26 Choledochal Cyst

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Chapter 25 / Common Bile Duct Stones and Exploration 321

stone clearance, a completion cholangiogram should be performed. A cystic

duct drainage tube can be left in place if ndings on the cholangiogram are

equivocal. is tube can be used postoperatively for cholangiography and

radiographic treatment of retained stones if necessary. After LTCBDE, the

cystic duct stump should be secured with an endoloop or a suture ligature

and not be clipped.

Laparoscopic Choledochotomy

Laparoscopic choledochotomy (Fig. 25.6) is another excellent approach to

CBD stones when the CBD diameter is 6 mm or greater. e anterior wall

of the CBD is bluntly dissected and a longitudinal choledochotomy created

in the anterior wall of the bile duct distal to the cystic duct insertion. e

choledochotomy should be fashioned to accommodate the diameter of the

largest stone. Two stay sutures can be placed in the common duct and used

to tent up the anterior CBD wall to facilitate incision. A larger choledocho-

scope (3.3 mm, 2.4-mm working channel) can then be placed into the bile

duct and stones extracted with baskets or balloon catheters. e choledo-

chotomy is then closed over a T-tube with a 4-0 absorbable suture.

Advantages of laparoscopic choledochotomy relative to LTCBDE

include the ability to remove larger stones (>1 cm), to remove stones from

the proximal hepatic ducts, to remove multiple stones, and to use biliary

lithotripsy to fragment impacted stones. e disadvantages of laparoscopic

choledochotomy are that it requires a T-tube and considerable laparo-

scopic suturing skill to close the choledochotomy.

Clearance of all CBD stones is achieved in 75% to 95% of patients

with laparoscopic CBD exploration. Results from several randomized

FIGURE 25.6 Laparoscopic choledochotomy. (From Lillemoe K, Jarnigan W.

Master techniques in surgery: hepatobiliary and pancreatic surgery.

Philadelphia, PA: Lippincott Williams & Wilkins, 2012.)

Section III / Biliary322

prospective trials comparing a single-stage approach (laparoscopic

holecystectomy + laparoscopic common bile duct exploration) versus a

two-stage approach (laparoscopic cholecystectomy +either pre- or post-

operative ERCP) are shown in Table 25.2. e morbidity and mortality of

laparoscopic CBD exploration are similar to laparoscopic cholecystectomy

alone. e overall hospital length of stay tends to be shorter for patients

managed with a one-stage approach versus a two-stage approach.

Open Common Bile Duct Exploration

Open CBD exploration is performed much less frequently with the

increased use of endoscopic, percutaneous, and laparoscopic techniques

to remove CBD stones. e rst step is to perform a full Kocher maneuver

mobilizing the duodenum so that a hand can be placed behind the head

of the pancreas and the distal CBD palpated. Any impacted stones may be

milked proximally. e supraduodenal bile duct is then exposed, and two

stay sutures are placed in the CBD just below the cystic duct. e anterior

wall of the bile duct is then elevated with the stay sutures and a longitudi-

nal choledochotomy made. e bile duct then can be explored for stones.

Rigid instruments should not be used to extract stones because they can

injure the delicate ductal epithelium. A soft rubber irrigating catheter can

be used to gently ush out any stones or debris. Balloon-tipped catheters

then can be passed proximally and distally into the ducts to retrieve stones.

Adequate clearance of the duct should be conrmed visually with exible

choledochoscopy. Remaining stones can be removed by irrigation or by the

use of instruments such as stone forceps, wire baskets, or balloon catheters.

A T-tube should be placed in the bile duct and the choledochotomy closed

with 4-0 absorbable suture. Completion cholangiography is performed

before closing the abdomen to rule out the presence of retained stones or a

bile leak around the T-tube. Postoperatively, a T-tube cholangiogram is per-

formed 3 to 7 days after the exploration. If the cholangiogram is normal, the

tube can be clamped and the tube pulled 3 to 6 weeks later. If retained stones

are detected, the tract is allowed to mature, and percutaneous extraction

can be performed by a radiologist in 4 to 6 weeks. Open CBD exploration

can be accomplished with almost no mortality in patients younger than age

60 years but carries mortality of up to 4% in septic patients.

