Acute invasive fungal rhinosinusitis: our experience with 18 cases

Abstract

Acute invasive fungal rhinosinusitis (AIFRS) is a rapidly progressive life threatening infection that is seen most commonly among immunocompromised patients. We present a case series of 18 patients clinically and histopathologically diagnosed with AIFRS with a mean follow-up of 9.11 ± 2.51 months (range 6-17). Demographic data, apparent symptoms and signs, underlying disorders, and outcomes are discussed. The mean age was 39.56 ± 20.66 years (range 2-75). The most common underlying diseases were diabetes mellitus (50 %) and leukemia (44.44 %). Mucosal biopsy confirmed fungal invasion of the nasal mucosa in all cases. The main fungi were Rhizopus oryzae (55.56 %), Absidia mucor (16.67 %), and Aspergillus fumigatus (27.78 %). Headache and facial pain (77.8 %), facial paresthesia (55.6 %), and ophthalmoplegia (33.3 %) were the most common symptoms and signs. Computed tomography and endoscopic findings showed various stages of sinonasal (100 %), pterygopalatine fossa (55.56 %), orbital (44.45 %), and cerebral (5.56 %) involvement. All patients underwent serial surgical debridement (3.78 ± 1.80 times; range 2-8) simultaneously with systemic antifungal therapy and proper management of the underlying disease. The most extreme case with brain involvement survived and recovered with no evidence of recurrent disease following treatment. All patients were considered cured after two endoscopic negative histopathologic evaluations. Three patients (16.67 %) died, one from uncontrolled leukemia and two due to renal failure. AIFRS is a potentially fatal condition, however, early diagnosis and management of the underlying disease accompanied with systemic antifungal and aggressive serial surgical intervention appears to be effective in reducing mortality in most patients.

A multi‐institutional review of outcomes in biopsy‐proven acute invasive fungal sinusitis

“…The results of this study confirm that surgery and antifungal therapy are important predictors of disease survival . A study of 59 patients by Piromchai et al showed that increasing time from diagnosis of AIFS to treatment was a negative predictor of mortality .…”

“…Timely diagnosis of AIFS is difficult because early clinical findings are nonspecific. Symptoms may include fever, nasal congestion, facial swelling, epistaxis, facial/nasal pain, rhinorrhea, or headache . Extensive disease can present with visual changes, cranial neuropathies, and necrosis .…”

“…Treatment of AIFS includes systemic antifungal therapy, timely surgical debridement, and correction of the underlying cause of immunosuppression . Early debridement may be key, as it provides histologic confirmation, fungal speciation, and directs antifungal therapy. Further, as absolute neutrophil count (ANC) recovery is correlated with improved survival, immune‐stimulating therapies (ISTs) are used with unknown efficacy .…”

Benefit of Endoscopic Surgery in the Management of Acute Invasive Skull Base Fungal Rhinosinusitis

“…14,16 Others have reported higher prevalence of DM than hematologic malignancy. 17 One patient in our cohort was immunosuppressed following solid organ transplantation, a sequela which has also been frequently reported. 12,13,15,16 Other comorbidities associated with AIFRS that were not seen in our cohort include human immunodeficiency virus/acquired immunodeficiency syndrome, chronic steroid use, renal or liver failure, and autoimmune (HIV/AIDS) diseases such as systemic lupus erythematosus and rheumatoid arthritis.…”

Treatment outcomes in acute invasive fungal rhinosinusitis extending to the extrasinonasal area

“…A systematic review of AIFRS showed that the most common symptom was facial swelling (64.5%), followed by fever (62.9%), nasal congestion (52.2%), ophthalmoplegia (50.9%), facial pain (46.8%), and headache (46.3%) 6 . Conversely, a previous study reported that the most common symptoms were headache (61.1%), facial pain (55.6%), and ophthalmoplegia (33.3%) 11 . In another study, fever was the predominant presenting symptom, followed by facial pain, nasal obstruction, and rhinorrhea 12 .…”