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1. Biliary System
Dr. Arjun A. Pawar
MBBS, MS,
M. Ch. Pediatric Surgery,
DNB Pediatric Surgery,
FMAS,
FIAGES.
Consultant Pediatric and Neonatal Surgeon,
Pediatric Laparoscopic Surgeon
Pediatric Urologist
Assistant Professor
Department of General Surgery
MGM Medical College & Hospital
Chh. Sambhaji Nagar

2. Biliary System
Parts:
•Intra hepatic ducts
•Extra hepatic ducts
•Gallbladder
•Common Bile Duct

6. Common Surgical Diseases
•Gallstones
•CBD Stones
•Biliary tract tumours
•Biliary strictures
•Choledochal Cyst
•Biliary Atresia

7. Choledochal cyst
• Incidence -1 in 100,000 to 150,000 live births
• Etiology -remains unknown
Pancreati-cobiliary maljunction (PBM)- Asso
with CDC and Carcinoma of biliary duct & gall
bladder
• Pathology - Sludge, cholelithiasis, or
choledocholithiasis
relatively acellular and may lack a typical mucosal
lining.

8. Todani’s classification

9. Type 3- Subtypes
Type 1 - Subtypes

10. Clinical Presentation
• Abdominal pain, jaundice, and a palpable right upper
quadrant abdominal mass -classic triad- 20%
• Infantile form- occurs before 12 months of age, present
with obstructive jaundice, acholic stools and
hepatomegaly
• Adult form- occurs anytime after 12 months of age and
usually has a greater number of symptoms including
fever, nausea, vomiting, and jaundice.
• Complications- Rupture- Pain, sepsis, Perforation
peritonitis.
• Biliary obstruction leading to ascending cholangitis and
complications of pancreatitis.
• Cholelithiasis , Malignancy.

11. Diagnosis
• LABORATORY STUDIES-
Serum markers of obstructive jaundice
(e.g., conjugated hyperbilirubinemia and
increased serum alkaline phosphatase) ,
chronic cases- abnormal coagulation profile
• IMAGING STUDIES-
• Abdominal ultrasound-cystic mass in the porta hepatis,
in continuity with the biliary tree
• 71% to 97% sensitivity.

12. USG

13. Technectium-99 HIDA scan-
Photopenia is initially evident at the cyst followed by
filling and delayed emptying.
 sensitivities- with 100% for type I cysts and
67% for type IV
 distinguishing between choledochal cyst and
biliary atresia
 diagnosis of cystic rupture- contrast would
empty into the peritoneal cavity.

15. Abdominal computed tomography (CT)-
intrahepatic ducts, distal common bile ducts, and the
pancreatic duct,
• highly useful in identifying type IV and type V cysts.
• CT cholangiography- full anatomy of the biliary tree
can be delineated
• risk of using CT or CT cholangiography-contrast
toxicity that can cause nephrotoxicity or
hepatotoxicity and radiation exposure

16. Invasive cholangiography
• Endoscopic retrograde cholangiopancreatography
(ERCP),
• Percutaneous transhepatic cholangiography (PTC),
• Intraoperative cholangiogram(IOC)
• Risk of cholangitis and pancreatitis

17. MRCP
Magnetic resonance cholangiopancreatography (MRCP) -
-gold standard
diagnostic sensitivity of MRCP is 90% to 100

18. Surgical Management
Open approach/ Laparoscopic / Robotic
• Type 1- Excision
• Type 2- Simple cyst resection
• Type 3- ERCP with Sphincterotomy or Cyst
marsupialization
• Type 4- Cyst resection of hepatic ducts only & long
term follow up for lithiasis , cholangitis
• Type 5- Segmental resections or Liver transplant

19. Hepaticoduodenostomy

20. Roux-en-Y hepatico-jejunostomy
15 cm –from DJ
40 cm- Roux
limb
Retrocolic
manner
End to side
anastomosis

21. Complications
• Anastomotic stricture,
• Recurrent ascending cholangitis,
• Bowel obstruction,
• Portal hypertension, and
• Malignancy.
• Stone formation

22. Biliary atresia
• Obliterative disorder of intra and extra-hepatic biliary
tree
• Progressive Panductular Obliterative Cholangiopathy
(PPOC)
• Liver cirrhosis and death
• 1: 10000 live births
• Female-to-male ratio -1.4 to 1.7 to 1
• Biliary atresia was first described by John Thompson in
1892
• Ladd was the first to report successful surgical
correction of biliary atresia
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23. • In the late 1950s Kasai and his colleagues in Japan
noted that bile flow from the porta hepatis was
possible after excision of the entire fibrotic
extrahepatic biliary tree.
• Kasai achieved the first long-term “cure” of a
patient deemed to have the noncorrectable form of
biliary atresia
• Kasai’s hepatic portoenterostomy- standard
operative treatment for biliary atresia-
• Liver transplantation, popularized by Starzl in the
early 1960 – Definitive therapy

24. Biliary Atresia- Classification- JAPS

25. Etiology
• Exact etiology of biliary atresia is unknown and
likely multifactorial.
• 20%- associated with other malformations
• TORCH (toxoplasmosis, others, rubella,
cytomegalovirus, herpes simplex virus)
• Other proposed factors - bile duct ischemia,
abnormal bile acid metabolism, pancreaticobiliary
maljunction, and the effect of certain
environmental toxins.

