Kasai procedure

Copyright © 2004 Pearson Education, Inc., publishing as Benjamin Cummings
KASAI PROCEDUREKASAI PROCEDURE
EMAD KANDIL, MDEMAD KANDIL, MD

OR
Preop Phenobarb

OR

Post-op
• Hemodynamically stable
• Started on steroids
• Reported 1 greenish stools

GB
• no inflammation seen

PORTA HEPATIS
• fibrotic contain nerves
and small blood vessels,
no bile duct components

Liver Bx
• Bridging fibrosis
• Chronic and focal acute
inflammation
• Marked cholestasis and
giant cell transformation
of hepatocytes c/W
extrahepatic ducts
dilation

Copyright © 2004 Pearson Education, Inc., publishing as Benjamin CummingsCopyright © 2004 Pearson Education, Inc., publishing as Benjamin Cummings
KASAI PROCEDUREKASAI PROCEDURE
EMAD KANDIL, MDEMAD KANDIL, MD

Biliary Atresia
• Obliteration or discontinuity of the
extrahepatic biliary system
• MC cause of death as a result of liver
disease in early childhood in the
Western world
• MC surgically treatable cause of
cholestasis encountered during the
newborn period
• If not corrected surgically, 2ry biliary
cirrhosis results
• First reported by Professor John
Burns from University of Glasgow in
1817 Mowat AP, etal(Earlier identification of biliary atresia and
hepatobiliary disease) Arch Dis Child 72:90-92, 1995

Biliary Atresia
TYPES
• subdivided into 2 distinct forms:
• (1) isolated biliary atresia (postnatal form):( 65-90%)
• (2) Associated situs inversus or polysplenia/asplenia
with or without other congenital anomalies
(fetal/embryonic form):(10-35%)

Biliary Atresia
TYPES
• Type I: atresia of the CBD, while the proximal ducts are
patent
• TypeII:atresia of the hepatic duct, with cystic structures
found in the porta hepatis
• Type III (>90%):atresia of the R&L hepatic ducts to the
level of the porta hepatis.
• Of great importance, these variants should not be
confused with intrahepatic biliary hypoplasia, which
comprises a group of distinct and surgically
noncorrectable disorders.

Pathophysiology
• Poorly understood.
• Congenital malformation of biliary
ductular system:
• Failure of recanalisation
• Ductal plate malformation
• Imbalanced apoptosis
• Disorganised cell turnover
• Disordered epithelial-
mesenchymal interaction

Pathophysiology
• Ischaemic insults
• Abnormal bile acid metabolism
• Pancreaticobiliary maljunction
• Immunologic dysfunction
• Type III (MC type) is characterized by a progressive
inflammatory lesion, suggesting a role for infectious
and/or toxic agents causing bile duct obliteration.
• Infectious agents:
• Elevated antibody titers to :
• Reovirus type 3
• Rotavirus
• CMV (25%)

Pathology
• 3 month old child died with
extrahepatic biliary atresia

Pathology
• Macroscopic appearance of
explanted liver showing large
central nodules surrounded
by cirrhotic liver (centimeter
scale).

Pathology
• Microscopically, numerous
brown-green bile plugs

Pathology

Frequency
• In the US:
• overall incidence of
1/10,000-15,000 live
births.
• Internationally:
• The incidence highest in
Asian populations
• ??more common in
Chinese Vs Japanese
infants.

Mortality/Morbidity• Long-term survival rate
(Post Kasai):
• 47-60% at 5 years
• 25-35% at 10 years.
• Overall survival for > 20 y was
reported as 22%.
• 30%, bile flow is inadequate
following surgery, and these children
succumb to complications of biliary
cirrhosis in the first few years of life
unless liver transplantation is
performed. •Nio M. The Outcome of Surgery for Biliary Atresia a
•nd the Current Status of Long Term Survivors.
•Tohoku J Exp M 1997

Race & Sex
• Race:
• Asian populations.
• Black infants X2 > white
infants.
• Sex: Females > males.

