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Choledochal cyst

  Choledochal cyst can also be called choledochal cystic dilatation, which is mostly congenital developmental malformation. The ratio is about 3 to 4 to 1. Symptoms often appear in children and adulthood, and the clinical manifestations are intermittent upper abdominal pain, mass in the upper right abdomen, and jaundice, known as the triad of choledochal cyst, nausea, vomiting, anorexia, diarrhea, and weight loss, etc.

  This disease is rarely seen. In 1948, Davis found 1 case among 17,981 gallbladder surgery patients at Mayo Clinic, and Smith collected only 2 cases of choledochal cyst from 787,000 admitted patients. However, in the past 20 years, the reports of this disease in the world literature have been increasing, and abroad there have been more than 1500 cases, of which 1/3 come from Japan, so it is considered that this disease is more common in Japan; the literature in China has reached nearly 400 cases. Therefore, due to the limited number of past cases, it may be due to insufficient understanding and low diagnostic rates, and it cannot be considered a rare disease. The incidence rate of women is about 3 to 4 times higher than that of men, and women are generally higher than men.

Table of Contents

What are the causes of choledochal cyst?
What complications can choledochal cyst easily lead to?
What are the typical symptoms of choledochal cyst?
How should choledochal cyst be prevented?
What laboratory tests are needed for choledochal cyst?
6. Diet recommendations and禁忌 for common bile duct cyst patients
7. Conventional methods of Western medicine for the treatment of common bile duct cyst

1. What are the causes of common bile duct cyst disease

  The etiology of common bile duct cysts has not been fully understood. There have been theories such as abnormal bile duct cavitation during embryonic period, virus infection, maldevelopment of the distal common bile duct nerves and muscles, etc. In the late 1960s, Babbitt proposed that there is a close relationship between abnormal pancreatobiliary drainage and the disease, especially after the 1970s, Japanese scholar Komi Nobuhiko established the Pancreatobiliary Drainage Abnormality Research Society, pushing the relevant research to a deeper level. The role of abnormal pancreatobiliary drainage in the pathogenesis of congenital bile duct dilatation has attracted more and more attention. Currently, most scholars believe that it is a congenital disease.

  1. Malformation of the biliary tract during embryonic development

  The formation of the extrahepatic bile duct system usually occurs between the 5th and 7th weeks of fetal development. At this time, the epithelial cells in the bile duct system proliferate, forming solid cell cords, which later become hollow and fuse to form the lumen of the bile duct. If a part of the epithelial cells overproliferates, when hollowing out and reconnecting, the distal part may become narrow while the proximal part over-hollows, which may lead to bile duct dilatation. Some scholars also propose that the ectopic opening of the pancreatobiliary drainage into the duodenum at a distant position is one of the pathological changes of congenital bile duct dilatation, suggesting that this lesion may be the cause of the elongation of the common channel of pancreatobiliary drainage.

  2. Obstruction at the end of the common bile duct

  Due to the obstruction at the end of the common bile duct, the bile excretion is not smooth, leading to secondary dilatation of the proximal common bile duct.

  3. Maldevelopment of the distal common bile duct nerves and muscles

  Some scholars have found that the distal wall of the common bile duct in patients with congenital bile duct dilatation lacks ganglion cells, indicating that the lack of ganglion cells may lead to a decrease in the rhythmic movement of the common bile duct, and the maldevelopment of the distal muscle functionally or structurally may cause obstruction of the common bile duct, bile excretion disorders, and an increase in pressure within the proximal bile duct, ultimately leading to varying degrees of bile duct dilatation. Related research conducted by Chinese scholars also found that the muscular layer at the end of the common bile duct cyst has significant thickening, and there are abnormal ganglion cells.

  4. Genetic factors

  Although there is no evidence to confirm a definite genetic pathway for the disease, there have been reports of familial occurrence both internationally and in China. The author has performed surgery on a 48-year-old father and an 11-year-old daughter who both suffered from congenital bile duct dilatation.

