Age / Sex : 15 / M

Chief complaint: dyspnea

What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case.

(답안은 한 개만 보내주시기 바랍니다)

Courtesy: 최수정 (Soo Jung Choi ), 강릉 아산병원 (Kangneung Asan Hospital)

Gorham’s disease

Discussion

Findings: MR imagings show marrow signal change with T1 low and T2 high signal intensities. Those lesions are strongly enhanced after Gd-enhancement. Axial enhanced MR images show that osseous lesion is extended to the adjacent soft tissue and left side epidural vascular structure is engorged. Chest CT shows massive pleural fluid collection, and it turned out to be chylothorax.

Differential Diagnosis: Gorham’s disease, aggressive hemangioma, Tuberculosis, metastasis.

Diagnosis: Gorham’s disease

Discussion: Gorham’s disease is a rare disorder characterized by proliferation of vascular channels resulting in destruction and resorption of osseous matrix. Chylous pericardial and pleural effusions may occur due to mediastinal extension of the disease process from the involved vertebra, scapula, rib or sternum and can be life threatening. MR findings of Gorham’s disease are nonspecific and variable depending on the degree of neovascular progression and fibrosis, but usually hyper-intensity on T2WI and well enhanced like the pattern of hemangiomatous and/or lymphangiomatous tissue. In a reported case of Gorham’s disease of the radius, MRI showed hyper-intensity on T2WI with small areas of flow-void and prominent vessels suggesting a vascular structure like the present case.

References:

1. Yoo SY, Hong SH, Chung HW, Choi JA, Kim CJ, Kang HS. MRI of Gorham's disease: findings in two cases. Skeletal Radiol. 2002 May;31(5):301-306.

2. Dipak V. Patel, MD, MSc Orth, MS Orth, FCPS Orth. Gorham’s Disease or Massive Osteolysis. Clin Med Res. May 2005; 3(2): 65–74.