Hepatic hydrothorax
| Hepatic hydrothorax | |
|---|---|
 | |
| Chest X-ray showing a hepatic hydrothorax in a person with cirrhosis | |
| Symptoms | Cough, shortness of breath, and respiratory failure |
| Causes | Liver cirrhosis, ascites |
| Diagnostic method | Pleural fluid analysis |
| Treatment | Medical management, thoracentesis, liver transplant, palliative care |
| Prognosis | Poor |
Hepatic hydrothorax is a rare form of pleural effusion that occurs in people with liver cirrhosis. It is defined as an effusion of over 500 mL in people with liver cirrhosis that is not caused by heart, lung, or pleural disease. It is found in 5–10% of people with liver cirrhosis and 2–3% of people with pleural effusions. It is much more common on the right side, with 85% of cases occurring on the right, 13% on the left, and 2% on both.[1] Although it is most common in people with severe ascites, it can also occur in people with mild or no ascites. Symptoms are not specific and mostly involve the respiratory system.
The condition is diagnosed based on the existence of liver cirrhosis and fluid build-up in the abdomen (ascites) and analysis of the fluid. The fluid has a low protein content. Mainly, the condition is treated by medical management, such as diet adjustment and usage of diuretics. Cases which do not respond, known as refractory hepatic hydrothorax, the condition is treated with a transjugular intrahepatic portosystemic shunt (TIPS). The only curative treatment is a liver transplant. Prognosis is usually poor.
Symptoms
The condition generally has no specific symptoms as it generally occurs with ascites and other manifestations of increased pressure in the portal vein caused by liver disease. Pleural fluid causes symptoms far more easily than ascitic fluid, due to the lower volume of the pleural cavity as compared to the abdominal cavity. The main symptoms are usually related to the symptoms of liver cirrhosis and ascites.[2] Less often, it may be the only manifestation of chronic liver disease. The symptoms depend on many factors, such as the effusion's volume, how fast it accumulates, and the presence of cardiopulmonary disease. The condition may cause no symptoms and be incidentally detected by medical scans, or it may cause large pleural effusions that result in respiratory symptoms like cough, shortness of breath, low blood oxygen, and respiratory failure.[3] In general, people are more sensitive to pleural effusions then ascites; much smaller effusions can cause symptoms.[4] Most people have progressive difficulty breathing and reduced tolerance for exercise. Rarely, there may be acute cases that accumulate fluid rapidly and result in circulatory collapse.[5] In some people, the main symptoms of people with cirrhosis are respiratory and related to the effusion.
Mechanism
The condition has no known precise, defined mechanism, but several have been proposed, which are similar to those behind ascites.[1][2] The most accepted theory is that fluid originating from ascites travels through defects in the diaphragm into the pleural cavity. These defects exist in the normal population. The defects are usually less than 1 cm and are more common of the right side, possibly because of the increased prevalence of tendon tissue from its proximity to the liver. Through a microscope, they look like discontinuities in the bundles of collagen that make up the tendon part of the diaphragm.[2] In hepatic hydrothorax, the pressure created by ascites and the thinning of the diaphragm caused by malnutrition cause the defects to become larger. Blebs of peritoneum can herniate through these defects; if they burst, a pleuroperitoneal communication is created. Fluid moves from the abdomen to the pleural cavity via a pressure gradient between the cavities. If the fluid accumulates faster than it can leave via pleural membrane absorption, hepatic hydrothorax results.[1]
Diagnosis
The most noticeable symptoms are usually those of cirrhosis and portal hypertension.[2] Most affected people show signs of end-stage liver disease. Diagnosis involves extracting the fluid via thoracentesis; after this, the fluid is analyzed to diagnose and rule out other causes.[5] The fluid can be analyzed for serum, protein, albumin, lactate dehydrogenase, and cell count. The fluid is a transudate and similar to fluid found in ascites.[2] There may be a higher protein and albumin content in hepatic hydrothorax due to the pleura absorbing the water.[3] To rule out heart-related causes of pleural effusion, an echocardiogram can be performed. Pleuroperitoneal communications are best detected by peritoneal scintigraphy. Hydrothorax without ascites has been reported to occur in as many as 20% of people with cirrhosis, but only detected in 7% of cases via CT scan and ultrasound.[2]
Management
