call girls in munirka DELHI 🔝 >༒9540349809 🔝 genuine Escort Service 🔝✔️✔️
Doppler ultrasound of the portal system - Pathological findings
1. Doppler of the portal system
Pathological findings
Samir Haffar M.D.
Assistant Professor of Gastroenterology
2. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
3. Causes of portal hypertension
Pre-sinusoidal Congenital hepatic fibrosis
Sarcoidosis
Schistosomiasis
Lymphoma
Hyperdynamic Arterio-portal fistula or malformation
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Intra-hepatic
Post-sinusoidal Cirrhosis
Causes Disease
Extra-hepatic Portal vein thrombosis or compression
most common cause
Supra-hepatic Budd-Chiari syndrome
Right heart insufficiency
4. Doppler US signs of PHT in cirrhosis
• P-S collaterals Highly sensitive & specific
• Portal vein Dilated PV
Decreased mean velocity (< 15 cm/sec)
To-and-fro flow /Hepatofugal flow
Increased pulsatility (VPI)
Arterio-portal fistula
• Hepatic vein Compression (Pseudo-portal flow)
• Hepatic artery Enlargement & tortuosity
Increased RI & PI
Harkanyi Z. Ultrasound Clin 2006 ; 1 : 443 – 455.
5. Porto-systemic collaterals
High sensitivity & specificity for PHT
• Tributary collaterals
“Drain normally into PS”
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Coronary vein (left gastric)
Short gastric veins
Branches of SMV & IMV
• Developed collaterals
“Developed or recanalized”
Recanalized umbilical vein
Spleno-renal collateral
Gastro-renal collateral
Spleno-retroperitoneal collateral
6. Common spontaneous porto-systemic collaterals
More than 20 P-S collaterals described
Patnquin1 H et al. Am J Roentgenol 1987 ; 149 : 71 – 76.
Most common: LGV – PUV – Spleno-renal – Gastro-renal
7. P-S collaterals / Coronary vein
Most prevalent (80-90%) – Most clinically important
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Sagittal view slightly superior
Tortuosity of CV as it extends
superiorly toward GE junction
Sagittal paramedial view
Flow in CV directed superiorly
& away from splenic vein
8. P-S collaterals / Gastroesophageal collateral
Gastroesophageal collateral veins close to diaphragm
McGahan J et al. Diagnostic ultrasound, Informa Healthcare, 2nd edition, 2008.
Longitudinal view of left liver lobe
9. Normal umbilical vein anatomy
UV communicates with umbilical segment of LPV
Travels down anterior abdominal wall toward umbilicus
Eventually drains into systemic system via inferior epigastric vein
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
10. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Hepatofugal flow within UV
Similar color Doppler viewLongitudinal US of LLL
Dilated umbilical vein (10 mm)
P-S collaterals / Recanalized umbilical vein
PUV observed only in hepatic or suprahepatic blockage
11. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
N Engl J Med 2005 ; 353 : e19.
Sagittal panoramic view
PUV traveling to periumbilical region
where it becomes tortuous
P-S collaterals / Recanalized umbilical vein
Caput medusae
12. Recanalized UV in ligamentum teres
UV ramifies into smaller PU collaterals when it proceeds inferiorly
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Sagittal color Doppler view US of LLL
P-S collaterals / Recanalized umbilical vein
13. Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Detectable flow within UV
Flow directed away from LPV
Indicating recanalization & PHT
Similar color Doppler viewLongitudinal US of LLL
UV extending from LPV
Diameter: 1.8 mm
P-S collaterals / Recanalized umbilical vein
14. Porto-systemic collaterals
• Coronary vein & umbilical vein are the easiest
& most productive to analyze
• Other collaterals detected sonographically
albeit with more difficulty in some cases
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
15. P-S collaterals / Spleno-renal collateral
Yamada M et al. Abdom Imaging 2006 ; 31:701 – 705.
Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005.
Transverse color Doppler US
Splenic vein feeding large
splenorenal collaterals
Flow direction from SV to LRV
Reversed or to-and-fro flow in SV
Schematic drawing
16. P-S collaterals / Spleno-renal collateral
Flow inversion in splenic vein
Flow inversion in SV increases dg of spleno-renal shunt
Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005
17. P-S collaterals / Short gastric veins
Sato T et al. J Gastroenterol 2002 ; 37 : 604 – 610.
Short gastric vein as inflowing vessel to gastric varices
18. P-S collaterals / Gastro-renal collateral
Yamada M et al. Abdom Imaging 2006 ; 31 : 701 – 705.
Maruyama H et al. Acad Radiol 2008 ; 15 : 1148 – 1154.
From cranial & dorsal side to
caudal & ventral side into LRV
Long-axis view of GRS
GRS LRV
From SV at confluence
coursing backward to join LRV
Schematic drawing
19. P-S collaterals / Lumbar & epigastric collaterals
Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005
Large collateral vein between LK & lower pole of spleen
shunting blood from splenic hilum to lumbar & epigastric veins
20. P-S collaterals / Superior mesenteric vein
Flow toward SMV in sup branch
Flow away from SMV in inf branch
Color Doppler view
2 mesenteric branches
of superior mesenteric vein
Semicoronal view of SMV
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
21. P-S collaterals / Inferior mesenteric vein
Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005.
Hepatofugal flow in IMV originating from PV confluence
22. P-S collaterals / IMV & rectal venous drainage
Wachsberg RH. Am J Roentgenol 2005 ; 184 : 481 – 486.
Peri-rectal varices
Transverse US through bladderLeft parasagittal CDUS
Hepatofugal flow in dilated IMV
23. P-S collaterals / Gallbladder varices
Harkanyi Z. Ultrasound Clin 2006 ; 1 : 443 – 455.
Sepentine area in wall of GB
Cystic vein to anterior abdominal wall or patent PV branches
Most commonly observed in PV thrombosis (30%)
24. Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005
P-S collaterals / Omental varices
Transverse view with linear transducer (7-MHz)
Omental varices just beneath abdominal wall
25. P-S collaterals / Spleno-retroperitoneal collateral
Prominent varices surrounding posterior aspect of spleen
Owen C et al. J Diag Med Sonography 2006 ; 22 : 317 – 328.
