[Spinal localization of Gorham's syndrome. Case report]

The authors report a case of massive osteolysis of the spine in an eighteen years old boy presenting a T9 collapse without neurological symptoms associated to a right chylothorax which disappeared after 5 days of drainage. MRI showed an increased signal on T1 and T2 weighted sequences. An orthopaedic treatment with a cast was decided as a first attempt. Because on increasing pain and kyphosis, surgery was considered, with posterior segmental fixation with Hartshill rectangle from T4 to L5. The result was satisfactory after 6 months of follow-UP. The vanished bone disease is a rare affection of unknown aetiology. 132 cases were published, 20 with spinal localization. The prognosis is uncertain with a mortality of 16 per cent. The treatment of bony lesions is difficult. Spinal localizations are best treated by segmental fixation extended on normal vertebrae.