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Spontaneous Intrahepatic Portosystemic Shunt in Budd–Chiari Syndrome
Spontaneous Intrahepatic Portosystemic Shunt in BuddeChiari Syndrome Peng-Xu Ding, Zhen Li, Xin-Wei Han, Wen-Guang Zhang, Peng-Li Zhou, and Zhong-Gao Wang, Zhengzhou, China
BuddeChiari syndrome (BCS) caused by hepatic venous outflow obstruction may result in portal hypertension and the development of intrahepatic collaterals that bypass the obstruction. Spontaneous intrahepatic portosystemic venous shunt (SIPSVS) is uncommon and may be associated with portal hypertension. SIPSVS is extremely rare in patients with BCS and has not been well documented. We report a case of SIPSVS in a 42-year-old woman with BCS caused by membranous obstruction and chronic thrombosis in the inferior vena cava (IVC). A direct vascular communication between the left portal vein and IVC was confirmed by sonography and a computed tomography angiography scan. The patient underwent successful percutaneous balloon angioplasty of the IVC. Surgical or endovascular treatment for SIPSVS was not carried out because the patient was asymptomatic and remained asymptomatic in terms of encephalopathy at a 1-year follow-up.
BuddeChiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at different levels from the small hepatic veins (HVs) to the junction of the inferior vena cava (IVC) and the right atrium, regardless of the cause of the obstruction.1 The development of intrahepatic collaterals is a distinctive feature of BCS and is found in >80% of cases.2 Two forms of intrahepatic collaterals may develop3,4: (1) those that communicate with systemic veins via the subcapsular vessels and (2) those that shunt blood from the occluded segments to the nonoccluded segments of the HV. However, direct communication between portal and systemic veins is rare. Spontaneous intrahepatic portosystemic shunt (SIPSVS) is defined as a
Department of Vascular and Endovascular Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. Correspondence to: Peng-Xu Ding, MD, Department of Vascular and Endovascular Surgery, First Affiliated Hospital of Zhengzhou University, 1 East Jian She Road, Zhengzhou, Henan 450052, China; E-mail: [email protected] Ann Vasc Surg 2014; 28: 742.e1–742.e4 http://dx.doi.org/10.1016/j.avsg.2013.06.031 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: December 27, 2012; manuscript accepted: June 19, 2013; published online: February 2, 2014.
communication between the portal and systemic venous circulation, and is at least partially located inside the liver. Unlike dilated paraumbilical veins caused by portal hypertension, SIPSVS is generally considered rare, even though advanced imaging modalities have successfully revealed these abnormalities. We present a case of combined BCS and SIPSVS that was diagnosed in a 42-year-old woman by color duplex ultrasonography and a computed tomography angiography scan.
CASE REPORT A 42-year-old woman presented with abdominal distension and anorexia of 2 months’ duration. The physical examination revealed percussion pain in the hepatic region and prominent dilation of the subcutaneous veins. Viral hepatitis panel tests revealed negative hepatitis B surface antigen (HBsAg) and antibody against hepatitis C virus. Abdominal ultrasound (Fig. 1) and multislice computed tomography (CT) scans (Fig. 2) revealed a membranous obstruction with chronic thrombosis in the proximal IVC. The right HV and the middle HV were completely occluded. The left HV was patent, and there was an aneurysm between the left portal vein and the left HV. Based on these findings, we planned percutaneous balloon angioplasty of the IVC obstruction. The IVC was 742.e1
742.e2 Case reports
Annals of Vascular Surgery
day after the procedure for approximately 6 months. Ultrasound revealed that the IVC was patent at 6 months of follow-up (Fig. 5). The initial clinical presentation had completely disappeared at 6 months of follow-up. No surgical or endovascular intervention was performed for SIPSVS because the patient did not have any symptoms of hepatic encephalopathy for 1 year postoperatively.
