eMedicine Specialties > Radiology > Musculoskeletal

Hemophilia, Musculoskeletal Complications: Imaging

Author: Ray F Kilcoyne, MD, † Former Professor Emeritus, Department of Radiology, University of Colorado Health Sciences Center
Contributor Information and Disclosures

Updated: Mar 21, 2008

Radiography

Findings

In 1977, Arnold and Hilgartner published a classification scheme for staging hemophilic arthropathy that has been widely used.6 The radiographic progression of disease is divided into 5 stages (see the Table in Clinical Details, and see Images 15-16).

Degree of Confidence

The early changes of effusion and synovitis in hemophilic arthropathy are poorly seen on radiographs.

False Positives/Negatives

Early soft-tissue abnormalities are not well depicted on radiographs. Radiographic findings of other diseases may mimic hemophilia in a given joint.

Computed Tomography

Findings

Masses that develop in a patient with hemophilia can be evaluated by computed tomography (CT) scanning, which is good for defining pseudotumors, whether they occur in soft tissues, in cortical bone, or in the medullary cavity. Soft-tissue hemorrhage that causes neurovascular compromise can also be evaluated with this modality. In particular, pathology in bone, such as an osseous pseudotumor, is well seen with CT scanning.

Degree of Confidence

Soft-tissue changes are better seen with MRI than with CT.

Magnetic Resonance Imaging

Findings

Soft-tissue changes are depicted well on MRI, including joint effusion, hemarthrosis, synovitis, and hemosiderin deposition.

Degree of Confidence

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.

False Positives/Negatives

Effusion and synovitis may develop from causes other than hemophilia, such as trauma or infection. In patients with PVNS, hemosiderin deposition may look exactly like that which is caused by hemophilia in a given joint. JRA 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.2

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.

Ultrasonography

Findings

Soft tissues are seen well with ultrasonography. A mass that is suspected of being a joint effusion or a soft-tissue pseudotumor can be demonstrated quickly with this modality.

Degree of Confidence

If the acoustic window is adequate, the finding of a sonolucent mass on ultrasonography is highly suggestive of fluid. Mixed signal intensity is likely due to a soft-tissue mass, such as a hematoma.

False Positives/Negatives

  • The inability to place the ultrasound transducer over the area of interest precludes scanning.
  • Bone blocks the sound waves and limits the ability to see subchondral cysts and erosions.
  • Cartilage cannot be adequately differentiated from bone.

Nuclear Imaging

Findings

Bone scans are highly sensitive for detecting areas of increased osteoblastic activity; the scans are useful for surveying the entire skeleton for disease. Follow-up bone scans can be used to determine the effectiveness of a patient's treatment. Radioisotopes, such as phosphorus-32 (32 P), can be injected therapeutically into a joint to decrease the amount of bleeding/effusion.9

Degree of Confidence

Bone scans combine high sensitivity with low specificity. Therefore, a negative bone scan should exclude acute joint hemorrhage or synovitis.

False Positives/Negatives

The lack of specificity of bone scans makes it difficult to determine the exact cause of a positive bone scan. The findings are more helpful in the acute phase of the disease than at other times.

Angiography

Findings

Before the development of MRI, there was a place for angiography, especially in the evaluation of pseudotumors. Currently, angiography is not used in the evaluation of musculoskeletal complications of hemophilia.

More on Hemophilia, Musculoskeletal Complications

Overview: Hemophilia, Musculoskeletal Complications
Imaging: Hemophilia, Musculoskeletal Complications
Follow-up: Hemophilia, Musculoskeletal Complications
Multimedia: Hemophilia, Musculoskeletal Complications
References
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References

  1. Aronova-Tiuntseva Y, Herreid CF. Hemophilia: "The Royal Disease". National Center for Case Study Teaching in Science. Available at http://ublib.buffalo.edu/libraries/projects/cases/hemo.htm. Accessed August 30, 2007.

