Polycystic Liver Disease (PLD)

Polycystic Liver Disease (PLD) is a medical condition of liver caused by multiple cysts in liver. The cysts are of different sizes and eventually affect the function of liver. Polycystic liver disease may be associated with similar cystic disease of kidney. In this article, we will discuss various causes, symptoms, and treatment for Polycystic Liver Disease.

Polycystic Liver Disease (PLD)

How Is Polycystic Liver Disease (PLD) Defined?

  • Genetic Disease-1
    • Polycystic liver disease is Autosomal Dominant Genetic Mutation Disease
    • Genetic mutation has been observed in isolated liver cystic disease and; polycystic disease involving liver and kidney.1, 2
  • Types of Polycystic Liver Disease (PLD)-
    • Isolated Cystic Disease -
      • Isolated cystic disease affects only liver.
      • Isolated cystic disease is less common than polycystic liver disease.
      • The isolated liver cystic disease is comprised of only 10 to 20% of all liver polycystic liver disease.
    • Polycystic Disease-
      • Polycystic liver disease involves liver and kidney.
      • Polycystic liver disease is more common type of cystic liver disease than isolated cystic liver disease.
      • The disease is observed in 80 to 90% of patient suffering with liver cyst.
  • Cystic Liver-
    • Liver surface shows cystic swelling during ultrasound examination. Swelling appears like grapes projecting on the surface of liver.
    • Multiple cysts are also lodged within the inner liver tissue.
    • Internal cystic growth slowly causes compression of portal vein and liver cells.
    • Surface cyst may grow substantial in size causing compression of surrounding soft tissue.
  • Age-
    • Polycystic disease is rarely observed in children
    • Polycystic liver disease is common among elderly as compared to middle age and younger population.
  • Sex-
    • Polycystic liver disease is common among female patient.
  • Asymptomatic Solitary or Few Cysts-1
    • Solitary cyst is always asymptomatic and diagnosed during routine examination of CT Scan and MRI.
    • Multiple small cysts are also often asymptomatic and may present a symptoms like purities which is often ignored for several years.3
    • Solitary and multiple small cysts are mostly seen in younger patients.
    • Cases has been published of
  • Multiple Cyst-
    • Large Area- Multiple cyst occupies large area of liver and causes compression of normal liver cells.
    • Liver Failure- Compression and destruction of surrounding normal cells by growing cyst may cause liver failure. Liver failure is very rare.
    • Large Cyst- Large cysts cause severe pressure on surrounding organs. Compression or ischemic changes of surrounding organs causes multiple symptoms.
    • Polycystic liver disease is rarely fatal.

Causes Of Polycystic Liver Disease (PLD)

  • Genetic Mutation 4-
    • Polycystic Liver Disease is an inherited medical condition.
  • Female Hormones-
    • Polycystic liver disease is more common among females.
    • Patient diagnosed of polycystic liver disease have shown the number of cyst to increase during pregnancy.
    • Follow up MRI and CT Scans of females diagnosed of polycystic liver disease have shown increased number of cyst when prescribed contraceptive steroid hormones.
    • The number of polycystic liver disease is higher in postmenopausal female who are taking estrogen or progesterone or both the hormones.
  • Age-
    • Higher number of cysts is observed in elderly patients.

Symptoms And Signs Of Polycystic Liver Disease (PLD)

Non-Specific Symptoms of Polycystic Liver Disease (PLD)

  • Loss of weight
  • Loss of appetite
  • Fatigue and weakness

Specific Symptoms and Signs of Polycystic Liver Disease (PLD)-

Symptoms and Signs Caused By Large Cyst-

  • Abdominal Pain-
    • Caused by irritation of peritoneum and distension of liver surface.
    • Abdominal pain is also caused by pressure on surrounding soft tissue.
  • Abdominal Distention-
    • Symptom is caused by pressure resulting in compression of intestine and stomach.
  • Shortness of Breath-
    • Larger cyst elevates diaphragm and compresses lung causing short of breath.
  • Back Discomfort-
    • Larger cyst causes pressure on paravertebral nerves and vertebrae, which results in back pain.

Symptoms and Signs Caused By Liver Failure-

  • Jaundice
  • Esophageal Bleeding- Obstruction of portal vein causes enlarged esophageal vein known as varieces. Enlarged vein may rupture resulting in life threatening bleeding.
  • Encephalopathy- Increased bilirubin level in blood may cause symptoms of encephalopathy resulting in confusion and coma.
  • Ascites- Low serum protein and portal vein hypertension can result in ascites.

Symptoms and Signs Caused By Ischemia-

  • Partial compression of surrounding arterial blood vessels results in ischemic changes of organ.
  • Portal Hypertension- Caused by cyst compressing portal vein.
  • Multiple large cysts within liver tissue cause obstruction of venous drainage.

Symptoms and Signs of Infection-

  • Fever with chills

Diagnosis of Polycystic Liver Disease (PLD)

Diagnoses of Asymptomatic Polycystic Disease-

  • Incidental finding when routine MRI or CT scan study of abdomen is performed

Radiological Studies-

  • CT Scan
  • MRI
  • Ultrasound Examination

Treatment For Polycystic Liver Disease (PLD)

Treatment For Asymptomatic Polycystic Liver Disease (PLD)-

  • Observation-
    • Watch the progress of the disease
  • Treatment-
    • Treatment is avoided if disease is asymptomatic
    • Large asymptomatic cysts are often removed to prevent liver failure.

Treatment For Symptomatic Polycystic Liver Disease (PLD)-

  • Aspiration of Cyst 5
    • Aspiration of the cyst is performed to reduce size of the cyst
    • Procedure is performed to decompress the cyst to eliminate pressure on surrounding organs.
    • This procedure is temporary, since cyst will grow back to original or larger size.
    • Needle placement inside the cyst is performed under the guidance of MRI or CT scan.
  • Sclerotherapy 5
    • The cyst to be treated is identified with MRI and CT scan.
    • Needle is placed inside the cyst under the guidance of CT scan, ultrasound or MRI.
    • Cyst is aspirated once the needle is placed inside the cyst.
    • Following aspiration phenol or alcohol is injected in the cyst to form the scar tissue so cyst walls will adhere to each other and cyst will collapse.
    • Recurrence of cyst is rare.
  • Excision of Cyst or Liver Resection 4, 5
    • Large cyst is excised by open surgery.
    • The liver and cyst is exposed following laparotomy and cyst is isolated.
    • Cyst is carefully dissected and removed.
    • Cyst may be difficult to isolate and remove. In such cases, partial liver resection is performed.
  • Liver Transplantation4
    • Indication- If all other treatments fail to relieve symptoms.

References:

1. Advances in management of polycystic liver disease.

Everson GT1, Helmke SM, Doctor B.

Expert Rev Gastroenterol Hepatol. 2008 Aug;2(4):563-76.

2. Autosomal recessive and dominant polycystic kidney diseases.

Sessa A1, Righetti M, Battini G.

Minerva Urol Nefrol. 2004 Dec;56(4):329-38.

3. Polycystic liver disease presenting as pruritus.

Johnson DK, Panchili S, Kolasseri S, Mavali RT., Ann Gastroenterol. 2014;27(1):76-78.

4. Management of polycystic liver disease.

Everson GT1, Taylor MR.

Curr Gastroenterol Rep. 2005 Feb;7(1):19-25.

5. Current treatment status of polycystic liver disease in Japan.

Ogawa K1, Fukunaga K, Takeuchi T, Kawagishi N, Ubara Y, Kudo M, Ohkohchi N.Hepatol Res. 2013 Dec 6.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: June 10, 2014

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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