congenital choledochal cyst
Introduction
Introduction to congenital choledochal cyst Congenital choledochal cysts, also known as cystic dilatation of the common bile duct, are mostly congenital malformations. Once the disease is diagnosed, early surgery is needed to reduce biliary cirrhosis caused by biliary infection and obstructive jaundice. Cystectomy, hepaticojejunal Roux-Y anastomosis is the current standard procedure for the treatment of choledochal cysts. Due to laparoscopic cyst resection and common jejunal Roux-Y anastomosis for biliary reconstruction, the surgical trauma is small, and the child recovers quickly, which can achieve patency drainage. basic knowledge The proportion of sickness: 0.002%-0.003% Susceptible people: children Mode of infection: non-infectious Complications: jaundice
Cause
Causes of congenital choledochal cyst
The cause is still not fully understood. There have been embryonic bile duct cavitation abnormalities, viral infection theory, distal common bile duct nerve, and muscular dysplasia. In the late 1960s, Babbitt proposed a close relationship with pancreaticobiliary confluence anomalies. Especially after the 1970s, Japanese scholar Komi Nobuhiko founded the episode of the pancreaticobiliary confluence anomaly. After the research was pushed deeper, the pancreaticobiliary confluence was congenital. The role played by the pathogenesis of biliary dilatation has attracted more and more attention. At present, most scholars believe that this is a congenital disease.
Biliary embryo developmental malformation (20%):
The formation of the extrahepatic bile duct system is mostly in the 5th to 7th week of the fetus. At this time, the epithelial cells in the biliary system tube proliferate, forming a solid cell cord, and the posterior vacuoles form and fuse into the lumen of the biliary tract. If a part of the epithelial cells proliferate excessively, the distal stenosis and the proximal hyperventilation may form an expansion of the bile duct when vacuolization is repeated. Some scholars have suggested that the occlusion of the pancreaticobiliary duct to the distal duodenal opening is one of the pathological changes of congenital biliary dilatation, suggesting that this lesion may be the cause of the extension of the common pathway of pancreaticobiliary duct.
Distal nerve and muscle dysplasia of the common bile duct (15%):
Some scholars have found that congenital biliary dilatation of the common bile duct wall lacks ganglion cells, suggesting that the lack of ganglion cells may lead to a decrease in rheumatoid rhythmic movement, while distal muscle function or structural dysplasia may cause the common bile duct. Obstruction, biliary discharge disorder, and increased pressure in the proximal biliary tract eventually lead to different degrees of bile duct dilatation. In related studies conducted by Chinese scholars, it was also found that the muscle layer at the end of the stenosis of the common bile duct cyst was significantly thickened and the ganglion cells were abnormal.
Hereditary factors (20%):
Although there is no evidence to confirm that the disease has a positive genetic pathway chain, there are reports of family morbidity both internationally and domestically. The author performed surgery on a 48-year-old father and an 11-year-old daughter with congenital biliary dilatation.
In 1916, a Japanese scholar, Muji, performed a necropsy on a patient with congenital biliary dilatation and found that the lower end of the dilated common bile duct had a common pathway of pancreatic duct and bile duct. The concept of pancreaticobiliary confluence was first proposed. Later, in the late 1960s and early 1970s, American Babbitt and Japanese ancient taste Xinyan conducted a more detailed study on the pathological changes of pancreaticobiliary ductal abnormalities and the relationship with this disease, and proposed the abnormal pathogenic theory of pancreaticobiliary duct. The authors also performed an animal model of puppies with abnormal pancreaticobiliary duct junction and distal stenosis of the common bile duct. The animal model of bile duct dilatation was successfully established, suggesting that abnormal pancreaticobiliary ductal flow leads to reflux of pancreatic juice into the biliary tract, which eventually leads to bile duct dilatation. These studies support the pathogenesis theory of pancreaticobiliary confluence.
In recent years, many scholars have found that the two types of congenital biliary dilatation, the most common type of congenital biliary dilatation in the clinic, are not completely consistent with cystic and fusiform types. Therefore, the cause may be due to congenital malformations caused by a variety of factors.
Prevention
Congenital choledochal cyst prevention
Early diagnosis as soon as possible, timely surgical treatment is the key to the prevention of this disease, should be supplemented with a variety of fat-soluble vitamins (A, D, K, etc.) to maintain a good nutritional status.
Complication
Congenital choledochal cyst complications Complications
The most common is jaundice, which is often the first symptom to be noticed in children. It can be intermittent or progressive. The jaundice can be itchy, the stool is gray, and the dark urine is bile.
