1. Teo duong mat BS Hieu BVND1 E

Biliary atresia is characterized by luminal obstruction of the extrahepatic bile duct with a fibrous ductal remnant representing the obliterated duct in the porta hepatis - The prima

Definition: Biliary atresia is a condition in which the

normal extrahepatic biliary system is disrupted

Progressive damage of extrahepatic and intrahepatic

bile ducts secondary to inflammation may occur,

leading to fibrosis, biliary cirrhosis, and eventual liver

failure

Biliary atresia is characterized by luminal obstruction

of the extrahepatic bile duct with a fibrous ductal

remnant representing the obliterated duct in the porta

hepatis

- The primary therapy is surgical.

BILIARY ATRESIA

® Prevalence: Approximately 1 in 10,000-15,000 births

® Male: Female = 2/1

==

BILIARY ATRESIA

2 distinct clinical forms:

- fetal-embryonic (or syndromic): is characterized by early

cholestasis, appears in the first 2 weeks of life, accounts for

10-35% of all cases In this form, the bile ducts are

discontinuous at birth, and 10-20% of affected neonates

have associated congenital defects: s\\s inversus,

oo ysplenia, malrotation, intestinal atresia, and cardiac

anomalies

perinatal (or acquired): accounts for the remaining 65-

90% cases 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 . -free

interval

duct is patent, allowing for

primary anastomosis of the extrahepatic bile duct to the

bowel.)

- B(Inoperable): does not have the patency shown in.

—_—— —_—._

BILIARY ATRESIA

Kasai classification: 3 main types

type I: the common bile duct is obliterated while the proximal bile ducts are patent

type Ila: atresia of the hepatic uct, the cystic and common bile ducts are patent

- type IIb: the cystic, common bile duct: and hepatic ducts are obliterated

on II]: atresia refers to iscontinuity of the right and the left hepatic ducts to the level of the porta hepatis, more than 90% of cases

BILIARY ATRESI

[maging modalities:

- Ultrasonography: the initial investigation in patients

with suspected biliary atresia

- Hepatobiliary scintigraphy

- Magnetic resonance cholangiopancreatography

(MRCP)

- Surgical and percutaneous cholangiography

- Endoscopic retrograde cholangiopancreatography

( ERCP)

BILIARY ATRESIA

© Ultrasonography:

- Technique: fasting at least 4 hours before examination

2 times obtained: 60 -go minutes after patient was fed

- The gallbladder ghost triad: a gallbladder length less than

1.5cm(1.9cm), a thin or indistinct gallbladde wall, and an

irregular and lobular contour

- The triangular cord sign( TC sign): a circumscribed, focal,

triangular or tubular echogenic density more than 3 mm thick

located cranial to the portal vein bifurcation

- Central biliary cysts and choledochal cysts may be associated

with biliary atresia

- For evaluation of gallbladder contraction, the examination was

repeated 60-go min after the infant was fed

- The volume of the gallbladder was calculated using the following

equation: V= 0,52 x width x width x length

- Gallbladder contraction was evaluated by calculating the contraction

index (Cl) as follows:

Cl(%)= 100 x (fasting Volume — postprandial Volume)/ fasting Volume

- Anormal C! = 86% = 18% (mean + SD) in 6-week-old infants(value for

patients younger than 12 weeks old) and 67% + 42° in 4-month-old

(value for patients 12 weeks old or older )

If the Cl less than the mean + SD, the gallbladder was described as

uncontracted

(Sonographic Diagnosis of Biliary Atresia in Pediatric Patients Using the “Triangular Cord” Sign Versus

Gallbladder Length and Contraction)

Gallblader

Gallbladder ghost triad in babies with biliary atresia Longitudinal scans of the gallbladder in (A) a 3-week- old girl and (B) a 5-week-old boy demonstrate a short

gallbladder, an irregular or

lobulated contour, anda

relatively indistinct lining and wall

(arrows) Gallbladder is

1.0 cm long

® Small, abnormal

gallbladder (arrow) in 8-

week-old infant with BA

has irregular wall and

abnormal shape

® Abnormal gallbladder

(arrow) in 10-week-old

infant with BA Note the

more subtle irregularity

of the wall (arrowheads)

of this 21-mm-long gallbladder

(b) Schematic drawing represents- TE-SIGN: the anatomic relationship between

the fibrous ductal remnant and

a) Photograph of surgical one of ze

obliterated extrahepatic bile ducts blood vessels around the porta

shows the fibrous ductal remnant hepatis The triangular, cone-

(black arrowheads) in the porta | shaped, fibrous ductal remnant hepatis, atretic gallbladder (arrow), P

and fibrous common bile duct (white (black arrowheads, green) Is

arrowhead) The fibrous ductal positioned anterior and slightly

remnant is a triangular cone-shaped superior to the portal vein (long mass arrow, blue) and the hepatic artery ;

(short arrow, red)

the triangular cord ina

baby with biliary atresia,

which appears as a focal echogenic triangular or ovoid density just

cranial to the bifurcation

of the portal vein

' 3s-day-old male infant

with biliary atresia

Sonogram reveals tubular echogenic cord

(arrows)

® 1s-day-old female neonate with unknown cause of infantile

cholestasis Sonogram

reveals tubular

echogenic cord (arrows)

“Triangular cord” was

0.3-0.4 cm wide and 1.3-

1.6 cm long.

