Abstract

Background: Mucormycosis is a life-threatening infection with a tendency for angioinvasion that may lead to progressive dissemination. Disseminated mucormycosis, defined as the involvement of two or more non-contiguous sites, is rare in children, and data concerning its management and outcome are scarce. The aim of this study was to assess the contemporary management strategies and outcomes of disseminated mucormycosis in the pediatric population. Methods: We conducted a retrospective search in six large tertiary medical centers for all cases of disseminated mucormycosis that occurred between 2009–2020 in patients aged 1–20 years. Results: Twelve cases were identified. Underlying conditions included hematological malignancies (n = 10), solid tumor (post-autologous hematopoietic stem cell transplantations; n = 1), and solid organ (liver) transplantation (n = 1). In all cases, amphotericin B formulations were administered as first-line therapy; in eight cases, they were also administered in combination with an echinocandin or triazole. Seven patients underwent surgical debridement procedures. The six-week mortality was 58%. Among the patients diagnosed between 2009–2015, one of the six survived, and of those diagnosed between 2016–2020, four of the six were salvaged. Conclusions: Disseminated mucormycosis is a life-threatening and often fatal disease, and improved diagnostic and therapeutic strategies are needed. Nevertheless, in this population-based study, five patients (42%) were salvaged through combined liposomal amphotericin/triazole treatment and extensive surgical interventions.

Highlights

  • Mucormycosis is a life-threatening infection in immunocompromised children, caused by fungi of the order Mucorales

  • Fresh specimens were sent to a central reference laboratory (Clinical Microbiology Laboratory, Rambam Health Care Campus, Haifa, Israel) for DNA sequencing and analysis

  • The polymerase chain reaction (PCR) products were separated by electrophoresis in ethidium bromide stained 2% agarose gels, sequenced on a 3130 Genetic Analyzer capillary electrophoresis DNA sequencer (Applied Biosystems, Carlsbad, CA, USA) and analyzed using the Basic Local Alignment Search Tool (BLAST)

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Summary

Introduction

Mucormycosis is a life-threatening infection in immunocompromised children, caused by fungi of the order Mucorales It is characterized by a rapid clinical course, with a tendency for angioinvasion that may lead to progressive dissemination, tissue infarction, and necrosis [1]. Several clinical entities of mucormycosis have been described, including rhino-orbito-cerebral, cutaneous, pulmonary, and gastrointestinal patterns of disease [2,3,4,5] All of these may potentially evolve into disseminated disease, defined as the involvement of two or more non-contiguous sites. Conclusions: Disseminated mucormycosis is a life-threatening and often fatal disease, and improved diagnostic and therapeutic strategies are needed In this populationbased study, five patients (42%) were salvaged through combined liposomal amphotericin/triazole treatment and extensive surgical interventions

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