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Sickle Cell Disease, Acute Chest Syndrome
KEY FACTS
Terminology
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New pulmonary opacity on chest radiograph + ≥ 1 additional symptom (such as fever, cough, sputum production, tachypnea, dyspnea, or hypoxia) in setting of sickle cell disease (SCD)
Imaging
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Upper & middle lobe opacities more common in children
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Lower lobe disease more common in adults
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Initial chest radiograph may be normal (46%)
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Opacity may not appear until 2-3 days after symptoms develop
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Opacities on CT may be more extensive than on radiograph
Pathology
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Potential causes: Infection (30%), pulmonary fat embolism (9%), pulmonary infarction (18%), & rib infarction
Clinical Issues
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Acute chest syndrome (ACS) most common in patients aged 2-4 years; incidence ↓ with age
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Fever, cough, & tachypnea most common symptoms in patients < 10 years of age
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Pain (chest, extremity, abdominal) more common in adolescents & adults
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Risk factors: Asthma, smoking, abdominal surgery, trauma
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ACS 2nd most common cause of hospitalization in patients with SCD after pain crisis
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ACS most common cause of premature death in patients with SCD
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Mortality 4-9x higher in adults than in children
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Treatment
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Supportive: Oxygen, antibiotics, pain control, IV fluids, incentive spirometry, & blood transfusions
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Prevention: Pneumococcal vaccine, Haemophilus influenzae vaccine, & hydroxyurea
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AP radiograph of the chest shows an opacity in the right upper lobe
in a 1 year old with sickle cell disease presenting with fever & dyspnea.
in a 1 year old with sickle cell disease presenting with fever & dyspnea.
AP radiograph of the chest in the same patient 12 hours later shows worsening opacification & volume loss of the right upper lobe
. Acute chest syndrome is defined as a new pulmonary opacity in a symptomatic patient with sickle cell disease. In children, it occurs more commonly in the upper & middle lobes.
. Acute chest syndrome is defined as a new pulmonary opacity in a symptomatic patient with sickle cell disease. In children, it occurs more commonly in the upper & middle lobes.
AP radiograph shows common thoracic findings in sickle cell disease: Cardiomegaly, a left lower lobe opacity
, & a pleural effusion
. Note the avascular necrosis in the left humeral head
.
, & a pleural effusion . Note the avascular necrosis in the left humeral head .
AP radiograph shows multifocal right lung opacities
in this sickle cell disease patient with respiratory distress & fever, consistent with acute chest syndrome. Note the cardiomegaly.
in this sickle cell disease patient with respiratory distress & fever, consistent with acute chest syndrome. Note the cardiomegaly.
TERMINOLOGY
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