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Abstract and Figures

Sickle-cell disease is common among patients of Afro-Caribbean origin. Though it can precipitate neurological conditions, it only rarely causes neurosurgical problems, with very few reported cases. We describe the case of a 7-year-old girl with a background of sickle-cell disease (SCD) brought into an acute neurosurgical unit in extremis, signs of a raised ICP, and with no history of recent trauma. Following further investigations, an acute drop in the hemoglobin and hematocrit levels were noted, with the cause of her presentation being attributed to a sickling crisis causing skull convexity infarction and resulting in spontaneous bilateral extradural hematomas requiring emergency evacuation. We review the current literature and propose the pathophysiological mechanism behind this phenomenon.
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REVIEW ARTICLE - VASCULAR
A review of spontaneous intracranial extradural
hematoma in sickle-cell disease
Samantha Hettige
1
&Agbolahan Sofela
1,2
&Sanj Bassi
1
&Chris Chandler
1
Received: 14 July 2015 /Accepted: 3 September 2015 /Published online: 16 September 2015
#Springer-Verlag Wien 2015
Abstract Sickle-cell disease is common among patients of
Afro-Caribbean origin. Though it can precipitate neurological
conditions, it only rarely causes neurosurgical problems, with
very few reported cases. We describe the case of a 7-year-old
girl with a background of sickle-cell disease (SCD) brought
into an acute neurosurgical unit in extremis, signs of a raised
ICP, and with no history of recent trauma. Following further
investigations, an acute drop in the hemoglobin and hemato-
crit levels were noted, with the cause of her presentation being
attributedto a sickling crisis causing skull convexity infarction
and resulting in spontaneous bilateral extradural hematomas
requiring emergency evacuation. We review the current liter-
ature and propose the pathophysiological mechanism behind
this phenomenon.
Keywords Sickle-cell disease .Extradural hematoma .Skull
convexity infarct .Spontaneous .Non-traumatic
Introduction
Non-traumatic intracranial extradural hematomas (EDHs) are
rare, and reports have associated their etiology with infections
[3,9,14,20,25,29,31,33,37], coagulation disorders [39,
43], hemodialysis, cardiac surgery [28,38], neoplasms [6,15,
18,22,41], and vascular malformations [35]. The first case
report of a spontaneous EDH was described in 1951 [37].
Sickle-cell disease, SCD, is a very common genetic disor-
der of red blood cells commonly affecting people of Afro-
Caribbean descent (affects up to 7.3 % of the worldsblack
population) [23]. It is characterized by sickle-shaped stiff cells
and causes intermittent obstruction to intravascular blood
flow. An acute episode of flow blockage in small blood ves-
sels causing end tissue infarction is one of the manifestations
of a crisis[10,38].
There is currently no known cure for the disease, but pa-
tients presenting acutely are managed symptomatically with
supportive therapy such as analgesia, intravenous and oral
fluids, intravenous antibiotics, if a source of infection is iso-
lated (e.g., acute chest syndrome), and prophylactic low mo-
lecular weight heparin as patients tend to have vaso-occlusive
crises; especially in the cerebro-vascular system, with hemor-
rhages being relatively rare (75 % are ischemic, 25 % are
hemorrhagic) [30]. Here we describe a child with sickle-cell
crisis who presented in coma due to spontaneous bilateral
EDHs.
Case report
A 7-year-old Afro-Caribbean girl was found unresponsive in
bed by her parents. On admission, she had a Glasgow Coma
Score of 3, with a right fixed and dilated pupil, and with no
history of recent trauma. After intubation and ventilation, a
CT scan showed the presence of a large right parietal
extradural hematoma, with significant midline shift, and a
smaller left parietal EDH (Fig. 1). She was known to suffer
from sickle-cell disease and suffered 34 crises a year. Her
regular medication included penicillin V, folic acid, and zinc
supplements. She had recently been discharged from the hos-
pital with a recurrent episode of acute crisis (causing head-
aches) the day before she was found unresponsive. Blood tests
*Agbolahan Sofela
asofela@doctors.org.uk
1
Department of Neurosurgery, Kings College Hospital, London, UK
2
c/o CL Chandler, Department of Neurosurgery, Kings College
Hospital, Denmark Hill, London SE5 9RS, UK
Acta Neurochir (2015) 157:20252029
DOI 10.1007/s00701-015-2582-6
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
... The most frequent etiology of spontaneous EDH observed in the present study was SCA, accounting for 27.4% of all 95 patients listed in ►Table 1. Sickle cell anemia, characterized by changes in the shape of red blood cells and intermittent intravascular obstruction of blood flow, represents a common genetic disorder, especially among African Americans (1:600). 62,63 Epidural hematoma is the most common neurosurgical emergency complication in patients with SCA 48 ; however, it still is a rare manifestation. Nevertheless, in patients with SCA, EDH should be suspected if the patient starts presenting with sudden headaches or other signs of intracranial hypertension, leading to a search for bone lesions and hemostatic disorders, such as thrombocytopenia. ...
