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Operative Treatment of Choledochal Cysts

Benjamin N.J. Thomson, O. James Garden

The earliest description of a choledochal cyst is by Douglas in 1952, involving a 17-year-old girl
with jaundice, fever, and a painful mass in the right hypochondrium. The incidence of choledochal
cysts in Western countries is about 1 in 200,000 live births; the incidence is higher in Asia. Presen-
tation is usually in childhood, and 25 % are diagnosed in the first year. Frequent association with
other hepatobiliary diseases, such as hepatic fibrosis, has been noted. There is also an association
with an aberrant pancreaticobiliary duct junction.
Surgical resection is required to prevent episodes of sepsis and pain and because of the associa-
tion with cholangiocarcinoma. In the Western literature, the incidence of cholangiocarcinoma is
lower than in Japanese series. Cystenterostomy should no longer be performed. The classification
of choledochal cysts is based on a system proposed by Alonso-Lej et al. and later modified by

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Todani et al. (Am J Surg 1977, 134:263–9.):
Type I: Solitary cyst characterized by fusiform dilatation of the common bile duct (most

-- common) (. Fig. 78.1a)
Type II: Diverticulum of the common bile duct (. Fig. 78.1b)

- Type III: Choledochocele (. Fig. 78.1c)

Type IV: Communicating intrahepatic and extra-hepatic cysts (second most common)

- (. Fig. 78.1e)
Type V: Caroli disease. Intrahepatic cystic disease with no choledochal cyst (. Fig. 78.1 f)

Patients may also have a combination of intrahepatic and extrahepatic cysts (. Fig. 78.1d).

.. Fig. 78.1 

P.-A. Clavien, M. G. Sarr, Y. Fong, M. Miyazaki (Eds.), Atlas of Upper Gastrointestinal and Hepato-Pancreato-Biliary Surgery,
DOI 10.1007/978-3-662-46546-2_78, © Springer-Verlag Berlin Heidelberg 2016
 714 Section IV  •  Biliary Tract and Gallbladder

Indications and Contraindications

--
1
Indications Symptoms (jaundice, pain, cholangitis)
2
-- Type I choledochal cyst
Type II choledochal cyst
3
4
- Type IV choledochal cyst
Previous cystenterostomy

Type III choledochal cysts are usually managed with endoscopic treatment. Suspected or con-
firmed malignant transformation should be managed with cyst excision and consideration of
5 pancreaticoduodenectomy.

Contraindications
-- Severe liver disease (e. g., Child-Pugh C cirrhosis)

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6 Severe portal hypertension
Coagulopathy
7
Investigation/Preparation

--
8
Clinical: Chronic liver disease, prior surgery
78
- Laboratory tests: Bilirubin, alkaline phosphatase, ALT, albumin, prothrombin time, platelets
Radiologic tests: Noninvasive – ultrasound, abdominal CT scan, MRCP; Invasive – ERCP if
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- intrahepatic ducts are not visualized on MRCP
Operative preparation: Antibiotics to cover biliary pathogens

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Chapter 78  •  Operative Treatment of Choledochal Cysts
715 78
Procedure

Step 1 Access and exposure

A right subcostal incision is used, with extension to the left rectus if required. Exposure is achieved
with a retractor (Omnitract retractor; Thompson retractor; Doyen’s blades); the liver is retracted.
The incision can be tailored to body habitus and to achieve best access. Laparoscopic approaches
are described and should employ the same principles as outlined below.
Intraoperative ultrasound is performed to identify the anatomy and extent of the cyst, to as-
sess cyst wall thickness and intrahepatic extension, and to identify intracyst calculi, which often
are found.
The gallbladder is dissected in retrograde fashion with division of the cystic artery. The gall-
bladder is often shrunken.
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 because
of dense, inflammatory adhesions. The key is to avoid intramural (cyst) dissection by sharp dis-
section in the adventitial plane around the vessels from which the cyst (and nodes) are dissected.
The gallbladder is left in continuity to facilitate retraction of the cyst. Dissection may be further
aided by the placement of a vessel loop around the cyst. (. Fig. 78.2)

.. Fig. 78.2 
 716 Section IV  •  Biliary Tract and Gallbladder

Step 2 Kocherization and mobilization of the porta hepatis

1
Full kocherization is performed to allow access to the lower bile duct and a retropancreatic ap-
2 proach (. Fig. 78.3).

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.. Fig. 78.3 

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Chapter 78  •  Operative Treatment of Choledochal Cysts
717 78
Step 3 Mobilization of the cyst

The cyst is now mobilized inferiorly to its lowest extent into the head of the pancreas, avoiding
damage to the pancreatic duct. Intraoperative ultrasound may provide 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 (. Fig. 78.4).

.. Fig. 78.4 
 718 Section IV  •  Biliary Tract and Gallbladder

Step 4 Opening the choledochal cyst

1
It may be necessary to open the cyst to enable visualization of the pancreatic duct, at its insertion
2 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 (. Fig. 78.5a). A view is shown looking
into the pancreas with a partly mobilized cyst (. Fig. 78.5b).
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.. Fig. 78.5 
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Chapter 78  •  Operative Treatment of Choledochal Cysts
719 78
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 divided distal duct is seen
with the pancreatic duct on view and closure started (. Fig. 78.6).

.. Fig. 78.6 
 720 Section IV  •  Biliary Tract and Gallbladder

Step 6 Reconstruction
1
The cyst is mobilized superiorly to the confluence of the right and left hepatic ducts. Assess care-
2 fully 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
3 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 inter-
4 rupted 4-0 PDS sutures. A Roux-en-Y limb of 70 cm of proximal jejunum should be used.
A drain should be left adjacent to the hepaticojejunostomy. The cyst is removed and the he-
5 paticojejunostomy started (. Fig. 78.7).

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14 .. Fig. 78.7 

15
16 Complications

17 Biliary leak

-- A small amount of bile drainage can be expected in the first 48 hours; it will usually settle.

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18 Continuing leakage should be managed conservatively with drainage.
Keep an eye open for amylase-rich fluid if there is difficult pancreatic dissection and/or
19 persistent nonbile leak.

20 Late complications

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- Cholangitis (20–40 %), usually in association with an anastomotic stricture.

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