Answer
In 1977, Arnold and Hilgartner published a classification scheme for staging hemophilic arthropathy that has been widely used. [19, 6] 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. This image depicts stage III joint disease, as determined by the Arnold-Hilgartner classification.
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 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-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 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 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 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 with hemophilia. This image shows the development of osteonecrosis (arrow) in the talar dome.
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 [19] (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 |
-
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-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.
-
Magnetic resonance image from a patient with hemophilia. This image shows dark, synovial masses that erode the cartilage and produce subchondral cysts (arrows).
-
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 previous image. This image shows a large hemarthrosis that is distending the suprapatellar recess. Image courtesy of Javier Beltran, MD.
-
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.
-
Radiograph in a patient with hemophilia. This image depicts a pseudotumor that is deforming the cortex of the femur (arrow). Other ossified masses in the soft tissues (arrowheads) are probably soft-tissue pseudotumors.
-
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).
-
Computed tomography scan of the pelvis in a patient with hemophilia. This image demonstrates a giant pseudotumor (a large expansile lytic lesion that involves the right iliac bone and extends into the sacrum, with inhomogeneous internal attenuation). Image courtesy of Javier Beltran, MD.
-
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.
-
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 with hemophilia. This image shows the development of osteonecrosis (arrow) in the talar dome.
-
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.
-
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.
-
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. This image depicts stage IV joint disease, as determined by the Arnold-Hilgartner classification.
-
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.
Tables
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 |
