Presentation
Patient from Nigeria with a history of HgbSS disease presented with chest pain for three hours. Rule out acute chest syndrome.
Patient Data
1. Mild diffuse cardiomegaly with mild central pulmonary vascular congestion in this patient with sickle cell disease.
2. Mild diffuse bilateral bronchial wall thickening may be due to reactive airway disease or a chronic finding. Minimal fibro-atelectasis in the right mid lung zone. No focal pneumonia, effusion or pneumothorax. Lungs otherwise clear.
3. Mild anterior wedging and biconcavity of some thoracic vertebral bodies, mild diffuse endplate sclerosis and H shaped configuration of the lower-thoracic vertebral bodies on frontal projection.
4. Diffuse splenomegaly in the upper abdomen best evident on the lateral view.
Case Discussion
Sickle cell anemia is a red blood cell disease with often severe chronic conditions if the patient inherits both parents' HgbS gene (rather than another gene configuration). On imaging, signs of sickle cell disease may be extramedullary hematopoiesis, osteonecrosis, myonecrosis and osteomyelitis 1. Other chronic manifestations of sickle cell disease are lung fibrosis, pulmonary hypertension, hyperkinetic circulation related to severe anemia, and thoracic skeletal abnormalities; the latter include H-shaped vertebrae, biconcave endplates due to bone softening, centrally-notched H-shaped vertebrae typical of infarction, and rib abnormalities due to infarction 2.
This case was submitted with supervision and input from:
Soni C. Chawla, M.D.
Health Sciences Clinical Professor,
Department of Radiological Sciences,
David Geffen School of Medicine at UCLA.
Attending Pediatric Radiologist,
Olive View - UCLA Medical Center.
Unable to process the form. Check for errors and try again.