CHAPTER 81

Biliary Atresia
Sanjay Krishnaswami
Richa Lal
Katrine Lofberg
Alaa Fayez Hamza

Introduction

Biliary atresia (BA) is a neonatal disease characterised by the inflammatory and sclerotic obliteration of part or all of the extrahepatic biliary tree,
with varying involvement of the intrahepatic bile ducts. Although seen in
only a small percentage of jaundiced neonates, this disorder is one of the
more common structural causes of neonatal cholestatic jaundice.
If left untreated, biliary atresia is almost uniformly fatal. Hepatic
failure, infection, or bleeding secondary to portal hypertension causes
death by the age of 1 to 2 years in the vast majority of patients.1,2 In the
current era, despite the diagnostic difficulties and technical challenges
that BA poses, early recognition and the proper performance of a Kasai
portoenterostomy procedure can be life saving. When available, liver
transplantation can serve as the ultimate therapy for those who have
end-stage disease.

Demographics

Based mostly on Western series, the incidence of biliary atresia is

between 1:8,000 and 1:17,000 live births, with an overall female preponderance of up to 1.7:1.3 In most of Africa, incidence data are not
available, but individual institutional reports suggest that up to five
children with this disorder are encountered yearly in many centres.46
Therefore, although cholestasis secondary to infectious causes is more
common, BA is encountered fairly frequently in Africa.4,7,8 No specific
genetic factors are associated with the disease, but associated congenital malformations occur in 1120% of the cases.3 The most commonly
associated anomaly is polysplenia; other associated anomalies include
intestinal atresias, abdominal situs inversus, malrotation, and genitourinary and cardiac anomalies.

Aetiology/Pathology and Classification

Many aetiologies for the disease have been proposed, including genetic
factors, congenital developmental causes such as a failure of recanalisation or antenatal ischaemia, and viral or other infectious causes. None
have been proven, however, and the pathogenesis remains unknown.
Because two groups of patients are affectedthose with other congenital anomalies and those with a late foetal or perinatal anomaly that
appears to occur in isolationthere may in fact be an interplay of these
various aetiologies in the development of BA in any individual patient.
Microscopic examination of the biliary system reveals fibrosis
of the ductules with varying degrees of inflammation. Early in the
disease, bile duct proliferation, biliary plugs, and mild portal fibrosis
are present. Later in the disease, this fibrosis extends intrahepatically
and will manifest as bridging fibrosis of the biliary structures.
Classification of biliary atresia is generally based upon the
macroscopic location of the fibrotic biliary cord remnants (Figure 81.1).
The most common type is complete fibrosis of the entire extrahepatic
biliary system, seen in up to 7080% of cases. Gallbladder and common
bile duct patency with obliteration of the porta and hepatic duct is the
next most frequent (1220%). Absence of portions of the biliary system
with fibrosis of the remaining portions as well as distal fibrosis with
proximal hilar cyst variants can also be seen. Macroscopically, these

(A)

(B)

(C)

Source: Karrer FM, Pence JC. Biliary atresia and choledochal cyst. In: Ziegler MM, ed.
Operative Pediatric Surgery. McGraw-Hill, 2003. Reproduced with permission from the McGrawHill Companies.

Figure 81.1: Illustration of common types of biliary atresia: (A) complete

extrahepatic biliary obliteration (most common, affecting about 80%); (B)
proximal obliteration, distal patency; and (C) common hepatic duct cystic
dilatation with portal and distal common bile duct obliteration.

mucus-containing cyst variants can be confused for a fibrotic hilar

cone or for a choledochal cyst. As these cysts do not communicate
with the biliary tree, all such structures need to be resected. Failure to
recognise this variant can lead to a nonfunctional anastamosis with the
sequestered cyst.3

