Critical Care Medicine

Pleural Disease: Hepatic Hydrothorax and Hemothorax

Pleural Disease: Hepatic Hydrothorax and Hemothorax

1. Description of the problem

What every clinician needs to know

Hepatic Hydrothorax

A hepatic hydrothorax is a pleural effusion, usually greater than 500 mls, in a cirrhotic patient without evidence of primary pleural, pulmonary or cardiac disease. Typically these effusions are right sided, however, bilateral or left sided effusions are not uncommon. A small percentage of patients develop a hydrothorax in the absence of ascites.

Hemothorax

Hemothorax is defined as blood in the pleural space.

Clinical features

Hepatic Hydrothorax

The presenting symptoms and signs of hepatic hydrothorax are related to direct compression of the adjacent lung. These include cough, shortness of breath, hypoxemia, chest pain and fatigue. When accompanied by fever and malaise, secondary infection of the pleural fluid should be considered and is associated with increased mortality.

Hemothorax

The presenting symptoms and signs of hemothorax are related to direct compression of the adjacent lung. These include cough, shortness of breath, hypoxemia, chest pain and fatigue. In addition, if the pleural bleeding is rapid, the patient may experience symptoms and signs of acute blood loss, including shock. When accompanied by fever and malaise, secondary infection of the pleural fluid should be considered and is associated with increased mortality.

2. Emergency Management

Hepatic Hydrothorax

When the effusion is large it may cause acute symptoms and signs that require emergency management. In some cases even a moderate sized effusion may become critical, due to poor cardiopulmonary reserve or because of the presence of another acute process. In either case, thoracentesis represents the primary intervention required.

As a general rule, no greater than 2 liters of fluid should be removed during the procedure to avoid the risk of re-expansion pulmonary edema, a potentially life-threatening complication. In most cases, removal of this volume will ameliorate the presenting symptoms and signs. Although considered safe, complications of thoracentesis occur including hemothorax, pneumothorax, air embolism, empyema and hemoptysis.

ICU Hemothorax

Early surgical consultation is recommended when a hemothorax is suspected. Coagulation parameters should be measured immediately and corrected if abnormal. It is important to assure that the patient has packed red blood cells available. Initial management of hemothorax usually involves chest tube insertion for the purpose of blood evacuation, lung re-expansion and to assess for further bleeding. A 32-36 French or greater chest tube should be used as it provides superior drainage in adults; while small-bore catheters are not recommended.

Following chest tube placement, a repeat chest radiograph should be obtained to both confirm tube position and assess completeness of hemothorax evacuation. The decision to undergo surgical intervention is guided by both the rate and volume of bleeding, as measured by chest tube output and blood transfusion requirements. Indications for subsequent surgical intervention include chest tube drainage of 1500 mls in 24 hours, accumulation of >200 mls of bloody drainage per hour-over a 2-4 hour period-and/or persistent transfusion requirements to maintain hemodynamic stability.

Depending upon the expertise available and the expected source of bleeding, it may be possible to address the problem thoracoscopically. Generally, however, thoracotomy is the common approach when persistent bleeding is present, the hemothorax is massive or, certainly, if one anticipates a major vessel injury as the source of bleeding.

3. Diagnosis

Establishing the diagnosis

Hepatic Hydrothorax

A cardiac or pulmonary etiology should be excluded in all patients with a pleural effusion(s). Echocardiography is helpful for diagnosing right heart failure or pericardial effusion. A CT scan of the chest should be performed in all patients with suspected hepatic hydrothorax to evaluate for lung, pleural or mediastinal disease that may account for the fluid accumulation.

CT may also reveal ascites which, when present, may help aid in the diagnosis of hepatic hydrothorax. Hepatic ultrasound is helpful for identifying masses, and doppler study of the portal and hepatic veins is used to assess patency.

Thoracentesis is the diagnostic test of choice in patients presenting with a suspected hepatic hydrothorax. Pleural fluid analysis typically demonstrates a transudate. Although the pleural fluid accumulates from migrated ascites, it may differ in the concentration of total protein and albumin when compared to ascites likely due to different absorptive properties of the thoracic pleura.

Cytologic analysis should be performed on the pleural fluid if malignancy is suspected. In patients with fevers, pleuritic pain and/or encephalopathy, fluid gram stain and culture should be obtained and a parapneumonic effusion or infection of a hepatic hydrothorax (spontaneous bacterial empyema [SBEM]) should be suspected.

Criteria for diagnosing SBEM include serum/pleural fluid gradient >1.1 g/dL, polymorphonuclear (PMN) leukocyte count >500 mm3 or positive fluid culture with an absence of pneumonia or contiguous infection.

