Sickle cell disease

Case contributed by Dr Mostafa Mahmoud El Feky

Presentation

Bilateral hip pain, back pain and pelvic pain.

Patient Data

Age: 30 years
Gender: Female
X-ray

X-Ray pelvis

Cortical irregularities and heterogeneous texture of both femoral heads.

MRI

Mri hip joint

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.

CT

CT abdomen

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 heterogenous marrow is in keeping with osteonecrosis.

MRI

MRI lumbar spine

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 ischaemia and infarction, as well as haemolytic anaemia. There is multiple forms of skeletal involvement in this case such as H-shaped vertebrae, osteonecrosis of pelvic bones and bilateral AVN 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 anaemia, and results from microvascular endplate infarction. Hepatosplenomegaly is also noted which occurs in some haematological disorders and chronic anaemias.

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Case information

rID: 55022
Case created: 14th Aug 2017
Last edited: 23rd Aug 2017
Inclusion in quiz mode: Included

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