DISCUSSION
Fig. 1. Color duplex ultrasonography on sagittal view reveals an intrahepatic shunt between the left portal vein and the inferior vena cava. accessed through the right femoral vein. Cavography was performed to identify the location and size of the thrombus and the occluded section of the IVC. The IVC pressure was then measured (25 cm H2O). Hepatic venography was not performed because the diagnosis of the HV was certain.5 After cavography, a 5-French straight catheter with multiple side holes guided by a hydrophilic guidewire (Cook Medical, Bloomington, IN) was inserted into the left portal vein through the IVC, the left HV, intrahepatic collaterals, and the portal vein. Cavography was then performed and revealed a SIPSVS (Fig. 3). The patient received a bolus of 5,000 IU heparin at the beginning of the procedure, followed by continuous infusion at 1,000 IU/hr to maintain the activated clotting time above 300 sec. A curved Brockenbrough needle (Medtronic, Minneapolis, MN) was then transfemorally introduced into the distal portion of the obstruction. Under the guidance of multiangle fluoroscopy, the angle of the needle was adjusted to ensure that the needle tip was directed to the catheter that was placed into the proximal portion of the obstruction from the right internal jugular vein. At this point, the obstruction was penetrated slowly by advancing the Brockenbrough needle. After crossing of the IVC membranes, a super stiff exchange wire (0.035in Amplatz Super Stiff Guidewire; Boston Scientific, Natick, MA) was inserted and positioned into the superior vena cava through the obstructive IVC. Balloon angioplasty of the IVC membranous obstruction was then performed with a 16-mm balloon catheter (Cook Medical). Subsequently, the IVC was dilated with a 30-mm Coda balloon catheter (Cook Medical). The inferior vena cavagraphy and IVC pressure measurement (8 cm H2O) were performed, and we confirmed that sufficient IVC blood flow was restored (Fig. 4). Postoperatively, the patient was given subcutaneous low molecular weight heparin at 5,000 IU every 12 hrs for 5 days. Warfarin was administrated orally to maintain the international normalized ratio at 2e3 from the second
BCS is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the levels of the hepatic venules, large HVs, IVC, or the right atrium, regardless of the cause of obstruction.1 Hepatic venous outflow tract obstruction leads to portal hypertension and to the development of intrahepatic and extrahepatic collaterals that bypass the obstruction.6 Intrahepatic collaterals are a specific diagnostic criterion for BCS,2 except for the paraumbilical vein, which is also observed in portal hypertension.7 With recent improvements, ultrasound, computed tomography, and magnetic resonance imaging scans have detected asymptomatic SIPSVS in an increasing number of patients. However, SIPSVSs are rarely seen,8 especially in patients with BCS. Park et al.9 categorized SIPSVSs into 4 different morphologic types. The most common type of SIPSVS is a single large tube of constant diameter that connects the right portal vein to the IVC. Type I includes patent paraumbilical veins, which are located in the liver. The shunts of type I are considered to be collateral pathways that develop in the hepatic parenchyma as a result of portal hypertension. The second type of SIPSVS is a localized peripheral shunt, in which single or multiple communications are found between peripheral branches of the portal vein and hepatic veins in one hepatic segment. The third type is aneurysmal, with peripheral portal and hepatic veins being connected through an aneurysm. The fourth type has multiple communications between peripheral portal and HVs diffusely in both lobes. According to this classification, our patient had a type 3 connection between the left HV and left branch of the portal vein. Most of the reported patients with this type of shunt were symptomatic, presenting with liver failure and portal hypertension. Recently, Lautz et al.10 classified intrahepatic portosystemic shunts, focusing on treatment options, considering that intrahepatic fistulas arising from a portal branch are usually suitable for endovascular closure. SIPSVS is a rare vascular pathology, and most of the reported cases have been associated with liver cirrhosis. Martin et al.11 reported that this disorder
Vol. 28, No. 3, April 2014
Fig. 2. (A) A computed tomography angiography (CTA) scan reveals that the inferior vena cava is a membranous obstruction with a small amount of inferior vena cava thrombosis (arrow). (B) An axial portal venous phase computed tomography scan reveals that the right and
Case reports 742.e3
middle hepatic veins cannot be identified. (C) A CTA scan with 3-dimensional reconstruction reveals an aneurysm connected with the left portal and the left hepatic veins (arrow).
