Sickle cell crisis: A crisis of a different sort?

Abstract

A 9-year-old boy with sickle cell disease (SCD) was presented to the emergency department with acute headache and swelling over his bilateral temporoparietal region. There was no history of antecedent trauma, fever, vomiting or other features of an intercurrent illness. On arrival, his blood pressure was 112/62 mm Hg, heart rate was 98/min and his Glasgow Coma Scale score was 15/15. There was evidence of significant scalp tenderness over the bilateral temporoparietal region. A complete neurological examination including direct and consensual pupillary response was unremarkable. Initial investigations revealed haemoglobin of 9.6 g/dL, leucocyte count of 6.8/mm, platelet count of 219/mm and a normal coagulation profile. His current medications included hydroxyurea and penicillin prophylaxis. He underwent an urgent CT of the head followed by MRI of the brain, which revealed abnormalities as depicted in figures 1,2 edpract;103/6/290/F1F1F1Figure 1CT head. edpract;103/6/290/F2F2F2Figure 2MRI brain. QUESTION 1: Is this one of the most common neurological presentation seen in sickle cell crisis? QUESTION 2: How common is this presentation in paediatric SCD? QUESTION 3: What is the best way to manage this child?

Spontaneous Extradural Hematoma in a Sickle Cell Anemia Patient with Hyperinflammation and Thrombotic Microangiopathy Successfully Treated with Eculizumab: A Case Report and Review of the Literature

“…16 Moreover, the event of subperiosteal hemorrhage has been shown to occur concomitantly in twothirds of flat bone infarctions. 17 A literature search on PUBMED yielded 19 cases of extradural hematomas associated with cranial bone infarction in SCD patients presenting with VOC from 1987 to 2022 13,[18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] (►Table 1). The involvement of the cranial bones in these patients during VOC appears to arise from the occlusion of their vasculature and subsequent ischemia as alluded to earlier.…”

Caregivers’ experience of seeking care for adolescents with sickle cell disease in a tertiary care hospital in Bahrain

“…As children with SCD move into adolescence, sickle cell pain becomes recurrent, acute, and unpredictable [ 13 ]. The sickle cell crisis, a sudden appearance of body pain lasting for several hours resulting from vaso-occlusive complications, is the main challenge for both patients and their caregivers, leading to poor psychological well-being and overall health of patients and their caregivers [ 14 – 16 ].…”