What is the role of MRI in the workup of hemophilia?

Updated: Sep 27, 2019
  • Author: Lars J Grimm, MD, MHS; Chief Editor: Felix S Chew, MD, MBA, MEd  more...
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Answer

Soft-tissue changes are depicted well on MRI, including joint effusion, hemarthrosis, synovitis, and hemosiderin deposition (see the images below).

Magnetic resonance image from a patient with hemop Magnetic resonance image from a patient with hemophilia. This image shows dark, synovial masses that erode the cartilage and produce subchondral cysts (arrows).
Axial gradient-echo magnetic resonance image from Axial gradient-echo magnetic resonance image from a patient with hemophilia. This image demonstrates hemosiderin deposition in the synovium as hypointense material that outlines the joint capsule. Image courtesy of Javier Beltran, MD.
Sagittal magnetic resonance image of the ankle joi Sagittal magnetic resonance image of the ankle joint in a patient with hemophilia. This image shows extension of abnormal joint fluid from the ankle joint into the subtalar joint. Note the dark hemosiderin posterior to the subtalar fluid (arrow).
Axial T2-weighted magnetic resonance image of the Axial T2-weighted magnetic resonance image of the pelvis. This image demonstrates a large pseudotumor of the pelvis with sacral extension. Image courtesy of Javier Beltran, MD.
Sagittal T2-weighted magnetic resonance image of t Sagittal T2-weighted magnetic resonance image of the lower extremity in a patient with hemophilia. This image demonstrates hemosiderin-laden synovium with low signal intensity that outlines the capsule of the tibiotalar joint. Image courtesy of Javier Beltran, MD.
Coronal T2-weighted magnetic resonance image of th Coronal T2-weighted magnetic resonance image of the knee in a patient with hemophilia. This image demonstrates pseudotumor of the knee, a lytic lesion in the lateral femoral condyle, as well as the characteristic manifestations of hemosiderin deposition in the knee joint and the subchondral cystic changes in the medial femoral condyle. Image courtesy of Javier Beltran, MD.

MRI offers excellent soft-tissue contrast resolution and spatial resolution. Special techniques have been developed and are undergoing development to improve the visualization of articular cartilage.

Effusion and synovitis may develop from causes other than hemophilia, such as trauma or infection. In patients with pigmented villonodular synovitis, hemosiderin deposition may look exactly like that which is caused by hemophilia in a given joint. Juvenile rheumatoid arthritis can cause knee deformity in a way that is similar to that in hemophilia, and bleeding may cause ectopic ossification in the soft tissues that looks like posttraumatic myositis ossificans.

Masses are especially common in the pelvis. Starker described these masses as pseudotumors in 1918; they are seen in 2% of patients with hemophilia. [18]

The 3 forms of pseudotumor are intraosseous, subperiosteal (or cortical), and soft tissue. The intraosseous form is most common in the femur, pelvic bones, tibia, and hand bones; the lesions can be variably sized. The pseudotumor is usually well demarcated, but it may also be bubbly and destructive. The lesion may simulate malignancy such as that from Ewing sarcoma, metastasis, or infection because of the pseudotumor's aggressive appearance. The subperiosteal type leads to cortical atrophy, subperiosteal new-bone formation, and soft-tissue extension. This is seen most commonly in the fibula. The soft-tissue form of the mass is surrounded by a fibrous capsule and may cause deformity of the adjacent bone.

Regarding other findings, calcification in hemosiderin may simulate other calcified masses on MRIs. Chondrocalcinosis occurs more commonly in calcium pyrophosphate deposition disease or primary hyperparathyroidism than in hemophilia. Septic arthritis can develop in a child with hemophilia; this may be a problem in early diagnosis. Contracture of soft tissues around joints may cause impingement on blood vessels or nerves.


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