Author Year Treatment N

Duct

Clearance

(%)

Morbidity

(%)

Length

of Stay

(days)

Rhodes 1998 LC+ERCP

LC+LCBDE

3.5

Cuschieri 1999 ERCP+LC

LC+LCBDE

133

Nathanson 2005 LC+ERCP

LC+LCBDE

7.7

6.4

Noble 2009 ERCP+LC

LC+LCBDE

Rogers 2010 ERCP+LC

LC+LCBDE

LC, laparoscopic cholecystectomy; ERCP, endoscopic retrograde cholangiopancreatography; LCBDE,

laparoscopic common bile duct exploration.

Select Randomized Trials of One- versus Two-Stage

Management of CBD Stones

TABLE

25.2

Chapter 25 / Common Bile Duct Stones and Exploration 323

DRAINAGE PROCEDURES

Patients with an impacted CBD stone at the ampulla that cannot be

removed with a CBD exploration or those with multiple stones in a non-

dilated duct may require a transduodenal sphincteroplasty. A sphinc-

teroplasty also is indicated in the presence of an ampullary stenosis or a

choledochocele. e rst step in a sphincteroplasty is to perform a Kocher

maneuver. A small longitudinal duodenotomy is made over the ampulla,

and two stay sutures are placed on each side of the ampulla to elevate it.

A small incision is made at the 11 o’clock position in the sphincter taking

care to avoid the pancreatic duct, which is usually found at the 5 o’clock

position. e sphincterotomy is extended through the sphincter (approxi-

mately 1.5 cm) and the impacted stone removed. e bile duct and duo-

denal mucosa are then approximated with interrupted 4-0 absorbable

sutures. e duodenotomy should be closed transversely to prevent nar-

rowing of the duodenal lumen.

Patients with grossly dilated bile ducts (>2 cm), multiple stones

(>5), intrahepatic stones, primary CBD stones, or a distal biliary stricture

should be considered for a biliary drainage procedure. e two options

are a choledochoduodenostomy and a Roux-en-Y choledochojejunostomy.

Choledochoduodenostomy can be performed in either a side-to-side or an

end-to-side fashion. Advantages of a choledochoduodenostomy are that

it can be performed rapidly, it requires only one anastomosis, and the bile

duct still can be accessed endoscopically. However, a side-to-side anasto-

mosis leaves the distal CBD in continuity and can lead to the “sump syn-

drome.” In this situation, food debris from the duodenum can enter the

distal limb of the CBD and obstruct the anastomosis or the pancreatic duct

orice leading to cholangitis or pancreatitis. Long-term complications of

choledochoduodenostomy include a small but real incidence of cholangitis

and liver abscess.

Roux-en-Y hepatico- or choledochojejunostomy also is an excellent

option for biliary drainage. is operation is performed by dividing the

hepatic or common duct and doing an end-to-side anastomosis to a 60-cm

Roux limb of jejunum. Development of the sump syndrome is not a concern

because a hepatico- or choledochojejunostomy completely diverts bile ow

from the alimentary stream.

CONCLUSIONS

CBD stones may be approached endoscopically, laparoscopically, or by

open CBDE. Choice of therapy depends on both patient considerations as

well as local endoscopic and surgical expertise. Patients with primary CBD

stones must be considered for a biliary drainage procedure.

Choledochal Cyst

Alessandro Paniccia and Barish H. Edil

Section III / Biliary326

FIGURE 26.1 Classication of biliary cysts.

A. Type I. B. Type II. C. Type III. D. Type IV.

E. Type V. (From Lipsett PA, Pitt HA,

Colombani PM, et al. Choledochal disease:

a changing pattern of presentation. Ann

Surg 1994;220(5):644.)

Chapter 26 / Choledochal Cyst 327

Type III cyst, also known as choledochocele, is a dilation of the com-

mon bile duct conned to the wall of the duodenum. Type III CC represents

less than 10% of all cysts.

