Biliary Atresia : Latest Update

Dr. Sastiono, SpB, SpBA

Effect of age at Kasai Portoenterostomy on the surgical outcome: A Review of the literature.

Masaki Nio, Motoshi Wada, Hideyuki Sasaki, Hiromu Tanaka

Surg Today DOI 10.1007/s00595-014-1024-z , Springer Japan 2014

1-30 days

Group 1 : 1-30 days, group2 : 31-60days gruop3 :61-90 days, group 4 : 91-120 days

group 5 :121- 150 days ; group 6 : > 151 days

Impact of age at Kasai operation on short and long term outcome of type III biliary atresia at a single institution

Masaki Nio, Hideyuki Sasaki, Motoshi Wada, Takuro Kazama,Kotaro Nishi, Hiromu Tanaka

J Pidiatr Surg 2010 ; 45 : 2361-63

57

7

50

LDLT in Cipto M Hospital

80%

Pediatric

Adult

Biliary

Atresia

• Diagnostic

Cholestasis?

Practical message

Jaudice

Age > 2

weeks dark urine

acholic

stool

Don’t be late to consult...

1 2

GGT

4 5

>250

Non visualied

GB

Triangular cord

Direct Bil ＞１mgr

３

-Diagnostic

-Kasai /Primer Liver transplant?

-Age

Laennec’s Scoring System for Staging Fibrosis on Liver Biopsy

Stage Name Septa (thickness and number)

Criteria Score

0 No definite fibrosis - - 0

1 Minimal fibrosis +/- No septa or rare thin septum; may have portal expansion or mild sinusoidal fibrosis

1

2 Mild fibrosis + Occasional thin septa; may have portal expansion or mild sinusoidal fibrosis

2

3 Moderate fibrosis ++ Moderate thin septa; up to incomplete cirrhosis

3

4A Mild, definite, or probable cirrhosis

+++ Marked septation with rounded contours or visible nodules. Most septa are thin (one broad septum allowed)

4

4B Moderate cirrhosis ++++ At least two broad septa, but not very broad septa and less than half of biopsy length composed of minute nodules

5

4C Severe cirrhosis +++++ At least one very broad septum or more than half of biopsy length composed of minute nodules (micronodular cirrhosis)

6

Jaudice

Age > 2

weeks dark urine

acholic

stool

1 2 GGT

4 5 >250

US :triangular cord

-Non visualized GB

Direct Bil ＞１mgr

３

Updating

MMP-7 Serum Matrix Metalloproteinase-

7 Differentiate Biliary Atresia to other form

of Cholestasis

ARFI acoustic radiation force impulse

-

-

-Liver panel

-Ultrasound

-Biopsy

- PTC

-IOC

-Biopsy

Kasai Porto

Enterostomy

ATRESIA BILIER

• POST OPRERATIVE FOLLOW UP

- Jaundice Free

- Cholangitis

- Portal Hypertension

Paediatric Hepatobilier Surgery

• January to October 2019 : 69 Operations

• Intra-operative Cholangiography : 14 cases

• Kasai procedure : 12 cases

• LDLT : 5 cases

• Hepatectomy due to Hepatoblastoma : 5 cases

• Choledocal cyst excision and hepaticojejunostomy Roux en Y : 8 cases

Most common diagnosis

• Billiary atresia : 31 cases

• Choledocal duct cyst : 10 cases

• Hepatoblastoma : 8 cases

• Trauma, allagyle syndrome, teratoma, neonatal hepatitis, etc :

Terima Kasih

ATRESIA BILIER

• WHEN?

Tidak Operasi Sampai Usia

- 1 tahun 50% -80% pasien meninggal

- 3 tahun 90% -100% pasien meninggal

Jaudice

Age > 2

weeks dark urine

acholic

stool

1 2 GGT

4 5 >250

US :triangular cord

-Non visualized GB

Direct Bil ＞１mgr

３

Updating

MMP-7 Serum Matrix Metalloproteinase-

7 Differentiate Biliary Atresia to other form

of Cholestasis

ARFI acoustic radiation force impulse

-