... 64 The symptoms presented by SCA patients differ from the classical symptoms of EDH caused by trauma, which often have a lucid interval in clinical condition, as suggested by Babatola et al. 2 Among homozygous patients, chronic hemolytic anemia may occur, with increased susceptibility to infections, vaso-occlusive crises, and cerebrovascular disorders (especially cerebral ischemia), which can worsen the clinical conditions of the patient. 63 A SCA crisis may increase the hematopoietic demand on cranial medullary tissue, predispose to bone margin disruption and subsequent hemorrhage. All patients reported by Mishra et al. 65 had an SCA crisis that preceded the EDH. ...
... 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. 63 In contrast, studies hypothesized that cases of spontaneous EDH are not associated with bone infarction and may occur due to abnormalities in cranial anatomy, 2 so that chronic expansion of hematopoietic tissue may rupture the inner and outer margins of the bone, causing extravasation of blood and medullary tissue into the subgaleal or epidural spaces. Another proposed mechanism has been less discussed and considers that the insufficient venous drainage in sickle cell pathology is possibly responsible for edema and hemorrhage. ...
Article
Full-text available
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.
... It is known that most cases of spontaneous AEDH are induced by three major causes, sickle cell disease, adjacent paranasal sinusitis, and cancer spreading to the skull or dura mater. [1][2][3][4][5][6][7][8][9][10][11] Other minor causes of spontaneous AEDH have been vascular abnormalities, coagulopathies, autoimmune diseases, anticoagulant treatment, after heart surgery, hemodialysis, or skull bone neoplasms. [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27] However, there are three reports of intracranial spontaneous AEDH without any causative disease or abnormality, which is called "idiopathic AEDH. ...
... Intracranial spontaneous AEDHs are induced mainly by three major causes, such as sickle cell disease, adjacent sinusitis, and skull or dural metastases. [1][2][3][4][5][6][7][8][9][10][11] The pathogenetic mechanisms of bleeding to the epidural space of the major causes are presumed to be as follows. ...
... Sickle cell disease was reported as the cause in 25 cases. 3,6) It is known that sickle cell disease induces bony infarction in long bones. It also induces skull bone infarction, and recanalization of embolized vessels produces hemorrhage. ...
Article
Full-text available
A 36-year-old woman presented with sudden onset of a right-sided headache that awoke her from sleep. She had no episodes of trauma or abuse. She was initially able to speak, but fell into a coma within an hour. The right pupil was dilated, with slow pupillary reflexes to light on both sides, and she showed left hemiparalysis. Computed tomography scan showed a right acute epidural hematoma, approximately 4 cm in thickness, and there were no findings of trauma such as skin wounds, subcutaneous hematomas, or skull fractures. In the emergency room, decompression of intracranial pressure by one burr hole was performed, and her dilated right pupil improved to normal size. She was then moved to the operating room, and hematoma removal was performed by craniotomy. Her blood pressure trended downward despite rapid blood transfusion and vasopressor therapy. There were no abnormal findings apparent intraoperatively, except for oozing from the whole surface of the dura mater and epidural space. Her consciousness improved postoperatively, and her left hemiparalysis improved within a few days. No causative diseases, risk factors, or vascular abnormalities were found on laboratory and radiological surveys. Two months postoperatively, the bone flap was removed because of infection. Eight months postoperatively, a cranioplasty using artificial skull was performed, and her postoperative course was uneventful. One year after the initial surgery, she has no neurological deficits, and there has been no recurrence of epidural hematoma.
... In a recent review on spontaneous hematomas in SCD, it was found that most of the patients were of the homogenous hemoglobin SS (HgbSS) genotype, and the most common location was the frontal area. Neither side appears to be more common than the other (left versus right and/or bilateral), and the majority of cases had skull infarcts that were confirmed radiologically and/or intra-operatively with around an 18.2% mortality rate [10]. ...
... Although there are reports of spontaneous SGHs in the literature, to our knowledge none of them presented with isolated spontaneous SGHs as in this case. One case was similar to our case but with EDH on one side of the head and an SGH on the other side [10]. This finding was attributed to a skull infarction, which could also have been the reason for the hematoma in our case. ...
Article
Full-text available
Sickle cell disease is one of the most commonly inherited conditions on the Arabian Peninsula. We report a case of a 17-year-old boy, who had previously been diagnosed with sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase (G6PD) deficiency and recently presented to our hospital with spontaneous subgaleal hematoma (SGH), which was managed conservatively. We also present a literature review on the topic of spontaneous intra- and/or extra-cranial bleeds.
