Sistem bilier

Gross anatomy of the human biliary tract Where bile goes in the biliary tract

Schematic representation of gallbladder musculature

Enterohepatic circulation of bile salts

Gallbladder filling and bile flow during fasting

The mechanisms of gallbladder function

 Ductus biliaris intra hepatal
a. Ductus biliaris hepatal kiri →dari liver segmen II, III, IV
b. Ductus biliaris hepatal kanan → dari liver segmen V, IV, VII, VIII
- bagian anterior dari segmen V dan VIII
- Bagian posterior dari segmen VI dan VII
Ductus dari anterior dan posterior → duct biliari hepatal
kanan → atas dari V. porta cabang kanan
c. Ductus biliaris dari lobus caudatus
Mengalirkan ke duct bil hepatal kanan dan kiri

Ductus biliaris extra hepatal

- Duct hepaticus kanan dan kiri ke duct hepaticus comunis
- Ductus hepaticus comunis dan ductus cysticus ke duct bilaris
comunis
- Duct cysticus * panjang ± 1-2 cm
* Spiral fold (Valve of heister
mempertahankan patency)
- Duct biliary comunis → Lig hepatoduodenale ada 3 komponen:
1. Duct Bil Comm→ anterior kanan
2. Arteria hepatica →anterior kiri
3. V. porta →posterior
- Duct biliari comm melintasi bagian post dari duodenum superior ke
duodenum decendens ± 3 – 8 cm dari spinc pilori
- Sebelum memasuki duodenum decendens bersama dgn duct
pancreatic Wirsungi → papila of Vater

