https://doi.org/10.1016/j.hpb.2022.09.

010 HPB

REVIEW ARTICLE

Diagnosis and management of choledochal cysts

Zachary J. Brown1, Azarakhsh Baghdadi2, Ihab Kamel2, Hanna E. Labiner1, D. Brock Hewitt1 &
Timothy M. Pawlik1
1
Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, and 2Department of Radiology, John
Hopkins University, Baltimore, MD, USA

Abstract
Background: Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic
bile ducts. We review the pathophysiology, diagnosis, and management of CCs.
Methods: MEDLINE/PubMed and Web of Science databases were queried for “choledochal cyst”, “bile
duct cyst”, “choledochocele”, and “Caroli disease”. Data were synthesized and systematically reviewed.
Results: Classified according to the Todani Classification, CCs are generally believed to arise sec-
ondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct
union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis,
liver abscess, liver cirrhosis and malignant transformation (3–7.5%). Radiological and endoscopic im-
aging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper
management. Management is generally guided by cyst classification with complete cyst excision
necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall
survival after choledochal cyst excision is 95.5%.
Conclusion: Most CCs should undergo surgical intervention to mitigate the risk of cyst related com-
plications such as cholangitis and malignant transformation.

Received 2 April 2022; accepted 28 September 2022

Correspondence
Timothy M. Pawlik, Department of Surgery, The Ohio State University Wexner Medical Center, 395 W.
12th Ave., Suite 670. E-mail: [email protected]

Introduction above eligibility criteria. Searches and article extraction were

conducted by the first author (ZJB) and reviewed by the author
Choledochal cysts (CCs) are rare cystic dilations of the intra-
team. Articles identified from the search strategy were uploaded
hepatic and/or extrahepatic bile ducts.1 Approximately 80% of
screened for relevant data, which were subsequently extracted
CCs are diagnosed in childhood – most often presenting with a
and synthesized.
right upper quadrant mass, abdominal pain and jaundice.2–4 The
greater use of cross-sectional imaging has increased detection of
Classification, pathophysiology and etiology
CCs among adults5 with the incidence of CCs being four-fold
In 1959, Alonso-Lej, Rever, and Pessagno were the first to pro-
higher among females.1,6 Additionally, CCs occur more
pose a classification system for CCs.8 The Alonso-Lej classifica-
frequently in Asian versus Western populations with an inci-
tion system classified CCs into three types according to the
dence of 1 in 13,000 versus 1 in 150,000 people, respectively.7 We
nature of the dilation of the extrahepatic bile ducts (Table 1).
herein review the pathophysiology, diagnosis, and management
Based upon the Alonso-Lej classification, type I CCs are
of CCs.
congenital cystic dilations of the common bile duct (CBD), type
II cysts are congenital diverticulum of the CBD with a narrow
Methods
neck, while a type III cyst are a congenital choledochocele with
A systematic review was performed utilizing MEDLINE/PubMed dilation of the intraduodenal portion of the CBD.8
and Web of Science databases with the end of search date January Subsequently, Todani and colleagues combined the Alonso-Lej
31, 2022. In PubMed, the terms “choledochal cyst,” “bile duct extrahepatic biliary cyst classification system with intrahepatic
cyst,” “choledochocele,” and “Caroli disease” were searched in the CC of Caroli disease.9 The Todani classification of CCs is
title. Articles published in English were assessed according to the therefore based upon the extent of extrahepatic, as well as

