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Polycystic liver disease

 

What is the happening deal with Polycystic liver disease?

Polycystic liver disease is very a rare condition that causes cysts to grow throughout the liver. A normal liver has a smooth, uniform appearance. A polycystic liver might look like a cluster of very large sets of grapes. Cysts also can grow independently in different parts of the liver. The cysts, if they get too numerous or large, may cause discomfort and health complications. Abdominal discomfort from swelling of the liver may occur. However, most affected individuals do not have any symptoms. In some cases, polycystic liver disease appears to occur randomly, with no apparent cause. But most people with polycystic liver disease do not have symptoms and live a normal life.

 

Who are more to be affected with Polycystic liver disease?

Liver cysts seem to be caused by the same genetic changes that lead to cysts in the kidney. In the liver, the cysts develop in the bile ducts or liver tubules rather than in liver cells. This means that liver cysts do not replace functioning cells and do not cause liver failure, even when they make the liver very large.

The most significant factor contributing to massive polycystic liver disease is female sex. Women are uniquely susceptible to massive polycystic liver disease, as opposed to men who have the same genetic mutation. It is believed that female steroid hormones may influence several of the factors responsible for secretion by and growth of liver cysts. In my studies, and in studies conducted by others, the risk of having massive polycystic liver disease is related to female gender, pregnancy, and exposure to exogenous sources of female steroid hormones such as contraceptive steroids or use of female hormone replacement therapy by postmenopausal women.

 

What are the symptoms of liver cysts ?

For most of the people with polycystic liver disease there are no symptoms, and these cysts are only found during an ultrasound scan. Occasionally, the liver cysts will impinge on the vascular structures of the liver, leading to portal hypertension and variceal bleeding. Patients with liver cysts can also experience impingement on the venous drainage of the liver, causing a “pseudo” Budd-Chiari syndrome, which blocks the venous drainage from the liver.

Symptoms develop as the cysts grow larger and more numerous:

   > Bloating and increase in size of the abdomen

   > Early fullness on eating

   > Heartburn or vomiting

   > Change in bowel habit or haemorrhoids (piles)

   > Pain.

 

At very rare cases , the cysts may cause complications:

   > Cysts may bleed or burst.

   > Cysts may become infected, causing pain and fever.

 

What are the associated diseases of Polycystic liver disease?

Caroli syndrome is a rare congenital liver disorder characterized by enlargement (dilation) of the small branches of the bile ducts inside the liver. If symptoms occur, the most common are abdominal pain, fever, and yellowing of the skin (jaundice). Caroli syndrome occurs because of abnormal prenatal development.

Choledochal cyst may develop if the common bile duct is malformed, leading to the formation of a cyst. This condition typically appears between the ages of two or three years and adolescence.

Neoplastic cysts are abnormal tissue growths that may be benign or malignant. Benign tumors may cause abdominal discomfort or bleeding within the sac that lines the abdominal cavity

 

How is the diagnosis of Polycystic Liver Disease done ?

> A family member of yours affected with PLD and are under the age of 40, and are infected with more than one cyst.

 > A family member of yours with PLD, are older than 40 having more than three cysts.

  Magnetic resonance imaging, computed tomography scan, and ultrasound are used to take pictures of the liver to see if cysts are present. The image is used for diagnostic purposes.

 

Treatment of Polycystic Liver Disease :

>Treatment may not be necessary in many cases of polycystic liver disease. Removal of the fluid in large cysts may be required in PLD patients with high level symptoms.

> Surgery is perfomed to open up the cyst and remove the fluid that is called as cyst fenestration

>Surgery is performed to remove the part of the liver most affected by the cysts

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