As the condition is causes by leaking ascitic fluid, treatment centers around managing ascites. Some individuals respond to medical management. In up to 26% of cases, the condition does not respond to medical management, in which case it is known as a refractory hepatic hydrothorax. For these individuals, the first treatment of choice is the insertion of a transjugular intrahepatic portosystemic shunt. The only curative treatment is a liver transplant. Additionally, treatment involves addressing the underlying cause of the liver disease, such as alcohol use or viral hepatitis.[4]
Medical management
Medical management is the main treatment. Although it is simple, cheap, and noninvasive, it has a high rate of failure and comes with a risk of acute kidney injury and kidney failure. Reducing sodium in the diet and using diuretics may help reduce ascites and stop the growth of the effusion. The goal of medical management is a low sodium diet of 70-90 mmol per day and to lose.5 kg/day of weight for those without edema, and 1 kg/day for those with edema. Usually, diet modification is not enough; then, diuretics are the next line of treatment. A distal agent and a loop diuretic can be used together to cause the kidneys to excrete least 120 mEq/day of sodium via urine. The amount of sodium excreted in urine is monitored before and during treatment to adjust diuretic dosage based on response.[3] Additionally, vasoconstrictors, such as midodrine, may help increase salt output by the kidneys.[4]
Refractory hepatic hydrothorax
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For those with refractory hepatic hydrothorax, the only definite treatment is a liver transplant. However, the majority of people with this condition are unsuitable for transplantation, and the majority of those that are die awaiting it. However, other treatments can improve symptoms, increase survival, and, ideally, give time until a liver transplant in available.[2]
Transjugular intrahepatic portosystemic shunt
The main treatment in those with refractory hepatic hydrothorax is the insertion of a transjugular intrahepatic portosystemic shunt (TIPS). TIPS decompresses the portal system, reducing portal venous pressure and fluid in the abdomen; it is estimated to work in 70-80% of cases. However, it does not improve the prognosis in those with end-stage liver disease.[6] In people with serious liver dysfunction, TIPS may cause liver failure, as it shunts blood away from the liver.
Thoracocentesis
Thoracocentesis, though typically safe, only provides temporary benefit, as fluid tends to return quickly.[7] Other possible complications may include pain, empyema, hemothorax, and subcutaneous emphysema.[3] Repeat usage of thoracocentesis increases the risk of complications; a review has indicated that the cumulative risk of complications such as pneumothorax and hemothorax approaches 12%.[7] In cases with ascites, initially performing paracentesis to drain the ascitic fluid can help reduce the chance of recurrence.[4]
Other treatments
In cases where TIPS is contradicted, another treatment option is to insert an indwelling pleural catheter (IPC).[8] Pleural treatments generally have a high complication rate;[7] in a case study, those receiving IPC had greater complication rates despite undergoing significantly less procedures.[4] As a last resort, pleurodesis can be used for those without ascites; by irritating the pleura together, it can repair any defects in the diaphragm. However, it requires multiple procedures and general anesthesia. Additionally, the amount of pleural fluid produced can overcome pleurodesis, causing it to fail.[7] Complications may include empyema, sepsis, and septic shock. Chest tubes are contradicted, as they can cause loss of protein, infection, pneumothorax, hemothorax, and electrolyte imbalances. Additionally, removing them may pose a challenge, as the fluid tends to return extremely quickly afterwards.[3] Palliative care can also help symptoms; for those resistant to disease-related treatment, no preferred methods exist to manage symptoms for this condition.[4]
Prognosis
The prognosis is poor, and the mortality rate is high. The median survival time for people with this condition is 8–12 months.[3] The pleural fluid can become infected, resulting in spontaneous bacterial pleuritis.[5] A Child-Pugh score greater than or equal to 10, MELD score greater than 15, higher creatinine levels, and no response to TIPS indicates an increased risk of death. Chest tube usage generally indicates a poor prognosis, with 1-year mortality rates of nearly 90% in one case study.[4]
Epidemiology
The condition is found in 5–10% of those with cirrhosis and portal hypertension[9] and 2–3% of all pleural effusions.[1] It is most common in the presence of decompensated cirrhosis.[5]
References
- ^ a b c d Garbuzenko, Dmitry Victorovich; Arefyev, Nikolay Olegovich (2017-11-08). "Hepatic hydrothorax: An update and review of the literature". World Journal of Hepatology. 9 (31): 1197–1204. doi:10.4254/wjh.v9.i31.1197. ISSN 1948-5182. PMC 5680207. PMID 29152039.