Type IV cyst refers to multiple cystic dilations of the biliary tract and

is further subclassied into type IVa where multiple cysts are present in the

intrahepatic and extrahepatic bile ducts and type IVb where multiple cysts

only involve the extrahepatic bile duct. Type IVa cyst is the second most

common type of CC representing 30% to 40%, while type IVb only accounts

for less than 5% of all cysts.

Type V cyst (Caroli disease) involves only the intrahepatic biliary

ducts and can be unilobar or bilobar. It represents less than 10% of all CCs.

DIAGNOSIS

Presentation

e historically described pathognomonic triad of abdominal pain, jaun-

dice, and right upper quadrant palpable mass is only present in 10% to 17%

of children. However, 85% of children present with at least two symptoms of

the triad compared to only 25% of adults.

Vague right upper quadrant abdominal pain associated with nausea

and emesis is often the presenting symptom in adults. Laboratory evalua-

tion of liver function and pancreatic enzymes frequently reects common

conditions such as cholangitis or pancreatitis, where concurrent pancreati-

tis may be present in 30% to 70% of adult patients. Additionally, concurrent

cystolithiasis and cholecystolithiasis are present in approximately 70% of

patients. As a result, an interval of up to 6 years from the rst presenting

symptoms to the diagnosis of cystic disorder of the biliary ducts is relatively

common. is delay in diagnosis often leads to additional procedures such

as cholecystectomy or other surgical exploration. Most often, adults are

diagnosed with CC during evaluation and treatment of complications such

as cholecystitis or acute pancreatitis. Diagnosis may also be discovered

incidentally during radiologic investigation for other problems. In contrast

to other cyst types, patients with type III choledochal cyst (choledochocele)

commonly present with acute pancreatitis.

Although rare, hepaticolithiasis and intrahepatic abscess have been

described as presenting complications of type IV and V bile duct cysts. is

can eventually lead to secondary biliary cirrhosis and portal hypertension

with subsequent liver failure.

Cyst rupture and biliary peritonitis have been also described as pre-

senting symptoms but usually in children. ese situations are extraordi-

narily rare.

e most common malignancy associated with CC disease is cholan-

giocarcinoma, though the presence of CC also increases the risk of developing

gallbladder cancer.

Imaging

Demonstration of the continuity of the cystic lesion with the biliary duct

is paramount in distinguishing it from other intra-abdominal cysts such

as pancreatic pseudocyst, biliary cystadenoma, or echinococcal cysts. In

patients with equivocal bile duct dilation (i.e., 1 to 2cm), the presence of

APBDJ strongly suggests the diagnosis of CC.

Abdominal ultrasound (US) is often the primary and the most cost-

eective imaging modality in diagnosing bile duct cyst; the sensitivity of US

ranges from 71% to 97%.

Endoscopic ultrasound has been described in the literature in order

to overcome the intrinsic limitations of transabdominal US such as body

habitus, bowel gas, the presence of overlying intra-abdominal structures,

Section III / Biliary328

and poor visualization of distal bile duct. It also gives you information of the

pancreas and biliary duct anatomy.

Computed tomography (CT) scan is now widely available and pres-

ents several advantages in the diagnosis of CC. Some of these advantages

include characterization of intrahepatic bile ducts, dilation evaluating the

cysts surrounding structures, and more visualization of the distal bile duct

and the pancreatic head. It also allows for evaluation of malignancy that

may mimic CC or may be originating from a CC. (i.e., very thick cyst wall or

portal lymphadenopathy).

Computed tomography cholangiography (CTCP) though not widely

available currently has 90% sensitivity for diagnosis of CC and can correctly

visualize the bile duct anatomy prior to a surgical procedure.

Magnetic resonance cholangiography (MRCP) is becoming the gold

standard in diagnosis with a reported sensitivity ranging between 90% and

100%. ough MRI/MRCP is relatively more expensive than CT, MRCP oers

similar excellent cross-sectional imaging evaluation as well as superior

visualization of the entire biliary tree (including APBDJ).

