1. Biliary Tract Anomalies
What is the most common cause of icterus in neonates? Physiologic jaundice of the newborn
When it begins? 2nd or 3rd day
Which component of bilirubin rises? Indirect
How long it continues? Beyond 2 weeks you have to check
Prof. Dr. Y. Hakan Çavuşoğlu

2. Biliary Atresia

3. Biliary atresia (BA) is a relatively rare obstructive condition of the bile ducts causing neonatal jaundice.
The pathologic findings in BA are characterized by inflammatory sclerotic obliteration of all or a part of extrahepatic biliary tree.
Atretic segment changes according to case to case.

4. History BA was first described by Thompson in 1892.
In 1916 Holmes, concluded that successful surgical treatment for congenital atresia of the bile ducts was theoretically possible in at least 16% of cases.
In the late 1950s Kasai defined hepatic portoenterostomy, did not gain immediate acceptance > over time became the standard operative treatment for BA.
Liver transplantation, popularized by Starzl in the early 1960s, is the only readily available salvage treatment for children with biliary atresia and progressive liver dysfunction.

5. Etiology Associated anomalies were reported up to 30%.
Etiology of biliary atresia remains unknown and likely multifactorial.
There is no ideal animal model.
Infection: viral
Embryologic: pancreaticobiliary ductal malunion
Metabolic
Immunologically mediated inflammation
Vascular theories.
Associated anomalies were reported up to 30%.

6. Embryology
The biliary system originates from the hepatic diverticulum of the foregut at 4weeks’ gestation.
(biliary system originates from endoderm)
Hepatic diverticulum differentiates into cranial and caudal buds.
Cranial bud gives rise to liver and extrahepatic bile ducts.
Caudal bud differentiates into superior and inferior buds.
Superior bud gives rise to gallbladder and cystic duct ,
Inferior bud gives rise to right and left ventral pancreas.

7. Embryology II
At the sixth week, the ventral pancreatic bud and common bile duct rotate around the duodenum clockwise by 180 degrees, and common bile duct at this point enters the duodenum at the left posterior surface.

8. Japanese Association of Pediatric Surgeons Classification
Tip I: 10%
atresia of the common bile duct (CBD)
Tip II: 2%
atresia of the CBD and the common hepatic duct
Tip III: 88%
atresia of all extrahepatic bile ducts up to the portahepatis
4 subtip ve 6 subgrup da mevcut.
As Holmes predicted.

9. Clinic presentation
Incidence: In Europe 1 in 18,000 live births. Higher in Asian populations. In Japan 1 in 10,000 live births.
There is a slight female predominance. F/M = 1/0.58
Mean gestational age: weeks
Mean birth weight: g
Non specific prenatal history.
Mature healthy babies

10. The cardinal signs and symptoms of biliary atresia are jaundice, clay-colored stools, and hepatomegaly
Jaundice in infants that persists longer than 2 weeks should not be considered physiologic, especially if the predominant fraction is conjugated bilirubin.
In rare cases jaundice begin after 2-3 weeks.
However, meconium staining is normal in 40% patients.

11. The cardinal signs and symptoms of biliary atresia are jaundice, clay-colored stools, and hepatomegaly
Jaundice in infants that persists longer than 2 weeks should not be considered physiologic, especially if the predominant fraction is conjugated bilirubin.
In rare cases jaundice begin after 2-3 weeks.
However, meconium staining is normal in 40% patients.

12. Clay-colored stool

13. First couple of months they thrive as usual
First couple of months they thrive as usual. After 3-4 months their general condition worsen.
Slight hepatomegaly.
Spleen may be palpable or unpalpable.
Acid is rare before 10th week.

14. Diagnosis
No single test can reliably be used to differentiate biliary atresia from other causes of jaundice in the infant.
A combination of data obtained from a complete clinical assessment, blood tests, imaging studies, and pathologic evaluation is often used to arrive at a provisional diagnosis in almost all cases.
The final diagnosis, however, is always confirmed at surgical exploration.
Laparoscopy-assisted cholangiography has been used to display the anatomic structure of the biliary tree.

