Spontaneous Epidural Haematoma in Sickle Cell Anaemia: Case Report and Literature Review

Babatola, B; Yusuf, Ayodeji Salman; Abiola, Asha M; Kanu, Okezie Obasi; Oladele, Arigbabu S

doi:10.4103/2006-8808.110271

Cited by 21 publications

(7 citation statements)

References 14 publications

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“…Three pathophysiologic explanations have been proposed over the years for the causation of this rare entity: (1) Vaso-occlusion of the haematopoietically active calvarial diploic bone resulting in bone infarction and subsequent leaking of blood and proteinaceous material in the subperiosteal, epidural, or subgaleal space [38–41, 43, 45]. (2) Acute rapid expansion of hematopoiesis with resultant microfracture of already thinned inner cortex and extravasation of blood and hematopoietic tissue [24, 28, 36]. (3) Sludging of sickle cells in the diploic veins hampering venous drainage and oozing of blood due to vascular injury and elevated back pressure.…”

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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“…It is known that coagulopathies may be responsible for spontaneous bleeding, including epidural bleeding. 41 In the present study, the observed coagulopathies (►Table 3) were: sickle cell anemia (SCA), 2,42,43,44,45,46,47,48,49,50 coagulation factor XIII deficiency, 51,52 hypofibrinogenemia, 53,54 vitamin K deficiency, 55 congenital afibrinogenemia, 56,57 immune thrombocytopenic purpura, 27,58 myelodysplastic syndrome, 59 disseminated intravascular coagulation (DIC), 60 and liver disease 61 . Coagulation disorders and pericranial infections are the two major categories of pathologies with which EDH is associated.…”

Section: Coagulopathiesmentioning

confidence: 57%

“…64 Bone infarction has been related to hematoma cases, possibly due to periosteal elevation and disruption of the bone margin. 2 Bone infarction has been reported in long bones, the spine, the sternum, and the ribs and is commonly associated with adjacent edema and hemorrhage. 66 A literature review conducted in 2015 noted an anatomical correlation between bone infarction and EDH location, but the direct cause has not yet been established.…”

Section: Coagulopathiesmentioning

confidence: 99%

Nontraumatic Intracranial Epidural Hematoma: Systematic Review of the Literature

Junior

Medeiros

Pereira³

2022

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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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.

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“…[10] Neurosurgical disorders reported in sickle cell patients in the literature include but are not limited to spontaneous epidural hematoma, anaplastic ependymoma, salmonella epidural abscess, moyamoya disease, salmonella enteritis brain abscess, intracranial aneurysms, and vertebral osteomyelitis with epidural abscess. [47815172226] Our study covered a broad spectrum of neurosurgical disorders ranging from trauma, neoplastic, vascular to cosmetic. The high female preponderance in our study is likely attributable to the more prolonged survival of females with sickle cell disease compared to males.…”

Section: Discussionmentioning

confidence: 99%

Outcome of cranial surgery in Nigerian patients with hemoglobinopathies: A retrospective study

et al. 2019

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Background: Surgical intervention in patients with hemoglobinopathies has been extensively reviewed in the literature, but information on the outcome of cranial surgery in this patient population in sub-Saharan Africa is limited. Methods: This is a retrospective study of patients with hemoglobinopathies, who underwent brain surgery in our facility. The review covered a 5-year period. We examined patient- and surgery-related variables and described the surgical complications as well as the 60-day mortality. Results: A total of nine procedures (eight under general anesthesia and one under local anesthesia) were performed on seven patients with hemoglobinopathy during the study period. Eight (88.9%) of these were done in female patients and one (11.1%) in a male patient. Six (66.7%) were performed in patients with no previous history of blood transfusion. Hb SC accounted for five (55.6%), Hb SS for three (33.3%), and Hb CC for one (11.1%) procedure, respectively. Three (33.3%) of these procedures were brain tumor-related, three (33.3%) trauma-related, one (11.1%) cosmetic, one (11.1%) vascular, and one for a postoperative complication. Only one (11.1%) procedure was associated with preoperative blood transfusion, whereas there was a need for blood transfusion following five (55.6%) of the procedures. There was a mortality rate of 11.1% (1 case). Other complications were recorded after three (33.3%) of the procedures and none with five (55.6%) of the procedures. Conclusion: Neurosurgery is possible and safe in patients with hemoglobin disorders. Adequate preoperative preparation, proper anesthetic techniques, meticulous surgery, and excellent postoperative care can help optimize outcome of surgical intervention in this patient population.

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Spontaneous Epidural Haematoma in Sickle Cell Anaemia: Case Report and Literature Review

Cited by 21 publications

References 14 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

Nontraumatic Intracranial Epidural Hematoma: Systematic Review of the Literature

Outcome of cranial surgery in Nigerian patients with hemoglobinopathies: A retrospective study

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