Bacteria commonly associated with spontaneous bacterial peritonitis (SBP) are similar to those found in SBEM and include E. Coli, Streptococcus species, Enterococcus, Klebsiella or Pseudomonas and approximately 50% of episodes of SBEM are associated with spontaneous bacterial peritonitis.

Intraperitoneal injection of 99mTc-sulpher colloid or 99mTc-human serum albumin is a described method used to confirm the diagnosis of hepatic hydrothorax. Patients undergo therapeutic thoracentesis followed by intraperitoneal radioisotope injection. Detection of the marker in the pleural cavity is confirmatory. This rarely is necessary.

ICU Hemothorax

Hemothorax is typically diagnosed using clinical and radiologic techniques. Small hemothoraces may be hard to recognize clinically and auscultation of the chest may be unremarkable. In large or expanding hemothoraces, lung auscultation demonstrate decreased breath sounds over the affected lung field with associated dullness to percussion.

Cardiopulmonary instability may develop due in part to a decrease in circulating blood volume and effective hematocrit. Pulmonary and cardiac compression may result as the volume of blood in the chest increases contributing to cardiopulmonary instability.

An upright chest X-ray remains the initial study of choice in the acute evaluation of hemothorax. For a hemothorax to be detected on a chest X-ray, 400-500 mls of fluid must be present in the pleural space. Supine films are less helpful for estimating the quantity of fluid in the chest due to fluid layering. It is almost always easier to assess fluid (or air) accumulation on an ICU film if the X-ray is taken with the patient in the upright, as opposed to supine, position.

Lateral supine films may help to differentiate a layering effusion from pulmonary contusions or other intraparenchymal process. The clinician should have a high index of suspicion of a hemothorax when a new effusion is seen on chest X-ray following a thoracic intervention.

Ultrasound may be useful for identifying the presence and quantifying the volume of pleural fluid.

Chest CT scan is a highly accurate study for identifying and characterizing fluid in the pleural cavity. CT scan differentiates between parenchymal and pleural process and can be helpful for evaluating persistent opacities on chest radiograph after tube thoracostomy.

Normal lab values

Typical results for pleural fluid in hepatic hydrothorax include:

1. Cell count <1000/cm3

2. Protein concentration <2.5 g/dL

3. Polymorphonuclear leukocyte (PMN) count <250/mm3

4. Pleural fluid/serum total protein ratio <0.5

5. Pleural fluid/serum lactate dehydrogenase ratio <0.6

6. Pleural fluid/serum albumin gradient >1.1

7. pH >7.4

8. Pleural fluid glucose level similar to serum level

How do I know this is what the patient has?

Hepatic hydrothorax should be suspected in patients with a history of portal hypertension and/or cirrhosis who presents with a pleural effusion. Ascites is usually present along with other features of cirrhosis.

Differential diagnosis

One must consider alternative diagnoses for a unilateral or bilateral pleural effusion.

When the effusion is unilateral, If the patients is acutely ill with fevers and/or WBC elevation, must consider empyema or parapneumonic effusion related to an underlying pneumonia. If the effusion is on the left side, with fevers - particularly in the alcoholic patient - Borhave syndrome (ruptured esophagus) is a consideration.

Once a transudate is confirmed, then CHF, or a low albumin are the most likely alternative etiologies. Other causes for a transudative effusion include pulmonary emboli, hypothyroidism, and early lymphatic obstruction. The differential for an exudative effusion is much longer and includes inflammatory and malignant processes.

4. Specific Treatment

Hepatic Hydrothorax

Thoracentesis represents the primary intervention required when symptoms or signs need treatment urgently. If symptoms or signs are mild and tolerable, then medical therapy should be the first intervention. The medical management of hepatic hydrothorax should focus largely on controlling the ascites. This is accomplished by reducing gradually the extracellular fluid volume, but not at the expense of tissue perfusion.

Patients are maintained on a sodium-restricted diet while trying to achieve negative fluid balance through the use of diuretics, including spironolactone and a loop diuretic (furosemide). Non-steroidal anti-inflammatory agents are avoided due to their sodium retention activity and increased risk of AKI in this patient population. Definitive treatment of hepatic hydrothorax requires treatment of the underlying liver failure, including medical management to liver transplant.

When medical therapy fails to control the effusion, and it returns, thoracentesis is indicated. If the hepatic hydrothorax is refractory to medical therapy, repeated thoracenteses is usually required, however, the duration of benefit is variable. Prior to performing thoracentesis, a chest x-ray or CT scan is helpful to characterize the amount of fluid present.

