Congenital bile duct dilatation

　　There are many theories about the etiology, and there is no conclusion yet. Most believe it is a congenital disease. Some also believe that acquired factors are involved in its formation. The main theories are three:

　　1The theory of congenital malformation believes that during the period of embryonic development, the primitive bile duct cells proliferate into a cord-like solid mass, and then gradually become hollow and贯通. If some part of the epithelial cells overproliferate, then during the hollowing and贯通, they will overhollow and form an expansion. Some scholars believe that the formation of bile duct dilatation requires the joint participation of congenital and acquired factors. The thin development of the bile duct wall caused by excessive proliferation and overhollow formation of bile duct epithelial cells during the embryonic period is a congenital factor, and the acquired factors such as the obstruction of the distal end of the common bile duct secondary to pancreatitis or ampulla inflammation, and the subsequent increase in intraductal pressure, will eventually lead to the formation of bile duct dilation.

　　2The theory of the common channel of pancreatobiliary ducts believes that due to the failure of the common bile duct and the main pancreatic duct to separate normally during embryonic development, the junction between them is far from the ampulla of Vater, forming a common bile duct channel that is too long, and the junction angle between the main pancreatic duct and the common bile duct is nearly perpendicular. Therefore, the site of pancreatobiliary anastomosis is not at the duodenal papilla, but outside the duodenal wall, where there is no sphincter, thus losing the sphincter function, causing the pancreatic juice and bile to reflux. When the secretion of pancreatic juice is excessive and the pressure increases, it causes the bile duct to dilate. In recent years, retrograde pancreatobiliary造影 has confirmed that the flow of pancreatobiliary convergence is as high as90～100%, and the fact that amylase content in the dilated bile ducts is increased. In animal experiments, it was observed that after the pancreatic juice enters the bile duct, the activity of amylase, trypsin, and elastase is enhanced, which may be the main cause of bile duct injury.

　　3The virus infection theory believes that biliary atresia, neonatal hepatitis, and bile duct dilatation are caused by a single cause, which is the result of inflammatory infection of the hepatobiliary system. After viral infection, the liver undergoes giant cell transformation, the bile duct epithelium is damaged, leading to luminal obstruction (biliary atresia) or wall weakness (bile duct dilatation). However, those who support this theory have decreased.

　　Once diagnosed, this disease should be treated as soon as possible. If delayed too long, it not only increases the child's pain but also increases the chance of complications. The malformation of cystic dilatation in the lesion and the relative stenosis of the distal bile duct lead to poor bile drainage and even obstruction, which is the root cause of complications.

　　主要包括：

　　1recurrent ascending cholangitis

　　2bile cirrhosis

　　3perforation or rupture of bile duct

　　4recurrent pancreatitis

　　5formation and wall cancer

　　The diagnosis of this disease can be considered based on the three main symptoms that appear intermittently since childhood, namely, abdominal pain, abdominal mass, and jaundice. If the symptoms recur, the possibility of diagnosis is greatly increased. The cystic type cases are mainly characterized by abdominal mass, with an earlier onset age. Diagnosis can be made by palpation combined with ultrasound examination. The fusiform type cases are mainly characterized by abdominal pain symptoms, and in addition to ultrasound examination, ERCP or PTC examination is also required for accurate diagnosis. There is no significant difference in the symptoms of jaundice between the two types, and they can occur in both. The first symptom in most cases occurs in1～3years, but the final diagnosis is often delayed much later. The cystic type is in1years, but the final diagnosis is often delayed much later. The cystic type is in1/4years, while the fusiform type is more common in1After the age of one, the disease mainly manifests as abdominal pain and jaundice.

　　1、The abdominal mass, abdominal pain, and jaundice are considered the classic triad symptoms of the disease. The abdominal mass is located in the upper right abdomen, below the costal margin, and the large ones can occupy the entire right abdomen. The mass is smooth and spherical, and may have a distinct cystic elasticity. When the cyst is filled with bile, it can present a solid sensation, resembling a tumor. However, there is often a change in size, with the mass increasing in size during the onset of infection, pain, and jaundice, and then slightly shrinking after the symptoms subside. Small bile duct cysts, due to their deep location, are not easily palpable. Abdominal pain occurs in the upper middle abdomen or the upper right abdomen, with varying nature and intensity of pain, sometimes presenting as persistent distension, and sometimes as colicky pain. The patient often assumes a flexed knee prone position and refuses to eat to alleviate symptoms. The onset of abdominal pain suggests biliary tract outlet obstruction, with increased pressure in the common bile duct, leading to reciprocal flow of pancreatic juice and bile, causing symptoms of cholangitis or pancreatitis. Therefore, fever is often accompanied by clinical symptoms, and nausea and vomiting may also occur. The increase in blood and urine amylase levels is often observed during symptom onset. Jaundice is usually intermittent and is often the main symptom in infants, with the severity of jaundice directly related to the degree of biliary tract obstruction. In mild cases, jaundice may not be present clinically, but it may temporarily appear after infection and pain, with feces becoming pale or grayish and urine becoming darker. All these symptoms are intermittent. Due to the unobstructed outlet of the distal bile duct, reciprocal bile and pancreatic flow can lead to clinical symptom onset. When bile can flow smoothly, symptoms are reduced or disappear. The interval between attacks varies, with some attacks being frequent and some being asymptomatic for a long time.