BILIARY TREE
Terdiri atas:
• Ductus Hepaticus D/S
• Ductus Hepaticus Communis
• Ductus Cysticus
• Ductus Choledochus
• Vesica Felea
DUCTUS HEPATICUS
• D. Hepaticus D & S à D. Hepaticus Comunis
• Panjang 4 cm
• Berjalan Turun dalam pinggir bebas omentum minus
• Bersatu dg D. Cysticus à D. Choledochus
DUCTUS CYSTICUS
• Panjang 4 cm Hubungkan collum VF & D. Hepaticus
Fisiologi
Comm.à D. Choledochus
• Sel hepatosit à Empedu 500-1500 ml/hari
DUCTUS CHOLEDOCHUS
• Di luar waktu makan à disimpan & mengalami pemekatan
• Panjang 8 cm
• Pengaliran cairan empedu:
• Perjalanan: didalam pinggir bebas omentum minus,
– Sekresi empedu oleh liver
didepan Foramen Epiploicum Winslow
– Kontraksi kandung empedu
• Depan Pinggir kanan V Porta
– Tahanan sphincter koledokus
• Belakang Bagian I Duodenum
• Dalam keadaan puasa à empedu dialirkan ke kandung
• Sebelah kanan A Gastroduodenalis
empedu
• Dlm alur permukaan posterior Caput Pancreas
• Setelah makan à kandung empedu kontraksi à sphincter
• Bersatu dg D. Pancreaticus Major
relaksasi à empedu mengalir ke duodenum
• Menembus dinding medial bag II Duodenum
• Ampula Vater à Spincter Odii & Papilla duodenum major
à Lumen Duodenum
DUCTUS BILIER
Anastomose Duct Chole- docus dg Pancreas • Relaksasi Spincter Odii
• Bergabung didalam duodenum  Sekresi Cholecystokinin
• Masuk ke Duodenum sendiri- sendiri  Kontraksi Ritmik Bladder
• Bergabung diluar Duodenum menjadi 1 ductus D. KANDUNGAN DAN FUNGSI EMPEDU
Spinchter Oddi melingkari duct. Choledochus pada papilla vateri • Kandungan Utama : garam empedu.
Komp Inorganik: Cl, HCO3,Na,K
VESICA FELLEA / KANDUNG EMPEDU Komp Organik: as. empedu, garam empedu, fosfolipid
• Buah Alpukat / Pear, panjang 6-10 cm (lechitin), Kolesterol, Bilirubin Conjugated
• Menempel pada Fossa Vesica Fellea di Lobus Quadratus • Asal: Kolesterol à Asam Cholic , As Keno dioksicholic à
Hepar. konjugasi dg glisin dan taurin
• Volume 30-40 cc • Fungsi: Metabolisme Lemak
• Permukaan lumen à Rugae / Crypte  Emulsifikasi partikel lemak
Bagian-bagian Vesica Felea:  Membentuk Micelus yg mudah larut àmembantu
• Fundus: Bulat menonjol di inferior hepar, setinggi costa IX absorbsi lemak dan vit A,D,E,K
kanan yg berpotongan dg Midclavicular Line , terutama E. SIRKULASI ENTEROHEPATAL
banyak otot polos • 94% garam empedu direabsorbsi di usus halus à difusi +
• Korpus : jaringan fibrotik (elastis) transport aktif
• Infundibulum = Hartman’s Pouch • Garam empedu rata-rata mengalami 18 kali sirkulasi
• Collum : melanjut ke duct cysticus sebelum dibuang ke faeces (+2.5 gram/hari)
Batas-batas
Anterior KOLESTASIS
• Dinding Abdomen &Permukaan visceral hepar di Lobus Mekanisme terjadinya:
Quadratus 1. Obstruksi Saluran Empedu (ekstrahepatal):
Posterior Neoplasma, Batu Ductus Choledochus, Stenosis Duct
• Colon Transversum & Bagian I & II duodenum Choledochus, Pancreatitis
Vascularisasi – Kolestasis sering total
• A. Cystica à a Hepatica Dextra (pada cystohepatic Triangle – Terapi : Bedah
of Callot) 2. Disfungsi / Kerusakan Hepatosit (Intrahepatal):
• V. Cystica à Cabang Dextra dari V Porta – Kolestasis tidak total
Innervasi : – Terapi : Non Bedah
• Parasimpatis ( n Vagus) à Pl. Coeliacus Akibat Kolestasis:
• Simpatis à dari T6-T8 Medula Spinalis • Empedu tidak ada dalam GIT à gangguan absorbsi lemak,
à via Ganglion Coeliacus plexus Coeliacus vit ADEK, ggn sintesa faktor II, VII dan X
• Afferen à T6-T8 Medula Spinalis • Bilirubin Direk kà ikterus, as empedu subkutis k à gatal.
Microscopis • Produksi bahan-bahan tertentu dlm sel hati k
Dinding terdiri dari 3 lapis • Histo PA à timbunan pigmen pd kanalikuli Hepar
• Mucosa • Pelebaran sal.empedu intra & ekstra hepatal à
 Epitel Columner Simpleks Hepatomegali
 Lamina Propia • Kolestasis à bakteri k à kolangitis
 Sinus RockItansky Aschoff Penyebab
• Muscularis • Keganasan :
 Otot Polos tipis, tidak teratur – Primer : Hepatoma
 Dibagian Luar à Lapisan perimuscular – Sekunder :Metastasis dari kolorektal, payudara dll
• Lapisan Serosa Peritonium • Infeksi :
FISIOLOGI SISTEM EMPEDU – Akut : Kolesistitis, kolangitis
A. PRODUKSI – Kronis : kolesistitis
Dibagi 2 bagian: • Batu :
• Sel Hepatosit (50%) à dirangsang as empedu – Kandung Empedu , Saluran empedu
 Isi: garam empedu, kolesterol, zat organik lain • Keganasan :
 Produksi dirangsang oleh asam empedu – Kandung empedu : adenokarsinoma
• Epitel Sekretorius Ductus + ductulus – Saluran empedu : kolangiokarsinoma
 Isi: Larutann encer ion Na dan Bicarbonat
 Produksi dirangsang Sekretin
Total Produksi 600-1200 ml / hari
B. PENYIMPANAN
• Produksi à dikosongkan ke duodenum / disimpan dlm
kandung Empedu
• Dalam kandung empedu dipekatkan 15 kali
• Volume maks kandung empedu: 30 – 60 cc
• Sekresi empedu selama 12 jam→sekitar 450 cc→dpt
disimpan→ air, Na+, Cl-, elektrolit lain diabsorbsi oleh
mukosa empedu (melalui transport aktif)→memekatkan
garam empedu, kolesterol, lesitin dan bilirubin
C. PENGOSONGAN KANDUNG EMPEDU
• Kontraksi Bladder
 Rangsangan N Vagus
 Sekresi Cholecystokinin
 • Peristaltik Usus

