Karen L. Krok; Andrés Cárdenas

Disclosures

Semin Respir Crit Care Med. 2012;33(1):3-10. 

In This Article

Management

Patients with hepatic hydrothorax tend to have advanced liver disease with significant complications related to portal hypertension. They should be referred for a liver transplant evaluation as definitive treatment for hepatic hydrothorax. In patients awaiting liver transplant and in those who are not transplant candidates, the aims of therapy for hepatic hydrothorax are twofold: (1) to relieve symptoms and (2) to prevent pulmonary complications (see Table 2 ). An algorithm for the management of hepatic hydrothorax is outlined in [Fig. 1].

Figure 1.

Treatment algorithm for hepatic hydrothorax. *Diuretics should be started with furosemide 40 mg/day and spironolactone 100 mg/day. If there is no response, diuretics may be increased in a stepwise fashion every 5 days by doubling doses (ratio of 40:100 mg), furosemide up to 160 mg/day and spironolactone up to 400 mg/day. TIPS, transjugular intrahepatic shunt.

Diuretics

The first step in the management of a hepatic hydrothorax is a simple and inexpensive treatment—to create a negative sodium balance by restricting sodium intake and administering diuretics. Patients should be on a 2000 mg/day sodium-restricted diet. Diuretics (typically furosemide and spironolactone) should be coadministered as well to produce a renal excretion of sodium of at least 120 mEq of sodium/day. The best initial regimen is the combination of furosemide 40 mg/day and spironolactone 100 mg/day.[42] If there is no response, compliance with diet and medications should be addressed, and then diuretics may be increased in a stepwise fashion every 5 days by doubling the doses; spironolactone up to 400 mg/day and furosemide up to 160 mg/day. The goal is to achieve an average weight loss of 0.5 kg/day in patients without edema and 1 kg/day in those with peripheral edema.[43,44] Patients should also be instructed to avoid nonsteroidal antiinflammatory drugs (NSAIDs), which can cause sodium retention and renal failure.

Unfortunately some patients are unable to tolerate the appropriate amount of diuretics without increasing their creatinine or developing hyponatremia. In a retrospective study of 405 cirrhotic patients admitted over a 5-year period with ascites, seven of 27 patients (25.9%) were refractory to medical treatment.[3] In a prospective study of 60 cirrhotic patients with hepatic hydrothorax, 13 (21.7%) were considered refractory.[30] In these patients other treatment modalities are required and will be discussed later in this article.

Thoracentesis and Paracentesis

In patients who are symptomatic, a thoracentesis needs to be performed to relieve the dyspnea, respiratory distress, or hypoxia. There are well-documented risks associated with a thoracentesis, including a pneumothorax and hemothorax, but it is usually safe and well tolerated. However, when a thoracentesis is required more than once every 2 to 3 weeks in patients on maximal sodium restriction and optimal diuretics, alternative treatments must be considered because the complication rate increases as more procedures are needed.

As a general rule, no more than 2 L of fluid should be removed because of the risk of pulmonary edema and hypotension.[45] However, this "2 liter rule" was developed in patients with causes of the pleural effusion other than cirrhosis; patients with cirrhosis may be able to tolerate the removal of larger volumes of fluid. Given the relatively small volume of fluid removed at thoracentesis we do not administer intravenous albumin to avoid circulatory dysfunction unlike its routine use with large-volume paracentesis.

Coagulopathy is not considered a contraindication to therapeutic thoracentesis. One study demonstrated no increased risk of bleeding in patients with prothrombin times up to twice the midpoint of the normal range, or platelet counts of more than 50,000/μL; the authors concluded that prophylactic transfusions are not necessary.[21,46] Patients with a serum creatinine more than 6 mg/dL had greater blood loss compared with those with lower values.

A large-volume paracentesis can also offer similar rapid relief of respiratory distress in those with ascites and hepatic hydrothorax and should be attempted prior to a thoracentesis. Angueira and Kadakia[47] found a significant increase in total lung capacity and symptomatic improvement within 2 hours after an average of 3.5 L of ascitic fluid was removed by a paracentesis.

