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Interventional Case Record
Interventional Case Record
Contributed by : Akash Shah
Contributed by : Akash Shah
Endovascular Recanalization and IVC Stenting in a case of complete membranous occlusion of IVC in a patient causing Budd-Chiari Syndrome
Endovascular Recanalization and IVC Stenting in a case of complete membranous occlusion of IVC in a patient causing Budd-Chiari Syndrome
Introduction:
Introduction:
Inferior vena cava (IVC) obstruction is one of the causes of Budd-Chiari Syndrome (BCS). Primary IVC thrombosis produces a membrane of varying thickness or stenosis of varying degrees in the hepatic portion. Clinically, it is characterized by distinct subcutaneous venous collaterals that are less pronounced in hepatic vein thrombosis. In contrast, the collateral veins in hepatic vein thrombosis are similar to those seen in portal hypertension. These membranes are thought to be a congenital abnormality, but they are more frequently acquired. IVC obstruction is more common in Asian male population, and in western countries.BCS is found mostly in women and its predominant cause is hepatic vein obstruction.
Inferior vena cava (IVC) obstruction is one of the causes of Budd-Chiari Syndrome (BCS). Primary IVC thrombosis produces a membrane of varying thickness or stenosis of varying degrees in the hepatic portion. Clinically, it is characterized by distinct subcutaneous venous collaterals that are less pronounced in hepatic vein thrombosis. In contrast, the collateral veins in hepatic vein thrombosis are similar to those seen in portal hypertension. These membranes are thought to be a congenital abnormality, but they are more frequently acquired. IVC obstruction is more common in Asian male population, and in western countries.BCS is found mostly in women and its predominant cause is hepatic vein obstruction.
In contrast to hepatic vein thrombosis, formation of a membrane may be an outcome of recurrent thrombosis. BCS has been shown to be associated with prothrombotic states. Diagnosis can be established by clinical examination, Doppler ultrasound, coCT, MRI, and venography. (1) (2)
In contrast to hepatic vein thrombosis, formation of a membrane may be an outcome of recurrent thrombosis. BCS has been shown to be associated with prothrombotic states. Diagnosis can be established by clinical examination, Doppler ultrasound, coCT, MRI, and venography. (1) (2)
IVC Obstruction can treated with surgery but there is a high morbidity and mortality. Percutaneous transluminal balloon angioplasty is an alternative and effective form of treatment. Endovascular balloon dilatation, with or without stenting of hepatic veins and/or IVC has been reported earlier by various authors as a safe and excellent method. Endovascular angioplasty with stent placement for complete membranous obstruction of the suprahepatic inferior vena cava is safe and effective, and the long-term results are excellent. (3).
IVC Obstruction can treated with surgery but there is a high morbidity and mortality. Percutaneous transluminal balloon angioplasty is an alternative and effective form of treatment. Endovascular balloon dilatation, with or without stenting of hepatic veins and/or IVC has been reported earlier by various authors as a safe and excellent method. Endovascular angioplasty with stent placement for complete membranous obstruction of the suprahepatic inferior vena cava is safe and effective, and the long-term results are excellent. (3).
THe goal of treatment is to ensure long-term patency of the IVC with minimal mortality and morbidity. In cases of compression by tumors, it is restricted to palliation to relieve symptoms. Endovascular management can restore hepatic / IVC venous outflow in selected patients. This decompresses liver congestion, improves hepatic function, and arrests progression to hepatic destruction. Because angioplasty alone is prone to high re-occlusion rates, the recommendations are to place stents in the IVC or HVs. (4).
THe goal of treatment is to ensure long-term patency of the IVC with minimal mortality and morbidity. In cases of compression by tumors, it is restricted to palliation to relieve symptoms. Endovascular management can restore hepatic / IVC venous outflow in selected patients. This decompresses liver congestion, improves hepatic function, and arrests progression to hepatic destruction. Because angioplasty alone is prone to high re-occlusion rates, the recommendations are to place stents in the IVC or HVs. (4).
IVC recanalization with stenting is a physiological procedure that maintains natural blood flow in the IVC. It can minimize the risk of hepatic encephalopathy, and remains the first-line treatment option for patients with (5) (6). However, TIPS has less portal vein blood perfusion in the liver than recanalization and a relatively higher risk of hepatic encephalopathy due to increased blood ammonia level and impaired liver function after shunt placement (7). The treatment of BCS with an expandable metallic IVC stent was introduced to decrease the re-stenosis rate after angioplasty. A study found that most studies adopted recanalization + stenting as a first-line treatment because of its long term patency rates and excellent results. Also, the risk of hepatic encephalopathy after recanalization is lower than TIPS/DIPS. TIPS/DIPS has only been applied as an alternative treatment option for selective cases of BCS, but it may have a high risk of complication after shunt implantation (8).
IVC recanalization with stenting is a physiological procedure that maintains natural blood flow in the IVC. It can minimize the risk of hepatic encephalopathy, and remains the first-line treatment option for patients with (5) (6). However, TIPS has less portal vein blood perfusion in the liver than recanalization and a relatively higher risk of hepatic encephalopathy due to increased blood ammonia level and impaired liver function after shunt placement (7). The treatment of BCS with an expandable metallic IVC stent was introduced to decrease the re-stenosis rate after angioplasty. A study found that most studies adopted recanalization + stenting as a first-line treatment because of its long term patency rates and excellent results. Also, the risk of hepatic encephalopathy after recanalization is lower than TIPS/DIPS. TIPS/DIPS has only been applied as an alternative treatment option for selective cases of BCS, but it may have a high risk of complication after shunt implantation (8).
Case Presentation:
Case Presentation:
A 25 year old woman a known to have of Budd-Chiari Syndrome presented with complaints of abdominal distension and swelling in both lower limbs since two years, pain in abdomen, and general fatigue since one year. She had recurrent ascites despite being on medical management.
A 25 year old woman a known to have of Budd-Chiari Syndrome presented with complaints of abdominal distension and swelling in both lower limbs since two years, pain in abdomen, and general fatigue since one year. She had recurrent ascites despite being on medical management.
She had short segment IVC occlusion (?Membranous in nature) with involvement of the ostium of right hepatic vein. An ultrasound was performed which revealed severe narrowing of the hepatic-suprahepatic segment of the IVC with no colour flow within, with numerous collaterals. A MRI Abdominal Venogram was performed which revealed the total extent of the occlusion involving the IVC and the hepatic veins. A strong suspicion of a membranous type of IVC obstruction was made. Middle and left hepatic veins along with an accessory right hepatic venous branch were seen perfusing major part of liver volume. She had an IVC gram performed earlier this year which revealed a complete occlusion at the level of the junction of the hepatic and suprahepatic segment of the IVC with collateral venous channels draining into the azygous-hemiazygous system.
She had short segment IVC occlusion (?Membranous in nature) with involvement of the ostium of right hepatic vein. An ultrasound was performed which revealed severe narrowing of the hepatic-suprahepatic segment of the IVC with no colour flow within, with numerous collaterals. A MRI Abdominal Venogram was performed which revealed the total extent of the occlusion involving the IVC and the hepatic veins. A strong suspicion of a membranous type of IVC obstruction was made. Middle and left hepatic veins along with an accessory right hepatic venous branch were seen perfusing major part of liver volume. She had an IVC gram performed earlier this year which revealed a complete occlusion at the level of the junction of the hepatic and suprahepatic segment of the IVC with collateral venous channels draining into the azygous-hemiazygous system.
She had first presented in early 2019 with complaints of dyspepsia, post-prandial fullness and bloating, was treated with PPIs and Amitriptyline. In the latter half of the same year she had a pre-term vaginal delivery at 8 months during which her abdominal ultrasound was performed, revealing a coarse hepatic echotexture and partially recanalised para-umbilical vein. Soon into 2020, she underwent a UGI scopy revealing a small esophageal varix for which she took medical management (Figure 1).
She had first presented in early 2019 with complaints of dyspepsia, post-prandial fullness and bloating, was treated with PPIs and Amitriptyline. In the latter half of the same year she had a pre-term vaginal delivery at 8 months during which her abdominal ultrasound was performed, revealing a coarse hepatic echotexture and partially recanalised para-umbilical vein. Soon into 2020, she underwent a UGI scopy revealing a small esophageal varix for which she took medical management (Figure 1).
Fig. 1: UGI scopy revealing a small esophageal varix
Her blood investigations were as follows: CBC-13/7200/1.57L, OT/PT-24/19, TP/alb 6.7/3.5, T/D bili-0.9/0.2. She was lost to follow up for 3 years, after which she presented in 2023 with complaints of bloating, epigastric fullness and post prandial fullness. She underwent an abdominal ultrasound which now revealed hepatomegaly with a coarse echotexture, caudate lobe hypertrophy and many venous collaterals in the periesophageal, perigastric and peri-renal regions. Another UGI scopy was performed (Figure 2) .
Her blood investigations were as follows: CBC-13/7200/1.57L, OT/PT-24/19, TP/alb 6.7/3.5, T/D bili-0.9/0.2. She was lost to follow up for 3 years, after which she presented in 2023 with complaints of bloating, epigastric fullness and post prandial fullness. She underwent an abdominal ultrasound which now revealed hepatomegaly with a coarse echotexture, caudate lobe hypertrophy and many venous collaterals in the periesophageal, perigastric and peri-renal regions. Another UGI scopy was performed (Figure 2) .
Fig. 2: Esophagus reveals 3 small varices, Stomach : normal, duodenum: D1 and D2 is normal.
This documented an increase in the esophageal varices since 2020. A liver elastography performed on her revealed increased liver stiffness (23 kPa). On increasing suspiciousness of hepatic dysfunction, contrast enhanced MRI was performed with abdominal venography (Figures 3, 4 and 5) which revealed a cirrhotic hepatic morphology with changes of early portal hypertension with a segmental IVC obstruction with a high index of suspicion of membranous IVC obstruction.
This documented an increase in the esophageal varices since 2020. A liver elastography performed on her revealed increased liver stiffness (23 kPa). On increasing suspiciousness of hepatic dysfunction, contrast enhanced MRI was performed with abdominal venography (Figures 3, 4 and 5) which revealed a cirrhotic hepatic morphology with changes of early portal hypertension with a segmental IVC obstruction with a high index of suspicion of membranous IVC obstruction.
Fig. 3: The liver exhibits a cirrhotic morphology with changes of early portal hypertension.
Fig. 4: There is an abrupt narrowing at the junction of the hepatic/suprahepatic IVC with involvement of the ostium of the right hepatic vein with multiple intrahepatic veno-veno collaterals and a dilated lumbar venous plexus draining into the azygos - hemiazygos venous system.
Fig. 5: Chronic occlusion of a short segment of the retrohepatic segment of the IVC was noted with numerous collaterals [azygous-hemiazygous) from the suprahepatic IVC. Due to the chronic hepatic venous outflow tract obstruction [HVOTO], there were secondary hepatic parenchymal changes including an enlarged liver (RL. vertical span 19.1cm] with diffuse surface nodularity and iregulanty hypertrophied caudate lobe, widened and dysmorphic porta and interlobar fissures.
With careful assessment of all the relevant clinical history and examination with review of radiological imaging, the plan for management was decided in the form of recanalization of the IVC by balloon dilatation + placement of a balloon mounted IVC stent for long term recanalization and patency.
With careful assessment of all the relevant clinical history and examination with review of radiological imaging, the plan for management was decided in the form of recanalization of the IVC by balloon dilatation + placement of a balloon mounted IVC stent for long term recanalization and patency.
After adequate pre-procedural patient and relative counselling, obtaining of an informed consent, and pre-operative preparation-medical, surgical and anaesthesia fitness was completed.
After adequate pre-procedural patient and relative counselling, obtaining of an informed consent, and pre-operative preparation-medical, surgical and anaesthesia fitness was completed.
Venous access was obtained via 6 French sheath in the right femoral vein. Anticoagulation was administered in the form of unfractionated heparin during the procedure. Pre-intervention venogram was performed via a 4Fr pigtail catheter which was guided to reach as close to the level of obstruction as possible.