Fig. 3. Intraoperative transfistula portography reveals an intrahepatic portocaval shunt between the left portal vein and the inferior vena cava via the left hepatic vein.
may also occur in patients with BCS, but the etiology of SIPSVS remains unclear. Hepatic encephalopathy is the major concern in SIPSVS. The size of the shunt and the tolerance to toxic metabolites may be factors that determine the development of hepatic encephalopathy.12 Because of the rarity of this disorder, the choice of treatment is still controversial.13,14 The traditional management of SIPSVS is surgery, including shunt ligation and hepatic resection. Angiographic embolization has become a safe and effective alternative to surgery in the past decade.14,15 Surgical or endovascular treatment options were postponed in our patient because she had a small-sized shunt and no cerebral manifestations suggesting hepatic encephalopathy. The patient
Fig. 4. Inferior vena cavogram reveals complete normalization of the diameter and flow characteristics after balloon angioplasty.
remains asymptomatic at 1 year of follow-up and we will continue to monitor her as an outpatient. Recent studies have shown that primary BCS should be regarded as a multifactorial disease, and that the co-occurrence of several prothrombotic
742.e4 Case reports
Fig. 5. Color duplex ultrasonography on sagittal view reveals that the inferior vena cava was widely patent at 6 months of follow-up.
disorders leads to thrombosis.16e18 A previous study found that several prothrombotic conditions were evident in at least 35% of BCS patients. Anticoagulation therapy is important for long-term success of the procedure because restenosis/thrombosis of the recanalized segment may occur, leading to early failure of the procedure. To prevent restenosis and thrombosis of the IVC, warfarin was administrated orally to our patient to maintain an international normalized ratio of approximately 2.5 after the procedure for approximately 6 months. In summary, SIPSVS is an uncommon disorder associated with hepatic cirrhosis and portal hypertension. SIPSVS is extremely rare in patients with BCS. SIPSVS can be treated with surgical or endovascular procedures, but it is also safe if patients remain asymptomatic for encephalopathy.
REFERENCES 1. Valla DC. Primary Budd-Chiari syndrome. J Hepatol 2009;50:195e203.
Annals of Vascular Surgery
2. Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis 2008;28:259e69. 3. Redmond PL, Kadir S, Cameron JL. Transhepatic venous collaterals in a patient with the Budd-Chiari syndrome. Cardiovasc Intervent Radiol 1988;11:285e7. 4. Cho OK, Koo JH, Kim YS, et al. Collateral pathways in Budd-Chiari syndrome: CT and venographic correlation. AJR Am J Roentgenol 1996;167:1163e7. 5. Kamath PS. Budd-Chiari syndrome: radiologic findings. Liver Transpl 2006;12(11 suppl 2):S21e2. 6. Cho KJ, Geisinger KR, Shields JJ, et al. Collateral channels and histopathology in hepatic vein occlusion. AJR Am J Roentgenol 1982;139:703e9. 7. Chaubal N, Dighe M, Hanchate V, et al. Sonography in Budd-Chiari syndrome. J Ultrasound Med 2006;25:373e9. 8. Lin ZY, Chen SC, Hsieh MY, et al. Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause. J Clin Ultrasound 2006;34:22e6. 9. Park JH, Cha SH, Han JK, et al. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 1990;155:527e8. 10. Lautz TB, Tantemsapya N, Rowell E, et al. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 2011;46:308e14. 11. Martin L, Dondelinger RF, Trotteur G. Treatment of BuddChiari syndrome by metallic stent as a bridge to liver transplantation. Cardiovasc Intervent Radiol 1995;189:196e9. 12. Santamaria G, Pruna X, Serres X, et al. Congenital intrahepatic portosystemic venous shunt: sonographic and magnetic resonance imaging. Eur Radiol 1996;69:76e8. 13. Kantarci F, Mihmanli I, Kara B, et al. Spontaneous intrahepatic portosystemic venous shunt in leptospirosis: is it a rare association or coincidence? Eur Radiol 2003;13(suppl 6): L235e6. 14. Grimaldi C, Monti L, Falappa P, et al. Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 2012;47:27e31. 15. Kim IO, Cheon JE, Kim WS, et al. Congenital intrahepatic portosystemic venous shunt: treatment with coil embolisation. Pediatr Radiol 2000;30:336e8. 16. Primignani M, Barosi G, Bergamaschi G, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology 2006;44:1528e34. 17. Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011;3:169e77. 18. Janssen HL, Garcia-Pagan JC, Elias E, et al. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38: 364e71.
