polycystic liver
Introduction
Introduction of polycystic liver Polycystic liver disease, more than half of patients with polycystic kidney disease, polycystic liver often invade the entire liver, and a few patients with polycystic liver disease are limited to one or half of the liver. The majority of polycystic liver disease is congenital, that is, the formation of hepatic cysts due to some abnormalities in congenital development. Patients with polycystic liver disease usually have symptoms until 30-50 years of age, and female patients with large cysts can interfere with the delivery process. The cyst gradually grew slowly. The disease complained of abdominal swelling and he felt the lumps. If the cyst oppresses adjacent organs, the symptoms may appear. Excessive abdominal pain can occur unless there is a combination of intracapsular hemorrhage, rupture, or rupture of the cyst. Sometimes there may be abdominal fullness or heavy pressure, loss of appetite, nausea and vomiting. Occasionally caused by biliary obstruction caused by jaundice. Treatment is mainly a low-copper diet, reduced copper absorption, increased copper excretion, and liver transplantation. The preferred drug is D-penicillamine. Liver transplantation is the best way to treat the disease. basic knowledge The proportion of illness: 0.005-0.08% Susceptible people: more common in 30-50 years old Mode of infection: non-infectious Complications: cirrhosis
Cause
Polycystic liver disease
Congenital dysplasia (30%):
Most of the polycystic liver disease is congenital and is caused by intrahepatic bile duct developmental disorders. During embryonic development, the excess bile duct degenerates itself without being connected to the distal bile duct. If the extrabiliary bile duct does not degenerate and absorb, and gradually expands in segments and sacs, polycystic liver can be formed. Polycystic liver disease is often accompanied by polycystic kidney disease, pancreatic cyst, lung or spleen cyst and other malformations, such as cerebral aneurysms, diverticulum, double ureter, horseshoe kidney or ventricular septal defect, etc., can also be used as evidence for its congenital dysplasia. Polycystic liver can occur in different members of the same family and is non-dominant.
Genetic (25%):
It is a phenomenon similar between biological parents and offspring, and between offspring. If the symptoms appear in an infancy or childhood are autosomal recessive disorders, adult polycystic liver is autosomal dominant.
pathology:Most of the polycystic liver cysts are multiple, a few are single, but also due to multiple atrial fusion into a single. Most of the polycystic liver affects the whole liver. The capsule contains clear bile-free liquid. The liver is enlarged and deformed. The liver surface can be seen as gray-white cysts of different sizes. It can be as small as a needle tip, as big as a child's head, and the liver section is honeycomb. . Cysts can also be concentrated in one lobe of the liver, with more involvement in the right lobe. The size of the cyst can range from 8 to 10 cm in size, but rarely exceeds 10 cm. The cyst fluid can be as much as 2000ml. There may be clear liquid, serum or gelatinous liquid in the thin wall of the capsule. It may be bloody or purulent when there is bleeding or infection. It does not contain bile, and the specific gravity is usually 1.010.
Cysts generally increase slowly with age, but liver volume can remain unchanged for years. When the cyst is ruptured, it can fuse into larger cysts. Histology showed no change in hepatic lobular structure, normal hepatocytes, cyst surrounded by fibrous envelope, and its cyst wall was composed of collagen connective tissue, lined with columnar epithelium or flat bile duct epithelium, and the outer layer was collagen-like tissue. If there is more fluid in the capsule, the epithelium can be flattened or even absent. In addition to liver and kidney, other organs such as pancreas, spleen, lung, ovary, seminal vesicle may also have cysts, which may also be associated with other congenital malformations including spina bifida and multi-finger deformity. The cysts are generally normal liver tissue. Fibrosis and bile duct hyperplasia can also occur in patients with chronic disease, and liver function damage, cirrhosis and portal hypertension can be caused in the advanced stage.
Prevention
Polycystic liver disease prevention
1. In normal times, care should be taken to avoid collision between the right intercostal space and the right upper abdomen to avoid cyst rupture.