26. Cirrhosis & PHT / Diameter of portal vein
1 Weinreb J et al. Am J Roentgenol 1982 ; 139 : 497 – 499.
2 Goyal AK et al. J Ultrasound Med 1990 ; 9 : 45 – 48.
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Diameter: 16.9 mm
Sign of portal hypertension
Longitudinal view of MPV
Contoversy on normal PV diameter
Up to 13 mm in one study1
Up to 16 mm in another study2
Unusual large PV: good sign of PHT
Normal PV size: do not exclude PHT
27. Cirrhosis & PHT / Portal vein velocity
Low velocity: good indicator of PHT
Normal velocity: do not exclude PHT
Controversy on normal PV velocity
Difficult to rely on velocity for dg
Normal mean velocity: 15 – 18 cm/sec
Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
Shrunken liver & irregular margin
Vmax: 10 cm/s
Diagnosis of PHT
Triplex image of PV
28. Portal vein pseudoclot – Incorrect velocity
Cirrhotic patient with portal hypertension
Slower flow in portal vein
demonstrated
Velocity scale: 7 cm/s
Rubens DJ et al. Ultrasound Clin 2006 ; 1 : 79 – 109.
Velocity scale: 20 cm/s
Good flow in HA anteriorly
No flow in adjacent PV
29. Cirrhosis & PHT / Portal vein flow
Normal flow
Kok Th et al. Scand J Gastroenterol 1999 ; 34 (Suppl 230) : 82 – 88.
Reversed flow
Advanced PHT
SOS
Porto-systemic shunt
To and fro flow
Advanced PHT
Heart failure
Arterio-portal fistula
30. Cirrhosis & PHT / To-and-fro flow in PV
Cardiac cycle
Hepatopetal & hepatofugal with each heart beat
Seen before frank hepatofugal flow
Wachsberg RH et al. RadioGraphics 2002 ; 22 : 123 – 140.
Duplex US of LPV during suspended respiration
31. Cirrhosis & PHT / To-and-fro flow in PV
Respiratory cycle
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
On real-time US, these alterations corresponded to respiratory cycle
Transverse color Doppler US of left portal vein
Hepatopetal flow Hepatofugal flow
32. Cirrhosis & PHT / To-and-fro flow in PV
Compression
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Transverse CDUS of left portal vein
Hepatopetal flow Hepatofugal flow
33. Causes of to-and-fro flow
Exaggerated pulsatility
Minimum velocity below baseline
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
- Portal hypertension
- Tricuspid regurgitation
- Right heart failure
- Aerterio-portal vein fistula
34. Cirrhosis & PHT / Reversed flow of PV
Hepatopetal flow in HA & hepatofugal flow in PV
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Not pathognomonic feature of cirrhosis
Severe PHT – Rare
35. Hepatopetal flow in HA
Hepatofugal flow in PV
Color Doppler of peripheral liver
Arterial flow above baseline
Portal venous below baseline
Duplex Doppler of same area
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Cirrhosis & PHT / Reversed flow in PV branches
36. Cirrhosis & PHT / Reversed flow in PV branches
Mansour MA et al. Vascular Diagnosis. Elsevier-Saunders, Philadelphia, 1st edition, 2005.
Right anterior PV branch
Hepatofugal flow
Right posterior PV branch
Hepatopetal flow
38. Hepatofugal portal / TIPS
Right portal vein to right hepatic vein
Hwang HJ et al. J Clin Ultrasound 2009 ; 37 : 511 – 524.
Reversion of hepatofugal flow
Stent devoid of color signals
Malfunction of TIPS
1 week after TIPS
Hepatofugal flow in RPV
Vigorous color flow in stent
Immediately after TIPS
39. Arterio-portal fistula / High-flow hemangioma
Hwang HJ et al. J Clin Ultrasound 2009 ; 37 : 511 – 524.
65-year-old man with high-flow hemangioma in LLL
Hypoechoic nodule with intratumoral flow
Peritumoral hepatofugal flow in segmental PV
Hepatopetal flow in proximal PV
40. Arterio-portal fistula / Post-liver biopsy
Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
Vascular lesion between
HA & PV branches
Inverted flow in PV
Oblique CDUSOblique gray-scale US
Focal echogenic area
in region of biopsy
Spectral Doppler US
High-velocity flow
Low-resistance flow
Turbulent flow
42. Helical portal vein flow
Near bifurcation
• Normal subjects 2%
• Severe liver disease 20%
• TIPS
• Post-liver transplantation Donor PV > recipient PV
• Portal vein stenosis
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
43. Helical portal vein flow
If not properly recognized, it can produce
the mistaken impression of PV flow reversal
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
44. Helical portal vein flow
Mimic of hepatofugal flow
Wachsberg RH et al. RadioGraphics 2002 ; 22 : 123 – 140.
Hepatopetal flow within liver confirms that net flow is hepatopetal
45. Cirrhosis & PHT / Prominent hepatic artery
Enlarged HA with tortuous or „„corkscrew‟‟ appearance
Increased flow in HA to compensate decreased flow in PV
Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
46. Causes of enlargement of hepatic artery
• Cirrhosis
• Hepatic diseases associated with alcoholism
• Congenital hepatic fibrosis
• Vascular tumors
• Hereditary hemorrhagic telangiectasia
Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
47. Parallel channel sign
von Herbay A et al. J Clin Ultrasound 1999 ; 27 : 426 – 432.
Gray-scale US
IH parallel channel sign
Suspicious of dilated IHBD
Color & pulsed Doppler US
Flow in both intra-hepatic lumina
Portal vein & hepatic artery
Absence of dilated intra-hepatic bile duct
48. Parallel channel sign
von Herbay A et al. J Clin Ultrasound 1999 ; 27 : 426 – 432.
