1 - Pediatric Department, Hospital de São Francisco Xavier, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal
2 - Neuroradiology Department, Hospital Beatriz Angelo, Lisbon, Portugal
3 - Pediatric Department, Hospital de Dona Estefània, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
4 - Pediatric Neurology Unit, Hospital de Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
5 - Hematology Unit, Hospital de Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
6 - Neuroradiology Unit, Hospital de Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
Publicação em versão integral: J Pediatr 2021 Dec 20;S0022-3476(21)01229-4. DOI: 10.1016/j.jpeds.2021.12.023
A17-year-old African male with homozygous sickle cell disease was admitted with a vaso-occlusive crisis of the lower limbs and lower back, further complicated with an acute thoracic syndrome. After 6 days of treatment with intravenous fluids, analgesics, and antibiotics, he developed a holocranial headache, nausea, and photophobia. Findings of the physical examination showed biparietal painful boggy masses (Figure 1). Neurologic examination was unremarkable, and there were no signs of intracranial hypertension. Ultrasound scan of the skull showed 2 heterogeneous fluid collections, compatible with subgaleal hematomas. To exclude intracranial hemorrhage, computed tomography scan of the head was performed, which revealed an anterosuperior frontal epidural hematoma of 6 mm (Figures 2 and 3). Coagulopathy was excluded, and a conservative approach was decided. The masses were progressively reabsorbed, and he was discharged 22 days after admission, totally asymptomatic. One month later, cranial magnetic resonance imaging was performed, showing a reduction of the subgaleal hematomas, a complete resolution of the epidural hematoma, but also T2 hyperintense areas in the calvaria compatible with bone infarction (Figure 4). Nontraumatic spontaneous epidural hematoma is extremely rare, potentially fatal, and it may be concurrent with calvarial bone infarction and subgaleal hematoma. The pathophysiology is still not fully understood and various explanations have been proposed: vaso-occlusion of the hematopoietically active skull bone with subsequent infarction and leaking of blood; rupture of epidural vessels next to the infarcted bone; acute expansion of hematopoiesis resulting in disruption of already thinner cortex skull bone, causing extravasation of blood to subgaleal or epidural spaces; and finally sludging of sickle cells causing insufficient venous drainage with subsequent congestion and hemorrhage. Epidural hematoma is associated with high mortality; however, when concurrent with subgaleal hematoma and/or bone infarction, the survival rate seems to be 100%. Nevertheless, as subgaleal hematomas can be associated with an underlying epidural hematoma, the threshold for requesting brain imaging should be low.
Keywords: Cranial mass; Epidural hematoma; Neurorradiology; Spontaneous cranial mass; Sickle cell disease