Congenital choledochal cyst, also known as choledocholithiasis, is the most common congenital anomaly, which can coexist with other diseases.
What is the reason?
The etiology of congenital choledochal cyst is still controversial. In recent ten years, with the progress of morphological bile enzymology and animal experimental research on the disease, we have a deeper understanding of its etiology. Most scholars believe that the disease is related to the obstruction of distal common bile duct caused by abnormal confluence of biliary and pancreatic ducts.
1. Congenital dysplasia of pancreaticobiliary junction leads to abnormal confluence. The so-called confluence anomaly refers to the congenital malformation of the junction of pancreaticobiliary duct outside the duodenal wall or at the junction of duodenum. Since Babbitt proposed in 1969 that abnormal pancreaticobiliary confluence is the cause of common bile duct dilatation, this theory has been emphasized in recent years. For example, the distal ends of the pancreatic duct and the common bile duct are abnormally connected at right angles to the common bile duct or
2. Biliary duct dysplasia 1936 Yotsu-Yanagi first proposed that in embryonic stage, the proliferation of primitive bile duct epithelial cells is unbalanced, such as excessive proliferation of the distal end, which will narrow the distal end and expand the proximal end during cavitation, thus forming this disease. Congenital elastic fiber deficiency of common bile duct wall will gradually expand when the internal pressure of bile duct increases, and its distal end will not be blocked.
3. Virus infection In recent years, it has been found through histopathological changes that hepatitis B virus, cytomegalovirus and adenovirus can cause obstruction or weakening of bile duct wall, leading to bile duct malformation.
4. Abnormal nerve distribution Some scholars compared the nerve distribution of fetal common bile duct with the distribution of ganglion cells in the distal narrow segment of children with choledocholithiasis, and found that the number of nerve fiber bundles and ganglion cells in the narrow segment of children was significantly lower than that in the control group. Therefore, it is considered that the occurrence of choledocholithiasis is related to the abnormal distribution of nerve plexus and ganglion cells at the distal end of the common bile duct, but whether the abnormal development of ganglion cells is a congenital disease or an acquired secondary disease needs further study.
(2) Pathogenesis
1. Pathological changes: The common bile duct showed spherical cyst or spindle dilatation in different degrees. The diameter of the tube wall ranges from 2 ~ 3 cm to 20 ~ 30 cm, and the thickness of the tube wall ranges from 2 ~ 3 mm to nearly 0.5 cm. The size of the tumor is not proportional to the age of the child. The mucosa and muscularis of the common bile duct can not maintain mucosal shedding, inflammation and infiltration, and the muscularis is variable and hypertrophy. A large number of fibrous connective tissues are stored in the capsule. Thick bile sometimes has sediment-like stones. In some cases, bile bacteria taken out during surgery are positive. Due to long-term chronic bile obstruction, liver cirrhosis can be caused to varying degrees. The liver is brownish green and hard, usually after decompression (relieving "back pressure"). Liver cirrhosis improved in more than 2 ~ 6 months, and the clinical symptoms also improved. However, without surgical treatment, repeated infection can cause chronic intrahepatic cholangitis, increase portal vein pressure of progressive biliary cirrhosis, and secondary splenomegaly and hypersplenism. Rupture of varicose veins in the lower esophagus causes massive hematemesis and bloody stool, which can cause biliary tract infection and stone formation due to the growth of cholestasis bacteria, and can cause extrahepatic bile duct dilatation, cyst rupture and bile overflow, leading to biliary peritonitis. Long-term repeated infection of pancreatic juice reflux can cause malignant transformation of bile duct epithelium or acute and chronic pancreatitis. Therefore, according to its pathological changes, early surgical treatment should be actively carried out.
2. Typing Congenital Bile Duct Dilatation can occur in intrahepatic and/or extrahepatic bile ducts, and choledochal cyst is the most common. However, with the development of imaging diagnosis technology, other types of diseases, especially cystic dilatation of intrahepatic bile duct (Caroli disease), are more common than before, and the classification method proposed by Flanigan is simple and practical.
(1) Cystic dilatation of common bile duct: 77% were cystic or spindle dilatation.