  5. Pathogenic theory of abnormal pancreatobiliary drainage

  In 1916, Japanese scholar Kageyama discovered during the autopsy of a patient with congenital bile duct dilatation that the dilated common bile duct had a common channel with the pancreas duct, which was longer than usual, and for the first time proposed the concept of abnormal pancreatobiliary drainage. Later, in the late 1960s and early 1970s, American Babbitt and Japanese Komi Nobuhiko conducted more detailed research on the pathological changes of abnormal pancreatobiliary drainage and its relationship with the disease, and proposed the pathogenic theory of abnormal pancreatobiliary drainage. The author also conducted experiments on a canine animal model with abnormal pancreatobiliary drainage and distal common bile duct stenosis, successfully creating an animal model of bile duct dilatation, indicating that abnormal pancreatobiliary drainage leads to the reflux of pancreatic juice into the bile duct, ultimately leading to bile duct dilatation. These studies all support the pathogenic theory of abnormal pancreatobiliary drainage.

  6. The theory of multiple factors combined to cause disease

  In recent years, many scholars have found that the pathological changes between the two most commonly existing types of congenital bile duct dilatation, cystic type and fusiform type, are not completely consistent. Therefore, it is believed that the etiology may be due to congenital developmental malformation caused by multiple factors.

2. What complications can choledochal cysts easily cause

  Choledochal cyst is the most common congenital biliary malformation in clinical practice. This condition is also known as congenital choledochal cyst, congenital dilatation of the choledochus, primary dilatation of the choledochus, etc. The complications of choledochal cysts include the following points:

  1. Bile stasis

  Due to biliary obstruction, bile stasis causes the cyst to gradually enlarge, leading to cholestatic liver, which gradually develops into biliary cirrhosis, severely impairing liver function.

  2. Portal hypertension

  (1) Compression by large cysts

  Large cysts compress the portal vein and superior mesenteric vein, causing the portal vein blood to flow back to the liver to be obstructed, resulting in extrahepatic portal hypertension.

  (2) Advanced biliary cirrhosis

  The formation of extensive fibrous strands and fibrotic nodules in the liver can compress the small branches of the intrahepatic portal vein and the hepatic sinusoids of the hepatic lobules, causing them to narrow or occlude. This obstructs portal vein blood flow, increases pressure, and leads to intrahepatic portal hypertension. In addition, due to liver cirrhosis, the sinusoids become narrow or occluded, and shunts between the portal vein and hepatic artery open up, allowing hepatic arterial blood to enter the portal venous system, further increasing portal vein pressure. This type of patient accounts for 25%.

  3. Cholangitis

  Due to poor bile excretion, local resistance decreases, making it susceptible to bacterial infection, leading to acute cholangitis, intrahepatic infection, and even liver abscesses. Recurrent cholangitis often leads to the swelling, thickening, and fibrosis of the bile duct wall, making the distal bile duct even narrower, and even occluded.

  4. Pancreatitis

  Abnormal junction of the pancreatic and bile ducts can cause bile juice in the high-pressure bile ducts to reflux into the pancreatic duct, damaging the pancreatic acini and ducts, leading to autolysis of pancreatic tissue and the development of pancreatitis.

  5. Stone formation within the cystic cavity

  Due to bile stasis, biliary tract infection, and bile duct stenosis, the composition of bile can change, which is conducive to the formation of bilirubin gallstones. Wang Changlin reported a case of a 13-year-old patient who had a large number of small granular bilirubin gallstones removed from a choledochal cyst, and there were also many stones in the left and right hepatic ducts. We also have 3 cases of pediatric choledochal cysts that are also complicated by bilirubin gallstones. Yamaguchi summarized 1433 cases, and the incidence of concomitant stones was 8.0%.

