Choledochal cysts - Introduction, Classification, Pathogenesis & Management

Choledochal Cysts
Vaskar Humagain,
Intern

 Choledochal cysts are focal or diffuse dilatations of the biliary tree
 Most commonly present in childhood but increasingly being

recognized in adults.
 represent significant clinical challenges where proper evaluation

and management are paramount to prevent serious clinical
sequelae.

 incidence of choledochal cysts varies significantly throughout the

world.
 In Asia, incidence is as high as 1 in 1000 population with 50% cases

representing from Japan
 In Western Countries, choledochal cysts occur less frequently with

reported cases ranging from 1:13,000 to 1:150,000 population.

 Occur more commonly in females with a M:F ratio of 1:3-4
 Classically present in childhood, but recent series report as

many as 25% of cases presenting in adults.

 Proper management of choledochal cysts requires consideration of their

classification.
 Original Classification by Alonso-Lej and associates exclusively involved the

extrahepatic duct
 The classification was revised by Dr. Todani and colleagues in 1977 to include

intrahepatic cystic anomalies

 Type I (50-85%): They are characterized by cystic or fusiform dilation

of the common bile duct.
 Type IA is defined by cystic dilation of the entire extrahepatic biliary

tree,
 Type IB is defined by focal, segmental (often distal) dilation of the

extrahepatic bile duct.
 Type IC is defined by smooth, fusiform (as opposed to cystic) dilation

of the entire extrahepatic bile duct.

 Type II ( 2%): true diverticula of the extrahepatic bile duct and

communicate with the bile duct through a narrow stalk.
 Type III ( 5%) : Cystic dilatation of the intraduodenal portion of the

extra hepatic common bile duct; also known as a choledochocele
 Type IV (30-40%): Involve multiple cysts of the intrahepatic and

extrahepatic biliary tree; IV A > IV B
 Type V: Caroli’s Disease

 Cause not currently known. Most cysts are congenital in nature.
 It is unclear whether cases of choledochal cysts diagnosed in adults

are acquired or late manifestations of congenital cysts.
 There may be multiple mechanisms involved in the creation of biliary

cysts
 The high incidence of biliary cysts in Asia suggests a role for either

genetic or environmental factors.

 Congenital weakness in the bile duct wall
 Abnormal biliary epithelial proliferation before bile duct cannulation is

complete
 Bile duct obstruction or distension in the prenatal or neonatal periods
 Fetal viral infection

 Pancreaticobiliary maljunction

 Pancreaticobiliary maljunction is defined as an extramural junction

of the pancreatic and biliary ducts in the duodenum beyond the
intramural sphincter function
 characterized by a long common channel (typically over 2 cm)
 Increased reflux of pancreatic juice into the biliary tree -- >

 Biliary atresia , Duodenal atresia, Colonic atresia, Imperforate anus
 Pancreatic arteriovenous malformation, Heterotopic pancreatic tissue

 Multiseptate gallbladder
 OMENS plus syndrome
 Ventricular septal defect, Aortic hypoplasia,

 Congenital absence of the portal vein
 Familial adenomatous polyposis
 Autosomal recessive and autosomal dominant polycystic kidney

disease

 Children: thick and dense fibrotic cyst wall with evidence of acute

and chronic inflammation.
 Adult: common findings are inflammation, erosions, sparseness of

mucin glands, and metaplasia
 Type III cysts are most often lined by duodenal mucosa, although

they sometimes are lined by bile duct epithelium.

 Classic triad : pain, jaundice, and abdominal mass. ( ~ 10%)
 Infants commonly present with elevated conjugated bilirubin

(80%), failure to thrive, or an abdominal mass (30%).
 In patients older than 2 years of age, abdominal pain is the most

common presenting symptom.
 Intermittent jaundice and recurrent cholangitis are also

common, especially in patients with a type III cyst.

