Choledochal cysts are congenital anomalies of the bile ducts, occurring in approximately 1 in 10,000 to 13,000 live births, and are more prevalent in women and Asians. They are classified into five types according to Todani's classification, with Type I being the most common. Complications include pancreatitis, cholangitis, and an increased risk of cholangiocarcinoma, with treatment options varying based on the type of cyst.

1. CHOLEDOCHAL CYST
CHOLEDOCHAL CYST
HAMED RASHAD
HAMED RASHAD
Professor of surgery Banha
Professor of surgery Banha
faculty of medicine - Egypt
faculty of medicine - Egypt

2. Background
Background
•
• Congenital anomalies of the bile ducts.
Congenital anomalies of the bile ducts.
•
• First found western literature 1723
First found western literature 1723 (Vater and
(Vater and
Ezler).
Ezler).
•
• First systematic description of 96 cases in
First systematic description of 96 cases in 1959.
1959.
•
• Classification of 5 types
Classification of 5 types (Todani 1977)
(Todani 1977)

3. Definition
Definition
 Cyst of the choledocal duct
Cyst of the choledocal duct
 Approximately 1 in 10.000-13.000 live births
Approximately 1 in 10.000-13.000 live births
 More common in women (3-4:1)
More common in women (3-4:1)
 More common in Asian (up to 1:1000) ,
More common in Asian (up to 1:1000) ,
especially in Japan
especially in Japan

4. Etiology
Etiology
• Long common channel of the terminal bile duct
Long common channel of the terminal bile duct
and pancreatic duct (more than 1 cm from the
and pancreatic duct (more than 1 cm from the
sphincter)
sphincter)
• Reflux of the pancreatic enzyms
Reflux of the pancreatic enzyms
• Possible genetic influence, common with other
Possible genetic influence, common with other
anomalies (congenital hepatic fibrosis, intrahepatic
anomalies (congenital hepatic fibrosis, intrahepatic
biliary cysts, and polycystic kidney disease)
biliary cysts, and polycystic kidney disease)
• Possible congenital duct wall anomaly
Possible congenital duct wall anomaly

5. Etiology (2)
Etiology (2)
• Weakness of the wall of the bile duct (Babbitt,
Weakness of the wall of the bile duct (Babbitt,
1973; Bismuth, 1999; Flanigan, 1975)
1973; Bismuth, 1999; Flanigan, 1975)
• Obstruction of the distal choledochus (Spitz, 1977)
Obstruction of the distal choledochus (Spitz, 1977)
• Combination of obstruction and weakness (Kato,
Combination of obstruction and weakness (Kato,
1974)
1974)
• Reflux of pancreatic enzymes into the CBD
Reflux of pancreatic enzymes into the CBD
secondary to an anomaly of the pancreaticobiliary
secondary to an anomaly of the pancreaticobiliary
junction (Babbitt, 1969; Kato, 1981; Miyano, 1979;
junction (Babbitt, 1969; Kato, 1981; Miyano, 1979;
Okada, 1990; Todani, 1984, Yamashiro, 1984)
Okada, 1990; Todani, 1984, Yamashiro, 1984)

6. Etiology (3)
Etiology (3)

7. Mortality/morbidity
Mortality/morbidity
•
• Infants and children – pancreatitis,
Infants and children – pancreatitis,
cholangitis and histological evidence
cholangitis and histological evidence
of hepatocellular inflammation
of hepatocellular inflammation
and damage.
and damage.
•
• cholangiocarcinoma 9-28%.
cholangiocarcinoma 9-28%.

8. Clinical Presentation
Clinical Presentation
 Jaundice
Jaundice
 Pain in RUQ
Pain in RUQ
 Mass (rare)
Mass (rare)

9. Symptoms
Symptoms
•
• Jaundice
Jaundice
•
• Pale stools (putty colour)
Pale stools (putty colour)
•
• Palpable mass right upper abdominal quadrant
Palpable mass right upper abdominal quadrant
•
• Hepatomegaly
Hepatomegaly
•
• Acute pancreatitis
Acute pancreatitis

