Neuroimaging in Sickle Cell Disease: A Review

Abstract

Sickle cell disease is the most common hereditary hemoglobinopathy, which results in abnormally shaped and rigid red blood cells. These sickle-shaped red blood cells cause vaso-occlusion and ischemic phenomena that can affect any organ in the body. As a common cause of disability, the neurological manifestations of sickle cell disease are particularly important. Neuroimaging has a crucial role in the diagnosis, management, and prevention of the complications of sickle cell disease. These complications can affect the brain parenchyma, vasculature, and skull and can be ascribed directly or indirectly to a vasculopathy of small and large vessels. Vaso-occlusion can cause ischemic stroke. Ischemic damage in the absence of an acute neurological deficit, and therefore only apparent on neuroimaging, is termed silent cerebral ischemia. Weakening of the arterial walls can cause aneurysms. In its most severe form, a vasculopathy of the terminal internal carotid arteries can progress to moyamoya syndrome, characterized by steno-occlusive disease and the formation of friable collateral arteries. Rupture of aneurysms or friable collateral arteries is a potential cause of intracranial hemorrhage. The skull and vertebrae may be affected by extra-medullary hematopoiesis, due to severe anemia, or iron deposition, due to chronic red blood cell transfusion. Impaired blood supply to bone is associated with osteomyelitis and osteonecrosis. Fat embolization syndrome is a rare complication of osteonecrosis, which may cause devastating neurological impairment. Awareness and early recognition of the diverse manifestations of sickle cell disease on neuroimaging is crucial to ensure optimal treatment in a complex patient cohort.

Pediatrik Sella ve Parasellar Bolge Goruntulemesi

“…Sella ve parasellar bölge değerlendirilmesinde bölgenin büyük arterlerinin normal sinyal yokluğuna ve damar lümenlerinin büyüklüklerine dikkat edilmelidir. Çocukluk çağında nörofibromatozis tip 1 ve orak hücreli anemiye bağlı olarak gelişen vaskülopatilerde internal karotid arterlerde ve proksimal orta ve anterior serebral arterlerdeki daralmalar ve oklüzyonlar gözlenebilir [36,37].…”

A framework for advancing sustainable magnetic resonance imaging access in Africa

“…19 The cerebrovascular implications of systemic disorders such as sickle cell disease with a high prevalence rate in SSA make neuroimaging particularly important in the region. 20 Beyond NCDs, MRI can play a paramount role in the management of infectious diseases, such as HIV and malaria. [21][22][23] Furthermore, in the context of a growing global cancer burden, access to imaging services can avert millions of cancer deaths annually worldwide.…”

“…Recent evidence in stroke imaging suggests that the incidence and prevalence of stroke in Africa has grown to two to three times the number of cases observed in Western Europe and the United States 19 . The cerebrovascular implications of systemic disorders such as sickle cell disease with a high prevalence rate in SSA make neuroimaging particularly important in the region 20 . Beyond NCDs, MRI can play a paramount role in the management of infectious diseases, such as HIV and malaria 21–23 …”

A Framework for Advancing Sustainable MRI Access in Africa

“…Of note, the high prevalence of sickle cell disease in SSA makes neuroimaging particularly important due to the cerebrovascular implications of the disorder. Beyond NCDs, MRI can play a paramount role in the management of infectious diseases, such as HIV and malaria [20][21][22] .…”