Abnormal Pancreaticocholang Synoflow Syndrome, also known as congenital choledochal cyst.

bubble_chart Pathogenesis

The various biliary and pancreatic diseases caused by abnormal pancreaticobiliary ductal junction have garnered clinical attention, but there is no consensus on classification. In 1985, Miyano et al. proposed a new classification method for clinical application, which can be divided into: Type I (long common channel type), further divided into: Type I_a ── cystic dilatation type, equivalent to congenital choledochal cyst (CBDⅠ); Type I_b ── fusiform dilatation type (CDBⅡ); Type I_c ── non-dilated bile duct type; Type II, other complex junctional anomalies.

bubble_chart Pathological Changes

Due to the accumulation of gall fel and varying degrees of liver dysfunction, the synthesis of coagulation factors collectively known as vitamin K-dependent factors (II, VII, IX, X) within liver cells is affected. Approximately 1/6 of patients may experience spontaneous perforation of the common bile duct. The causes of perforation may be related to congenital weakness of the common bile duct wall, terminal stenosis, twisting causing gall fel excretion disorders or secondary infections, malformation at the pancreaticobiliary junction, pancreatic fluid reflux, and excessive bile duct pressure.

bubble_chart Clinical Manifestations

Obstruction of bile excretion leads to insufficient absorption of fat-soluble vitamin K, which can cause specific abnormalities in the coagulation process. Severe deficiency often manifests as spontaneous bleeding, such as systemic ecchymosis, hematoma, gastrointestinal bleeding, epistaxis, and bleeding in joints, muscles, and the intracranial space. Some patients frequently experience abdominal pain and elevated serum pancreatic amylase levels, which are symptoms of pancreatitis.

Based on clinical symptoms and signs, a diagnosis can be made. Further CT scans and B-ultrasound examinations should be conducted to rule out diseases such as hepatitis and cholangitis.

bubble_chart Treatment Measures

Once a clear diagnosis is made, timely surgical treatment is advisable.