Chylothorax in Gorham's disease.
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.
Lee
Won Sup
WS
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea. heo1013@plaza.snu.ac.kr
Kim
Sung Han
SH
Kim
Inho
I
Kim
Hark Kyun
HK
Lee
Keun Seok
KS
Lee
Sang Yoon
SY
Heo
Dae Seog
DS
Jang
Bong-Soon
BS
Bang
Yung-Jue
YJ
Kim
Noe Kyeong
NK
D000328:Adult; D002916:Chylothorax / Q000150:complications / Q000473:pathology* / Q000532:radiotherapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D010015:Osteolysis, Essential / Q000150:complications / Q000473:pathology* / Q000532:radiotherapy*; D018700:Pleurodesis; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome