Clinical characteristics and outcome of invasive fungal sinusitis in children with hematological malignancies

Abstract

Invasive fungal sinusitis (IFS) is a rare disease that requires careful attention and prompts management due to its high mortality among pediatric patients with hematological malignancies. This is a retrospective analysis of pediatric patients with hematological malignancies treated at Children's Cancer Hospital Egypt 57 357 (CCHE) through the period from 2008 till 2016 with proven IFS. Thirty-four patients were diagnosed with IFS. Five (15%) patients had an invasive rhino-cerebral fungal disease. Mucorales were isolated in 50% (n = 17) patients, Aspergillus in 38% (n = 13) patients, and mixed fungal in 12% (n = 4) patients. Sinuses were the only localized site in (45%). Extra-nasal spread was reported in 20 patients; Sino-pulmonary in 35% (n = 12), sino-cerebral in 15% (n = 5), and sino-orbital in 5% (n = 2) patients. Combined antifungal therapy with surgical debridement was done in 59% of patients with a better outcome when compared to those who received only medical antifungal treatment (P = .01). The overall mortality rate at week 12 was 35% (n = 12), and IFS attributable mortality was 20% (n = 7). IFS with cerebral extension carried the highest mortality rate for both 12-week all-cause (P = .04) and fungal-attributable (P = .01) mortality. Pediatric patients with hematologic malignancies are susceptible to invasive fungal sinusitis (IFS). Surgical debridement, combined with antifungal therapy, improves outcomes among those patients. IFS patients with cerebral extension had a higher risk of mortality.

Lay summary: We studied the characteristics of invasive fungal sinusitis in children with hematological malignancies. Mucormycosis was the most common cause. Surgical debridement, combined with anti-fungal therapy, improves outcomes. Patients with rhino-cerebral fungal disease had a higher risk of mortality.

Keywords: Invasive fungal sinusitis (IFS); aspergillosis; children; hematological malignancies; rhino-cerebral mucormycosis.