Clinical Presentation

Most children with biliary atresia appear normal at birth but become
increasingly jaundiced after 3 to 6 weeks of age. Additionally, in those
children with physiologic jaundice from birth that does not resolve
spontaneously, BA should be suspected because persistence of jaundice
after 2 to 3 weeks of life should always be considered pathologic until
proven otherwise. This sign, coupled with the findings of progressively
acholic stools and dark urine, is suggestive of BA.
As the liver becomes progressively obstructed, it grows in size and
firmness. Malabsorption of fat-soluble vitamins resulting from hepatic
obstruction and failure can lead to diarrhoea, anaemia, and malnutrition.
Overall growth of the infant may therefore appear normal in the first few
weeks or months after birth, but most patients develop failure to thrive
once liver failure is more significant. Prolonged bleeding can be seen
from the umbilical stump in such cases. Finally, splenomegaly, bleeding
oesophageal varices, and other signs of portal hypertension can also be
significant parts of the examination in patients who present with advanced
illness.9 By recent African reports, up to two-thirds of patients present late
in the illness after advanced signs of liver failure are present.10
The value of the experienced paediatrician in recognising the
jaundiced baby with persistently acholic stools cannot be overstated. It
is important to recognise these and other physical findings early because
successful outcomes after surgery for biliary atresia are tied closely to
age at performance of Kasai portoenterostomy.11 Note, however, that
the physical signs and symptoms of BA described above overlap with
myriad other causes of jaundice in infancy (discussed in the next
section), and none of these findings should be considered as conclusive
for BA. Rather, early recognition of signs of persistant jaundice should
prompt swift referral to centres capable of completing the diagnosis and
instituting definitive surgical intervention, if warranted.

Biliary Atresia 477

Investigation

The differential diagnosis of neonatal jaundice is prodigious and

includes such surgically correctable obstructive lesions as biliary atresia and choledochal cyst as well as inborn metabolic errors, congenital
infectious causes, and other causes, as listed here:
physiologic: immaturity of glucuronyl transferase (resolves quickly);
breast milk feeding;
hematologic: Rh/ABO blood group incompatibility, hemolytic diseases (spherocytosis);
infectious: the TORCH (TOxoplasmosis, Rubella, Cytomegalovirus
(CMV), Herpes) complex; syphillis, hepatitis, and others;
genetic/metabolic: 1-antitrypsin deficiency, galactosaemia, tyrosinaemia, cystic fibrosis (CF), hypothyroidism, Gauchers disease,
iron storage disease;
hepatocellular dysfunction: Gilberts disease, Crigler-Najjar syndrome;
neonatal hepatitis;
total parenteral nutrition (TPN)-related cholestasis; and
extrahepatic processes: BA, choledochal cyst, Alagille syndrome
(arteriohepatic dysplasia), inspissated bile plug syndrome, bile duct
stenosis or stricture, spontaneous perforation of the bile duct.

Testing

The main goal of early testing is to rapidly differentiate jaundice

due to obstruction from that due to other causes. A proper combination of serology testing, imaging, and biopsy prior to or during an
operative cholangiogram leads to an accurate diagnosis in >95% of
patients.12 Conventional serum liver function tests are nonspecific.
Hyperbilirubinaemia, usually in the 512 g/L range, is typical of the
early stage of the disease. However, bilirubin fractionation is not useful
in distinguishing obstructive jaundice due to BA from the more common
intrahepatic cholestatic diseases (e.g., neonatal hepatitis) because there
is elevation of indirect, unconjugated as well as direct, conjugated bilirubin in both diseases. Similarly, alanine transaminase (AST) and aspartate transaminase (ALT) are also often elevated in both cases, although
extreme elevations of these are unusual in BA. In contradistinction to
this, alkaline phosphatase and gamma-glutamyl transpaptidase (GGT)
are often very elevated, and low levels of these may suggest an alternate
diagnosis. Alterations in hepatic function tests, such as prothrombin
time, partial thromboplastin time, and albumin, are typically seen only
in advanced cases of BA. Serum testing should also be done to screen
for both inborn metabolic errors and classic infectious causes such as
TORCH and syphilis, among others. An exhaustive hunt to rule out all
these possibilities can take many weeks, so one should not wait for the
results of testing prior to imaging, which may help confirm or eliminate
the possibility of an obstructive, mechanical cause of jaundice.1,9