(2) Diverticulum dilatation of common bile duct: it can be diverticulum dilatation of common bile duct or gallbladder, mostly saccular dilatation of the side wall of common bile duct, with narrow base or short pedicle connected with the side wall of common bile duct, accounting for 2% ~ 3. 1%.
(3) Cystic dilatation of the end of the common bile duct: also known as choledocholithiasis, the end of the common bile duct is dilated and embedded in the duodenum, accounting for 65,438 0%.
(4) Cystic dilatation of intrahepatic and extrahepatic bile ducts including Caroli disease accounted for 65,438 08.9%.
(5) Cystic dilatation of intrahepatic bile duct is Caroli's disease.
What are the manifestations of editing this passage and how to diagnose it?
Jaundice, abdominal pain and mass are the three basic symptoms of this disease, but not all patients have three main symptoms in their medical history or when they see a doctor. Clinically only 1 or two patients have all three symptoms, accounting for only 20% ~ 30%.
1. Recurrent abdominal pain The nature and degree of pain in the right upper abdomen or middle upper abdomen are different, sometimes it is colic, traction pain or slight swelling pain. Secondary infection may be accompanied by fever, sometimes nausea and abdominal pain, accounting for about 80% ~ 90%.
2. The tumor is located at the lower upper edge of the costal margin of the right upper abdomen and covered by the hepatic margin. Huge tumors can exceed the midline of the abdomen. As shown in figure 1, the tumor surface is smooth and spherical. Small cystic choledochal cyst is not easy to be found because of its deep position. When infected with pain and jaundice, the tumor can first enlarge and then shrink. About 70% of patients were treated for right epigastric tumor.
About 50% cases of jaundice have jaundice, and the degree of jaundice is directly related to the degree of biliary obstruction. Jaundice usually recurs, accompanied by various infections and fever. Most of the above symptoms are intermittent attacks. Due to the obstruction of the distal exit of the common bile duct, bile duct infection occurred, which relieved the symptoms or disappeared after a few days of treatment. Some children have frequent seizures, and some can have seizures once every few months, except for the above symptoms. Jaundice can be accompanied by nausea and vomiting, and white clay can appear. The urine color of stool is deepened, and some children, especially infants, will have symptoms of acute biliary peritonitis, high fever, abdominal distension and even shock when they have cyst perforation. According to the clinical manifestations of abdominal pain, jaundice and cystic mass in the right upper abdomen, a preliminary clinical diagnosis should be made. Some cases do not have the "three main symptoms", so laboratory and auxiliary examination should be carried out to help diagnose.
What tests should I do?
Most of the patients' hematuria and feces showed obstructive jaundice, and a series of tests of obstructive jaundice were abnormal, including the obvious increase of serum bilirubin, mainly direct bilirubin, alkaline phosphatase and γ -glutamyltranspeptidase, and there were different degrees of acute liver function abnormalities. In a few patients, all the examination indexes are basically normal, and inflammatory changes such as peripheral blood cell count and neutropenia can be seen in those infected in the capsule. A considerable proportion of cases of this disease, especially in the course of spindle, found that pancreatic amylase in hematuria increased and was misdiagnosed as simple acute pancreatitis. There are indeed cases of pancreatitis in clinic, but most cases are due to abnormal pancreaticobiliary confluence, and pancreatic juice will return to bile duct or even intrahepatic bile duct. In the capillary bile duct, pancreatic amylase can return to the blood circulation through the hepatic venous sinus, leading to multiple pseudopancreatitis.
1.b-ultrasound is the simplest and non-invasive examination method, which can initially obtain the hypoechoic area with clear sub-hepatic boundary, determine the size of cyst and the degree of distal bile duct stenosis, and understand the degree and scope of intrahepatic bile duct dilatation and whether it is combined with bile duct. This method has the advantages of no damage, no radiation, convenience, low cost and dynamic observation, and the diagnostic accuracy can reach 94%.
2.CT scanning can determine whether the bile ducts inside and outside the liver are dilated, the location and shape of the dilated bile ducts, the degree of stenosis at the distal end of the common bile duct, and the location and shape of the intrahepatic bile duct, which is helpful for the selection of surgical methods. Lesions can be displayed by cross-sectional images, and tubular structures can be displayed by intravenous injection of iodine contrast agent, which has high qualitative value, similar to B-ultrasound, but it can only pass through bile duct and cannot display the length of obstructive bile duct, and the radiation is expensive, which is a supplement to B-ultrasound.