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Table 1 Alonso-Lej classification of choledochal cysts ducts without (type 1A) or with (type 1B) dilation of the
Choledochal Characteristics common channel. Type II is defined as an acute dilation between
cyst type the ducts without (type IIA) or with (type IIB) dilation of the
I Congenital cystic dilation of the common duct common channel. Type III has several subclassifications with
II Congenital diverticulum of the common duct with a type IIIA being similar to a pancreatic divisum. Type IIIB has no
narrow neck. duct of Wirsung. TypeIIIC also has several subclassifications
III Congenital choledochocele with dilation of the where type IIIC1 has a small communicating duct between the
intraduodenal portion of the common bile duct. main duct and accessory duct, type IIIC2 has a common channel
made up of common and accessory ducts of the same caliber, and
type IIIC3 is characterized by total or partial dilation of the
intrahepatic biliary involvement (Table 2). Based on the Todani
ductal system.17
Classifiation, type I CCs are characterized by a fusiform dilation
Abnormal pancreaticobiliary maljunction, which is charac-
of the CBD with normal intrahepatic ducts, type II CCs are a
terized as union of the pancreatic and CBD outside of the
focal supraduodenal diverticulum, and type III CCs, also known
duodenal wall, has been associated with type I and IV CCs;
as choledochoceles, are intraduodenal cystic dilations of the
however, these abnormalities are often noted among patients
CBD. Type IV CCs are characterized by intrahepatic and extra-
without CCs – calling into question the actual impact on CC
hepatic ductal dilation or multiple areas of extrahepatic dilation
risk. Interestingly, pancreaticobiliary maljunction has been
without involvement of the intrahepatic duct; type V CCs, also
linked to an increased risk of biliary tract cancer without the
known as Caroli Disease, is characterized by variable degrees of
presence of a CC.18,19 The pathophysiology and etiology of CC
intrahepatic bile duct dilation. Type I CCs are more often seen in
may also have a hereditary component as there have been reports
children, while type IV are more prevalent in adults.7,9
of multiple occurrences of CC within the same family.20 Inter-
Although the etiology of CCs is not well understood, most CCs
estingly, similar to the pathophysiology of Hirschsprung disease,
likely develop as a result of an anomalous pancreaticobiliary duct
which is characterized by the absence of migration of ganglion
union (APBDU). An APBDU is characterized by a long common
cells to the distal colon, Kusunoki et al. demonstrated a signifi-
channel (>10 mm proximal to the ampulla) of the pancreatic
cant reduction of ganglion cells in the narrow portion of the CC
duct and CBD that predisposes to reflux of pancreatic fluid into
compared with the dilated portion.21 As such, the absence of
the biliary tree leading to biliary duct inflammation, increased
ganglion cell migration may be another mechanism associated
pressure, and, eventually, biliary ductal dilation.10–12 The CBD
with CC formation.
and pancreatic duct usually unite within the sphincter of Oddi to
form a common channel with a normal length of 0.2–1.0 cm.13
Complications
An anomalous union creates an abnormal channel that allows
Presenting symptoms of CCs may include abdominal pain and
pancreatic enzymes to enter the biliary tree weakening the CBD
jaundice. In a multi-institutional analysis of 394 patients who
wall that facilitates cyst formation.14 In support of this theory,
underwent resection of CCs, 84.5% of patients with CC
biliary manometry has documented high concentrations of
presented with symptoms while 15.5% of individuals were
pancreatic enzymes in CC fluid.15 Of note, while only 2% of
asymptomatic. Adults were more likely to present with abdom-
individuals without CCs have an APBDU, up to 96% of patients
inal pain, while children were more likely to present with jaun-
with CCs have a documented APBDU.16
dice. Other CC-related symptoms may include cystolithiasis,
Komi et al. classified the AUPBD into three types according to
cholecystitis, pancreatitis, liver abscess, and liver cirrhosis.22–24
the ductal union and the angle of the junction of the pancreatic
Pancreatitis may be particularly common among patients with
duct and CBD. Type I is defined as a right angle between the
CCs with a prevalence of up to 70%.25,23,26–28 Pancreatitis may
be secondary to the altered pancreaticobiliary ductal anatomy
Table 2 Todani classification of choledochal cysts characterized by the long common channel, as well as the
Choledochal Characteristics development of cystolithiasis.23,27 In particular, pancreatitis may
cyst type be due to bile reflux into the pancreatic duct.29,28 Other clinical
I Fusiform or cystic dilation of the common hepatic complications of CC include development of hepatolithiasis as a
duct or the common bile duct. result of bile stasis or migration of intra-cystic stones, which can
II Supraduodenal diverticulum occur more often among patients with type IV CCs.30 Bouts of
III Intraduodenal cystic dilation of the bile duct. recurrent biliary obstruction and resultant cholangitis can lead to
IVa Dilation of both the intrahepatic and extrahepatic the development of intrahepatic abcesses.31,32 Furthermore,
ducts. portal hypertension may develop secondary to biliary cirrhosis or
IVb Multiple areas of extrahepatic dilation without fibrosis, portal vein thrombosis, or Caroli disease.33,24
involvement of the intrahepatic ducts The most feared complication of CCs is malignant trans-
V Dilation of only the intrahepatic bile ducts formation. Among patients with CC, the incidence of malignant

