Anomalies of biliary tree
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This document discusses various anomalies of the biliary tree that can occur during embryonic development, including choledochal cysts, anomalous pancreatobiliary junction, annular pancreas, and biliary atresia. It describes the embryology of the biliary system and pancreas. Common anomalies are discussed such as their presentation, diagnosis using imaging modalities, classification systems, and management approaches including various surgical procedures.
Anomalies of biliary tree
- 1. Anomalies of Biliary Tree Dr. Shambhavi Sharma Resident 1st year – General Surgery PAHS
- 2. Embryology
- 3. Embryology • Beginning in the fourth week of gestation liver bud arises from the distal extent of the foregut • As the liver parenchyma develops the cells between it and the foregut proliferate, forming the precursor to the bile duct. • Between the 4th and 5th weeks of gestation the gallbladder primordium buds off the caudal extent of the bile duct giving rise to the gallbladder and cystic duct.
- 4. • This bud lies in close proximity to the ventral pancreatic bud • The shared stalk rotates posteriorly and medially to join the dorsal pancreatic bud
- 5. • The ventral pancreatic bud gives rise to the head,uncinate process • The duct of Wirsung, typically joins with the common bile duct (CBD) at ampulla of vater and drain in 2nd part of duodenum • duct draining the dorsal pancreatic bud will fuse with the duct draining the ventral pancreatic bud(duct of santorini )
- 6. • This duct may fail to fuse (known as pancreas divisum) and/or drain directly into the duodenum at the minor papilla
- 7. Type I (Classic):The coalition between dorsal and ventral pancreatic ducts is absent. 70% cases. Type II (Absent ventral duct): The minor papillae depletes the entire pancreas although the major papilla and exhausts the bile duct. 20-25%. Type III (Functional): The conduit between dorsal and ventral pancreatic ducts is filamentous or insufficient. 5-6% of the discovered malformations.
- 8. Annular pancreas failure of the ventral bud to rotate with the duodenum, causing envelopment of duodenum – Lecco's theory – Baldwin's theory – Hypertrophy and fusion of the ventral and dorsal buds before rotation of the gut, resulting in complete encirclement of the duodenum
- 9. Anomalous pancreatobiliary junction (ABPJ) anomalous union of the pancreatic and bile ducts outside the duodenal wall resulting in a long common channel (usually > 15-mm) • APBJ is seen in up to 90-100% of cases of congenital choledochal cysts • .
- 10. • The presence of a long common channel allows reflux of pancreatic secretions into the biliary system, possibly resulting in choledochal cyst • Conversely, reflux of bile into the pancreatic duct can cause relapsing or chronic pancreatitis
- 11. • Dorsal Agenesis – complete and partial • Ansa pancreatica • Santorinicele & Wirsungocele • Bifid tail of the pancreas • Ectopic pancreas • Intra-pancreatic accessory spleen • Congenital pancreatic cysts
- 12. ANSA PANCREATICA
- 13. Anomalies in gall bladder
- 14. Anomalies of Biliary tree • Fibropolycystic liver disease – Choledochal cyst – Congenital hepatic fibrosis – Biliary hamartoma – Autosomal dominant polycystic disease – Caroli disease • Biliary atresia
- 15. Choledochal cyst • Congenital anomaly – supported by fact that increasing number are detected antenatal ultrasound • Cystic dilatation of ducts of Biliary tree • Highest incidence in Japan • Often associated with pancreatobiliary maljunction – reflux of pancreatic juice into bile duct – damages the bile duct which dilates (Babbit theory)
- 16. Presentation • Presents before the age of 10 years (60 % of cases) • Asymptomatic – in neonates due to increase use of antenatal ultrasound • Clinical feature – triad (all three triad present in less than 50% of cases) – Obstructive jaundice - 75 % – Abdominal pain in right upper quadrant - 50 % – Palpable abdominal mass - 30 %
- 17. Types of choledochal cyst • Alonso-Lej et al. – modified by Todani 60% 30%
- 18. Diagnosis - Imaging • Modalities – Abdominal ultrasonography – first line investigation – CT scan with 3d reconstruction of biliary tree anatomy – Magnetic resonance cholangiopancreatography (MRCP) – HIDA scanning – Cholangiography – excellent anatomical delineation – • Ontable cholangiography • ERCP route • Percutaneous route
- 19. Type 1
- 21. Type 4
- 22. Complication • Pancreatitis • Cholangitis • Gallstone and CBD stone • Secondary biliary cirrhosis • Rupture of cyst and peritonitis • Cholangiocarcinoma
- 23. Management • Only surgical management is definitive • Goal of operation – Allow free hepato-enteric bile flow – Remove all cyst mucosa (to decrease all the malignant potential) – Exclude common channel and prevent pancreatobiliary reflux – Minimize risk of cholangitis
- 24. Surgery • Access – Position – supine with back slightly extended – Incision – High transverse or subcostal incision
- 25. Procedure – depends on morphology of the cyst • Type 1 – excision of cyst with its mucosa and reconstruction of Roux-en-Y hepaticojejunostomy – Cyst excision and hepaticojejunostomy • Type 2 – excision of diverticulum and suturing of CBD wall is done • Type 3 – Endoscopic sphincterotomy with cyst excision