Causes of Unconjugated Hyperbilirubinemia Causes of Posthepatic Jaundice

 Increased RBC breakdown Upper-third obstruction
 Acute hemolysis  Polycystic liver disease,
 Chronic hemolytic disorders  Caroli disease
 Large hematoma resorption, multiple blood transfusions  Hepatocellular carcinoma
 Gilbert syndrome  Oriental cholangiohepatitis
 Decreased hepatic bilirubin conjugation  Hepatic arterial thrombosis (e.g., after liver transplantation
 Gilbert syndrome or chemotherapy)
 Crigler-Najjar syndrome types I and II  Hemobilia (e.g., after biliary manipulation)
 Familial unconjugated hyperbilirubinemia  Iatrogenic bile duct injury (e.g., after laparoscopic
Causes of Hepatic Jaundice cholecystectomy)
 Hepatitis: Viral, Autoimmune, Alcoholic  Cholangiocarcinoma (Klatskin tumor)
 Drugs and hormones  Sclerosing cholangitis
 Diseases of intrahepatic bile ducts Middle-third obstruction
 Liver infiltration and storage disorders  Cholangiocarcinoma,
 Systemic infections  Sclerosing cholangitis
 Total parenteral nutrition  Papillomas of the bile duct
 Postoperative intrahepatic cholestasis  Gallbladder cancer,
 Cholestasis of pregnancy  Choledochal cyst
 Benign recurrent intrahepatic cholestasis  Intrabiliary parasites
 Infantile cholestatic syndromes  Mirizzi syndrome
 Inherited metabolic defects  Extrinsic nodal compression (e.g., from breast cancer or
 No identifiable cause (idiopathic hepatic jaundice lymphoma)
 Iatrogenic bile duct injury (e.g., after open cholecystectomy)
 Cystic fibrosis
 Benign idiopathic bile duct stricture
Lower-third obstruction
 Cholangiocarcinoma
 Sclerosing cholangitis
 Papillomas of the bile duct
 Pancreatic tumors
 Ampullary tumors
 Chronic pancreatitis
 Sphincter of Oddi dysfunction
 Papillary stenosis
 Duodenal diverticula
 Penetrating duodenal ulcer
 Retroduodenal adenopathy (e.g., lymphoma, carcinoid)
Sistem Bilier (Lanjutan)
Cholelithiasis
Terbentuknya batu di saluran empedu  paling sering dalam kandung
empedu [gall bladder]
Di barat  20% pada dewasa dan lanjut usia
Bisa asimptomatis selama masa hidup pasien
Tiga jenis: batu kolesterol, pigmen/bilirubin dan campuran
Patofisiologi
Kondisi normal à ada keseimbangan antar kadar bile acids,
kolesterol dan phospholipids
Ketidakseimbangan 3 komponen tersebut à predisposisi
terbentuknya lithogenic bile yang akan diikuti pembentukan batu
empedu tipe kolesterol
Batu empedu tipe pigmen tersusun dari calcium bilirubinate dan
memiliki dua bentuk utama:
Hitam à hemolisis & penyakit liver
Coklat à infeksi traktus biliaris oleh bakteri
Faktor predisposisi
Ras Meksiko-AMerika, Indian-Amerika
Wanita : Pria = 2 : 1  4 “F” : fat forties fertile female
Wanita yang mengkonsumsi pil kontrasepsi yang mengandung
estrogen tinggi
Insiden penyakit meningkat seiring bertambahnya usia
Anamnesa/Subyektif
• Seringkali asimptomatis sepanjang hidup pasien
• Keluhan paling sering: nyeri intermitten di bawah ribcage
kanan, bisa menjalar ke punggung
• Mual, dengan atau tanpa muntah bisa muncul
• Beberapa makanan, terutama yang mengandung lemak
tinggi dapat memicu gejala
• Pasien dapat mengalami serangan nyeri abdomen akut à
billiary colic , feses acholic dan kencing seperti teh
Px Fisik/Obyektif
• Px fisik bisa normal
• Rasa tidak nyaman bisa dipicu dengan palpasi dalam pada
RUQ abdomen
• Murphy’s sign [+] à acute cholecystitis à bisa disertai
demam dan takikardi
• Jaundice [+] à jika terjadi obstruksi partial/komplit
• Komplikasi:
– Pancreatitis à nyeri abdomen lebih difus,
termasuk di epigastrium dan kuadran kiri atas
abdomen
– Severe hemorrhagic pancreatitis à 15% pasien à
mortalitas tinggi karena kegagalan sistem multiorgan
• Charcot triad à Nyeri pada RUQ abdomen, Demam,
Jaundice/icterus à berkaitan dengan obstruksi duktur
biliaris komunis dan cholangitis
Px Laboratorium
Pada cholelithiasis yang tidak mengalami komplikasi à normal
Lakukan px darah rutin à DL, elektrolit, liver enzyme, bilirubin
DL à leukositosis à acute cholecystitis à butuh terapi lebih
urgen
50% pasien cholelithiasis simptomatis à abnormal transaminase
PT/aPTT à bisa abnormal pada pasien yang mengalami jaundice
berat akibat disfungsi absorbsi vitamin K
ALP dan amylase serum à meningkat setiap serangan akut
Px Penunjang/Imaging
• Rontgen à 15% cholelithiasis à radioopaqe
• Ultrasound à
– paling sensitif dan spesifik
– deteksi batu empedu & pelebaran saluran empedu intra
maupun ekstrahepatik
– Melihat penebalan dinding empedu maupun adanya cairan
pericholecystic à tanda acute cholecystitis
 CT Scan à workup nyeri abdominal tanpa adanya tanda/gejala
terlokalisir/spesifik
Tx Medikamentosa
• Agen disolusi
– Ursodeoxycholate à efektif untuk batu kolesterol
yang kecil dan terletak dalam kandung empedu
– Tingkat rekurensi tinggi à 50-60%
• Extracorporeal shock-wave lithotripsy (ESWL)
– Menghancurkan batu empedu menjadi fragmen2 lebih
kecil à keluar spontan melalui duktus biliaris ke
duodenum
– Tidak direkomendasikan lagi untuk cholithiasis karena
tingginya tingkat kegagalan, morbiditas [jaundice,
pancreatitis], dan tingkat rekurensi
Tx Bedah
• Cholecystectomy merupakan tx untuk px cholelithiasis
simptomatis
• Tx cholelithiasis asimptomatis à kontroversial
– Beberapa ahli bedah à cholecystectomy karena
potensi munculnya cholecystitis & choledocholithiasis
– Cholelithiasis asimptomatis dapat
dipertimbangkan menjalani cholecystectomy berdasar
kriteria:
– Sedang menjalani pengobatan yang dapat
menimbulkan “masking-effect” gejala cholelithiasis
[kortikosteroid, analgesik, narkotik]
– Batu empedu dengan diameter > 2 cm
– Pasien dengan “porcelain gallbladder”
– Pasien yang mengalami sensory neuropathy pada
abdomen
– Pasien yang direncanakan untuk mendapat
transplan organ [selain liver]
Komplikasi berkaitan dengan perjalanan penyakit
• Migrasi batu ke ductus biliaris komunis (choledocholithiasis)
• Inflamasi akut dinding kandung empedu [cholecystitis]
• Gall bladder gangrene
• Gallstone ileus
• Pancreatitis
• Bilioenteric fistula
Komplikasi berkaitan dengan cholecystectomy
• Bleeding
• Infeksi
• Cedera pada ductus biliaris komunis
Prognosis
• Dubia et bonam
• Tingkat mortalitas/morbiditas 0.1 – 0.5%
IkTERUS
Kadar bilirubin dalam darah:
Normal: 0,3 - 0,6 mg%, subikterus: >1,5 mg%
Ikterus : >3 mg%
BILIRUBINEMI BILIRUBINURI UROBILINURI STERKOBILIN
Normal 0,3 s/d 0,6 % negatif 150 s/d 200 mg% per 24 jam
Direk N, Indirek
Ikterus Hemolitik meningkat Negatif 2+ / N
Direk N, Indirek
Okterus o.k disfungsi meningkat Negatif Positif N atau meningkat
Hepatosit Normal
Direk meningkat,
Hepatitis Indirek 2+ Normal/+ Menurun
Normal/meningkat Negatif
Direk meningkat,
Obstruksi Total Indirek N 2+ Negatif Negatif