3. Endoscopic retrograde cholangiopancreatography (ERCP) with duodenal fiberscope inserted through duodenal papilla in children can show the whole picture of pancreaticobiliary duct, especially the abnormal confluence of pancreaticobiliary duct, and provide reliable basis for the choice of treatment methods. For children who need general anesthesia, ERCP injury is relatively small, and adults only need mucosal infiltration anesthesia to have no obvious organic injury. Angiography is easy to succeed and can obtain better diagnostic effect than PTC. At present, ERCP can also be used to diagnose abnormal pancreaticobiliary junction in newborns abroad. More effectively, the contrast agent can be directly injected into bile duct and pancreatic duct through duodenoscopy, and the whole picture of bile duct cyst can be directly displayed. This is a routine examination method for adults, but it requires certain conditions and equipment for children and is widely used in older children.
4. Percutaneous cholangiography (PTC) can:
① Understanding the location of cystic dilatation of intrahepatic bile duct can provide guidance for surgical selection.
② To know whether there is abnormal confluence of pancreatic duct and pathological changes of distal pancreaticobiliary duct.
③ Make a definite diagnosis and understand the degree of proximal and distal bile duct stenosis.
④ Take bile for bacteriological examination. Before puncture, prothrombin time should be checked. If the coagulation mechanism is not good, PTC will inject the contrast agent directly into the intrahepatic bile duct with a fine needle. By developing intrahepatic and extrahepatic bile ducts, biliary tract malformation can be directly observed. However, due to the need for general anesthesia, the risk of puncture failure is high, and ERCP is often used instead.
5. Radionuclide hepatobiliary scanning with 99mTc can visually and dynamically observe the shape and function of hepatobiliary system, as well as the location, size, shape and excretion of bile duct cysts.
6. Barium meal examination of upper digestive tract showed that the diameter of duodenal window was flattened before and after widening, and the left and right diameters were widened. The lateral radiograph shows that the descending part of the duodenum moves forward, as shown in Figure 2, and the diagnosis can be made.
7. When the X-ray cyst of upper digestive tract is large, a soft tissue mass with smooth edge and uniform density can be seen in the upper right abdomen, which can be manifested as the displacement of gastric antrum and duodenum to the lower left, and the curve of duodenum expands into an arc impression; The lateral radiograph shows that the duodenal framework is enlarged and displaced forward due to the compression of the stomach and duodenum, but it is difficult to diagnose the spindle bile duct dilatation by ordinary X-ray examination.
8. Cholangiography Oral or intravenous cholangiography is mostly unclear due to the dilution of contrast media. When the liver function is seriously damaged, it is not appropriate to replace intraoperative cholangiography with more advanced examination methods, which has basically stopped clinically. Direct injection of contrast agent into gallbladder or bile duct shows the nature and scope of the whole intrahepatic and extrahepatic bile duct, which is very helpful for diagnosis and determination of surgical methods.
9. Magnetic resonance cholangiopancreatography (MRCP) is the latest cholangiography method at present. It does not need contrast agent to be processed by computer, leaving only clear three-dimensional images of bile duct and pancreatic duct. It is a high-tech non-invasive imaging technology that matured in the 1990s and was applied in clinic, using magnetic resonance imaging. Vibration, a special imaging technology, can obtain clear imaging effect of pancreaticobiliary duct, and even clearly judge whether there is abnormal pancreaticobiliary duct confluence. In recent years, a large number of clinical studies show that the diagnosis of bile duct dilatation alone is far from suitable for guiding surgery correctly.
What are the confusing diseases in editing this paragraph?
The early stage of this disease should be differentiated from biliary atresia and hepatitis, and sometimes from right hydronephrosis, pancreatic cyst, hepatic hydatid cyst, omentum and mesenteric cyst. B-ultrasound and biochemical examination can generally be used for differential diagnosis, and CTERCP can be used to assist differential diagnosis when necessary.
