Sickle cell disease

Case contributed by Dr Mostafa El-Feky

Presentation

Bilateral hip pain, back pain and pelvic pain.

Patient Data

Age: 30 years
Gender: Female

X-Ray pelvis

x_ray

Cortical irregularities and heterogeneous texture of both femoral heads.

Hip joints

mri

Cortical irregularity of both femoral heads. Altered marrow texture with geographic areas of hypointensity on T1WI and hyperintensity at T2WI, more affecting the right femoral head with extension to the neck and visualized proximal femoral shaft marrow (bilateral avascular necrosis).

Mild bilateral hip joint effusion.

Altered marrow texture with heterogeneous appearance of the bone marrow of the pelvic bones with multiple marrow lesions extending to the bone cortex is suggestive of osteonecrosis.

Abdomen

ct

Mild hepatosplenomegaly.

Central depression of the vertebral bodies giving the appearance of H-shaped vertebrae.

Diffuse diminished bone density with prominent trabecular pattern of the spine and pelvic bones.

Cortical irregularities of the pelvic bones with multiple cortical defects associated with underlying multiple osteolytic bony lesions and heterogeneous marrow are in keeping with osteonecrosis.

Lumbar spine

mri

Central depression of the vertebral bodies giving the appearance of H-shaped vertebrae.

Case Discussion

Sickle cell disease is an autosomal recessive condition that manifests as multisystem ischemia and infarction, as well as chronic hemolytic anemia.

There are multiple forms of skeletal involvement in this case: H-shaped vertebrae, osteonecrosis of pelvic bones and bilateral avascular necrosis of the femoral heads

H-shaped vertebrae (codfish) are a characteristic finding of sharply delimited central endplate depression, seen in approximately 10% of patients with sickle-cell anemia, and results from microvascular endplate infarction.

Hepatosplenomegaly is also noted, which seen in some hematological disorders and chronic anemias.

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