Abnormal Pancreatico-Biliary Ductal Union Syndrome

　　The main causes of the biliary pancreas maljunction syndrome are genetic factors and body factors. The biliary pancreas maljunction syndrome is an autosomal recessive genetic disease. The various biliary and pancreatic diseases caused by biliary and pancreatic duct maljunction have attracted clinical attention, but there is no consensus on classification. In 1985, Miyano et al. proposed a new classification method convenient for clinical application, which can be divided into type Ia - cystic dilatation type, equivalent to congenital choledochal dilatation (CBDⅠ); type Ib - spindle-shaped dilatation (CBDⅡ type); type Ic - no bile duct dilatation type; type II, for other complex maljunctions.

　　Due to bile stasis and varying degrees of liver dysfunction, the synthesis of coagulation factors II, VII, IX, X, which are collectively referred to as vitamin K-dependent factors, is affected in liver cells. Approximately 1/6 of patients may experience spontaneous perforation of the common bile duct. The cause of perforation may be related to congenital thinning of the bile duct wall, terminal narrowing, twisting, which causes obstruction of bile excretion or secondary infection, malformation at the site of the biliary and pancreatic duct junction, retrograde pancreatic juice flow, and increased intraductal pressure.

　　In addition to general symptoms, the biliary pancreas maljunction syndrome can also cause other diseases. The disease often manifests as spontaneous bleeding, such as gastrointestinal bleeding, epistaxis, and intracranial hemorrhage. Some patients may have symptoms of pancreatitis.

　　The biliary pancreas maljunction syndrome is caused by the obstruction of bile excretion, leading to insufficient absorption of fat-soluble vitamin K, which can cause specific abnormalities in the coagulation process. In severe cases, spontaneous bleeding can occur, such as purpura, hematoma, epistaxis, and joint and muscle bleeding. Some patients may have frequent abdominal pain and increased levels of pancreatic amylase in the blood, showing symptoms of pancreatitis.

　　The etiology of the biliary pancreas maljunction syndrome is not yet clear, and it may be related to environmental factors, genetic factors, dietary factors, and emotional and nutritional factors during pregnancy. Therefore, it is not possible to prevent the disease directly by targeting the etiology. Early detection, early diagnosis, and early treatment are of great significance for indirect prevention. Due to the obstruction of bile excretion, there is a disturbance in the absorption of vitamin K, which can lead to insufficient intake of vitamin K over a long period of time, affecting blood coagulation function. Therefore, it is necessary to check blood coagulation function when necessary to provide vitamin K supplementation.

　　The main clinical examination methods for congenital abnormality of pancreaticobiliary fistula syndrome are B-ultrasound examination, contrast examination, CT examination, and MRI examination, as follows:

　　1. B-ultrasound examination

　　The site of the common bile duct is shown as a localized expanded anechoic area, which is often spherical or elliptical, spindle-shaped, with clear cyst wall, connected to the hepatic duct at the proximal end, and the gallbladder is often pushed to the anterior abdominal wall, and there may be shadow of stones in the cystic cavity.

　　2. Contrast examination

　　Under the premise that liver function is normal, physiological accumulation method of contrast enhancement can show spherical or fusiform dilated common bile duct, and retrograde cholangiopancreatography endoscopic retrograde cholangiopancreatography (ERCP) can show dilated common bile ducts by directly injecting contrast medium into the bile ducts.

　　3. CT examination

　　Visible dilated cystic cavities coexist with normal gallbladders.

　　4. MRI examination

　　Magnetic resonance cholangiopancreatography (MRCP) can show dilated common bile ducts and the entire bile duct system in spherical or fusiform shapes.

　　Patients with congenital abnormality of pancreaticobiliary fistula syndrome should have a light and nutritious diet, eat more fresh vegetables, and pay attention to dietary balance. It is advisable to eat foods rich in protein but not greasy, such as fish and lean meat. Smoking, alcohol, tea, coffee, and spicy刺激性 foods should be avoided.

　　Congenital abnormality of pancreaticobiliary fistula syndrome belongs to surgical diseases, and should be treated with early surgery repair once diagnosed to avoid hemostatic disorders caused by factor deficiency, which can induce hemorrhage of tissues and organs throughout the body. For patients with a tendency to hemorrhage, vitamin K should be supplemented in time, and for patients with factor deficiency, the corresponding lacking factors should be actively supplemented to relieve tissue and organ ischemia.