Imaging

The two most useful imaging modalities in further differentiating surgically amenable causes from other causes of cholestasis are ultrasound
(US) and nuclear hepatobiliary imaging. Although operator-dependant,
US is available in many African centres and offers a safe and noninvasive way to evaluate the jaundiced neonate. It can be used to assess
the size of the gallbladder and intra- and extrahepatic biliary ducts and
to visualise gallstones. Although the presence of a cystic extrahepatic
or intrahepatic ductal dilatation typically rules out BA, the disease is
suspected if there is a very small or absent gallbladder, extrahepatic
ducts are not at all visualised, or the cone-shaped fibrotic portal plate
is seen.13 The presence of polysplenia or pre-duodenal portal vein lends
further support to a diagnosis of biliary atresia. Doppler use helps to
correctly interpret the adjacent hepatic artery as a vascular rather than
a biliary duct structure.

Figure 81.2: Nuclear hepatobiliary scan demonstrating prompt uptake of

technecium tracer with no excretion into the gut after 24 hours. This image is
suspicious for biliary atresia, but could still be consistent with neonatal hepatitis.
Excretion into the gut at any time rules out biliary atresia.

Nuclear imaging is performed with intravenous administration of

technecium-99m, which is taken up by the liver. In neonatal hepatitis,
there is delayed technecium uptake due to hepatocellular dysfunction
and delayed or absent excretion into the gut. In BA, uptake is usually
prompt (especially early in the disease) but there is no gut excretion
(Figure 81.2). Therefore, visualisation of tracer in the intestine rules
out BA, whereas absence of tracer visualisation in the intestine could
be due to either process. In resource-poor locations without nuclear
imaging techniques, it may be judicious to proceed straight to either
percutaneous biopsy or operative biopsy and cholangiogram via a
laparoscopic or open technique.

Management

If, after initial investigation, the diagnosis of BA is still suspected, prompt

operative intervention is warranted to definitively determine whether the
patient has BA and, if so, to perform a Kasai portoenterostomy procedure.

Preoperative Considerations

In addition to routine preoperative care for any abdominal procedure, a

dose of vitamin K (1 mg/kg) can be given several days prior to surgery.
Coagulation factors (prothrombin time/international normalization
ratio/partial thromboplastin time, or PT/INR/PTT) should be checked
to ensure suitability for operation, and type-specific blood should be
available. Routine bowel preparation is not necessary, but a dose of
preoperative broad-spectrum antibiotic is given prior to incision.

Initial Intraoperative Considerations: Cholangiogram

and Liver Biopsy

Traditionally, the operation commences with a small right upper quadrant

transverse or oblique incision. However, if available, laparoscopy can
also be utilised for the initial portion of the procedure. If a gallbladder is
visualised and is patent, an operative cholangiogram is the next manoeuvre, wherein the tip of the gallbladder is cannulated with an angiocatheter
and contrast is instilled under fluoroscopy. Contrast in the duodenum and
continuity with the intrahepatic ducts rules out BA (Figure 81.3)
If biliary atresia is ruled out and a biopsy has not previously been
done, a generous wedge biopsy of the liver is performed and the
incision(s) is (are) closed. An adequate liver biopsy specimen that has
at least five portal tracts, reported by an experienced pathologist done
after 46 weeks of age, should have an overall accuracy of 96%. The
biopsy not only helps to differentiate BA from neonatal hepatitis, but
also points towards specific aetiologies of neonatal hepatitis as well as
1-antitrypsin deficiency and storage disorders such as Niemann-Pick
disease. In addition to being sent for pathologic exam, a portion of the
biopsy specimen should be sent for viral and bacterial cultures.
If no gallbladder or a fibrotic gallbladder is present, or if
cholangiogram reveals a lack of either the proximal or distal extrahepatic
ducts (Figure 81.4), the right upper quadrant incision should be widened
or the procedure converted to open from laparoscopic in preparation for
hepatic portal exploration and the Kasai procedure.