  6、出血

  因胆道梗阻致肝功能损害,加上胆盐不能进入肠道,脂溶性维生素吸收障碍,肝脏对凝血因子的合成发生障碍,可致出血,包括皮肤瘀斑、牙龈出血、消化道出血等。

  7、囊肿穿孔或破裂

  囊肿逐渐增大,囊内压力逐渐增加,在囊壁的薄弱处,易因腹压突然增加或上腹部的外伤而发生穿孔,引起急性胆汁性腹膜炎。中国台湾、日本和中国都有这方面的报告,占1.8%。

  8、囊肿癌变

  囊肿内长期胆汁淤滞、反复发作胆管炎及胰胆管汇合异常,胰液反流入囊腔等,可使胆管壁发生炎症、溃疡、再生或化生,在此基础上诱发癌变。

3. 胆总管囊肿有哪些典型症状

  胆总管囊肿一般多出现于儿童和成年期,据统计25%的病例在生后第1年作出诊断,60%在10岁前作出诊断,23%是在40岁以上才作出诊断,年龄最大者为78岁。临床表现为间歇性上腹痛、右上腹肿块和黄疸,称为胆总管囊肿三联征,但仅占总数的1/3。55%~60%的病例有腹痛,60%~75%有肿块,65%~80%有黄疸。临床表现颇不相同,可以完全不痛,或为上腹部能耐受之钝痛,甚至是右上腹剧烈绞痛。黄疸是最常见的症状,在儿童往往为最先注意到的症状,可为间歇性或进行性;婴儿进行性黄疸,应考虑到先天性胆道闭锁。黄疸重者可有皮肤刺痒、粪便灰白,尿暗黑色为胆汁尿的表现,黄疸程度与胆道梗阻和感染程度有直接关系。上腹或右季肋部可触及肿物,大的可占全右腹部,扪之有囊性感,固定不活动,小型囊肿可稍有移动。

  其次,如果发生感染后,则出现明显触痛和反跳痛。如果是十二指肠内胆总管膨出,则同时有十二指肠梗阻症状。此外,尚可出现恶心、呕吐、厌食、腹泻以及体重减轻等,发生感染则有寒战、发热、白细胞计数增加。

4. 胆总管囊肿应该如何预防

  对于胆总管囊肿的预防,应该早期尽快明确诊断,确定是否患病才是主要,及时手术治疗是此病预防的关键。同时还应该补充各种脂溶性维生素(A、D、K 等),以维持良好的身体状况。保持营养平衡等等。

5. 胆总管囊肿需要做哪些化验检查

  胆总管囊肿首先应该先进行诊断,根据患儿幼年起间歇出现典型的腹痛、黄疸、腹部包块三大症状应考虑本病,但部分不具有三大症状,需结合实验室检查、腹部B超、CT和胆道造影等做出诊断。

  再进行实验室检查:肝功能试验,查明黄疸的性质,对术前诊断亦属必要。

  Then proceed with other auxiliary examinations:

  1. X-ray examination

  It is of great help in diagnosis. The upper abdominal plain film shows a dense shadow of a mass, and gastrointestinal X-ray barium meal or low-pressure duodenal barium meal shows that the stomach is compressed and displaced to the left anterior position, and the duodenum to the left anterior-inferior position, with an enlarged duodenal flexure. Barium enema examination shows that the colon-hepatic flexure is displaced downward. Cholescintigraphy often fails, and when the serum bilirubin is greater than 3mg/dl, oral or intravenous contrast enhancement is not possible; only intravenous cholangiography may still be valuable before jaundice appears. Small cysts can be expected to be visualized; large cysts may not be visualized due to dilution of the contrast agent. Percutaneous liver biopsy for cholangiography may confirm the cystic dilation of the common bile duct, but there is a concern about leakage of bile. Retrograde cholangiography through a fiberoptic duodenoscope may be the most valuable for diagnosing choledochal cysts if successful, but 50% of cases have increased amylase levels postoperatively, and individual cases may die due to induced pancreatitis. Cholangiography during surgery is the most effective, as it can outline the contour of the cyst and the condition of intrahepatic bile ducts, providing favorable evidence for determining the treatment plan.

  2. Ultrasound examination

  A constant liquid level segment can be found. The tomography instrument can reliably show the position and size of the abdominal cyst. Liver radionuclide scanning helps identify the condition of intrahepatic bile ducts, as well as the position and size of the choledochal cyst. Selective abdominal aortic angiography may show a large area of avascular region, and suggest the location of the cyst, etc.

6. Dietary taboos and recommendations for patients with choledochal cyst

  Cholangiocystoma is the most common congenital biliary tract malformation in clinical practice. The main lesion refers to a part of the common bile duct that expands into a cystic or spindle shape, sometimes accompanied by congenital malformations of intrahepatic bile ducts. Generally, patients with this condition should pay attention to the following dietary principles.

