Korean J Thorac Cardiovasc Surg 2019; 52(1): 44-46  https://doi.org/10.5090/kjtcs.2019.52.1.44
Chylous Manifestations and Management of Gorham-Stout Syndrome
Sungbin Cho, M.D.1, Seung Ri Kang, M.D.1, Beom Hee Lee, M.D.2, Sehoon Choi, M.D., Ph.D.1
1Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine, 2Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine
Corresponding author: Sehoon Choi, Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
(Tel) 82-2-3010-3904 (Fax) 82-2-3010-6966 (E-mail) choishn@gmail.com
Received: August 20, 2018; Revised: September 12, 2018; Accepted: September 13, 2018.; Published online: February 5, 2019.
© The Korean Journal of Thoracic and Cardiovascular Surgery. All rights reserved.

cc This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Gorham-Stout disease (GSD) was first described by Gorham and colleagues in 1954, but its precise mechanism and cause remain to be elucidated. In this condition, voluminous and potentially fatal chylous effusions into the thorax can occur. Herein, we describe a case of GSD in which the patient presented with massive pleural effusions and mottled osteolytic bone lesions. We performed multiple operations, including thoracic duct ligation using video-assisted thoracoscopic surgery and thoracotomic decortication, but these procedures did not succeed in preventing recurrent pleural effusion and chest wall lymphedema. After administering sirolimus (0.8 mg/m2, twice a day) and propranolol (40 mg, twice a day), the process of GSD in this patient has been controlled for more than 2 years.
Keywords: Gorham-Stout disease, Pleural effusion, Sirolimus, Chylothorax

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