Laporan Kasus

An. Yeni Aprilia/ 1 bulan Anamnesis: Kuning sejak 2 minggu setelah lahir BAK makin kuning BAB makin putih Pemeriksaan Fisik: Keadaan umum baik Icterus, hepatomegali

Laboratorik:
Bilirubin direk 24 g/dL Bilirubin total 31 g/dL Alkali phosphatase 678 SGOT 324 SGPT 264

USG
Liver normal, IHBD/IHBD tak melebar GB kecil 6.7 mm

Operasi
Kasai procedure

Anatomy: Two different forms

1. syndromic BA 2. Non-syndromic BA

The French classification

1. Type 1 (3%): Atresia limited to common bile duct 2. Type 2 (6%): Cyst in the liver hilum communicating with dystrophic intrahepatic bile ducts 3. Type 3 (19%): Gallbladder, cystic duct and common bile duct patent 4. Type 4 (72%): Complete extrahepatic biliary atresia

Aetiology:
Unknown, morphogenesis of bile ducts Virus, genetic component

Diagnosis:
Prenatal diagnosis Clinical features Ultrasonography Cholangiography Liver biopsy Others Absence of medical causes of neonatal cholestasis

Treatment: Two steps:

1) 2) 1) 2) 3) 4) 5) in the neonatal period, the Kasai operation subsequent liver transplantation Transverse supra-umbilical incision Confirm the diagnosis After section of the falciform and left triangular ligaments, exteriorise the liver Excise the extrahepatic biliary tree Preparing a 45 cm Roux en Y loop

The Kasai operation: hepatoporto-enterostomy:

Treatment: The Kasai operation: hepatoporto-enterostomy:

6) Make an anastomosis between the cut edge of the transsected tissue in the porta hepatis and the antimesenteric side of the Roux loop

The tehnic, based on anatomical pattern:

in type 1: cholecysto-enterostomy, or hepaticoenterostomy. in type 2: kysto-enterostomy. in type 3: hepatoporto-cholecystostomy

Treatment: Post operative course:

The indication of corticosteroids remains controversial

Complication after successful Kasai operation:

Cholangitis Portal hypertension Hepatopulmonary syndrome and pulmonary hypertension Intrahepatic biliary cavities Malignancies

Treatment: Outcome after unsuccessful Kasai operation

biliary cirrhosis continues liver transplantation is required

Overall outcome of BA patients

Overall survival of BA patients at 5 and 10 years was 71% With improvements of the overall management of BA patients, an overall survival of 90% is nowadays realistic