　　2The typical triad symptoms, which were previously considered to be mandatory symptoms of the disease, are actually not. In early cases, the three major symptoms do not appear simultaneously. In recent years, with the increasing number of early diagnoses, it has been found that there are more cases with fusiform dilatation, and those with the triad symptoms are still insufficient.10％. Most cases have only one or two symptoms. According to various reports, in60～70% of the cases have an abdominal mass that can be palpated,60～90% of the cases have jaundice, although jaundice is obviously obstructive, in fact, many patients are diagnosed with hepatitis and confirmed only after repeated attacks. Abdominal pain also lacks typical manifestations, so it is easy to be misdiagnosed as other abdominal conditions. Multiple bile duct dilatations inside and outside the liver generally appear late, and symptoms appear only when the intraliver cysts are infected.

　　3Caroli disease: Caroli1958In the year, the multifocal cystic dilatation of intralobular bile ducts in the liver was first described, therefore, congenital intrahepatic bile duct dilatation is also known as Caroli's disease, which belongs to congenital liver cystic fibrous lesions, and is believed to be an autosomal recessive inheritance, more common in males, mainly seen in children and young adults. Early reported cases did not have liver fibrosis and portal hypertension, but in subsequent reports2/3The cases are accompanied by congenital liver fibrosis and are often accompanied by various renal lesions, such as polycystic kidney disease, and in the late stage, complications such as liver cirrhosis and portal hypertension. According to Sherlock's classification, they are divided into four categories: congenital liver fibrosis, congenital intrahepatic bile duct dilatation, congenital common bile duct dilatation, and congenital liver cysts, collectively known as liver and biliary fibrocystic disease. One or more lesions can coexist in the liver and biliary system. This disease is characterized by bile duct inflammation and calculi caused by bile duct dilatation and bile stasis in the liver, but due to the atypical clinical symptoms, it can occur at any age, and recurrent right upper quadrant abdominal pain, fever, and jaundice may occur. During an attack, the liver becomes明显肿大, and after the infection is controlled, the liver often shrinks rapidly with the improvement of symptoms. Liver function damage is not proportional to clinical symptoms. In the early stages, it is often diagnosed as cholecystitis or liver abscess, and if there are other fibrocystic lesions such as congenital liver fibrosis or extrahepatic bile duct dilatation, the symptoms are more complex, and symptoms such as liver cirrhosis, extrahepatic biliary obstruction, and urinary tract infection may occur. Diagnosis is often difficult, and often requires surgical intervention to confirm the diagnosis. In recent years, with the application of diagnostic methods such as ultrasound imaging and various bile duct造影 techniques, accurate diagnosis of intraliver lesions can be obtained, and therefore, case reports have also increased, but often include secondary bile duct dilatation caused by other reasons, which has led to confusion in the concept of Caroli's disease.

　　1、Relief measures:Treatment during the acute phase of symptoms, adopt fasting2～3Days to reduce the secretion of bile and pancreatic juices, alleviate the pressure inside the bile duct. Use antispasmodics to relieve pain, antibiotics3～5Days to prevent and control infection, and corresponding symptomatic treatment, often achieve the purpose of alleviating symptoms. Given its frequent attacks and various complications, timely surgical treatment is recommended.

　　2、Preoperative preparation:The surgical preparation performed is a general preoperative preparation.

　　1、Biochemical examination of blood and urine amylase, which should be regarded as routine examination during the onset of abdominal pain, is helpful for diagnosis.

　　2、Type B ultrasound imaging.

　　3、Percutaneous liver puncture cholangiography (PTC).

　　4、Endoscopic retrograde cholangiopancreatography (ERCP).

　　5、Gastrointestinal barium meal examination.

　　6、Intraoperative bile duct造影.

　　Among the various examination methods, ultrasound examination and biochemical tests are generally performed first at the clinical level, and if an abdominal mass is palpated, the diagnosis can be established. If the mass is not palpated clinically but there is a suspected diagnosis by ultrasound, an ERCP examination is required. If limited by equipment and age, a PTC examination is performed, and other examination methods are supplemented if necessary.

　　1、Dietary care for congenital choledochal cyst: various fat-soluble vitamins (A, D, K, etc.) should be supplemented to maintain good nutritional status.

　　2、Natural vitamin A exists only in animal foods such as: animal liver, eggs, cream, and fish liver oil; the carotenoids contained in plants can be converted into vitamin A in the liver.

