congenital liver cyst
Introduction
Introduction to congenital hepatic cyst Congenital hepatic cyst (congenitalcystofliver) is a common clinical liver benign disease, belonging to congenital dysplasia, clinically often divided into multiple hepatic cysts or polycystic liver (multiple cysts polycystic disease of liver) and single hepatic cysts. basic knowledge Sickness ratio: 0.01%-0.02% Susceptible people: no specific population Mode of infection: non-infectious Complications: abdominal pain cholangitis
Cause
Causes of congenital hepatic cysts
(1) Causes of the disease
It is not very clear, there are two views: one is the developmental intrahepatic bile duct or lymphatic development disorder, or the intrahepatic vagus bile duct formation; the first is the intrahepatic infection caused by cholangitis, causing intrahepatic small bile duct atresia, proximal The small bile duct gradually expands cystically, forming cysts, and congenital developmental disorders can be caused by heredity, such as adult polycystic liver disease (APLD), which is an autosomal dominant hereditary disease.
According to the etiology of Debakey, congenital hepatic cysts can be divided into two major categories: primary hepatic cysts and primary biliary cysts. The former are divided into: 1 isolated hepatic cysts (can be divided into single or multiple Hepatic cysts; 2 multiple (polycystic) hepatic cysts (ie polycystic liver), the latter are divided into: 1 localized intrahepatic bile duct dilatation; 2 intrahepatic bile duct multiple cystic dilatation (ie Calori disease) This article only describes congenital hepatic cysts, or true cysts, to distinguish between pseudocysts caused by trauma, inflammation, and tumors.
(two) pathogenesis
Single hepatic cysts can be small or large, the small ones are only a few millimeters in diameter, the larger ones can reach more than 20cm in diameter, and even occupy the entire liver, but the liver cysts seen in the clinic are large, containing fluid. The amount is often above 500ml, up to 17,000ml. In one case of Shanghai Oriental Hepatobiliary Surgery Hospital, there is 1 case of cystic fluid up to 5000ml. The cyst is round or oval, mostly single-room and multi-atrial. Sometimes there are pedicles, cysts have a complete capsule, the surface is milky white, there are also blue-gray, the wall thickness is different, the thick can reach 0.5 ~ 5.0mm, the inner layer is columnar epithelial cells, the outer layer is fibrous tissue It is covered with a large bundle of biliary vascular bundles. The surrounding hepatocytes are often atrophied and degenerated due to compression. The cyst fluid is mostly clear and transparent or mixed with bile. If it is combined with intracapsular hemorrhage, it may be brown, and the cyst fluid is neutral or alkaline. The density is 1.010~1.022, containing a small amount of albumin, mucin, cholesterol, red blood cells, bilirubin, tyrosine or bile.
Multiple hepatic cysts are more common than single-shot cysts. The size of cysts is different. The smaller ones are mung beans or can only be found under the microscope. The larger ones can reach more than 1000ml. The cysts can be scattered in the whole liver or in the liver. The leaf is more common in the right liver. The cut surface of the specimen is changed in a honeycomb shape. The wall of the capsule can be divided into two layers. The inner layer is epithelial cells. The shape can vary depending on the size of the cyst. The cyst is larger due to the compression of the epithelial cells. Columnar cells and goblet cells are flat or disappear; medium-sized cysts, only goblet cells; small cysts, columnar cells and goblet cells, the outer layer of collagen-like tissue, a large number of cysts can be seen Small bile ducts and normal liver cells, the cystic fluid is clear and transparent, generally does not contain bile. Multiple hepatic cysts rarely cause portal hypertension and esophageal varices, but may be combined with bile duct stricture, cholangitis and hepatitis. In the advanced stage, liver function damage may occur. Ascites, jaundice, splenomegaly, esophageal varices or abdominal varicose veins.
Prevention
Congenital hepatic cyst prevention
Do a good job of pregnancy check, early detection of congenital hepatic cysts, and targeted treatment.