  1. Minimize the intake of fats, especially animal fats, avoid eating fatty meat and fried foods, and as much as possible, replace animal fats with vegetable oils.

  2. A considerable number of gallbladder inflammation and gallstone disease are indeed related to excessively high cholesterol levels and metabolic disorders in the body, so it is necessary to limit foods with high cholesterol content such as fish roe, yolks of various eggs, and the livers, kidneys, hearts, and brains of various carnivorous animals.

  3. Cooking should be done by steaming, boiling, stewing, or braising, and it is strictly forbidden to consume large amounts of fried, baked, roasted, smoked, or preserved foods.

  4. Increase the intake of foods rich in high-quality protein and carbohydrates such as fish, lean meat, dairy products, fresh vegetables, and fruits to ensure energy supply, thereby promoting the formation of glycogen in the liver and protecting the liver.

  5. Increase the consumption of foods rich in vitamin A such as tomatoes, corn, and carrots to maintain the integrity of gallbladder epithelial cells, prevent the shedding of epithelial cells to form the core of stones, and thus trigger stones or cause stones to grow larger and more in number.

  6. If possible, drink fresh vegetable or fruit juices such as watermelon juice, orange juice, and carrot juice more frequently, and increase the frequency and quantity of meals to enhance the secretion and excretion of bile, and alleviate inflammation and bile stasis.

  7. Reduce the intake of foods rich in fiber such as cabbage and celery to avoid increased peristalsis in the gastrointestinal tract due to difficult digestion, which may trigger biliary colic.

  8. Quit smoking and drinking and reduce the intake of spicy and irritating foods and strong seasonings, such as mustard oil, to avoid stimulating the gastrointestinal tract and triggering or aggravating the condition.

  9. It is advisable to consume light, easy-to-digest, low-fiber, temperature-appropriate, non-irritating, and low-fat liquid or semi-liquid foods. One should not indulge in eating and drinking to seek temporary satisfaction, as this may cause unnecessary trouble and even trigger bile duct bleeding, which may be life-threatening.

7. Conventional Methods of Western Medicine for Treating Choledochal Cysts

  Through the summary of experience from a large number of cases in recent years, people's treatment methods for choledochal cysts tend to be unified. The cyst internal drainage and cyst resection with choledochojejunal anastomosis that were used in the past for treating children with choledochal cysts have been abandoned due to a high incidence of complications such as postoperative intracystic infection, anastomotic stenosis, stone formation, gastric reflux, and even canceration. Currently, it is advocated that regardless of the age of the child or the severity of the symptoms, the radical resection of the cyst with choledochojejunostomy Roux-Y anastomosis is the standard surgical procedure for treating choledochal cysts. A detailed understanding of the changes in the child's entire biliary and pancreatic duct system before and during surgery, while handling abnormal lesions of intraportal bile ducts and pancreatic ducts as well as the common duct, is the key to reducing postoperative complications. It is usually the method that doctors will take to treat choledochal cysts.

  1. Full-thickness Cyst Resection

  For children with a short history of illness, cystic dilatation of the common bile duct is common, the cyst wall is usually very thin, the inflammation around the portal vein is mild, and the tissue adhesion is not severe. A full-thickness and complete resection of the cyst can be performed.

  2. Cyst Endometrial Resection

  If the child has a long history of illness and recurrent cyst infections, the cyst may be severely adherent to the surrounding tissue, the anatomical levels are unclear, and the full-thickness resection of the cyst may result in excessive bleeding, and there is a possibility of injury to large blood vessels and pancreatic tissue. Cyst endometrial resection can be performed to avoid free cyst wall.

  3. Cyst External Drainage

  For cases where bile duct perforation causes cholestatic peritonitis, severe cholangitis, and it is difficult to remove cysts due to poor overall conditions of children, cyst external drainage can be performed to reduce the impact on children, solve the problem of bile duct obstruction, and then perform radical surgery again after the child's condition improves. Abdominal cyst drainage, percutaneous puncture of cysts or bile duct drainage are all effective methods that can be chosen.

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