1. Choledochal cyst with abdominal mass as the prominent manifestation and no jaundice in the right upper abdomen or upper abdomen mass should be differentiated from hepatic cyst, retroperitoneal cyst, hydronephrosis, renal embryonic tumor, omental cyst and mesenteric cyst.
(1) Hepatic echinococcosis: It is different from cholangiectasis in that when patients come into contact with animals such as dogs and sheep in animal husbandry areas, the cysts will gradually increase. Both B-ultrasound and CT showed that the eosinophil count of extrahepatic common bile duct was normal, the positive rate of casoni test (intradermal echinococcosis test) was as high as 80% ~ 95%, and 80% of complement fixation test was positive.
(2) Hepatic cyst: The liver is hard, with nodular feeling and no tenderness. Liver function tests are generally normal. Patients with polycystic liver disease sometimes have polycystic lesions of kidney, pancreas or spleen at the same time. B-ultrasound and CT can clearly show that the cyst is located in the liver and the extrahepatic biliary tract is normal.
(3) Retroperitoneal cystic masses: cystic teratoma, lymphangioma, etc. It is difficult to distinguish it from cystic dilatation of common bile duct without jaundice from symptoms and signs. Besides bile duct dilatation, B-ultrasound CT can basically be distinguished from ERCP.
(4) Right hydronephrosis: Physical examination is not easy to distinguish from bile duct dilatation, but hydronephrosis is often filled in the lateral lumbar triangle, especially with B-ultrasound intravenous pyelography (IVP) or retrograde cholangiopancreatography (ERCP), which is easy to distinguish.
(5) Wilms tumor: The main differences are:
① Children with fast tumor growth, high blood pressure or hematuria are generally more seriously ill.
② The tumor was solid with moderate hardness.
③ X-ray plain film of abdomen showed that the tumor pushed the intestine to the inside, and sometimes there were scattered punctate calcification in the tumor. Intravenous pyelography shows that the renal pelvis and calyx are deformed or crushed without development, or only a small amount of contrast agent appears in the renal pelvis.
(6) Pancreatic cyst: Pseudopancreatic cyst in children is closely related to trauma. Cysts are mostly located in the left upper abdomen or navel, often accompanied by abdominal pain, elevated urine sugar and blood sugar, and elevated or normal serum amylase. It is not difficult to distinguish by B-ultrasound CT or ERCP.
2. The common bile duct cyst with jaundice as the prominent manifestation should be differentiated from the right abdominal retroperitoneal tumor of bile duct atresia cholangiocarcinoma compressing the common bile duct.
(1) Biliary atresia: The main differences are:
① After birth 1 ~ 2 weeks, the child developed cholestatic jaundice, which deepened rapidly, but the urine without gap was dark brown, the stool was light yellow, and then it developed into clay stool.
② Yellow staining of skin and sclera is obvious, and ascites or portal hypertension may occur in the later stage of the disease.
③ Ultrasound examination showed that there was no gallbladder in the common bile duct or only atrophic gallbladder, and bile duct dilatation showed extrahepatic bile duct dilatation.
(2) Periampullary carcinoma of common bile duct: The main differential points are:
① Most of the patients are middle-aged and above, and the course of disease is short.
② Jaundice is progressive deepening rather than intermittent.
③ Symptoms such as emaciation and anemia may appear when the general condition deteriorates rapidly.
④ The large lump is palpable, but hard and nodular.
⑤CTB ultrasound or MRI can find the solid tumor in the ampulla of the distal common bile duct, while the canceration rate of cholangiocarcinoma with congenital cholangiectasia is high. More than half of the patients complained of intermittent abdominal pain and fever after cholangiocarcinoma. Compared with congenital cholangiectasis without canceration, this frequency is slightly higher, about 30% have jaundice and touch the abdominal mass. When they have back pain and emaciation, it shows that they are in the advanced stage due to cancer. There is no specific manifestation after the change, which is easy to be confused with the primary disease. Therefore, once the shadow of tumor is found in the dilated bile duct, such as B-ultrasound CTERCP, it should be highly suspected that it is extremely difficult to analyze more than 300 cases of cancer, and the focus is limited to the early muscularis, with less than 10 cases.
