A patient with hemophilia A or B (hereditary Factor 8 or 9 deficiency) who experiences intra-articular hemorrhage may develop a disabling arthropathy. Pettersson et al developed a radiographic score for affected joints. The presence of effective prophylaxis has reduced the severity of this complication.
Radiologic findings:
(1) osteoporosis
(2) enlargement of epiphysis
(3) irregularity of subchondral surface
(4) narrowing of the joint space
(5) subchondral cyst formation
(6) erosion of joint margins
(7) incongruence between joint surfaces
(8) deformity
(9) ankylosis
|
Radiologic Change |
Finding |
Points |
|
osteoporosis |
absent |
0 |
|
|
present |
1 |
|
enlargement of epiphysis |
absent |
0 |
|
|
present |
1 |
|
irregularity of subchondral surface |
absent |
0 |
|
|
slight |
1 |
|
|
pronounced |
2 |
|
narrowing of joint space |
absent |
0 |
|
|
< 50% |
1 |
|
|
> 50% |
2 |
|
subchondral cyst formation |
absent |
0 |
|
|
1 cyst |
1 |
|
|
> 1 cyst |
2 |
|
erosion at joint margins |
absent |
0 |
|
|
present |
1 |
|
incongruence between joint surfaces |
absent |
0 |
|
|
slight |
1 |
|
|
pronounced |
2 |
|
deformity (angulation and/or displacement of articulating bones) |
absent |
0 |
|
|
slight |
1 |
|
|
pronounced |
2 |
|
ankylosis |
absent |
0 |
|
|
present |
13 |
from Table 2, page 154, Pettersson (1980)
where:
• Narrowing of joint space does not cover situation where the narrowing = 50%. In the implementation I have set this to 1 point.
• Scoring of ankylosis is given on page 154, top of second column.
radiologic joint score =
= MAX(points for ankylosis, SUM(points for first 8 parameters))
Interpretation:
• minimum score for a joint: 0
• maximum score for a joint: 13
• If multiple joints are examined, then the maximum score is the number of joints examined times 13 points. Funk et al examined 6 joints (both knees, both ankles, both elbows) giving a total of 78 points per patient (6 * 13).
|
Joint Score |
History of Intra-articular Bleeding |
Reduced Joint Function |
|
0 |
present in some patients |
none |
|
2 |
present hardly ever |
none or slight |
|
3 or 4 |
always present |
always present |
|
> 4 |
always present |
rather good correlation between increased score and reduced function |
from Table 3, page 158, Pettersson (1980)
Limitations:
• Gilbert (1993, page 6) indicates that there may be little correlation between the clinical examination and radiologic score, and that treatment decisions should not be based solely on the X-ray examination.
Specialty: Surgery, orthopedic
