A Massive Extradural Hematoma in Sickle Cell Disease and the Importance of Rapid Neuroimaging

Abstract

Sickle cell disease (SCD) is an inherited hemoglobinopathy leading to several serious organ complications and early death. It is mostly found in equatorial countries like Tanzania. Extradural hematoma (EDH) is a rare, but serious complication to SCD and may have debilitating consequences. Hitherto, there is no report of EDH in SCD where neuroimaging has been available before, during, and after such an event. Here, we describe a young female SCD patient who developed EDH that required surgical evacuation. She had made full recovery after three months. Neuroimaging performed two years prior to this event was unremarkable except for multiple small cerebral infarcts. On admission, neuroimaging revealed a subgaleal hematoma, possibly indicating disruption of the skull cortex due to increased hematopoiesis. Three months after evacuation of the hematoma, neuroimaging showed evidence of brain atrophy and the previously reported cerebral infarcts and multifocal bone infarction, but no vasculopathy. Possibly, disruption of the skull cortex with subsequent bleeding caused the EDH. As the differential diagnoses of neurological complications in SCD are many and some complications are reversible, neuroimaging should be performed without delay.

Multifocal Calvarial Infarction with Epidural Hemorrhage in a Patient with Sickle Cell Vaso-Occlusive Crisis: A Case Report

“…In the current literature, surgical evacuation was pursued in patients presenting with large epidural hematomas with significant mass effect and worsening neurological symptoms. [3][4][5][6][7][8][9][10] As reported by Hettige et al, approximately 50% of patients in the current literature had surgical evacuations of their epidural hematomas. 9 Smaller epidural hematomas without significant neurologic symptoms were managed conservatively; analgesics, frequent neurologic checks, and close interval imaging follow-up were the mainstays of treatment.…”

“…Acute calvarial infarction with epidural hemorrhage is an extremely uncommon presentation of sickle cell vasoocclusive crisis. [3][4][5][6][7][8][9][10] Multifocal sites of involvement as well as superimposed subgaleal fluid collections, as seen in this case, is even less commonly reported in the existing literature. [8][9][10] The majority of these patients present with vague headaches with or without scalp swelling; focal neurologic deficits are infrequently reported.…”

“…In addition, these patients were managed medically for vaso-occlusive crisis, often with periodic simple blood transfusions. [3][4][5][6][7][8][9][10] Treatment with exchange transfusion was not explicitly stated in any of the current literature. Nonetheless, strong consideration should be given to exchange transfusion therapy in the patient presenting with vaso-occlusive crisis and intracranial symptoms to prevent potentially life-threatening complications such as acute stroke.…”

“…The nonspecific nature of the presenting symptoms highlights how this diagnosis can be overlooked if not within the working differential diagnosis. [3][4][5][6][7][8][9][10] When a patient with known sickle cell anemia presents with headache or scalp swelling, this rare complication should enter the clinical differential diagnosis and further evaluation with imaging should be pursued.…”

“…2 Very rarely, calvarial infarction with associated extra-axial hemorrhage has been reported with sickle cell anemia. [3][4][5][6][7][8][9][10] Imaging recognition of this rare presentation of vaso-occlusive crisis is essential in providing timely treatment.…”