478 Biliary Atresia

Kasai Roux-en-Y Portoenterostomy

The portoenterostomy procedure, first described by Kasai and Suzuki in

1959,14 uncovers patent biliary microductules proximal to the level of
extrahepatic biliary fibrosis and allows these structures to drain directly
into a segment of defunctionalised intestine.
The procedure begins with separation of the gallbladder from its
liver bed down to the junction of the cystic and common bile ducts.
1. The peritoneum over the hepatoduodenal ligament is opened, exposing
the biliary and hepatic arterial structures (Figure 81.5). The fibrous
common duct is dissected and transected at its margin with the duodenum.
2. The entire gallbladder and fibrous common duct is placed on
traction in a superior direction. The cystic artery is ligated, being
careful to definitively identify it from the right hepatic artery.

Figure 81.3: Intraoperative cholangiogram with contrast filling hypoplastic

proximal and distal duct system with emptying into duodenum: (A) contrast-filled
catheter within gallbladder; (B) left and right hepatic ducts; (C) intrapancreatic
common bile duct. Patient had Allagille syndrome, not biliary atresia.

3. The ductal remnants are then dissected from the adjacent hepatic
artery and portal vein until the right and left branches of the portal vein
are identified (Figure 81.6).

4. Further dissection reveals the widening of the fibrotic duct into a

cone-shaped mass entering the liver superiorly. This fibrotic hepatic
endplate is then transected at the point at which it is flush with the liver
edge. In order to identify this level, small branches of the portal vein to
the central portion of the fibrotic endplate must often be divided such
that the most superior dissection is actually behind the portal vein.
(Figure 81.7).
5. If there is discontinuity of the proximal biliary tree, meticulous
exploration over the bifurcation of the portal vein will lead to
identification of the fibrotic cone. Once the endplate is divided, cautery
on this portion of the liver should be avoided, and a moist gauze
should be placed here while attention is turned to the construction of a
Roux-en-Y intestinal limb. The completed portal dissection is shown
in Figure 81.8.

6. A 40-cm Roux-en-Y limb is constructed by dividing the bowel 10

15 cm after the ligament of Treitz. The distal cut edge is advanced in a
retrocolic fashion up to the level of the liver. A handsewn end-to-end
or side-to-side jejunojejunostomy re-establishes intestinal continuity.
The mesenteric defect at the jejunojejunostomy is closed, and the Roux
limb is tacked to the colonic mesentery at its retrocolic window to
prevent internal hernia and excessive tension on the portoenterostomy
(Figure 81.9).

C
D

Figure 81.4 : Intraoperative cholangiogram demonstrating emptying of contrast into

the duodenum with no proximal duct filling (proximal biliary atresia): (A) contrast
being administered through a small but patent gallbladder; (B) stenotic but patent
common bile duct passing intrapancreatic and emptying into the duodenum; (C)
contrast in the duodenum; (D) retrograde filling of the pancreatic duct.

7. The portoenterostomy is performed in a single layer, end-to-side

or end-to-end fashion, using long-term absorbable sutures in an
interrupted or running fashion, being careful to take meticulous full
thickness bites of only the periphery of the porta hepatis (Figures
81.10 and 81.11). This ensures that the portoenterostomy incorporates
the entire portion of tissue that may contain biliary channels and that
risk of microductular structure compromise is minimised.

8. The completed portoenterostomy is shown in Figures 81.12

and 81.13. A closed-suction drain can be placed posterior to the
portoenterostomy, and the abdomen is closed in layers.
To limit potential postoperative cholangitis, many modifications
of the original portoenterostomy have been proposed over the years
since Kasai first described the technique. These have included initial
externalisation of the biliary conduit via a stoma and an antireflux
intussusception valve within the Roux limb, among others. However,
as none of these have been successful in preventing cholangitis, and in
fact have been associated with unique complications of their own, their
use should be discouraged.

Postoperative Considerations

Figure 81.5. Biliary atresia: view of porta hepatis and hepatoduodenal ligament
prior to dissection: (A) fibrotic gallbladder; (B) duodenum; (C) falciform ligament;
(D) approximate location of fibrotic endplate.

