vision in one eye, and had no recurrence of orbital inflammation. ANCA-associated orbital vasculitides are rare, but must be kept in mind in the differential diagnosis of acute orbital inflammatory syndromes.
Department of Ophthalmology, Dalhousie University , Halifax , Canada. babak.maleki@gmail.com
Babak
B
Maleki
Alejandra A
AA
Valenzuela
Weei-Yuarn
WY
Huang
J Godfrey
JG
Heathcote
D000208:Acute Disease; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D003131:Combined Modality Therapy; D003161:Compartment Syndromes / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D011241:Prednisone / Q000627:therapeutic use; D014657:Vasculitis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone