Embedded Files
Welcome to the Academic and Educational pages of the
Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Interventional Case Record
Interventional Case Record
Sharp retrograde hepatic vein recanalization in a patient with Budd–Chiari Syndrome
Sharp retrograde hepatic vein recanalization in a patient with Budd–Chiari Syndrome
.Contributed by : Viraj Patel
.Contributed by : Viraj Patel
In 1845, George Budd:, a British physician , described a clinical triad of abdominal pain, ascites, and hepatomegaly in three patients with hepatic vein thrombosis. Hans Chiari, a Austrian pathologist, in 1899, added the pathological details, describing the "obliterating endophlebitis of the hepatic veins".
Introduction:
Introduction:
Budd–Chiari Syndrome (BCS) is characterized by obstruction of hepatic venous drainage at the level of the hepatic veins or inferior vena cava (IVC). The prevalence is approximately 1 in 100,000 individuals worldwide. The most common causes include hypercoagulable states such as myeloproliferative disorders, protein C/S deficiency, or oral contraceptive use in women. Infertility in women with BCS is uncommon and may result from hormonal imbalance secondary to hepatic dysfunction. Advances in interventional radiology (IR) have made endovascular management— including angioplasty and stenting—the preferred treatment option, offering restoration of hepatic venous outflow with minimal invasiveness.
Budd–Chiari Syndrome (BCS) is characterized by obstruction of hepatic venous drainage at the level of the hepatic veins or inferior vena cava (IVC). The prevalence is approximately 1 in 100,000 individuals worldwide. The most common causes include hypercoagulable states such as myeloproliferative disorders, protein C/S deficiency, or oral contraceptive use in women. Infertility in women with BCS is uncommon and may result from hormonal imbalance secondary to hepatic dysfunction. Advances in interventional radiology (IR) have made endovascular management— including angioplasty and stenting—the preferred treatment option, offering restoration of hepatic venous outflow with minimal invasiveness.
Case presentation:
Case presentation:
A 28 year old woman presented to the gynecology clinic with primary infertility for three years. She had regular menses and no prior pregnancies. There was no history of oral contraceptive use, jaundice, or chronic illness.
A 28 year old woman presented to the gynecology clinic with primary infertility for three years. She had regular menses and no prior pregnancies. There was no history of oral contraceptive use, jaundice, or chronic illness.
Laboratory findings showed normal LFT profile , normal coagulation profile and normal hormonal profile (FSH, LH, estradiol, TSH).
Laboratory findings showed normal LFT profile , normal coagulation profile and normal hormonal profile (FSH, LH, estradiol, TSH).
Radiological findings:
Radiological findings:
Ultrasonography of the abdomen and pelvis showed mild hepatomegaly with caudate lobe hypertrophy and coarse echotexture. The uterus and ovaries were norml. There was no collection in pouch of Douglas.. Doppler examination showed reversal of flow in the right and middle hepatic veins; the left hepatic vein (LHV) was patent with slow flow. There were intrahepatic veno-venous collaterals. (Fig 1,2 and Video 1)
Ultrasonography of the abdomen and pelvis showed mild hepatomegaly with caudate lobe hypertrophy and coarse echotexture. The uterus and ovaries were norml. There was no collection in pouch of Douglas.. Doppler examination showed reversal of flow in the right and middle hepatic veins; the left hepatic vein (LHV) was patent with slow flow. There were intrahepatic veno-venous collaterals. (Fig 1,2 and Video 1)
MRI with MR venography showed focal stenosis of the left hepatic vein near its confluence with the IVC . There were intrahepatic collateral channels filling the right and middle hepatic veins (RHV, MHV) suggestive of Budd–Chiari Syndrome. (Fig 3).
MRI with MR venography showed focal stenosis of the left hepatic vein near its confluence with the IVC . There were intrahepatic collateral channels filling the right and middle hepatic veins (RHV, MHV) suggestive of Budd–Chiari Syndrome. (Fig 3).
video 1.mp4
Fig 1, Video 1 (top), Fig. 2 (bottom) USG abdomen and HV Doppler showing reversal of flow in RHV and MHV , ostial occlusion and slow flow of LHV and large intrahepatic venovenous collaterals.
Fig 3 – – MRI Abdomen and MR venography showing Occlusion of All 3 HV ostia , Multiple intrahepatic veno-venous collaterals. Findings suggesting diagnosis of Budd-Chiari Syndrome.
Interventional technique:
Interventional technique:
Under ultrasound and fluoroscopic guidance, percutaneous right hepatic venous access was established using a short chiba needle and NEFF set. Following this, MHV ostium and LHV were cannulated through venovenous collaterals from the RHV access using a microcatheter. A venogram was performed from all three hepatic veins showing filling of all three hepatic veins from venovenous collaterals. (Video 2,3,4,5).
Under ultrasound and fluoroscopic guidance, percutaneous right hepatic venous access was established using a short chiba needle and NEFF set. Following this, MHV ostium and LHV were cannulated through venovenous collaterals from the RHV access using a microcatheter. A venogram was performed from all three hepatic veins showing filling of all three hepatic veins from venovenous collaterals. (Video 2,3,4,5).
video 2.mp4video 3.mp4Video 2 & 3 Venograms Obtained from Percutaneous RHV access.
video 4.mp4Video 4 MHV ostium cannulated through veno-venous collaterals from RHV using microcatheter and venogram was perpormed..
video 5.mp4Video 5 LHV cannulated from RHV access and venogram was performed,
LHV was cannulated under ultrasound guidance and using a Chiba needle and access secured by NEFF se. A right jugular venous access was also obtained. Simultaneous venography from LHV and IVC , confirmed a short segment occlusion of LHV at the confluence of the left hepatic vein with the IVC. (Video 6,7).
LHV was cannulated under ultrasound guidance and using a Chiba needle and access secured by NEFF se. A right jugular venous access was also obtained. Simultaneous venography from LHV and IVC , confirmed a short segment occlusion of LHV at the confluence of the left hepatic vein with the IVC. (Video 6,7).
video 6.mp4Video 6 Percutaneous access obtained into LHV, secured by passing Neff set.
video 7.mp4Video 7 IVC and LHV venograms performed simultaneously demonstrating short segment occlusion at the LHV ostium.
Attempts were made to cross the occlusion from RHV, LHV and IVC access using catheter and hydrophilic guide wires. However this was unsuccessful.. Hence a right IJV access was obtained using 10F short sheath. Using a Colapinto and long chiba needle the patent confluence of middle and left hepatic vein was punctured and a guidewire was negotiated into the left hepatic vein which in turn was snared from percutaneous left hepatic venous access. (Video 8,9,10,11).
Attempts were made to cross the occlusion from RHV, LHV and IVC access using catheter and hydrophilic guide wires. However this was unsuccessful.. Hence a right IJV access was obtained using 10F short sheath. Using a Colapinto and long chiba needle the patent confluence of middle and left hepatic vein was punctured and a guidewire was negotiated into the left hepatic vein which in turn was snared from percutaneous left hepatic venous access. (Video 8,9,10,11).
video 8.mp4video 9.mp4Video 8 & 9 – Crossing of ostial occlusion using Colapinto and CHIBA needle from IVC.
video 10.mp4video 11.mp4Video 10 & 11 Snaring of microwire passed from IVC through LHV Access.
Pre-dilatation was performed with a 6 mm balloon to allow the passage of catheter over the wire from IJV access. (Fig 4). A 4F catheter was advanced over 0.018 wire and exchanged for a 0.035 system. A LHV venogram was performed from IVC assess. (Video 12). LHV ostial venoplasty was done till disappearance of the waist. (Video 13,14). A balloon mounted metallic stent (9 × 37 mm) was deployed across the stenosis. (Video 15,16)
Pre-dilatation was performed with a 6 mm balloon to allow the passage of catheter over the wire from IJV access. (Fig 4). A 4F catheter was advanced over 0.018 wire and exchanged for a 0.035 system. A LHV venogram was performed from IVC assess. (Video 12). LHV ostial venoplasty was done till disappearance of the waist. (Video 13,14). A balloon mounted metallic stent (9 × 37 mm) was deployed across the stenosis. (Video 15,16)
Fig 4 – Balloon dilatation to allow passage of the catheter over the wire from IJV access.
video 12.mp4Video 12 LHV venogram from IVC access.
video 13.mp4video 14.mp4Video 13 & 14 Plasty of tight LHV Ostial Stenosis till disappearance of waist
video 15.mp4video 16.mp4Video 15 & 16 LHV stent deployment
Post-stent venography demonstrated free flow of contrast from the hepatic vein into the IVC without residual narrowing. (Video 17,18).
Post-stent venography demonstrated free flow of contrast from the hepatic vein into the IVC without residual narrowing. (Video 17,18).
video 17.mp4Video 17 Post Stent Deployment Venogram from LHV
video 18.mp4Video 18 RHV & LHV venogram obtained post LHV stenting demonstrating all venous drainage through recanalized LHV
The patient tolerated the procedure well and was started on warfarin (target INR 2–3).Follow up Doppler and CECT of the abdomen was performed after one week. This showed a patent stent with normal hepatic venous flow. (Fig 5 and Video 19)
The patient tolerated the procedure well and was started on warfarin (target INR 2–3).Follow up Doppler and CECT of the abdomen was performed after one week. This showed a patent stent with normal hepatic venous flow. (Fig 5 and Video 19)
video 19.mp4Fig 5 & Video 19 – Caption 16– Follow up CT after 1 week of LHV Stenting showing good flow across LHV stent.
Discussion:
Discussion:
Budd–Chiari syndrome can manifest in various ways depending on the extent and chronicity of venous obstruction. While the classic triad is hepatomegaly, ascites, and abdominal pain is often seen, some cases remain asymptomatic and are detected incidentally. The right hepatic vein drains directly into the IVC; the left and middle hepatic veins often unite to drain the rest of the liver in approximately 80% of the cases. Small accessory hepatic veins from the pericaval liver segments drain directly into the IVC . The intrahepatic venovenous collaterals and the hepatic vein occlusion can be appreciated well on imaging modalities like ultrasonography, triple phase CT and MRI [1] . Formation of intrahepatic venovenous collateral vessels in patients with hepatic venous obstruction is the body’s own compensatory mechanism to decompress the liver, and if these collateral vessels are adequate, it can explain the absence of symptoms in these patients.
Budd–Chiari syndrome can manifest in various ways depending on the extent and chronicity of venous obstruction. While the classic triad is hepatomegaly, ascites, and abdominal pain is often seen, some cases remain asymptomatic and are detected incidentally. The right hepatic vein drains directly into the IVC; the left and middle hepatic veins often unite to drain the rest of the liver in approximately 80% of the cases. Small accessory hepatic veins from the pericaval liver segments drain directly into the IVC . The intrahepatic venovenous collaterals and the hepatic vein occlusion can be appreciated well on imaging modalities like ultrasonography, triple phase CT and MRI [1] . Formation of intrahepatic venovenous collateral vessels in patients with hepatic venous obstruction is the body’s own compensatory mechanism to decompress the liver, and if these collateral vessels are adequate, it can explain the absence of symptoms in these patients.
In women, infertility may result from chronic hepatic dysfunction leading to altered estrogen metabolism, anovulation, or hypothalamic-pituitary-gonadal axis disturbance. Improvement in hepatic flow after stenting can normalize venous pressure and hormonal balance, leading to increased chances of conception. [2,3]
In women, infertility may result from chronic hepatic dysfunction leading to altered estrogen metabolism, anovulation, or hypothalamic-pituitary-gonadal axis disturbance. Improvement in hepatic flow after stenting can normalize venous pressure and hormonal balance, leading to increased chances of conception. [2,3]
Endovascular management (balloon angioplasty and stenting) is now considered the first-line treatment for short-segment hepatic vein or IVC stenosis, as it restores hepatic venous outflow, reduces portal pressure, and prevents progression to cirrhosis. Surgical shunts or liver transplantation are reserved for cases with diffuse obstruction or hepatic failure.[4,5]
Endovascular management (balloon angioplasty and stenting) is now considered the first-line treatment for short-segment hepatic vein or IVC stenosis, as it restores hepatic venous outflow, reduces portal pressure, and prevents progression to cirrhosis. Surgical shunts or liver transplantation are reserved for cases with diffuse obstruction or hepatic failure.[4,5]
This case emphasizes that Budd–Chiari syndrome can present atypically as infertility. Timely imaging diagnosis and interventional stenting can restore hepatic venous outflow and lead to significant clinical recovery, including reversal of infertility.[6] Interventional radiology plays a crucial role in both diagnosis and management of BCS.[7]
This case emphasizes that Budd–Chiari syndrome can present atypically as infertility. Timely imaging diagnosis and interventional stenting can restore hepatic venous outflow and lead to significant clinical recovery, including reversal of infertility.[6] Interventional radiology plays a crucial role in both diagnosis and management of BCS.[7]
References:
References:
1. Menon KV, Shah V, Kamath PS. The Budd–Chiari syndrome. N Engl J Med. 2004;350(6):578–585.
1. Menon KV, Shah V, Kamath PS. The Budd–Chiari syndrome. N Engl J Med. 2004;350(6):578–585.
2. Eapen CE, Velissaris D, Heydtmann M, et al. Long-term outcome of hepatic vein stenting in Budd–Chiari syndrome. Gut. 2006;55(6):878–884.
2. Eapen CE, Velissaris D, Heydtmann M, et al. Long-term outcome of hepatic vein stenting in Budd–Chiari syndrome. Gut. 2006;55(6):878–884.
3. Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd–Chiari syndrome: a review by the European Group for the Study of Vascular Disorders of the Liver (VASCU-LIVER). J Hepatol. 2003;38(3):364–371.
3. Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd–Chiari syndrome: a review by the European Group for the Study of Vascular Disorders of the Liver (VASCU-LIVER). J Hepatol. 2003;38(3):364–371.
4. Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in Budd–Chiari syndrome. Hepatology. 2004;39(2):500–508.
4. Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in Budd–Chiari syndrome. Hepatology. 2004;39(2):500–508.
5. Qi X, Han G, Guo X, et al. Transjugular stenting for Budd–Chiari syndrome: long-term outcomes. Liver Int. 2013;33(5):679–689.
5. Qi X, Han G, Guo X, et al. Transjugular stenting for Budd–Chiari syndrome: long-term outcomes. Liver Int. 2013;33(5):679–689.
6. Rautou PE, Moucari R, Cazals-Hatem D, et al. Pregnancy in women with treated Budd–Chiari syndrome: maternal and fetal outcomes. J Hepatol. 2009;51(3):475–481.
6. Rautou PE, Moucari R, Cazals-Hatem D, et al. Pregnancy in women with treated Budd–Chiari syndrome: maternal and fetal outcomes. J Hepatol. 2009;51(3):475–481.
7. Zhang W, Qi X, Zhang X, et al. Management of Budd–Chiari syndrome: an update on current therapeutic strategies. Clin Res Hepatol Gastroenterol. 2015;39(5):570–577.
7. Zhang W, Qi X, Zhang X, et al. Management of Budd–Chiari syndrome: an update on current therapeutic strategies. Clin Res Hepatol Gastroenterol. 2015;39(5):570–577.
Google Sites
Report abuse