Biliary Atresiadoccdn.simplesite.com/d/a8/e9/287104482457151912/f5281825...Impact of age at Kasai...
Transcript of Biliary Atresiadoccdn.simplesite.com/d/a8/e9/287104482457151912/f5281825...Impact of age at Kasai...
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
-