Choledochal cysts - Introduction, Classification, Pathogenesis & Management

Choledochal Cysts Vaskar Humagain,

Intern

INTRODUCTION

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.

EPIDEMIOLOGY 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.

EPIDEMIOLOGY

CLASSIFICATION 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

TODANI CLASSIFICATION 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 1 A

Type II

TODANI CLASSIFICATION

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

Type III

Type IV A

Type IV B

Type V

PATHOGENESIS

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

PATHOGENESIS

APBJ – BABBIT THEORY

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 -- >

ASSOCIATED DEVELOPMENTAL ANOMALIES

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

PATHOLOGY 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.

PRESENTATION 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.

DIAGNOSIS 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

DIFFERENTIAL DIAGNOSES Bile Duct Tumors Biliary Obstruction Cholangiocarcinoma Pancreatitis, Acute

MANAGEMENT

Important Clinical Considerations

Operative management

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

COMPLICATIONS

Pancreatitis

Suppurative cholangitis

Gallstone and CBD stone formation

Rupture of cyst

Cholangio carcinoma in CBD

CHOLEDOCHAL CYST AS A DIAGNOSTIC PITFALL: A CASE REPORTUTA WAIDNER*, DORIS HENNE-BRUNS AND KLAUS BUTTENSCHOENJOURNAL 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.

CONGENITAL CHOLEDOCHAL CYST: ANALYSIS OF 1,433 PATIENTS IN THE JAPANESE LITERATUREMUNEYUKI YAMAGUCHI, MD, JAPAN THE AMERICAN JOURNAL OF SURGERYVOLUME 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.

REFERENCES 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

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UpToDate, 21.2, LWW

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