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Living Donor Liver Transplantation in Budd-Chiari Syndrome: A Single-Center Experience
OUTCOMES
Living Donor Liver Transplantation in Budd-Chiari Syndrome: A Single-Center Experience G.S. Choi, J.B. Park, G.O. Jung, J.M. Chun, J.M. Kim, J.I. Moon, C.H.D. Kwon, S.J. Kim, J.W. Joh, and S.-K. Lee ABSTRACT Budd-Chiari syndrome (BCS), which is characterized by hepatic venous outflow obstruction due to occlusion of the major hepatic vein and/or the inferior vena cava (IVC), is rare. Traditionally, a caval resection is advocated for these patients; however, such a manenver renders living donor liver transplantation (LDLT) impossible. We encountered BCS in 4/377 LDLT patients during a 5-year period (January 2003 to December 2007). This report examine the various surgical modifications in these 4 patients, who underwent to LDLT for BCS. Resection of right hepatic vein (RHV) with an adjacent fibrotic part of the IVC with direct anastomosis of the graft RHV to the IVC was performed in 2 patients. One patient underwent retrohepatic IVC excision and reconstruction with a cryopreserved autologous IVC graft. The fourth patient, with a preexisting mesoatrial shunt for BCS, underwent conversion of this to a RHV atrial shunt. Graft and patient survivals were 100%. There were few complications in either donors or recipients. LDLT for BCS can be performed safely with adequate venous drainage techniques and with anticoagulant therapy and good follow-up for early diagnosis and treatment of recurrence leading to excellent long-term results. ARIOUS TREATMENT modalities exist for BuddChiari syndrome (BCS), a veno-occlusive disorder resulting in hepatic outflow obstruction, hepatic congestion, liver failure, or portal hypertension.1,2 These options include surgical portocaval shunts,3 angioplasty or transjugular intrahepatic portosystemic shunt,4,5 and liver transplantation (OLT).6 – 8 Traditional teaching suggests that OLT should be offered only after failure of conventional therapy, such as anticoagulation, diuretics, or portosystemic shunts.9,10 Therapeutic strategies depend on the functional status of the liver. Patients with cirrhosis, malignancy, or hepatic encephalopathy warrant consideration for OLT. Available data for OLT performed for BCS show excellent long-term survival and patency.6 – 8 In cadaveric donor OLT, the inferior vena cava (IVC) is generally replaced
V
© 2010 Published by Elsevier Inc. 360 Park Avenue South, New York, NY 10010-1710 Transplantation Proceedings, 42, 839 – 842 (2010)
with the donor IVC, because the donor liver graft contains the segment of IVC and hepatic veins. In contrast, in living donor liver transplantation (LDLT), specific venous drainage procedures must be devised regarding the level and size of the stenotic or the obliterated retrohepatic IVC. In this report, we have discussed our results and presented a literature review of surgical techniques and outcomes in LDLT for BCS patients. From the Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. Address reprint requests to Suk-Koo Lee, MD, PhD, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, Korea. E-mail: [email protected] 0041-1345/10/$–see front matter doi:10.1016/j.transproceed.2010.02.045 839
840
CHOI, PARK, JUNG ET AL Table 1. Donor and Recipient Demographics Patient
Gender Age (y) MELD Donor age and gender Viral hepatitis Clinical presentation Previous intervention Hepatocellular carcinoma (largest tumor size, tumor number) Graft to body weight ratio Liver weight (g) Follow-up (mos)
#1
Female 44 7 32 M None Uncontrolled ascites TACE* Yes (1.5 cm, #1) 1.43% 884 24
#2
#3
#4
Male 45 12 17 M None Variceal bleeding EVL No
Female 50 11 23 M HBV Uncontrolled ascites TACE Yes (2 cm, #2)
Female 41 15 44 F None HCC TACE Yes (2.1 cm, #4)
0.78% 2400 18
1017 13
1.2%
1.1% 944 23
Abbreviations: TACE, transarterial chemoembolization; EVL, endoscopic variceal ligation.
