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Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Interventional Case Record
Interventional Case Record
Trans-collateral loop technique for retrograde recanalization of occluded hepatic vein in a patient with Budd-Chiari syndrome.
Trans-collateral loop technique for retrograde recanalization of occluded hepatic vein in a patient with Budd-Chiari syndrome.
Contributed by : Maurya K. Patel (medical student)
Contributed by : Maurya K. Patel (medical student)
George Budd, a British internist, described three cases of hepatic vein thrombosis due to abscess-induced phlebitis in 1845, and Hans Chiari, an Austrian pathologist, added the first pathologic description of a liver with “obliterating endophlebitis of the hepatic veins” in 1899.
Introduction:
Introduction:
Budd Chiari syndrome is characterized by hepatic venous outflow obstruction presenting with right upper quadrant pain, ascites, jaundice, and pedal edema. It may lead to fulminant hepatic failure in a small minority of patients. The management includes anticoagulation, TIPS, endovascular stenting , and rarely surgical decompression. We present a case of Budd chiari syndrome which was managed by endovascular retrograde canalization of the obstructed segment with balloon dilatation and stenting.
Budd Chiari syndrome is characterized by hepatic venous outflow obstruction presenting with right upper quadrant pain, ascites, jaundice, and pedal edema. It may lead to fulminant hepatic failure in a small minority of patients. The management includes anticoagulation, TIPS, endovascular stenting , and rarely surgical decompression. We present a case of Budd chiari syndrome which was managed by endovascular retrograde canalization of the obstructed segment with balloon dilatation and stenting.
Case Presentation:
Case Presentation:
A 35 year old man presented to our center with a six month history of progressive exercise intolerance, abdominal distension and right upper quadrant pain in the abdomen. The patient had slight weight loss and sleep disturbances due to the distended abdomen. There was no history of melena, clay-coloured stools, drug intake or fever.
A 35 year old man presented to our center with a six month history of progressive exercise intolerance, abdominal distension and right upper quadrant pain in the abdomen. The patient had slight weight loss and sleep disturbances due to the distended abdomen. There was no history of melena, clay-coloured stools, drug intake or fever.
On examination, the abdomen was tense and distended. The patient was afebrile . Blood analysis revealed increased liver enzymes, with predominant hepatocellular changes (aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were 140 U/L and 96 U/L respectively). Bilirubin levels were also increased (total bilirubin of 4.0 mg/dL, with a direct level of 2.53 mg/dL). INR levels were 2.6. Abdominal ultrasound showed moderate free fluid in the abdomen, increased liver size to 16 cm and altered liver echotexture . Abdominal CT revealed moderate ascites, homogenous peripheral liver enhancement and delayed attenuation,homogenous caudate lobe enhancement and hypertrophy,and non-opacified hepatic veins suggestive of occlusion. A possible diagnosis of chronic Budd-Chiari syndrome was made and the patient further evaluated.
On examination, the abdomen was tense and distended. The patient was afebrile . Blood analysis revealed increased liver enzymes, with predominant hepatocellular changes (aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were 140 U/L and 96 U/L respectively). Bilirubin levels were also increased (total bilirubin of 4.0 mg/dL, with a direct level of 2.53 mg/dL). INR levels were 2.6. Abdominal ultrasound showed moderate free fluid in the abdomen, increased liver size to 16 cm and altered liver echotexture . Abdominal CT revealed moderate ascites, homogenous peripheral liver enhancement and delayed attenuation,homogenous caudate lobe enhancement and hypertrophy,and non-opacified hepatic veins suggestive of occlusion. A possible diagnosis of chronic Budd-Chiari syndrome was made and the patient further evaluated.
Doppler ultrasound revealed long segment occlusion of the left hepatic vein at the ostium, complete thrombosis of the middle hepatic vein at the ostium and a short segment occlusion of the right hepatic vein at the ostium. An MR venogram confirmed the above findings.
