Bilateral chylothorax caused by pleuropulmonary lymphangiomyomatosis: a challenging problem in thoracic surgery

Massive left-sided pleural effusion in a 35-year-old man was initially diagnosed as idiopathic spontaneous chylothorax and treated with serial thoracenteses and left thoracotomy. Six weeks later, a right thoracotomy was performed for contralateral chylothorax, and histologic examination revealed lymphangiomyomatosis. The patient survived this rare and potentially fatal disease. We have found no previously published case of bilateral lymphangiomyomatosis treated with separate thoracotomies because of bilateral chylothorax.