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' iN \ l ) - s re 7 i h Li J = 4 zn | A naw yy cy Ñ' _ PC AC THANH `» A A | = : £ —_ Ff ta: | = & 3 = fy = & a a ch `, ˆ General:

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

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BILIARY ATRESIA

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

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==

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

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— > — "¬ — %* a RY ATRESIA: Classification Classification 1: - A(operated): 10-15%, a major

portion of the extrahepatic bile ducts are patent (the proximal common hepatic

duct is patent, allowing for

primary anastomosis of the extrahepatic bile duct to the

bowel.)

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—_—— —_—._

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

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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

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BILIARY ATRESIA

- 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

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—_ Ƒ— =.=.-.ễ— oO —— Gallbladder contraction

- 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

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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

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64-day-old female infant with biliary atresia

Sonogram shows

abnormal gallbladder

(arrows) Gallbladder is

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® Small, abnormal

gallbladder (arrow) in 8-

week-old infant with BA

has irregular wall and

abnormal shape

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® Abnormal gallbladder

(arrow) in 10-week-old

infant with BA Note the

more subtle irregularity

of the wall (arrowheads)

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(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 ;

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xo ¬ Triangular cord Transverse (a) and longitudinal (b) scans of

the triangular cord ina

baby with biliary atresia,

which appears as a focal echogenic triangular or ovoid density just

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' 3s-day-old male infant

with biliary atresia Sonogram reveals

tubular echogenic cord

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® 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-

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® Transverse sonogram

shows TC sign (arrow) in

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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

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

triangular echogenic area with biliary atresia (a)

(cursors) in the anterior Longitudinal scan shows TC

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

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".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

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= — — ~_~-

Sonography sign accuracy

® The diagnostic accuracy of the triangular cord

sign( TC sign) was 95%, sensitivity was 93%, and specificity was 96%

71%, sensitivity was 72%, and specificity was 69% © The diagnostic accuracy of gallbladder contraction

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

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-Hepatobiliary scir ® A technetium-labeled iminodiacetic acid (IDA) ae is typically used, include” Tc (technetium-ggm

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

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mm —_ — Biliary atresia In a 2- week-old boy: ® Hepatobiliary scan obtained after a 35- minute delay demonstrates absence of

activity in the central

common bile duct and

small intestine

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phy -

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

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

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-

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

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ỷ—_— nam = mm šÝ - Liver biopsy: =>" Pathologic changes in

Pathologic changes in biliary atresia: a marked neonatal hepatitis: lobular

disarray, giant cell degree of fibrosis, bile duct

transformation, and proliferation, and portal

mononuclear lobular inflammation

infiltrate

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- —— — SS ndoscopic retrograde — cholangiopancreatography( ERCP)

® 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)

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® 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

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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)

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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

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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

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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

uct

(b) Oblique coronal thick-slab MR T2- Geighted single-shot fast SE MR

cholangiogram shows a choledochal cyst (short arrow) and the gallbladder (arrowheads) The triangular area of high signal intensity (long arrow) located in the porta hepatis is not continuous with the choledochal cyst or gallbladder Intraoperative

cholangiogram (not shown)

demonstrated a choledochal cyst, but the triangular structure at the porta

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BILIARY ATRESIA

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

anastomosis ofthe bowel to the bed of the porta

hepatis)

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