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transformation has been reported to be around 3–5%.1 The true ERCP as the imaging modality of choice to delineate cyst anat-
incidence of malignant transformation in CCs remains un- omy. The benefits of MRCP include its non-invasive nature and
known, however. The risk of malignancy has been strongly its ability to define cyst anatomy relative to surrounding
linked with increasing age, as the risk of cancer – while not zero structures.39,7,41,35,42
– is very low among the pediatric CC population.11 The asso- Given the identification of increasing numbers of asymp-
ciation of age with malignancy risk has been postulated to be tomatic patients, indications and timing of surgery– especially
related to the long-term chronic exposure of the biliary epithe- among individuals at the extremes of the age spectrum – can be
lium to the pancreatic fluid, especially among patients with nuanced. For example, given that the risk of malignancy is
APUBD. The risk of CC malignancy is also higher among pa- relatively low, elderly patients who have multiple comorbidities
tients with type I or type IV CCs with a marked lower incidence may not benefit from operative intervention for CC. Clinicians
among patients with type V CC; cancer has rarely been reported that management is largely dictated by CC type, accurate imaging
among patients with type II or III CCs.34 In a pooled analysis of to determine CC extent and type is critical to determine treat-
78 studies, the prevalence of malignancy was 7.5% (434 cancer ment planning. In addition, when an operation is being
diagnoses out of 5780 patients); cholangiocarcinoma and gall- considered accurate anatomic delineation of relationship of bile
bladder cancer were the most common malignancies.2 In duct to the pancreatic duct is important. Aberrant biliary and
particular, adenocarcinoma is the most common histologic pancreatic anatomy may be common and needs to be identified
subtype comprising 73–84% of cancers; other histologies using preoperative imaging.43 Additionally, prior to surgical
include anaplastic carcinoma (10%), undifferenced cancer intervention, any underlying biliary infection should be
(5–7%), and squamous cell carcinoma (5%).35 controlled with either broad-spectrum antibiotics and/or endo-
Major post-operative morbidity, grade 3 or higher, is similar scopic or percutaneous drainage procedures.
between children and adults; overall peri-operative morbidity
may be higher, however, among adults (35.1% vs 16.3%, Management based upon cyst type
p < 0.001). Adults more often have complications involving Historically, CCs were treated surgically with cyst enterostomy.
seromas (3.1% vs 0%, p = 0.04), wound infections (9.7% vs This operative approach was associated with a high incidence of
1.5%, p < 0.001), and perihepatic abscesses (7.7% vs 0%, long-term complications such as stricture, jaundice, cholangitis,
p < 0.001), while children more often have anastomotic leaks need for repeat surgery, and persistent risk of chol-
(3.0% vs 0%, p = 0.01) and GI tract perforations (3.0% vs 0%, angiocarcinoma.44 Even though partial cyst excision or cyst en-
p = 0.01).1 terostomy may relieve the biliary obstruction, complete cyst
excision is required to mitigate the risk of malignancy.7,44 As
Imaging noted, the risk of malignancy varies significantly based on CC
Radiological and endoscopic imaging is the cornerstone of CC type and, therefore, so does the operative approach.
diagnosis. The initial work-up for a patient suspected of having a
CC generally begins with an ultrasound (US) or computed to- Type I
mography (CT) scan. US is generally utilized more often in the Treatment of type I CC (Figs. 1–3) should include complete cyst
pediatric population, while adults are more likely to undergo a excision with Roux-en-Y hepaticojejunostomy (HJ). If the CC
CT.1 The key imaging feature of CCs on US, CT, or magnetic extends into the pancreatic head, pancreaticoduodenectomy
resonance imaging (MRI) is a bile duct in continuation with (PD) may sometimes be needed to remove the CC completely,
areas of cystic dilation.36 Communication with the biliary tree is however “coring out” the entire CC behind the pancreatic head
important to differentiate a choledochal cyst from a mucinous can often be accomplished. The risk of leaving residual CC
cystic neoplasm (MCN) of the liver, as a MCN does not should always be balanced against the increase morbidity of
communicate with the biliary tree.36 Additionally, dilation of the performing a PD.35 Biliary reconstruction typically involves a
bile ducts should not be associated with an identifiable me- standard HJ. Hepaticoduodenostomy (HD) had previously been
chanical obstruction.37 Use of a hepatobiliary contrast agent may considered an option as it provides the advantage of access to the
assist in determining a possible connection with the biliary tree.38 biliary tract via endoscopy for future interventions if needed.45
Full delineation of cyst anatomy is imperative for proper The ability to perform a HD may be limited, however, by the
management. After US or CT, cholangiography with percuta- mobility of the duodenum and the inability to perform a
neous transhepatic cholangiography, endoscopic retrograde tension-free anastomosis. In addition, some data suggest that HD
cholangiopancreatography (ERCP), or magnetic resonance may be associated with increased risk of gastric and biliary
cholangiopancreatography (MRCP) may be helpful to charac- cancers, as using an operative approach that allows for continued
terize more fully the cyst anatomy. ERCP provides diagnostic and exposure of the biliary tree to pancreatic fluid is counter-
therapeutic benefit allowing for interventions such as stent intutive.46 As such, HD has largely been abandoned. Rather,
placement to address cyst-related complications such as chol- complete cyst excision and HJ reconstruction is considered the
angitis.39,40 At most centers, MRCP is preferentially utilized over treatment of choice for type I CC.