Chest Tube Placement

Various authors have cautioned against the placement of a chest tube in patients with cirrhosis because they are fraught with multiple complications: pneumothorax, hemothorax, empyema, electrolyte abnormalities, and hepatorenal syndrome.[39,48,49] Despite this plea, many patients still have chest tubes placed when they present with respiratory distress and a hydrothorax.

Orman and Lok[40] reviewed the records of 17 patients admitted to a tertiary care center with a chest tube placed for hepatic hydrothorax. Sixteen out of 17 patients had at least one complication, and 12 patients had more than one complication. These included 11 patients with acute kidney injury, seven patients with a pneumothorax, and five patients with an empyema. Liu and colleagues[48] reviewed the outcomes of 59 patients with cirrhosis undergoing chest tube placement and found a complication rate of 80% and a 27% mortality; a caveat of this study is that it included patients who had a chest tube placed for a pneumothorax or empyema, which are indications that require a chest tube to be placed.

The drainage of large volumes of protein-rich pleural fluid may lead to renal failure and electrolyte disturbances in this patient population. It can also be difficult to remove the chest tube because there is often a rapid reaccumulation of fluid once the chest tube is clamped.

In lieu of placing a chest tube other modalities must be considered in the treatment of patients with an uncomplicated hepatic hydrothorax. It should also be noted that the presence of SBEM is not an indication for a chest tube placement unless frank pus is noted on the thoracentesis.

Transjugular Intrahepatic Portosystemic Shunt

Transjugular intrahepatic portosystemic shunt (TIPS) is a procedure that creates an anastomosis between the portal and hepatic veins, decompressing the hepatic and splanchnic vascular bed and resulting in a reduction in portal pressure. It is now the standard of care in patients with refractory hepatic hydrothorax. Contraindications to placing a TIPS are age > 70, significant hepatic encephalopathy, large portal vein thrombosis, right-sided heart failure, elevated pulmonary arterial pressures, and an elevated model for end-stage liver disease (MELD) score (typically greater than 18). This elevated MELD score is a marker for more severe hepatic dysfunction. Because TIPS shunts blood away from the liver and reduces the effective portal perfusion to the liver, it can precipitate liver failure in patients with already significant hepatic dysfunction. In a carefully selected population though, TIPS can lead to significant improvements in the complications related to portal hypertension.

Several groups have reported a beneficial effect of TIPS in patients with hepatic hydrothorax. Unfortunately, few of the studies described the rigor with which the refractoriness of the hydrothorax to medical therapy was determined. Despite this shortcoming, most series have demonstrated response rates in the range of 70 to 80%.[20,50–56]

The most recently published and largest series to date was reported by Dhanasekaran and colleagues in 2010.[55] In their study 73 patients with refractory hepatic hydrothorax had a TIPS placed. Fifty-nine percent of patients had a complete response, 20% had a partial response, and 21% had no response to TIPS placement. The short-term survival rates at 30, 60, and 90 days were 81, 78, and 72%, respectively. The long-term survival rates at 1, 3, and 5 years were 48, 26, and 15%, respectively. These survival rates are equivalent to other previously published rates; Wilputte and colleagues[56] found that the 30-, 90-, and 150-day mortality was 14, 25, and 53%, respectively, after TIPS placement. Encephalopathy developed in 15% of patients.[55]

Risk factors for mortality after TIPS placement for hepatic hydrothorax have been described. These include a Child-Pugh score ≥ 10, a pre-TIPS MELD score > 15, and an elevated pre-TIPS creatinine.[55,56] In addition, a lack of response in the hydrothorax after TIPS placement is associated with an increased mortality rate.