BuddeChiari syndrome (BCS) caused by hepatic venous outflow obstruction may result in portal hypertension and the development of intrahepatic collaterals that bypass the obstruction. Spontaneous intrahepatic portosystemic venous shunt (SIPSVS) is uncommon and may be associated with portal hypertension. SIPSVS is extremely rare in patients with BCS and has not been well documented. We report a case of SIPSVS in a 42-year-old woman with BCS caused by membranous obstruction and chronic thrombosis in the inferior vena cava (IVC). A direct vascular communication between the left portal vein and IVC was confirmed by sonography and a computed tomography angiography scan. The patient underwent successful percutaneous balloon angioplasty of the IVC. Surgical or endovascular treatment for SIPSVS was not carried out because the patient was asymptomatic and remained asymptomatic in terms of encephalopathy at a 1-year follow-up.
BuddeChiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at different levels from the small hepatic veins (HVs) to the junction of the inferior vena cava (IVC) and the right atrium, regardless of the cause of the obstruction.1 The development of intrahepatic collaterals is a distinctive feature of BCS and is found in >80% of cases.2 Two forms of intrahepatic collaterals may develop3,4: (1) those that communicate with systemic veins via the subcapsular vessels and (2) those that shunt blood from the occluded segments to the nonoccluded segments of the HV. However, direct communication between portal and systemic veins is rare. Spontaneous intrahepatic portosystemic shunt (SIPSVS) is defined as a
Department of Vascular and Endovascular Surgery, First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. Correspondence to: Peng-Xu Ding, MD, Department of Vascular and Endovascular Surgery, First Affiliated Hospital of Zhengzhou University, 1 East Jian She Road, Zhengzhou, Henan 450052, China; E-mail: [email protected] Ann Vasc Surg 2014; 28: 742.e1–742.e4 http://dx.doi.org/10.1016/j.avsg.2013.06.031 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: December 27, 2012; manuscript accepted: June 19, 2013; published online: February 2, 2014.
communication between the portal and systemic venous circulation, and is at least partially located inside the liver. Unlike dilated paraumbilical veins caused by portal hypertension, SIPSVS is generally considered rare, even though advanced imaging modalities have successfully revealed these abnormalities. We present a case of combined BCS and SIPSVS that was diagnosed in a 42-year-old woman by color duplex ultrasonography and a computed tomography angiography scan.
CASE REPORT A 42-year-old woman presented with abdominal distension and anorexia of 2 months’ duration. The physical examination revealed percussion pain in the hepatic region and prominent dilation of the subcutaneous veins. Viral hepatitis panel tests revealed negative hepatitis B surface antigen (HBsAg) and antibody against hepatitis C virus. Abdominal ultrasound (Fig. 1) and multislice computed tomography (CT) scans (Fig. 2) revealed a membranous obstruction with chronic thrombosis in the proximal IVC. The right HV and the middle HV were completely occluded. The left HV was patent, and there was an aneurysm between the left portal vein and the left HV. Based on these findings, we planned percutaneous balloon angioplasty of the IVC obstruction. The IVC was 742.e1
742.e2 Case reports
Annals of Vascular Surgery
day after the procedure for approximately 6 months. Ultrasound revealed that the IVC was patent at 6 months of follow-up (Fig. 5). The initial clinical presentation had completely disappeared at 6 months of follow-up. No surgical or endovascular intervention was performed for SIPSVS because the patient did not have any symptoms of hepatic encephalopathy for 1 year postoperatively.