Gray-scale US
IH parallel channel sign
Suspicious of dilated IHBD
Color & pulsed Doppler US
Blood flow in anterior structure
No flow in posterior structure
Confirmation of dilated intra-hepatic bile duct
49. Cirrhosis & PHT / Changes of hepatic artery flow
Kok Th et al. Scand J Gastroenterol 1999 ; 34 (Suppl 230) : 82 – 88.
Decreased diastolic flow
ESLD
Reversed diastolic flow
ESLD
Normal flow
Normal in most
patients
50. Cirrhosis & PHT / Pulsatility index of HA
Cirrhotic patients vs controls – Correlation with HVPG
Schneider AW et al. J Hepatol 1999 ; 30 : 876 – 881.
PI: 0.85
20 controls
0.92 ± 0.1
PI: 1.22
50 cirrhotic patients
1.14 ± 0.18
P< 0.001
Directly correlated with HVPG
52. Damping index of HV waveform
Severe portal hypertension : HVPG > 12 mmHg
Kim MY et al. Liver International 2007 ; 27 : 1103 – 1110.
Minimum velocity of downward HV
Maximum velocity of downward HV
Damping index =
Normal value: < 0.6
Severe portal hypertension: ≥ 0.6
53. Damping index of HV waveform in cirrhosis
DI: 0.26
HVPG: 7 mmHg
DI: 0.72
HVPG: 15 mmHg
Kim MY et al. Liver International 2007 ; 27 : 1103 – 1110.
DI of 0.6: Sen 76%, Sp 82, & AUC 0.86 for severe PHT
54. Doppler in cirrhosis / PHT
Prognostic implications
• Collaterals PUV High bleeding risk in surgery
Reversed LGV High bleeding risk of EV
S-R shunt Low bleeding risk of EV
• Portal vein Low flow High risk of HE
Inversed flow CI for TIPS & porto-caval shunt
Congestion index High bleeding risk of EV
• Hepatic artery Increased PI ESLD
• Hepatic vein Monophasic ESLD
Increased DI Severe PHT (> 12 mmHg)
55. Transjugular Intrahepatic Portosystemic Shunt
TIPS
Highly effective for
– Reducing ascites
– Recurrent variceal hemorrhage
– Improving quality of life
High rate of stenosis or thrombosis
High rate of hepatic encephalopathy
57. Follow-up of TIPS by Doppler US
• 24 to 48 hours (baseline)
• 3 months
• 6 months
• 12 months
• Annually thereafter
Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70.
Real goal of surveillance
Detect stenosis before complete thrombosis
58. TIPS / Normal
Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70.
Stent within liver parenchyma
Hepatopetal flow in MPV
Hepatofugal flow in RPV
Color Doppler of TIPS Color & pulsed Doppler of TIPS
Monophasic pulsatile flow
Velocity: 106 cm/sec
59. TIPS / Mirror image artifact
If not recognized: migration into heart (emergency intervention)
If uncertainty persists: chest radiograph
Wachsberg RH. Ultrasound Quarterly 2003 ; 19 : 139 – 148.
Stent on either side of diaphragm
Mirror image artifact Variant of mirror image artifact
Stent above diaphragm
True TIPS visible by rotating probe
60. TIPS / migration
Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70.
Proximal portion migrated out of PV into parenchymal tract
This resulted in complete thrombosis of stent
Longitudinal view of TIPS
61. TIPS – Stenosis
Middleton WD et al. Ultrasound Quarterly 2003 ; 19 : 56 – 70.
Mid TIPS
Mean portal vein Right portal vein
Mid TIPS Distal TIPS
Vel 26 cm/sec
Aliasing 371 cm/sec 98 cm/sec
Hepatopetal flow
62. TIPS / occlusion
Ricci P et al. J Ultrasound 2007 ; 10 : 22 – 27.
Homogeneous hyperechoic intraluminal material
without any color flow within TIPS
63. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
64. Portal vein thrombosis
• Etiology Extra-hepatic: multiple causes
Cirrhosis ± HCC: complete – partial
Budd-Chiary syndrome: 15% – poor prognosis
• Sensibility Equal to CT – Power Doppler increase Sen
• False positive Very low portal flow
• Partial Gray scale better than color Doppler
• Indications Before hepatic surgery
Before porto-caval shunt
Before hepatic transplantation
66. Superior mesenteric vein thrombosis
Pancreatic cancer
Sagittal view of pancreas & SMV
Thrombosed
SMV
Mass in
Pancreatic neck
Shunt between SMV
& systemic venous return
http://www.sonographers.ca
67. Superior mesenteric vein thrombosis
Transverse image of SMA & SMV
http://www.ultrasoundcases.info
SMA
SMV
68. Intestinal infarction
Considered from presentation until resolution of pain
• Ascites
• Thinning of intestinal wall
• Lack of mucosal enhancement of thickened wall
• Development of multi-organ failure
Intestinal infarction is likely
Surgical exploration should be considered
69. Ultrasound in ischemic bowel
Thickening of small bowel wall
Loss of layering structure of wall
Chen MJ et al. J Med Ultrasound 2006 ; 14 : 79 – 85.
Thickening of small bowel wall
Bright flecks within the wall
70. Portal vein gas
Acute transmural mesenteric infarction
Tritou I et al. J Clin Ultrasound 2011 (in press).
Wiesner W et al. Radiology 2003 ; 226 : 635 – 650.
Intrahepatic PV gas in
periphery of both lobes
CECT scan
Tiny echogenic foci
in liver parenchyma
Gray-scale US
Vertical bidirectional
spikes on PV waveform
Duplex of MPV
71. Acute thrombosis of portal vein
Complete thrombosis
http://www.sites.tufts.edu
Echogenic material visualized within portal vein
Increased diameter of portal vein
72. Partial thrombosis of portal vein
Echogenic material occluding lumen of PV by ≈ 50%
Sacerdoti D et al. J Ultrasound 2007 ; 10 : 12 – 21.
