jaundice pada kasus bedah anak.pdf
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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
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