Particularly challenging in children is proper distinction between

biliary atresia and bile duct cyst. In this situation, a

technetium HIDA

scan will demonstrate continuity of the cystic lesion with the bile duct and

eventually emptying of contrast into the bowel. Retention of contrast in the

biliary system is typical of biliary atresia or any condition causing distal

common bile duct (CBD) obstruction. e sensitivity of a hepatobiliary imi-

nodiacetic acid (HIDA) scan, however, is subject to wide variation ranging

from 100% for type I to only 67% for type IVa. is inaccuracy is mainly due

to HIDA inability to visualize the intrahepatic bile ducts.

In light of the increased accuracy of MRCP and CTCP, invasive chol-

angiography modalities such as percutaneous transhepatic cholangiogram

(PTC) and endoscopic retrograde cholangiopancreatography are applied

less frequently. However, in the scenario where biliary drainage is inade-

quate (type IV/V), PTC and stent placement may be required.

An exception is made for type III CC in which endoscopy is considered

the principal diagnostic modality because of its potential to also provide

therapeutic intervention. is modality allows cannulation of the papilla of

Vater and eventually opacication of the dilated intramural common bile

duct. In addition, sphincterotomy is therapeutic for these cysts.

MANAGEMENT

Surgical treatment of CCs has undergone signicant changes during the

last century. Several surgical techniques have been described including cyst

marsupialization, choledochorrhaphy, and internal drainage via cystenter-

ostomy to the duodenum or jejunum. e results with the above techniques

have been unsatisfying due to signicant associated morbidity and mortal-

ity such as ascending cholangitis, anastomosis stricture, stone formation,

and a remarkable 30% incidence of postoperative malignancy occurring

15 years earlier than primary malignancy. erefore, any treatment after

complete resection is of historical interest only.

e general consensus today is that surgical management depends on

cyst type and that complete cyst excision should be obtained in order to

decrease the risk of associated malignancy.

Type I CC is successfully treated by complete cyst resection with a

Roux-en-Y hepaticojejunostomy either in an open fashion or, when techni-

cally feasible, with a laparoscopic approach.

e technical aspects of this operation involve mobilization of the

hepatic exure and wide Kocher maneuver because the cyst extends

Chapter 26 / Choledochal Cyst 329

posterior to the duodenum into the head of the pancreas. Dissection on the

anterior cyst is continued caudally into the pancreatic head until the bile

duct narrows, which is the inferior portion of the cyst. is area is ligated,

and then, the cyst is reected cephalad. Dividing the bile duct and displacing

the cyst anteriorly allow identication of the portal vein. Posterior dissection

is continued until the bile duct is transected just distal to the hepatic duct

conuence. Care should be taken not to injure the right hepatic artery, which

commonly passes posterior to the bile duct and anterior to the portal vein.

Depending on bile duct size, some biliary surgeons prefer to reconstruct this

anastomosis across soft Silastic transhepatic stents. A 60-cm limb is brought

up in a retrocolic fashion for a Roux-en-Y hepaticojejunostomy. ere is an

associated 7% complication rate including early anastomotic leak, pancre-

atic leak due to injury to the pancreatic duct, and bowel obstruction. Late

complications include cholangitis, pancreatitis, and anastomotic stricture.

e important point is that complete cyst excision is required to prevent

future degeneration of CC into cholangiocarcinoma.

Type II CC can be treated with simple cyst excision. e procedure

of choice depends on cyst size and location. Commonly used surgical

approaches are an extrahepatic biliary resection and Roux-en-Y reconstruc-

tion or complete excision with primary closure over a T tube. In order to

prevent segmental bile duct narrowing, the defect in the wall of the common

bile duct should be closed transversely or over a T tube.

Type III CCs have an extremely low risk of malignancy. ese cysts

more commonly present with pancreatitis or abdominal pain. ey may

also be diagnosed in the setting of pancreatic or biliary obstructive symp-

toms or much more rarely gastric outlet syndrome. Dierent treatments

have been proposed ranging from simple endoscopic sphincterotomy to

complete cyst excision through a lateral duodenostomy. If operation is

undertaken, it is of primary importance to identify the pancreatic duct in

order to avoid accidental injuries. Cannulation of the pancreatic duct with

Silastic tubes prior to cyst resection can be used to identify the pancreatic

duct and avoid injuries. Eventually the cyst can be excised and sphincter-

oplasty can be performed. Some authors believe that the pancreatic and

biliary duct should be routinely separated and reanastomosed to the duo-

denum in order to avoid pancreaticobiliary mixing. Most authorities feel

that endoscopic “unroong” provides adequate therapy for type III CC.