History
• Typical symptoms include variable degrees of
jaundice, dark urine, and light stools.
• In most cases, acholic stools are not noted at birth
but develop over the first few weeks of life.
• Appetite, growth, and weight gain may be normal.

Physical Exam
• No findings are pathognomonic for the disorder.
• These infants typically are full term and may manifest
normal growth and weight gain during the first few
weeks of life.
• Hepatomegaly may be present early, and the liver is
often firm or hard to palpation.
• Splenomegaly (suggests progressive cirrhosis with
portal hypertension).
• Direct hyperbilirubinemia(physiologic unconjugated
hyperbilirubinemia rarely persists beyond 2W)
• Cardiac murmurs

Lab Studies
• Bilirubin: direct bili > 2.0 mg% or 20% of T. bili
• T. bili (6- to 12-mg%) range
• ALP, GGTP, AST, ALT:
• no single test discriminates between biliary atresia
and the other causes of neonatal cholestasis.
• Serum alpha1-antitrypsin with Pi typing:
• Alpha1-antitrypsin deficiency MC inherited liver
disease that presents with neonatal cholestasis

Ultrasonography
• Exclude specific anomalies of the extrahepatic biliary
system, particularly choledochal cyst. (Today, a
diagnosis of choledochal cyst should be made in
utero by fetal U/S).
• In biliary atresia, may demonstrate absence of the
gallbladder.
• Sensitivity and Specificity do not exceed 80%

Triangular Cord Sign
• Magd A. Kotb etal, PEDIATRICS
Vol. 108 No. 2 August 2001, 416-
420

Hepatobiliary Scintiscanning (DISIDA)
• Evidence of intestinal excretion of radiolabel
confirms patency of the extrahepatic biliary system.
• Two cautionary notes:
• Reliability of the scintiscan is diminished at very
high conjugated bilirubin levels (>20 mg%)
• 10% rate of false-positive/false-negative
diagnostic errors.

Kasai Portoenterostomy
• Introduction in 1957.
• Not widely accepted by the
western world until 1970s.
• The only palliative treatment for
patients with biliary atresia
• Increased 5-year survival from
0% to 60%
Kasai M, Kimura S, Asakura Y, Suzuki Y, Taira Y, Obashi E.
Surgical treatment of biliary atresia. J Pediatr Surg 1968;3:665-675

Hepaticoportojejunostomy

Hepaticojejunostomy

Hepaticoportoduodenostomy

Hepaticoduodenostomy

Portocholecystostomy

Factors Influencing Outcome
• Age (before 60 days)
• Hyaluronic Acid
• Experience or caseload of a
surgical centre
• Preop Jaundice: no
relationship with the outcome
• Serum transaminases and
bilirubin have no predictive
value

Age @ Surgery
• Surgery done before age of 60 days is associated with
favourable outcome.
• Some centres provide acceptable results with operation
done between 60-90 days.
• Sendai series revealed a 10 years survival rate of 73%
for those operated on before 60 days, but only 11% for
those underwent surgery after 90 days of life.
Valayer J. Conventional Treatment of Biliary Atresia:
Long Term Results. J Ped Surgery 1996;31:1546-1551

Billirubin
• Serum transaminases and bilirubin have no predictive
value on the outcome.
• However, the rapidity with which bilirubin returns to
normal after Kasai operation is an important predictive
factor for long-term survival.
• Serum bilirubin <1 mg/dL within 3 months after
operation implicates good long-term outcome.
Ohhama Y. Early Prediction of Long Term Survival and the
Timing of Liver Transplantation after the Kasai Operation.
J Ped Surgery2000;35:1031-1034.

