Jaundice

pada

Kasus Bedah Anak

Dr Sastiono Divisi Bedah Anak FKUI/RSCM

Bilirubin Metabolism An Overview

Blood: Conjugated & Un-conjugated.
Conjugated Bilirubin (Direct bilirubin):

Bilirubin + Glucuronic acid. Soluble in H2O.

Unconjugated Bilirubin (Indirect bilirubin):

Bilirubin (No Glucuronic acid). Soluble in Alcohol.

Stool: Stercobilin

Urine Urobilinogen 2

Age > 2 weeks Check total bilirubin & direk

Cholestasis
-Bilirubin direct > 1mg/dl -Bilirubin direct > 20% of Total Bilirubin

Exercise
Total bilirubin 13.2 20.3 3.5 Bilirubin direk 2.4 5.2 0.9 Cholestasis ? No Yes No

Obstuctive Cholestasis
Biliary Atresia , Choledochal Cyst , inspisated bile syndrome , Alagille syndrome , Gall stones , Cystic Fibrosis , etc

Cholestasis

Hepatocelullar Cholestasis
-Idiopathic Neonatal Hepatitis -Viral Infection :CMV,HIV -Bacterial Infection:UTI,Sepsis,Syphilis -Genetic /Metabolic Disorders -TPN -Etc

DIAGNOSIS
Pemeriksaan Penunjang Rutin Darah tepi lengkap, gambaran darah tepi, urin rutin, tinja 3 porsi dan biokimia darah.

Kolestasis intrahepatis

Kolestasis ekstrahepatis

AST(SGOT)/ALT (SGPT) GGT Bilirubin serum

+++

+ +++

++++ ++

Etiology of cholestasis at CMH (2000-2003)

No 1 2 3 4 5 6 Etiology Extrahepatic biliary atresia UTI Sepsis CMV Alagille syndrome Others % 23 17 14 5,5 2 9,5

Strategy 1. Initial screening 2. Biliary atresia 3. Further investigation

1. Initial screening
Diagnosis:
UTI Sepsis Hypothyroid Panhypopituitarism

2. Biliary atresia

Pale Stools

Warrants urgent referral to exclude biliary atresiaSurgery <8 weeks leads to much better prognosis

GGT > 250

Liver US

Practical message

1
Age > 2 weeks

2
acholic stool

3
GGT
>250

non visulised GB

Dont be late to consult...

USG Patensi duktus (-) Biopsi hati

Bile duct paucity

Patensi duktus (+) Infeksi (-) Biopsi Hati Hepatitis neonatus

Draf Formulir Standard pelayanan Medis 2002 Unit Kerja Kelompok Gastrohepatologi IDAI

Infeksi (+) Medikamentosa

Atresia bilier Kolangiografi intraoperatif Operasi Kasai

ATRESIA BILIER
WHEN?
Dioperasi Pada Usia - 2 bulan 80% pasien bebas ikterus - 2-3 bulan 40% -50% pasien bebas ikterus - 3-4 bulan 25% pasien bebas ikterus - 4 bulan < 20% pasien bebas ikterus

ATRESIA BILIER
WHEN?
Tidak Operasi Sampai Usia - 1 tahun 50% -80% pasien meninggal - 3 tahun 90% -100% pasien meninggal

TERAPI
Operasi Portoenterostomi (Operasi Kasai) Operasi Transplantasi Hati

OPERASI KASAI

OPERASI KASAI
Faktor Prognosis - Usia pada saat operasi - Pola anatomis - Sindrom polisplenia - Pengalaman rumah sakit

FAKTOR PROGNOSIS
Usia Pada Saat Operasi - 2 bulan 80% pasien bebas ikterus - 2-3 bulan 40% -50% pasien bebas ikterus - 3-4 bulan 25% pasien bebas ikterus - 4 bulan < 20% pasien bebas ikterus

FAKTOR PROGNOSIS
Pola Anatomik Atresia: - Atresia of common bile duct only 100% - Complete extrahepatic atresia 65,4%

FAKTOR PROGNOSIS
Pengalaman Rumah Sakit - 2 pasien per tahun 54% - 3-5 pasien per tahun 59,8% - 20 pasien per tahun 77,8%

Choledochal cyst
-Type 1 Cystic or fusiform dilatation of choledochus (most frequent) -Type 2 Choledochus diverticulum -Type 3 Choledochocele -Type 4 Combination of intrahepatic and extrahepatic cysts -Type 5 Isolated intrahepatic duct cysts, single or multiple (Carolis disease)

