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, tachypnea and/or chest pain on a background of established sickle cell disease.
There is no single underlying etiology to acute chest syndrome but rather a variety of infectious and noninfectious causes, including 5:
- pulmonary infarction
- fat embolism
- rib or sternal infarct causing atelectasis (from splinting)
Point-of-care lung ultrasonography in the acute chest syndrome may reveal one of the following patterns;
- alveolar consolidation
- the most common abnormality found, with a posterobasal regional predilection
- air bronchograms may be visualized
- anterior subpleural consolidations
- lung rockets
- three or more B-lines per sonographic field, typically 3 cm apart (B3 lines) - defines the sonographic interstitial syndrome
- bilateral diffuse anterolateral interstitial syndrome may be observed 7
- pleural effusions 9
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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- 8. Razazi K, Deux JF, de Prost N, Boissier F, Cuquemelle E, Galactéros F, Rahmouni A, Maître B, Brun-Buisson C, Mekontso Dessap A. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. (2016) Medicine. 95 (7): e2553. doi:10.1097/MD.0000000000002553 - Pubmed
- 9. Colla JS, Kotini-Shah P, Soppet S, Chen YF, Molokie R, Prajapati P, Prendergast HM. Bedside ultrasound as a predictive tool for acute chest syndrome in sickle cell patients. (2018) The American journal of emergency medicine. 36 (10): 1855-1861. doi:10.1016/j.ajem.2018.07.006 - Pubmed
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