Nasogastric drainage is continued for several days after the operation

until gut function resumes. At that time, an oral diet is given as tolerated. Perioperative intravenous antibiotics, such as a cephalosporin, are
administered by most surgeons for at least the first 1224 hours. Many

Biliary Atresia 479

surgeons administer long-term oral antibiotics (e.g., trimethoprim/sulfa,
2 mg/kg per day) as well as choleretic agents, such as ursodeoxycholic
acid (1015 mg/kg per day) upon discharge in the hope of lessening the
incidence of cholangitis following a Kasai procedure.11
Steroids are purported to benefit biliary function after a Kasai
procedure through stimulation of salt-independent bile flow in addition
to their marked anti-inflammatory, immunosuppressive, and scar-limiting
properties. They were originally proposed for use along with broadspectrum antibiotics in cases of postoperative cholangitis and in cases
of sudden cessation of bile flow in a previously well-functioning
portoenterostomy.1,14 More recently, the routine use of steroids in the
postoperative period has become standard for some surgeons. Although its
use remains controversial, especially in light of reports showing equivocal
benefits, this practice is widespread. The evidence for steroids comes from
multiple studies reporting improved short- and long-term outcomes after
standard postoperative steroid use as reflected by decreased bilirubin level
several months after the Kasai procedure, decreased mortality at one year
postoperatively, and improved jaundice-free survival with native liver at
four- to five-year follow-up.1517 Although many of these studies involved
small numbers of patients and many potentially confounding variables
exist, none of the studies noted significant adverse reactions from steroid
use, such as infectious complications or wound issues. The optimal dose
and length of steroid treatment to achieve positive effects is unknown and
varied across the studies cited. One author of this chapter prefers a threeweek tapering course of oral prednisone (2 mg/kg per day) beginning at
hospital discharge.

Figure 81.6: Porta after isolation of hepatic arteries and partial dissection
of fibrotic endplate vessel loops around hepatic arteries: (A) portal venous
confluence; (B) fibrotic ductal structures (medial portion of fibrous endcone
already divided and reflected laterally).

Complications

Although the usual operative complications, such as severe bleeding

and anastomotic leak, can occur, these are rareeven in patients with
fairly advanced disease. The most common issues postoperatively are
cholangitis, nutritional deficiencies, and portal hypertension.
Cholangitis occurs in one-third to two-thirds of patients; its
incidence is highest within the first several years after a Kasai
procedure. It is thought that bacteria in the Roux-en-Y conduit and
bile stasis combine to cause this problem. The onset of cholangitis is
heralded by fever, leukocytosis, and an increase in bilirubin. Most cases
are responsive to supportive treatment with fluids and early institution
of broad-spectrum antibiotics covering intestinal flora. In refractory
cases, one can consider a short burst of intravenous corticosteroids, as
previously described. If not already being used prior to the first episode
of cholangitis, chronic oral suppressive antibiotics, ursodeoxycholic
acid, or oral steroids may be of benefit to prevent recurrent episodes.18
Reoperation to prevent cholangitis (by creation of an antireflux valve or
lengthening of the Roux limb) is generally unsuccessful.3
Portal hypertension is the most serious delayed complication,
seen in up to 5070% of long-term survivors.19 It can occur even in
patients who initially had a successful Kasai procedure and usually
manifests as ascites, variceal bleeding, or hypersplenism. Treatment
with diuretics, beta blockers, or variceal banding and splenectomy or
splenic embolisation can be successful. However, without the possibility
of liver transplantation, many of these patients will succumb to this
portal hypertension.
Severe nutritional deficiency can accompany liver disease secondary
to BA. Without adequate bile flow for intestinal fat absorption, children
can develop essential fatty acid deficiency. Until adequate bile flow
is achieved, it is prudent to administer formula feeds with a high
percentage of medium-chained triglycerides that can be absorbed
directly without the assistance of bile salts. Additionally, affected
neonates can develop deficiencies of the fat-soluble vitamins (K, E,
A, and D), which can result in diseases such as rickets (vitamin D
deficiency) or severe coagulopathy (vitamin K deficiency). Monitoring
vitamin levels and/or supplementation of these vitamins as available
can prevent significant morbidity.

