Congenital liver cyst in children
Introduction
Introduction to congenital hepatic cyst in children 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 The proportion of illness: 0.02% Susceptible people: children Mode of infection: non-infectious complication:
Cause
Causes of congenital hepatic cyst in children
(1) Causes of the disease
Hepatic cysts are mostly congenital, often combined with other organ cysts, such as renal cysts, foreign scholars have investigated 184 patients with renal cysts and their families, found that 173 autopsy, 64 patients with hepatic cysts, and 70% The family of renal cysts has a family history, and he suggests that the two diseases are caused by a single gene.
(two) pathogenesis
Regarding the pathogenesis, hepatic cysts are generally thought to be caused by abnormal bile duct development in the hepatic hilar region of the embryonic period. Moschowitz conducted a study and found that the cystic wall of the fetal cystic liver is lined with bile duct epithelium and rectangular epithelial cells, which he believes to be due to the vagus bile duct epithelium. Combined with inflammatory hyperplasia and obstruction, the contents of the lumen are retained to form cysts. Some scholars believe that the extrahepatic bile ducts do not degenerate spontaneously during embryonic development, and do not connect with the distal bile ducts, forming cysts, and the physiology of congenital hepatic cysts. The situation has been less well understood in the past. Everson et al. (1990) conducted a percutaneous balloon infusion and a secretin test in clinical patients. (Considering cystic endothelium is a bile duct epithelial cell, secretin regulates epithelial secretion. The results showed that the electrolyte composition of the cyst fluid was the same as that of the serum. The -GT of the cyst fluid was higher than the serum level, the glucose content was low (<0.83 mmol/L, ie 15 mg/dl), and the component secreting IgA was present, indicating that the bile duct was present. The expression of epithelial cells was the same; the intracapsular pressure was measured to be 16-40 cmH2O, which was higher than the pressure in the biliary tract, indicating that the secretion of cyst fluid was active, and the cyst fluid Containing IgA, IgG, activated complement G, injection of secretin within 10min, the secretion of cystic fluid continues to increase, the secretion of cystic fluid of congenital hepatic cyst may be regulated by endocrine, but lack of insight, congenital hepatic cyst can be isolated Single hair involving a leaf or multiple diffuse involvement of the whole liver, cysts vary in size, the largest can reach tens of centimeters, the small can be like a needle tip, a huge hepatic cyst (the largest cyst containing cystic fluid reported to be 18,000 ml) can cause Obvious symptoms, such as abdominal distension, compression, physical activity and limited food intake; cysts themselves can also have complications, such as intracapsular hemorrhage caused by swelling of the cyst causing abdominal pain; other rare complications are cystic perforation, bacterial infection, biliary and due to Obstructive jaundice caused by cyst compression, portal hypertension, compression of the inferior vena cava, etc., the liver has a strong proliferative compensatory ability, so generally does not cause cirrhosis or liver failure, the simple cyst of the liver generally does not malignant, but There are also records of malignant transformation of simple hepatic cysts (Ameriks, 1972), with a thin wall, lined with monolayers of cubic or columnar epithelial cells, and bile duct epithelial cells. Like, the cells have no hyperplasia or dysplasia (atypsia) phenomenon, the cysts are mostly clear serous liquid, but also can be yellowish, but can not detect bile pigment; with intracapsular hemorrhage can be coffee-like turbidity, generally not It is bright red, accompanied by intracapsular infection. The cyst fluid contains a large number of white blood cells. During the process of gradual expansion and expansion, the cyst compresses the adjacent liver tissue, causing the cyst-containing liver to shrink, and the remaining liver tissue is compensatory due to Under pressure, the liver cells disappear, but the blood vessels and bile ducts do not disappear, and protrude into the sac and become wrinkled, forming the wall of the cyst. Therefore, there is a large number of vascular structures on the wall of the capsule. The specific gravity of the cystic fluid is 1.010~1.022, and there are traces. Protein, bilirubin, cholesterol, glucose and various enzymes such as -GT, lactate dehydrogenase (LDH), etc., contain IgG, IgA in the protein, indicating that the cystic epithelium has a secretory function.
