Extrahepatic Biliary Atresia

MAMC.
PSU2015 ©vivekgharpure 1
M.Ch. (AIIMS) 1986
Asso Prof MGM Medical
College
Asso Prof GMCH
Independent Practice
Journal of Neonatal Surgery
Marathoner
Trekking
Indian National Trust for Art
and Cultural Heritage
Recreational mathematics
MAMC.PSU2015 ©vivekgharpure 2
Pediatric Surgery
Biliary atresia
Paradigm Shift
Diagnosis of EHBA hasn’t changed much over
30 years.
Management has undergone a paradigm shift.
Looking at the recent papers on EHBA,
Nobody cares about whether the anastomosis
will function or not; how to prevent
cholangitis, ;
Everyone writes about issues related to the
transplanted liver
EHBA personalities
Cesar Roux, 1857-1934. Morio Kasai 1922 - 2008

Swiss surgeon
EHBA personalities
Thomas Starzl
First successful human liver
transplant in 1967
Established clinical utility of
Cyclosporin
Hypoplasia of bile ducts- Alagille Syndrome is
a different altogether. Not included with
biliary atresia
Associated anomalies are
polysplenia/asplenia syndrome, cardiac
lesion, situs inversus, absent vena cava,
preduodenal portal vein, malrotation,
meckel’s diverticulum,
Differentials
• Alagille Syndrome
• Caroli Disease
• Cholestasis
• Cystic Fibrosis
• Cytomegalovirus Infection
• Galactose-1-Phosphate Uridyltransferase
Deficiency (Galactosemia)
• Hemochromatosis, Neonatal
• Herpes Simplex Virus Infection
• Lipid Storage Disorders
• Rubella
• Syphilis
• Toxoplasmosis MAMC.PSU2015 ©vivekgharpure 10
Presentation
• Regardless of etiology, the clinical
presentation of neonatal cholestasis is
remarkably similar in most infants.
• Typical symptoms - variable degrees of
jaundice, dark urine, and light stools.
• most infants are full-term, although a higher
incidence of low birthweight may be
observed.
• acholic stools are not noted at birth
-develop over the first few weeks of life.
Appetite, growth, and ©vivekgharpure
MAMC.PSU2015 weight gain normal.11
Diagnosis
Shah I et al. Trop Gastroenterol. Jul-Sep 2012
Clay colored stools best predictor or EHBA.
Alk phosphatase or GGTP not suggestive.
N=33
Workup
USG - absent gallbladder and no
dilatation of the biliary tree. A ill
informed radiologist is likely to
interpret this as ‘NORMAL’. Gall
bladder is difficult to see in the
newborn, undilated bile ducts – not
seen in conventional bile duct
pathology. Patient may believe the
baby does not have bile duct
pathology
Workup
• HIDA scan – more reliable than USG. Now
available in many places
• ERCP – Technically difficult.
• Duodenal intubation – Unreliable
• Liver Bx- Requires experienced pathologist.