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patients with a type I CC underwent complete cyst excision,

cholecystectomy and bilioenteric anastomosis. There were no
peri-operative moralities, one patients developed pancreatitis
and anastomotic leak post-operatively which was managed
conservatively.35

Type II
Management of type II CC (Fig. 4) needs to take into account the
connection of the cyst to the bile duct relative to the size of the
connection. For CCs with a small cyst neck, the connection can
be ligated or closed primarily. Conversely, larger connections
may require closure over a T-tube after excision or en-bloc cyst
excision with the adjoining bile duct with a Roux-en-Y HJ
reconstruction. The decision should be based on anatomic and
technical considerations as the data would suggest no clear
Figure 1 Type I choledochal cyst in a 27-year-old female. Coronal benefit of en-bloc CC excision and reconstruction over cyst
MRCP image shows fusiform dilatation of the common duct (arrow) resection alone.48
and normal intrahepatic ducts In a retrospective European multicenter study, 350 patients with
congenital CCs were identified of which 19 were type II CCs (17 in
As a result of recurrent episodes of cholangitis and chronic adults and 2 in children). The biliary diverticulum was located in
inflammation in adult patients, CC excision may sometimes be the upper, middle, or lower third of the bile duct in 58%, 21%, or
more technically challenging among adult versus pediatric pa- 21% of cases respectively. Five patients were asymptomatic while
tients.47 In particular, the posterior aspect of the CC may be 14 patients presented with symptoms such as abdominal pain,
adherent to the anterior aspect of the portal vein making com- weight loss, jaundice or cholangitis. One patient presented with a
plete removal not feasible without dramatically increasing the synchronous cancer. All patients underwent resection of the
complexity of the operation. In these instances, resection of the diverticulum and 58% of cases required a concomitant resection
anterior wall of the cyst may be resected with fulguration of the of the extrahepatic bile duct. The post-operative complication rate
mucosa on the posterior wall mucosa. If the inflammatory ad- was 15.3% with 5.3% of patients experiencing a serious compli-
hesions are too dense or the patient has severe portal hyper- cation and no deaths were reported. Two patients had complica-
tension, a choledochocystojejunostomy remains an alternative, tions which required additional treatment including one patient
albeit less optimal, treatment option.23,24,27 In a case series from who underwent an R1 resection for a synchronous cancer and
a single institution, 10 adults patients with CC were identified presented with an anastomotic stenosis with cholangitis, intra-
from 1998 to 2013, of which eight were type I CC. All the hepatic stones, and liver atrophy.49

