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Radiological features of Gorham's disease.
R Kotecha1, L Mascarenhas, H A Jackson
1Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA.
Clinical Radiology
|March 20, 2012
Summary
Gorham's disease imaging reveals common bone lesions, often in vertebrae. Extra-osseous findings like soft tissue and splenic involvement are frequent, aiding diagnosis.
Area of Science:
- Radiology
- Pediatric Imaging
- Oncology
Background:
- Gorham's disease, also known as vanishing bone disease, is a rare disorder characterized by progressive bone loss.
- Accurate diagnosis relies heavily on characteristic imaging findings.
Purpose of the Study:
- To outline the key imaging features of Gorham's disease across plain radiography, CT, and MRI.
- To correlate imaging findings with clinical presentation and disease extent.
Main Methods:
- Retrospective review of imaging studies (radiography, CT, MRI) for eight pediatric patients diagnosed with Gorham's disease.
- Detailed assessment of bone, soft tissue, and visceral involvement on all imaging modalities.
Main Results:
- Vertebral bone lesions were most common at diagnosis.
- CT revealed generalized osteopenia, lytic lesions, and heterogeneous bone density.
- MRI showed hyperintense T1 signal in bone marrow, with frequent soft-tissue lymphangiomatous lesions, chylous pleural effusions, and splenic involvement.
Conclusions:
- Splenic and soft-tissue involvement are common in Gorham's disease.
- Combined osseous and extra-osseous findings on imaging are highly suggestive of the disease.
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