Symptom
Symptoms of congenital choledochal cyst common symptoms jaundice abdominal pain dull pain nausea cyst dark urine cold biliary atresia anorexia biliary obstruction
Symptoms occur mostly in children and adulthood. According to statistics, 25% of cases are diagnosed in the first year after birth, 60% are diagnosed before the age of 10, and 23% are diagnosed before the age of 40. The oldest is 78 years old. Clinical manifestations of intermittent upper abdominal pain, right upper quadrant mass and jaundice, called triad of choledochal cyst, but only accounted for 1/3 of the total, 55% to 60% of cases have abdominal pain, 60% to 75% have lumps, 65% to 80% have jaundice, clinical manifestations are quite different, can be completely painless, or dull pain that can be tolerated in the upper abdomen, or even severe cramps in the right upper quadrant. Jaundice is the most common symptom, and is often the most common in children. Symptoms noted first may be intermittent or progressive; infants with progressive jaundice should consider congenital biliary atresia, severe jaundice may have skin itching, fecal gray, dark urine black for bile urine, jaundice and Biliary obstruction is directly related to the degree of infection. The upper abdomen or right rib can touch the mass. The large one can occupy the right abdomen. The sac is sexy, fixed and inactive. The small cyst can move slightly. After infection, Then there is obvious tenderness and rebound tenderness, if it is Finger intestinal bile duct bulging, while the symptoms of duodenal obstruction, in addition, they can still experience nausea, vomiting, anorexia, diarrhea and weight loss, infection, there are chills, fever, increased white blood cell count.
Examine
Examination of congenital choledochal cyst
Liver function test: It is necessary to find out the nature of jaundice. Preoperative diagnosis is also necessary. Most patients have blood, urine and feces examined by obstructive jaundice. A series of examinations of obstructive jaundice are abnormal, including serum bilirubin. Prime, mainly direct bilirubin increased significantly, alkaline phosphatase and -glutamyl transpeptidase also increased, may have varying degrees of acute liver dysfunction, a small number of patients can be basically normal indicators Inflammatory changes such as increased white blood cell count and increased neutrophils in peripheral blood can be seen in patients with cysts.
There is a considerable proportion of cases in this disease, especially in the process of fusiforms, blood is found, urinary pancreatic amylase is increased, and is misdiagnosed as simple acute pancreatitis. Clinical actual cases do include pancreatitis, but most In the case of abnormal pancreaticobiliary confluence, the pancreatic juice will flow back into the bile duct and even the intrahepatic bile duct. In the capillary bile duct, pancreatic amylase can be caused by the hepatic sinus and flow back into the blood circulation, mostly non-true pancreatitis.
X-ray inspection
It is very helpful for diagnosis. The upper abdominal plain film can be seen with dense mass shadow, gastrointestinal X-ray barium angiography or low-profile duodenal angiography. It can be seen that the gastric pressure is shifted to the left and the duodenum is moved to the left and the left. Position, duodenal curvature increased, X-ray barium enema examination showed colonic hepatic flexion forward and downward, gallbladder angiography often failed, when serum bilirubin is greater than 3mg / dl, oral or venography is not possible Intravenous cholangiography may be valuable only before jaundice occurs, small cysts are expected to be displayed; large cysts are diluted due to contrast agents, and cholangiopancreatography may be confirmed by skin liver puncture. Sexual expansion, but there are concerns about leakage of bile, retrograde intubation angiography with fiberoptic duodenoscopy, if successful, the most valuable for the diagnosis of choledochal cyst, but 50% of cases have amylase increased after surgery, individual cases Can be caused by pancreatitis death, intraoperative cholangiography, the most effective, can depict the contour of the cyst and intrahepatic bile duct status, can provide a favorable basis for determining the treatment plan.
2. Ultrasound examination
A constant liquid level can be found. The tomograph can reliably display the location and size of the abdominal cyst. The liver radionuclide scan can help identify the intrahepatic bile duct status, as well as the location and size of the common bile duct cyst. Arterial angiography shows a large area of avascular area, suggesting the location of the cyst.
Diagnosis
Diagnosis and diagnosis of congenital choledochal cyst
According to the child's early onset of typical abdominal pain, jaundice, abdominal masses should be considered in the three major symptoms, but some do not have three major symptoms, combined with laboratory tests, abdominal B-ultrasound, CT and cholangiography to make a diagnosis .
The main manifestation of jaundice should be differentiated from jaundice hepatitis, common bile duct atresia, and hemolytic jaundice. The main manifestations of abdominal mass should be differentiated from the common retroperitoneal and intraperitoneal masses in children, such as hydronephrosis. Renal embryonal tumor, teratoma, liver tumor, mesenteric and omental cysts; patients with abdominal pain need to be differentiated from intussusception, intestinal diverticulum, biliary tract mites, cholecystitis, etc.; intrahepatic biliary cyst should be differentiated from congenital hepatic cyst .