- 26. Procedure – depends on morphology of the cyst • Type 4 – If cyst is adherent to portal vein posteriorly, the part of cyst wall over the portal vein is left behind – Mucosa of that part is removed – Lily’s operation • Cholecystectomy is done in all types
- 27. Alternatives • External drainage • Cyst excision and hepaticoduodenostomy • Mucosectomy and hepaticojejunostomy • Choledochocyst-enterostomy
- 28. Biliary atresia • Congenital • Fibrosis of extra and intra hepatic biliary tree • Male predilection • Incidence – 1:10000 • Obstructive cholangiopathy with inflammation, destruction and obliteration of extrahepatic biliary tree (predominantly) • Can be – Perinatal acquired – 70 % - not associated with other congenital anomalies – Fetal/embryonic – 30% - associated with other congenital anomalies
- 29. Presentation • Asymptomatic at birth • Symptomatic – Within 3 months of life • Obstructive jaundice • Steatorrhoea • Pale stool • Hepatomegaly • Eventually causes cirrhosis of liver
- 30. Classification
- 31. Diagnosis - Imaging • Modalities –Abdominal ultrasonography –MRCP –HIDA scan –On table cholangiography
- 32. Ultrasonography Triangular cord sign – fibrous remnant of CBD
- 34. HIDA scanning
- 35. Management • Only surgical management available • Based on correction chances –Correctable – 10 % • Roux-en-Y jejunal anastomosis –Non-correctable – 90 % • Hepaticoportojejunostomy – Kasai operation • Definitive – Liver transplantation
- 36. Procedure • Ligate the common bile – transect the common bile duct • Dissect up to porta hepatis – transect bile duct at the level of porta hepatis and portal plate is created • Roun – en – Y anastomosis - jejunum is connected to porta hepatis
- 37. Post-operative management • Antibiotics • Corticosteroids – iv followed by oral • Ursodeoxycholic acid (1 year)
Editor's Notes
- #25: Procedure The choledochal cyst and gall bladder are mobilized, keeping to a plane of dissection which lies between the peritoneum and cyst wall. This plane is entered anteriorly, and is then extended around the sides of the cyst, taking great care to avoid damaging the hepatic artery, which may be very adherent to the cyst wall. The cyst is lifted forwards from the portal vein and is encircled proximally and (where possible) distally. The common hepatic duct is then divided at the level of the bifurcation and the cyst and gallbladder are reflected forwards (Fig. 12.6). This allows the dissection to proceed distally, as far towards the pancreaticobiliary junction as possible, whilst avoiding damage to the pancreas and related structures. At this point the bile duct is divided and the cyst and gallbladder are removed en bloc. The distal end of the common bile duct is either ligated or oversewn, depending on size. The common hepatic duct is carefully examined to exclude any proximal stenoses. If these are present, they can usually be resected from the divided end of the duct.25 The common hepatic duct is then anastomosed to a 40 cm retrocolic roux loop of jejunum with interrupted absorbable sutures. It is important to achieve a wide anastomosis, which is proximal to any ductal strictures.25,52 The abdomen is then closed, leaving a small suction drain in place.
- #28: External drainage In very complicated cases, particularly where there is obstruction, uncontrolled ascending cholangitis or cyst rupture, it may be preferable to establish some form of temporary external drainage, prior to carrying out definitive surgery.4 This can be achieved by percutaneous transhepatic cholangiodrainage where appropriate3 or by open T-tube cholecystotomy. Formal cyst excision and hepaticojejunostomy should be carried out once the general condition of the patient has improved. Cyst excision and hepaticoduodenostomy This procedure is somewhat simpler than the conventional operation, as it avoids the need to create a roux loop. It also has the merit of producing a more physiologically ‘normal’ anatomical result.51 However, it is believed that these patients are more vulnerable to duodenohepatic reflux, stasis and cholangitis, particularly where the intrahepatic ducts are dilated,3 and for this reason most authors prefer the roux loop hepaticojejunostomy. Mucosectomy and hepaticojejunostomy In longstanding choledochal cysts, the cyst wall may be so thick and adherent that it is very difficult to separate it from adjacent structures such as the hepatic artery and portal vein. In these cases it may be preferable to open the cyst and excise the cyst lining from within, without attempting to excise the cyst itself. Complete removal of the cyst mucosa in this way diminishes the risk of malignancy and allows a hepaticojejunostomy to be carried out in the usual manner. Choledochocyst-enterostomy This operation is technically straightforward and may have a role in the acute situation where radical excision is hazardous.4 However, it is very unsatisfactory in the long run, because of the high incidence of stasis, calculus formation, cholangitis and malignancy. Indeed, most surgeons would suggest that such cases should go on to have a formal cyst excision and hepaticojejunostomy in due course.53