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Published on: January 17, 2018
Sphenoidal sinogenic extradural empyema associated with juvenile myelomonocytic leukemia
N Guignard1, T Roujeau2, L Saumet3
1Department of ENT and Head and Neck Surgery, University Hospital Gui de Chauliac, 80 Avenue Augustin Fliche, 34295, Montpellier Cedex 5, University of Montpellier, France.
Abstract:
Intracranial empyema is a rare but serious complication of sinusitis in children. Myelodysplastic/myeloproliferative syndromes (MMS), including juvenile myelomonocytic leukemia (JMML), can lead to immunosuppression, thus favouring infections. We report the case of a sphenoid sinogenic retro-clival extradural empyema in a 14-year-old female patient associated with JMML. Treatment consisted in an endonasal transphenoidal drainage of the empyema associated with intravenous antibiotherapy. The patient was thereafter enrolled in chemotherapeutic treatment with Azacitidine. The disease progressed to blast phase, indicating bone marrow graft. This is the first reported case of an endocranial complication of bacterial sinusitis associated with MMS in a child.
Insights
This case report details a rare intracranial empyema in a child with juvenile myelomonocytic leukemia (JMML). Early diagnosis and treatment of this serious complication are crucial for managing associated infections.
Area of Science:
- Pediatric Neurology
- Pediatric Hematology
- Infectious Diseases
Background:
- Intracranial empyema is a severe complication of sinusitis, particularly in immunocompromised children.
- Myelodysplastic/myeloproliferative syndromes (MMS), such as juvenile myelomonocytic leukemia (JMML), increase infection risk due to immunosuppression.
Observation:
- A 14-year-old female patient with JMML developed a sphenoid sinogenic retro-clival extradural empyema.
- The patient underwent endonasal transsphenoidal drainage and intravenous antibiotic therapy.
Findings:
- Despite treatment, the patient's JMML progressed to blast phase, necessitating a bone marrow transplant.
- This case highlights the first reported instance of an intracranial complication of bacterial sinusitis linked to MMS in a pediatric patient.
Implications:
- This case underscores the importance of recognizing and managing intracranial empyema in pediatric patients with hematologic malignancies.
- Prompt intervention for sinogenic intracranial empyema is critical, even in the context of underlying immunosuppressive conditions like JMML.