3. Choledochal cysts with abdominal pain as the prominent symptom and acute right epigastric pain or epigastric pain as the prominent symptom should be differentiated from biliary ascariasis, acute cholecystitis, acute pancreatitis and intussusception.
(1) Biliary ascariasis:
① Sudden drilling-like pain in the right upper abdomen or upper abdomen can be relieved or returned to normal after the attack, with severe symptoms and mild signs.
② No jaundice, sometimes mild.
③ There is no mass in the right upper abdomen or upper abdomen.
④ Ultrasonic examination showed that there was a worm-like echo in the common bile duct, and the common bile duct could be slightly dilated, but there was no worm-like echo in the common bile duct. ERCP showed cystic or spindle dilatation of common bile duct, bile duct dilatation and abnormal pancreaticobiliary confluence, but biliary ascaris was not found.
(2) Acute cholecystitis: It often occurs in adults, with fever, pain, tenderness and muscle tension. Murphy's signs are obvious. Sometimes the gallbladder moves with breathing and dilates shallower than the common bile duct. Jaundice is deeper and wider. If there is jaundice, it is lighter. B-ultrasound can easily distinguish the common bile duct without cystic or spindle dilatation in acute cholecystitis.
(3) Intussusception: The main symptom of this disease is regular paroxysmal abdominal pain. Abdominal mass is oval or rectangular, easy to move and slightly tough. Most of them are located in the upper right. There are jam-like barium enema or air enema to see the typical cup-shaped shadow of intussusception head.
(4) Acute pancreatitis: Abdominal pain is more common in adults, often located in the middle of the upper abdomen to the left, which may involve the left waist, back and left shoulder. Severe cases may cause shock, nausea, vomiting and fever, as well as peritoneal irritation. Biochemical examination showed that the amylase in hematuria increased significantly. B-ultrasound CT showed that the swollen pancreas and common bile duct were normal. It is particularly noteworthy that about 20% ~ 40% of patients with congenital cholangiectasia show hyperamylasemia, and some cases have increased urinary amylase. The so-called "pseudopancreatitis" is caused by the reverse flow of amylase in capillary bile duct. In this case, pancreatic lesions are mostly mild.
1. Bile stasis Due to biliary obstruction, bile retention, cysts gradually increase, and the liver gradually develops into biliary cirrhosis, which seriously damages liver function.
2. There are two causes of portal hypertension: (1) giant cyst compression: giant cyst compression of portal vein and superior mesenteric vein leads to portal vein blood flow obstruction and extrahepatic portal hypertension; (2) Late biliary cirrhosis: extensive fibrochords and sclerosing nodules are formed in the liver, squeezing small branches of portal vein and hepatic sinuses of hepatic lobules, narrowing or blocking portal vein blood flow, increasing pressure and forming intrahepatic portal hypertension; In addition, due to liver cirrhosis, the sinus cavity is narrowed or the short circuit between portal vein and hepatic artery is blocked.
3. Cholangitis is prone to bacterial infection, leading to acute cholangitis, intrahepatic infection and even liver abscess. Cholangitis often causes swelling, hypertrophy and fibrosis of the bile duct wall, which narrows or even occludes the distal bile duct.
4. Abnormal confluence of pancreaticobiliary duct in pancreatitis can make bile in high-pressure bile duct return to pancreatic duct, damage pancreatic duct and acinar pancreatic juice penetrate into pancreatic parenchyma, resulting in autolysis of pancreatic tissue and pancreatitis.
5. The formation of gallstones in gallbladder cavity can be changed by cholestasis, biliary tract infection and bile duct stenosis, which is beneficial to the formation of bile pigment stones. Wang Changlin reported 1 case 13-year-old patient took out a large number of small particles of bile pigment stones from the common bile duct cyst, and there were also many stones in the left and right hepatic ducts. We have 3 cases of choledochal cyst in children. Yamaguchi summary 1433 cases with stones, accounting for 8.0%.
6. Bile duct obstruction and bile salt can't enter the bleeding liver function damage caused by intestinal fat-soluble vitamin absorption disorder. Obstacles in the synthesis of coagulation factors in the liver can lead to bleeding, including skin ecchymosis, gingival bleeding and gastrointestinal bleeding.