PATIENTS AND METHODS During a 5-year period at our institution (January 2003 to December 2007), we performed LDLT in 377 patients including 4 (1.06%) with a primary diagnosis of BCS: 1 male and 3 females. Their mean age was 44 years (Table 1). Three patients were transplanted as a primary procedure. One patient had undergone surgical treatment using a mesoatrial shunt with a ringed PTFE graft 10 years before OLT (Fig 1A). Patients were approved for LDLT after complete evaluation by our liver transplant selection committee. All 4 patients received right lobe grafts. All donors underwent a complete preoperative evaluation, which involved a comprehensive medical examination including laboratory values, ultrasonography, computed tomography (CT) with reconstruction, magnetic resonace (MR) imaging with MR cholangiography, volumetry and psychiatric clearance. The donor hepatectomy was performed in standard fashion through a right subcostal incision with left and upper midline extensions. The recipient work-up included laboratory values, ultrasonography, and computed tomography (CECT with reconstruction). Recipient hepatectomy was performed through a bilateral subcostal incision with an upper midline extension. The portal high hilar dissection was described previously.11 The preservation of recipient IVC was achieved by meticulous dissection of the caudate lobe from the IVC, despite the presence of many short hepatic veins between the caudate lobe and the retrohepatic IVC. Portal venous and arterial anastomoses were completed in standard fashion. A duct- to-duct biliary anastomosis was done with a combination of a posterior running and an anterior interrupted row of absorbable sutures. Posttransplant immunosuppression included induction with basiliximab, and maintenance with tacrolimus, mycophenolate mofetil, and steroid. Steroid was weaned at 4 months after transplantation. In the postoperative period, color Doppler ultrasound was performed on days 1 and 5. 99m Tc-DISIDA scan and CT angiography were performed as per our routine OLT protocol on day 14.
2 showed venous outlet obstruction at the level of the major hepatic veins but the IVC was patent. The orifice of the obliterated right hepatic vein was resected with freshening
RESULTS
The demographic features of the recipients and donors are listed in Table 1. There were no significant donor complications. Three patients had hepatocellular carcinoma and 1, a history of variceal bleeding with endoscopic treatment. No patient had evidence of hypercoagulopathy. Patients 1 and
Fig 1. (A) Pretransplant CT angiography reconstruction of patient with previous mesoatrial shunt. (B) Posttransplant CT follow-up focusing to hepatic venous drainage.
LDLT AND BUDD-CHIARI SYNDROME
of the adjacent IVC for anastomosis of the right hepatic vein of the graft to the IVC in end to side fashion using the piggy-back method without venovenous bypass. In the case of patient 3, we detected a 2.5-cm obliteration of the retrohepatic IVC around the orifice of hepatic veins. After caval sparing during total hepatectomy, the obliterated IVC was dissected, removed and interposed with a cryopreserved cadaveric IVC graft. Due to the presence of extensive collateral vessels a venovenous bypass was not needed (Fig 2). Patient 4 had a history of surgical treatment using a mesoatrial shunt from the superior mesenteric vein to the right atrium using a ringed PTFE graft 10 years prior as primary treatment for BCS (Fig 1A). The liver and the retrohepatic IVC, which was totally obliterated, were removed and the previous mesoatrial shunt resected near the mesenteric vein, and then closed. The hepatic vein of the right lobe graft was anastomosed to the PTFE graft to established drainage to the atrium through the previous mesoatrial shunt graft (Fig 1B). Immediate postoperative anticoagulation utilized low-molecular-weight heparin, which was later converted to long term therapy with anti-platelet agents only. In all cases, follow-up Doppler and CT scans revealed that all vascular structures were patent. Graft and patient survival was 100%. Postoperatively, 1 patient displayed a biliary stricture, which was managed with endoscopic retrograde cholangiopancreatographic dilatation and stent placement. Another patient display cytomegalovirus infection requiring readmission for ganicclovir treatment.
