Spontaneous Non-traumatic Epidural Hematoma in Sickle Cell Disease: A Case Report

Abstract

Spontaneous non-traumatic epidural haematoma is an uncommon but potentially fatal neurological complication of sickle cell disease. This case report describes a 13-year-old girl with homozygous sickle cell disease who was admitted with a non-febrile vaso-occlusive crisis after inadequate hydration during a period of extreme heat. On admission, she was conscious and had no sensory or motor deficit, but she presented with diffuse osteoarticular pain. Laboratory testing showed severe anaemia, with haemoglobin at 6.6 g/dL, elevated C-reactive protein at 97 mg/L, hyperbilirubinaemia and elevated D-dimer, while malaria testing was negative. Initial treatment included intravenous rehydration and step-2 analgesia, with an early reduction in pain intensity. Two hours later, she developed a sudden severe diffuse headache in a non-traumatic context, followed by altered consciousness, a Glasgow Coma Scale score of 12/15 and left hemibody motor deficit. Emergency cranio-cerebral computed tomography demonstrated three extra-axial hyperdense biconvex lesions consistent with epidural haematomas in the right parieto-occipital, left parietal and left frontal regions, with mass effect and a 9 mm midline shift. A multidisciplinary team recommended urgent craniotomy with haematoma evacuation. During the surgical procedure, the patient developed cardiorespiratory arrest, and resuscitation was unsuccessful. Review of reported cases suggests that spontaneous epidural haematoma in homozygous sickle cell disease is rare, is often associated with headache or vaso-occlusive crisis, and may be linked to cranial bone infarction, marrow expansion or venous congestion. This case emphasises the need for urgent neuroimaging when acute non-traumatic headache, altered consciousness or focal neurological deterioration occurs in patients with sickle cell disease. Early recognition may support faster therapeutic decision-making in this high-risk context.