SicklER: Acute Radiologic Manifestations of Sickle Cell Disease in the Emergency Department

Congress:

ECR 2019

Poster Number:

C-1291

Type:

Educational Exhibit

Keywords:

Emergency, CT, MR, Ultrasound, Education, Acute

Authors:

T. Rashid¹, D. Sadowsky¹, T. Li¹, U. Hasan¹, C. Lugo², L. LeCompte², P. Gerard²; ¹Valhalla, NY/US, ²Valhalla/US

DOI:

10.26044/ecr2019/C-1291

DOI-Link:

https://dx.doi.org/10.26044/ecr2019/C-1291

Fig. 1: Presentation of acute chest syndrome in a 26 year old female with history of...

Fig. 2: 27 year old male with history of sickle cell disease who presents with...

Fig. 3: 27 year old female with history of sickle cell disease who presents with...

Fig. 4: 14 year old female with a history of sickle cell disease who presents with...

Fig. 5: 27 year old female with sickle cell disease with history of left frontal lobe...

Fig. 6: MRA head and neck in the same 27 year old female with sickle cell disease with...

Learning objectives

The goals of this educational exhibit include a clarification of the pathophysiology and radiographic features of several different presentations of sickle cell disease, including pathophysiology and radiographic features of acute chest syndrome (ACS), osteonecrosis, ischemic stroke, SVC syndrome, and splenic sequestration crisis.

Background

Sickle cell disease is an autosomal recessive disease which results from a point mutation in the gene that codes for the beta globin chain of hemoglobin, resulting in sickled red blood cells which impair blood flow in the deoxygenated state. Possessing one copy of this trait confers a protective effect against malaria and survival advantage. Globally, 4.4 million people are estimated to have sickle cell disease, with an additional 43 million having sickle cell trait. 80% of sickle cell disease occurs in Sub-Saharan Africa, with...

Findings and procedure details

Acute Chest Syndrome:Vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell disease. Will present in a patient with acute chest pain as a new opacification of the lung fields on a chest x-ray. Other sequelae of sickle cell disease, including rib enlargement, cardiomegaly, and bone infarcts can also be clues which point to chronicity. The chest x-ray is usually diagnostic of this entity: a CT scan of the thorax may show a mosaic perfusion pattern which could also be associated with...

Conclusion

Imaging plays a key role in identifying acute complications of sickle cell disease. Knowledge of and prompt recognition by the radiologist of the many different sequelae of such complications is essential in guiding appropriate management and the prevention of morbidity and mortality.

Personal information

For further questions, please contact: Dr. Perry Gerard, MD, MBA, FACR New York Medical College Westchester Medical Center Valhalla, NY 10595 914-493-8267 [email protected]

References

1. GBD 2015 Mortality and Causes of Death Collaborators. Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systemic analysis for the Global Burden of Disease Study 2015. Lancet 2016; 388:1459-1544. 2. Piel FB, Hay SI, Gupta S, et al. Global burden of sickle cell anaemia in children under five, 2010-2050: modeling based on demographics, excess mortality, and interventions. PLoS Med 2013; 10:e1001484. 3. Onwubalili JK. Sickle cell disease and infection. J Infect 1983; 7:2. 4....