® Transverse sonogram

shows TC sign (arrow) in

7-week-old infant with

BA Area of increased

Sonogram in 10-week-old

infant with BA shows TC sign

(arrow) superior to portal

vein (arrowhead)

The TC sign (cursors) asa

thick, tubular, echogenic area

along the anterior aspect of the right portal vein (long

arrow)

(b) Transverse scanshows Images ofa 3s-day-old boy

aspect ofthe portal veinin sign (cursors) with a 5.1-mm-

".x

`" &

Images of a 36-day-old boy with

biliary atresia but no

demonstrable TC sign (a)

Longitudinal US scan shows

EARPYV is 3.0 mm thick between

cursors

(b) Surgical specimen of the

fibrous ductal remnant is a pattern of fibrous hepatic

duct (arrowheads) in the

porta hepatis

= — — ~_~-

Sonography sign accuracy

® The diagnostic accuracy of the triangular cord

sign( TC sign) was 95%, sensitivity was 93%, and

specificity was 96%

© The diagnostic accuracy of gallbladder length was

71%, sensitivity was 72%, and specificity was 69%

© The diagnostic accuracy of gallbladder contraction

was 77%, sensitivity was 85%, and specificity was 73%

(Sonographic Diagnosis of Biliary Atresia in Pediatric Patients Using the

“Triangular Cord” Sign Versus Gallbladder Length and Contraction)

DISIDA sega yl-iminodiacetic

acid) and" Tc tebrofenid (trimethylbromo-iminodiacetic acid)

® Infants with biliary atresia usually have normal hepatocyte uptake of the radiotracer if they are younger than 2 months of age

® If excretion of radiotracer into the

bowel is seen, biliary atresia is

virtually excluded If radiotracer

excretion is absent after 24 hours (as

it is in the image below), biliary atresia is suspected

® Hepatobiliary scan

obtained after a 35-

minute delay

demonstrates absence of

activity in the central

common bile duct and

small intestine

phy -

© Surgical cholangiography is typically performed by

injecting contrast material through the gallbladder If no

communication is seen between the biliary tree and the

gastrointestinal tract, biliary atresia is diagnosed

urgical and percutaneous cholangiog

© Ultrasonography-guided percutaneous

cholecystocholangiography is a relatively new technique in

which radiographic contrast material is injected into the

gallbladder under ultrasonographic guidance and the

extrahepatic biliary system is viewed with fluoroscopy

® Liver biopsy is often used to confirm the diagnosis of

biliary atresia and may be done at the same time as

surgical or percutaneous cholangiography

-

Surgical and percutaneous cholangiography

Biliary atresia and central

cyst:

(A) Oblique sonogram

demonstrates a large cystic structure in the porta

hepatis

(B) Intraoperative

cholangiogram

demonstrates filling of the

cyst and mildly dilated intrahepatic ducts but no communication with the duodenum m

Pathologic changes in biliary atresia: a marked

neonatal hepatitis: lobular

disarray, giant cell degree of fibrosis, bile duct

infiltrate

® ERCP allows direct visualization of the extrahepatic biliary tree

with the injection of F250) DẸTE contrast agent into the

extrahepatic biliary system through the papilla of Vater It

requires a general anesthetic, substantial expertise, and the

availability of sufficiently small endoscopes

® This technique can show obstruction in the common bile duct

and enables visualization of the extrahepatic biliary system

distal to the common hepatic duct and the extrahepatic biliary

system with bile lakes at the porta hepatis

® ERCP, while not an alternative to noninvasive imaging, can be

used to avoid surgery in approximately 25% of cases

(a study by Petersen et al)

® Findings in infants with biliary atresia include

incomplete visualization of the extrahepatic biliary

system and periportal high signal intensity on T2-

weighted magnetic resonance imaging (MRI) scans

(which may represent cystic dilatation of fetal bile

ducts with surrounding fibrosis)

Triangular Area of High Signal Intensity in the Porta Hepatis at T2-weighted MR Cholangiography with US Correlation

Images obtained in a 13-day-old

female neonate with acholic stool (a)

Transverse gray-scale US image of the

liver shows an echogenic triangular

cord (arrowheads) that contains a

linear hypoechoic lesion (arrow)

Oblique coronal thick-slab T2- weighted single-shot fast SE MR cholangiogram shows a triangular area of high signal intensity (arrow) in the porta hepatis and does not show the extrahepatic bile duct

a 57-day-old female infant with

cholestatic jaundice and acholic stool

power Doppler US image shows a

tubular cystic lesion (straight arrow),

which does not have a flow signal,

anterior to the portal vein (curved

arrow) Cursors indicate the

maximum depth of this cystic lesion

Oblique coronal T2-weighted

single-shot fast SE MR cholangiogr am demonstrates an

inver ted tr riangular area of high

signal intensity (arrow) anda

normal ¢ gallbladder (arrowheads)

in a 59-day-old female infant with

persistent jaundice and acholic

stool (a) Transverse power

Doppler US image of the liver

shows a small ovoid hypoechoic

lesion (arrow) within a triangular t

cord that abuts the portal vein

Oblique coronal thick-slab T2-

weighted single-shot fast SE

MR cholangiogram shows a small triangular area of high

signal intensity (arrow) inthe

porta hepatis

Images obtained in a 40-day-old

female infant with jaundice and

acholic stool (a) Oblique gray-scale

US image of the liver shows a

tria ngular hypoechoic lesion (long

straight arrow) within a periportal

echogenic mass, a dilated common

bile duct (short straight arrow), and

the portal vein (curved arrow)

Cursors indicate the maximum width

(0.79 cm) of the dilated common bile

or gallbladder Intraoperative cholangiogram (not shown) demonstrated a choledochal cyst, but the triangular structure at the porta

hepatis was not visualized

BILIARY ATRESIA

® Treatment: Kasai portoenterostomy( a Roux-en-Y

anastomosis ofthe bowel to the bed of the porta

hepatis)

© Prognostic: patient had BA will have good prognosis

if be operated as soon as possible before 3 months of

age

® Final stage: biliary cirrhosis must be undergone liver

transplatation.

Thank you for your attentionl