Venous access was obtained via 6 French sheath in the right femoral vein. Anticoagulation was administered in the form of unfractionated heparin during the procedure. Pre-intervention venogram was performed via a 4Fr pigtail catheter which was guided to reach as close to the level of obstruction as possible.
Video 1._AP_DSA.mp4Video 2.LAT_DSA.mp4Videos 1&2 : Pre-intervention IVC gram from left common femoral vein access using 4Fr pigtail catheter documenting IVC obstruction with numerous collaterals [azygous-hemiazygous). There are No patent venous channels directly draining into the right atrium.
There was a short, completely occluded segment (thick web) between the right atrium (RA) and IVC with multiple collaterals. After a few unsuccessful attempt to puncture the membrane with a curved tip Glidewire, the thick web was punctured with the distal tip of a stiff wire through a 4Fr headhunter (H1) catheter (Figure 6). Through a 4Fr headhunter (H1) catheter (Figure 6). After confirming the position of the H1 catheter by contrast injection, the sheath was advanced over the needle into the RA, beyond the occlusion (Videos 3 &4).
There was a short, completely occluded segment (thick web) between the right atrium (RA) and IVC with multiple collaterals. After a few unsuccessful attempt to puncture the membrane with a curved tip Glidewire, the thick web was punctured with the distal tip of a stiff wire through a 4Fr headhunter (H1) catheter (Figure 6). Through a 4Fr headhunter (H1) catheter (Figure 6). After confirming the position of the H1 catheter by contrast injection, the sheath was advanced over the needle into the RA, beyond the occlusion (Videos 3 &4).
Fig. 6:: Using an ultra stiff (AUS) wire to pass through the IVC occlusion through a 4 Fr Headhunter (H1) catheter, the occlusion was successfully crossed.
Video 3._AP_RA_entry.mp4Video 4._LAT_RA_entry.mp4Videos 3,4 :Video 3&4 : A venogram obtained after crossing of the IVC occlusion by placing 4Fr Headhunter (H1) catheter confirming successful contrast passage into the right atrium from the IVC. No abnormal contrast extravasation / leak is seen.
Then a J-tip glide wire was negotiated across the web and was placed in the left subclavian vein (Figure 7). The membrane was first dilated with a 7 x 60mm balloon, and patency across the IVC into the RA was achieved (Figure 8&9).
Then a J-tip glide wire was negotiated across the web and was placed in the left subclavian vein (Figure 7). The membrane was first dilated with a 7 x 60mm balloon, and patency across the IVC into the RA was achieved (Figure 8&9).
Fig 7. Right atrium was successfully crossed using glide wire manipulation reaching the proximal end of the left subclavian vein. 4Fr H1 catheter was passed over it and parked with its tip in the left subclavian vein.
Fig 8. : IVC ballooning and dilatation for venous recanalization using 7 x 60 mm balloon documenting pre-ballooning waist at the junction of hepatic and suprahepatic segment of IVC which disappeared upon progressive serial dilatation.
Fig 9 : Post IVC recanalization with balloon plasty, the IVC gram showing contrast passing into the right atrium through a narrowed segment of hepatic-suprahepatic IVC. The length of the narrowed segment of the IVC was calculated using the marker pigtail catheter . The Length of the narrowed IVC segment was approximately 4 cm.
Fig 10 After progressive serial dilatations of the right common femoral venous access, Aa14 Fr Long sheath was passed over a ultra stiff wire across the narrowed segment of the IVC with distal tip of the long sheath within the RA.
The selected stent was passed over the stiff wire and expanded using an over the wire 12 x 60mm Balloon at the required position and level (Figure 11). Post balloon expansion, the IVC gram showed patency of the deployed balloon mounted stent (Videos 5&6).
The selected stent was passed over the stiff wire and expanded using an over the wire 12 x 60mm Balloon at the required position and level (Figure 11). Post balloon expansion, the IVC gram showed patency of the deployed balloon mounted stent (Videos 5&6).
Fig 11 Dilatation of the narrowed patent lumen of the IVC performed by progressive inflation of the 12 x 60mm balloon.
