Pneumothorax Ex-vacuo or “trapped lung” in the setting of hepatic hydrothorax

A 24-year-old male with hepatitis C contracted at birth developed cirrhosis complicated by variceal bleeding, portal hypertension, ascites, and a persistent right-sided hepatic hydrothorax that required thoracenteses every four to six weeks. The effusions were transudative in nature (fluid to serum total protein ratio 0.4, fluid to serum LDH ratio 0.5) and without evidence of infection, pleural, or parenchymal abnormalities consistent with a hepatic hydrothorax. Given his refractory effusions, an indwelling pleural cavity drainage catheter was placed at an outside hospital 8 months prior to his orthotopic liver transplantation (OLT) for self-drainage of the effusion at home. The pleural catheter was removed 1 month after its placement when he was referred for OLT in order to minimize the chance of infectious complications. He was subsequently managed successfully with diuretics and therapeutic thoracenteses as needed for symptomatic relief. At the time of liver transplantation he had undergone approximately ten thoracenteses.

He subsequently underwent an uncomplicated OLT. A pre-operative chest x-ray revealed a large chronic right-sided pleural effusion. He was successfully extubated after OLT and doing well so no immediate intervention was performed on the effusion. On post-operative day (POD) #5, the patient developed respiratory distress with new left sided infiltrates consistent with pneumonia. He was started on antibiotics and a right-sided therapeutic thoracentesis was performed with removal of 1.3 liters of fluid. Post-thoracentesis chest imaging revealed a large hydropneumothorax suspicious for a pneumothorax ex-vacuo. A small-bore chest tube was inserted and placed on suction without any additional re-expansion of the lung or any change in his respiratory status (Figure 1). The chest tube was discontinued 2 days later and a subsequent chest computed tomography (CT) confirmed the presence of a large right-sided hydropneumothorax with underlying visceral and parietal pleural thickening and fibrosis causing restriction and constriction of the underlying right lung, consistent with “trapped lung” (Figure 2). Because there was no evidence of any endobronchial obstruction on CT we elected not to perform a diagnostic bronchoscopy to rule out endobronchial lesions. The patient’s respiratory status improved with antibiotics and no infection was identified in the pleural fluid cultures. Thoracic surgery was consulted for possible video-assisted thoracoscopic surgery (VATS); however, because the patient was doing well without any complaints of dyspnea and without hypoxemia, the decision was made to defer the surgery until his immunosuppression was tapered down in order to minimize wound healing complications. He was discharged home on POD #20 without need for supplemental oxygen. A repeat chest x-ray 1 month later showed improvement in the hydropneumothorax, and a chest x-ray obtained 6 months later showed near-complete re-expansion of his right lung without any surgical intervention (Figures 3A-C).

A 68-year old female with non-alcoholic steatohepatitis (NASH) complicated by a refractory right-sided hepatic hydrothorax (fluid to serum total protein ratio 0.3, fluid LDH < 2/3 upper limits of normal serum LDH) presented for an OLT evaluation. The diagnosis of NASH was made 14 years prior and for the 1 year prior to presentation to our hospital, she had required near weekly thoracenteses. The patient was subsequently admitted to our hospital with shortness of breath. Following a therapeutic thoracentesis, she was noted to have a pneumothorax. A 16 French chest tube was placed with no air leak or re-expansion of the lung following chest tube placement. A CT of the chest confirmed the presence of a hydropneumothorax without evidence of endobronchial obstruction. Thoracic surgery was consulted to consider her for definitive management of her pleural effusion because of the persistent high volume drainage (~700 cc per day) from the chest tube. The patient underwent VATS decortication and chemical pleurodesis with resolution of the hydropneumothorax. On POD #20 after her VATS, the patient underwent a successful OLT and did not suffer any further pulmonary or pleural complications.

A 74-year old male with cirrhosis secondary to hepatitis B complicated by a refractory right-sided hepatic hydrothorax (fluid to serum total protein ratio 0.2, fluid LDH < 2/3 upper limits of normal serum LDH) requiring near weekly thoracenteses for the preceding two years presented for an OLT evaluation. After a thoracentesis, he was found to have a pneumothorax and a small bore chest tube was placed by interventional radiology without subsequent re-expansion of the lung. A bronchoscopy was performed and did not reveal any endobronchial abnormalities. Given the complex loculated nature of the effusion, thoracic surgery recommended a thoracotomy with decortication. The operation was complicated by significant post-operative bleeding, and the patient was taken back to the operating room 3 days following the initial thoracotomy due to continued bleeding. Diffuse oozing was found intra-operatively from the parenchymal surface of the lung and the chest wall. The patient suffered a cardiac arrest and expired shortly after surgery.

A 57-year old male with cirrhosis related to NASH diagnosed seven years prior, presented for an OLT evaluation. Prior to presentation, he had multiple chest tubes placed for a refractory right-sided hepatic hydrothorax (fluid to serum total protein ratio < 0.5, fluid LDH < 2/3 upper limits of normal serum LDH) with failed attempts at pleurodesis and had an indwelling pleural cavity drainage catheter placed at an outside hospital. Due to concerns for infection, the pleural catheter was later removed and he was managed with diuretics and frequent thoracenteses. Transjugular intrahepatic porto-systemic shunt (TIPS) was not initially felt to be an option secondary to a reported portal vein thrombosis. Approximately 3 months after the removal of the pleural catheter, another indwelling pleural catheter was placed by an outside hospital secondary to the patient’s difficulty with tolerating diuretics. He eventually underwent successful TIPS placement for the refractory hepatic hydrothorax, and the second indwelling pleural catheter was removed approximately 2 months after its placement. After removal of the second indwelling pleural catheter, chest x-ray revealed a large loculated hydropneumothorax (Figure 4), suspicious for trapped lung. Given that the patient remained asymptomatic without any oxygen requirement, the decision was made to defer any surgical intervention. He was discharged from the hospital and a follow-up chest x-ray one month later revealed significant improvement of his right-sided hydropneumothorax (Figure 5). He is currently doing well two years later and continues to await OLT.