Complication
Congenital hepatic cyst complications Complications abdominal pain cholangitis
Complications are rare. The most common symptoms are intracapsular hemorrhage. The clinical manifestations are sudden and severe abdominal pain and cyst enlargement. This kind of complication is almost seen in women over 50 years old, but in a very small number of patients, the abdominal pain is mild or not. Under hemorrhage, the contents of the capsule were observed to be fluid under ultrasound. In addition, when the cyst was ruptured and there was infection in the capsule, chills and high fever could occur. The duodenum could still form internal hemorrhoids; portal hypertension, etc. Kasai et al. Three cases of hepatic cysts were reported to have cancerous changes. It was pointed out that if the cysts were found to be turbid, the irregular wall nodules should be alert to malignant changes. In addition, multiple cysts may be complicated with bile duct stricture and cholangitis.
Symptom
Symptoms of congenital hepatic cysts Common symptoms Abdominal mass jaundice Upper abdomen pain Liver enlargement Severe pain Portal hypertension Painful ascites Hemorrhagic bile retention
Patients with congenital hepatic cysts are often asymptomatic when they are young. Symptoms gradually appear after 35 to 40 years of age. They are characterized by upper abdominal masses, pain in the liver area and upper abdomen, usually painful. If there is intracapsular hemorrhage, it can also be accompanied by severe abdominal pain. Cysts compressing adjacent organs can also cause reduced eating, pain, vomiting, jaundice, etc. A small number of severe hepatic cysts can also cause ascites, portal hypertension, and patients with other organ cysts can show some performance, often early in clinical examination There is no positive finding. For larger hepatic cysts, the body can touch the right upper quadrant, the surface is smooth, the quality is tough, and the infection may be accompanied by tenderness.
Examine
Examination of congenital hepatic cysts
Congenital hepatic cysts without comorbidities, even if the cysts are large, usually do not affect liver function, therefore, laboratory tests rarely have positive results, when comorbidities, patients can be expressed as liver bilirubin, transaminase, alkali Sex phosphatase, transpeptidase increased; patients with co-infection can appear white blood cells and elevated levels of performance.
1. X-ray: Abdominal plain film can be found that the cystic wall of the hepatic cyst is curved or full-vessel calcification; pneumoperitoneography sometimes shows a smooth and neat protruding patch on the surface of the liver; when the cyst compresses the gastrointestinal tract, barium meal can be seen The gastrointestinal tract can be seen; arterial angiography can show that the arteries branch around the cyst is spherical, no tumor blood vessels, and there is a clear filling defect area in the parenchyma. The general X-ray imaging has certain diagnostic significance for hepatic cysts, but there is no Specificity, generally not used.
2. B-ultrasound: a circular or elliptical anechoic area in the liver, which can be single or multiple, scattered in the liver parenchyma, the wall of the capsule is a thin band of light echoes, and the surrounding tissue is clearly demarcated, multi-atrial There may be single or multiple spaced bands of light in the cystic sac, the thickness and width are different, the diagnosis of ultrasound is easy, the accuracy and specificity are high, and it is easy to follow up, which is helpful for identification of extrahepatic abdominal cysts. Kidney B super is listed as a routine.
3. CT: A typical congenital hepatic cyst presents a smooth, sharp, rounded or elliptical low-density shadow with a CT value similar to or slightly higher than water. The cyst is close to the hepatic envelope or adjacent to each other. It shows a very thin cyst wall, even thickened and calcified cyst wall, the cyst is mostly single-atrial, even visible separation, no enhanced performance in the cyst after intravenous injection of contrast agent, CT scan if hepatic cyst is found, especially In the case of polycystic lesions, the kidneys, spleen and pancreas should be routinely scanned. If multiple cysts are also found, it can be used as an auxiliary diagnosis basis for APLD.
4. Radionuclide imaging: With the use of radionuclide 198Au, 131I and other liver scans, it is helpful to determine whether there is a space-occupying lesion in the liver. It is helpful for identifying cysts in the liver or outside the liver. Round or large radial defects or sparse, which is helpful for the diagnosis of cyst location.
5. MRI: The sensitivity of diagnosis of hepatic cysts is higher than that of CT. It can show cysts of 1cm in size and can distinguish cystic dilatation of bile ducts. However, MRI is difficult to differentiate between hepatic cysts and cavernous hemangioma.
