Extrahepatic biliary atresia CMC Chitwan Medical college

1. Biliary Atresia
Anup Shrestha
CMC

2. Introduction
• Biliary atresia (BA) is a progressive, idiopathic,
fibro-obliterative disease of the extrahepatic
biliary tree that presents with biliary obstruction
exclusively in the neonatal period.
• Overall incidence is low (approximately 1 in
10,000 to 20,000 live births )
• BA is the most common indication for liver
transplantation in children.

3. Japanese Association of Pediatric
Surgeons classification
• In type I, or “distal” BA,
atresia affects only the CBD,
with the GB and hepatic
ducts remaining patent.
• type IIa, in which the
gallbladder and CBD are both
present and patent
• Type IIb, in which the GB,
CBD, and hepatic ducts are all
obliterated
• type III or “complete,” :
obliteration of both
intrahepatic bile ducts as well
as the entire extrahepatic
biliary

4. Distinct clinical forms
• Fetal-embryonic (or syndromic): is characterized by
early cholestasis, appears in the first 2 weeks of life.
- In this form, the bile ducts are discontinuous at birth,
and affected neonates have associated congenital
defects: situs inversus, polysplenia, malrotation, intestinal
atresia, and cardiac anomalies.
• Perinatal (or acquired): This form is typically found in
neonates and infants aged 2-8 weeks.
- Progressive inflammation and obliteration of the
extrahepatic bile ducts occurs after birth.
- -This form is not associated with congenital anomalies,
and infants may have a short jaundice –free interval.

5. Etiology
• Genetic: Recent work has identified mutations in jagged 1, a
Notch signaling ligand, and the left right patterning gene
Nodal Cofactor Cryptic (CFC1).
• Immunologic: Overexpression of adhesion molecules in
biliary epithelium.
-Aberrant expression of class I and II HLAs
-Expression of Fas ligand and increased apoptosis of bile duct
epithelial cells
• Vascular Abnormalities : impaired arterial flow may lead to
necrosis and fibrous obliteration of extrahepatic bile ducts
• Viral :Reovirus type3, Rotavirus, Cytomegalovirus,
Papillomavirus
• Environmental/miscellaneous- Gestational use of drugs
(amphetamines, alcohol), phytotoxins, mycotoxins, Industrial
toxins, gestational diabetes, maternal age

6. Epidemiology
• It is most commonly observed in East Asian
countries, with a reported incidence as high as
1 in 5,000 to 10,000 live births
• More common in females than in males
• Chinese infants seem to be particularly at risk

7. Clinical Features
• Jaundice lasting longer than the first 2 weeks
of life
• Acholic stools
• Dark urine
• Hepatomegaly
• Vitamin K deficiency
• Coagulopathy
• Cardiac Murmurs

8. Workup
• Labs- LFT
• infants show moderate elevations in total
bilirubin, which is commonly 6-12 mg/dL
• . Elevated Alkaline phosphatase (ALP), gamma-
glutamyl transpeptidase(GGTP), serum
aminotransferases, serum bile acids
• albumin, total protein are in normal range.

9. USG
• A shrunken gallbladder
despite fasting and lack
of intrahepatic bile
ducts.
• The presence of a
hyperechogenic hilum
on ultrasound, or
“triangular cord sign

10. Hepatobiliary Iminodiacetic
Acid (HIDA) Scan
• Lack of bile excretion into the
intestine on HIDA scan is consistent
with BA.
• The specificity is 93% specific, and
94.6% accurate in diagnosing BA.
• Limitation-
-infants with severe hepatitis may
exhibit impaired radiotracer uptake and
thus have limited excretion into the
bowel.
- Additionally, given the progressive
nature of BA, some infants may initially
demonstrate some flow of radiotracer
into the duodenum with the gradual
obliteration of this tract later in the
disease

11. MRCP
• Provide more detailed definition of the
biliary tree.
• Findings- include incomplete visualization
of the extrahepatic biliary system and
periportal high-signal intensity on T2-
weighted MRI scans
• Sensitivity and specificity of greater than
90%

12. ERCP
• ERCP allows direct
visualization of the
extrahepatic biliary tree.
• It requires a general
anesthetic, substantial
expertise, and the
availability of sufficiently
small endoscopes.

