Bile Ducts and Anatomic Abnormalities Flashcards
(20 cards)
1
Q
Causes of biliary obstruction: intrahepatic vs extrahepatic
A
Intrahepatic:
- Primary liver disease
- Secondary
2
Q
Intrahepatic causes of biliary obstruction
A
- Primary liver disease (metabolic).
- Alagille syndrome
- PFIC
- Alpha-1-Antitrypsin - Secondary.
- Infection
- Endocrine (TSH/T4)
3
Q
Alagille Syndrome
A
3/7 criteria for diagnosis.
- Facial features (prominent eyes)
- Cardiac: TOF, PA, PS
- Posterior embryotoxon
- Skeletal anomalies (Butterfly vertebrae)
- Bile duct paucity
4
Q
Pruritis: common symptom in chronic cholestatic liver disease. Medication options
A
- Antihistamines (Atarax)
- Actigall
- Rifampin
- Zofran
- Cholestyramine
- Naltrexone
- Dronabinol
- Zoloft
5
Q
Pruritis Surgical treatment
A
- partial external biliary diversion (stoma and drain bile). effective but not pretty. can cause M&M due to electrolyte shifts
- internal ileal bypass (ileocolonic diversion). this changes enterohepatic biliary circulation, this is more cosmetically appealing, but not always effective.
6
Q
PSC
A
- A/W IBD (UC)
- Sxs: pruritis, CLD
- Dx: Liver bx/ERCP
- No specific therapy: Ursodiol
7
Q
Extrahepatic obstruction
A
- BILIARY ATRESIA
- Choledochal Cyst
- Tumor, LCH
- Gallstone
- Cholecystitis
8
Q
Biliary atresia: 2 forms
A
- Embryonic (fetal): ~20%
- Perinatal (Acquired) ~80%
9
Q
Embryonic BA
A
- 100% splenic anomalies (polysplenia, asplenia).
- Portal vein anomalies: preduodenal, absence of IVC, cavernous transformation.
- Situs inversus 50%
- Malrotation
- Cardiac anomalies 50%
10
Q
Diagnosis of Biliary Atresia:
A
- History: acholic stool, no infection, (rule out Fam Hx of A1AT).
- PEx: rule our Alagille syndrome.
- Labs: elevated conjugated bilirubin, GGT.
- Liver biopsy: Bile duct proliferation, Periportal edema, bile duct plugs
11
Q
Confirmation of diagnosis of BA
A
- Intraoperative cholangiogram -> Kasai portoenterostomy.
12
Q
Subtypes of BA
A
- Type 1: atresia of CBD
- Type 2a: atresia of CHD
- Type 2b: atresia of CBD, CHD, Cystic duct
- Type 3: atresia of CBD, CHD, cystic duct, hepatic ducts (most common >90%)
13
Q
Subtypes of BA
A
14
Q
Treatment of BA
A
- Roux-en-Y portoenterostomy (Kasai procedure)
15
Q
Post-op treatment of Kasai
A
- Await return of bowel function, advance diet, see if stools are green
- IV Abx to reduce risk of postoperative cholangitis (PO Bactrim for 6 months).
- Steroids for anti-inflammatory effect ?
- USDA to improve bile flow
- Discharge within 4-5 days
16
Q
Kasai complications
A
- Cholangitis: 50%, usually with 2 yeras of Kasai. Fever, jaundice, decreased pigment in stools. Draw blood cultures, start broad spectrum antibiotics.
- Abrupt cessation of active bile drainage: rule out cholangitis, may consider revision of Kasai.
- Cirrhosis/ESLD: even after successful Kasai, cirrhosis may progress. Liver transplant needed in over 2/3 of patients by age 20.
17
Q
Kasai outcomes
A
- 1/3 have sustained bile flow, clearance of jaundice, no long term sequelae
- 1/3 progressive liver dysfunction,
- 1/3 never drain bile after Kasai, progressive liver failure, salvage by early liver transplantation.
18
Q
Biliary reconstruction in liver transplant
A
- Duct-to-Duct (older patients, size matched grafts).
- Roux-en-Y (younger kids, or PSC driven liver transplants).
19
Q
Bile leak
A
- Anastomotic leak can occur early in post op course (peritonitis, drain output is yellow-green and bilirubin level is higher than serum bilirubin level). Also elevated serum bilirubin. Etiology: technical or hepatic artery thrombosis.
- Leak from cut surface of reduced-size graft.
- Management: Percutaneous drainage.
If cut surface leak, most self resolve with time. If anastomotic leak: reoperation vs ERCP/stent vs PTC
20
Q
A