Figure 2 Type I choledochal cyst in a 58-year-old male. Coronal MRCP image (a) shows fusiform dilatation of the common duct (arrow) and
normal intrahepatic ducts (arrowheads). Coronal reconstruction of CT in the portal venous phase (b) shows similar findings

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Figure 3 Common Channel in a 7-year-old female with type I choledochal cyst. Coronal MRCP (a) shows a long common channel (arrow) in a
patient with anomalous pancreaticobiliary duct union (APBDU) and type I Choledochal cyst. A stone is seen in the distal common duct. Notice
diffuse dilatation of the extrahepatic common duct. Percutaneous transhepatic cholangiography (PTC) (b) confirms the diagnosis

an endoscopic approach with ERCP allows for clear delineation

of pancreatic-biliary anatomy, information critical to avoid the
significant morbidity associated with pancreatic duct injury.55–57
Endoscopic treatment of choledochoceles has been demon-
strated to be durable and be associated with good long term
outcomes.52
A recent systematic review was performed to evaluate patients
with choledochocele. Thirteen cases of choledochocele were in
pediatric patients. Abdominal pain was the most common
presenting symptom and US was the most common diagnostic
modality utilized. Five pediatric patients underwent endoscopic
sphincterotomy and eight patients underwent open trans-
duodenal cyst excision. In addition, 58 cases of adults with
choledochocele were identified and similar to the pediatric
population, abdominal pain was the most common presenting
symptom and US was the most frequently used imaging mo-
dality. In the adult population, 36% of patients underwent
Figure 4 Type Il choledochal cyst in a 52-year-old male. Coronal endoscopic intervention and 52% of patients underwent open
MRCP shows a focal diverticulum arising for the distal common bile surgical management including five patients who underwent a
duct. The communication with the distal common duct is well PD. Post-operative mortality was reported in four patients. Seven
demonstrated (arrowhead). The intra and extra hepatic ducts are adult patients did not undergo treatment. Three adult patients
normal had malignancy in the choledochocele, and no malignancies
were reported in the pediatric population.58

Type III Type IV

Given the anatomic location of type III CC (Fig. 5), complete The treatment of type IV CCs (Figs. 6 and 7) remains a challenge
surgical excision of the choledochocele would require a PD or due to the various presentations of the distribution of intra- and
transduodenal excision, which can be technically challenging and extra-hepatic disease. If the disease involves one side of liver, then
associated with morbidity. As such, given the very low incidence partial hepatic resection with concomitant excision of the
of malignant transformation, the accepted management of extrahepatic bile ducts with Roux-en-Y HJ reconstruction is
choledochoceles generally includes an endoscopic approach that preferred. In general, patients who derive the most benefit from
may include sphincterotomy or sphinteroplasty.7,34,50–52,11,53 partial hepatectomy are individuals with dilation and stenosis of
Among patients with choledochoceles, ERCP offers better visu- intrahepatic ducts, intrahepatic duct stones, or parenchymal at-
alization of ductal anatomy compared with MRCP.54 In addition, rophy as these patients are more prone to cholestasis, abscesses,

