Chylotamponade

Abstract

Gorham-Stout syndrome is a rare disorder that leads to extensive lymphangiomatosis in bones with resorption of adjacent bone matrix. As a result, the disease is also known as massive osteolysis or “vanishing bone disease.” Although the disease primarily occurs in bone, it may also involve adjacent soft tissues. We report a case of Gorham-Stout syndrome presented with chylotamponade as a result of extensive intrathoracic lymphatic hyperproliferation. A 50-year-old woman, who is known to have Gorham-Stout syndrome, presented with progressively worsening dyspnea for 1 week. She was hemodynamically stable and her cardiorespiratory examination was unremarkable except for elevated jugular venous pressure. Chest radiography revealed an enlarged cardiac silhouette and a small left pleural effusion. Transthoracic echocardiography confirmed a large pericardial effusion with evidence of tamponade. Urgent pericardiocentesis was performed, and ≈1.2 L of milky fluid, identified a s chyle on biochemistry, was drained. She became asymptomatic soon after pericardiocentesis; however, pericardial fluid reaccumulated over the next few days. She had been diagnosed as having Gorham-Stout syndrome from the age of 12 years. Massive osteolysis of the mandible developed, which required reconstruction. Osteolysis subsequently recurred in the reconstructed mandible. Also, bone involvement in the skull base had …