Management of Extensive Central Nervous System Cladophialophora bantiana Infection in a 9-Year-Old Child

Juri Boguniewicz, Gail J. Demmler-Harrison, Timothy E. Lotze, Imad T. Jarjour, William E. Whitehead, Jessica Frontiero, Ankhi Dutta, Thomas Fogarty, Jill V. Hunter, Oluwaseun B. Ogunbona, Aishwarya V. Pareek, Lindsay H. Cameron

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Pediatric central nervous system (CNS) phaeohyphomycosis is a rare invasive fungal infection associated with high mortality. Methods: We describe a child with progressive neurologic symptoms whose ultimate diagnosis was Cladophialophora bantiana-associated CNS phaeohyphomycosis. We discuss her clinical presentation, medical and surgical management and review the current literature. Results: A 9-year-old female presented with acute onset of headaches, ophthalmoplegia and ataxia. Initial infectious work-up was negative, including serial fungal cerebrospinal fluid cultures. Over 2 months, she experienced progressive cognitive and motor declines, and imaging revealed worsening meningitis, ventriculitis and cerebritis. Ultimately, Cladophialophora was detected by plasma metagenomic next-generation sequencing (mNGS). Fourth ventricle fluid sampling confirmed the diagnosis of C. bantiana infection. Given the extent of her disease, complete surgical resection was not feasible. She required multiple surgical debridement procedures and prolonged antifungal therapy, including the instillation of intraventricular amphotericin B. With aggressive surgical and medical management, despite her continued neurologic deficits, she remains alive 3 years after her initial diagnosis. To our knowledge, this is one of a few published pediatric cases of CNS phaeohyphomycosis and the first with the causative pathogen identified by plasma mNGS. Conclusion: CNS phaeohyphomycosis is a serious, life-threatening infection. The preferred management includes a combination of surgical resection and antifungal therapy. In cases complicated by refractory ventriculitis, intraventricular antifungal therapy can be considered as adjuvant therapy. Direct sampling of the CNS for pathogen identification and susceptibility testing is the gold standard for diagnosis; however, the use of plasma mNGS may expedite the diagnosis.

Original languageEnglish (US)
Pages (from-to)E481-E486
JournalPediatric Infectious Disease Journal
Volume41
Issue number11
DOIs
StatePublished - Nov 1 2022
Externally publishedYes

Keywords

  • Cladophialophora bantiana
  • Xylohypha bantiana
  • intraventricular amphotericin
  • phaeohyphomycosis
  • plasma metagenomic next-generation sequencing

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Microbiology (medical)
  • Infectious Diseases

Fingerprint

Dive into the research topics of 'Management of Extensive Central Nervous System Cladophialophora bantiana Infection in a 9-Year-Old Child'. Together they form a unique fingerprint.

Cite this