Diagnosis
Diagnosis and diagnosis of congenital hepatic cyst
diagnosis
Isolated hepatic cysts often have no clinical symptoms. They are rarely diagnosed before birth. Generally, they should be noticed when they are large enough to cause compression symptoms or complications. Therefore, patients with large liver and asymptomatic disease without liver damage should When thinking about the possibility of cyst disease, individual should be identified with huge ovarian cysts. When hepatomegaly is found in patients with polycystic kidney disease, the diagnosis of polycystic liver disease should be particularly suspected. The true polycystic liver should be associated with intrahepatic bile duct. Dilation identification.
Differential diagnosis
Bacterial liver abscess
Acute onset, more chills and relaxation type hyperthermia, body temperature can reach 39 ~ 41 ° C; liver area or upper abdomen pain, sometimes involving the shoulder; liver swelling is located under the costal margin, the tenderness is obvious, the intercostal space is fixed Painful area; white blood cell count increased, there may be nuclear left shift; blood bacterial culture half positive; B-ultrasound: honeycomb hypoechoic network structure or liquid dark area, blurred edge of the lesion, rough echo, irregular; CT: It is a round or round-shaped low-density area. Although the density is similar to that of cysts, after the contrast agent is enhanced, the abscess is generally strengthened, forming a reinforcing ring. The separation of the multi-atrial cyst is also enhanced. The presence of a target or dual target is a characteristic CT manifestation of hepatic abscess, and a pussy that is odorous by a diagnostic puncture guided by B-ultrasound can be diagnosed.
2. Amoebic liver abscess
Slow onset, long course of disease, low fever, history of dysentery before onset, liver enlargement is significant and tenderness is obvious; serum immunoassay is positive; B-ultrasound, CT and other imaging findings are similar to bacterial liver abscess; B-guided diagnosis Sexual puncture can be diagnosed by taking chocolate-like pus.
3. Hepatic hydatid disease
Most of the patients are from pastoral areas, and the onset is slow. There are often irregular pains in the upper abdomen. The texture of the upper abdomen is different. It can be sexy or tough. Sometimes it can be characterized by characteristic echinococcosis (hydatid thrill). ); blood picture can be expressed as eosinophilia; indirect hemagglutination test, positive complement test, positive rate of intradermal allergy test, but also a lot of false positive; B-ultrasound: lesion wall more than double Structure, thicker wall, there may be circular dark areas (agrons) of different sizes in the cyst cavity, such as small round dark areas (large sac small sac) in the dark dark area, Characteristics of the disease, the water canal sign in the cystic cavity can be seen when the inner capsule falls off; CT: the wall of the capsule has a slightly higher density of annular shadows, and most cysts have a higher density of the mother capsule and a lower density of the ascus in the cyst. Internal, is a characteristic manifestation of hepatic hydatid disease.
4. Liver tumors with cystic lesions
It is mainly identified by imaging diagnosis: 1 liver cystadenoma or cystadenocarcinoma: under the B-ultrasound, there is a cystic cavity with no echogenic area, but the cyst wall is not smooth, and a part of the part has a relatively strong echo of the papilla into the cystic cavity. If it is malignant, the follow-up can be seen in the cavity with irregular flocculent mass-like structural echoes, gradually filling the cystic cavity and transforming into a non-uniform disordered echo of cystic mixture; 2 metastatic liver cancer and part of the original When the central liver liquefaction necrosis occurs in the hepatocellular carcinoma, B-ultrasound is surrounded by a layer of hypoechoic dark circles around the enhanced photosphere, while the center of the cluster presents another echo-free or hypoechoic region, the so-called target sign or cattle. Bull eye sign.
5. Cysts in the rest of the extrahepatic abdominal cavity
Giant hepatic cysts sometimes need to be differentiated from pancreatic cysts, common bile duct cysts, hydronephrosis, mesenteric cysts, and large ovarian cysts. Generally, it is not difficult to identify by asking about medical history, physical examination, B-ultrasound, CT, etc.