2. Because the father or mother has the disease, it will cause the child to have the disease. The cyst can not be found in infancy and childhood, and can not be detected during pregnancy. Therefore, if you have parents and relatives of this disease, you need to pay more attention. 3, family history should pay attention to diet, do not drink, but not irritating food, life, work, rest are regular, not too tired. In addition, you can go to the hospital regularly to check and treat according to the actual situation. 4, if the volume is small, no clinical symptoms can be observed, symptoms or large volume can be actively surgical treatment, regular review.Complication
Polycystic liver complications Complications cirrhosis
Cysts generally increase slowly with age, but liver volume can remain unchanged for years. When the cyst is ruptured, it can fuse into a larger cyst. When the cyst of the liver is large enough to affect health, such as digestive malabsorption, or complications such as cyst rupture and infection, consider surgical treatment. If you have acute severe abdominal pain, hematemesis, or black stool, you should go to the hospital. Fibrosis and bile duct hyperplasia can also occur in patients with chronic disease, and liver function damage, cirrhosis and portal hypertension can be caused in the advanced stage.
Symptom
Polycystic liver symptoms Common symptoms Indigestion nausea and vomiting Upper abdomen mass appetite decreased liver enlargement Right upper abdominal pain
After suffering from multiple hepatic cysts, the first manifestation is gastrointestinal symptoms. Such as indigestion, loss of appetite, nausea, vomiting and upper right abdominal pain, but not to a degree. The liver is swollen and the right upper quadrant is swollen, and it is sexy and has no obvious tenderness. There may be chills and fever after secondary infection.
The liver surface of multiple hepatic cysts can reach scattered cystic nodules. Such as intracapsular hemorrhage, combined infection or pedicle cyst torsion, may have acute abdominal performance. It is rare to have a large cyst to compress the common bile duct or hepatic duct with jaundice. There may be no positive signs, and some patients may touch the abdominal mass.
Examine
Polycystic liver disease examination
Laboratory inspection
Cysts and hemorrhage occasionally anemia, white blood cell count is generally normal, but also up to 76 × 10 / L. Changes in liver function tests were not proportional to liver enlargement, with only occasional indirect bilirubin and mild increase in BSP. Alkaline phosphatase increase is only seen in some cases. Blood urea nitrogen and creatinine can be increased in polycystic kidney and kidney units.
Film degree exam
B-ultra-visible dark areas and cysts of different sizes can be seen in the liver scan. Gastrointestinal barium meal is seen in the stomach and duodenum, colon displacement. Intravenous pyelography can have signs of polycystic kidney disease. Laparoscopy showed an enlarged liver with many cysts on its surface. Liver biopsy sometimes results in cystic wall tissue. CT shows a round, low-density lesion with a clear shape and clear boundary. The edge is linear, the density of the capsule is uniform, and there is no compartmentalization. The flat scan can confirm the diagnosis. After strengthening, the contrast is clearer. When bleeding or secondary infection, CT Value >20Hu. Hepatic angiography sees the avascular zone, forcing the blood vessels to shift.
Clinically, for the age of more than 30 years old, the appearance is healthy, hepatomegaly, liver function is normal and accompanied by polycystic kidney disease. Family history, suspected of polycystic liver disease. After the symptoms of oppression and special examinations above the line, if the diagnosis is still suspicious, it is feasible to explode.
Diagnosis
Polycystic liver diagnosis
diagnosis
Diagnosis can be performed based on clinical performance and laboratory tests.
Differential diagnosis
The identification of polycystic liver and simple hepatic cysts depends on family history, and evidence of the presence of other organ cysts helps to identify the two.
The identification of polycystic liver disease and hepatic hydatid disease depends on the epidemiological history. The latter also has cyst wall calcification, eosinophilia, Casoni test and echinococcosis complement test positive.
The difference between polycystic liver disease and liver cancer needs to be examined. Although liver cancer can be liquefied, there may be multiple liquefaction sections, but the liver enlarges very rapidly, and the liver texture is hard. The CT enhanced scan shows that the blood supply is substantial. And there are other features of liver cancer that are easy to distinguish.