Source: Karrer FM, Pence JC. Biliary atresia and choledochal cyst. In: Ziegler MM, ed.
Operative Pediatric Surgery. McGraw-Hill, 2003. Reproduced with permission from the McGrawHill Companies.

Figure 81.7: Fibrotic extrahepatic bile ducts with branches of portal vein to endplate.

Figure 81.8: Intraoperative photo of completed dissection of porta prior to

portoenterostomy in another patient: (A) portal vein confluence; (B) right hepatic
artery; (C) portal plate after resection of fibrous cone; arrows mark anterior and
posterior edges.

480 Biliary Atresia

Outcomes

Figure 81.9: Completed jejunojejunostomy of Roux limb: (A) proximal jejunum

at ligament of Treitz; (B) end-to-side jejunojejunostomy; (C) Roux limb to liver
passing retrocolic, secured to transverse colon mesentery.

Given that biliary atresia was previously a uniformly fatal disease, the
Kasai operation has dramatically improved the survival rates of infants
with this disease. However, the results are far from perfect. In general,
by Japanese and Western reports, 6080% of patients will achieve
initial improvement in jaundice and early success with portoenterostomy, defined by some as normalisation of serum bilirubin to less than
2 mg% at 3 months following the procedure. Of these, one-half will
have permanent relief and the other one-half will have progressive
liver failure. The patients who never achieved initial improvement go
on to experience progressive liver failure. The best five-year survival
(up to 60%) has been reported by the Japanese Biliary Atresia Registry.
Rates in most African reports are significantly lower, likely due to
the patients advanced age at the time of diagnosis and morbidities of
advanced disease.6,10

Prognostic Factors

Figure 81.10: Distal Roux limb (superior to its retrocolic passage) being opened
on its antimesenteric aspect in preparation for end-to-side anastomosis with
porta: (A) side of Roux limb; (B) portal endplate; (C) pylorus posterior and
adjacent to tip of Roux limb.

The most important long-term prognostic factors in predicting outcome

after the Kasai procedure appear to be age at presentation, achievement
of primary biliary drainage, experience of the centre, and occurrence
of cholangitis.
Age at the time of the Kasai procedure is perhaps the most important
prognostic indicator. Most reports state that the best outcomes, in both
initial success and long-term survival, occur in patients younger than
age 6070 days at the time of operation (68% 10-year survival). The
Japanese Biliary Atresia Registry extends such rates to patients up to
90 days of age, with a precipitous drop in success after that point.3,11,20
Most series report performance of a Kasai procedure in infants after
70 days of age as a risk factor for failure; however, some do not agree
with this.2123
Although it is a very important postoperative prognostic factor,
primary biliary drainage is not a guarantee of the long-term success of
a portoenterostomy. Rather, it is a requirement for success in that nearly
all patients who do not achieve primary biliary drainage will never do
so. Surgical treatment via a second (redo) Kasai procedure for patients
who did not achieve primary drainage as well as for those who had
initial drainage but subsequent cessation, was met with initial interest
secondary to case reports and small series of successes. Recent reports,
however, suggest that redo portoenterostomy is not a useful strategy in
the vast majority of patients.24

Role of Liver Transplantation

Figure 81.11: Posterior row of portoenterostomy anastomosis complete. Vessel

loops around portal venous and hepatic arterial branches posterior to Roux limb: (A)
inferior border of portal plate; (B) mucosal edge of jejunum ready for anterior row
sutures; (C) lateral sutures leading to edge of posterior portion of anastomosis.