Ohhama Y. Early Prediction of Long Term Survival and the Timing of Liver
Transplantation after the Kasai Operation. J Ped Surgery2000

Ohhama Y. Early Prediction of Long Term Survival and the Timing of
Liver Transplantation after the Kasai Operation. J Ped Surgery2000

Hyaluronic Acid
• Serum level was found to be a useful early marker to
predict the outcome of a patient after Kasai operation.
• HA is cleared rapidly from the circulation by the liver via
receptor mediated endocytosis into sinusoids.
• During progressive liver disease, these cells undergo
transformation impairs the uptake of
metabolites such as HA.
• >450g/L has similar predictive power as surgical age.
Dhawan A. Serum hyaluronic acid as an early prognostic maker in
biliary atresia. J Ped Surgery 2001;36:443-446

Survival Data
• A nationwide survey in Japan revealed 16% out of
2013 patients have survived for more than 10 years.
Only about half of these patients are jaundice free after
10 years.
• Miyano T. Current Concept of the Treatment of Biliary Atresia.
World J Surg 1993
• A similar survey in North America in 1989 reported
long-term survival rate of 25%.
Karrer FM. Biliary atresia registry. J Ped Surgery 1990
• Another, Japanese series produced the best result in
terms of survival. Fifty-nine percents survived for 10
years after Kasai with >80% of them are jaundice free.
Overall survival for > 20 y was reported as 22%.
• Nio M. The Outcome of Surgery for Biliary Atresia and the Current Status
of Long Term Survivors. Tohoku J Exp M 1997

Howard ER. J Ped Surgery 2001;36:892- 897.

Overall Survival (without liver transplantation)
(Grouped by centre caseload)
Patrick J McKiernan ,Lancet 2000
-Group-A centres
(>5 cases/year)
-Group-B centres
(<5 cases/year).

Overall Survival
(Grouped by centre caseload)
Patrick J McKiernan ,Lancet 2000
-Group-A centres
(>5 cases/year)
-Group-B centres
(<5 cases/year).

Seamless Management of Biliary Atresia in England and Wales
(1999-2002)
• Before 1999, infants born in the UK with suspected
biliary atresia were investigated in regional centres,
and, if confirmed, a Kasai operation was done there.
• Since 1999, all infants with suspected biliary atresia in
England and Wales, UK, have been referred to one of
three designated centres where both the Kasai
operation and liver transplantation (if necessary) could
be done.
Dolgin SE.,Lancet. 2004,24;363(9418):1354-7.

Outcome of infants with Biliary Atresia (Jan, 1999, to June, 2002)
Dolgin SE.,Lancet. 2004,24;363(9418):1354-7.

Seamless Management of Biliary Atresia in England and Wales
(1999-2002)
51%30%Native Liver Survival
89%85%Overall Survival
1999–2002(4y)1993–1995(5y)
Our early results suggest that surgical outcome can be improved
by centralization of care to supra-regional centers.
Dolgin SE.,Lancet. 2004,24;363(9418):1354-7.

4-year Actuarial Survival rates
-overall survival :89%
-4-year survival with
native liver:51%
Dolgin SE.,Lancet. 2004,24;363(9418):1354-7.

Postsurgical Complications
• Unsuccessful Anastomosis
• MC complication
• 1/3 of all patients, bile flow is inadequate following
surgery, and then succumb to biliary cirrhosis in the
first few years of life unless OLT is performed
• Cholangitis
• 93% of patients before 1 year of age
• 30-40% at 5 years post surgery
• Some patients may present as late as 13 years after
Kasai
• The incidence is reduced by antibiotic prophylaxis
• Early and prompt antibiotic administration helps in
restoring good liver function.
Gottrand F. AJDC 1991;145:213-215

Postsurgical Complications (cont.)
• Portal Hypertension and Variceal Bleeding
• >60%
• Recurrent uncontrolled variceal bleeding
is an indication for transplantation
• Hepatocellular Ca
• OLT is the only option for long-term survival.
• Hepatopulmonary Syndrome
• uncommon a/w chronic liver disease
• Triad of liver dysfunction, arterial hypoxaemia and intrapulmonary
vascular dilatation.
• Dyspnoea, particularly orthodeoxia, and cyanosis.
• Mortality rate 41%
• OLT

