Cystic liver disease ( PCLD – polycystic liver disease) is a rare liver disease in which the liver is riddled with cysts (cavities filled with fluid). Gene mutations on chromosomes 6 and 19 are known to cause cystic liver, so cystic liver is a hereditary disease. A cystic liver should not be confused with a liver cyst.
What is the cyst liver?
A cystic liver is usually a congenital malformation of the liver. The organ is interspersed with cysts. For hyperesthesia definition and meaning, please visit howsmb.com.
In contrast to the much more common liver cysts, cystic liver is a very rare disease that is often genetic.
The disease is characterized by slow, progressive expansion of the liver and possible abdominal changes.
Cyst liver is generally a benign disease that occurs primarily in women over the age of 40.
Causes
The most common cause of cyst liver is the so-called autosomal dominant hereditary polycystic kidney disease (abbreviated: ADPKD).
About 58 percent of all affected patients have already developed a cyst liver by the age of 20, 85 percent by the age of 30 and 95 percent by the age of 40. A cystic liver is usually also observed in ADPKD, with polycystic kidney disease occurring more frequently than autosomal dominant hereditary polycystic liver disease. In ADPKD, renal function deteriorates due to cystic remodeling of the renal tissue.
As a result, kidney failure occurs, which can only be treated with the help of dialysis as long as no donor organ is available. Among other things, affected patients also develop malformations of the vessels in the cerebral arteries ( aneurysms ), changes in the heart valves and benign protuberances in the colon wall. The disease course of polycystic kidney disease is similar to the disease course of cystic liver.
Symptoms, Ailments & Signs
Cystic liver is usually a benign condition that initially causes no symptoms. Due to a genetic defect, however, the liver is interspersed with cysts from birth. However, this does not initially affect the liver function. However, a cyst liver has the property of becoming larger and larger over time.
This increases the liver mass. Due to their increasing size, they can later press on the stomach and intestines and cause these organs to be crowded out. Then there are the typical symptoms caused by the mass. Symptoms include an early feeling of satiety, loss of appetite, nausea, vomiting and weight loss.
Furthermore, the increase in size of the liver can increasingly lead to shortness of breath and heart problems ( cardiac arrhythmia ). Irrespective of this, however, the liver function remains intact for the time being. However, this can be increasingly limited with further cyst formation. Any liver dysfunctions are then caused by impaired liver blood flow or bile drainage.
Bile drainage problems also result in yellowing of the skin and eyes ( jaundice ). Bleeding from the cysts is very rare. Ruptures and infections of the cysts can also occur. Therefore, despite the generally good prognosis of the cyst liver, a surgical intervention is sometimes necessary in individual cases to alleviate the symptoms. If the liver is completely riddled with cysts, however, liver function can fail completely. Then a liver transplant must be performed.
Diagnosis & History
Cystic liver is primarily manifested by a slow increase in the size of the organ due to the increasing number and size of the cysts. This usually leads to a displacement of other organs in the body.
A cyst liver is usually about ten times larger than a normal organ. Typical symptoms of a cystic liver include pain, an increase in abdominal circumference, an early feeling of satiety, nausea and vomiting, and abdominal wall hernias. Under certain circumstances, those affected may also experience shortness of breath and cardiac arrhythmias.
If the contents of the cyst become infected or the cyst ruptures (both with and without bleeding), serious complications can occur. In rare cases, the cyst liver involves a severe impairment of the liver function.
An ultrasound (sonography) is the safest way to diagnose a cyst liver. If other family members are already affected by the disease, the medical history can provide further information on the exact type of cystic liver. Computed tomography or magnetic resonance imaging can also provide further information, especially in the case of existing symptoms. Elevated laboratory parameters (e.g. bilirubin) or an elevated tumor marker (CA 19-9) alone do not allow any statements to be made about the severity of the disease.
Complications
A cystic liver can cause various complications. If the liver disease is not treated in time, it can lead to cardiac arrhythmias, shortness of breath and gastrointestinal problems. Further growth of the cyst causes abdominal pain and possibly also cramps, always associated with a strong feeling of discomfort and illness.
If the cyst’s contents become infected or a cyst ruptures, it can cause serious complications. First of all, there is a risk that the entire liver will become inflamed, which will severely impair its ability to function. It can also lead to blood poisoning, which in the worst case can be fatal.
When treating a cystic liver, the main risks come from surgical procedures. A liver transplant carries the risk that the body will reject the new organ. Infections, allergic reactions and secondary diseases such as osteoporosis can also occur.
