Management of Spontaneous Epidural Hematoma in the Setting of Vaso-occlusive Crisis among Pediatric Patients with Sickle Cell Disease: A Case Series and Scoping Literature Review

Rutvi Patel, Hailey Reisert, Margaret Keymakh, Eleni Drakou, Jessica Briggs, Joshua Cohen, Jimmy S. Lee, Daniel Lax, Kaitlin Strumph, Mandana Behbahani, Amanda Baker, Andrew Kobets

Research output: Contribution to journalArticlepeer-review

Abstract

Spontaneous epidural hematoma (EDH) is a rare sickle cell disease (SCD) complication. We report 3 pediatric cases with SCD and spontaneous EDH and 1 with subgaleal hematomas in the setting of vaso-occlusive crises and elaborate on their presentation and management. Through a scoping review, we identified 71 additional cases reported from 1970 to 2024 and highlighted notable features. We highlight skull infarction as a prognostic factor associated with 88% lower odds of death relative to patients without skull infarction (P<0.01). We clarify optimal management strategies like early and aggressive treatment of disseminated intravascular coagulation (DIC), as 57% of patients with DIC died such that DIC was associated with 7.56 times higher odds of death (P=0.01).

Original languageEnglish (US)
Pages (from-to)38-46
Number of pages9
JournalJournal of Pediatric Hematology/Oncology
Volume47
Issue number1
DOIs
StatePublished - Jan 1 2025

Keywords

  • epidural hematoma
  • sickle cell disease
  • vaso-occlusive crisis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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