  2. Resnick D. Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia, Pa: WB Saunders Co; 2002.

  3. Roosendaal G, Lafeber FP. Blood-induced joint damage in hemophilia. Semin Thromb Hemost. Feb 2003;29(1):37-42. [Medline].

  4. Nuss R, Kilcoyne RF. Diagnosis by imaging of haemophilic joints. In: Rodriguez-Merchan EC, ed. The Haemophilic Joints: New Perspectives. Malden, England: Blackwell Publishing; 2003:24-9.

  5. Pettersson H, Ahlberg A, Nilsson IM. A radiologic classification of hemophilic arthropathy. Clin Orthop Relat Res. Jun 1980;149:153-9. [Medline].

  6. Arnold WD, Hilgartner MW. Hemophilic arthropathy. Current concepts of pathogenesis and management. J Bone Joint Surg Am. Apr 1977;59(3):287-305. [Medline]. [Full Text].

  7. National Heart, Lung, and Blood Institute. Hemophilia. NHLBI Diseases and Conditions Index. Available at http://www.nhlbi.nih.gov/health/dci/Diseases/hemophilia/hemophilia_all.html. Accessed August 30, 2007.

  8. Bernabeu-Taboada D, Martin-Hervas C. Sonography of haemophilic joints. In: Rodriguez-Merchan EC, ed. The Haemophilic Joints: New Perspectives. Malden, England: Blackwell Publishers; 2003:30-5.

  9. Nuss R, Kilcoyne RF, Geraghty S, et al. MRI findings in haemophilic joints treated with radiosynoviorthesis with development of an MRI scale of joint damage. Haemophilia. May 2000;6(3):162-9. [Medline].

  10. Doria AS, Babyn PS, Lundin B, et al, for the Expert MRI Working Group of the International Prophylaxis Study Group. Reliability and construct validity of the compatible MRI scoring system for evaluation of haemophilic knees and ankles of haemophilic children. Haemophilia. Sep 2006;12(5):503-13. [Medline].

  11. Jorge Filho D, Battistella LR, Lourenço C. Computerized pedobarography in the characterization of ankle-foot instabilities of haemophilic patients. Haemophilia. Mar 2006;12(2):140-6. [Medline].

  12. Kilcoyne RF, Lundin B, Pettersson H. Evolution of the imaging tests in hemophilia with emphasis on radiography and magnetic resonance imaging. Acta Radiol. Apr 2006;47(3):287-96. [Medline].

  13. Lundin B, Berntorp E, Pettersson H, et al. Gadolinium contrast agent is of limited value for magnetic resonance imaging assessment of synovial hypertrophy in hemophiliacs. Acta Radiol. Jun 2007;48(5):520-30. [Medline].

  14. Pergantou H, Matsinos G, Papadopoulos A, Platokouki H, Aronis S. Comparative study of validity of clinical, x-ray and magnetic resonance imaging scores in evaluation and management of haemophilic arthropathy in children. Haemophilia. May 2006;12(3):241-7. [Medline].

  15. Zukotynski K, Jarrin J, Babyn PS, et al. Sonography for assessment of haemophilic arthropathy in children: a systematic protocol. Haemophilia. May 2007;13(3):293-304. [Medline].

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Further Reading

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Keywords

hemophilic arthropathy, hemophilic soft-tissue bleeding, pseudotumor, hemophilia A, classic hemophilia, factor VIII deficiency, hemophilia B, Christmas disease, factor IX deficiency, bleeding disorder, Arnold-Hilgartner staging/classification, hemarthrosis

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Contributor Information and Disclosures

Author

Ray F Kilcoyne, MD, † Former Professor Emeritus, Department of Radiology, University of Colorado Health Sciences Center
Ray F Kilcoyne, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, International Skeletal Society, Radiological Society of North America, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Medical Editor

Amilcare Gentili, MD, Professor of Clinical Radiology, University of California at San Diego; Consulting Staff, Department of Radiology, Thornton Hospital; Chief of Radiology, San Diego VA Health Care System
Amilcare Gentili, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Javier Beltran, MD, Chair, Department of Radiology, Maimonides Medical Center
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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