DISCUSSION
Fig. 1. Color duplex ultrasonography on sagittal view reveals an intrahepatic shunt between the left portal vein and the inferior vena cava. accessed through the right femoral vein. Cavography was performed to identify the location and size of the thrombus and the occluded section of the IVC. The IVC pressure was then measured (25 cm H2O). Hepatic venography was not performed because the diagnosis of the HV was certain.5 After cavography, a 5-French straight catheter with multiple side holes guided by a hydrophilic guidewire (Cook Medical, Bloomington, IN) was inserted into the left portal vein through the IVC, the left HV, intrahepatic collaterals, and the portal vein. Cavography was then performed and revealed a SIPSVS (Fig. 3). The patient received a bolus of 5,000 IU heparin at the beginning of the procedure, followed by continuous infusion at 1,000 IU/hr to maintain the activated clotting time above 300 sec. A curved Brockenbrough needle (Medtronic, Minneapolis, MN) was then transfemorally introduced into the distal portion of the obstruction. Under the guidance of multiangle fluoroscopy, the angle of the needle was adjusted to ensure that the needle tip was directed to the catheter that was placed into the proximal portion of the obstruction from the right internal jugular vein. At this point, the obstruction was penetrated slowly by advancing the Brockenbrough needle. After crossing of the IVC membranes, a super stiff exchange wire (0.035in Amplatz Super Stiff Guidewire; Boston Scientific, Natick, MA) was inserted and positioned into the superior vena cava through the obstructive IVC. Balloon angioplasty of the IVC membranous obstruction was then performed with a 16-mm balloon catheter (Cook Medical). Subsequently, the IVC was dilated with a 30-mm Coda balloon catheter (Cook Medical). The inferior vena cavagraphy and IVC pressure measurement (8 cm H2O) were performed, and we confirmed that sufficient IVC blood flow was restored (Fig. 4). Postoperatively, the patient was given subcutaneous low molecular weight heparin at 5,000 IU every 12 hrs for 5 days. Warfarin was administrated orally to maintain the international normalized ratio at 2e3 from the second
BCS is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the levels of the hepatic venules, large HVs, IVC, or the right atrium, regardless of the cause of obstruction.1 Hepatic venous outflow tract obstruction leads to portal hypertension and to the development of intrahepatic and extrahepatic collaterals that bypass the obstruction.6 Intrahepatic collaterals are a specific diagnostic criterion for BCS,2 except for the paraumbilical vein, which is also observed in portal hypertension.7 With recent improvements, ultrasound, computed tomography, and magnetic resonance imaging scans have detected asymptomatic SIPSVS in an increasing number of patients. However, SIPSVSs are rarely seen,8 especially in patients with BCS. Park et al.9 categorized SIPSVSs into 4 different morphologic types. The most common type of SIPSVS is a single large tube of constant diameter that connects the right portal vein to the IVC. Type I includes patent paraumbilical veins, which are located in the liver. The shunts of type I are considered to be collateral pathways that develop in the hepatic parenchyma as a result of portal hypertension. The second type of SIPSVS is a localized peripheral shunt, in which single or multiple communications are found between peripheral branches of the portal vein and hepatic veins in one hepatic segment. The third type is aneurysmal, with peripheral portal and hepatic veins being connected through an aneurysm. The fourth type has multiple communications between peripheral portal and HVs diffusely in both lobes. According to this classification, our patient had a type 3 connection between the left HV and left branch of the portal vein. Most of the reported patients with this type of shunt were symptomatic, presenting with liver failure and portal hypertension. Recently, Lautz et al.10 classified intrahepatic portosystemic shunts, focusing on treatment options, considering that intrahepatic fistulas arising from a portal branch are usually suitable for endovascular closure. SIPSVS is a rare vascular pathology, and most of the reported cases have been associated with liver cirrhosis. Martin et al.11 reported that this disorder
Vol. 28, No. 3, April 2014
Fig. 2. (A) A computed tomography angiography (CTA) scan reveals that the inferior vena cava is a membranous obstruction with a small amount of inferior vena cava thrombosis (arrow). (B) An axial portal venous phase computed tomography scan reveals that the right and
Case reports 742.e3
middle hepatic veins cannot be identified. (C) A CTA scan with 3-dimensional reconstruction reveals an aneurysm connected with the left portal and the left hepatic veins (arrow).
