1 - Serviço de Neurorradiologia, Centro Hospitalar Universitário do Porto
2 - Unidade de Neurologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central
3 - Unidade de Hematologia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central
4 - Serviço de Neurorradiologia, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central
- CMIN Summit’22, 29 junho a 1 junho 2022, Porto
Introduction
Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy, which manifests as multisystem ischemia and infarction, as well as haemolytic anaemia. Although its epidemiology is extremely variable depending on geography, it is a relatively uncommon pathology in Western Europe. Our aim is to highlight brain and spine imaging findings, within the broad spectrum of its manifestations/complications, some of which can be quite unusual and often despised.
Methodology
We present a broad spectrum of brain and spine imaging findings of SCD, found in patients from a single level III Pediatric Hospital in Lisbon, between 2010 and 2022.
Results
The morphological changes of red blood cells that occur in SCD promote ischemia/infarction as the main multi-systemic manifestation. Different patterns of ischemic brain injury and associated vasculopathy can occur, but also haemorrhagic complications, whether parenchymal, extra-axial or even epicranial. Fat or septic brain embolization may also occur, with typical imaging patterns. Bone infarctions, whether of the vault or spine are relatively common in these patients. There is also increased infectious susceptibility due to functional asplenia, justifying some cases of brain or spinal abscesses, as well as osteomyelitis.
Conclusion
Familiarity with imaging findings of brain and spinal complications of SCD, even the less commonly described in the literature, may prompt diagnosis and prognostic stratification, and promote measures to control progressive vasculopathy, protecting against early and end-organ damage.
Keywords: Sickle cell disease; neuroimaging; brain imaging; spine imaging