CASE 71
By: Dr. Amna Kashgari MD,SSC-Rad
HISTORY: A 7-year-old girl with bone pain.
What are your findings?
What is your diagnosis?
Mention three musculoskeletal complication of this entity.
FINDINGS:
Frontal radiograph of the chest demonstrates central cupping of the end-plate of the thoracic vertebral bodies (H- shaped vertebrae).
DIAGNOSIS:
Sickle cell anemia.
Complication:
Bone infarction.
Avascular necrosis of the femoral head.
Osteomyelitis.
PEARLS AND DISCUSSION:
Sickle cell anemia is the most common hemoglobin variant in the world (HbS) (Autosomal recessive). The abnormal (sickled) red blood cells (RBCs) removed from the circulation and destroyed at increased rates, leading to chronic anemia.
The sickled RBCs cause vascular occlusion, which leads to tissue ischemia and infarction.
Clinical presentation usually presents after 6 months of age. The SCA is characterized by heterogeneity in clinical and hematologic severity:
Hematologic: Chronic anemia, acute splenic sequestration, aplastic crisis, and acute vasoclusive episodes.
Gastrointestinal: Pigmented gallstones.
Neurologic: Transient ischemic attacks, infarctive stroke, intracerebral hemorrhage and retinopathy.
Cardiac: Acute myocardial infarction and high output failure.
Pulmonary: Acute chest syndrome (including pneumonia and infarction due to in situ thrombosis).
Renal: Papillary necrosis and focal glomerulosclerosis.
Infectious: Bacteremia, osteomyelitis, meningitis.
Bone: Marrow hyperplasia, Vertebral sclerosis due to many small infarcts beginning early in life, central cupping of the end-plate (H- shaped vertebrae) and vertebral collapse.
Other MSK manifestation: Dactylitis also known as hand-foot syndrome (Most often occurs between 6 and 18 months of age) and generalized bone infarction.
Complications:
Fractures – Resulting from full thickness cortical necrosis.
Osteomyelitis – Necrotic bone marrow fertile site for secondary infection (most common organism is Salmonella).
Growth disturbances: Usually secondary to deficiency in the nutrient artery circulation to the central region of the metaphyseal side of the growth plate.
FURTHER READING:
Gael J. Lonergan, David B. Cline, MAJ et al. From the Archives of the AFIP:Sickle Cell Anemia. RadioGraphics 2001; 21:971–994.
Vivian C. Ejindu, Andrew L. Hine et al. Musculoskeletal Manifestations of Sickle Cell Disease. RadioGraphics 2007; 27:1005–1021.
