Abstract
Background: Due to the difficulties in the definite diagnosis, data on brain imaging in pediatric patients with central nervous system (CNS)-invasive mold infection (IMD) are scarce. Our aim was to describe brain imaging abnormalities seen in immunocompromised children with CNS-IMD, and to analyze retrospectively whether specific imaging findings and sequences have a prognostic value. Methods: In a retrospective study of 19 pediatric patients with proven or probable CNS-IMD, magnetic resonance imaging (MRI)-findings were described and analyzed. The results were correlated with outcome, namely death, severe sequelae, or no neurological sequelae. Results: 11 children and 8 adolescents (11/8 with proven/probable CNS-IMD) were included. Seven of the patients died and 12/19 children survived (63%): seven without major neurological sequelae and five with major neurological sequelae. Multifocal ring enhancement and diffusion restriction were the most common brain MRI changes. Diffusion restriction was mostly seen at the core of the lesion. No patient with disease limited to one lobe died. Perivascular microbleeding seen on susceptibility weighted imaging (SWI) and/or gradient-echo/T2* images, as well as infarction, were associated with poor prognosis. Conclusions: The presence of infarction was related to poor outcome. As early microbleeding seems to be associated with poor prognosis, we suggest including SWI in routine diagnostic evaluation of immunocompromised children with suspected CNS-IMD.
Highlights
Despite the availability of potent antifungal compounds, invasive fungal infections, in particular those affecting the central nervous system (CNS), are associated with high morbidity and mortality in immunocompromised patients
The deceased patients suffered from ALL (n = 4), severe combined immunodeficiency (SCID), chronic granulomatous disease (CGD), and myelodysplastic syndrome (MDS)
All 5 children with severe neurological sequelae had ALL as a primary disease; age varied between 3 and 14 years
Summary
Despite the availability of potent antifungal compounds, invasive fungal infections, in particular those affecting the central nervous system (CNS), are associated with high morbidity and mortality in immunocompromised patients. Early diagnosis and treatment of IMD of the CNS seems to be associated with better outcomes. Non-invasive procedures such as brain imaging studies play a key role in the early detection of CNS-IMD. We aimed to describe the brain imaging abnormalities seen in immunocompromised children with CNS-IMD, and to analyze whether specific MRI findings and sequences have a prognostic value. Due to the difficulties in the definite diagnosis, data on brain imaging in pediatric patients with central nervous system (CNS)-invasive mold infection (IMD) are scarce. CNS-IMD, and to analyze retrospectively whether specific imaging findings and sequences have a prognostic value. Methods: In a retrospective study of 19 pediatric patients with proven or probable CNS-IMD, magnetic resonance imaging (MRI)-findings were described and analyzed.
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