Gorham's disease with chest wall involvement : a case report and a review of the literature

Abstract EN

Introduction : Gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones.

Diagnosis of Gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial.

No specific treatment is available.

Management options include surgery, radiation therapy and medical therapy.

We aimed to present the first case of Gorham’s disease with chest wall involvement in Iran.

By review of the literature we discussed important issues of this rare disease including clinical findings, diagnosis and treatment options.

Case Presentation : We present a 48-year-old man with a history of dyspnea following a blunt chest trauma who was admitted to our clinic several times due to reaccumulation of pleural fluid and chylothorax.

Gorham's disease was finally established according to clinical manifestations and radiological findings including massive osteolysis in his left ribs and also histological examination.

Discussion : According to review of the literature and considering all treatment modalities the patients was successfully treated with a combination of radiotherapy, pamidronate and thalidomide.

We suggest that this disease should be considered among differential diagnoses of patients with chest pain, pleural effusion and / or chylothorax with an unknown reason and more importantly history of chest trauma.

In suspected cases, it is essential to examine biopsy specimens of the bone adjacent to the inflammated tissues in order to confirm diagnosis.