Presentation and outcome of rhino-orbital-cerebral mucormycosis in patients with diabetes

Aim: To report presentation and outcome of rhino-orbital-cerebral mucormycosis (ROCM) exclusively in patients with diabetes mellitus. Methods: Retrospective, non-comparative, interventional analysis of the medical records of 35 patients with ROCM among 22 316 patients with diabetes seen over the last 12 years. Results: A cohort of 23 men and 12 women with a mean (SD) age of 47.3 (14.4) years ( range 18-70 years) was studied. Five patients had type 1 diabetes mellitus, 29 had type 2 diabetes mellitus, and one had secondary diabetes. Nine patients had ROCM as the first clinical manifestation of diabetes. The mean ( SD) blood glucose at presentation was 20.6 ( 8.3) mmol/l ( range 10.0 to 53.3 mmol/l) and 17 patients had ketosis/ketoacidosis. Ophthalmic symptoms and signs were pronounced: external ophthalmoplegia ( 89%), proptosis (83%), visual loss (80%), chemosis (74%), and eye lid gangrene (14%). Non-ophthalmic manifestations included sinusitis (100%), nasal discharge/ulceration ( 74%), infranuclear VI nerve palsy (46%), palatal necrosis (29%), cerebral lobe involvement (20%), and hemiparesis (17%). Computed tomography/magnetic resonance imaging showed involvement of paranasal sinuses in all patients with ethmoid (86%) and maxillary ( 80%) sinuses being most frequently involved. Orbital involvement was observed in 80% of patients with cavernous sinus thrombosis in 11%, and internal carotid occlusion and hydrocephalus in 3% each. All were treated with amphotericin B (3-3.5 g) and 26 ( 74%) patients underwent appropriate surgery. Twenty one patients (68%) survived with a mean ( SD) follow up of 39.6 (34.1) months ( range 10 months to 11 years). Factors related to poor survival included delay in diagnosis and treatment ( p < 0.05), facial and/or eye lid gangrene ( p < 0.05), hemiplegia ( p < 0.05), cerebral invasion by mucorales ( p < 0.05), and treatment with amphotericin B alone ( p < 0.05). Conclusions: In patients with diabetes and ROCM, ROCM was the presenting manifestation in one fourth of the patients. Ophthalmic and extensive cerebral involvement predominated in the clinical picture. Delay in treatment due to late presentation and associated complications were major determinants of the survival outcome in these patients.