Doppler ultrasound revealed long segment occlusion of the left hepatic vein at the ostium, complete thrombosis of the middle hepatic vein at the ostium and a short segment occlusion of the right hepatic vein at the ostium. An MR venogram confirmed the above findings.
Interventional technique:
Interventional technique:
Based on the above findings a transjugular approach was established and a guide wire was passed into the IVC. We tried to probe at the ostia of the hepatic veins but were unable to cannulate the main hepatic veins because of the occlusion. A repeat MR venogram showed a large collateral vein draining the right hepatic vein that was then accessed through a transjugular route. [Figure 1].
Based on the above findings a transjugular approach was established and a guide wire was passed into the IVC. We tried to probe at the ostia of the hepatic veins but were unable to cannulate the main hepatic veins because of the occlusion. A repeat MR venogram showed a large collateral vein draining the right hepatic vein that was then accessed through a transjugular route. [Figure 1].
Fig. 1
A guide wire was then positioned in the collateral vein. Following initial balloon dilatation, the wire was passed retrogradely through the right hepatic venous occlusion and kept free floating in the right atrium. [Figure 2a] A second internal jugular vein approach was established and another wire was passed with a Snare to pull the free wire in the right atrium and thus a loop with proper tension was established[Figure 2b].
A guide wire was then positioned in the collateral vein. Following initial balloon dilatation, the wire was passed retrogradely through the right hepatic venous occlusion and kept free floating in the right atrium. [Figure 2a] A second internal jugular vein approach was established and another wire was passed with a Snare to pull the free wire in the right atrium and thus a loop with proper tension was established[Figure 2b].
Fig. 2a
Fig. 2b
Figure 3 (below) shows the photograph of the neck with 5F and 6F sheaths. A balloon catheter was passed through the occlusion [Figure 4] and inflated at the site. A stent was then deployed,
Figure 3 (below) shows the photograph of the neck with 5F and 6F sheaths. A balloon catheter was passed through the occlusion [Figure 4] and inflated at the site. A stent was then deployed,
Fig. 3
Fig. 4
Post-Operative angiogram [Figure 5] revealed adequate flow through the stent. On 7th day follow up the patient had improved clinically and ascites had resolved. A repeat angiogram at three months post procedure had shown good retrograde flow through the stent.
Post-Operative angiogram [Figure 5] revealed adequate flow through the stent. On 7th day follow up the patient had improved clinically and ascites had resolved. A repeat angiogram at three months post procedure had shown good retrograde flow through the stent.
Fig. 5
Discussion:
Discussion:
Budd Chiari syndrome is an uncommon disorder characterized by hepatic venous outflow tract obstruction along the venous course from hepatic venules upto the junction of inferior vena cava and the right atrium. Obstructions due to pericardial disease, cardiac causes and sinusoidal obstruction syndrome are not included in this syndrome.[1]. There is a known etiological factor in 80% of the cases and 20% are idiopathic.The state of hypercoagulability is the major risk factor for developing Budd-Chiari syndrome. The most important causes are myeloproliferative disease like polycythemia vera and essential thrombocythemia , malignancy (by direct compression or vascular invasion), liver lesions (hepatic cysts, cystadenoma, invasive aspergillosis etc. due to their space occupying nature), oral contraceptives and pregnancy etc.[2] On the basis of duration, the disease may be classified as fulminant, sub-acute, acute, or chronic. The subacute form is the most common and has an insidious onset as in our case. Only one in three patients with the subacute variant have obstruction of all the three hepatic veins. [3]
Budd Chiari syndrome is an uncommon disorder characterized by hepatic venous outflow tract obstruction along the venous course from hepatic venules upto the junction of inferior vena cava and the right atrium. Obstructions due to pericardial disease, cardiac causes and sinusoidal obstruction syndrome are not included in this syndrome.[1]. There is a known etiological factor in 80% of the cases and 20% are idiopathic.The state of hypercoagulability is the major risk factor for developing Budd-Chiari syndrome. The most important causes are myeloproliferative disease like polycythemia vera and essential thrombocythemia , malignancy (by direct compression or vascular invasion), liver lesions (hepatic cysts, cystadenoma, invasive aspergillosis etc. due to their space occupying nature), oral contraceptives and pregnancy etc.[2] On the basis of duration, the disease may be classified as fulminant, sub-acute, acute, or chronic. The subacute form is the most common and has an insidious onset as in our case. Only one in three patients with the subacute variant have obstruction of all the three hepatic veins. [3]
Right upper quadrant pain, ascites, jaundice, pedal edema, breathlessness on exertion, etc. are the most common presenting features of patients with Budd Chiari syndrome. Diagnosis mainly relies on sonography, Computed Tomography (CT), and other vascular imaging modalities like color Doppler, MR venogram are also useds. The treatment options include medical management with anticoagulants (low molecular weight heparin and warfarin) and treating the underlying cause. Second line options include stenting of the short segment occlusion, transjugular intrahepatic portosystemic shunt (TIPS) or surgical decompression (which is rare now), and the last option is liver transplantation.[2,4] . In our patient the anticoagulant therapy was not sufficient to prevent the progression of the disease.
