Chylous Manifestations and Management of Gorham-Stout Syndrome

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.

Chylous Manifestations and Management of Gorham-Stout Syndrome / 대한흉부외과학회지

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.

Surgical Options for Malignant Mesothelioma: A Single-Center Experience

BACKGROUND: We investigated the surgical outcomes of patients who underwent therapeutic surgery for malignant pleural mesothelioma (MPM) at a single center. METHODS: A retrospective review of 21 patients who underwent therapeutic surgery for MPM from January 2001 to June 2015 was conducted to assess their outcomes. The patients' characteristics and postoperative course, including complications, mortality, overall survival, and recurrence-free survival, were analyzed. RESULTS: Of the 21 patients who underwent therapeutic surgery, 15 (71.4%) underwent extrapleural pneumonectomy, 2 pleurectomy (9.5%), and 4 excision (19.1 %). The median age was 57 years (range, 32–79 years) and 15 were men (71.4%). The mean hospital stay was 16 days (range, 1–63 days). Median survival was 14.3 months. The survival rate was 54.2%, 35.6%, and 21.3% at 1, 3, and 5 years, respectively. In patients' postoperative course, heart failure was a major complication, occurring in 3 patients (14.3%). The in-hospital mortality rate was 2 of 21 (9.5%) due to a case of severe pneumonia and a case of acute heart failure. CONCLUSION: A fair 5-year survival rate of 21.3% was observed after surgical treatment. Heart failure was a major complication in our cohort. Various surgical methods can be utilized with MPM, each with its own benefits, taking into consideration the severity of the disease and the comorbidities of the patient. Patients with local recurrence may be candidates for surgical intervention, with possible satisfying results.

Management of Coronary Sinus Ostial Atresia during a Staged Operation of a Functional Single Ventricle

Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

Aortic Root Reimplantation in a Patient Who Underwent an Arterial Switch Operation

Neo-aortic insufficiency associated with root enlargement following an arterial switch operation is a serious late complication. To achieve successful surgical correction of this condition, multiple factors should be considered, including the individual patient's anatomy, the challenging nature of the redo procedure, and the patient's young age. However, limited publications have described the use of valve-sparing techniques for the treatment of neo-aortic insufficiency associated with root enlargement following an arterial switch operation. Herein, we report our recent experience of a valve-sparing aortic root procedure with ascending aorta and hemiarch replacement despite the presence of a discrepancy in leaflet size and nearby severe adhesions.