73. Partial thrombosis of portal vein
Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
Black & white ultrasound
Partial echogenic thrombus
Color & pulsed Doppler
Complete filling of main PV
obscuring the clot
74. Non-malignant PV thrombosis in cirrhosis
Systematic review – Many unresolved issue
• Incidence 10 – 25%
• Pathophysiology Cirrhosis no longer hypocoagulable state
• Clinical findings Asymptomatic disease
Life-threatening condition
• Management Not addressed in any consensus publication
1st line treatment: warfarin or LMWH
2nd line treatment: thrombectomy, TIPS
Tsochatzis EA et al. Aliment Pharmacol Ther 2010; 31 : 366 – 374.
75. Diagnosis of malignant PV thrombosis
• Color Doppler US* PV > 23 mm in diameter
“AASLD” Arterial-like flow on Doppler
Increased serum α-FP
• FNA CT- or US-guided
• CEUS Contrast-Enhanced Ultrasound
* DeLeve L et al. AASLD practice guidelines: Vascular disorders of the liver.
Hepatology 2009 ; 49 : 1729 – 1764.
76. Portal vein thrombus in HCC
Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
FNA of portal vein thrombus confirmed HCC
Gray-scale US image
Thrombus in PV & its branches
Color Doppler image
Vascularity within thrombus
Low-resistance arterial waveform
77. Malignant PV thrombosis / CEUS
38 pts (15 benigns - 23 malignants) – Conclusive (37/38)
Dănilă M et al. Medical Ultrasonography 2011 ; 13 : 102 – 107.
Gray-scale US
Malignant PVT Arterial phase
Enhancement
Portal phase
Wash-out
Late phase
Wash-out
Contrast-Enhanced US
78. Portal vein pseudoclot – Augmentation
Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13.
Color Doppler US of main portal vein
At rest
No detectable flow
Compression of lower abdomen
Augmented portal venous flow
79. Portal vein pseudoclot – Incorrect angle
Velocity: 24 cm/sec
Wall filter: medium
Angle 90°
Velocity: 7 cm/sec
Wall filter: medium
Angle < 90°
Radiol Clin N Am 2006 ; 44 : 805 – 835.
80. Chronic portal vein thrombosis
Portal cavernoma
Parikh et al. Am J Med 2010 ; 123 : 111 – 119.
Hepatopetal collaterals around thrombosed portal vein
82. Tchelepi H et al. Ultrasound Clin 2007 ; 2 : 415 – 422.
Transverse color US of stomach
Multiple dilated gastric varices
P-S collaterals / Isolated gastric varices
Collaterals via short gastric veins
Isolated gastric varices
Hepatopetal flow in LGV
Splenic vein thrombosis
83. P-S collaterals / Transcapsular collaterals
Chronic PVT due to necrotizing pancreatitis or surgery
Seeger M et al. Radiology 2010 ; 257 : 568 – 578.
Transcapuslar collateral
from SB varices to PVs
Color Doppler image
Submucosal varices
in small-bowel loop
US image
Ectopic intestinal varices
& transcapsular collaterals
Schematic diagram
84. Portal cholangiopathy
• Definition Biliary & GB abnormalities in EHPVO
• Frequency 70 – 100% (symptomatic or not)
• Mechanism Mechanical extrinsic compression
Biliary ischemic injury
• Manifestation Majority asymptomatic
RUQ quadrant pain
Cholestasis & cholangitis
Secondary biliary cirrhosis
• Management Directed to symptomatic patients only
Besa C et al. Abdom Imaging 2011 (in press).
85. Portal cholangiopathy
Biliary & GB wall abnormalities in EHPVO
Gallbladder varices
producing wall thickening
Cavernoma of portal vein
Associated with dilated bile ducts
Besa C et al. Abdom Imaging 2011 (in press).
Cholangiographic images essential to confirm diagnosis
86. MRC in portal cholangiopathy
Besa C et al. Abdom Imaging 2011 (in press).
Multiple smooth strictures
due to extrinsic compression
Stenosis with marked dilatation
of proximal biliary tree
Differentiate from bile duct cancerDifferentiate from PSC
87. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
88. Budd-Chiari syndrome
Narayanan Menon KV et al. N Engl J Med 2004 ; 350 : 578 – 85.
Occlusion of a single hepatic vein usually clinically silent
Two or three hepatic veins can be occluded without clear symptoms
89. Diagnosis of BCS
AASLD practice guidelines*
• Doppler US Most effective & reliable diagnostic mean
Experienced examiner aware of dg suspicion
• MRI or CT Confirmatory study
Experienced Doppler examiner not available
• Liver biopsy Diagnosis not done by non-invasive imaging
• Venography When diagnosis remains uncertain
* DeLeve L et al. AASLD practice guidelines: Vascular disorders of the liver
Hepatology May 2009 ; 49 : 1729 – 1764.
90. Doppler US in BCS
Obstruction &/or collaterals of HV or IVC*
* DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764.
Obstructed HV Presence of solid endoluminal material
Hyperechoic cord replacing normal vein
Reversed flow in large hepatic vein
Dilatation of vein upstream to obstacle
HV collaterals Sipder web in vicinity of HV ostia
Subcapsular or HV to intercostal or HV veins
Caudate lobe hypertrophy with dilated veins
IVC Web – Thrombosis – Inversion of flow
91. BCS / Solid endoluminal material in HV
Solid endoluminal material in middle & left hepatic veins
Narrowing at distal end of middle hepatic vein as it joins IVC
Chaubal N et al. J Ultrasound Med 2006 ; 25 : 373 – 379.
Transverse subcostal image
92. BCS / Hyperechoic cord
Hepatic veins transformed to fibrotic cords
“Hepatic vein star”
Boozari B et al. J Hepatol 2008 ; 49 : 572 – 580.
93. BCS / Reversed flow in large HV
Inverted flow in right hepatic vein
Normal flow in middle hepatic vein
Right intercostal view
Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
94. BCS / Reversed flow in HV upstream to obstacle
Solid endo-luminal material in distal part of MHV
Reverse flow in proximal part of MHV
95. BCS / Sipder web in vicinity of HV ostia
Vilgrain V. Eur Radiol 2001 ; 11 : 1563 – 1577.
Segev D L. Liver Transpl 2007 ; 13 : 1285 – 1294.
