A lethal form of Gorham disease associated with extensive musculoskeletal pneumatosis: case report and review of the literature

Skeletal Radiol. 2008 Nov;37(11):1041-6. doi: 10.1007/s00256-008-0525-5. Epub 2008 Jul 16.


We report here the imaging findings of a rare case of a lethal form of Gorham disease in a young female patient. Multimodality imaging findings over 13 year-follow-up demonstrated progressive wide spread skeletal and soft tissue abnormalities with permeative osteolysis, pathological fractures and severe skeletal deformities. Unusual extensive osseous and soft tissue pneumatosis was illustrated on cross-sectional studies. The progressive nature of this form of Gorham disease and the subsequent complications eventually culminated in patient's death.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Chylothorax / etiology
  • Diagnosis, Differential
  • Disease Progression
  • Emphysema / etiology
  • Fatal Outcome
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Osteolysis, Essential / complications
  • Osteolysis, Essential / diagnosis*
  • Tomography, X-Ray Computed