POLA KELAINAN LFT

Penyakit Kolestasis Infiltrasi Campuran
Hepatoseluler
Prototip Viral Batu Ca Metastase Cerosis
Penyakit Hepatitis akut Duct Chol hepar Alkohol
Bilirubin 4 s/d 8 6 s/d 20 <4 (umumnya) 2 s/d 10
mg/dl
Alkali 1 s/d 2 x n 2 s/d 5 x n 2 s/d 4 x n 1 s/d 2 x n
Fosfatase
Transaminase 500 s/d 1.000 < 200 < 100 < 400
Unit/ml
Protrombin Meningkat Meningkat Normal Meningkat
Time (bila parah) Bila cronis Sedikit
Gambaran SGPT<SGOT Alk Phosp Jarang icterus
Diagnostik (Umumnya) tetap Meningkat parah
walau BR menurun
Peningkatan PT Peningkatan PT Pembesaran Peningkatan PT
tdk dpt dikoreksi dapat dikoreksi hepar/Irreguler tdk dpt dikoreksi
dgn Vit K dgn Vit K dgn Vit K

GAMBARAN PERBEDAAN LFT PADA KOLESTASIS B.R DIREK ANTARA KELAINAN HEPATO SELULER DAN OBSTRUKSI BATU

Hepato seluler Obstruksi Batu empedu

Bilirubin mg/dl 4 s/d 8 6 s/d 20
Alkali phosphatase 1 s/d 2 X N 2 s/d 5 x N
Transaminase 500 s/d 1.000 < 200
Unit/ml
Prothrombin Time Meningkat Meningkat
Koreksi Vit K Koreksi vit K berhasil
gagal
 Cholangitis
Penyebab utama: choledocholithiasis
• Penyebab lain:
– Intervensi/manipulasi traktus biliaris
– Keganasan hepatobiliar
 Mortalitas/morbiditas à tinggi jika tidak diterapi
à 13-88%

Anamnesa/Subjektif
Riwayat choledocholithiasis atau manipulasi pada traktus biliaris
disertai demam, nyeri RUQ, dan jaundice [Charcot triad]
– Demam à 95%
– Nyeri RUQ à 90%
– Jaundice à 80%