 U/S abdomen : to detect the presence
 CT scan – more appropriate in adults.
 MRCP
 Cholangiography: gold standard , PTC or ERC in adults and

intraoperative cholangiography in small children
 Liver function tests

 Bile Duct Tumors
 Biliary Obstruction
 Cholangiocarcinoma
 Pancreatitis, Acute

 Important Clinical Considerations
 Operative management

 Type I: excision of the cyst with its mucosa and reconstruction by

Roux-en-Y hepatico-jejunostomy
 Type II: excision of the diverticulum and suturing of the CBD wall
 Type III: endoscopic sphincterotomy is done.

 Type IV: Extrahepatic biliary resection, cholecystectomy, and biliary

reconstruction
 Type V: Liver transplantation, hepatectomy

 Pancreatitis
 Suppurative cholangitis
 Gallstone and CBD stone formation
 Rupture of cyst
 Cholangio carcinoma in CBD

UTA WAIDNER*, DORIS HENNE-BRUNS AND KLAUS BUTTENSCHOEN
JOURNAL OF MEDICAL CASE REPORTS 2008, 2:5 DOI:10.1186/1752-1947-2-5

A 19-year-old Russian woman (height, 1.69 m; weight, 54 kg) with
non-specific upper abdominal pain presented to a local hospital for
evaluation. She complained of recurrent pain for weeks. Clinical
examination revealed neither jaundice nor a palpable abdominal
mass. The clinical laboratory data were normal.


 Ultrasonography revealed a hypoechogenic, nearly

spheric, homogenous formation with a smooth contour in direct
contact with the underside of the liver and without any
intermediate layer. The finding was most compatible with a large
hepatic cyst.


Computer tomography showed a clearly
limited, hypodense, homogenous structure with a transverse
diameter of 11 cm in the immediate vicinity of the liver, anterior
to the right kidney, and posterior to the gall bladder


 Cystic echinococcosis was excluded serologically.
 The larger structure was interpreted as a congenital hepatic cyst

due to the direct contact to segment 5 of the liver.
 The smaller structure was judged as an independent hepatic cyst

because it resembled the large cyst


 laparoscopic fenestration of the large cyst was done
 the cyst was approached via the inferior border.
 A puncture was performed, which resulted in the evacuation of

more than 100 ml of bile.
 Then, the cyst was opened by a 4 × 3 cm incision.
 Laparoscopic evaluation of the inner cyst revealed two bile ducts


 Bilirubin increased to 6.21 mg/dl and the patient developed

jaundice on second POD
 Due to these ambiguous findings, the patient was transferred to
our university hospital on the third postoperative day. Computer
tomography showed incipient pancreatitis. After re-evaluation of
the original computer tomography, a large choledochal cyst
involving the distal part of the common bile duct was recognized.
The patient underwent repeat surgery on the fourth day after the
original surgery, and a large choledochal cyst, Todani type 1A, with
a diameter of 8–10 cm was found


This case report highlights the difficulties involved in making a
correct diagnosis and the operative treatment for a choledochal
cyst.

MUNEYUKI YAMAGUCHI, MD, JAPAN
THE AMERICAN JOURNAL OF SURGERY
VOLUME 140, ISSUE 5, NOVEMBER 1980, PAGES 653–657

 Five of the author's cases and 1,428 cases from Japan's literature

are discussed. Half of the patients were infants. The ratio of men
to women was 1 to 3. One hundred fifty-one patients had
malformation of the pancreaticobiliary system, which is said to be
a cause of congenital choledochal cyst. All of the patients have
been followed up. Excision of the cyst is the best procedure for
preventing ascending cholangitis and cystic cancer. Roux-Y
hepaticojejunostomy is also effective for reconstruction of the bile
duct because it rarely causes ascending cholangitis.

 Bailey and Love’s Short Practice of Surgery, 26th

Edition, 2012, Taylor Francis Group
 Maingot’s Abdominal Operations, 11th Edition, 2007, M.J.

Zinner, et.al., Mc Graw Hills Access Surgery
 SRB’s Manual of Surgery, 3rd Edition, 2008, Jaypee Publications
 UpToDate, 21.2, LWW
 Icons Courtesy of Google Images

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