Prevention
Congenital hepatic cyst prevention in children
According to the survey data, there is a family tendency, and there may be multiple organ cysts. At present, there are no effective preventive measures. Regular examination should be performed during pregnancy. If the child has obvious tendency or abnormality of multiple cysts, especially large multiples. Sexual cysts should be aborted in time to avoid the birth of children with the disease.
Pregnant women should avoid harmful factors as far as possible, including away from smoke, alcohol, drugs, radiation, pesticides, noise, volatile harmful gases, toxic and harmful heavy metals, etc., so as not to cause genetic mutations.
Complication
Pediatric congenital hepatic cyst complications Complication
Complications are rare. The most common symptom is intracapsular hemorrhage. The clinical manifestations are sudden and severe abdominal pain and cyst enlargement. However, in a very small number of patients, the abdominal pain is mild or not, and the contents of the capsule are observed to be fluid under ultrasound. In addition, when the cyst is ruptured and there is a concurrent infection in the capsule, there may be chills and high fever; compression of the duodenum can still form internal hemorrhoids; portal hypertension, etc. In 1977 Kasai et al reported three cases of hepatic cyst wall cancer, indicating if It was found that the cyst was turbid, and the irregular wall of the cyst should be alert to malignant changes. In addition, multiple cysts may be complicated with bile duct stricture, cholangitis, obstructive jaundice, portal hypertension, and inferior vena cava compression.
Symptom
Congenital hepatic cyst symptoms in children Common symptoms Ascites abdominal mass severe pain internal bleeding jaundice pain upper abdominal pain
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 in children
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. B-ultrasound is the main method for the diagnosis of congenital hepatic cysts. It is non-invasive and accurate. Most congenital hepatic cysts can be diagnosed by B-ultrasound. B-ultrasound can determine the size, number and distribution of lesions, and can identify liver. External cysts, biliary cysts, parasitic cysts, liver abscesses and tumor liquefaction; at the same time, B-ultrasound can also be treated.
2. Computer tomograph (CT) is an effective diagnostic tool, just like B-ultrasound. CT scan can show the size, shape, location, distribution of cysts and other organs in the abdominal cavity. Can provide a more comprehensive liver scan film, therefore, more objective and comprehensive, in recent years, the application of CT positioning liver puncture technology to participate in the treatment of congenital hepatic cyst, other magnetic resonance, radionuclide scan, abdominal plain film Selective celiac angiography, gastrointestinal angiography, etc. can also be used for the diagnosis and differential diagnosis of congenital hepatic cysts, but compared with B-ultrasound and CT scan, there is no advantage, laparoscopy is an invasive examination, for the liver surface Cysts can be diagnosed and treated with fenestration. However, it is difficult to observe cysts in the liver parenchyma, so it is rarely used clinically.
Diagnosis
Diagnosis and diagnosis of congenital hepatic cyst in children
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
1. Other cystic masses in the liver such as intrahepatic biliary cyst, liver abscess, liver parasitic cyst (hydatidosis) and liver tumor liquefaction necrosis, etc., sometimes need to be identified clinically, and some cases are misdiagnosed as liver The cyst is treated, the intrahepatic biliary cyst is punctured into bile, the ERCP is developed, the liver abscess generally has a history of infection, the puncture liquid is purulent, and the culture has bacterial growth; the liver echinococcosis is positive for the serum complement test (Cassonis test). Liver tumors are accompanied by other symptoms and biochemical changes.
2. Extrahepatic cysts such as choledochal cyst, pancreatic cyst, mesenteric cyst and gallbladder hydrops, clinical combination of special examination including B-ultrasound, CT scan, ERCP, etc., general differential diagnosis is not difficult, but some cases are still misdiagnosed until surgery The diagnosis can be confirmed at the time of treatment.
3. According to medical history, physical signs and laboratory tests on liver function and liver cancer, it can be differentiated from hepatocellular carcinoma.