Px Laboratorium
• DL, Liver function test, dan kultur darah
• Hasil laboratorium yang umumnya ditemui:
– Leukositosis ,Hyperbilirubinemia Antimicrobial Therapy
– Peningkatan ALP, transaminase & kadar serum • All patients having acute cholangitis
amylase • As soon as the diagnosis suspected or established
– Kultur darah (+) pada 50% paien • Gr I à 2 or 3 days
– Multiple organisme diidentifikasi pada 60% pasien • Gr II and III à 5-7 days
à aerobic [E.Coli, Klebsiella, Enterococcus] & • Biliary penetration
anaerobic [Bacteroides fragilis]
• Px Pencitraan à USG abdomen Antibiotika Untuk acut cholagitis grade 1
Terapi Medikamentosa Cefaxolin, Cefmetazole, cefotiam, oxacepem,flomoxef,
• Antibiotik IV broad-spectrum ampicilin sulbactam
• Koreksi cairan dan elektrolit Antibiotike untuk grade 2 dan 3 (moderate dan severe)
• Obstruksi à tekanan biliar tinggi à mengganggu sekresi Pilihan I:
bilier dari antibiotik à dekompresi & drainage Ampicilliin/sulbactam, Cefoperazone, ceftriaxon, ceftazidime,
• Percutaneous transhepatic biliary drainage (PTBD) à cefepime, cefozopran,aztreonam
alternatif metode drainage bilier non bedah Salah satu diata ditambah metronidazole
Terapi Bedah Pilihan kedua
• Endoscopic biliary drainage dan dekompresi Cifrofloxacin,levofloxacin, pazufloxacin
• Surgical decompression à tepat untuk pasien yang gagal Satu diatas ditambah metronidazole
menjalani [atau ketika fasilitas tidak memadai] Meropenem, imipenem,, cilastin, doripenem
endoscopic/transhepatic drainage
Komplikasi Biliary Drainage
• Pyogenic liver abscess • Endoscopic
• Acute renal failure • Percutaneous Transhepatic (PTCD)
• Open Drainage
Diagnostic criteria (Final Version of Tokyo Guidelines • Internal (stent) or external (nasobiliary) (?)
A.Clinical context and manifestations Timing (?)
1. History of biliary disease • As soon as possible or within 24 hrs
2. Fever and/or chills à Cholecystectomy is indicated after the resolution of acute
3. Jaundice cholangitis
4. RUQ or upper abdominal pain
B. Laboratory data Prognosis
1. Evidence of inflammatory response • Death will occur unless early and appropriate biliary
2. Abnormal liver function test drainage is performed and systemic antibiotics are
C. Imaging Findings administered
1. Biliary dilatation, or evidence of an • Mortality varies 2,5%-65%
etiology (stricture, stone, stent, etc) • Cause of death : - MOF w/ irreversible shock
Suspected diagnosis : two or more items in A
Definite diagnosis :
1. Charcot’s Triad (2+3+4)
2. Two or more items in A + both items in B+C
Severity Assessment
• Mild (gr. I) : respond to initial medical tx/
• Moderate (gr. II) : doesn’t respond
• Severe (gr.III) : associated w/ the onset of dysfunction at
least in any one of the following :- cardiovascular system, -
nervous system, - respiratory system, - kidney, - liver, -
hematological system
 Acute Cholecystitis
Acute inflammatory disease of the gallbladder
Incidence Gall Bladder Drainage
• 3%-10% of all w/ abdominal pain Endoscopic (ENGBD)
• <50 yr = 6,3% Percutaneous Transhepatic (PTGBD, PTGBA) Then followed by
• >50 yr = 20,9% surgery (cholecystectomy)
Etiology
90%-95% caused by cholecystolithiasis Complications
5%-10% caused by acalculous cholecystitis Perforation of the gallbladder
Risk Factors Biliary peritonitis
• Cholelithiasis Pericholecystic abscess
• Drugs Billiary fistula
• 5F, Pregnancy Prognosis
• AIDS Mortality : 0-10%
Patophysiology Risk of death tends to be higher in :
Obstruction of the neck of gallbladder or cystic duct  increase - elderly, - diabetes, - post cholecystostomy
gallbladder pressure
Classification
- Edematous chelecystitis
- Necrotizing cholecystitis
- Suppurative cholecystitis
- Chronic cholecystitis

Diagnostic Criteria

A. Local signs of inflammation

1. Murphy’s sign
2. RUQ pain/mass/tenderness
B. Systemic signs of inflammation
1. Fever
2. Elevated CRP 3. Elevated WBC count
C. Imaging findings (USG, MRI, CT)

Severity Assessment
Mild (gr. I) : healthy patient, no organ dysfunction, mild inflammatory
changes in gallbladder
Moderate (gr. II) : associated w/
1. Elevated WBC count (>18000/mm)
2. Palpable tender mass in RUQ
3. Duration of complaint > 72 hrs
4. Marked local inflammations
Severe (gr. III) : associated w/ dysfunction of any one of the following
1. Cardiovascular
2. Neurological
3. Respiratory
4. Renal
5. Hepatic
6. Hematological

Antimikrobial
 Cholecystectomy

Indications for LC
Symptomatic cholelithiasis
Biliary colic
Acute cholecystitis
Gallstone pancreatitis
Asymptomatic cholelithiasis
Sickle cell disease
Total parenteral nutrition
Chronic immunosuppression
No immediate access to health care facilities (e.g., missionaries,
military personnel, peace corps workers, relief workers)
Incidental cholecystectomy for patients undergoing procedure
for other indications
Acalculous cholecystitis (biliary dyskinesia)
Gallbladder polyps >1 cm in diameter
Porcelain gallbladder

