Gorham's disease

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.

Contents

Synonyms

Synonyms include:[1]

  • Disappearing Bone Disease
  • Essential Osteolysis
  • Gorham's Syndrome
  • Gorham-Stout Syndrome
  • Idiopathic Massive Osteolysis
  • Massive Gorham Osteolysis
  • Massive Osteolysis
  • Morbus Gorham-Stout Disease
  • Progressive Massive Osteolysis
  • Vanishing Bone Disease
  • Phantom Bone Disease

Etiology

The etiology of the disease remains unclear.

Signs and symptoms

Gorham's disease.jpg

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.

Gorham's disease may cause pathologic fractures, and acute or chronic pain. In children it may cause bone deformity. When detected early, radiographic evidence of decreased bone mass can be found.

Involvement of the thoracic skeleton may cause mediastinal involvement resulting in pericardial or pleural effusions. This may be due to the patient suffering from a combination of lymphangiomatosis (LYMF) and Gorham's disease.

Treatment

Therapeutical measures mainly focus on limiting the progression of the disease, and medical treatment options include the following:

  1. Radiation therapy
  2. Bisphosphonates (anti-osteoclastic effect)
  3. 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 reabsorbed.

Additional treatment required for the correction of mediastinal complications include pleurodesis, pleurectomy, thoracic duct ligation.

Although the above treatments have been tried in various cases, they have not had consistent success.

External links

References

  1. ^ "NORD - National Organization for Rare Disorders, Inc.". http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Gorham%27s%20Disease. Retrieved 2008-04-02. 

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