Video 5. AP_DSA_post_stenting.mp4Video 6._ LAT_DSA_post_stenting.mp4Videos 5 6 Post balloon expansion , IVC gram showsg improved patency and flow from IVC into the RA with visualisation of few collaterals.
Further Re-dilatation and balloon expansion of the stent was performed using a 16 x 40 mm balloon (Figure 12).
Further Re-dilatation and balloon expansion of the stent was performed using a 16 x 40 mm balloon (Figure 12).
Fig 12 Re-dilatation and expansion of the stent using a 16 x 40 mm balloon to reduce the obstruction and improve patency.
Post balloon expansion and final stent deployment gram was taken using the 4Fr Pigtail catheter placed in the hepatic segment of the IVC (Videos 7&8) which showed no residual IVC narrowing or obstruction and smooth, free flow across the stent with immediate disappearance of most of the collaterals documented on the pre-procedure IVC gram.
Post balloon expansion and final stent deployment gram was taken using the 4Fr Pigtail catheter placed in the hepatic segment of the IVC (Videos 7&8) which showed no residual IVC narrowing or obstruction and smooth, free flow across the stent with immediate disappearance of most of the collaterals documented on the pre-procedure IVC gram.
Video 7.AP_DSA_post_stenting.mp4Video 8._LAT_DSA_post_stenting.mp4Videos 7&8 : After the final balloon dilatation using 16 x 40 mm balloon, , the IVC gram shows smooth flow into the RA and disappearance of collaterals .
A post procedure radiograph was taken (Figure 13) to confirm proper positioning of the IVC stent in situ after withdrawal of all hardware from within the patient.
A post procedure radiograph was taken (Figure 13) to confirm proper positioning of the IVC stent in situ after withdrawal of all hardware from within the patient.
Fig 13 : Post Procedure Radiograph documenting deployed IVC stent in situ in position.
The patient tolerated the procedure well and was monitored in the recovery room with removal of venous sheaths in both lower limbs with advice of strict lower limb immobilization for at least 6 hours. Procedural success was defined as venographic evidence of no residual IVC narrowing/occlusion and smooth physiological flow through created lumen and disappearance of collaterals seen on pre-intervention gram with absence of significant gradients across the level of obstruction. After intervention, satisfactory antegrade flow was observed in IVC. Normal flow spectrum was also visualised on color Doppler ultrasound. Post-procedure the patient was advised oral anticoagulation so as to maintain international normalised ration (INR) of 2–3.
The patient tolerated the procedure well and was monitored in the recovery room with removal of venous sheaths in both lower limbs with advice of strict lower limb immobilization for at least 6 hours. Procedural success was defined as venographic evidence of no residual IVC narrowing/occlusion and smooth physiological flow through created lumen and disappearance of collaterals seen on pre-intervention gram with absence of significant gradients across the level of obstruction. After intervention, satisfactory antegrade flow was observed in IVC. Normal flow spectrum was also visualised on color Doppler ultrasound. Post-procedure the patient was advised oral anticoagulation so as to maintain international normalised ration (INR) of 2–3.
