J Korean Med Sci.  2002 Dec;17(6):826-829. 10.3346/jkms.2002.17.6.826.

Chylothorax in Gorham's Disease

• Lee WS
• Kim SH
• Kim IH
• Kim HK
• Lee KS
• Lee SY
• Heo DS
• Jang BS
• Bang YJ
• Kim NK

• KMID: 648796
• DOI: http://doi.org/10.3346/jkms.2002.17.6.826

Abstract

A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.

Keyword

Gorham's Disease; Osteolysis; Radiotherapy; Pleurodesis; Chylothorax

MeSH Terms

Adult
Chylothorax/complications/*pathology/radiotherapy
Female
Humans
Magnetic Resonance Imaging/methods
Osteolysis, Essential/complications/*pathology/*radiotherapy
Pleurodesis
Time Factors
Tomography, X-Ray Computed
Treatment Outcome