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Operative Treatment of Choledochal CystsBenjamin N.J. Thomson, O. James Garden

Introduction

The earliest description of choledochal cysts is by Douglas in 1952 of a 17-year-old girlwith jaundice, fever and a painful mass in the right hypochondrium. Choledochal cystsin Western countries have an incidence of around 1 in 200,000 live births. There is ahigher incidence in Asia. Presentation is usually in childhood and 25% are diagnosed in the first year. Frequent association with other hepatobiliary diseases, such as hepaticfibrosis, has been noted. There is an association with an aberrant pancreaticobiliary duct junction.

Surgical resection is required to prevent episodes of sepsis and pain and because of the association with cholangiocarcinoma. In the Western literature the incidence of cholangiocarcinoma is lower than in Japanese series. Cyst-enterostomy should nolonger be performed. Classification is by Alonso-Lej et al.■ Type I Solitary cyst characterized by fusiform dilatation of the common bile

duct (most common) (A)■ Type II Diverticulum of the common bile duct (B)■ Type III Choledochocoele (C)■ Type IV Intrahepatic cysts in association with a choledochal cyst (second most

common) (E)■ Type V Caroli’s disease. Intrahepatic cystic disease with no choledochal cyst (F)

(Adapted from Todani et al. Am J Surg 1977; 134:263–269.)

Patients may also have a combination of intrahepatic and extrahepatic cysts (D).

A B C

D E F

Indications and Contraindications

Indications ■ Symptoms (jaundice, pain, cholangitis)■ Suspected or established malignancy■ Type I choledochal cyst■ Type II choledochal cyst■ Type IV choledochal cyst■ Previous cyst-enterostomy

Contraindications ■ Severe liver disease (e.g., Child-Pugh C cirrhosis)■ Severe portal hypertension■ Coagulopathy

Investigation/Preparation

Clinical: Chronic liver diseaseLaboratory tests: Bilirubin, alkaline phosphatase, ALT, albumin, prothrombin

time, plateletsRadiologic tests: Non-invasive; ultrasound, abdominal CT scan, invasive MRCP;

ERCP if intrahepatic ducts are not visualized on MRCPOperative preparation: Antibiotics to cover biliary pathogens

SECTION 4 Biliary Tract and Gallbladder642

Procedure

STEP 1 Right subcostal incision, with extension to the left rectus if required

Exposure with Doyen’s blades (see figure on page 644).Omnitract retractor for retraction of the liver.Duodenum kocherized.

STEP 1a

The incision needs to be smaller if: (a) there is no apex to the wound, (b) the incisionneeds to be moved superiorly, (c) there is a wider blade on the retractor or (d) less liveris needed on view.

STEP 1b

Intraoperative ultrasound is performed to identify the anatomy and extent of the cyst,to assess cyst wall thickness and intrahepatic extension, and to identify frequently found intracyst calculi.

Retrograde dissection is done of the gallbladder with division of the cystic artery.The cystic duct is followed to its insertion into the common bile duct/cyst.Mobilization of the cyst is performed from the portal vein and hepatic arteries.This dissection may be difficult due to dense inflammatory adhesions. The key is

to avoid intramural (cyst) dissection by dissection in the adventitial plane around thevessels from which the cyst (and nodes) are dissected.

The gallbladder is left in continuity to facilitate retraction of the cyst. This may be further aided by the placement of a vessel loop around the cyst.

Operative Treatment of Choledochal Cysts 643

STEP 2 Kocherization and mobilization of porta-hepatis

A full kocherization is performed to allow access to the lower bile duct and a retropancreatic approach.


STEP 3

The cyst is now mobilized inferiorly to its lowest extent into the head of thepancreas avoiding damage to the pancreatic duct.

Intraoperative ultrasound may have provided useful information in this regard.Ultrasonic dissection (CUSA) may aid in dissecting the cyst from the pancreas.Multiple small vessels may be divided with diathermy.


STEP 4 Opening the choledochal cyst

The cyst may require to be opened to enable visualization of the pancreatic duct,at its insertion into the common bile duct.

The cyst is controlled at its lower end above the junction of the bile duct and the pancreatic duct, which is preserved carefully (A). A view is shown looking into thepancreas with a partly mobilized cyst (B).


STEP 5 Repair of the distal common bile duct

If residual cyst lining remains it can be cauterized cautiously with diathermy current.The lower common bile duct is repaired with interrupted 5-0 PDS sutures just at its

junction with the pancreatic duct, damage to which is avoided carefully. The divideddistal duct is seen with the pancreatic duct on view and closure started.


STEP 6

The cyst is mobilized superiorly to the confluence of the right and left hepatic ducts.Assess carefully for aberrant ducts, which may need to be divided separately.The dissection may need to be extended along the extrahepatic course of the left duct

if it is ectatic.Some advocate extended liver resection for cysts extending into the left or right duct.It is possible to anastomose to the cyst at the confluence of the ducts, if this extends

more proximally, rather than performing liver resection.A hepaticojejunostomy is performed in an end-to-side manner with a single layer of

interrupted 4-0 PDS sutures. A Roux-en-Y limb of 70cm of proximal jejunum should beused.

A drain should be left adjacent to the hepaticojejunostomy. The cyst is removed andthe hepaticojejunostomy started (A, B).

Complications

Biliary Leak■ A small amount of bile drainage can be expected in the first 48hours, and will usually

settle.■ Continuing leakage should be managed conservatively with drainage.■ Keep an eye open for amylase-rich fluid if there is difficult pancreatic dissection

and/or persistent nonbile leak.

Late Complications■ Cholangitis (20–40%) usually in association with an anastomotic stricture.
