choledochal cysts - introduction, classification, pathogenesis & management
DESCRIPTION
PowerPoint presentation on Choledochal Cysts, also known as biliary cyst, uploaded by Dr. Vaskar Humagain, first presented in 31st December, 2013. This presentation contains all the information about Choledochal Cysts, the original and revised Todani classification of choledochal cysts, pathogenesis, other associated congenital anomalies, clinical features in infant and adult, management of choledochal cysts. Comments are highly welcome :)TRANSCRIPT
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.
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
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.
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
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
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
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
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
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
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
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
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
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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