Gray-scale US
Small interwoven veins near IVC
Hepatic venogram
Typical “spider web” pattern
96. BCS / Large subcapsular vein
Large tortuous subcapsular vein draining into IVC
Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
97. BCS / HV draining into another HV
Occluded RHV draining through collateral vessel into MHV
Flow away & toward transducer in same vessel
“Bicolored hepatic vein”
Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
98. BCS / Collateral from HV to caudate lobe vein
Brancatelli G et al. Am J Roentgenol 2007 ; 188 : W168 – W176.
Transverse Doppler US at level of caudate lobe
Lack of flow in distal portion of MHV
Collateral from MHV to caudate lobe vein
100. BCS / Caudate lobe hypertrophy
Erden A. Eur J Radiol 2007 ; 61 : 44 – 56.
Sagittal gray-scale US
Enlarged caudate lobe
Antero-posterior diameter: 7.6 cm
101. BCS / Dilated caudate lobe vein
75% of cases
Bargalló X et al. Am J Roentgenol 2003 ; 181 : 1641 – 1645.
Mildly dilated caudate vein
7 mm
Largely dilated caudate vein
21 mm
Caudate vein (≥ 3 mm) suggests diagnosis
Except for cardiac failure
102. BCS / Membranous obstruction of IVC
Kandpal H et al. RadioGraphics 2008 ; 28 : 669 – 689.
30-year-old woman, abdominal pain & distention of 3 y duration
Ostial HV narrowing
Multiple IH collaterals
Tapered IVC occlusion
at cavo-atrial junction
Reversed flow in IVC
Loss of cardiac pulsations
103. Budd-Chiari syndrome & liver hydatid disease
Retrospective study of 13 patients with HDL & BCS
Yilmaz C et al. Radiol Oncol 2009 ; 43 : 225 – 232.
Heterogeneous mass representing degenerated & collapsed membranes
Large subcapsular vein draining into suprahepatic IVC
104. BCS / IVC thrombosis
Behçet disease – Secondary BCS
Sagittal image of IVC distended with echogenic thrombus
Secondary BCS due to renal cell carcinoma
Rumack CM, Wilson SR, & Charboneau JW. Diagnostic Ultrasound.
Elsevier-Mosby, St. Louis, Missouri, USA, 3rd edition, 2005.
105. BCS / Thrombosis of PV
15% of patients – Poor prognosis
Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
Thrombosis of portal vein
Hepatofugal flow in right portal vein
Dilated hepatic artery
106. BCS / Benign regenerative nodules
Multiple (> 10) – Small ( < 4 cm) – Hypervascular
Vilgrain V et al. Radiology 1999 ; 210 : 443 – 450.
Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
Two iso- & hyperechoic nodules
surrounded by thin hypoechoic halo
Low resistance arterial waveform
with high velocity
107. Proposed diagnostic strategy for BCS
Valla DC. Gut 2008 ; 57 : 1469 – 1478.
DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764.
108. Doppler US in SOS
Non specific
• Main PV Decreased, to-and-fro, or reversed flow
• Hepatic artery Significant elevation of RI (> 0.80)
• Hepatic veins Normal direction – Monophasic flow
• IVC Patent with flow toward heart
McGahan J et al. Diagnostic ultrasound, Informa Healthcare, 2nd edition, 2008.
* Lassau N et al. Radiology 1997 ; 204 : 545 – 552.
• US findings Thickened GB wall – Ascites
109. Sinusoidal obstruction syndrome (SOS)
BMT for acute myelogenous leukemia
Desser TS et al. Am J Roentgenol 2003 ; 180 : 1583 – 1591.
Contrast-enhanced CT
Heterogeneous hepatic enhancement
Color & duplex US of HV
Monophasic flow in MHV
Imaging currently not diagnostic by itself
110. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
111. Respiratory variation of IVC
Berzigotti A & Piscaglia F. Ultraschall Med 2011 ; 32 : 548 – 571.
Dilated IVC diameter (> 20 mm)
Absence of normal respiratory variation of IVC diameter
Being similar in expiration & inspiration
Longitudinal scan of IVC
112. Tricuspid regurgitation
Type 1 (mild)
S wave < D wave
Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098.
Type 2 (moderate)
No flow in ventricular systole
Type 3 (severe)
Retrograde flow in ventr systole
113. Combined tricuspid regurgitation & right-sided HF
Biphasic waveform
Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098.
Retrograde A, S, & V waves
combined to form a single wave
Single antegrade wave
& single retrograde wave
114. Constrictive pericarditis
Retrograde pulse between D & A
Scheinfeld MH et al. RadioGraphics 2009 ; 29 : 2081 – 2098.
Typical of constrictive pericarditis
CT scanSchematic drawing Pulsed Doppler
115. Severe right heart failure
Increased pulsatility in portal vein
Dietrich CF. Ultrasound of the liver. EFSUMB – European Course Book
Cardiac dysfunction if Vmin reaches 0 or reversed
Correlate PV waveforms with HV waveforms
116. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
117. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935.
Hemangioma / CEUS
Baseline US Arterial phase
Peripheral enhancement Sustained enhancement
Portal vein phase
118. Focal nodular hyperplasia
Venturi A et al. J Ultrasound 2007 ; 10 : 116 – 127.
“spoke-wheel pattern”
Arteries radiating toward periphery
Color Doppler US Power Doppler US
119. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935.
Focal nodular hyperplasia / CEUS
Arterial phase
Hypervascular mass
Stellate vascular pattern
Equal to liver enhancement
Central nonenhancing scar
Portal vein phaseBaseline US
Hypoechoic mass in
caudate lobe
120. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935.