As a general rule, no greater than 2 liters of fluid should be removed during the procedure to avoid the risk of re-expansion pulmonary edema, a potentially life-threatening complication. Although considered safe, complications of thoracentesis occur including hemothorax, pneumothorax, air embolism, empyema and hemoptysis.

Hemothorax

See emergent management seciton for the initial treatment of hemothorax.

Persistent blood seen on chest radiographs and CT scans following tube thoracostomy is an independent predictor of the development of an empyema and should be treated with early VATS, within 3-7 days of hospitalization.

Blood tends to organize over time and forms a thick peel that may result in a "trapped" non-expanding lung. This phase requires a surgical intervention (video-assisted thoracoscopy or thoracotomy) to remove the peel to facilitate lung expansion. Early thoracoscopy (before day 3) for retained hemothorax has been associated with a reduction in operative difficulty, contamination/infection of clot, and hospital length of stay compared to those performed later. Additional benefits include fewer pulmonary complications, shorter time to recovery, and less long-term disability.

VATS can be attempted in the retained hemothorax later; however, the conversion rate to open thoracotomy increases with longer delays. There should be a low threshold for early referral for a VATS cleanout when a patient has retained blood despite adequate chest tube drainage.

Reports of using thrombolytics for treating subacute (6-13 day old) collections in patients at high risk for thoracotomy have been reported; however, surgical intervention is the treatment of choice when feasible.

Refractory cases

Hepatic Hydrothorax

In patients who require repeat thoracentesis, despite maximal medical therapy at a frequency of more than every 2 weeks, they should be considered for transjugular intrahepatic portosystemic shunt (TIPS) procedure. The TIPS procedure involves fluoroscopic placement of a metal stent within the liver between the portal and hepatic venous system. This results in a decrease in portal pressure and subsequent decrease in ascites production.

Symptomatic improvement is reported in 70-80% of patients with refractory hepatic hydrothorax. Complications of TIPS include shunt thrombosis, hepatic encephalopathy and worsening pulmonary hypertension. Although TIPS may provide symptomatic relief from hepatic hydrothorax, it is typically considered a "bridge" to transplantation.

Should a chest tube be placed?

No, not for an uncomplicated hepatic hydrothorax! It is important to stress that although chest tube drainage may provide immediate symptomatic relief of dyspnea in patients with a hepatic hydrothorax, one should always try to avoid chest tube placement. This relates to the subsequent persistent high volume chest tube output, which culminates in massive fluid shifts, protein and electrolyte depletion, infection and bleeding risk.

Is chest tube placement followed by sclerosis ever a therapeutic option?

No! Chest tube placement followed by administration of a sclerosing agent (e.g. talc, tetracycline) has limited success due to rapid fluid accumulation in the pleural cavity, which inhibits visceral-parietal pleural coaptation and symphysis. The high volume nature of these effusions, by providing a route of easy egress for ascites, makes these effusions recalcitrant to pleurodesis. It is, therefore, misguided to presumptively place a chest tube with the intention of subsequently performing a pleurodesis.

If a patient is dyspneic because of the effusion, then palliation can be temporarily achieved with thoracentesis, as further control of the ascites is initiated, but placing a chest tube is almost always a mistake.

Are there any reasonable surgical options?

Yes. Successful outcomes utilizing video assisted thoracoscopy surgery (VATS) talc pleurodesis have been reported, although repeat procedures are frequently necessary. Surgical repair of diaphragmatic defects with or without surgical pleurodesis has been reported with some success. Using a VATS approach, diaphragmatic defects are identified and closed using sutures. Other methods described include VATS repair using pleural flap or mesh only reinforcement.

These procedures should be considered palliative and may be attempted as an alternative to frequent thoracentesis and TIPS. Pleurodesis and/or surgical diaphragm repair should be reserved for patients who have failed conservative measures and the increased morbidity and mortality of operating on a patient with hepatic failure must be considered before considering any of these approaches a rational option.

Cases of tunneled pleurocutaneous catheter placement for managing symptomatic hepatic hydrothorax have been reported. Advantages cited for this technique include ease of insertion and avoidance of repeat thoracentesis and its associated complications. The major risk of an indwelling catheter relates to infection risk, which may be associated with increased mortality in this patient population.

Peritoneo-venous shunt (Le Veen shunt) diverts ascites from the peritoneal cavity into the venous system and was used in the past for managing hepatic hydrothorax. Due to their limited efficacy and high complication rate, Le Veen shunts are no longer used for treating hepatic hydrothorax.

