Spontaneous extradural and subgaleal hematoma: A rare neurosurgical crisis of sickle cell disease

Abstract

Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery. The association of epidural hematomas in sickling hemoglobinopathies is reviewed. In all cases, we noticed one episode of sickle cell crisis just before the occurrence of spontaneous EDH. Perhaps this crisis puts an extra demand over the hematopoietic skull tissue disrupting inner and outer skull margins leading to spontaneous EDH and subgaleal hematoma.

Spontaneous Subgaleal Hematoma in a Sickle Cell Disease Patient: A Case Report

“…Silent and acute parenchymal infarctions and moyamoya vasculopathy comprise most neurological complications of SCD [ 1 ]. Two-third of the central nervous system complications are ischemic; intracerebral hemorrhages are less common [ 2 ]. Subarachnoid or spontaneous extradural hematomas are even less common than the other two complications [ 2 ].…”

“…Two-third of the central nervous system complications are ischemic; intracerebral hemorrhages are less common [ 2 ]. Subarachnoid or spontaneous extradural hematomas are even less common than the other two complications [ 2 ]. These central nervous system (CNS) complications can reach up to 6.9% in children with SCD compared to 0.5% in healthy children [ 1 ].…”

“…Sickle cell disease (SCD) is one of the most commonly inherited autosomal recessive conditions in people of African, Indian, Arabian Peninsula, and Mediterranean descent [ 1 , 2 ]. The oxygen-carrying hemoglobin molecules of red blood cells are affected in this hemoglobinopathy, predisposing the blood cells to a sickle-shape that may occlude small arteries and cause tissue hypoxia and micro/macro infarcts [ 1 , 2 ]. Five to seven percent of the worldwide population is estimated to have hemoglobinopathies with Africa having the highest prevalence of SCD [ 3 , 4 ].…”

Spontaneous Epidural and Corpus Callosum Hemorrhage in Sickle Cell Disease – An Unusual Presentation in a Ghanaian Patient

“…Sub-galeal hematomas are more associated with epidural hematomas with underlying bone infarction. Mishra et al [ 9 ] analyzed prior published reports and found skull bone infarction in seven out of nine cases reviewed. From the reviewed case reports in adults, the mean age at presentation was found to be 19 years, which is comparable to our patient’s reported age of 18 years.…”

“…The majority of the previous reports identify skull bone infarction in the same area as the extradural hematoma and the commonest interpretation is that extradural hematomas are a complication of bone infarction disrupting the cortical bone, causing periosteal elevation and subsequent bleeding into the extradural space. There could also be a spontaneous rupture of epidural vessels in the vicinity of the infarcted bone [5][6][7][8][9].…”