Radiological features of Gorham's disease

To describe the key findings on plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) of Gorham's disease.

Eight children diagnosed with Gorham's disease between 1999 and 2009 were included. All imaging studies performed on each patient were reviewed with special attention to the extent of bone, soft tissue, and visceral involvement.

All patients had bone lesions at diagnosis, most commonly in the vertebrae. CT showed generalized osteopenia, multiple lytic lesions, and heterogeneous bone density. MRI demonstrated altered signal intensity in bone marrow that was hyperintense on T1 imaging. Seven patients had soft-tissue lymphangiomatous lesions adjacent to identified osseous lesions. Four patients had chylous pleural effusions: three with bilateral and one with unilateral involvement. The spleen was involved in six patients.

Splenic lesions and soft-tissue involvement are common in patients with Gorham's disease. The presence of extra-osseous lesions along with characteristic bone lesions on plain radiography may be pathognomonic of Gorham's disease.