Abstract:Trauma to the skull causing injury to the middle meningeal artery, middle meningeal vein, or dural venous sinuses is responsible for most cases of epidural hemorrhage (EDH). Spontaneous EDH is a rare entity in clinical practice. Common causes include sinusitis, coagulation abnormalities, dural metastasis, and Langerhans cell histiocytosis. Isolated nontraumatic EDH is an exceedingly rare complication of sickle cell disease (SCD). We report a case of spontaneous EDH in a patient with SCD and review the world li… Show more
“…Subarachnoid hemorrhage is the most common type of intracranial hemorrhage, with EDH rare. However, spontaneous EDH in SCD is being increasingly recognized [ 3 , 4 , 8 , 9 ], albeit without clear data on its frequency or risk factors.…”
Section: Discussionmentioning
confidence: 99%
“…The pathophysiology of spontaneous EDH is not entirely understood. The three main proposed mechanisms for this entity are related to bony infarction, hematopoietic marrow expansion, and altered blood flow due to increased viscosity [ 4 , 8 , 9 ]. The first hypothesis states that infarction of the skull leads to periosteal elevation and disruption of the cortical bone margin, resulting in leakage bleeding into the epidural space.…”
Section: Discussionmentioning
confidence: 99%
“…The first hypothesis states that infarction of the skull leads to periosteal elevation and disruption of the cortical bone margin, resulting in leakage bleeding into the epidural space. This is suggested by the coexistence of subgaleal or orbital hematomas in those with EDH due to SCD [ 4 ]. MRI of the skull commonly shows bone/ marrow hyperintensity due to edema, with contrast enhancement [ 9 ].…”
Section: Discussionmentioning
confidence: 99%
“…Non-traumatic or spontaneous EDH is an uncommon occurrence, with implicated etiologies including infections, coagulopathies, advanced renal disease, and malignancy, among others [ 1 ]. While cerebrovascular events, particularly ischemic stroke, are common in sickle cell disease (SCD) [ 2 , 3 ], spontaneous EDH secondary to SCD is rare, with only a few reports in English literature [ 4 ].…”
Epidural hematoma (EDH) classically occurs secondary to trauma. Spontaneous EDH is uncommon and can be a rare complication of sickle cell disease (SCD). We report the case of a 20-year-old Indian male with sickle cell anemia, who presented with a sickling bony crisis and suffered a non-traumatic EDH within 24 hours of admission.
A 20-year-old male presented with generalized body pain, suggestive of a sickling bony crisis. He was promptly admitted and received standard treatment for the same. The next day, he developed severe right-sided headache, associated with orbital pain, decreased movements on the right side, and altered sensorium. He had a Glasgow coma scale score of 8/15, and reduced power of the right upper limb and lower limb. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed a left-sided large parieto-temporal epidural hematoma with midline shift and mass effect. He underwent emergency decompressive craniotomy and evacuation of the hematoma, following which he recovered well, with no residual deficits.
Spontaneous EDH is being increasingly reported in SCD. Possible mechanisms include skull bone infarction, altered skull bone anatomy due to extramedullary hematopoiesis, and venous congestion due to sluggish blood flow in diploic veins. In our patient, altered skull anatomy appeared to be the causative mechanism. Early identification of EDH and aggressive neurosurgical management is crucial to survival and a good prognosis.