Contraindications to LC
Absolute
Unable to tolerate general anesthesia
Refractory coagulopathy
Suspicion of gallbladder carcinoma
Relative
Previous upper abdominal surgery
Cholangitis
Diffuse peritonitis
Cirrhosis and/or portal hypertension
Chronic obstructive pulmonary disease
Cholecystoenteric fistula
Morbid obesity
Pregnancy

Technique of OC. A. Gallbladder in situ with the cystic duct isolated

and the cystic artery ligated and divided. B. The gallbladder has been
taken down from the liver bed and a catheter placed in the cystic duct
for an intraoperative cholangiogram. C. Gallbladder completely
removed with the cystic duct stump and proximal stump of the cystic
artery remaining. D. The abdomen is closed with a closed-suction
drain placed through a separate stab incision
 Bile duct and GB cancer
Bile duct Cancer GB cancer
 Average age 60 years
 Ulcerative colitis is a common associated condition
 Subtypes: (1) periductal infiltrating, (2) papillary or intraductal,
and (3) mass forming-nodular
 Location: 85% extrahepatic
Risk Factors
Definite risk factors
Primary sclerosing cholangitis (1% per year)
Liver fluke infection (Opisthorchis viverrini)
Hepatolithiasis (10%)
Biliary malformation (10% choledochal cysts, Caroli's)
Thorotrast
Probable risk factors
Hepatitis C , Cirrhosis, Toxins (dioxin, polyvinyl chloride)
Biliary-enteric drainage procedures
Staging
Presentation
 T1: Tumor involving biliary confluence ± unilateral extension
to 2° biliary radicles
 T2 Tumor involving biliary confluence ± unilateral extension
to 2° biliary radicles AND Ipsilateral portal vein involvement ± AJCC staging
ipsilateral hepatic lobe atrophy
 T3 Tumor involving biliary confluence + bilateral extension
to 2° biliary radicles
 OR Unilateral extension to 2° biliary radicles with
contralateral portal vein involvement
 OR Unilateral extension to 2° biliary radicles with
contralateral hepatic lobe atrophy
 OR Main or bilateral portal venous involvement
Presentation Treatment
Obstructive jaundice, Cholangitis (10%), Palpable mass
Liver cirrhosis, Cachexia
Diagnosis
 Blood work
 CA19-9: Its sensitivity &specificity for detection of CCA in PSC
are 79% and 98%, respectively, at a cutoff value of 129 U/mL.
 Imaging (US, CT, MRI/MRCP, ERCP, PTC, EUS, PET/CT)
Treatment and prognosis
 Surgical resection
 Adjuvant and neoadjuvant treatments : Mayo Protocol
The average patient with adenocarcinoma of the bile duct survives
less than a year. The overall 5-year survival rate is 15%.
Following a thorough radical operation, 5-year survival is about
40%. Biliary cirrhosis
Surgery
 Local lymph node metastases (N1) are not an absolute
contraindication to surgical treatment, because they do not
significantly influence outcomes in hilar CCA
 Predominantly in the elderly
 Incidentally diagnosed at an early stage after cholecystectomy
for cholelithiasis (1%)
 Approximately 90% of patients have gallstones.
 The 20-year risk of developing cancer for patients with
gallstones is less than 0.5% for the overall population and 1.5%
for high-risk groups
Risk Factors
 Larger stones (3 cm) tenfold increased risk
The risk is higher in patients with symptomatic pts
 Polypoid lesions, particularly in polyps >10mm
 The calcified "porcelain" gallbladder (20%)
selective mucosal calcification (7%)
 Choledochal cysts have an increased risk of developing
cancer anywhere in the biliary tree, but the incidence is highest
in the gallbladder.
Other Risk Factors
 Anomalous pancreatobiliary duct junction
 Obesity and pregnancy
 Chronic inflammatory bowel disease
 Polyposis coli
 Mirizzi syndrome
 Bacterial and Salmonella infections
 Industrial exposure to carcinogens
 Familial tendency
Pathology
 Adenocarcinomas 90% . Squamous , adenosquamous, oat cell,
 Papillary (10%), nodular, and tubular
 Lymphatics are present in the subserosal layer only. Therefore
cancers invading but growing through the muscular layer have
minimal risk of nodal disease
 40% have distant metastasis at Dx
Abdominal discomfort, right upper quadrant pain, nausea, and
vomiting.
Jaundice, weight loss, anorexia, ascites, and mass
Blood work
Imaging (UD, CT, MRI/MRCP, ERCP, PTC, PET/CT)
Stage 0: Carcinoma in situ
Stage I: T1/2 N0 M0: invades lamina propria, muscle layer,
perimuscular connective tissue
Stage II: T3 N0/1 M0 T3: perforates the serosa and/or directly
invades the liver and/or one adjacent organ
Stage III: T4: invades any main vessel
Stage IV: M1: distant metastases, including metastases in lymph
nodes at the pancreatic body and tail
and prognosis
 Surgery (Laparascopic /Open)
 Adjuvant therapy
The 5-year survival rate of all patients is less than 5%, median
survival of 6 months.
T1 treated with cholecystectomy 90% 5-year survival
T2 lesions treated with an extended cholecystectomy and
lymphadenectomy is over 70%
Advanced but resectable gallbladder cancer are reported to have
5-year survival rates of 20 to 50%.
Choledochal Cysts

