Liver cyst is a fluid filled cavity that varies in size from a few millimeters to several centimeters. Typically, polycystic liver disease is characterized by more than one cyst in the liver and falls in this rare category of disease when there are more than 20 cysts in the liver. Polycystic liver disease is a rare inherited disorder that can be divided into two types, namely, isolated autosomal dominant polycystic liver disease (when the cysts are found exclusively in liver) and autosomal dominant polycystic kidney disease (when the cysts are present in kidneys as well as liver). Both these diseases are autosomal dominant diseases and autosomal dominant polycystic kidney disease (ADPKD) is the more common disease ranging approximately 80-90% of all the cases.
It is considered a genetic disorder with autosomal dominant inheritance. It is more common in females than in males and the susceptibility increases with growing age. It is thought to be related to female sex hormones and susceptibility increases in females after pregnancy, exposure to oral contraceptives and postmenopausal females on hormone replacement therapy. The liver cysts are usually asymptomatic until and unless they are very large. When large, they present with symptoms of abdominal distension and discomfort, postprandial fullness, back discomfort and shortness of breath. An abdominal imaging (ultrasound, CT scan and MRI) will typically show signs of hepatomegaly and macrocysts in the liver.
What Is The Treatment For Liver Cysts?
It is a general misconception that liver cysts are benign and do not require any intervention. However, when the cyst is too large it needs symptomatic relief by reducing the size of liver that can be provided with many treatment options. The treatment measures include conservative approach, invasive and medical approach.
Aspiration and Sclerotherapy: This involves aspiration of the cystic fluid and injecting a sclerosing agent following the aspiration. In general, a dominant cyst (greater than 5cm) is chosen that is responsible for the symptoms of abdominal discomfort, so that symptomatic relief can be provided. The cystic fluid is aspirated with the help of a catheter with an aspiration needle and later on injected with a sclerosing agent. The common sclerosing agents used are ethanol, minocycline and tetracycline, which help in the destruction of epithelial lining of the cyst, so that more fluid production is hindered. Pain is a common complication at the time of ethanol sclerotherapy.
Fenestration: This can be achieved either laparoscopically or open surgery. In this, a surgical window (also known as deroofing) is created along with cystic fluid drainage. Nowadays, mostly laparoscopic fenestration is adopted; however, some cases may be suitable for open fenestration due to its better accessibility and visibility.
Segmental Hepatic Resection: This procedure involves removing (resectioning) a part of liver. Resectioning is done in cases where there are many cysts in a segment of liver and is done only in cases of extreme increase in hepatic volume. For a better resectional outcome, there should at least be about 20-30% normal liver parenchyma.
Resection is considered when the symptoms are severe with severe liver enlargement due to cysts and sole fenestration will not be able to reduce the liver volume. There is an increased chance of liver bleeding and bile leakage along with risk of adhesions that might complicate future transplantation of liver.
Liver Transplantation: It is the only cure for liver cysts and is carried out in severe polycystic liver cases. It is only indicated in patients who have severe symptoms that are severely impairing quality of life of the patient as well as in cases where portal hypertension and nutritional compromise are major untreated complications.
Apart from surgical intervention, there are medicinal options that are being researched and studied; these include somatostatin analogs (octreotide and lanreotide) and mammalian target of rapamycin (mTOR) inhibitors (sirolimus and tacrolimus). The clinical trials on these medications have been promising, but their safety, efficacy; dose and duration need to be further studied before introducing them in the market for individuals.
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