Sickle Cell Disease
Intermittent left flank pain as a child • Xray of the Week
What is the diagnosis?
Figure 1. A. Sagittal CT of the chest. B.and C. Axial CT of chest and abdomen.
Figure 2.
A. Sagittal CT of chest. H-shaped vertebrae due to endplate depressions of the vertebral bodies (red arrows) caused by central growth plate infarction. Sclerosis in the spine and sternum (blue arrow) due to medullary bone infarcts.
B. Axial CT of chest. Cardiomegaly (yellow arrows) due to anemia.
C. Axial CT of abdomen. Densely calcified small spleen (green arrows) indicating autospenectomy.
Discussion
Sickle cell disease (SCD) a hereditary (autosomal recessive) condition common in people of African descent which causes a hemoglobinopathy, resulting in anemia and ischemia/infarction of multiple organs. Up to 8% of the African population is homozygous for sickle cell as it confers a resistance to malaria. SCD often presents during sepsis or dehydration as a painful vaso-occlusive crisis such as bone,chest or abdominal pain depending on the affected organ.
Musculoskeletal manifestations of SCD are due to chronic anemia resulting in extramedullary hematopoiesis, vaso-occlusive crises resulting in bone infarcts, and much less commonly osteomyelitis. Bone infarcts are initially radiolucent on CT, then progress to regions increased attenuation as as fibrosis and sclerosis replace the infarcted bone. As in this case, vertebral body infarcts are seen as a central, squared off endplate depression referred to as the Lincoln log or H-shaped vertebra. (Fig. 1A) Pathognomonic for SCD, but seen in only 10% of cases, this deformity is caused by central growth plate infarction. Anemia causes extramedullary hematopoiesis and leads to cardiomegaly. (Fig. 1B) Splenic infarction is very common such that by age 5 close to 95% of children with SCD have functional autosplenectomy. As seen here, this is manifested by as small densely calcified spleen. (Fig. 1C)
Management of vaso-occlusive crises is supportive with oxygen, hydration and analgesia. Treatment with hydroxyurea tends to lessen the severity of vaso-occlusive crises. Bone marrow transplantation can be curative.
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Kevin M. Rice, MD is president of Global Radiology CME and serves as the Chief of Staff of Valley Presbyterian Hospital in Los Angeles, California and is a radiologist with Renaissance Imaging Medical Associates. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. Dr. Rice co-founded Global Radiology CME with Natalie Rice to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field. In 2016, Dr. Rice was nominated and became a semifinalist for a "Minnie" award for the Most Effective Radiology Educator.
Follow Dr. Rice on Twitter @KevinRiceMD
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