Classification of choledochal cysts. The five types of choledochal cysts,

according to Todani et al.
TOKYO GUIDE LINES 2013
Tokyo guideline
• First Guideline for acute cholangitis and acute cholecystitis
• 2007, 2013
• 2013, 11 articles

TERMINOLOGY, ETIOLOGY AND EPIDEMIOLOGY

Cholangitis
• Definition
– Morbid condition
– Acute infection and inflammation in the bile duct

• Pathophysiology
– Onset involve 2 factor
• Increase bacteria in bile duct
• ↑ intraductal pressure

• Historical
– 1887, Charcot use “hepatic fever”, Charcot’s triad
– 1959, Reynold and Dargan use “ Acute obstructive cholangitis”,
Reynold’s pentad
– Longmire’s classification (not use)
• Acute suppurative cholangitis ~ Charcot triad
• Acute obstructive suppurative cholangitis ~ Reynold’spentad

Cholecystitis
• Definition
– Acute inflammation disease of Gall bladder
• Pathophysiology
– Gall stone => most common cause
– Obstuction at GB neck or cystice duct
– Increase GB pressure
– => acute cholecystitis
– 2 factor determine progression
• Degree of ocstruction
• Duration of obstructions
TRAKEOESOFAGEAL FISTEL
Patofisiologi terjadinya Kategori Waterston
Esofagus dan trakea berkembang dari foregut. Pada umur embryo 4-6
minggu, kaudal foregut membentuk divertikulum ventral yang
A. berat badan lahir ≥ 2 ½ kg, KU baik
berkembang menjadi trakea. Lipatan trakeoesofageal longitudinal
B. 1. berat badan lahir 1,8-2 ½ kg, KU baik
akan menyatu membentuk septum yang membagi foregut menjadi
2. berat badan lahir ≥ 2 ½ kg, dengan pneumonia sedang atau
tabung laringotrakeal di sebelah ventral dan di sebelah dorsal menjadi
disertai kelainan congenital lain
esofagus. Gangguan pada pembentukan septum ini mengakibatkan
C. 1. berat badan lahir < 1,8 kg
pemisahan yang inkomplet sehingga akan menimbulkan atresia dan
C. 2. berat badan lahir ≥ 2 ½ kg dengan pneumonia berat dan
fistel.
kelainan congenital lain yang berat

Operasi
Thorakotomi posterolateral Dekstra Retropleural.

Postoperatif

 Dirawat di neonatal intensive care unit

 Cairan intravenus
 Antibiotik profilaksis dilanjutkan
 Nutrisi lewat transanatomotic nasogastric tube diberikan pada
hari ke 2-3 post op
 Oesophagogram dilakukan 7 hari post op, dan jika tidak
didapatkan leakage thorax drain dapat di lepas
Diagnostik  Oral feeding bisa dimulai setelah thorax drain dilepas
 Polihidramnion, pd USG antenatal: gas lambung (-), distended  Fisiotherapi napas dilakukan secara terus menerus dengan
esophageal pouch suction nasopharingeal
 Adanya gelembung busa putih halus yg banyak pada mulut dan
hidung, walau telah disuction atau hipersalivasi
 Riwayat nafas berbunyi dan disertai batuk dan tersedak dan
biru
 Saat makan tersedak, batuk, sesak, sianosis
 Jika ada TEF, mungkin dpt tjd abdominal distention
 VACTERL
 Pada pemasangan NGT, akan terhenti pada panjang ± 10 – 12 cm
 Pada pemeriksaan RÖ thorax mungkin tampak kompresi dan
deviasi trakea. Aspirasi pneumoni pada segmen posterior dari
lobus superior, NGT yang melingkar pada mediastinum ,
pemeriksaan dg kontras jarang dilakukan krn dpt menambah
resiko aspirasi pneumoniti