Discussion
Discussion
Inferior vena cava obstruction can result from thrombosis secondary to hypercoagulable disorders, extrinsic compression by tumours, infective phlebitis, inflammation, trauma, surgery, or in many cases remains idiopathic. In longstanding cases, it results in swelling of extremities, pain, venous ulceration and impaired liver and renal functions. The course of the disease can be rapidly fatal, or at times it may be confused with other causes of cirrhosis and portal hypertension. This form of chronic IVC obstruction is common in developing countries. (9)
Inferior vena cava obstruction can result from thrombosis secondary to hypercoagulable disorders, extrinsic compression by tumours, infective phlebitis, inflammation, trauma, surgery, or in many cases remains idiopathic. In longstanding cases, it results in swelling of extremities, pain, venous ulceration and impaired liver and renal functions. The course of the disease can be rapidly fatal, or at times it may be confused with other causes of cirrhosis and portal hypertension. This form of chronic IVC obstruction is common in developing countries. (9)
Morphologically, IVC obstruction can be segmental, diffuse, discrete complete membranous or incomplete membranous obstruction. Discrete membranous obstruction now is believed to be a sequel of thrombosis and subsequent partial recanalisation. (10)
Morphologically, IVC obstruction can be segmental, diffuse, discrete complete membranous or incomplete membranous obstruction. Discrete membranous obstruction now is believed to be a sequel of thrombosis and subsequent partial recanalisation. (10)
Diagnosis can be established by clinical examination, colour Doppler sonography, contrast-enhanced computed tomography (CECT), magnetic resonance imaging (MRI), and venography. Once the diagnosis is established, inferior vena cavography is needed to guide endovascular techniques. (11)
Diagnosis can be established by clinical examination, colour Doppler sonography, contrast-enhanced computed tomography (CECT), magnetic resonance imaging (MRI), and venography. Once the diagnosis is established, inferior vena cavography is needed to guide endovascular techniques. (11)
Treatment of IVC obstruction includes surgical (direct visualisation of obstruction and removal or venous bypass grafts) or interventional (balloon dilatation ± stenting). (5,6,7) Surgery though successful, carries higher risk of complications in patients who already had impaired liver function. Mortality in surgical group is higher–33–40% (Kimura et al. & Iwaragi et al.). Stents have revolutionised the endovascular management and to maintain long-term patency of IVC. (12). Goal of treatment is to give a long-term patency of IVC with minimal mortality and morbidity. In case of tumour compression, it is restricted to palliation to relieve symptoms. Endovascular management can restore hepatic / IVC venous outflow in selected patients. This decompresses liver congestion, improves hepatic function, and arrests progression to hepatic destruction.
Treatment of IVC obstruction includes surgical (direct visualisation of obstruction and removal or venous bypass grafts) or interventional (balloon dilatation ± stenting). (5,6,7) Surgery though successful, carries higher risk of complications in patients who already had impaired liver function. Mortality in surgical group is higher–33–40% (Kimura et al. & Iwaragi et al.). Stents have revolutionised the endovascular management and to maintain long-term patency of IVC. (12). Goal of treatment is to give a long-term patency of IVC with minimal mortality and morbidity. In case of tumour compression, it is restricted to palliation to relieve symptoms. Endovascular management can restore hepatic / IVC venous outflow in selected patients. This decompresses liver congestion, improves hepatic function, and arrests progression to hepatic destruction.
Because angioplasty alone is prone to high re-occlusion rates, the recommendations are to place stents in the IVC or HVs. IVC Recanalization with stenting is a physiological procedure that maintains natural blood flow in the IVC. It can minimize the risk of hepatic encephalopathy, and remains a first-line treatment option for patients with BCS (13). However, TIPS has less portal vein blood perfusion in the liver than recanalization and a high risk of hepatic encephalopathy due to the formation of a blood ammonia level and impaired liver function after shunt placement. The treatment of BCS with an expandable metallic stent was introduced to decrease re-stenosis rate after angioplasty. A study found that most studies adopted recanalization (44.28%) as a first-line treatment because it is a relatively simple and quick procedure. Also, the risk of hepatic encephalopathy after recanalization is lower than TIPS/DIPS. TIPS/DIPS has only been applied as an alternative treatment option for selective cases of BCS, but it may have a high risk of complication after shunt implantation. However, several previous studies have reported high patency rate and good long-term outcome of TIPS/DIPS for BCS (14). Liver transplantation is a second surgical option for BCS when rapidly progressive liver failure occurs before or after TIPS (15).
Because angioplasty alone is prone to high re-occlusion rates, the recommendations are to place stents in the IVC or HVs. IVC Recanalization with stenting is a physiological procedure that maintains natural blood flow in the IVC. It can minimize the risk of hepatic encephalopathy, and remains a first-line treatment option for patients with BCS (13). However, TIPS has less portal vein blood perfusion in the liver than recanalization and a high risk of hepatic encephalopathy due to the formation of a blood ammonia level and impaired liver function after shunt placement. The treatment of BCS with an expandable metallic stent was introduced to decrease re-stenosis rate after angioplasty. A study found that most studies adopted recanalization (44.28%) as a first-line treatment because it is a relatively simple and quick procedure. Also, the risk of hepatic encephalopathy after recanalization is lower than TIPS/DIPS. TIPS/DIPS has only been applied as an alternative treatment option for selective cases of BCS, but it may have a high risk of complication after shunt implantation. However, several previous studies have reported high patency rate and good long-term outcome of TIPS/DIPS for BCS (14). Liver transplantation is a second surgical option for BCS when rapidly progressive liver failure occurs before or after TIPS (15).