Diagnosis
-Clinical features : 0-3 mo : biliary atresia > 1 yo : mass , pain , cholestasis -USG -MRCP

Management
-Emergency Setting : External Drainage -Elective Setting : Internal Drainage Cyst Excision
-Hepatico jejunostomy Roux en Y -Segmentectomy for IHBD dilatation

No Cyst Excision
-Cyst-Duodenostomy -Cyst-Jejunostomy Roux en Y

Liver Transplantation

Clinical manifestation Alagille Syndrome

PFIC
Progressive Familial Intrahepatic Cholestasis
PFIC1-ATP8B1

deficiency PFIC2- ABCB11 deficiency PFIC3-ABCB4 deficiency

Low/N GGT High GGT

Summary
Jaundice at the age > 2 weeks - check bilirubin fraction Cholestasis is always abnormal-need further examination Use the strategy for finding the Dx Use clues for searching the Dx

Terima Kasih

Clinical Clues
Clinical feature Family history, consanguinity, dysmorphic feature Bruising, petechiae or bleeding Hypoglycemia Suggesting Metabolic/inherited disease Vit K deficiency Secondary to metabolic disease Hypopituitarism Acute liver failure Intrauterine infection Inborn error of metabolism Advance liver disease Intrauterine infection Inborn error of metabolism

Splenomegaly

Ascites

Clinical Clues (2)

Clinical feature Dysmorphic Cardiac Murmur Sick Infant Micropenis Cataracts Situs Inversus Retinal probs Mass Cutan Haemangioma White Hair Bile Stained Hernia Suggesting Trisomies, Alagilles Alagilles, EHBA Sepsis, HLH,cong infec Panhypopituitarism Rubella, Galactosaemia EHBA TORCHS, Alagilles Choledochal Cyst Liver Haemangioma HLH Spont Perforation of Bile Duct

GAMBARAN KLINIS
Dua Bentuk Atresia Bilier 2. Tipe Perinatal (80%) Cholestasis perinatal Ductular remnant (+) Kelainan kongenital lain (-)

Classification of Choledochal cyst -Type 1 Cystic or fusiform dilatation of choledochus (most frequent) -Type 2 Choledochus diverticulum -Type 3 Choledochocele -Type 4 Combination of intrahepatic and extrahepatic cysts -Type 5 Isolated intrahepatic duct cysts, single or multiple (Carolis disease)

Classifi cation of Biliary Atresia Type Incidence (%) Description

1 5% Level of obstruction within the common bile duct. The gallbladder therefore contains bile. Typically these are cystic. The more proximal intrahepatic ducts are abnormal (scanty, irregular, and etiolated), sometimes with a hazy, cloud-like appearance rather than as actual ducts. 2 3% Level of obstruction within the common hepatic duct. The gallbladder will not contain bile but a transection of the proximal remnant should show two distinct bile-containing lumens 3 >90% Obstruction is within the porta hepatis with no visible bile-containing proximal lumen. The gallbladder may look normal but if so will only contain clear mucus. A communication with the duodenum may be shown on cholangiography. Fig. 55.2

Cholestasis?

Many infants with Cholestatic appear otherwise healthy and grow normally. Misleading physician into believing that is physiologic or caused by breast-feeding, when in fact it may be caused by biliary atresia.

ATRESIA BILIER
Kausa Ekstrahepatik
Biliary atresia Cholechochal cyst Spontaneous perforation of the common bile duct Choledocholithiasis Neonatal sclerosing cholangitis Bile duct stenosis,Compression by tumors or masses

FAKTOR PROGNOSIS
Sindrom Polispenia - Sindrom polispenia (+) 48,3% - Sindrom polispenia (-) 69,9%

RSCM
Ikterus, urin gelap, tinja akholik Hiperbilirubinemia terkonjugasi SGOT, SGPT, GGT, PT, alb/glob, kolesterol, trigliserida, asam empedu, TORCH, TSH, FT4. USG
Draf Formulir Standard pelayanan Medis 2002 Unit Kerja Kelompok Gastrohepatologi IDAI

Bilirubins Journey
Macrophage Heme Unconjugated Bilirubin (UCB) Conjugated Bilirubin (CB) Liver

Unconjugated Bilirubin (UCB)

Bile Duct

Intestinal Lumen

Urobilin/Stercobilin

Conjugated Bilirubin (CB)