Post Op Meds
• Steroids:
• In the immediate postop
• anti-inflammatory agent
• Nonspecific stimulant of bile salt-
independent bile flow.
• Ursodeoxycholic acid (ursodiol):
• Enhance bile flow
• may improve outcomes, and
associated with minimal toxicity.
• Bactrim:
• Prophylactic for cholangitis

Quality of Life
• 22 patients who survived >
20y
• 3 women were married and 1
had completed a normal
pregnancy.
• Normal growth was achieved
in all except 1 who had
Turner syndrome.
• 2ry sexual characteristics
were normal in all patients.
except the patient with Turner
syndrome
• School performance was
normal in 8, 1 year below
normal for 11, and 2-3 years
subnormal for 7 patients Nio M. Current Status of 21 Patients Who Have
Survived More than 20 years since Undergoing
Surgery for Biliary Atresia. J Ped Surgery 1996

Quality of Life
Nio M. Current Status of 21 Patients Who Have
Survived More than 20 years since Undergoing
Surgery for Biliary Atresia. J Ped Surgery 1996

Quality of Life
• A recent comparison of quality of life in long-term biliary
atresia survivors from King’s College and Tohoku
University
• The quality of life measurements were comparable
between 2 centres except small reductions in the
scores for general health and vitality among the
Japanese.
Howard ER. Survival Patterns in Biliary Atresia and
Comparison of Quality of Life of Long-Term Survivors in
Japan and England. J Ped Surgery 2001;36:892- 897.

Reoperation
• 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

Liver Transplantation
• Extrahepatic biliary atresia is MC
diagnosis in children requiring
OLT(>50% of patients with liver
transplants in most series)
• Consider OLT early in patients who
do not achieve clearing of jaundice
following Kasai.
• However, in most series the primary
indications for OLT are the
symptoms of end-stage liver
disease.
• OLT will salvage patients with failed
Kasai with 10-year posttransplant
survival of 71%. Altman, R. Peter, etal, Annals of
Surgery. 226(3):348-355, 1997

Liver Transplantation
• Overall, a recent review demonstrated that 66% of
infants undergoing the Kasai procedure ultimately
required OLT, including more than 50% of patients
who initially achieved bile drainage.
• OLT as the primary treatment for biliary atresia may
be indicated only for patients > 120d of age with an
enlarged and hard liver.
• Kasai, M, etal(Surgical limitation for biliary
atresia: indication for liver transplantation)J
Pediatr Surg. 1989 Sep;24(9):851-4.

Medical/Legal Pitfalls
*With improved survival rate of 70-80% with liver
transplantation, there are different opinions pertaining
to the choice of treatment for BA. Some recommended
primary liver transplantation as a curative approach.
*> 60% of infants undergoing the Kasai procedure
ultimately require OLT
*Organ procurement problems??

Laparoscopic Kasai
Esteves E,et al. Laparoscopic Kasai portoenterostomy
for biliary atresia. Pediatr Surg Int 2002;18:737–40.

Laparoscopic Kasai

Robot Surgery
• (A good surgeon must have the eyes of an eagle, a
lion's heart and the hands of a lady)
D r L. Willoughby, 1935
• The da Vinci® robotic surgical cart

Robot Surgery
Computer-assisted
robot-enhanced technology
allows complex GI surgery to be
performed using MIS
Lorincz A, etal(Robot-assisted minimally
invasive Kasai portoenterostomy)Surg
Endosc. 2004 Jul;18(7):1136-9. 2004

Robot Surgery
Mariano ER, etal(Anesthetic concerns for robot-assisted
laparoscopy in an infant)Anesth Analg. 2004 Dec;99(6):1665-7,

Kasai procedure

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