Gorham's disease
Gorham's disease is a rare congenital disorder characterized by proliferation of vascular channels that cause massive destruction of bone matrix. The disease was first described by Gorham et al. in 1954.
Etiology
The etiology of the disease remains unclear, but there is no evidence of underlying malignancy or infection.
Signs and symptoms
The presentation of the disease varies considerably, and a high index of suspicion is needed to reach an early diagnosis. Dull musculoskeletal pain and progressive weakness is typical, with a protracted but progressive development of symptoms. Involvement of the thoracic skeleton may cause mediastinal involvement resulting in pericardial or pleural effusions.
Treatment
Therapeutical measures mainly focus on limiting the progression of the disease, and medical treatment options include the following:
- Radiation therapy
- Bisphsphonates (Anti-osteoclastic effect)
- ALPHA-Interferon
Surgical removal of lesions and various reconstructive/supportive procedures as well as prosthesis are of value, however bone grafts tend to be of limited effect as they are often quickly resobed.
Additional treatment required for the correction of mediastinal complications include pleurodesis, pleurectomy, thoracic duct ligation. Template:WikiDoc Sources