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children were identified of whom 22 had cystic extrahepatic

dilation and 15 had fusiform dilation. Interestingly, the patents
with cystic dilation had greater dilation of the extrahepatic ducts
and a trend toward higher choledochal pressure while children
with fusiform dilation had higher bile amylase and were older.
Thus it has been proposed to subdivide type IV CCs further into
cystic and fusiform subgroups based upon this clinical and
pathophysiological evidence.67
A retrospective analysis was performed of 81 consecutive pa-
tients with type IV CCs including 17 children. One child and 29
adult patients had excision of extrahepatic bile ducts in addition
to a liver resection, while 16 children and 35 adults underwent
resection of extrahepatic bile ducts only. Overall, adults had
markedly higher rates of biliary stricture than children. For adult
patients, the group with resection of the extrahepatic bile ducts
only had a higher morbidity including biliary stricture, lithiasis,
and reoperation versus patients who had concomitant liver
resection. In addition, 5 adult patients had cholangiocarcinoma
Figure 5 Type III choledochal cyst (choledochocele) in a 32-year-old
while no children had a malignancy noted on final pathology.68
male. Coronal MRCP image shows cystic ductal dilatation of the distal
common bile duct (arrow). The intra and extra hepatic ducts are normal
Type V - Caroli Disease
The management of patients with Type V CC (Fig. 8) can be
cholangitis, hepatolithiasis, and cancer.59,60 Interestingly, pa- particularly complex. If there is focal biliary dilation within the
tients with type IV CCs are more likely to develop malignancy in liver, a prtial hepatectomy is the treatment of choice. Among
the extra-hepatic bile ducts.59 To this point, data have demon- patients with diffuse intrahepatic disease in which hepatectomy is
strated that excision of the dominant extrahepatic disease may be not an option, liver transplant can be considered. Alternative
key to mitigation of malignancy risk.59 A subset of patients with interventions, including biliary drainage procedures, are often
type IV CC will have a choledochocele component; these in- not effective at preventing recurrent episodes of cholangitis.11,69
dividuals may also benefit from a transduodenal Among patients with bilateral disease, attempts at nonsurgical
sphincteroplastecy.61,62,23,63 management of cholangitis and optimization of liver insuffi-
The King’s College Hospital classification has characterized ciency should be the focus of attention.70–73 While alternative
extrahepatic dilation of the bile ducts into a cystic or fusiform treatment options for patients with type V CC such as external
appearance.64–66 In a review of a single center series from King’s biliary decompression or internal drainage by chol-
College Hospital of pediatric patients with type IV CCs, 37 edochoduodenostomy or HJ, these methods may prove

Figure 6 Type IV choledochal cyst in a 35-year-old female. Coronal CT image in the portal venous phase (a) shows significant dilatation of the
common duct (arrow) and the left intrahepatic duct (arrowhead). Coronal MRCP image (b) shows diffuse dilatation of the common duct (arrow)

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Figure 7 Type lV choledochal cyst in a 40-year-old female. Doppler ultrasound (a) shows diffuse dilatation on of the common duct farrow.
Coronal CT imagine in the portal venous (b) shows dilatation of the common duct (arrow), as well as the left hepatic duct (arrowheads). The
gallbladder is also seen (small arrows). Coronal MRCP image (c) shows diffuse dilatation of the biliary tree. Axial T2 weighted image (d) shows
diffuse dilatation of the common duct with layering debris (arrow)