Fig. 3. Intraoperative transfistula portography reveals an intrahepatic portocaval shunt between the left portal vein and the inferior vena cava via the left hepatic vein.
may also occur in patients with BCS, but the etiology of SIPSVS remains unclear. Hepatic encephalopathy is the major concern in SIPSVS. The size of the shunt and the tolerance to toxic metabolites may be factors that determine the development of hepatic encephalopathy.12 Because of the rarity of this disorder, the choice of treatment is still controversial.13,14 The traditional management of SIPSVS is surgery, including shunt ligation and hepatic resection. Angiographic embolization has become a safe and effective alternative to surgery in the past decade.14,15 Surgical or endovascular treatment options were postponed in our patient because she had a small-sized shunt and no cerebral manifestations suggesting hepatic encephalopathy. The patient
Fig. 4. Inferior vena cavogram reveals complete normalization of the diameter and flow characteristics after balloon angioplasty.
remains asymptomatic at 1 year of follow-up and we will continue to monitor her as an outpatient. Recent studies have shown that primary BCS should be regarded as a multifactorial disease, and that the co-occurrence of several prothrombotic
742.e4 Case reports
Fig. 5. Color duplex ultrasonography on sagittal view reveals that the inferior vena cava was widely patent at 6 months of follow-up.
disorders leads to thrombosis.16e18 A previous study found that several prothrombotic conditions were evident in at least 35% of BCS patients. Anticoagulation therapy is important for long-term success of the procedure because restenosis/thrombosis of the recanalized segment may occur, leading to early failure of the procedure. To prevent restenosis and thrombosis of the IVC, warfarin was administrated orally to our patient to maintain an international normalized ratio of approximately 2.5 after the procedure for approximately 6 months. In summary, SIPSVS is an uncommon disorder associated with hepatic cirrhosis and portal hypertension. SIPSVS is extremely rare in patients with BCS. SIPSVS can be treated with surgical or endovascular procedures, but it is also safe if patients remain asymptomatic for encephalopathy.
REFERENCES 1. Valla DC. Primary Budd-Chiari syndrome. J Hepatol 2009;50:195e203.
Annals of Vascular Surgery
2. Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis 2008;28:259e69. 3. Redmond PL, Kadir S, Cameron JL. Transhepatic venous collaterals in a patient with the Budd-Chiari syndrome. Cardiovasc Intervent Radiol 1988;11:285e7. 4. Cho OK, Koo JH, Kim YS, et al. Collateral pathways in Budd-Chiari syndrome: CT and venographic correlation. AJR Am J Roentgenol 1996;167:1163e7. 5. Kamath PS. Budd-Chiari syndrome: radiologic findings. Liver Transpl 2006;12(11 suppl 2):S21e2. 6. Cho KJ, Geisinger KR, Shields JJ, et al. Collateral channels and histopathology in hepatic vein occlusion. AJR Am J Roentgenol 1982;139:703e9. 7. Chaubal N, Dighe M, Hanchate V, et al. Sonography in Budd-Chiari syndrome. J Ultrasound Med 2006;25:373e9. 8. Lin ZY, Chen SC, Hsieh MY, et al. Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause. J Clin Ultrasound 2006;34:22e6. 9. Park JH, Cha SH, Han JK, et al. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 1990;155:527e8. 10. Lautz TB, Tantemsapya N, Rowell E, et al. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 2011;46:308e14. 11. Martin L, Dondelinger RF, Trotteur G. Treatment of BuddChiari syndrome by metallic stent as a bridge to liver transplantation. Cardiovasc Intervent Radiol 1995;189:196e9. 12. Santamaria G, Pruna X, Serres X, et al. Congenital intrahepatic portosystemic venous shunt: sonographic and magnetic resonance imaging. Eur Radiol 1996;69:76e8. 13. Kantarci F, Mihmanli I, Kara B, et al. Spontaneous intrahepatic portosystemic venous shunt in leptospirosis: is it a rare association or coincidence? Eur Radiol 2003;13(suppl 6): L235e6. 14. Grimaldi C, Monti L, Falappa P, et al. Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 2012;47:27e31. 15. Kim IO, Cheon JE, Kim WS, et al. Congenital intrahepatic portosystemic venous shunt: treatment with coil embolisation. Pediatr Radiol 2000;30:336e8. 16. Primignani M, Barosi G, Bergamaschi G, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology 2006;44:1528e34. 17. Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011;3:169e77. 18. Janssen HL, Garcia-Pagan JC, Elias E, et al. Budd-Chiari syndrome: a review by an expert panel. J Hepatol 2003;38: 364e71.