Hepato-cellular carcinoma / CEUS
Baseline US Arterial phase
More echogenic
Hypervascular mass
Less echogenic
“Washed out”
Portal phase
121. Brannigan M et al. RadioGraphics 2004 ; 24 : 921 – 935.
Hepatic metastasis / CEUS
Arterial phase US image
Less echogenic
Hypovascular masses
Metastatic colon cancer
Baseline conventional US
Very subtle lesion
122. Doppler of the portal system
Portal hypertension
Portal vein thrombosis
Budd-Chiari syndrome & SOS*
Suspicion of cardiac disease
Characterization of hepatic mass
Miscellaneous
* SOS: Sinusoidal Obstruction Syndrome
123. Miscellaneous
• Biliary atresia
• Portal vein aneurysm
• Vascular shunts Porto-systemic PV to HV
Arterio-portal HA to PV
Arterio-systemic HA to HV
Portal-to-portal PV to PV
Systemic-to-systemic HV to HV
• HHT Hereditary Hemorrhagic Telangiectasia
• TAPVR Total Anomalous Pulmonary Vein Return
* Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
124. US of biliary atresia
High-spatial resolution real-time US
• Typical GB absent or small (< 15 mm)
Triangular cord sign (EARPV > 4 mm)
Hepatic subcapsular flow
• Others IHBD or CBD not dilated
Associated choledochal cyst
Cyst in porta hepatis (10%)
Pre-duodenal portal vein
Interrupted IVC: does not join right atrium
Situs inversus
EARPV: Echogenic Anterior wall of Right Portal Vein
Lee MS et al. Radiology 2009 ; 252 : 282 – 289.
125. Biliary atresia
29 biliary atresia – 35 neonatal cholestasis – 19 controls
EARPV: Echogenic Anterior wall of Right Portal Vein
Lee MS et al. Radiology 2009 ; 252 : 282 – 289.
GB length
1.4 cm
Hepatic subcapsular flowPositive TC sign
EARPV: 4 mm
Sen: 62%
Sp: 100%
Sen: 100%
Sp: 86%
126. Portal vein aneurysm
Prevalence: 0.6 per 1 000 persons
• Sites Confluence – Main PV – Intra-hepatic PV
• Pathogenesis Congenital: Marfan – Cutis laxa
Acquired: CLD, PHT, pancreatitis, trauma
• Diagnosis Extra-hepatic: focal dilatation > 21 mm
Intra-hepatic: focal dilatation > 9 mm
• Manifestation Small: asymptomatic
Large: thrombosis, rupture, compression
• Treatment Follow-up – anticoagulation – PC treatment
Koc Z et al. Am J Roentgenol 2007 ; 189 : 1023 – 1030.
127. Portal vein aneurysm
Swart J et al. Ultrasound Clin 2007 ; 2 : 355 – 375.
Bidirectional flow
“yin yang sign”
Color Doppler US
Focal dilatation of PV
4 cm in diameter
Gray-scale US Duplex Doppler US
Portal venous type flow
Bidirectional flow
128. Thrombosed portal vein aneurysm
Santana P et al. J Ultrasound Med 2002 ; 21: 701 – 704.
Power Doppler USTransverse US of SP confluence
No flow within aneurysmGiant portal vein aneurysm
SV
129. Aneurysm of portal vein bifurcation
Koc Z et al. Am J Roentgenol 2007; 189 : 1023 – 1030.
Diameter of portal vein bifurcation 21 mm
Significantly larger than remaining segments of same vein
Turbulent flow
130. Porto-systemic shunt
Portal vein to hepatic vein
Tri- or biphasic wave in PV suggests diagnosis
Cirrhosis – Trauma – Aneurysm – Congenital – HHT
131. Porto-systemic shunt / Cirrhosis
Most frequent cause – Associated with HE
Direct communication between portal vein & hepatic vein
Power Doppler US
PV
HV
Lane MJ et al. Am J Roentgenol 2000 ; 174 : 125 – 131.
132. Congenital extrahepatic porto-systemic shunts
Abernethy malformation ( 1793)
Type I Absence of intra-hepatic portal veins
Ia SMV & SV drain separately into IVC
Ib SMV & SV form common trunk drained by IVC
Type II Hypoplastic intra-hepatic portal veins
Morgan G et al. J Pediatr Surg 1994 ; 29 : 1239 – 1241.
133. Schematic drawing of Abernethy malformation
Hu GH et al. World J Gastroenterol 2008 ; 14 : 5969 – 5979.
Type IIType I bType I a
134. Abernethy malformation type Ib
Konstasa AA et al. Eur J Radiol 2010 (in press).
Color Doppler US
Direct emptying of
portal vein into IVC
CECT
Direct emptying of
portal vein into IVC
Biopsy (portal space)
Presence of bile duct & HA
Absence of PV
135. Congenital intra-hepatic porto-systemic shunts
Park’s classification (1990)
Type 1 Single tubular connection between RPV & IVC
Type 2 Multiple peripheral shunts within one segment
Type 3 Porto-systemic shunt through PV aneurysm
Type 4 Multiple peripheral shunts diffusely in 2 lobes
Park JH et al. Am J Roentgenol 1990 ; 155 : 527 – 8.
Type 5 Persistent ductus venosus (considered 5th type)
136. Park classification – Type 1
Single tubular connection between RPV & IVC
Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
Large vascular channel connecting RPV & MHV
RHV
MHV
Channel
Oblique color Doppler US
137. Park classification – Type 2
Multiple peripheral shunts within one segment
De Gaetano AM et al. Abdom Imaging 2007 ; 32 : 463 – 469.
Gray-scale US
Multiple serpiginous vessels in segment 3
between LPV & LHV
Waveform within shunt
Continuous portal-like flow
Waveform of LHV
Turbulent high velocity flow
138. De Gaetano AM et al. Abdom Imaging 2007 ; 32 : 463 – 469.
Park classification – Type 3
Porto-systemic shunt through PV aneurysm
Normal
triphasic pattern
Waveform of HV branch
Phasic pattern
Transmission from heart
Waveform of PV branch
Aneurysm between
PV & HV branches
Color Doppler US
139. Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
Monophasic portal flow
Triphasic HV flow
Spectral Doppler of shuntIntercostal RLL
Portal branch opens
into peripheral HV
Transverse LLL
Portal branches open
into peripheral HV
Park classification – Type 4
Multiple peripheral shunts diffusely in both lobes
140. Patent ductus venosus
17 reported cases in children*
* Yoshimoto Y et al. J Pediatr Surg 2004 ; 39 : E1 – E5.
Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
Venous duct connecting left portal vein & IVC
Oblique color Doppler US
IVC
MPV
LPV
DV
141. Arterio-portal shunt
Hepatic artery to portal vein
Low resistance flow in HA & arterialization of PV
Trauma – Cirrhosis – Aneurysm – Congenital – HHT
142. Arterio-portal shunt / Aneurysm
Dilated splenic artery
14 mm
Dilated splenic vein
22 mm
Splenic hilum
SV aneurysm
143. Arterio-portal shunt / Aneurysm
Splenic artery in hilum
RI: 0.41
Intra-splenic artery
RI: 0.61
Splenic vein in hilum
Arterialisation
146. Arterio-systemic shunt / HHT
Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
Dilated hepatic artery branch & right hepatic vein
Shunt of hepatic artery branch to right hepatic vein
150. Systemic-to-systemic shunt / HHT
Oblique color Doppler US
Large communication between right & middle hepatic veins
RHV
MHV
Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
152. Curaçao diagnostic criteria for HHT
1. Epistaxis Spontaneous, recurrent nose bleeds
2. Telangiectases Multiple, at characteristic sites:
Lips, oral cavity, nose, fingers
3. VMs GI, pulmonary, hepatic, cerebral, spinal
4. Family history First degree relative with HHT
Definite diagnosis If three criteria are present
Possible diagnosis If two criteria are present
Unlikely If fewer than two criteria are present
Criteria
Shovlin CL et al. Am J Med Genet 2000 ; 91 : 66 – 7.
153. US Doppler findings in HHT
Prevalence of hepatic involvement: at least 33%
• HA Dilated main HA & its branches
High-velocity flow (aliased or turbulent)
• Vms Porto-systemic shunt
Arterio-portal shunt
Arterio-systemic shunt
Systemic-to-systemic shunt
Liver biopsy should be avoided (high risk of bleeding)
Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
154. HHT / Dilated main hepatic artery
Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645.
Dilated hepatic artery: 8.7 mm
Measured 2 cm distal to its origin from celiac trunk
155. Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
HHT / Dilated HA branches – Straight
Transverse US of left lobe
“double channel sign” Dilated hepatic artery branch
Power Doppler US
156. HHT / Dilated HA branches – Tortuous
Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
Power Doppler USGray-scale US
157. HHT / IH arterial hypervascularization
Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645.
Tortuous small arterial branches
Color Doppler USGray-scale US
No abnormality demonstrated
158. Slightly dilated HA: 6.2 mm
High PSV: 129 cm/sec
Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
HHT / High velocity flow in HA
Duplex US of hepatic artery
159. US criteria for hepatic involvement in HHT
Criteria
Major criteria Dilated common hepatic artery (> 7 mm)
Intrahepatic arterial hypervascularization
Caselitz M et al. Hepatology 2003 ; 37 : 1139 – 1146.
Diagnosis: two major criteria or one major with two minor
Minor criteria PSV of proper hepatic artery > 110 cm/s
RI of proper hepatic artery < 0.60
Vmax of portal vein > 25 cm/s
Tortuous course of extrahepatic hepatic artery
Facultative findings Dilated portal vein (> 13 mm)
Dilated liver veins (> 11 mm)
Hepatomegaly (> 15 cm in MCL)
Nodular liver margin
161. Total Anomalous Pulmonary Venous Return
TAPVR
Infradiaphragmatic TAPVR the least common type
Pulmonary veins to left HV or left PV by large common channel
Flow almost always inhibited by one or more stenoses at this channel
Gallego C et al. RadioGraphics 2004 ; 24 : 755 – 772.
162. Infra-diaphragmatic TAPVR
Newborn with cyanosis & respiratory distress
CPV: Common Pulmonary Vein
Gallego C et al. RadioGraphics 2004 ; 24 : 755 – 772.
Transverse US of liver
Stenosis of CPV just
before it enters LHV
A
LHV
Stenosis
CPV
Longitudinal midline US
Vessel coming from thorax
with flow as in aorta
CPV
A
Aliasing in stenotic
segment of CPV & LHV
Oblique image of liver
A
CPV
LHV
Most prevalent portal systemic collateral present in 80% to 90%of patients with portal hypertension.Most clinically important ofthe portal systemic collaterals because its presence implies an increased risk for variceal hemorrhage.
Determination of flow direction in splenic vein increases the diagnostic confidence of S-R shunt.
Hepatic artery and the portal vein have blood flowing in opposite directions.
Although the role of Doppler sonography has decreased in the evaluation of the hepatic lesions with recent advances in CT andMR imaging, it should be kept in mind that Doppler sonography has the advantage over CT and MRI of demonstrating the direction of the flow of the hepatic vasculature.
Acceleration resulting from focal compression by regenerative nodulesValue of 0.6 of DI showed a sensitivity of 75.9% and a specificity of 81.8% for the presence of severe portal hypertension (hepatic venous pressure gradient >12mmHg) (AUC = 0.860).
Absolute contraindications to TIPS:1- Severe hepatic encephalopathy and liver failure2- Chronic portal vein thrombosis, especially those with narrowed and fibrotic veins or cavernous transformation Experienced centers are often successful in placing a shunt in patients with acute or subacute thrombosis.3- Severe right-heart failure with elevated central venous pressureRelative contraindications to TIPS1- Polycystic liver disease2- Systemic hepatic infections3- Hypervascular liver tumorsTechnical success rate for placement of TIPS is greater than 90%.The procedural complication rate ranges from 10 to 16%.Mortality related to the TIPS procedure is usually less than 2%.Primary patency:1 year: 25 - 66%2 years: 5 - 42% 3 years: 21%4 years: 13%5 years 13%Radiologic revision of malfunctioning shunt usually successful, resulting in primary assisted patency rate of approximately1 year: 85%2 year: 61%3 years: 46%4 years: 42%5 years: 36%
Early stenosis would be missed if one waited until the stent velocity dropped to 50 to 60 cm/second.