A study from China recently reported a case series of 115 patients in which stents were placed in the IVC and HVs with success rates of 94% and 87%, respectively. Patency reached 90% after a mean follow up of more than 45 months. When the disease is not amenable to thrombolysis, angioplasty with or without stenting has emerged as an excellent option in both elective and emergent situations. (16)
A study from China recently reported a case series of 115 patients in which stents were placed in the IVC and HVs with success rates of 94% and 87%, respectively. Patency reached 90% after a mean follow up of more than 45 months. When the disease is not amenable to thrombolysis, angioplasty with or without stenting has emerged as an excellent option in both elective and emergent situations. (16)
When Hirooka and Kimura (17) reviewed 205 cases of Budd–Chiari syndrome from world literature, one third had membranous obstructions. They analyzed the anatomical details of 150 cases, and divided them into 10 different types according to the topology of the membrane. The location of the membrane relative to the right, left and middle hepatic vein orifices, and membrane thickness varied a great deal from case to case; some orifices were occluded and others were patent, but the membrane was invariably in the hepatic portion of the IVC. Such variable topologies of the membrane and ostial occlusion strongly suggest that the membrane is an outcome of thrombosis rather than a congenital anomaly. Various names have been used in the literature to denote the occluding lesion in the hepatic portion of IVC, and these included ‘coarctation’, (18) ‘congenital stenosis’, (19) ‘congenital occlusion of the IVC in its diaphragmatic portion’, (20) ‘congenital membranous obstruction of IVC’, (21) ‘membranous obstruction with coarctation of IVC in its diaphragmatic portion’, (22) ‘membranous stenosis’, (23) ‘fibrous constriction of IVC’, ‘partial Budd–Chiari syndrome (segmental obstruction of the hepatic cava’) and, most frequently, ‘membranous obstruction of the IVC (MOVC) (24).
When Hirooka and Kimura (17) reviewed 205 cases of Budd–Chiari syndrome from world literature, one third had membranous obstructions. They analyzed the anatomical details of 150 cases, and divided them into 10 different types according to the topology of the membrane. The location of the membrane relative to the right, left and middle hepatic vein orifices, and membrane thickness varied a great deal from case to case; some orifices were occluded and others were patent, but the membrane was invariably in the hepatic portion of the IVC. Such variable topologies of the membrane and ostial occlusion strongly suggest that the membrane is an outcome of thrombosis rather than a congenital anomaly. Various names have been used in the literature to denote the occluding lesion in the hepatic portion of IVC, and these included ‘coarctation’, (18) ‘congenital stenosis’, (19) ‘congenital occlusion of the IVC in its diaphragmatic portion’, (20) ‘congenital membranous obstruction of IVC’, (21) ‘membranous obstruction with coarctation of IVC in its diaphragmatic portion’, (22) ‘membranous stenosis’, (23) ‘fibrous constriction of IVC’, ‘partial Budd–Chiari syndrome (segmental obstruction of the hepatic cava’) and, most frequently, ‘membranous obstruction of the IVC (MOVC) (24).
References:
References:
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8. Tripathi, D. et al. Long-term outcomes following percutaneous hepatic vein recanalization for Budd-Chiari syndrome. Liver Int. 37(1), 111–120
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9. Tripathi, D. et al. Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome. Aliment. Pharmacol. Ther. 39(8), 864–872.
10. Garcia-Pagán, J. C. et al. TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients. Gastroenterology 135(3), 808–815
10. Garcia-Pagán, J. C. et al. TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients. Gastroenterology 135(3), 808–815
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