MRCP for Diagnosis of EHBA
158 of 190 patients were interpreted as having
BA by 3D-MRCP; of those, 103 patients were
confirmed as having BA, whereas 55 patients
did not have BA.
MRCP 99% sensitive, but 36% specific
Negative predictive value 96%
Lab Work
Direct Hyperbilirubinemia
Alkaline phosphatase (AP), 5'
nucleotidase, gamma-glutamyl
transpeptidase(GGTP), serum
aminotransferases, serum bile acids
Alpha-1-Antitrypsin elevated 10% patients
with cholestasis.
Histology
Bile ductular
proliferation
Hepatocellular bile
staining -
consequence of
cholestasis.
Cholanagiogram
Definitive answer in obstructive jaundice
Then resect biliary tree and perform
anastomosis
If you are lucky and have bile flow, start
steroids.
DHC and UDCA used earlier, may cause more
fibrosis.
Prophylactic oral antibiotics to prevent
cholangitis.
Or treat cholangitis when occurs
Neonatal Jaundice
HIDA
excretion
steroids
No excretion
Op Chonangiogram
Patent bile ducts
steroids
EHBA
HPE
Bile flow
present
observe
absent
Steroids/UDCA
Bile flow
present
steroids
absent
MAMC.PSU2015 ©vivekgharpure
Consider
20
LDLT
Hypoplasia
Principles of Surgery
If the intrahepatic biliary tree is
unaffected, surgical reconstruction of
the extrahepatic biliary tract is
possible. This surgery is called
Kasai procedure (after Dr. Morio Kasai)
or hepatoportoenterostomy.
Not usually curative, but ideally buys
time until the child can achieve growth
and undergo liver transplantation.
Management
Patients with obstructive jaundice worked up,
prepared and undergo Portoenterostomy.
A variety of conduits used for drainage. Roux-
en-Y jenunal loop common; though Double
Y, gastric tube, Ileo-cecal segment; all have
been used.
Goal – Prevent Cholangitis. (thought to be
ascending, but not really)
From Morio Kasai’s original paper
Surgical Anatomy
Hepatico
Porto
Enterostomy
Drawings from Kasai’s Original Paper
Modifications
of
HPE
Endosurgical Management of EHBA
Lap vs Open
Time for Lap Kasai > Open (p < 0.05)
Complications 40% vs 84.6% (p < 0.05)
Hospital stay and analgesia shorter (p < 0.05)
No difference in outcomes (stool color and
other criteria)
Razumovskiĭ AIu
2014
Outcomes
30% babies fail to achieve satisfactory
bile flow and progress to cirrhosis,
portal hypertension, liver failure.
Even those babies who achieve
adequate bile flow after porto-
enterostomy, more than half of them
yet develop progressive cirrhosis
Cholangitis occurs in upto 50% patients
Prognosis
Age at surgery most significant factor.
Early surgery likely to have better
outcomes
Transplant free survival at 1 and 2 year
reported to be 54% and 47% in one
study (n=244)
Risk of transplant lower in children who
achieved bile flow within 12 weeks of
HPE
START Trial
Steroids in Biliary Atresia Randomized Trial
(START) was conducted in 140 infants (mean
age, 2.3 months) between September 2005 and
February 2011 in the United States; follow-up
ended in January 2013.
high-dose steroid therapy following surgery did
not result in statistically significant treatment
differences in bile drainage at 6 months, although
a small clinical benefit could not be excluded.
Steroid treatment was associated with earlier onset
of serious adverse events in children with biliary
atresia.
Factors predicting better outcomes
• Younger than 10 weeks (in some reports, 2
mo) at operation
• Preoperative histology and ductal remnant
size
• Presence of bile in hepatic lobular zone 1
• Absence of portal hypertension, cirrhosis,
and associated anomalies
• Experience of the surgical team
• Postoperative clearing of jaundice
American Association for Study of Liver Diseases
Criteria for Re-exploration
• Infants who become jaundiced after an
initial anicteric phase postoperatively
• Infants with favorable hepatic and
biliary duct remnant histology at initial
operation, who do not successfully
drain bile
• Infants who may have had an
inadequate initial surgery
Long Term Prognosis
• Overall, a review demonstrated that
66% of infants undergoing the Kasai
procedure ultimately required Liver
Transplant,
• including more than 50% of patients
who initially achieved bile drainage.
Long Term Prognosis
Bijl EJ, et al Neth Med J May 13
98/162 survivars had liver related
complications 20 years after HPE.
40% are complication free
Sundaram SS et al. J Pediatr. Oct 13
Health related quality of life poorer in
survivors of EHBA (n=221)
Long Term Prognosis
Chardot C J Hepatol. Jun 13
N = 1107
HPE 1044. Satisfactory bile drainage 38%
Survival with native liver after HPE
40% at 5 yr, 36% at 10 yr, 30% at 20 yr
20 yr survival 40% if HPE done at 1
month
588 patients 692 LTs
77% survival at 20 yrs Post Transplant
Long Term Morbidity of EHBA in
children with Native Livers
During the 12 preceding months, cholangitis
and bone fractures occurred in 17% and
5.5%, respectively. Health-related quality of
life was reported normal by 53% of patients.
However, only 1.8% met the study definition
of "ideal" outcome.
Nq Vl
J Pediatr Sep 2014
Primary LDLT
Wang Q Hepatogastroenterology. Jun 13
17/50 babies with EHBA received primary
LDLT (live donor liver transplant); 33 HPE
30 day survival – 84% and 82%
1 year survival – 83% and 60%
Jaundice free at 1 month – 100% and 70%
Suggest primary LDLT in selected patients ! !
Indian Situation
Incidence of EHBA 1:20,000
About 60,000 EHBA babies born in the

country every year
Number of LTs in country 1500-2000/year
Cost 12 lacs to 30 lacs
After all the advances in
diagnosis, management, this
still remains the most
important tool in diagnosis
of biliary atresia. Early
surgery still remains the
most significant factor in
long term prognosis.
This chart should be
displayed in every
pediatrician’s clinic.
Only then there is some
hope for the 60,000 new
cases of EHBA in the
country
Thank you

Extrahepatic Biliary Atresia

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MAMC.

Cesar Roux, 1857-1934. Morio Kasai 1922 - 2008

• ERCP – Technically difficult.

• Duodenal intubation – Unreliable

• Liver Bx- Requires experienced pathologist.

Suggest primary LDLT in selected patients ! !

About 60,000 EHBA babies born in the

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