Question
The Xray of a 17 Year Old Male is shown. Most Probable Diagnosis
A. Scurvy
B. Rickets
C. Rheumatoid Arthritis
D. Hemophilic Arthopathy
Show Answer
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Correct Answer » D Explanation |
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Ans:D. Hemophilic Arthropathy.
Haemophilic arthropathy – knee Periarticular osteoporosis and epiphyseal enlargement are seen at left knee radiograph with widened intercondylar notch.
There is also evidence of joint effusion in favour of hemarthrosis.
Haemophilic arthropathy
- It refers to permanent joint disease occurring in haemophilia sufferers as a long-term consequence of repeated haemarthrosis. Around 50% of patients with haemophilia will develop a severe arthropathy.
- Epidemiology
- Haemophilia is an x-linked recessive disease affecting males. Haemarthroses may be spontaneous or result from minor trauma and typically first occurs before the age of two and continues to occur into adolescence. It is usual for the same joint to be repeatedly involved. In adulthood, haemarthroses are uncommon. However, proliferative chronically inflamed synovium results in the development of haemophilic arthropathy.
- Pathology
- There is synovial hyperplasia, chronic inflammation, fibrosis, and haemosiderosis. The synovium mass erodes cartilage and subchondral bone leading to subarticular cyst formation
- Location
- Haemophilic arthropathy is often monoarticular or oligoarticular. Large joints are most commonly involved in the following order of frequency :
- knee
- elbow
- ankle
- hip
- shoulder
- Haemophilic arthropathy is often monoarticular or oligoarticular. Large joints are most commonly involved in the following order of frequency :
- Radiographic features
- Plain radiograph
- joint effusion seen in the setting of haemarthrosis
- periarticular osteoporosis: from hyperaemia
- epiphyseal enlargement with associated gracile diaphysis: from hyperaemia (appearances can be similar to juvenile rheumatoid arthritis and paralysis)
- secondary degenerative disease: symmetrical loss of joint cartilage involving all compartments equally with periarticular erosions and subchondral cysts, osteophytes and sclerosis
- widened intercondylar notch
- squared inferior margin of the patella
- bulbous femoral condyles
- flattened condylar surfaces
- Plain radiograph
- Treatment and prognosis
- Early factor VIII or IX replacement may prevent or delay joint destruction. Radiosynoviorthesis has been shown to be effective in reducing bleeding and effusion in selected cases. Surgical arthrodesis 6 or joint replacement 7 can be an effective treatment for the end-stage disease.
