Saturday, January 16, 2010

More on Hepatic Hydrothorax

Proof enough for me:

Hepatic hydrothorax.

Milanez de Campos JR, Andrade Filho LO, de Campos Werebe E, Pandulo FL, Filomeno LT, Jatene FB.

Division of Thoracic Surgery, Hospital das Clínicas of the University of São Paulo Medical School, São Paulo, Brazil.

Hepatic hydrothorax (HH) is an uncommon manifestation of cirrhosis with ascites. Pleural effusions form when ascitic fluid moves through diaphragmatic defects that have been opened by increased peritoneal pressure. The diagnosis is established clinically by finding a serous transudate and is confirmed by radionuclide imaging demonstrating communication between the peritoneal and pleural spaces. In end-stage liver disease, the management of hepatic hydrothorax is problematic and often does not respond to medical therapy. Therapeutic options for a refractory hepatic hydrothorax include therapeutic thoracentesis, talc slurry through a chest tube, peritoneovenous and pleurovenous shunting, thoracoscopic talc poudrage, transjugular intrahepatic portosystemic shunt (TIPS), thoracosopic diaphragmatic defect repair followed by talc poudrage, and lastly, liver transplant. TIPS can be used as a bridge for transplantation but is often complicated by encephalopathy. Video assisted thoracic surgery (VATS) with patching the defect and talc poudrage may provide symptomatic relief; however, the morbidity and mortality in these extremely ill patients is high. The only definitive treatment for refractory hepatic hydrothorax associated with end-stage cirrhosis is liver transplantation.

Hepatic Hydrothorax – An Uncommon Complication Of Cirrhosis

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

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.

Amlodipine 10 mg daily and Propranolol 40 mg BID.

Physical examination:
Normal vital signs.
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 tests would you order?
- 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 shoewd 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?
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.

What happened next?
1L of pleural fluid was drained via thoracentesis (pleural tap) and sent for analysis.

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.

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.

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