7. The perforation or rupture of cyst is increasing gradually, and the pressure in the cyst is increasing gradually. In the weak part of the cyst wall, perforation is easy to occur due to sudden increase of abdominal pressure or upper abdominal trauma, causing acute biliary peritonitis. China, Taiwan Province Province of China, Japan and China have reported this, accounting for 1.8%.
8. Recurrent cholangitis caused by long-term cholestasis and abnormal pancreaticobiliary confluence in cyst can cause bile duct wall inflammation and ulcer regeneration or metaplasia, and the canceration rate is 2.5%.
How to prevent editing this paragraph?
Early diagnosis and timely surgical treatment are the key to prevent this disease, and various fat-soluble vitamins (ADK, etc. ) should be supplemented to maintain a good nutritional status.
(a) the treatment of this disease, if not surgical treatment, is often due to repeated infection of biliary cirrhosis, common bile duct perforation or cancer, so when the child is diagnosed, it should be treated with surgery in time.
1. In principle, there are three requirements:
(1) The main purpose of the operation is to restore bile drainage into the intestine to avoid ascending cholangitis.
(2) Remove the dilated common bile duct to prevent cancer in the future.
(3) To prevent anastomotic stenosis in the future.
2. Surgical methods At present, the commonly used surgical methods are:
(1) Cyst resection, Roux-Y anastomosis of common hepatic duct and jejunum and anti-reflux valvuloplasty.
(2) Cyst excision, jejunum interposition instead of biliary tract, and anti-reflux giant flap surgery.
(3) Cyst-duodenal anastomosis and cyst resection-hepatoduodenal anastomosis are seldom used at present, because it is difficult to prevent reflux infection and anastomotic stenosis.
(2) Prognosis Due to the frequent occurrence of cholangitis and the above complications, surgery should be performed as soon as possible. Before 1970s, cystostomy or partial cystectomy plus ostomy was usually used. There are more and more reports of malignant transformation of bile duct cysts, and there are also reports of sporadic cases in China. The average age of primary cyst is 50 years old, but the average age of malignant cyst after intestinal drainage is 35 years old. The average age of patients with internal drainage is 25 years old, so the average time from internal drainage to malignant transformation is about 10 years. Primary malignant transformation can occur in bile duct (53%) or gallbladder (46%). However, after internal drainage, 85% of the malignant transformation occurred on the residual cyst, and only 9% of the patients in the gallbladder underwent partial cyst resection, and some cyst walls also became malignant. The reason why the cyst is easy to become cancerous after enterostomy may be that pancreatic juice easily flows into the cyst cavity when the biliary-pancreatic duct is deformed. The activation of pancreatic enzyme from the small intestine can aggravate the inflammatory changes in the cyst and accelerate the occurrence of canceration. Therefore, cyst internal drainage and partial cyst resection internal drainage are not advisable even after such surgery. Surgical patients should also consider a second cyst resection. At present, the accepted surgical methods for the treatment of type I bile duct cyst include cyst resection, Roux-en-Y hepaticojejunostomy or hepatoduodenal interposition jejunostomy. In 1950s, the mortality rate of this kind of operation was relatively high, around 30%, which has been obviously reduced to around 4% in recent years. The Second Affiliated Hospital of Harbin Medical University 1986 reported 4 1 case without death. So far, we have done nearly 30 cases without death. For type ⅱ bile duct cyst, cyst resection and bile duct-bile duct anastomosis can be performed. For type ⅲ bile duct-duodenum interposition jejunostomy, by cutting off the ends of the common bile duct and duodenum, the lateral back wall of the pancreatic duct can be cut off, the pancreatic duct can be found for protection, and part of the pancreatic head tissue can be removed. Bile duct cyst can be removed by cutting the front side wall of duodenum in intestinal cavity or adding Oddi sphincter incision, and then transplanting pancreaticobiliary duct in the back wall of duodenum. For type Ⅳ bile duct cyst confined to one lobe, local lobectomy is feasible. Liver transplantation is needed for extensive intrahepatic lesions, but various forms of intrahepatic bile duct internal and external drainage are not ideal.