Fig 2.
841
DISCUSSION
Patients with BCS, who display a poor quality of life secondary to venous obstruction, hepatomegaly, decompensated liver function, fulminant hepatic failure or hepatocellular carcinoma qualify for OLT in the current Model for End-Stage Liver Disease system. OLT for BCS was first performed in 1974. Since then, several series and registry analyses including 2 recent large series have documented the European and US experiences of liver transplantation for BCS.6 – 8 In patients with certain synthetic deficiencies, such as antithrombin III or protein C, liver transplantation offers the additional advantage of curing the genetic defects. Patients with BCS have multiple therapeutic options that may not be available to other patients on the transplant waiting list. Although liver transplantation has emerged as an increasingly safe therapeutic option for patients with BCS, its application should not be used liberally. Most literature on OLT for BCS concerns DDLT. Surgical techniques and long-term results of DDLT have reported 5-year survival rates of 45%–95%; BCS has been reported to recur in 0%–27% of cases.6 – 8 The deceased donor graft has an IVC segment with hepatic veins. Consequently one can apply techniques involving removal of the retrohepatic IVC as part of the donor hepatectomy and the cavocaval anastomosis in the recipient. Depending on the status of the collaterals, the standard operative technique of DDLT in BCS was used with or without a venovenous bypass procedure.
Interposed cyroperserved cadaveric vena caval graft and anastomosis of graft.
842
However, surgical techniques and long-term follow-up are lacking for LDLT in BCS; experiences have been mostly restricted to case reports only. From a technical perspective, LDLT provides substantial challenges in the setting of BCS. Several technical considerations like caudate lobe enlargement; diffuse fibrotic reactions in the retroperitoneum, which increase the complexity of steps requiring control of the IVC; portal vein thickening and thrombosis; as well as increased bleeding and subsequent hemostasis make LDLT challenging in these patients. In LDLT, the piggy-back technique is used with preservation of the recipient IVC. The anastomosis between the recipient hepatic vein cuff and the graft hepatic vein is performed using a partial liver graft in the absence of a donor IVC segment. Stenosis associated with BCS has been classified by Yamada et al into 2 types;8 partial or complete occlusion. In the latter, there is an absence of pressure gradient differences across the area of stenosis due to well-developed hemi-azygous veins, and the other is partial occlusion with the presence of pressure gradient differences across the area of stenosis. The latter is distinguished by the existence of a clinical symptom, such as ascites, lower extremity edema, and renal dysfunction. This classification correlates with surgical procedures. The key considerations in the surgical procedure for LDLT among patients with BCS is the management of a stenosed or occluded IVC, and the choice of techniques to reconstruct the graft hepatic veins.8 In situations where large pressure differences exist before and after the stenosis, lower extremity edema, distended abdominal flank, and back veins and albuminuria may occur. Patch plasty for IVC stenosis was recommended by Yamada et al.8 They described the necessity for the IVC patch plasty depending on the site and extension of the IVC defects after thrombectomy and resection of the dense fibrotic wall of the IVC. We were able to resect the hepatic vein cuff and adjacent fibrotic area, then performing a direct anastomosis to the IVC in patients 1 and 2. We did not need a venous patch plasty, due to partial resection of the IVC with a subsequent wide anastomosis hence minimizing the chances of a recurrence. IVC replacement becomes necessary when the degree of IVC stenosis or obstruction is extensive or when a metallic stent has been implanted into the diseased IVC during a previous intervention.12 In cases of complete occlusion of the IVC, resection of the segment with placement of a cryopreserved autologous graft is an option. In patient 3 the strictured fibrosed IVC was replaced by an autologous graft. If the retroperitoneum is severely scarred and fibrosed, and resection of the IVC is technically risky, a possible option could be a direct RHV-left atrial shunt. We realized this option in patient 4, who had a preexistent meso-atrial shunt. Here, the mesenteric end of the previous meso-atrial shunt was disconnected, and the RHV of the graft was anastomosed to the PTFE shunt, hence achieving direct drainage to the left atrium. This RHV-atrial PTFE graft
