Jaundicepada

Kasus Bedah Anak

Dr Sastiono Divisi Bedah Anak FKUI/RSCM

Bilirubin MetabolismAn Overview

Blood: Conjugated & Un-conjugated.

UrineUrobilinogen

Stool: Stercobilin

Conjugated Bilirubin (Direct bilirubin):

Bilirubin + Glucuronic acid.Soluble in H2O.

Unconjugated Bilirubin (Indirect bilirubin):

Bilirubin (No Glucuronic acid).Soluble in Alcohol.

2

Age > 2 weeks

Check total bilirubin & direk

Cholestasis

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

Total bilirubin Bilirubin direk Cholestasis ?

13.2 2.4

20.3 5.2

3.5 0.9

Exercise

Yes

No

No

Cholestasis

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

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 Etiology %

1 Extrahepatic biliary atresia 23

2 UTI 17

3 Sepsis 14

4 CMV 5,5

5 Alagille syndrome 2

6 Others 9,5

Strategy1. Initial screening2. Biliary atresia3. Further investigation

1. Initial screening

• Diagnosis:– UTI– Sepsis– Hypothyroid– Panhypopituitarism

2. Biliary atresia

Pale Stools

Warrants urgent referral to exclude biliary atresia-Surgery <8 weeks leads to much better prognosis

> 250GGT

Liver US

Practical message

Age > 2 weeks

acholic stool

Don’t be late to consult...

1 2GGT

3 4

>250 non visulised GB

USG

Patensi duktus (+)

Hepatitis neonatus

Biopsi Hati Medika-mentosa

Infeksi (-) Infeksi (+)

Patensi duktus (-)

Biopsi hati

Bile duct paucity

Atresia bilier

Kolangiografiintraoperatif

Operasi KasaiDraf Formulir Standard pelayanan

Medis 2002 Unit Kerja Kelompok Gastrohepatologi IDAI

Patensi duktus (-)

Biopsi hati

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 only100%

- 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 (Caroli’s 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 No Cyst Excision

-Hepatico jejunostomy Roux en Y

-Segmentectomy for IHBD dilatation

-Cyst-Duodenostomy-Cyst-JejunostomyRoux 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 feature SuggestingFamily history, consanguinity, dysmorphic feature

Metabolic/inherited disease

Bruising, petechiae or bleeding Vit K deficiency

Hypoglycemia Secondary to metabolic diseaseHypopituitarismAcute liver failure

Splenomegaly Intrauterine infectionInborn error of metabolismAdvance liver disease

Ascites Intrauterine infectionInborn error of metabolism

Clinical Clues

Clinical Clues (2)Clinical feature SuggestingDysmorphic Trisomies, AlagillesCardiac Murmur Alagilles, EHBASick Infant Sepsis, HLH,cong infecMicropenis PanhypopituitarismCataracts Rubella, GalactosaemiaSitus Inversus EHBARetinal probs TORCHS, AlagillesMass Choledochal CystCutan Haemangioma Liver HaemangiomaWhite Hair HLHBile Stained Hernia Spont Perforation of Bile Duct

GAMBARAN KLINIS

• Dua Bentuk Atresia Bilier2. Tipe Perinatal (80%)

Cholestasis perinatalDuctular 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 (Caroli’s disease)

Classifi cation of Biliary AtresiaType Incidence (%) Description1 ∼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 twodistinct bile-containing lumens3 >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 withthe duodenum may be shown on cholangiography.Fig. 55.2

Cholestasis?

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

ATRESIA BILIER

• Kausa EkstrahepatikBiliary atresiaCholechochal cystSpontaneous perforation of the common bile ductCholedocholithiasisNeonatal sclerosing cholangitisBile duct stenosis,Compression by tumors or masses

FAKTOR PROGNOSIS

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

Ikterus, urin gelap, tinja akholik

Hiperbilirubinemia terkonjugasi

USG

SGOT, SGPT, GGT, PT, alb/glob, kolesterol, trigliserida, asam empedu, TORCH, TSH, FT4.

RSCM

Draf Formulir Standard pelayanan Medis 2002 Unit Kerja

Kelompok Gastrohepatologi IDAI

Bilirubin’s Journey

Macrophage Heme

Unconjugated Bilirubin (UCB)

LiverUnconjugated Bilirubin (UCB)

Conjugated Bilirubin (CB)

Bile Duct

Intestinal Lumen Conjugated Bilirubin (CB)Urobilin/Stercobilin