Right upper quadrant pain, ascites, jaundice, pedal edema, breathlessness on exertion, etc. are the most common presenting features of patients with Budd Chiari syndrome. Diagnosis mainly relies on sonography, Computed Tomography (CT), and other vascular imaging modalities like color Doppler, MR venogram are also useds. The treatment options include medical management with anticoagulants (low molecular weight heparin and warfarin) and treating the underlying cause. Second line options include stenting of the short segment occlusion, transjugular intrahepatic portosystemic shunt (TIPS) or surgical decompression (which is rare now), and the last option is liver transplantation.[2,4] . In our patient the anticoagulant therapy was not sufficient to prevent the progression of the disease.
Endovascular stenting and TIPS are the alternate management approaches for Budd Chiari syndrome. In our patient, we identified a short segment occlusion of the right hepatic vein and decided to perform angioplasty with stenting as this has a physiological advantage of restoring the normal blood flow pattern compared to sinusoidal perforation in the more invasive TIPS.[5] . Also TIPS has the disadvantage of unwanted liver capsule perforation and possible infection at the time of the procedure.[6] Endovascular stenting can be performed by internal jugular vein approach or percutaneous transhepatic approach.But percutaneous transhepatic approach has the potential of serious bleeding complication[7] especially in our patient who had ascites and deranged coagulation time. Thus endovascular stenting through the internal jugular approach was selected. However, we were unable to pass the guide wire across the occlusion anterogradely. Hence a large collateral was identified draining the right hepatic vein into the IVC and was used to form a loop. We were successful in passing the guide wire across the occlusion in a retrograde manner and used a snare from the second puncture to pull the same. Thus we established a “Trans collateral Loop” upon which the balloon was passed and inflated at the occlusion.
Endovascular stenting and TIPS are the alternate management approaches for Budd Chiari syndrome. In our patient, we identified a short segment occlusion of the right hepatic vein and decided to perform angioplasty with stenting as this has a physiological advantage of restoring the normal blood flow pattern compared to sinusoidal perforation in the more invasive TIPS.[5] . Also TIPS has the disadvantage of unwanted liver capsule perforation and possible infection at the time of the procedure.[6] Endovascular stenting can be performed by internal jugular vein approach or percutaneous transhepatic approach.But percutaneous transhepatic approach has the potential of serious bleeding complication[7] especially in our patient who had ascites and deranged coagulation time. Thus endovascular stenting through the internal jugular approach was selected. However, we were unable to pass the guide wire across the occlusion anterogradely. Hence a large collateral was identified draining the right hepatic vein into the IVC and was used to form a loop. We were successful in passing the guide wire across the occlusion in a retrograde manner and used a snare from the second puncture to pull the same. Thus we established a “Trans collateral Loop” upon which the balloon was passed and inflated at the occlusion.
