Acute spontaneous intracranial epidural haematoma and disseminated intravascular coagulation in a paediatric sickle cell patient

Banerjee, Christopher; Yowtak, June; Fridlyand, Diana; Alleyne, Cargill H.

doi:10.1136/bcr-2018-224504

Cited by 8 publications

(14 citation statements)

References 38 publications

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“…MRI appears to be the most sensitive tool. CT scan has low yield especially in the acute phase of the disease [18]. Infarcted bone can appear normal intraoperatively although thinning of the inner cortex is frequently seen.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Saha

2019

Case Reports in Hematology

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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 literature regarding this rare entity. A 20-year-old African American female with sickle cell disease presented with vaso-occlusive crisis. About 24 hours after hospital admission, the patient had sudden deterioration of her mental status. An emergent CT scan of the head revealed a large right-sided frontoparietal epidural hematoma with midline shift, subfalcine, and uncal herniation. The patient underwent emergent hematoma evacuation but died 24 hours after surgery.

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Section: Discussionmentioning

confidence: 99%

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Saha

2019

Case Reports in Hematology

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“…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%

“…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 ]. In the second hypothesis, expansion of hematopoietic marrow in the skull, particularly after acute drops in hemoglobin, results in similar cortical thinning and blood extravasation.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous Epidural Hematoma in Sickle Cell Crisis: A Case Report

Joy¹,

Vasnaik²,

Bhat³

et al. 2022

Cureus

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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.

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“…Such connection is well known and has been described previously [ 44 ]. DIC of different severity among the SCD patients is one of the most significant outcomes of the vascular occlusions [ 45 - 47 ]. Another pathological outcome of such inflammation is ACS, which is the leading cause of morbidity and mortality for patients with SCD [ 48 ].…”

Section: Discussionmentioning

confidence: 99%

Azithromycin Reduces Markers of Vascular Damage in Pediatric Patients With Sickle Cell Disease

et al. 2021

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Background Immunomodulatory effects of macrolides in chronic inflammation are well known. In this study, we tested our hypothesis that azithromycin (AZT) can decrease inflammation in pediatric patients with sickle cell disease (SCD). Methods The use of AZT as an anti-inflammatory agent was evaluated in double-blind, placebo-controlled, cross-over study for 8 weeks of treatment with 8 weeks of washout. Blood samples were collected before (PRE) and after (POST) each 8-week treatment period. Repeated measures analysis of variance (ANOVA) with post hoc multiple comparison procedures and Chi-square test were used for statistical analysis of the data. Complete blood count, distribution of the lymphocyte subsets, and plasma levels of markers of vascular damage were analyzed. Results A significant decrease in the number of leucocytes and granulocytes was observed in AZT group following treatment. An opposite dynamic was observed in placebo group; numbers of granulocytes significantly increased at POST interval. All markers of vascular damage were reduced in AZT group at POST interval with overall significance (P = 0.026). The most prominent significant changes were observed in levels of myeloid-related protein 8/14 (MRP8/14), lipocalin A (NGAL), matrix metalloproteinases (MMP) 9, and insulin-like growth factor-binding protein (IGFBP) 4. Plasma level of C-reactive protein (CRP) was significantly decreased in AZT group as well. Conclusions Data suggested that AZT may be beneficial in management of microvascular injury in SCD.

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Acute spontaneous intracranial epidural haematoma and disseminated intravascular coagulation in a paediatric sickle cell patient

Cited by 8 publications

References 38 publications

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Spontaneous Epidural Hemorrhage in Sickle Cell Disease, Are They All the Same? A Case Report and Comprehensive Review of the Literature

Spontaneous Epidural Hematoma in Sickle Cell Crisis: A Case Report

Azithromycin Reduces Markers of Vascular Damage in Pediatric Patients With Sickle Cell Disease

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