- ^ a b c d e f g Singh, Amita; Bajwa, Abubakr; Shujaat, Adil (2013). "Evidence-Based Review of the Management of Hepatic Hydrothorax". Respiration. 86 (2): 155–173. doi:10.1159/000346996. ISSN 0025-7931. PMID 23571767. S2CID 34109215.
- ^ a b c d e f Lv, Yong; Han, Guohong; Fan, Daiming (2018-01-01). "Hepatic Hydrothorax". Annals of Hepatology. 17 (1): 33–46. doi:10.5604/01.3001.0010.7533. ISSN 1665-2681. PMID 29311408.
- ^ a b c d e f g Pippard, Benjamin; Bhatnagar, Malvika; McNeill, Lisa; Donnelly, Mhairi; Frew, Katie; Aujayeb, Avinash (2022-06-25). "Hepatic Hydrothorax: A Narrative Review". Pulmonary Therapy. 8 (3): 241–254. doi:10.1007/s41030-022-00195-8. ISSN 2364-1754. PMC 9458779. PMID 35751800.
- ^ a b c d Chaaban, Toufic; Kanj, Nadim; Bou Akl, Imad (2019-08-01). "Hepatic Hydrothorax: An Updated Review on a Challenging Disease". Lung. 197 (4): 399–405. doi:10.1007/s00408-019-00231-6. ISSN 1432-1750. PMID 31129701. S2CID 164216989.
- ^ Banini, Bubu A.; Alwatari, Yahya; Stovall, Madeline; Ogden, Nathan; Gershman, Evgeni; Shah, Rachit D.; Strife, Brian J.; Shojaee, Samira; Sterling, Richard K. (2020). "Multidisciplinary Management of Hepatic Hydrothorax in 2020: An Evidence-Based Review and Guidance". Hepatology. 72 (5): 1851–1863. doi:10.1002/hep.31434. ISSN 1527-3350. PMID 32585037. S2CID 220072866.
- ^ a b c d Pippard, Benjamin; Bhatnagar, Malvika; McNeill, Lisa; Donnelly, Mhairi; Frew, Katie; Aujayeb, Avinash (2022-09-01). "Hepatic Hydrothorax: A Narrative Review". Pulmonary Therapy. 8 (3): 241–254. doi:10.1007/s41030-022-00195-8. ISSN 2364-1746. PMC 9458779. PMID 35751800.
- ^ Haas, Kevin P.; Chen, Alexander C. (July 2017). "Indwelling tunneled pleural catheters for the management of hepatic hydrothorax". Current Opinion in Pulmonary Medicine. 23 (4): 351–356. doi:10.1097/MCP.0000000000000386. ISSN 1070-5287. PMID 28426468. S2CID 36048269.
- ^ Gilbert, Christopher R.; Shojaee, Samira; Maldonado, Fabien; Yarmus, Lonny B.; Bedawi, Eihab; Feller-Kopman, David; Rahman, Najib M.; Akulian, Jason A.; Gorden, Jed A. (2022-01-01). "Pleural Interventions in the Management of Hepatic Hydrothorax". Chest. 161 (1): 276–283. doi:10.1016/j.chest.2021.08.043. ISSN 0012-3692. PMID 34390708. S2CID 237054567.