Introduction Epidural hematoma (EDH) is generally a direct sequela of head injury. Spontaneous EDH is rarely described in the literature. Spontaneous EDH can be caused by infections of adjacent regions, vascular malformations of the dura mater, metastases to the bone skull, and disorders of blood coagulation. The preferred treatment is surgical. The prognosis is directly related to the size, location, and Glasgow Coma Scale score on admission and the underlying disease.
Methods A systematic literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. We performed the search in the PubMed/MEDLINE, Embase, and Scopus databases. Abstracts and articles were screened according to our inclusion and exclusion criteria.
Results The literature review yielded 1,156 records from the databases, of which a total of 164 full-text articles were included in the final synthesis, plus 22 additional relevant studies. A total of 89 case report studies were included, providing 95 unique patients. There was a predominance of coagulopathies as the main etiology of spontaneous EDH. A total of 45.3% of the patients presented lesions in > 1 brain lobe. The frontal lobe was the most prevalent location of EDH. The most used neuroimaging exam was computed tomography (CT). Surgical intervention was the most common treatment performed. A total of 24.2% of the patients died.
Conclusion Nontraumatic EDH represents an uncommon manifestation of several pathologies. Clinical investigation should be aware of such a possibility. Healthcare professionals should value the physical examination and clinical history of the patient. Given the scarcity of information on the pathogenesis of spontaneous EDH, further studies are needed to better define intervention strategies and therapies for these patients.