Budd chiari

1. BUDD-CHIARI
SYNDROME
MODRATOR: DR. RAMAKANTH, ASSISTANT PROFESSOR
PRESENTER: DR. ANURADHA

2. BUDD-CHIARI SYNDROME
 Also known as Hepaticvenous outflow tract obstruction
 Which includes a group of conditions characterized by obstruction to the outflow of blood
from liver secondary to involvement of one or more Hepatic veins, IVC, Right Atrium
 Incidence: 2.4 cases per million
 In Asians males, membranous webs
 In western country female and children 30-40 years age group, thrombosis is the cause.

3. ETIOLOGY
 Idiopathic
 Congenital: IVC webs , hepatic vein webs
 Venous thrombosis secondary to
 Dehydrartion
 Sepesis
 Polycythemia rubra vera
 Antiphspholipid syndrome
 Sickle cell disease
 Paroxysmal nocturnal hemoglobinuria
 Injury/ inflammation secondary to
 Phlebitis
 Autoimmune disease
 Bechets disease
 Tumor invasion
 Leiomyosarcoma of IVC

4. PATHOLOGY
 Partial/ complete hepatic venous outflow obstruction
 Increased Sinusoidal pressure
 Portal HTN and Liver congestion
 Hypoxia and hepatocyte dysfunction
 Necrosis
 Hepatic fibrosis and Cirrhosis

6. CLINICAL FEATURES
 ACUTE: Abdominal pain, Acites, tenderness, liver failure
 CHRONIC: PHTN, Acites, Pedal edema, UGIB, Large nodular liver
 CLASSIFICATION:
 PRIMARY: Due to endoluminal venous thrombosis, webs, endophlebitis
 SECONDARY: Lesion out side venous system caused by Tumors Abcess and
Cysts

9. IMAGING
 USG Acute findings:
 Hepatomegaly
 Hypoechoic Acites with or without Caudate lobe Hypertrophy
 SUBACUTE OR CHRONIC:
 Liver atrophy or normal size
 Hetrogenous echogeneicity of liver
 Portal HTN
 Caudate lobe enlargement
 Spleenomegaly
 Ascites

12. COLOUR DOPPLER
 Absent or Uniphasic flow in hepatic vein or IVC
 Intrahepatic collaterals which are comma shape
 Portal HTN features- Hepatofugal flow
 Caudate lobe vein diameter >3mm
 Enlarged hepatic atery diameter
 Short segment narrowing of IVC
 Webs- appears as hyperreflective linear focci in IVC
 Regenerative nodules

18. CT FINDINGS
 ACUTE BDS:
 Caudate lobe hypertrophy.
 Early enhancement of caudate lobe and central liver.
 FLIP-FLOP Phenomenon
 EARLY- central prominent enhancement
peripheral weak enhancement
 DELAY- central washout
peripheral enhancement
 Hepatic vein obstruction.
 Thrombosis of IVC.
 Spider web appearance of intra hepatic collaterals.
 Acites
 Spleenomegaly

20. CECT image (axial) showing MIDLE AND RIGHT
HEPATIC VEIN OBSTRUCTION AND IVC partial
THROMBOSIS
CECT image (axial) showing SPIDER WEB
APPEARANCE OF INTRA HEPATIC COLLATERALS

21. SUBACUTE AND CHRONIC CT
FINDINGS
 CHRONIC:
 Patchy enhancement of liver
 Collaterals
 Intrahepatic and portogastric
 Hepatic artery enlargement
 Chronic thrombosis in hepatic vein/ IVC
 Acites
 Spleenomegaly
 Regenerative nodules, seen as multiple 0.5-4 cms nodules which appear
Hyperdense in arterial phase iso or hypodense in venous phase

25. MRI FINDINGS IN BDS
 T2- Heterogenous increased signal in peripheral portion due to edema
 T2GRE & T1C- shows absent flow in hepatic vein or IVC
 T1C ACUTE BDS- shows Flip- Flop
 T1C CHRONIC BDS- show variable subacute changes
 Regenerative nodules T1 hyper, T2 hyper or Iso
 Where as in HCC T1 Hypo, T2 Hyper

28. DSA
 Non filling of obstructed or thrombosed HV/IVC
 Collaterals seen as spiders web

30. SULPHAR COLLOID SCAN
 Increased uptake by caudate lobe with reduced uptake by the rest of the
liver appears as HOT SPOT SIGN

31. ROLE OF INTERVENSIONS
1. TIPS
2. Stent
3. Balloon angioplasty
4. Medical management
1. Ascites- diuretics/ paracentesis
2. GIB- Balloon/ tamponade
3. Heparin for variable venous obstruction.

32. BALOON THERPY WITH OR WITHOUT
STENTING
 Short length stenosis of HV gives immediate relief but high recurrence
 SURGICAL MANAGEMENT: Congestion and fibrosis on biopsy needs
surgical management
 Portocaval or mesocaval stenting which is successfully portal vein and IVC
pressure> 10 mm.
 Liver transplantation is advisable in fulminant hepatic failure and cirrhosis
and end stage liver disease


33. TIPS
 TRANSJUGULAR INTRAHEPTIC PORTOSYSTEMATIC SHUNT
 INDICATIONS
 Acute vertical bleeding when pharmacologic therapy and endoscopic sclerotherapy
have failed
 Recurrent variceal bleeding
 Ascites refractory to medical management inn patients that require frequent drainage or
do not tolerate repeated drainage
 Hepatic hydrothorax that cannot be adequately managed with salt restriction and
diuresis
 Portal hypertensive gastropathy
 Hepatorenal syndrome
 Lower gastrointestinal and stomal varices
 Malignant compression of hepatic or portal veins

35. POST TREATMENT IMAGING
 Clinical response- decreased ascites, decreased caudate lobe size, colour
flow along the length of the stent.
 Reversal of blood flow in portal vein seen as indirect.

36. POST TREATMENT IMAGING

37. CONCLUSION
 Bud-Chiari Syndrome is hepatic venus outflow obstruction.
 Imaging Findings: -Caudate lobe hypertrophy
 -Hepatic vein/ IVC obstruction
 - Flip-flop appearance
 - Spider web appearance
 Imaging plays a vital role in the diagnosis and in particular Contrast Enhanced
Computed Tomography (CECT) represents the first line image technique in these
patients.

38. THANK YOU