Pediatric Invasive Fungal Rhinosinusitis in Immunocompromised Children With Cancer

OBJECTIVES: 1) To determine the factors that predispose towards invasive fungal rhinosinusitis (FS) in immunocompromised children with cancer, and 2) to propose practice guidelines for management of pediatric FS in immunocompromised patients. METHODS: Retrospective chart review of 9 patients who developed invasive FS compared to 8 patients who did not develop invasive FS during the study period. Presenting signs and symptoms, nasal endoscopic findings, radiographs, laboratory studies, histologic and microscopic samples, and outcomes were compared. RESULTS: Seventeen consecutive pediatric immunocompromised patients with hematologic and lymphoid neoplasms underwent nasal endoscopy and biopsy for possible FS. Nine patients were diagnosed with 10 episodes of FS; 1 patient developed FS with different organisms on 2 separate occasions separated by 6 months; 8 patients were not diagnosed with FS. Eight patients had acute myelogenous leukemia (AML); 6 patients had acute lymphoblastic leukemia (ALL); 1 patient had Burkitt's lymphoma, 1 patient had undifferentiated leukemia; and 1 patient had biphenotypic acute leukemia. All patients with FS had an absolute neutrophil count (ANC) 600 or less (range 0-600). All patients with FS had either persistent fevers or sinus symptoms (facial pain, nasal congestion, rhinorrhea). Sinus CT scans were abnormal in all patients with FS and without FS. Two patients with FS had maxillary sinus retention cysts. Operative endoscopic findings were helpful diagnostically when necrosis or ulceration was found. All patients in the non-FS group normalized their ANCs; 2 of the 9 patients in the FS group did not normalize their ANC. These 2 patients died from disseminated FS or from complications due to their immunosuppression. CONCLUSION/SIGNIFICANCE: All patients with FS had either persistent fevers or symptoms localized to the sinuses (facial pain, nasal congestion, or rhinorrhea). Endoscopic examination was helpful when necrosis was detected. We recommend directed biopsies of suspicious lesions, the middle and inferior turbinate, in immunocompromised, neutropenic pediatric patients with cancer who present with either persistent fevers or localizing symptoms to the sinuses. We favor the use of “rush” biopsies over frozen sections because of the better-quality sections and ability to perform appropriate stains.