15. Differential diagnosis of neonatal cholestasis

16. Ultrasonography US can rule out choledochal cyst.
‘Triangular cord sign’ is a triangular or tubular structure composed of fibrous tissue located cranial to the portal vein bifurcation.
Gall bladder may be visualized in 25% of cases.

17. Liver Biopsy
A percutaneous liver biopsy can help differentiate BA from other cholestatic conditions with a high degree of reliability.
Ductular proliferation is diagnostic for BA. Further findings of bile stasis with plugging, varying degrees of periportal inflammation and giant cell transformation further support the diagnosis of BA.
It may be difficult to differentiate BA from giant cell hepatitis.

18. Scintigraphy
Technetium-99m-DISIDA labeled hepatobiliary scintigraphy nucleotide uptake by hepatocytes is rapid, but excretion into the bowel is absent, even on delayed images. Excretion into the intestine rules out biliary atresia, but non excretion does not confirm the diagnosis.

19. Differential diagnosis
α1-Antitrypsin deficiency,
Cystic fibrosis,
Neonatal hepatitis syndrome
Idiopathic neonatal hepatitis
Infectious hepatitis in neonate

20. Biliary Atresia Idiopathic Neonatal Hepatitis 1/18,000 1/5,000-10,000
Rare familial.
More associated anomalies.
Mature babies.
Persistent clay-colored stools
Colored stool and bile in duodenum rules out BA.
Isotope uptake by hepatocytes is rapid, but excretion into the bowel is absent on scintigraphy.
Idiopathic Neonatal Hepatitis
1/5,000-10,000
20% familial.
Rare associated anomalies.
Premature and SGA babies.
Temporary failure of colored stools.
Isotope uptake is delayed but, excretion into the intestine may or may not be seen.

21. Surgical management
It is believed that age at operation is an important factor in outcome.
(before 8 weeks-90 days)

22. Surgical management Type 1 lesions; hepaticojejunostomi.
In other forms; Kasai portoenterostomy

23. Kasai portoenterostomy

24. Excision of the entire extrahepatic biliary tree with transection of the fibrous portal plate near the hilum of the liver

25. Roux-en-Y jejunostomy

26. Complications Cholangitis (most frequent complication)
elevated serum bilirubin,
leukocytosis,
normal to acholic stools,
in a febrile patient (>38.5°C)
Cessation of bile flow
Portal hypertension
Hepatopulmonary Syndrome
Intrahepatic cysts
Hepatic malignancy

27. Postoperative course
Early portoenterostomy (before 8th week) is the major factor in early and late postoperative course.
operative technique,
severity of liver disease,
gross and microscopic aspects of the biliary tree and portal plate,
presence of comorbid conditions
are a few of the many variables that can affect outcome
70-80% of patients have documented bile flow with efficient clearance of jaundice

28. Postoperative course
70-80% of patients have documented bile flow with efficient clearance of jaundice after portoenterostomy but BA is a progressive disease.
Despite the age is important for primary surgical treatment, hepatic portoenterostomy is the most reasonable first choice.

29. Postoperative course
Portoenterostomy and liver transplantation are complementary procedures.
Liver transplantation is indicated;
jaundice continues to worsen, failure of initial portoenterostomy with no bile drainage,
and progressive liver disease with complications.
Biliary atresia is the most common indication for liver transplantation in children.
60% survival at five years with portoenterostomy alone. The combination of hepatic portoenterostomy and liver transplantation has transformed the overall five-year survival of about 90%.

30. Choledochal Cysts

31. Choledochal cyst (CC) Congenital dilatation of the biliary tract.
The dilatation can be found along any portion of the biliary tract.
The most common site is the choledochus.
Etiology: There are many theories to explain the development of a CC, but none of these can explain the formation of the five different types of CC.

