Severe unilateral headache caused by skull bone infarction with epidural haematoma in a patient with sickle cell disease

Arends, Samuel; Coebergh, Jan; Kerkhoffs, J.L.; Gils, Adrianus van; Koppen, Hille

doi:10.1177/0333102411414441

Cited by 38 publications

(40 citation statements)

References 8 publications

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“…One of the most underrecognized complications of SCD is extradural or subdural hematoma in the absence of significant head trauma, 49,50 probably related to hypervascular areas of bone, 51 bone infarction, [52][53][54] or venous thrombosis 10 (see supplemental Figure 1B). Bony infarction in the orbit may be associated with periosteal hemorrhage and compressive optic neuropathy with risk of visual loss, whereas subgaleal hemorrhage, which may be substantial as there is a large potential space, may also occur.…”

Section: Extradural and Subdural Intracranial Hemorrhage May Occur Wimentioning

confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

210

234

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With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

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Section: Extradural and Subdural Intracranial Hemorrhage May Occur Wimentioning

confidence: 99%

Central nervous system complications and management in sickle cell disease

DeBaun

Kirkham²

2016

Blood

210

234

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“…Analysis of the extracted data resulted in a total of 22 cases (Table 1), including our index case, where spontaneous intracranial EDH has occurred [2,4,8,10,34]. In all cases, the patients presented within the first four decades of life (mean age is 15).…”

Section: Discussionmentioning

confidence: 99%

“…There are several cases where skull infarction related to sickle-cell disease has been described [2,10,12,13,24,34], although bony infarction in sickle-cell disease usually affects the long bones. The skull infarction in each case correlates anatomically with the spontaneous EDHs, but a direct causation is difficult to explain.…”

Section: Discussionmentioning

confidence: 99%

A review of spontaneous intracranial extradural hematoma in sickle-cell disease

et al. 2015

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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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“…It is thought that the expansion of intramedullary haematopoietic tissue results in the disruption of the inner and outer skull margins which may lead to thinning of cortical bones. These altered bone and periosteal structures as well as local vessel wall necrosis due to vaso-occlusion precipitate non-traumatic extravasation of blood and haematopoietic tissue into the subgaleal and epidural spaces [5].…”

Section: Discussionmentioning

confidence: 99%