Management dan Treatment

Preoperatif

 Cegah aspirasi
o Head up
o Pasang NGT
o continuous suctionin
 Cairan intravena D10 1/5 NS
 O2 , jika respiratory failure maka diperlukan intubasi
 Gastrotomy cito jika terjadi distensi lambung akut
 Antibiotik broad spectrum (ampisilin + gentamisin) karena ada
resiko infeksi paru akb aspirasi
 Bayi ditempatkan pada boks bayi dengan penghangat (warmer)
 Cari kelainan congenital lainnya.
CHOLEDOCHAL CYST
Treatment.
PREOPERATIVE:
Patofisiologi terjadinya
Menurut teori Babbitt, Todani: OPERATIVE:
Anomali drainase duktus pankreatikobiliari. Duktus pankreatikus  Type I: complete excision, a Roux-en-Y hepaticojejunostomy is
masuk ke CBD dengan sudut yang abnormal dan lebih proksimal performed to restore biliary-enteric continuity.
terhadap ampula Vater. Kelainan ini disebut ‘long common channel’  Type II: the cyst is excised in its entirety. The resultant
yang berakibat tidak efektifnya sphincter Oddi dan akibatnya defect in the common bile duct is closed over a T-tube.
terjadi reflux enzym pancreas yang akan merusak epitel duktus dan
selanjutnya menyebabkan dilatasi duktus.  Type III (choledochocele): depends upon the size the cyst.
Ada empat type:
Type I – kiste pada CBD
 3 cm or less can be treated effectively with endoscopic
Type IA is saccular in configuration and involves either the
sphincterotomy.
entire extrahepatic bile duct or the majority of it.
Type IB is saccular and involves a limited segment of the bile
 larger than 3 cm typically produce some degree of
duct.
duodenal obstruction. These lesions are excised surgically
Type IC is more fusiform in configuration and involves most or
through a transduodenal approach. If the pancreatic duct
all of the extrahepatic bile duct
enters the choledochocele, it may have to be reimplanted
Type II – divertikel pada CBD
into the duodenum following excision of the cyst.
Type III – choledochele (kista pada distal CBD)
Type IV A – Multicystic pada ekstra hepatic
Type IV B – Multicystic pada intra dan ekstra hepatic bile ducts  Type IV: completely excised and a Roux-en-Y
Type V – Multicystic intrahepatic (Caroli’s disease) hepaticojejunostomy is performed to restore continuity.

 Type V (Caroli disease): hepatic lobectomy. Patients with

bilobar disease who begin to manifest signs of liver failure,
biliary cirrhosis, or portal hypertension may be candidates for
liver transplantation.

 Lilly technique: Occasionally, the cyst adheres densely to the

portal vein secondary to long-standing inflammatory reaction.
In this situation, a complete, full-thickness excision of the cyst
Tipe 1 tipe 2 tipe 3 may not be possible. Lilly devised a technique, in which the
serosal surface of the duct is left adhering to the portal vein
while the mucosa of the cyst wall is obliterated by curettage or
cautery. Theoretically, this removes the risk of malignant
transformation in that segment of duct.

PANCREAS ANULARE

Tipe 3 tipe 5 Patofisiologi terjadinya

Menurut teori Lecco’s, annulus berasal dari primordial pancreas
Diagnostik ventral. Duktus pankreatikus dari jaringan anular melintas dari
Klinis – trias klasik: ikterus, mass, pain bagian anterior ke lateral dan bagian posterior akhirnya bergabung
Lab – bilirubin direk dan alkali phosphatase meningkat dengan duktus pankreatikus utama.
USG Ada dua tipe:
1) Ekstramural – menyebabkan obstruksi GI tinggi
2) Intramural – mengakibatkan ulserasi duodenum

Diagnostik
Klinis – Polihydramnion, bile stain vomit, abdominal distended (r.
epigastrium), konstipasi, dehidrasi, jaundice
Radiologik – double bubble

Treatment
PREOPERATIF – dekompresi lambung dengan NGT dan suction
continuous, Terapi cairan dan nutrisi intravena
OPERASI – Eksplorasi laparotomi urgent, insisi transversal
supraumbilikal, duodenoduodenostomy
POSTOPERATIF – NGT dengan suction continuous, th/ cairan dan
nurtrisi intravena, bila volume retensi ngt berkurang dan tidak bile
staining, dpt dimulai dengan oral.
ANORECTAL MALFORMATION (Alberto Pena -1990-
Insiden The cause of anorectal malformation is unknown
 1 – 4 : 5.000 Neonatus The average incidence in the worldwide is about 1 in 5000 live births
 Wanita > Pria Some of that have a genetic predisposition
Anomali penyerta Associated with many syndromes : ec. Down syndrome, cat’s eye
 Vater – Vacterl syndrome
 The Cat Eye syndrome
One concept  well known :
Klasifikasi High lession  supralevator
 Pria Low lession  infralevator
– Cutaneus/perineal fistula
– Recto uretral fistula Early detection
– Bulbar  Anamnesis à defecation +/-
– Prostatic  Physical Examination :
– Recto vesical fistula Anal (+)
– Anorectal agenesis (-) fistula Anal ( - ) à fistula +/-
– Rectal atresia  Imaging / X- photo studies :
 Wanita 1. Indication
– Cutaneus/perineal fistula 2. Timing
– Vestibular fist 3. Photo à Invertogram
– Anorectal agenesis (-) fist à Knee Chest position
– Rectal atresia
– Persisten cloaca Problems :
Pemeriksaan penunjang  General :
→ Foto polos (sinar horisontal) knee chest position à dehidration
Pengelolaan à Hypothermia
→ Colostomy à Multiple Anomalies
→ PSA (Post resection sagital approach)  Spesific :
à Infection
à Lower intestinal obstruction
à Peritonitis
à Septic

Management :
Depend and according to the problems :
à General problems

à Spesific problems