　　3、Cholesterol in the human body can be converted into vitamin D. Foods rich in vitamin D: fish, fish eggs, liver, egg yolks, butter, cream, cheese, meat, milk, fruits, nuts, vegetables, and grains, etc.

　　4、There are many foods rich in vitamin K. However, the best sources include dark green vegetables and cabbage, spinach, parsley, beetroot, cauliflower, and kale. In addition, specific vegetable oils such as soybean oil, rapeseed oil, cottonseed oil, and olive oil also contain vitamin K.

　　Surgical treatment:

      1、The external drainage technique is applied to individual severe cases, such as severe obstructive jaundice with liver cirrhosis, severe biliary tract infection, spontaneous bile duct perforation, and secondary treatment after the condition improves.

　　2、囊肿与肠道间内引流术如囊肿十二指肠吻合术，因有较多的并发症，胆管炎的反复发作、吻合口狭窄、结石形成等，以后转而采用囊肿空肠Roux-en-Y式吻合术，并在肠襟上设计各种式样的防逆流操作，但仍存在胰胆合流问题，因而术后还是发生胆管炎或胰腺炎症状，甚至需要再次手术，且术后发生囊壁癌变者屡有报道。所以目前已很少采用。

　　3、胆管扩张部切除胆道重建术近年主张具有根治意义的切除胆管扩张部位以及胆道重建的方法，从而达到去除病灶，使胰胆分流的目的。可采用生理性胆道重建术，将空肠间置于肝管与十二指肠之间，或加用防返流瓣，或采用肝管空肠Roux-en-Η τεχνική σύγκρισης Y, η σύγκριση της χολόχνας με την δωδεκαδάκτυλο, μπορεί να επιτευχθεί καλή επίδοση. Το κύριο είναι ότι η σύγκριση πρέπει να είναι αρκετά μεγάλη για να διασφαλιστεί η πλήρης διήθηση της χολής.

　　3Για τη θεραπεία της εκτεταμένης χολόχνασης εντός του ήπατος, η χολόχναση που προκύπτει από την εκτεταμένη χολόχναση εκτός του ήπατος, η μορφολογία της είναι σφαιρική εκτεταμένη, μετά την επέμβαση συνήθως μπορεί να επανέλθει στο φυσιολογικό. Αν είναι κυστική εκτεταμένη, είναι μίγματος, μετά τη χολοκυστική αποχώρηση, ανεξάρτητα από το μέγεθος της σύγκρισης, παραμένει η χολόχναση εντός του ήπατος, η λοίμωξη και η δημιουργία πέτρας ή καρκίνου, οπότε οι ασθενείς με περιορισμένη κυστική εκτεταμένη χολόχναση, οι περισσότεροι προτιμούν να εκτελέσουν την απομάκρυνση της μερίδας του ήπατος και της χολοκυστικής

　　4Για τη θεραπεία της νόσου Caroli, η πρόληψη και η θεραπεία της χολόχνασης είναι η κύρια μέθοδος, η μακροπρόθεσμη χρήση ευρύτατων αντιβιοτικών, αλλά η αποτελεσματικότητα της θεραπείας δεν είναι ικανοποιητική. Επειδή η πάθηση είναι πολύ εκτεταμένη, η χειρουργική θεραπεία δεν μπορεί συχνά να είναι επιτυχής. Αν η πάθηση περιορίζεται σε ένα φύλο, μπορεί να γίνει απομάκρυνση του φύλου του ήπατος, αλλά σύμφωνα με τις αναφορές, οι δυνατές απομακρύνσεις είναι λιγότερες από1/3Περιπτώσεις. Η μακροχρόνια πρόγνωση είναι πολύ κακή.

　　5Μετά την αποκατάσταση της καρκινώδους παθογένειας της χολόχνασης, ακόμα και αν επιτεύχθηκε ο στόχος της διήθησης της χολής και του πанкρεατικού υγρού, σε ορισμένες περιπτώσεις παραμένουν συχνά η κράμπες στο στομάχι, η αύξηση της αμυλάσης του πанкρεατικού αίματος και άλλες κλινικές εκφράσεις της χολόχνασης ή της παγκρεατίτιδας, που σχετίζονται με την εκτεταμένη χολόχναση εντός του ήπατος και την αномαλία της μορφολογίας του πанкρεατικού. Οι συμπτώματα μπορούν να ανακουφιστούν με την αποχή από τη διατροφή, την αντιφλεγμονώδη θεραπεία, την ανακούφιση από τις κράμπες και την ευδοκίμηση, καθώς και με την πάροδο του χρόνου, η διαίρεση μεταξύ των επεισοδίων θα επεκταθεί. Μέσα σε μακροχρόνια παρακολούθηση80% περιπτώσεων έλαβαν ικανοποιητικά αποτελέσματα.