Spontaneous Epidural Hematoma in Patients With Sickle Cell Disease: A Series of Two Cases of a Rare Neurological Complication

TL;DR

This paper reports two rare cases of spontaneous epidural hematoma in patients with sickle cell disease, highlighting the importance of early diagnosis for better outcomes.

Contribution

The paper adds to the limited literature by presenting two distinct clinical cases of a rare complication in sickle cell disease.

Findings

One patient with sickle cell disease had a spontaneous EDH managed conservatively with good outcomes.

Another patient required urgent surgery for a rapidly progressing EDH and showed improvement.

The cases stress the need for high suspicion of EDH during sickle cell crises for timely intervention.

Abstract

An epidural hematoma (EDH) arises most commonly in cases of cranial trauma. Sickle cell disease (SCD) is a hemoglobinopathy associated with multiple complications, and spontaneous EDH is one of its rare complications that usually arises during the crisis phase. The proposed mechanisms include skull bone infarction, cortical disruption, and marrow expansion, which can lead to hemorrhage. Owing to its rarity, it continues to pose diagnostic challenges for clinicians. We report two cases of spontaneous EDH with distinct clinical presentations and management. The first case was of an 18-year-old homozygous SCD patient, who presented with headache and nonspecific symptoms during a crisis. Neuroimaging showed an EDH, along with a subgaleal hematoma. The patient was managed conservatively with good clinical outcomes. The second case was of a young female who developed a rapidly progressive neurological deficit, and imaging showed an extensive epidural hematoma requiring urgent surgical decompression. The patient improved subsequently. These cases emphasize the significance of a high index of suspicion for EDH during the crisis phase of SCD for timely management and better clinical outcomes.

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