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

Updated: Nov 14, 2016
  • Author: Lars J Grimm, MD, MHS; Chief Editor: Felix S Chew, MD, MBA, MEd  more...
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Answer

Answer

In 1977, Arnold and Hilgartner published a classification scheme for staging hemophilic arthropathy that has been widely used. [8, 16] The radiographic progression of disease is divided into 5 stages (see the Table in Introduction, as well as the images below).

Radiograph of the leg in a patient with hemophilia Radiograph of the leg in a patient with hemophilia. This image depicts stage III joint disease, as determined by the Arnold-Hilgartner classification.
Radiograph of the leg in a patient with hemophilia Radiograph of the leg in a patient with hemophilia. This image depicts stage IV joint disease, as determined by the Arnold-Hilgartner classification.

Other radiographic images are shown below

Knee radiograph in a 37-year-old man with moderate Knee radiograph in a 37-year-old man with moderate factor IX hemophilia. This image shows bony excrescence on the lateral side of the femur is a hemophilic pseudotumor.
Radiograph of the lower extremity of the 3-year-ol Radiograph of the lower extremity of the 3-year-old daughter of the patient in the previous image. This image shows talar tilt. The girl not only inherited 1 diseased X chromosome with mild factor IX hemophilia from her father, but she also has Turner (XO) syndrome. The child's only X chromosome had the hemophilia gene.
Anteroposterior radiograph from a patient with hem Anteroposterior radiograph from a patient with hemophilia. This image demonstrates hemarthrosis with hemosiderin deposition. Note the irregularity of the articular surfaces and the presence of subchondral sclerosis with cysts. Image courtesy of Javier Beltran, MD.
Lateral radiograph from the same patient as in the Lateral radiograph from the same patient as in the previous image. This image shows a large hemarthrosis that is distending the suprapatellar recess. Image courtesy of Javier Beltran, MD.
Lateral elbow radiograph in a patient with hemophi Lateral elbow radiograph in a patient with hemophilia. This image shows an opaque joint effusion due to the presence of iron in the synovium (arrows).
Radiograph of the ankle in a 20-year-old patient w Radiograph of the ankle in a 20-year-old patient with hemophilia. This image shows the development of osteonecrosis (arrow) in the talar dome.
Radiograph of the shoulder of a 35-year-old man wi Radiograph of the shoulder of a 35-year-old man with hemophilia. This image shows advanced degenerative joint disease. The infiltrate in the lung is due to a fungal infection as a complication of the patient's positive human immunodeficiency virus (HIV) status.

The early changes of effusion and synovitis in hemophilic arthropathy are poorly seen on radiographs. Early soft-tissue abnormalities are not well depicted on radiographs. Radiographic findings of other diseases may mimic hemophilia in a given joint.

Table. Arnold-Hilgartner classification [8] (Open Table in a new window)

Stage

Findings

0

Normal joint

I

No skeletal abnormalities, soft-tissue swelling is present

II

Osteoporosis and overgrowth of the epiphysis, no cysts, no narrowing of the cartilage space

III

Early subchondral bone cysts, squaring of the patella, widened notch of the distal femur or humerus, preservation of the cartilage space

IV

Findings of stage III, but more advanced; narrowed cartilage space

V

Fibrous joint contracture, loss of the joint cartilage space, extensive enlargement of the epiphysis, substantial disorganization of the joint


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