Liver transplantation, where available, is principally useful in cases

of inadequate biliary drainage after portoenterostomy and in cases
of progressive hepatic dysfunction or refractory portal hypertension
despite initial portoenterostomy. Liver transplantation centres in North
America, Europe, and Japan now report up to 85% 5-year survival after
transplant for biliary atresia.25 Given these excellent results, some in the
West have advocated for primary liver transplantation in the neonate
with BA. However, the long-term success of portoenterostomy in a
significant percentage of patients, combined with the risks of long-term
immunosupression beginning early in life (infectious and malignant),
the expense of liver transplant, the shortage of donors, and the need
for intense follow-up, make this a less attractive initial option in these
patients. These issues are magnified in the African setting. In those
regions where transplantation is an option, it should be noted that performance of an initial Kasai procedure has not been shown to affect the
success of subsequent transplantation.26

Conclusion

Figure 81.12 : Completed Kasai procedure: (A) Roux-en-Y end-to-side

portoenterostomy anastomosis; (B) stomach and pylorus; (C) transverse colon
with retrocolic Roux limb behind it.

The Kasai procedure remains the mainstay of initial treatment for biliary atresia throughout the world. In addition, it may offer the only hope
for children with BA in most of Africa, where liver transplantation may
not be available.

Biliary Atresia 481

Despite the fact that many African reports state a poor outcome for
even those BA patients who undergo portoenterostomy, likely due to the
extremely late presentation of patients for the procedure, this should not
discourage the use of the Kasai procedure in general.6,10 It is therefore
important for the surgeon to advocate to the community for early referral
of patients with neonatal jaundice who may have biliary atresia. Time is
of the essence, so accepting centres should have expertise in the rapid
work-up of such patients. Subsequent thorough preoperative resuscitation
and preparation and meticulous technique in the performance of Kasai
portoenterostomy are crucial to the successful outcome of the patient who
presents with potentially salvageable liver disease.

Source: Altman RP, Buchmiller TL. The jaundiced infantbiliary atresia. In: Grosfeld JL, ONeill
JA, Coran AG, Fonkalsrud EW (eds.). Pediatric Surgery, 6th ed., Vol. 2. 2006, Pp 1609.

Figure 81.13 : Illustration of completed Kasai procedure.

Key Summary Points

1. Although not as common as infectious aetiologies of neonatal
cholestasis, biliary atresia is seen regularly in centres
throughout Africa.

5. A combination of ultrasound, nuclear imaging (if available), and

operative cholangiogram with or without biopsy will definitively
determine whether a patient has biliary atresia.

2. Persistent jaundice after 3 weeks of age is pathologic until

proven otherwise.

6. Kasai portoenterostomy is the cornerstone of therapy for biliary

atresia.

3. The differential diagnoses for cholestasis in the newborn

are large and include genetic/metabolic disorders, infectious
agents, hepatocellular dysfunction, neonatal hepatitis, and
extrahepatic obstructive disorders.

7. Modifications of the Roux-en-Y portoenterostomy, such as

externalisation of the biliary conduit and intussusception valves
within the Roux limb, offer no benefit.

4. Early referral to a centre that can rule out extrahepatic biliary

obstruction is vital to a successful outcome if biliary atresia is
found.

8. Cholangitis, the most frequent postoperative complication, is

usually responsive to broad-spectrum antibiotics.
9. Advanced age at the time of the Kasai procedure is one of the
most important indicators of poor outcome.

References
1.

Karrer FM, Lilly JR. Corticosteroid therapy in biliary atresia. J

Pediatr Surg 1977; 90:736.

2.

Adelman S. Prognosis of uncorrected biliary atresia: an update. J

Pediatr Surg 1978; 13:389392.

3.

Nio M, Ohio R, Miyano T, et al. Five- and 10-year survival rates

after surgery for biliary atresia: a report from the Japanese Biliary
Atresia Registry. J Pediatr Surg 2003; 38:997.

4.

Obanafunwa JO, Elesha SO. Childhood liver diseases in Jos,

Nigeria: a retrospective histopathological study. East Afr Med J
1991; 68:702706.

5.

Motala C, Ireland JD, Hill ID, Bowie MD. Cholestatic disorders of

infancyaetiology and outcome. J Trop Pediatr 1990; 36:218222.

6.

Mabogunje OA. Biliary atresia in Zaria, Nigeria: a review. Ann Trop

Paediatr 1987; 7:200204.

7.