5. Disease monitoring, follow-up and disposition

Hepatic Hydrothorax

After tapping a hepatic hydrothorax, the patient should be monitored closely for 24 hours for recurrence of the symptoms and signs and for complications related to the thoracentesis. Most importantly, a CXR should be done soon after completion of the procedure to both rule out a pneumothorax and to assess the impact of fluid removal. HgB should be followed for 6-24 hours to ensure there is no internal bleeding. Finally, medical therapy to control the ascites must be instituted otherwise the effusion will recur.

Hemothorax

After chest tube placement the patient should be monitored closely for 24 hours to assess for ongoing bleeding and for recurrence of associated symptoms and signs. Soon after the procedure a CXR should be done to assess for appropriate tube placement and to assess the impact of fluid removal. Chest tube output should be quantified every 1-2 hours and HgB should be checked every 6-8 hours. Close monitoring should continue for at least 12-24 hours, based on the etiology, volume, and rate of bleeding.

Follow-up chest X-Rays and CT scans should be done to assess for clearing of blood from the pleural space, since residual blood that fails to drain following tube thoracostomy is an independent predictor of the development of an empyema and should be treated with early VATS, within 3-7 days of hospitalization.

Blood tends to organize over time and forms a thick peel that may result in a "trapped" non-expanding lung. This phase requires a surgical intervention (video-assisted thoracoscopy or thoracotomy) to remove the peel to facilitate lung expansion. Early thoracoscopy (before day 3) for retained hemothorax has been associated with a reduction in operative difficulty, contamination/infection of clot, and hospital length of stay compared to those performed later. Additional benefits include fewer pulmonary complications, shorter time to recovery and less long-term disability.

VATS can be attempted in the retained hemothorax later; however, the conversion rate to open thoracotomy increases with longer delays. There should be a low threshold for early referral for a VATS clean out when a patient has retained blood despite adequate chest tube drainage.

Reports of using thrombolytics for treating subacute (6-13 day old) collections in patients at high risk for thoracotomy have been reported; however, surgical intervention is the treatment of choice when feasible.

Pathophysiology

Hepatic Hydrothorax

Hepatic hydrothorax originates from the direct passage of ascites across small defects in the diaphragm called fenestrations. These fenestrations are seen microscopically as discontinuities in collagen bundles in the tendinous diaphragm. Ascites is pushed through these fenestrations into the pleural space down a pressure gradient from the higher intra-abdominal pressure to the lower intra-pleural pressure.

Once the absorptive capacity of the pleura surfaces is overwhelmed, fluid accumulates, resulting in a pleural effusion. Additional mechanisms are thought to contribute to pleural fluid accumulation in hepatic hydrothorax including hypoalbuminemia (decreased colloid osmotic pressure), perito-pleural lymphatic drainage and azygous vein hypertension with plasma leakage, since not all patients diagnosed with this condition have coexisting ascites.

Hemothorax

Aside from trauma, most hemothoraces in the intensive care unit are related to interventions, including thoracentesis, chest tube insertion, central line or right heart catheter placement, and the bleeding source is usually venous in origin. Hemothorax also occurs following thoracic and cardiac surgery procedures.

Thoracentesis is frequently performed in ICU patients for evaluating pleural fluid, and bleeding associated with thoracentesis is usually minor and resolves without further interventions. Major bleeding may result from intercostal vessel laceration.

Injuries to the spleen and liver have been reported during thoracenteses; however, intra-abdominal bleeding is more common in these patients. A history of a bleeding disorder, anticoagulation medications and renal failure may increase the risk of bleeding in patients undergoing thoracentesis and other invasive thoracic procedures.

Hemothorax following chest tube insertion is a known complication. Blood draining from the chest tube is typically the first indication of an insertion bleeding complication. Post chest tube placement radiographs should always be obtained to confirm tube position and determine whether the indication for placement has been adequately addressed. A new opacity following tube placement should alert the clinician that a lung parenchymal injury occurred or that a hemothorax may be developing.

To best avoid a chest-related hemothorax, anticoagulation should be stopped at the appropriate time and coagulopathies should be reversed when possible. The chest tube should be tunneled directly on top of the desired rib. This decreases the risk of injury to the intercostal vein and artery which lie on the inferior aspect of the ribs. Muscular bleeding may occur and is usually self-limited; however, injury to muscular arterial and venous branches may result in persistent bleeding necessitating surgical intervention.