The removal of individual cysts can lead to bleeding, injury or inflammation, which in turn is associated with far-reaching complications. Finally, the administration of antibiotics and other medications can lead to serious side effects and interactions. If taken for a longer period of time, there is a risk of permanent organ damage.
When should you go to the doctor?
If there is a diagnosed genetic defect within the family, a comprehensive genetic test should be carried out immediately after the birth of the offspring. It must be checked whether the existing genetic mutation has been passed on to the offspring. If various health irregularities become apparent in the further course, a doctor’s visit is also necessary. Swelling, a feeling of tightness in the organism or general limitations in functional activities are worrying. In the case of disorders of the gastrointestinal tract, pain, nausea or vomiting, the cause must be clarified.
Changes in weight, a loss of appetite and a general feeling of illness or inner weakness are further signs of an existing illness. Difficulty breathing, cardiac arrhythmia and changes in the complexion should always be presented to a doctor. A yellow appearance of the affected person is characteristic of the cyst liver. A doctor should be consulted immediately before complications can arise.
If states of anxiety, insomnia or inner restlessness develop due to the disturbances in breathing activity, it is advisable to consult a doctor. A medical examination is required to make a diagnosis. A treatment plan is necessary to alleviate the symptoms. The constant increase in health impairments should be understood as a warning signal. Therefore they should be checked. If left untreated, life-threatening developments can occur.
Treatment & Therapy
If there are no symptoms, the cyst liver does not necessarily have to be treated. However, if treatment is to be carried out, this can be done by covering the cysts laparoscopically, removing individual segments of the liver or using a combination of both techniques.
If the liver is massively interspersed with cysts and this leads to a restriction of the liver function, a liver transplant is carried out in exceptional cases. Which procedure is actually used depends on the symptoms and complaints of the patient concerned. Surgical interventions can usually stop 90 percent of the symptoms, but a recurrence of the symptoms due to further growth of the cysts cannot be ruled out.
Patients who want to avoid surgery can resort to puncture of individual cysts, sclerotherapy or medication. A puncture – especially of individual large cysts – can usually lead to at least short-term relief of the symptoms, but there is also a 100 percent chance of a relapse. The probability of a recurrence is usually lower if the cysts are sclerosed.
Both procedures are only limited to a small number of cysts and there is usually no significant improvement in symptoms. Even if the intake of medication is to be viewed critically, primarily due to side effects and therapy costs, medication (e.g. somatostatin analogues) can cause a decrease in liver volume and a slowdown in cyst growth.
Prevention
A cystic liver cannot actually be prevented, especially if it is hereditary. Whether there are preventive measures can only be clarified by the doctor treating you after a detailed medical history has been taken – including information on illnesses in the family.
Aftercare
In the case of a cyst liver, those affected usually have only limited and very few direct follow-up measures available. For this reason, the early diagnosis and subsequent treatment of this disease are paramount in order to prevent the occurrence of other symptoms or complications. As a rule, the cyst liver cannot heal itself, so that the affected person is always dependent on a visit to a doctor.
In many cases, the disease can only be completely cured by liver transplantation. After such an operation, the person concerned should definitely rest and rest. Efforts or stressful and physical activities should be avoided in order not to unnecessarily burden the body. A healthy lifestyle with a healthy diet can also have a positive effect on the further course of the cyst liver.
Those affected should avoid obesity as much as possible. Various medications are often required. This can also limit some of the symptoms. Care should always be taken to ensure that the dosage is correct and that the medication is taken regularly. If anything is unclear or if there are side effects, always consult a doctor first. Under certain circumstances, the disease can also reduce the life expectancy of the patient.
You can do that yourself
In the case of a cyst liver, the possibilities for self-help are in most cases significantly limited and are only very rarely available to the person concerned.
The disease itself can usually only be completely cured by a liver transplant, although the symptoms can also recur after surgery. Those affected by the cyst liver are often dependent on the help and support of their own families in their everyday lives. Psychological support is also very important here, as this can also prevent or alleviate depression and other mental upsets. Regular check-ups by a doctor are also very useful in order to monitor the condition of the liver over the long term.
In general, a healthy lifestyle with a healthy diet can also have a positive effect on the further course of the cyst liver. Those affected should definitely refrain from smoking and also from drinking alcohol in order not to unnecessarily burden the liver. Since the cystic liver may also be hereditary, a genetic examination and counseling should be carried out in any case if you wish to have children in order to prevent this disease from recurring in the descendants.
