A choledochal cyst is a rare congenital anomaly of the biliary tract. It is often associated with an anatomical disorder at the junction of the bile and pancreatic ducts. The wall of the cyst is thickened to a width of 2-7 mm, it consists of fibrous tissue with elastic and muscle fibers. In most cases, dilatation begins just above the duodenum and ends at the junction of the left and right hepatic ducts (so-called type I, see below). The gallbladder does not show pathological changes, it can only be enlarged. Liver changes are only seen in older children with chronic disease (periportal fibrosis, rarely even cirrhosis). The main problem is the risk of malignancy of the cyst (formation of cholangiocarcinoma), which is 10-15% in untreated patients.
ascending cholangitis, liver fibrosis or cyst perforation develop relatively quickly.
Children older than 1 year of age
the most common symptom is abdominal pain;
there may be palpable resistance in the right hypochondrium;
obstructive jaundice tends to be intermittent.
The so-called typical trias (abdominal pain, icterus and palpable resistance) is often mentioned in the literature. However, this only occurs in 10% of patients.
accurately determine anatomical deviations of the pancreatobiliary junction;
proves the event. dilation of intrahepatic bile ducts;
MRCP
the type of cyst can be determined very precisely;
if available, the method of choice;
intraoperative cholangiography is currently only used for unclear intraoperative findings;
an MRI or CT examination with the use of a contrast agent can be added - these methods are suitable for patients with concurrent pancreatitis or when malignancy of the cyst is suspected;
biochemistry – liver function tests may be normal or pathological in the sense of obstructive jaundice and cholangitis, and amylase may be elevated in concurrent pancreatitis.
Therapy
For type I and IV, radical excision of the cyst with cholecystectomy and replacement of the bile ducts (hepaticojejunoanastomosis) is performed. Gallbladder cyst can be operated laparoscopically. In a classic operation, the procedure is as follows:
It starts with an oblique right-sided incision in the right subcostal area (possible extension to the left);
the liver is released from its suspensory apparatus and luxates through the surgical wound;
the gallbladder and cystically dilated choledochus are released;
the common bile duct is resected at the level of the bifurcation;
the distal part of the bile duct is cut in the area of the head of the pancreas and sutured with an absorbable suture;
if radical excision of the cyst is not possible, mucosectomy is performed (prevention of malignant reversal);
retrocolic, 40 cm of jejunum is excluded and a wide jejunal anastomosis is made to bifurcate the bile ducts in the hepatic hilum.
In type II, excision of the diverticulum is performed .
In type III, the choledochocele is resected from a transduodenal approach.
For type V, a liver lobectomy is performed for unilateral involvement, in case of bilateral involvement, liver transplantation is the only treatment .
