Acute chest syndrome (ACS) in sickle cell disease is a leading thoracic complication as well as leading cause of mortality in those affected by sickle cell disease. The diagnosis is made on the combination of new pulmonary opacity on chest x-ray with at least one new clinical symptom or sign.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Patients may present with acute fever, cough, wheezing, tachypnoea and/or chest pain on a background of established sickle cell disease.
There is no single underlying aetiology to ACS but rather a variety of infectious and noninfectious causes, including 5:
- pulmonary infarction
- fat embolism
- rib or sternal infarct causing atelectasis (from splinting)
A chest radiograph may also show other sequelae from sickle cell disease such as bone infarcts, rib enlargement and cardiomegaly (from anaemia).
May show a mosaic perfusion pattern that could be associated with a pleural effusion. The radiographic signs above may also be seen on CT.
General imaging differential considerations include:
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