Type IV cyst treatment is dictated by the presence or absence of intra-

hepatic involvement. When intrahepatic involvement is diuse, complete

cyst removal requires partial hepatectomy.

Type IVb is treated in the same fashion of type I cyst.

e treatment for type IVa cyst depends on the extent of the intra-

hepatic involvement and the presence of cirrhosis and/or portal hyperten-

sion. If the intrahepatic involvement is conned to a single lobe, a wide hilar

hepaticojejunostomy with segmental hepatectomy can be performed. If the

intrahepatic component is diuse and bilobar involvement is encountered,

extrahepatic biliary excision should still be performed, and drainage of

intrahepatic biliary tree should be considered with two large-bore Silastic

stents. e aim of this approach is to reduce intrahepatic biliary stasis, chol-

angitis, and stone formation potentially decreasing the risk of liver damage

and malignant transformation. Some authors recommend having trans-

plantation in this setting; treatment of patients with type IV CCs must be

determined in the setting of an experienced multidisciplinary group.

Type V cyst (Caroli disease) treatment is dictated by extent of intrahe-

patic disease and the presence or absence of cirrhosis, portal hypertension,

and liver failure. Early recognition of this disease is paramount in order to

avoid recurrent cholangitis, biliary stasis, and intrahepatic abscess formation.

Section III / Biliary330

Initial nonoperative management may be warranted to treat cholan-

gitis, intrahepatic stone formation, as well as biliary stasis in the attempt to

decrease progressive liver injury.

In the absence of portal hypertension, if there is diuse liver disease

extrahepatic bile duct excision, biliary enteric anastomosis and permanent

drainage of the intrahepatic cyst with large-bore Silastic stents may be con-

sidered. In cases of unilobar liver involvement, the treatment consists of

extrahepatic bile duct excision and segmental hepatectomy. Orthotropic

liver transplant is an option for Caroli disease with bilobar cystic involve-

ment. Outcomes of transplantation are comparable to thos e for other trans-

plant populations; however, the timing of transplant remains controversial.

OUTCOME/FOLLOW-UP

e treatment of choledocal cyst disease with complete resection is suc-

cessful in preventing degeneration into cancer. Early complications include

biliary anastomotic leak, pancreatic leak in case of accidental pancreatic

duct injury, and bowel obstruction due to intussusception or internal

hernia.

Late complications include biliary anastomotic stricture and recur-

rent cholangitis. ese complications are seen in approximately 25% to 35%

of the cases and are often associated with stone formation. Percutaneous

dilation of anastomotic stricture with biliary stent placement usually pro-

vides durable treatment.

Despite excision of CCs, the risk of malignancy in the remaining bile

ducts is estimated between 0.7% and 6% likely due to subclinical malignant

disease not detected at the time of surgery or incomplete cyst excision.

erefore, patients diagnosed with cystic disorder of the biliary ducts require

lifelong postoperative surveillance with serial imaging and monitoring of

serum liver enzymes and tumor markers (CA 19–9).

CONCLUSION

Although cystic disorder of the biliary ducts remains a rare condition, in

recent years, it is being recognized with increased frequency in the western

population.

e importance of CCs is related to the lifetime risk of malignancy of

approximately 10%. Cholangiocarcinoma remains the most common type

of cancer arising from CCs, though these patients also have an increased

risk for developing gallbladder cancer. Complete surgical resection of dis-

eased bile ducts is the treatment of choice and should be pursued when

technically possible in reasonable surgical candidates. When surgical

excision is not possible, interventions should be considered such as tran-

shepatic drains in order to decrease biliary stasis and improve symptom-

atology. Complete excision of the cyst can prevent biliary cancer. If CC

disease is left behind as seen with type IV and type V, lifelong management

and cancer surveillance are necessary. Even with complete CC excision,

lifetime surveillance should continue, as late complications are common.