26. Pathology-
Bile ductular proliferation -considered diagnostic for
biliary atresia.
Associated findings include bile stasis, periportal
inflammation, identification of giant cells, and
varying degrees of fibrosis

28. Clinical Features
• Progressive jaundice,
• Acholic stools,
• Dark urine, and
• Firm hepatomegaly in an infant
• Jaundice involves direct hyperbilirubinemia
• Eventually, signs of advanced liver disease such as
palpable hepatomegaly and splenomegaly, ascites,
failure to thrive, and malnutrition become present.
• If not treated, biliary atresia is fatal within the first
2 years of life

29. DIAGNOSIS
• BLOOD TESTS- liver function tests” –
Both the direct and indirect bilirubin levels are
elevated.
Transaminases are also mildly to moderately increased.
Alkaline phosphatase levels are often elevated
Gammaglutamyl transpeptidase (GGTP) level elevated
and very specific
Clotting cascade and serum albumin are abnormal in
advanced cases.
• TORCH family (Toxoplasmosis, Other viruses, Rubella,
Cytomegalovirus, and Herpes Simplex Virus) screening

30. Abdominal ultrasound
• Obtained in the fasting state to allow for filling of the
gallbladder
• Presence of triangular cord sign
• Increased resistivity index- preoperative HARI ≥0.8
should be considered as a risk factor for poor outcomes
in BA.
• Use of doppler techniques can improve the accuracy
• Echotexture of the liver, the presence of ascites, the
patency of the hepatic vasculature, and the anatomy of
the biliary structures can be assessed.
• Infants with biliary atresia, however, the ultrasound
reveals that the gallbladder is either shrunken or
normal appearing and the bile ducts are not easily
delineated.

32. HEPATOBILIARY SCINTIGRAPHY
• Use of isotopes of technetium 99m to assess excretion
of bile from the liver into the small intestine and
therefore biliary patency.
• Hida” (hydroxy iminodiacetic acid) scan
• Technetium-labeled compound diisopropyl
iminodiacetic acid (DISIDA) is more effective
• All jaundiced infants undergoing hepatobiliary
scintigraphy should be pretreated with phenobarbital
(5 mg/kg/day) for 5 days before the study
• Delayed assessment of isotope excretion at 24 hours is
warranted. When no isotope is seen in the intestine
after 24 hours, biliary obstruction is presumed and the
diagnosis of biliary atresia must be further pursued.

33. Liver Biopsy
• Most accurate nonsurgical diagnostic test.
• Presence of varying degrees of inflammation with
bile ductular proliferation is considered compatible
with the diagnosis of biliary atresia
• Bile stasis with plugging and giant cell
transformation further support the diagnosis
• Bile duct paucity syndromes can be readily
differentiated from biliary atresia.

34. Other tests
• Intubation of the duodenum viathe nasoduodenal
route with aspiration or prolonged collection of
duodenal fluid can exclude biliary atresiaif bile-
stainedfluid is obtained
• Magnetic resonance cholangiopancreatography
(MRCP) have superior accuracy in the diagnosis of
biliary atresia

35. Management
• Pre operative preparation
- broad-spectrum antibiotics,
-oral supplementation with fat-soluble vitamins (A, D, E, and
K)
-or an intramuscular injection of vitamin K (1 mg)
• Intra-operative cholangiogram
• Surgical management
Kasai porto-enterostomy
Variations as per anatomic characteristics
Variations to reduce cholangitis

36. Management
Operation involves excision of the
entire extrahepatic biliary tree with
transection of the fibrous portal plate
near the hilum of the liver.
Bilioenteric continuity is then
reestablished with a Roux-en-Y limb
Absence of the spleen or polysplenia-
alert for
associated anomalies such as
malrotation, preduodenal portal vein,
and interrupted inferior vena cava with
azygous continuation.

37. Roof top incision with delivery of the liver
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38. Excision of the fibrous ducts at portal plate

39. Kasai’s porto-enterostomy
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40. POSTOPERATIVE CARE
• nasogastric tube should continue for the first 48
hours
• third postoperative day, infants often have already
passed gas and stool and an oral diet can be started
• Intravenous antibiotics- subsequently converted to
long-term oral antibiotics
• closed suction drain is removed before discharge,
typically on the fifth postoperative day
• Antibiotics, a choleretic agent, fat-soluble vitamin
supplements, and an oral steroid taper have been
the routine protocol

41. Post op regimen

42. outcome
• Responders- 33%
• Transient responders-33%
• Non responders-33%
• Jaundice free 40 %
• Problems 20 %
• Transplant 40 %
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43. Complications
• NUTRITIONAL COMPLICATIONS- deficiency occurs
Supplements started- Nutritional parameters,
vitamin levels, and growth should be monitored
• CHOLANGITIS- 30 to 60%- treated symptomatically
• PORTAL HYPERTENSION- 34 to 76%- Ascites,
Esophageal varices, massive bleeding –life
threatening
• PORTAL HYPERTENSION–ASSOCIATED
HYPERSPLENISM - occurs in 16% to 35%- associated
thrombocytopenia can complicate episodes of
gastrointestinal bleeding

44. LIVER TRANSPLANTATION
• Liver failure due to biliary atresia represents the most
common indication for liver transplantation in the pediatric
age group.
General indications for liver transplantation in biliary atresia
include
• (1) failure of initial portoenterostomy with no bile drainage
and progressive liver disease;
• (2) episodic or inefficient bile drainage with slow
deterioration of liver function and development of growth
failure; and
• (3) development of one or more complications of chronic
liver disease
• Liver transplantation remains the most important rescue
therapy for children with biliary atresia after
portoenterostomy with 5-year survival rates expected to
exceed 90%.

45. Thank You