13. Preoperative liver biopsy
• Used method to help
exclude other causes
of neonatal jaundice .
• Findings suggestive of
BA include portal or
bridging fibrosis, bile
duct proliferation,
portal inflammation
and bile duct plugs

14. Surgical Management- the Roux-en-Y HPE
(Kasai procedure)
• First performed by
Morio Kasai in 1959
• In this procedure, the
extrahepatic biliary tree
is excised, and the
fibrous portal plate at
the hilum of the liver is
transected and
anastomosed to a Roux-
en-Y limb

15. Procedure
• Incision – rooftop or Chevron

16. Procedure
• the abdomen is grossly inspected to identify any
evidence of associated anomalies, such as
intestinal malrotation or abnormalities of the
spleen and portal vein
• the liver often appears cholestatic or fibrotic with
a fibrotic and shrunken-appearing gallbladder.
• If the gallbladder is normal in appearance, is
patent, the contents of the gallbladder may be
aspirated to assess.
• If clear (“white”) bile is aspirated, no additional
maneuvers are required. If the fluid is darker
appearing, it is recommended to proceed with a
cholangiogram

17. Intra-op Cholangiogram
• Is considered gold standard
for diagnosis of BA
• If contrast appears to freely
flow into both the
intrahepatic ducts as well as
the duodenum, BA may be
safely excluded.
• If a complete biliary tree is
not visualized, biliary-enteric
continuity must be restored

18. • The peritoneum overlying the
hepatoduodenal ligament is opened to
allow identification of the structures in
this area.
• Fibrous remnant of the distal common
bile duct is often present here.
• As dissection continues proximally, the
biliary remnant develops into a cone of
fibrotic tissue that is located at the
bifurcation of the main portal vein into
its left and right branches.
• –most important landmark during the
dissection of the portal plate and should
be the goal of every dissection

19. Roux Limb Construction
• Proximal jejunum is identified and
transected about 10 centimeters distal
to the ligament of Treitz
• The distal end, destined for the right
upper quadrant, is oversewn and the
Roux limb is measured to 40 to 50
centimeters. At this location, an end-
to-side jejunojejunostomy is created
with interrupted absorbable sutures.
• The oversewn end of the Roux limb is
carefully brought into the right upper
quadrant via a small defect created in
the avascular portion of the transverse
mesocolon.

20. Portoenterostomy
• An end-to-end HPE is created rather than an
end-to-side to avoid creating a long unused
blind end as the bowel continues to grow over
time.

21. Post-op care
• Drainage of gastric secretions with a nasogastric tube
should continue for the first 48 hours, then removed
• NPO can be broken at 2nd or 3rd day
• High calorie diet – 3 to 4 gm/pg/ day
• Drain removed at 5th P.O.D
• Ursodeoxycholic Acid for choleretic effect
• Antibiotics
• Vitamin A, D ,E ,K supplements
• Methylprednisolone should be given for it’s anti-
inflammatory and choleretic effects

22. Outcome
• 30-day postoperative mortality following HPE is
low, and is estimated to be 0% to 5%
• Patients in whom biliary drainage is adequate
develop pigmented stools with an associated
downtrend in serum bilirubin levels, typically
within the first 10 to 14 days after surgery.
• Half of patients will continue to experience liver
inflammation, fibrosis, and eventual liver failure
despite an initial good response following HPE.
• Ultimately need liver transplantation by a mean
age of 5.4 years

23. Long-term native-liver survival by age at
hepatoportoenterostomy
groups 1 and 2 are operative age < 6 days; group 3, 61 to 90
days; group 4, 91 to 120 days; group 5, 121 to 150 days;
group 6, >151 days

24. Complications
• Cholangitis- MC Complication
- Mechanism- reflux of intestinal content
- Bacterial Translocation
- Impaired portal lymphatic drainage
Prevention :- Initial construction of an adequate
Roux limb is fundamental
-Prophylactic antibiotics
- Intractable cholangitis : Liver Transplant should be
considered

25. Portal Hypertension
• It is estimated that as many as 30% to 70% of
patients will continue to have elevated portal
pressures following HPE
• Results in esophageal varices and ascites
• Half of the patient will have atleast one
episode of variceal bleeding
• β-blockers and endoscopic interventions
• (sclerotherapy or banding) as primary
prophylaxis in children

26. Hepatopulmonary Syndrome
• characterized by hypoxia associated with chronic
liver disease with evidence of intrapulmonary
shunting
• inability of the liver to metabolize vasoactive
substances may lead to abnormal shunting within
the lungs, ultimately resulting in symptoms of
dyspnea, platypnea, and orthodoexia
• No effective medical therapy
• Liver transplant – definitive treatment

27. Thank You