“…Subarachnoid hemorrhage is the most common type of intracranial hemorrhage, with EDH rare. However, spontaneous EDH in SCD is being increasingly recognized [ 3 , 4 , 8 , 9 ], albeit without clear data on its frequency or risk factors.…”
Section: Discussionmentioning
confidence: 99%
“…The pathophysiology of spontaneous EDH is not entirely understood. The three main proposed mechanisms for this entity are related to bony infarction, hematopoietic marrow expansion, and altered blood flow due to increased viscosity [ 4 , 8 , 9 ]. The first hypothesis states that infarction of the skull leads to periosteal elevation and disruption of the cortical bone margin, resulting in leakage bleeding into the epidural space.…”
Section: Discussionmentioning
confidence: 99%
“…The first hypothesis states that infarction of the skull leads to periosteal elevation and disruption of the cortical bone margin, resulting in leakage bleeding into the epidural space. This is suggested by the coexistence of subgaleal or orbital hematomas in those with EDH due to SCD [ 4 ]. MRI of the skull commonly shows bone/ marrow hyperintensity due to edema, with contrast enhancement [ 9 ].…”
Section: Discussionmentioning
confidence: 99%
“…Non-traumatic or spontaneous EDH is an uncommon occurrence, with implicated etiologies including infections, coagulopathies, advanced renal disease, and malignancy, among others [ 1 ]. While cerebrovascular events, particularly ischemic stroke, are common in sickle cell disease (SCD) [ 2 , 3 ], spontaneous EDH secondary to SCD is rare, with only a few reports in English literature [ 4 ].…”
Epidural hematoma (EDH) classically occurs secondary to trauma. Spontaneous EDH is uncommon and can be a rare complication of sickle cell disease (SCD). We report the case of a 20-year-old Indian male with sickle cell anemia, who presented with a sickling bony crisis and suffered a non-traumatic EDH within 24 hours of admission.
A 20-year-old male presented with generalized body pain, suggestive of a sickling bony crisis. He was promptly admitted and received standard treatment for the same. The next day, he developed severe right-sided headache, associated with orbital pain, decreased movements on the right side, and altered sensorium. He had a Glasgow coma scale score of 8/15, and reduced power of the right upper limb and lower limb. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain showed a left-sided large parieto-temporal epidural hematoma with midline shift and mass effect. He underwent emergency decompressive craniotomy and evacuation of the hematoma, following which he recovered well, with no residual deficits.
Spontaneous EDH is being increasingly reported in SCD. Possible mechanisms include skull bone infarction, altered skull bone anatomy due to extramedullary hematopoiesis, and venous congestion due to sluggish blood flow in diploic veins. In our patient, altered skull anatomy appeared to be the causative mechanism. Early identification of EDH and aggressive neurosurgical management is crucial to survival and a good prognosis.
“…Venous sinus thrombosis ( Figure 5J ) and reversible posterior leukoencephalopathy ( Figure 6 ) may also be associated with hemorrhage. There are reports of epidural hematomata in the absence of significant head trauma in SCD [ 26 , 27 ], probably related to hypervascular areas of bone. Figure 6.…”
Section: Qualitative Imaging In Acute Neurological Settingsmentioning
Introduction: Over the past decades, neuroimaging studies have clarified that a significant proportion of patients with sickle cell disease (SCD) have functionally significant brain abnormalities. Clinically, structural magnetic resonance imaging (MRI) sequences (T2, FLAIR, diffusion-weighted imaging) have been used by radiologists to diagnose chronic and acute cerebral infarction (both overt and clinically silent), while magnetic resonance angiography and venography have been used to diagnose arteriopathy and venous thrombosis. In research settings, imaging scientists are increasingly applying quantitative techniques to shine further light on underlying mechanisms. Areas covered: From a June 2020 PubMed search of 'magnetic' or 'MRI' and 'sickle' over the previous 5 years, we selected manuscripts on T1-based morphometric analysis, diffusion tensor imaging, arterial spin labeling, T2-oximetry, quantitative susceptibility, and connectivity. Expert Opinion: Quantitative MRI techniques are identifying structural and hemodynamic biomarkers associated with risk of neurological and neurocognitive complications. A growing body of evidence suggests that these biomarkers are sensitive to change with treatments, such as blood transfusion and hydroxyurea, indicating that they may hold promise as endpoints in future randomized clinical trials of novel approaches including hemoglobin F upregulation, reduction of polymerization, and gene therapy. With further validation, such techniques may eventually also improve neurological and neurocognitive risk stratification in this vulnerable population.
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