ineffective as disease remains present distal to the treated intra- left hepatectomy and one patient underwent an extended left
hepatic cysts.74 In one multicenter study, the majority of patients hepatectomy while two patients underwent liver transplant. Two
with Caroli Disease had disease confined to one hemi-liver cases were aborted due to finding metastatic cholangiocarcinoma
(69%) while the remaining 31% of patients had disease at the time of surgery; two patients were awaiting liver trans-
involving both hemi-livers. In this cohort, 75% of patients un- plantation.76 Four patients who underwent surgery had major
derwent hepatic resection while 19% underwent liver trans- post-operative complications requiring further surgery and there
plantation.69 Both resection and liver transplantation achieved were two perioperative mortalities; 14 patients had minor post-
good results with a 97% 5-year survival after liver resection and operative complications. At a median follow-up of 3.7 years, 26
89% 5-year survival after transplantation. patients were free of complications.76 In a separate series of 40
A recent series from a single center of patients with Caroli patients, the time between onset of symptoms and surgical
disease with or without liver fibrosis or cirrhosis identified 21 therapy was 26.5 months.77 Thirty-three patients underwent
patients; 19 patients were treated with liver resection and 2 with liver resection, four transplantation, and three patients under
transplantation. Both liver resection and transplantation were biliary enteric bypass due to transplant contraindications. Pa-
associated with acceptable post-operative morbidity and no tients undergoing operative therapy demonstrated improvement
deaths were reported in a follow-up at 5-years.75 In a separate in disease related symptoms, biliary complications, and anti-
study by Kassahun et al. that reported on 33 patients with Caroli biotic treatment.77
disease, 8 patients had diffuse disease and 25 patients had
localized disease within the liver. Liver resection was performed Minimally invasive approach to surgical management
in 27 patients; 10 patients underwent a right hepatectomy, three In addition to the traditional open approach, minimally invasive
patients had an extended right hepatectomy, 13 patients had a surgery (MIS) using laparoscopy or robotic techniques have been

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Figure 8 Caroli disease in a 40-year old male. Axial (a) and coronal (b) T2 images from MRCP show diffuse intrahepatic cystic ductal dilatation,
with sparing of the extrahepatic common duct. (c) Percutaneous transhepatic cholangiography (PTC) shows similar findings with cystic dilatation
of the intrahepatic ducts (arrows)

successfully utilized to treat CC. MIS may provide superior With greater experience, more centers are adopting minimally
visualization of the hilar structures, yet requires advanced skills invasive techniques to treat patients with CCs.88
in both MIS and hepatobiliary surgery. Experienced MIS sur-
geons can, however, achieve good technical outcomes.78 MIS Outcomes
management of CCs is more common among pediatric pa- Operative intervention for CC is generally well-tolerated, and
tients.79 While sample sizes are generally small and data scant, similar to other hepato-biliary procedures. Early complications
the evidence would suggest that there is no difference in the can include HJ leak, while HJ stricture can be a late complication,
incidence of post-operative complications among patients un- although both are uncommon. Overall, patients who undergo
dergoing open versus minimally invasive CC management.79 surgery for CC do well with a 95.5% five-year survival with no
Several studies have noted good technical outcomes with MIS significant differences in survival or major post-operative
cyst excision and reconstruction in adults with acceptable post- morbidity between children and adults. Not surprisingly, pa-
operative morbidity.80–84 In fact, other data have suggested tients who have a malignancy at the time of CC resection have a
that laparoscopic cyst excision is not only safe and feasible, but worse outcome. In a cohort of 394 patients with CC of which 135
may also be associated with lower risk of complications – were children and 259 were adults, ten adults and two children
especially among pediatric patients.85 In particular, compared had a malignancy noted on pathology following resection. Both
with laparoscopy, the robotic platform may be more ideal to pediatric malignancies were embryonal rhabdomyosarcoma,
perform the HJ reconstruction.86 Xie et al. reported safe use of while the adult tumors were adenocarcinoma. Six patients had
the robotic platform even for patients younger than 1 year old.87 type I CC, 5 with type IV, and 1 with type III. At last follow-up,

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