10. Classification
Classification
(Todani 1977)
(Todani 1977)
•
• Type I
Type I 80-90%
80-90%
•
• Type II
Type II
•
• Type III
Type III
•
• Type IV
Type IV
•
• Type V
Type V

11. Todani Classification
Todani Classification
• Type I - Cystic or fusiform dilatation of the CBD; most
Type I - Cystic or fusiform dilatation of the CBD; most
frequent type (90-95% of the cases).
frequent type (90-95% of the cases).
• Type II - Diverticulum of the CBD, with normal size CBD
Type II - Diverticulum of the CBD, with normal size CBD
• Type III - Choledochocele, a cystic dilatation of the distal
Type III - Choledochocele, a cystic dilatation of the distal
intramural portion of the CBD, typically protruding into the
intramural portion of the CBD, typically protruding into the
second portion of the duodenum
second portion of the duodenum
• Type IV - Cystic or fusiform dilatation of the CBD associated
Type IV - Cystic or fusiform dilatation of the CBD associated
with cystic, fusiform, or saccular dilatation of intrahepatic bile
with cystic, fusiform, or saccular dilatation of intrahepatic bile
ducts, also termed form fruste
ducts, also termed form fruste
• Type V - Cystic, fusiform, or saccular dilatation of the
Type V - Cystic, fusiform, or saccular dilatation of the
intrahepatic bile ducts associated with a normal CBD; may be
intrahepatic bile ducts associated with a normal CBD; may be
associated with hepatic fibrosis (referred to as Caroli disease)
associated with hepatic fibrosis (referred to as Caroli disease)

12. Type I choledochal cyst
Type I choledochal cyst
•
• Dilation of entire common hapatic
Dilation of entire common hapatic
and common bile duct or segments
and common bile duct or segments
of each.
of each.
•
• Sacular or fusiform.
Sacular or fusiform.

13. Type II choledochal cyst
Type II choledochal cyst
•
• Relatively isolated protrusion that
Relatively isolated protrusion that
project from common bile duct wall.
project from common bile duct wall.
•
• Connected by narrow stalk.
Connected by narrow stalk.

14. Type III choledochal cyst
Type III choledochal cyst
•
• Found in intraduodenal portion of the
Found in intraduodenal portion of the
common bile duct (choledochocade).
common bile duct (choledochocade).

15. Type IV choledochal cyst
Type IV choledochal cyst
•
• Multiple dilations of the
Multiple dilations of the
Intrahepatic and extrahepatic
Intrahepatic and extrahepatic
biliary tree.
biliary tree.
•
• Large extrahepatic cyst with
Large extrahepatic cyst with
multiple intrahepatic cysts.
multiple intrahepatic cysts.

16. Type v choledochal cysts
Type v choledochal cysts
•
• Dilation of intraphepatic biliary
Dilation of intraphepatic biliary radicles.
radicles.
•
• Numerous cysts with structures
Numerous cysts with structures
causing intraphepatic stone
causing intraphepatic stone
formation, obstruction and cholangitis.
formation, obstruction and cholangitis.

17. Types of choledocal cysts
Types of choledocal cysts

18. Investigations
Investigations
•
• USS abdomen
USS abdomen
•
• CT (93%) or MRI (100%) + cholangiogram
CT (93%) or MRI (100%) + cholangiogram
•
• HIDA scan
HIDA scan
•
• Plain AXR do not identify choledochal cysts
Plain AXR do not identify choledochal cysts
well.
well.

19. Radiology
Radiology

20. US
US

21. CT : choledocal cyst and pancreatitis
CT : choledocal cyst and pancreatitis

23. MR Cholangiography
MR Cholangiography

24. Therapy
Therapy
Type I-IV
Type I-IV
• Cystojejunostomy
Cystojejunostomy
• Total Excision and Cystojejunostomy
Total Excision and Cystojejunostomy
• Biliary reconstruction can be performed with a
Biliary reconstruction can be performed with a
Roux-en-Y hepaticojejunostomy as high as
Roux-en-Y hepaticojejunostomy as high as
possible, near the hilum of the liver (Hata, 1993;
possible, near the hilum of the liver (Hata, 1993;
Joseph, 1990; Kasai, 1970; Miyano, 1996)
Joseph, 1990; Kasai, 1970; Miyano, 1996)
Type V
Type V
• Hepatic Lobectomy
Hepatic Lobectomy

25. Treatment Type I
Treatment Type I
•
• Surgery
Surgery
•
• Complete excision with Rovx-en-y
Complete excision with Rovx-en-y
biliary end to side anastromosis to
biliary end to side anastromosis to restore
restore
biliary continuity to
biliary continuity to gastrointestinal track.
gastrointestinal track.

