1 - Neuroradiology Department - Hospital de Egas Moniz, Centro Hospitalar Lisboa Ocidental (CHLO), Lisbon, Portugal
2 - Neuroradiology Department - Hospital de São José, Centro Hospitalar Lisboa Central (CHLC), Lisbon, Portugal
3 - Neuroradiology Department - Hospital Central do Funchal, Funchal, Portugal
4 - Neuroradiology Department - Hospital de Santa Maria, Centro Hospitalar Lisboa Norte (CHLN), Lisbon, Portugal
- XVII Congresso da SPNR, 25 e 26 de Novembro de 2022, Lisboa
Introduction and Purpose: Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy characterized by the presence of hemoglobin S, which leads to abnormally shaped (sickled) red blood cells. The main manifestations of this disease are related to hemolytic anemia and vaso-occlusive crises, the latter being clinically threatening. The purpose of this work is to illustrate the findings that can be found in neuroimaging in patients with SCD.
Methods: The authors present several CT and MR images performed at CHLC with the cerebral and skeletal imaging findings most frequently found in patients diagnosed with SCD.
Results and Discussion: Sickle cell disease can have a wide variety of clinical presentations, usually manifesting in early childhood. Over their lifetime, approximately 25% of these patients will have a neurologic complication. Vasculopathy is the underlying cause of many radiological manifestations, including territorial infarction, “silent ischemia” and intracranial hemorrhage (intraparenchymal and subarachnoid hemorrhage). Territorial infarcts are mostly seen in the anterior circulation, and “silent ischemia” typically involves the “watershed” border zones. Occlusion or stenosis of the distal internal carotid artery may result in the development of moyamoya syndrome. There is a greater propensity for aneurysm formation in adults with SCD, which tend to be multiple and to appear in the posterior circulation. Less frequent complications include posterior reversible encephalopathy syndrome (PRES) and cerebral fat embolism secondary to the breakdown of infarcted bone. Extramedullary hematopoiesis may be especially evident in the skull due to diploic space widening (“hair-on-end” appearance). Skeletal complications include infarction and osteomyelitis. Spontaneous epidural hemorrhage and subperiosteal hemorrhage can be seen with calvarial bone infarction, and microvascular endplate infarction may result in Hshaped vertebrae.
Conclusions: The neurological and skeletal complications of SCD are relatively common, and the neuroradiologist needs to be familiar with their neuroimaging presentation.
Keywords: Sickle cell disease; neuroimaging; brain lesions; skeletal manifestations