Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
December 2022
Next Case of the Month Coming January 10...
Bone Infarction and Epidural Hemorrhage in Sickle Cell Disease
- Background:
- Sickle cell disease is a common inherited autosomal recessive disease that is characterized by abnormally shaped (sickle-shaped) red blood cells.
- The gene that causes sickle cell disease is more prevalent in populations of African origin; approximately 8% of African Americans and 40% of members of some African tribes carry the gene.
- It can involve virtually any organ system.
- The clinical manifestations of sickle cell disease vary and are classified as vaso-occlusion, chronic anemia, and infection.
- Clinical Presentation:
- Among the musculoskeletal manifestations of sickle cell disease are thrombosis of bone vessels and bone infarction. This process may occur anywhere in the skeleton and results directly from the sickling of red blood cells in the bone marrow, which causes stasis of blood and sequestration of cells.
- Infarcts typically occur in the medullary cavities and epiphyses and often are the source of painful bone crises.
- Subperiosteal hemorrhage is another known complication of sickle cell disease that may be implicated in the formation of the epidural hematoma seen in this case.
- Key Diagnostic Features:
- Bone infarction
- Plain radiograph: Classic description is of medullary lesion with central lucency surrounded by sclerosis with a serpiginous border.
- CT: Similar findings to plain film
- MRI:
- T1: Serpiginous peripheral low signal due to granulation tissue/sclerosis; peripheral rim may enhance postgadolinium; central signal is that of marrow.
- T2: Acute infarct may show ill-defined nonspecific area of high signal. Double-line sign: Hyperintense inner ring of granulation tissue and a hypointense outer ring of sclerosis; central signal is usually that of marrow.
- Differential Diagnoses:
- Enchondroma
- Healing nonossifying fibroma
- Normal red marrow
- Marrow tumor
- Treatment
- Management of vaso-occlusive crises includes oxygen, hydration, and analgesia.
- Anemia is usually well tolerated; however, blood transfusions may be necessary.
- Sickle cell anemia causes significant mortality and morbidity, with a decrease of 25–30 years in the average life expectancy.
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