There are very few cases describing the endovascular retrograde approach for recanalisation of hepatic vein occlusion in cases of Budd Chiari syndrome.[6,7,8,9 ] In a previous case [6],the collateral loop was established from the left hepatic vein to a lower right hepatic vein and the stent was deployed at the occluded confluence of veins. The patient had double right hepatic veins, out of which the lower one was connected to the left via a collateral. In another case [8]. the caudate lobe drained directly into the IVC and this small hepatic vein also had a shunt connecting it to the right hepatic vein. Thus the caudate-collateral was used to retrogradely cannulate the right hepatic vein. In a similar case by Weaver et al. [7] again, an engorged caudate vein was identified which had intrahepatic shunts to the right the hepatic vein and was cannulated to access the right hepatic venous occlusion. They named it the floss technique. In two cases by Sun et al. [9], there was obstruction of the retrohepatic portion of the IVC in patients of Budd chiari syndrome. A femoral access was established and the catheter was passed through accessory hepatic vein and into the IVC from where it was used to retrogradely access the occluded segment. Other studies have shown that the accessory collaterals may be recanalized to provide decompression. [10]. Our case did not have an enlarged caudate vein with an intrahepatic shunt but had a direct collateral from the right hepatic vein to the IVC.
There are very few cases describing the endovascular retrograde approach for recanalisation of hepatic vein occlusion in cases of Budd Chiari syndrome.[6,7,8,9 ] In a previous case [6],the collateral loop was established from the left hepatic vein to a lower right hepatic vein and the stent was deployed at the occluded confluence of veins. The patient had double right hepatic veins, out of which the lower one was connected to the left via a collateral. In another case [8]. the caudate lobe drained directly into the IVC and this small hepatic vein also had a shunt connecting it to the right hepatic vein. Thus the caudate-collateral was used to retrogradely cannulate the right hepatic vein. In a similar case by Weaver et al. [7] again, an engorged caudate vein was identified which had intrahepatic shunts to the right the hepatic vein and was cannulated to access the right hepatic venous occlusion. They named it the floss technique. In two cases by Sun et al. [9], there was obstruction of the retrohepatic portion of the IVC in patients of Budd chiari syndrome. A femoral access was established and the catheter was passed through accessory hepatic vein and into the IVC from where it was used to retrogradely access the occluded segment. Other studies have shown that the accessory collaterals may be recanalized to provide decompression. [10]. Our case did not have an enlarged caudate vein with an intrahepatic shunt but had a direct collateral from the right hepatic vein to the IVC.
Thus we would like to highlight the possibility of a trans collateral loop technique and re-emphasize the importance of identifying an intrahepatic collateral connection or an IVC-hepatic collateral connection in cases of Budd Chiari syndrome. It can serve as an important channel for endovascular stenting.
Thus we would like to highlight the possibility of a trans collateral loop technique and re-emphasize the importance of identifying an intrahepatic collateral connection or an IVC-hepatic collateral connection in cases of Budd Chiari syndrome. It can serve as an important channel for endovascular stenting.
References:
References:
1. Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21;13(19):2693-6.
1. Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21;13(19):2693-6.
2. Hitawala AA, Gupta V. Budd-Chiari Syndrome [Internet]. StatPearls - NCBI Bookshelf. 2023. Available from:
2. Hitawala AA, Gupta V. Budd-Chiari Syndrome [Internet]. StatPearls - NCBI Bookshelf. 2023. Available from:
3. Menon KVN, Shah V, Kamath PS. The Budd–Chiari Syndrome. New England Journal of Medicine/the New England Journal of Medicine 2004 Feb 5;350(6):578–85.
3. Menon KVN, Shah V, Kamath PS. The Budd–Chiari Syndrome. New England Journal of Medicine/the New England Journal of Medicine 2004 Feb 5;350(6):578–85.