Central venous catheter insertion is a commonly performed ICU procedure. The subclavian vein is located at the apex of the chest adjacent to the pleura and free wall vein penetration may cause hemothorax. Injuries to the jugular vein, subclavian artery and right atrium resulting in hemothorax have been reported. Venous injury at the left brachiocephalic superior vena cava junction occasionally occurs when inserting left sided internal jugular or subclavian lines.

Complications associated with pulmonary artery catheter insertion include cardiac arrhythmias, carotid and subclavian artery puncture, catheter associated sepsis and catheter induced pulmonary artery (PA) rupture. Hemothorax following catheter induced PA rupture is a life threatening complication associated with a high mortality. Urgent thoracotomy with direct injury repair is optimal; however, lobectomy or pneumonectomy may be required to control hemorrhage.

Efforts to avoid PA rupture include balloon inflation in large arteries prior to catheter advancement, minimizing time spent in the wedge position, and removal of balloon air prior to catheter withdrawal.

Epidemiology

NA

Prognosis

Hepatic Hydrothorax

The prognosis of patients with hepatic hydrothorax is largely dependant on the underlying liver disease.

Hemothorax

The prognosis of a hemothorax largely depends on the cause. If traumatic, the prognosis is excellent. If the cause of the hemothorax is malignancy, then the prognisis is grim and is clearly defined by the malignancy.

Special considerations for nursing and allied health professionals.

NA

What's the evidence?

Hepatic Hydrothorax

Gurung, P, Goldblatt, M, Huggins, JT, Doelken, P, Nietert, PJ, Sahn, SA. "Pleural fluid analysis and radiographic, sonographic, and echocardiographic characteristics of hepatic hydrothorax". Chest. vol. 140. 2011. pp. 448-53.

(Article provides detailed fluid analysis of patients undergoing thoracentesis for hepatic hydrothorax.)

Mercky, P, Sakr, L, Heyries, L. "Use of a tunnelled pleural catheter for the management of refractory hepatic hydrothorax: a new therapeutic option". Respiration. vol. 80. 2010. pp. 348-352.

(Authors describe succesful use of managing a patient with hepatic hydrothorax with a tunneled pleurex catheter)

Alonso, JC. "Pleural effusion in liver disease". Seminars in Respiratory & Critical Care Medicine. vol. 31. 2010. pp. 698-705.

Gurung, P, Goldblatt, M, Huggins, JT, Doelken, P, Nietert, PJ, Sahn, SA. "Pleural fluid analysis and radiographic, sonographic, and echocardiographic characteristics of hepatic hydrothorax". Chest. vol. 140. 2011. pp. 448-53.

Roussos, A, Philippou, N, Mantzaris, GJ, Gourgouliannis, KI. "Hepatic hydrothorax: pathophysiology diagnosis and management". Journal of Gastroenterology & Hepatology. vol. 22. 2007. pp. 1388-93.

(Review article focusing on the pathogenesis, clinical manifestations, diagnosis and treatment of hepatic hydrothorax).

Cerfolio, RJ, Bryant, AS. "Efficacy of video-assisted thoracoscopic surgery with talc pleurodesis for porous diaphragm syndrome in patients with refractory hepatic hydrothorax". Annals of Thoracic Surgery. vol. 82. 2006. pp. 457-9.

(Authors describe a surgical approach using video assisted thoracoscopy with talc pleurodesis for treating refractory hepatic hydrothorarax which proved successful in controlling effusions in 3/4 of patients.)

Hemothorax

DuBose, J, Inaba, K, Demetriades, D, Scalea, TM, O'Connor, J, Menaker, J, Morales, C, Konstantinidis, A, Shiflett, A, Copwood, B. "AAST Retained Hemothorax Study Group. Management of post-traumatic retained hemothorax: a prospective, observational, multicenter AAST study". The Journal of Trauma and Acute Care Surgery. vol. 72. 2012. pp. 11-22.

(Article reviews practice patterns and factors influencing the successful management of post-traumatic retained hemothorax and identifies predictors of the need for thoracotomy in these patients).

Mowery, NT, Gunter, OL, Collier, BR, Diaz, JJ, Haut, E, Hildreth, A, Holevar, M, Mayberry, J, Streib, E. "Practice management guidelines for management of hemothorax and occult pneumothorax". Journal of Trauma-Injury Infection & Critical Care. vol. 70. 2011. pp. 510-8.

Pneumatikos, I, Bouros, D. "Pleural effusions in critically ill patients". Respiration. vol. 76. 2008. pp. 241-8.

(Authors describe the evaluation and management of pleural effusions in critically ill patients with a focus on initial and subsequent management of hemothorax in these patients).
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