... Neurological sequelae, impaired cognition, and increased mortality have been reported in SCD patients affected by this complication [4,5]. e underlying mechanism leading to EDH is not known, but it has been associated with a number of other diseases (e.g., infection, cancer, bleeding disorder, and vasculopathy), as reviewed by Hettige et al. [6]; however, none of them has been convincingly shown to cause EDH. Page et al. suggested that a preexistent bone infarction in SCD could lead to rupture of nearby blood vessels [4]. ...
... Complications of the central nervous system rank as one of the most serious complications to SCD. Although EDH is rare, its long-term outcome can be debilitating [4,5] and the Case Reports in Hematology 3 associated mortality is high; in their review of 22 young SCD patients with EDH, Hettige et al. found that ∼1/3 of them died due to EDH [6]. erefore, if EDH is suspected in a SCD patient, neuroimaging should be performed without delay, which is feasible also in low-resource settings such as ours [10]. ...
Article
Full-text available
Sickle cell disease (SCD) is an inherited hemoglobinopathy leading to several serious organ complications and early death. It is mostly found in equatorial countries like Tanzania. Extradural hematoma (EDH) is a rare, but serious complication to SCD and may have debilitating consequences. Hitherto, there is no report of EDH in SCD where neuroimaging has been available before, during, and after such an event. Here, we describe a young female SCD patient who developed EDH that required surgical evacuation. She had made full recovery after three months. Neuroimaging performed two years prior to this event was unremarkable except for multiple small cerebral infarcts. On admission, neuroimaging revealed a subgaleal hematoma, possibly indicating disruption of the skull cortex due to increased hematopoiesis. Three months after evacuation of the hematoma, neuroimaging showed evidence of brain atrophy and the previously reported cerebral infarcts and multifocal bone infarction, but no vasculopathy. Possibly, disruption of the skull cortex with subsequent bleeding caused the EDH. As the differential diagnoses of neurological complications in SCD are many and some complications are reversible, neuroimaging should be performed without delay.
... Acute epidural hematoma (EDH) is classically associated with trauma. 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]. ...
Article
Full-text available
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.
... Sludging of sickle cells in the diploic veins leads to insufficient venous drainage and blood oozing due to vascular injury and elevated backpressure is another proposed mechanism. [6][7][8][9] The clinical presentation of EDH in SCD patients is usually preceded by sickle cell crisis in most of the reported cases and it differs from the classic description of post-traumatic EDH which is characterized by a lucid interval. [8] Prior to the EDH, patients typically present with crises, which are difficult to control with analgesics and fluids. ...
Article
Full-text available
Introduction: Sickle cell disease (SCD) is defined as an autosomal recessive disorder characterized by the production of abnormal hemoglobin S and is correlated with high morbidity and mortality. The clinical consequences of SCD include pain crisis, acute chest syndrome, and strokes. Spontaneous epidural hematoma is a rare manifestation in sicklers with few cases reported in the literature.[6] The pathophysiology is not completely understood. However, a few explanations have been reported over the years that include vaso-occlusion of the bone resulting in bone infarction, microfracture due to rapid expansion of hematopoiesis of the inner cortex, and sludging of the sickle cells in the diploic veins-all result in leaking of blood in the epidural or in the subgalea space. Patient concerns: A 14-year-old boy known to have SCD (Hb SS) presented to the Security Forces Hospital with a history of diffuse headache associated with nausea that started 12 h prior to presentation. Diagnosis: Computed tomography (CT) showed bilateral frontal epidural hematoma and subgaleal space. Intervention: A multidisciplinary team was created (hematology, neurology, neurosurgery, and interventional radiology) and a plan was formulated as follows: Continuous monitoring of the patient's neuro vital signs and transfuse the patient with blood and platelets in addition with Levetiracetam. Outcomes: The patient was discharged after 9 days of hospital admission. He has remained symptom-free post-transfusion. Post-discharge CT scan showed a reduction in the hematoma size. Conclusion: A high index of suspicion is needed for a prompt diagnosis and treatment of this rare complication of SCD. The management strategy of EDH depends on the level of consciousness of the patient upon presentation. Surgical approach with craniotomy and evacuation or conservative management have been used with full recovery of the patients.
Preprint
Full-text available
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While primarily a hematologic disease, sickle cell anemia is notorious for its multisystemic manifestations, particularly in episodes of vaso-occlusive crisis. Multifocal acute calvarial infarction with associated epidural hemorrhage has rarely been reported in sickle cell vaso-occlusive crisis. In this article, we reported a unique case of a 15-year-old male presenting with sickle cell vaso-occlusive crisis and neuroimaging findings of multifocal calvarial bone infarction and epidural hemorrhage. Radiologists and clinicians should be cognizant of this rare complication of sickle cell anemia to ensure appropriate diagnosis and timely treatment.
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