Muthuphei MN. Childhood liver diseases in Ga-Rankuwa Hospital,

South Africa. East Afr Med J 2000; 77:508509.

8.

Akang EE, Osinusi KO, Pindiga HU, Okpala JU, Aghadiuno PU.
Congenital malformations: a review of 672 autopsies in Ibadan,
Nigeria. Pediatr Pathol 1993; 13:659670.

9.

ONeill JA, Jr., Grosfeld JL, Fonkalsrud EW, Coran AG, Caldamone
AA, eds. Principles of Pediatric Surgery. 2nd ed. Mosby, 2004.

10. Mshelbwala PM, Sabiu L, Lukong CS, Ameh EA. Management of

biliary atresia in Nigeria: the ongoing challenge. Ann Trop Paediatr
2007; 27:6973.

11. Altman RP, Lilly JF, Greenfeld J, et al. A multivariable risk factor
analysis of the portoenterostomy (Kasai) procedure for biliary
atresia: twenty-five years of experience from two centers. Ann Surg
1997; 226:348; discussion 353.
12. Zerbini MC, Gallucci SD, Maezono R, et al. Liver biopsy in
neonatal cholestasis: a review on statistical grounds. Mod Pathol
1997; 10:793799.
13. Sera Y, Ikeda S, Akagi M. Ultrasonographic studies for the
diagnosis of infantile cholestatic disease. In: Ohi R, ed. Biliary
Atresia. Professional Postgraduate Services, 1987.
14. Kasai M, Suzuki H, Ohashi E, et al. Technique and results of
operative management of biliary atresia. World J Surg 1978;
2:571580.
15. Dillon PW, Owings E, Cilley R, et al. Immunosuppression as
adjuvant therapy for biliary atresia. J Pediatr Surg 2001; 361:80.
16. Meyers RL, Book LS, OGorman MA, et al. High-dose steroids,
ursodeoxycholic acid, and chronic intravenous antibiotics improve
bile flow after Kasai procedure in infants with biliary atresia. J
Pediatr Surg 2003; 38:406.
17. Muraji T, Tusagawa C, Nishijima E, et al. Surgical management
for intractable cholangitis in biliary atresia. J Pediatr Surg 1997;
32:1103; discussion 1106.
18. Bu LN, Chen HL, Chang CJ, et al. Prophylactic oral antibiotics
in prevention of recurrent cholangitis after the Kasai
portoenterostomy. J Pediatr Surg 2003; 38:509.

482 Biliary Atresia

19. Lilly JR, Weintraub WH, Altman RP. Spontaneous perforation of

the extrahepatic bile ducts and bile peritonitis in infancy. Surgery
1974; 75:664.

23. van Heurn LW, Saing H, Tam PK: Portoenterostomy for biliary
atresia: Long-term survival and prognosis after esophogeal
variceal bleeding. J Pediatr Surg 2004; 39:6.

20. Grosfeld JL, Fitzgerald JF, Predaina R, et al. The efficacy of

hepatoportoenterostomy in biliary atresia. Surgery 1989; 106:692;
discussion 700.

24. Hasegawa T, Kimura T, Sasaki T, et al. Indication for redo hepatic

portoenterostomy for insufficient bile drainage in biliary atresia:
Re-evaluation in the era of liver transplantation. Pediatr Surg Int
2003; 19:256.

21. Davenport M, Kerkar N, Mieli-Vergani G, et al. Biliary atresia: the

Kings College Hospital experience (19741995). J Pediatr Surg
1997; 32:479.
22. Maksoud JG, Fauza DO, Silva MM, et al. Management of biliary
atresia in the liver transplantation era: A 15-year, single-center
experience. J Pediatr Surg 1998; 33:115.

25. McDiarmid SV. Current status of liver transplantation in children.

Pediatr Clin North Am 2003; 50:1335.
26. Vacanti JP, Shamberger RC, Eraklis A, Lillehei CW. The therapy
of biliary atresia combining the Kasai portoenterostomy with liver
transplantation: a single center experience. J Pediatr Surg 1990;
25:149.