Hepatic Hydrothorax - An Uncommon Complication Of Cirrhosis

Author: M. Auron, M.D, Department of Hospital Medicine, Cleveland Clinic, OH
Reviewer: V. Dimov, M.D., University of Chicago, IL

A 63-year-old female was admitted to the hospital with a progressive dyspnea of one-week duration. She also had orthopnea but denied chest pain, cough, palpitations, wheezing, fever or chills. Past medical history was significant for well-controlled hypertension. Social history was positive for smoking; the patient denied using drugs or alcohol. Family history was unremarkable.

Medications

Amlodipine 10 mg daily and propranolol 40 mg BID.

Physical examination

Normal vital signs.
Anicteric.
Lung exam showed decreased air entry bilaterally, mostly on the right side with decreased vocal resonance and tactile fremitus, with dullness to percussion over the right infra-axillary and infra-scapular areas.
Abdominal and cardiac exam was unremarkable.
The patient had bilateral pitting edema of lower limbs.

What is the most likely diagnosis?

Pleural effusion.

What laboratory tests would you suggest?

- CXR (PA, lateral and decubitus).
- Complete blood count with differential (CBCD)
- Basic chemistry profile (BMP)
- Hepatic function panel (LFT) including coagulation profile (PT, INR and aPTT).
- Serum LDH
- Ascites fluid albumin, LDH, glucose, pH, cytology
- Thyroid function test
- BNP.
- Transthoracic echocardiogram to assess ejection fraction

Laboratory results

Chest roentgenogram revealed a large right sided pleural effusion.

LFTs showed hypoalbuminemia (2.5) and hyperbilirubinemia (Total 3.0, direct 2.5). INR was elevated (1.8) and PTT was normal. Transaminases were normal.

The remaining of the tests was normal.

What treatment would you start for this patient?

The patient was started on diuretics and a salt/fluid restricted diet. A CT of the chest and abdomen was requested for further evaluation of the effusion.

What happened?

The CT-scan of chest and abdomen showed liver appearance suggestive of cirrhosis without ascites.

Cirrhosis was confirmed via liver biopsy. Hepatitis serology was negative.

Work up for autoimmune hepatitis, alpha-1-antitrypsin deficiency, hemochromatosis and Wilson’s disease was negative. Alpha fetoprotein was normal. Lipid profile showed elevated total cholesterol (280 with elevated LDL 200), suggesting NASH (Non-alcoholic steatohepatitis) as the etiology of cirrhosis.


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What happened next?

One liter (1 L) of pleural fluid was drained via thoracentesis (pleural tap) and sent for analysis.


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The pleural effusion recurred despite conservative measures and frequent thoracocentesis. Hepatic hydrothorax was considered as the most likely diagnoses in the setting of recurrent transudative pleural effusion in a patient with cirrhosis. The patient underwent talc chemical pleurodesis and chest tube placement with clinical improvement and resolution of the effusion.

Final diagnosis

Hepatic hydrothorax secondary to NASH-induced cirrhosis.

What did we learn from this case?

Hepatic hydrothorax is a recurrent transudative pleural effusion that occurs in patients with cirrhosis even in the absence of ascites or other manifestations of portal hypertension. Most commonly is associated with persistent diaphragmatic defects associated with relatively slow forming ascites; this results in pleural effusion as long as the rate of ascites formation does not exceed the volume capacity of the pleural space. The only definitive treatment is liver transplantation.

References

1. von Bierbrauer A, Dilger M, Weissenbach P, Walle J. [Hepatic Hydrothorax - A Rare Cause of Pleural Effusion that is Difficult to Manage. Pneumologie. 2007 Nov 20.
2. Huffmyer JL, Nemergut EC. Respiratory dysfunction and pulmonary disease in cirrhosis and other hepatic disorders. Respir Care. 2007 Aug;52(8):1030-6.
3. Roussos A, Philippou N, Mantzaris GJ, Gourgouliannis KI. Hepatic hydrothorax: pathophysiology diagnosis and management. J Gastroenterol Hepatol. 2007 Sep;22(9):1388-93.
4. Cárdenas A, Arroyo V. Management of ascites and hepatic hydrothorax. Best Pract Res Clin Gastroenterol. 2007;21(1):55-75.
5. Garcia-Tsao G. Portal hypertension. Curr Opin Gastroenterol. 2002 May;18(3):351-9.
6. Gur C, Ilan Y, Shibolet O. Hepatic hydrothorax--pathophysiology, diagnosis and treatment--review of the literature. Liver Int. 2004 Aug;24(4):281-4.

Published: 01/21/2008
Updated: 06/08/2011

5 comments:

  1. very interested

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  2. any specific reason why thyroid function tests were done. also. is it useful to do these tests in acute illness.

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  3. "any specific reason why thyroid function tests were done."

    Hypothyroidism has been associated with pleural effusions, although rare:

    Hypothyroidism
    http://www.merck.com/mmpe/sec12/ch152/ch152f.html

    Hypothyroidism and pleural effusions.
    http://www.ncbi.nlm.nih.gov/pubmed/2135384

    "is it useful to do these tests in acute illness."

    Point well taken, considering sick euthyroid syndrome. However, if there is a rampant hypothyroidism, the TFTs can be helpful since the uncontrolled hypothyroidism itself may be the cause of the acute illness.

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  4. My son had hepatic hydrothorax and we were told a liver transplant would not necessarily prevent this from happening after surgery. The above statement "The only definitive treatment is liver transplantation" is contrary to this. I have serious questions of why he could not have been treated rather than the family encouraged to remove him from ventilator and let nature take it's course, which resulted in death. They did tell us he had 2 bacterial hospital acquired infections. I still believe they could have done something to save him. Can anyone who has experienced such as this shed light on my concern?

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  5. this condition is not so rare in cirrhotic patients. One patient of the clinic (Italy) had pleural effusion that recurred after several treatments. We were able to reduce the pleural effusion avoiding the danger of life, but not avoid completely the formation. The patient was already in the transplant list due to an advanced stage of HCV-related cirrhosis and for now his therapy is supportive.

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