Journal of Chest Surgery

  • 제52권1호
  • /
  • Pages.44-46
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  • 2019
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  • 2765-1606(pISSN)
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  • 2765-1614(eISSN)
대한심장혈관흉부외과학회 (The Korean Society for Thoracic and Cardiovascular Surgery)
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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)
  • 투고 : 2018.08.20
  • 심사 : 2018.09.13
  • 발행 : 2019.02.05
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초록

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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참고문헌

  1. Tie ML, Poland GA, Rosenow EC 3rd. Chylothorax in Gorham's syndrome: a common complication of a rare disease. Chest 1994;105:208-13. https://doi.org/10.1378/chest.105.1.208
  2. Wu JK, Hooper ED, Laifer-Narin SL, et al. Initial experience with propranolol treatment of lymphatic anomalies: a case series. Pediatrics 2016;138:e20154545. https://doi.org/10.1542/peds.2015-4545
  3. Gondivkar SM, Gadbail AR. Gorham-Stout syndrome: a rare clinical entity and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e41-8. https://doi.org/10.1016/j.tripleo.2009.08.043
  4. Kiran DN, Anupama A. Vanishing bone disease: a review. J Oral Maxillofac Surg 2011;69:199-203. https://doi.org/10.1016/j.joms.2010.05.088
  5. Jayaprakash B, Prajeesh B, Nair DS. Gorham's disease. J Assoc Physicians India 2013;61:941-3.
  6. Wang S, Lu J, You Q, Huang H, Chen Y, Liu K. The mTOR/AP-1/VEGF signaling pathway regulates vascular endothelial cell growth. Oncotarget 2016;7:53269-76. https://doi.org/10.18632/oncotarget.10756
  7. Cramer SL, Wei S, Merrow AC, Pressey JG. Gorham-Stout disease successfully treated with sirolimus and zoledronic acid therapy. J Pediatr Hematol Oncol 2016;38:e129-32. https://doi.org/10.1097/MPH.0000000000000514

피인용 문헌

  1. Update June 2019 vol.17, pp.3, 2019, https://doi.org/10.1089/lrb.2019.29065.fb
  2. A Large Skull Defect Due to Gorham-Stout Disease: Case Report and Literature Review on Pathogenesis, Diagnosis, and Treatment vol.11, 2019, https://doi.org/10.3389/fendo.2020.00037
  3. Gorham-Stout disease successfully treated with sirolimus (rapamycin): a case report and review of the literature vol.21, pp.1, 2020, https://doi.org/10.1186/s12891-020-03540-7