Pediatric Phaeohyphomycosis: A 44-Year Systematic Review of Reported Cases

Phaeohyphomycosis is an infection caused by pigmented fungi, which can be life-threatening in immunocompromised hosts and in disseminated disease. In adults with disseminated disease, mortality is as high as 79%. Data in children are derived from case reports and series. We conducted this study to review the characteristics of phaeohyphomycoses in children.

We conducted this study following the PRISMA 2020 guideline for reporting systematic reviews. We performed a review of the reported cases of pediatric phaeohyphomycoses in core bibliographic databases published in the English and Spanish language, between June 1977 and October 2021. We included all eligible cases in patients <18 years to determine the clinical characteristics, diagnosis, treatment, and outcomes.

A total of 130 cases were reviewed. The mean age was 8 years. The most common underlying conditions and risk factors included hematologic malignancies (32.5%), neutropenia (26.9%), steroid therapy (24.6%), trauma or surgery (23.1%), and children that received a transplant (14.6%). The most common presentation was localized infection (61.5%); skin and soft tissue infections were the most prevalent (25.4%). Exserohilum spp (20.8%) and Exophiala spp (17.7%) were the most common organisms isolated. Antifungal therapy remains as the most frequent treatment (87%). Overall mortality rate was 22.3% (localized 13.7% vs disseminated 37.3%).

The findings of this review suggest that phaeohyphomycoses in children have a better outcome compared to adults. We report a lower mortality rate in children when compared with adults in disseminated infection (37.3% vs 79%) and CNS infection (50% vs 60-70%). However, there is a wide variation in mortality rates according to the infection site, treatment, and underlying conditions. Prospective studies are needed.