Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Chylous Manifestations and Management of Gorham-Stout Syndrome

  • Cho, Sungbin (Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kang, Seung Ri (Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Beom Hee (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Choi, Sehoon (Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2018.08.20
  • Accepted : 2018.09.13
  • Published : 2019.02.05
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Abstract

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 ($0.8mg/m^2$, 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.

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References

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