Surgical Repair of Diaphragm Defects

Evaluation for defects in the diaphragm by open thoracotomy and by videothoracoscopy has been used to identify diaphragmatic defects. If the defect can be identified, biologic glue or sutures can be used to close and seal these defects.[10,23,57] In small, uncontrolled reports, good results have been seen in patients in whom the fenestrations can be localized and closed. Milanez de Campos and colleagues[58] performed 21 thoracoscopies in 18 patients with hepatic hydrothorax with an overall success rate of 48%. In the five patients where diaphragmatic defects were identified and closed, four had resolution of the hepatic hydrothorax, and the fifth patient developed empyema and died secondary to pneumonia and liver failure. Morbidity (57.1%) and mortality (38.9%) were high in this study during a follow-up period of 3 months, raising questions about the utility of such an approach. Although there are no head-to-head comparison studies, the mortality rate in this thoracoscopic study can be placed in the context of the lower 90-day mortality (25%) reported with TIPS.

Often the surgical techniques are combined with pleurodesis, which is described below.

Pleurodesis

Pleurodesis is a technique that consists of the ablation of the space between the parietal and visceral pleura with a sclerosing agent injected by tube thoracostomy. Transudative pleural effusions are notoriously difficult to pleurodese due to the absence of inflamed pleural surfaces that are required for successful adhering of parietal and visceral surfaces together. In addition, the rapid fluid reaccumulation prevents the visceral and parietal pleural surfaces from approximating long enough for the inflammatory process to result in pleural symphysis.[59,60]

Pleurodesis with tetracycline or talc has been used. The irritant is administered through a chest tube or by thoracoscopy. In the study described earlier by Milanez de Campos and colleagues,[58] aerosolized talc was effective in preventing recurrence of the effusion in only 10 patients (47.6%). Effusions recurred within 3 months in 43.7% of patients; in addition there were several complications noted, including fever, chest pain, empyema, incomplete reexpansion, pneumonia, and wound infection. A smaller study by Mouroux and colleagues[10] utilized video-assisted thoracoscopy (VATS) to repair diaphragmatic defects in addition to pleurodesis in eight patients with refractory hepatic hydrothorax. Hydrothorax resolution occurred in the six patients where a defect was found and repaired, with a follow-up of 7 to 36 months. In a similar study, Ferrante and colleagues[61] performed VATS and talc pleurodesis in 15 patients with refractory hepatic hydrothorax. No visual defect was found in any of these patients. Control of symptoms and resolution of the effusion were achieved in 11 patients (73%) in the first 30 days after the procedure, with eight patients remaining asymptomatic for a median follow-up of 5.5 months and three experiencing recurrence of the effusion between 45 and 60 days after the VATS. Unfortunately, complications included pain around the chest tube site, low-grade fever with leukocytosis, pleurocutaneous fistula, and empyema. Takayama and colleagues[62] reported on a technique of thoracoscopic pleurodesis using argon beam coagulation, fibrin glue, and minocycline in nine patients with refractory hydrothorax. In this small study all patients showed clinical improvement with two recurrences.

Finally Northup and colleagues[63] described a technique of a thoracoscopically guided mechanical pleurodesis followed by talc instillation combined with simultaneous extended percutaneous peritoneal drainage tube placement to allow complete adherence of the pleural space. In this small series of five patients, no visual defects were found. The drains were left in place for a mean of 12.6 days. Patients were followed for up to 132 days without recurrence of the effusion, although two patients received a liver transplant within 1 month of the procedure. The authors state that the success of their procedure was based on two aspects: (1) the meticulous mechanical abrasion (up to 30 minutes) of the entire diaphragmatic surface and (2) the addition of the peritoneal catheter aided in decreasing the hydrostatic pressure from the peritoneal cavity driving fluid into the pleural space. There were no deaths attributable to the procedure, although four patients required hospitalization for 3 or more weeks.

Continuous positive airway pressure in conjunction with pleurodesis may help to facilitate pleural apposition by creating a positive intrathoracic pressure gradient, which inhibits the flux of ascites into the pleural space.[63,64]

Although VATS with pleurodesis appears promising, most reports include small numbers of patients and are uncontrolled. Therefore, medical management and TIPS should be attempted first, and VATS with pleurodesis should be considered only if these measures fail.

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