26. Treatment Type II
Treatment Type II
•
• Total excision
Total excision

27. Treatment Type III
Treatment Type III
•
• 3 cm or less endoscopic removal.
3 cm or less endoscopic removal.
•
• >3 cm surgical excised.
>3 cm surgical excised.

28. Treatment Type IV
Treatment Type IV
•
• Dilated extraphepatic
Dilated extraphepatic
duct removed.
duct removed.

29. Treatment Type V
Treatment Type V
•
• Liver Transplantation
Liver Transplantation

30. Surgery
Surgery
Gallbladder
Choledochal Cyst

31. Cystic Duct
Gallbladder
Choledochal Cyst

32. Hepaticojejunostomy Roux-en-Y
Hepaticojejunostomy Roux-en-Y

33. Complications
Complications
 Cholangitis
Cholangitis
 Biliary stone formation
Biliary stone formation
 Anastomotic stricture
Anastomotic stricture
 Residual debris in the intrahepatic bile ducts
Residual debris in the intrahepatic bile ducts
 Intrahepatic bile duct dilatation
Intrahepatic bile duct dilatation
 Malignancy
Malignancy

34. References
References
 Btaiche I and Khalidi N (2002). Parenteral Nutrition - Associated
Btaiche I and Khalidi N (2002). Parenteral Nutrition - Associated
Liver Complications in Children.
Liver Complications in Children. Pharmacotherapy.
Pharmacotherapy. 22(2): 188- 211
22(2): 188- 211
 Howard E, Stringer M and Colombani P (2002) (2ND Ed).
Howard E, Stringer M and Colombani P (2002) (2ND Ed).
Surgery of the Liver, bile ducts and Pancreas in Children.
Surgery of the Liver, bile ducts and Pancreas in Children. Arnold.
Arnold.
London
London
 Piwko J, Caty M, Ryan R (2003). Neonatal considerations for the
Piwko J, Caty M, Ryan R (2003). Neonatal considerations for the
Paediatric Surgeon.
Paediatric Surgeon. www.emedicine.com/ped/topic2982.htm
 Puri P (2003) Newborn Surgery (2nd Ed).
Puri P (2003) Newborn Surgery (2nd Ed). Arnold.
Arnold. London
London
 Sadiq J, Nondi B, Lakhoo K (2009). An unusual variant of
Sadiq J, Nondi B, Lakhoo K (2009). An unusual variant of
choledochal cyst : a case report.
choledochal cyst : a case report. Journal of Medical Case Reports.
Journal of Medical Case Reports. 3 :
3 :
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54

35. References Cont.
References Cont.
 Sawyer M, Varma M, Murphy T (2009). Choledochal Cyst.
Sawyer M, Varma M, Murphy T (2009). Choledochal Cyst.
http://emedicine.medscape.com/article/366004-overview
 Strodtbeck F(2003) The pathophysiology of prolonged periods of
Strodtbeck F(2003) The pathophysiology of prolonged periods of
No Enteral Nutrition or Nothing by Mouth. Newborn and Infant
No Enteral Nutrition or Nothing by Mouth. Newborn and Infant
Nursing Reviews.
Nursing Reviews. www.medscape.com/viewarticle/458473
 Subramanian K, Yoon H and Toral J (2002). Extremely Low Birth
Subramanian K, Yoon H and Toral J (2002). Extremely Low Birth
Weight Infant.
Weight Infant. www.emedicine.com/ped/topic2784.htm
 Tortora G and Grabowski S (1993) (7TH Ed). Principles of
Tortora G and Grabowski S (1993) (7TH Ed). Principles of
Anatomy and
Anatomy and Physiology
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Harper Collins College Publishers. New
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36. THAK YOU
THAK YOU