32. Epidemiology and Etiology
The incidence of choledochal cyst ranges from 1 in 100,000 to 150,000 live births in Western populations with the incidence in the Japan reportedly as high as 1 in 10,000.
There is a well-documented female dominance (3 to 4:1).
The etiology of choledochal cyst remains unknown but is commonly accepted to be congenital in nature.
Distal obstruction,
weakness of the duct wall,
or a combination of the two
are the predominant hypotheses.
The etiologic basis for choledochal cyst is likely multifactorial.

33. Abnormal pancreatobiliary junction is characterized by a junction of the bile duct and pancreatic duct outside the duodenal wall with a long common duct channel leading to the duodenal lumen (at least 8mm).
While abnormal pancreatobiliary junction is a rare anomaly, with prevalence of 0.03% in population, it is present in 50-80% of patients with CC.
predispose to reflux of pancreatic juice into the biliary tree, result in increased amylase levels in bile, intraductal activation of proteolytic enzymes

34. Todani’s classification
Type I*
Ia: cystic dilatation of the CBD
Ib: fusiform dilatation of the CBD
Type II: diverticulum of the CBD
Type III: choledochocele (dilatation of the terminal CBD within the duodenal wall)
Type IV
IVa: multiple cysts of the extrahepatic and intrahepatic ducts
IVb: multiple extrahepatic duct cysts
Type V—intrahepatic duct cyst (single or multiple, as in Caroli disease).

35. Diagnosis 20-25% with prenatal ultrasound (with increasing frequency),
~60% first decade,
20% late childhood or adult.
accepted to be congenital in nature

36. Clinical Features Abdominal pain (88%), vomiting (46%), fever (28%),
jaundice (25%),
discolored stool (12%),
abdominal tumor (7%), and
classic triad (pain, jaundice, tumor) (2%).

37. Complications of CC Cholelithiasis, Cholangitis,
Acute or chronic pancreatitis,
Intraperitoneal cyst rupture,
Biliary cirrhosis,
Bleeding due to erosion of the cyst into adjacent vessels,
Cholangiocarcinoma (10-30%); late complication with a mean age at diagnosis of 32 years.

38. Imaging Ultrasonography is the initial imaging method of choice.
ERCP allows excellent definition of the cyst as well as the entire anatomy, but this investigation is invasive.
Magnetic resonance cholangiopancreatography (MRCP) is highly accurate in the detection and classification of the cysts.

40. Differential diagnosis
Should be differentiated from cysts that do not communicate with biliary tree including;
Pancreatic,
Mesenteric,
Hepatic cysts

41. Surgical Techniques
Cyst excision and a bilio-enteric anastomosis is the preferred approach for most patients.
Either open or laparoscopic approach.

42. Partial hepatectomy is indicated for the localized type of Caroli disease (type V) and liver transplantation is usually needed for diffuse disease.
Endoscopic unroofing of a choledochocele (type III) with sphincterotomy of the CBD, or sphincterotomy alone, are considered the preferred treatment for choledochocele.

43. Post-operative complications
Bile leak,
Cholangitis,
Anastomotic stricture,
Alkaline reflux gastritis,
Adhesive intestinal obstruction.
Post-excisional malignant disease is in 0.7-6% of patients.

44. Thanks for your attention
References
Nelson Textbook Of Pediatrics, WB Saunders, 2004
Surgery of the Liver, Bile Ducts and Pancreas in Children, Arnold, 2002
Operative Pediatric Surgery, McGraw Hill, 2003
Semin Pediatr Surg 1992; 1(2):
Pediatric Surgery Vol 2, Mosby, 1998
Ashcraft’s Pediatric Surgery, 6th Edition, Elsevier Saunders, 2014
Topazian M., Biliary Cvsts. In UpToDate, Chopra S., Rand E.B.(Ed) (Accessed on June 7, 2016)