Choledochal cyst

• Bile duct (choledochal) cyst is a rare congenital anomaly of the bile ducts.
• It is often associated with an anatomical disorder associated with the bile and pancreatic ducts.
• The cyst wall is deciphered to a width of 2-7 mm, consisting 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 widespread.
• Liver changes are only in older children with chronic disease (periportal fibrosis, rarely cirrhosis).
• The main problem is the risk of cyst malignancy (development of cholangiocarcinoma), which is 10-15% in untreated patients.

Classification of cysts

• There are 5 basic types (classification according to Todani):
  • type I - cystic dilatation of the external bile ducts (90-95% of all patients);
  • type II - bile duct diverticulum;
  • type III - cystic dilatation of the intraduodenal part of the bile duct (so-called choledochocele);
  • type IV - multiple cysts of extra- and intrahepatic bile ducts;
  • type V - cystic dilatation of intrahepatic bile ducts (so-called Caroli's disease).

Clinical picture

1. Children under 1 year of age
   • the first manifestation is obstructive jaundice;
   • ascending cholangitis, liver fibrosis or cyst perforation develop relatively quickly.
2. Children older than 1 year of age
   • the most common manifestation is abdominal pain;
   • there may be palpable resistance in the right hypochondria; obstructive jaundice tends to be intermittent.

• The so-called typical Triassic (abdominal pain, jaundice, and tactile resistance) are often mentioned in the literature.
• However, it occurs in only 10% of patients.

Diagnostics

1. Ultrasound of the abdomen
   • cyst size;
   • anatomical proportions of the proximal bile ducts;
   • vascular anatomy;
   • liver echogenicity;
2. ERCP
   • cyst size;
   • accurately determine the anatomical abnormalities of the pancreatobiliary junction; proves event. dilatation of intrahepatic bile ducts;
3. MRCP
   • the type of cyst can be determined very precisely;
   • if available, the method of choice;
4. perioperative cholangiography is currently used only for unclear perioperative findings;
5. contrast-enhanced MRI or CT scans may be added - these methods are suitable for patients with concomitant pancreatitis or suspected cyst malignancy;
6. biochemistry - liver function tests may be normal or pathological in the sense of obstructive jaundice and cholangitis, there may be increased amylase in concomitant pancreatitis.

Therapy

• In types, I and IV, radical excision of the cyst with cholecystectomy and replacement of the bile ducts (hepaticojejunoanastomosis) is performed.
• The choledochal cyst can be operated laparoscopically.
• The procedure for the classic operation is as follows:
  • It begins with a sloping right incision in the right lower jaw (possible extension to the left);
  • the livers are released from their suspension apparatus and luxate through the surgical wound;
  • the gallbladder and cystically dilated choledochus are released;
  • the common bile duct is resected at the bifurcation level;
  • the distal part of the bile duct is severed in the head area of ​​the pancreas and sutured with an absorbable suture;
  • if radical excision of the cyst is not possible, mucosectomy (prevention of malignant reversal) is performed;
  • 40 cm jejunum is excreted retrocollicaly and a broad jejunal anastomosis is made to bile the bile ducts in the hepatic hilum.
• In type II, diverticulum excision is performed.
• In type III, the choledochocele is resected from the transduodenal approach.
• In type V, liver lobectomy is performed for unilateral disease, in the case of bilateral disease, liver transplantation is the only treatment.

Early complications:

1. anastomosis dehiscence,
2. pancreatitis,
3. bleeding,
4. ileus.

Late complications:

1. stricture in the anastomosis,
2. recurrent cholangitis,
3. gallstones,
4. pancreatitis,
5. malignant reversal.

Links

External articles

• Žlučové cesty
• Játra
• Cholelitiáza
• Operační výkony na žlučníku a žlučových cestách
• Diagnostické zobrazovací metody při vyšetření žlučníku a žlučových cest
• Nemoci žlučníku a pankreatu u dětí

Used literature

• ŠNAJDAUF, Jiří a Richard ŠKÁBA. Dětská chirurgie. 1. vydání. Praha : Galén, 2005. ISBN 807262329X.