hepatic hydrothorax hepatic hydrothorax 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. although it is most common in people with severe ascites, people with mild or no ascites have had the condition. symptoms are not specific and mostly involve the respiratory system. hepatic hydrothoraxchest x-ray showing a hepatic hydrothorax in a person with cirrhosissymptomscough, shortness of breath, and respiratory failurecausesliver cirrhosis, ascitesdiagnostic methodpleural fluid analysistreatmentmedical management, thoracentesis, liver transplantprognosispoor 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. in cases that do not respond, known as refractory cases, the condition is treated with a transjugular intrahepatic portosystemic shunt (tips). the only curative treatment is a liver transplant. prognosis is usually poor. symptoms hepatic hydrothorax 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. less often, it may be the only manifestation of chronic liver disease. the symptoms depend on many factors, such as the effusion's volume, rate of accumulation, and the co-existence of cardiopulmonary disease. the disease 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. 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. in some cases, the main symptoms of people with cirrhosis are respiratory and related to the effusion. mechanism the disease has no known precise, defined mechanism, but several have been proposed. they are similar to the mechanisms behind ascites in cases of portal hypertension. the most medically accepted mechanism is that low albumin levels in blood reduce osmotic pressure and increase pressure in the azygos vein, causing it to leak plasma into the pleural cavity. 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. the tendency towards the right side could be due to the left side being more muscular and the right side being more tendinous due to the 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. in hepatic hydrothorax, the pressure created by ascites and the thinning of the diaphragm caused by malnutrition in cirrhosis cause the defects to become larger. blebs of peritoneum can herniate through these defects, and 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 the pleural membranes can absorb it, hepatic hydrothorax results. diagnosis peritoneal scintigraphy showing tracer in the pleural and abdominal cavities, suggesting a pleuroperitoneal communication the most noticeable symptoms are usually those of cirrhosis and portal hypertension. 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. 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. there may be a higher protein and albumin content in hepatic hydrothorax due to the pleura absorbing the water. 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. management as the disease is causes by leaking ascitic fluid, treatment centers around managing ascites. some cases 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. in these cases, the first treatment of choice is the insertion of a transjugular intrahepatic portosystemic shunt. the only curative treatment is a liver transplant. 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 usage of 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 lose .5 kg/day in cases without edema, and 1 kg/day in cases 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. refractory hepatic hydrothorax fluoroscopic image of tips in progress in cases of 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. 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 hypertension and ascites; it is estimated to work in 70-80% of cases. however, it does not improve the prognosis in cases with end-stage liver disease. 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. in cases 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. other possible complications may include pain, empyema, hemothorax, and subcutaneous emphysema. additionally, repeat thoracocentesis operations increase the risk of complications; a review has indicated that the cumulative risk of complications such as pneumothorax and hemothorax approaches 12%. other treatments in cases where tips is contradicted, another treatment option is to insert an indwelling pleural catheter (ipc). as a last resort, pleurodesis can be used in cases 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. 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 afterwards. prognosis the prognosis is poor and the mortality rate is high. the median survival time for cases with this condition is 8–12 months. the pleural fluid can become infected, resulting in spontaneous bacterial pleuritis. it is underdiagnosed and has a high death rate. epidemiology the condition is found in 5-10% of cirrhosis and portal hypertension cases and 2-3% of all pleural effusions. it is most common in the presence of decompensated cirrhosis.