Choledochal cyst, Gall bladder, Choledochal cyst treatment

1. By
DR RITESH DHANBHAR

2. Definetion
Defined as isolated/focal or
combined/diffuse congenital dilatation of extra or
intrahepatic biliary tree.

3. Etiology
1.Babbitt theory :
There is long common channel more than 2 cm. It causes
reflux of pancreatic juice into the bile duct, enzymatic destruction of
the bile duct wall, ductal wall weakening and dilatation.
2. During embryogenesis, there is abnormal early canalisation of
bile duct with distal obstruction causing increased proximal pressure,
weakening and ductal dilatation.
3. Reduced postganglionic autonomous neurons in the distal portion
of the cyst causing its poor function distally.

4. Histology
1. Histological features are Chronic inflammation, sparse
mucin glands and metaplasia.
2. Histological types
A. Glandular with normal cuboidal epithelium with
cavities in the mucosa.
B. Fibrotic type with fibrous cyst wall with thickened bile
duct.

5. Types (Todani Modification of Alonso-Lej
Classification)
Type I: Dilatation of
extrahepatic biliary tree
(60%).
• Type Ia—cystic.
• Type Ib—focal segmental
(saccular).
• Type Ic—fusiform.

6. Type II: Diverticulum of
extrahepatic biliary tree.
Type III: Choledochocele—
cystic dilatation of
intraduodenal part of CBD .

7. Type IV: Dilatation of extra- and intrahepatic or multiple
parts of extrahepatic biliary tree (30%).
• Type IVa: Dilatation of extrahepatic and intrahepatic biliary
tree. It is 2nd most common.
• Type IVb: Dilatation of multiple sections of the
extrahepatic bile duct.

8. • Type V: Dilatation of the only intrahepatic biliary
tree(Caroli’s disease).

10. Clinical feature
• Common in female
• Common in Japan
• Obstructive jaundice (80%), pain in right
hypochondrium,cholangitis.
• Mass per abdomen—mass is to the right and above
umbilicus, smooth, soft, not moving with respiration, not
mobile and resonant.
• Chield Failure to thrive.

11. Triad of choledochal cyst
1. Right upper quadrant pain
2. Jaundice
3. Palpable abdominal mass

12. Diagnosis
• US abdomen—unilocular cyst mainly in infants.
• CT scan—mainly to see intrahepatic biliary system.
• ERCP, Cholangiography—to see ductal anatomy.
• MRCP—to see status of pancreatic and biliary system
and pancreaticobiliary maljunction–ideal.
• Liver function tests.
• PTC to see intrahepatic biliary tree.

14. Treatment
• In type I—excision of cyst with its mucosa , cholecystectomy
and reconstruction by Roux-en-Y hepaticojejunostomy.
• In type II—excision of the diverticulum, cholecystectomy and
either suturing of the CBD wall or Roux-en-Y
hepaticojejunostomy can be done.
• In type III—endoscopic sphincterotomy is done. Excision is
also often needed. Intrahepatic dilatation is difficult to treat. If it
is localised, hepatectomy is sufficient, but if it is diffuse, liver
transplantation may be required.

16. • In type IV if cyst is adherent to portal vein posteriorly, that
part of the cyst wall over the portal vein is left behind. But
mucosa of the part should be removed (Lily’s operation).
• Type IVa-- Cholecystectomy and Roux-en-Y
hepaticojejunostomy , liver transplantation.
• Type IVb-- Resection of extrahepatic biliary tree with removal
of choledochal cyst along with cholecystectomy and Roux-
en-Y hepaticojejunostomy.
• Type V: Cholecystectomy , liver transplantation

18. • Cholecystectomy is a must in all types.
• If cyst has already turned into malignancy—
adenocarcinoma, then radical surgery and
chemotherapy is given.
• Liver transplantation is the choice in type IV and V
cases.