Important unanswered questions in cirrhotic portal vein thrombosis:Does occurrence of PVT alter the natural history of cirrhosis and therefore should asymptomatic patients be treated withthe goal of recanalization or prevention of further thrombus extension?Should all patients with cirrhosis and PVT be aggressively anticoagulated?Should this apply only to patients on transplantation waiting list?If recanalization does not occur should patients be offered second-line treatment with transjugularintrahepaticportosystemic shunts?How long should the interval be whilst being anticoagulated before considering therapy to have failed?How should patients be monitored?Is oral warfarin better than low-molecular weight heparin?
Consider only direct visualization of obstruction, and/or collaterals, of a hepatic vein or inferior vena cava, as definite evidence for the diagnosis.
Term “spiderweb” was initially used in description of angiographic findings in Budd-Chiari syndrome, and it means presence of very small interwoven veins.Interwoven: منسوجة
Hypertrophied subcapsular veins may shunt blood from liver to systemic veins (azygos vein, intercostal veins) or directly to inferior vena cava creating new intrahepatic and extrahepatic circulation.
“Bicolored” hepatic vein
HCC appears to be a significant long-term complication (11 of 97 patients followed-up for a mean of 5 years).αFP more specific for dg than with other liver diseases. Risk of HCC in long-standing IVC obstruction 70-fold higher than those with pure hepatic vein involvement.
HVOD frequently develops before day 20 of bone marrow transplantation.clinically suspected when there is jaundice, painful hepatomegaly, and fluid retention. Intensive myeloablativechemotherapy or radiation therapy before transplantation is presumed to be the cause.Clinical criteria for diagnosis: Seattle Criteria & Baltimore Criteria (weight gain – ascites – hepatomegaly - hyperbilirubinemia). The diagnosis may be supported by imaging, which will demonstrate the presence of hepatomegaly and ascites and rule out biliary obstruction due to benign or malignant causes, but imaging is currently not diagnostic by itself. The best-studied modality is gray-scale and color Doppler ultrasonography. The majority of studies suggest that no single ultrasound parameter is diagnostic for SOS.Findings that are highly suggestive of SOS are reversal of portal venous flow, attenuation of hepatic venous flow, gallbladder wall edema, and perhaps increased resistive indices to hepatic artery flow. A composite score of gray-scale and color Doppler ultrasound criteria has been proposed, but may be too cumbersome for routineclinical use. One study has suggested that the presence of flow in the para-umbilical vein is more common in moderate and severe SOS, but this will need to be validated by other investigators.
spoke-wheel:
Infradiaphragmatic total anomalous pulmonary venous return (TAPVR)
All or part of EHBT atretic – 1 in 15 000 live birthsMoyer et al introduced general guidelines for evaluation of cholestatic jaundice in infants.High-spatial resolution real-time US serves as a first-line screening tool with which to determine the cause of jaundice. Presence of triangular cord (TC) sign and an abnormal gallbladder (GB) on high-spatial resolution real-time US images is widely accepted as the diagnostic criterion for BA.Length of the GB on longitudinal scanning: GB length of at least 1.5 cm considered normal.Thickness of EARPV just proximal to RPV bifurcation site: triangular cord sign is EARPV thicker than 4 mm on longitudinal US images.
Causes of porto-systemic shuntCirrhosis Most frequent – Associated with HETrauma Blunt or penetratingPV aneurysm Rupture into hepatic veinCongenitalExtrahepatic “Abernethy malformation”Intrahepatic “Park classification” HHT Hereditary Hemorrhagic Telangiectasia
Medical literature contains approximately 100 published cases of congenital portosystemic shunts. First study conducted by Abernethy in 1793.Morgan and Superina classified extrahepaticportosystemic shunts into two types (1994).
The first type is the most common. Blood flow volumes are measured by multiplying the lumen area by the mean velocity at a given point. Portovenous shunt ratio is calculated by dividing the total blood flow volume in the shunt by that in the portal vein. Although the presence of a portosystemic shunt is considered abnormal in all cases, it has been demonstrated that shunt ratios of less than 24%–30% do not cause liver encephalopathy, even in cirrhotic patients.Portosystemic venous shunting causes hypergalactosemia, which, if it persists over a long period of time, leads to cataract formation.Portosystemic encephalopathy usually develops in adults.The natural history of portosystemic shunting depends on shunt ratio and patient age. Spontaneous closure is expected to occur in the first 2 years of life when an intrahepaticportosystemic shunt is found; close follow-up is recommended.In patients of all ages, shunt ratios above 60% should be corrected due to the risk of encephalopathy and liver dysfunction.Both embolization and surgical correction of the shunt have been described. When these measures fail, liver transplantation is the only therapeutic option.
DV is embryonic vascular structure that connects umbilical vein to IVC bypassing liver during the fetal life & closes immediately after birth.Functional closure is completed within the first few minutes of life, but true obliteration may not be complete for 15 to 20 dayMeasurement of postprandial serum galactose and ammonia concentrations can be used as screening tests in infants suspected of having a portosystemic shunt through the ductusvenosus.16 cases of PDV in children, excluding of neonates, have been reported. Most children were Japanese.The initial presentation included hypergalactosemia, liver dysfunction, encepalopathy, hypoxia, and tachypnea.Ultrasonography and angiography are useful in diagnosing PDV.
Causes of arterio-portal shuntTrauma Blunt or penetratingIatrogenic Liver biopsy – PTC Cirrhosis With or without HCCFocal liver lesion Benign or malignantAneurysm Rupture of arterial or venous aneurysmCongenital HHT – Ehlers-Danlos – Biliaryatresia
Causes of arterio-systemic shuntHepato-cellular carcinomaLarge hemangiomaHereditary Hemorrhagic Telangiectasia (HHT)
Causes of sytsemic-to-systemic shunt:BCSHHT
Causes of enlargement of hepatic artery:1- Cirrhosis2- Hepatic diseases associated with alcoholism3- Vascular tumors4- Hereditary hemorrhagic telangiectasia