Current opinion in pulmonary medicine
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Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis. ⋯ Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.
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Hepatic hydrothorax is a complication of end-stage liver disease that may have significant associated morbidity. Sodium restriction and diuretic therapy are the mainstays of treatment, though up to a quarter of patients will become refractory to this and will require a pleural drainage procedure. Thoracentesis, transjugular intrahepatic portosystemic shunt, and chemical pleurodesis all have variable success rates and associated complications in the management of hepatic hydrothorax. Indwelling tunneled pleural catheters (ITPC) have been successfully used to manage recurrent symptomatic malignant pleural effusions, and there is growing interest regarding their use in the management of hepatic hydrothorax. ⋯ ITPC may be an acceptable treatment option for the management of hepatic hydrothorax which are refractory to conventional medical management. Larger, randomized controlled trials are needed to further evaluate the safety and efficacy of these catheters for the management of nonmalignant pleural effusions.