4. Beckett, D., Olliff, S. Interventional Radiology in the Management of Budd Chiari syndrome. Cardiovasc Intervent Radiol 31, 839–847 (2008).
4. Beckett, D., Olliff, S. Interventional Radiology in the Management of Budd Chiari syndrome. Cardiovasc Intervent Radiol 31, 839–847 (2008).
5. Holland‐Fischer, P., Grønbæk, H., Astrup, L., Keiding, S., Nielsen, D. T., & Vilstrup, H. (2004). Budd‐Chiari and inferior caval vein syndromes due to membranous obstruction of the liver veins: successful treatment with angioplasty and transcaval transjugular intrahepatic porto‐systemic shunt. Scandinavian Journal of Gastroenterology, 39(10), 1025–1028.
5. Holland‐Fischer, P., Grønbæk, H., Astrup, L., Keiding, S., Nielsen, D. T., & Vilstrup, H. (2004). Budd‐Chiari and inferior caval vein syndromes due to membranous obstruction of the liver veins: successful treatment with angioplasty and transcaval transjugular intrahepatic porto‐systemic shunt. Scandinavian Journal of Gastroenterology, 39(10), 1025–1028.
6. Pereira da Silva F, Donato P, Caseiro-Alves F. Collteral loop approach from left to right liver lobe: Endovascular recanalization of a hepatic vein in Budd-Chiari syndrome. Eur J Radiol Open. 2016 Oct 24;3:251-253.
6. Pereira da Silva F, Donato P, Caseiro-Alves F. Collteral loop approach from left to right liver lobe: Endovascular recanalization of a hepatic vein in Budd-Chiari syndrome. Eur J Radiol Open. 2016 Oct 24;3:251-253.
7. Weaver JJ, Dobrow EM, Hsu EK, Monroe EJ. Single-access liver floss technique with antegrade hepatic vein access and recanalization in Budd-Chiari syndrome. Diagn Interv Radiol. 2018 Jan-Feb;24(1):38-41.
7. Weaver JJ, Dobrow EM, Hsu EK, Monroe EJ. Single-access liver floss technique with antegrade hepatic vein access and recanalization in Budd-Chiari syndrome. Diagn Interv Radiol. 2018 Jan-Feb;24(1):38-41.
8. Baptista M, Andrade LC, Furtado E, Donato P. Endovascular recanalization of a hepatic vein in Budd–Chiari Syndrome: a Collateral Loop-Guided approach. Journal of Vascular and Interventional Radiology [Internet]. 2015 Jan 1;26(1):135–7.
8. Baptista M, Andrade LC, Furtado E, Donato P. Endovascular recanalization of a hepatic vein in Budd–Chiari Syndrome: a Collateral Loop-Guided approach. Journal of Vascular and Interventional Radiology [Internet]. 2015 Jan 1;26(1):135–7.
9. Sun Z, Jiang J, Li W, Zhang X, Zhang X, He C. New approach to dilation of stenotic lesions through the accessory hepatic vein in Budd-Chiari syndrome. Journal of Vascular Surgery Cases [Internet]. 2015 Mar 1;1(1):42–5.
9. Sun Z, Jiang J, Li W, Zhang X, Zhang X, He C. New approach to dilation of stenotic lesions through the accessory hepatic vein in Budd-Chiari syndrome. Journal of Vascular Surgery Cases [Internet]. 2015 Mar 1;1(1):42–5.
10. Fu YF, Xu H, Zhang K, Zhang QQ, Wei N. Accessory hepatic vein recanalization for treatment of Budd-Chiari syndrome due to long-segment obstruction of the hepatic vein: initial clinical experience. Diagn Interv Radiol. 2015 Mar-Apr;21(2):148-53
10. Fu YF, Xu H, Zhang K, Zhang QQ, Wei N. Accessory hepatic vein recanalization for treatment of Budd-Chiari syndrome due to long-segment obstruction of the hepatic vein: initial clinical experience. Diagn Interv Radiol. 2015 Mar-Apr;21(2):148-53
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