CHOI, PARK, JUNG ET AL
showed good long-term patency at 2 years. A direct RHVleft atrial shunt can be an option for patients with preexisting meso-atrial shunts or when IVC excision is technically risky due to local conditions. Kuang et al13 reported unfavorable long-term results of using cryopreserved vein grafts for portal vein and hepatic artery replacement due to complications including aneurysm, thrombosis, and stricture. However, the long-term patency of autologous grafts has not been reported for vein replacement. Yamada et al8 presented 9 patients with BCS who were treated with LDLT. This series reported 88% 1- and 71% 3-year survivals, with 2 patients suffering recurrent BCS. We have had similar results with no recurrences at a mean follow-up of 20 months; these results are comparable to DDLT in BCS. In conclusion, LDLT may be performed safely for patients with BCS with suitable modifications in the surgical technique depending on the type of BCS and the local conditions. Long-term anticoagulant therapy and good follow-up for early diagnosis and interventional radiologic treatment of recurrent BCS can yield excellent outcomes after LDLT for BCS. REFERENCES 1. Menon KV, Shah V, Kamath PS: The Budd-Chiari syndrome. N Engl J Med 350:578, 2004 2. Klein AS: Management of Budd-Chiari syndrome. Liver Transpl 12:S23, 2006 3. Murad SD, Valla DC, de Groen PC, et al: Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 39:500, 2004 4. Molmenti EP, Segev DL, Arepally A, et al: The utility of TIPS in the management of Budd-Chiari syndrome. Ann Surg 241:978, 2005 5. Eapen CE, Velissaris D, Heydtmann M, et al: Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut 55:878, 2006 6. Segev DL, Nguyen GC, Locke JE, et al: Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl 13:1285, 2007 7. Mentha G, Giostra E, Majno PE, et al: Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol 44:520, 2006 8. Yamada T, Tanaka K, Ogura Y, et al: Surgical techniques and long-term outcomes of living donor liver transplantation for BuddChiari syndrome. Am J Transplant 6:2463, 2006 9. Campbell DA, Rolles K, Jamieson N, et al: Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome. Surg Gynecol Obstet 166: 511, 1988 10. Shaked A, Goldstein RM, Klintmalm GB, et al: Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. SGO 174:453, 1992 11. Lee KW, Joh JW, Kim SJ, et al: High hilar dissection: new technique to reduce biliary complication in living donor liver transplantation. Liver Transpl 10:1158, 2004 12. Shimoda M, Marubashi S, Dono K, et al: Utilization of autologous vein graft for replacement of the inferior vena cava in living-donor liver transplantation for obliterative hepatocavopathy. Transpl Int 20:804, 2007 13. Kuang AA, Renz JF, Ferrell LD, et al: Failure patterns of cryopreserved vein grafts in liver transplantation. Transplantation 62:742, 1996
Living Donor Liver Transplantation in Budd-Chiari Syndrome: A Single-Center Experience G.S. Choi, J.B. Park, G.O. Jung, J.M. Chun, J.M. Kim, J.I. Moon, C.H.D. Kwon, S.J. Kim, J.W. Joh, and S.-K. Lee ABSTRACT Budd-Chiari syndrome (BCS), which is characterized by hepatic venous outflow obstruction due to occlusion of the major hepatic vein and/or the inferior vena cava (IVC), is rare. Traditionally, a caval resection is advocated for these patients; however, such a manenver renders living donor liver transplantation (LDLT) impossible. We encountered BCS in 4/377 LDLT patients during a 5-year period (January 2003 to December 2007). This report examine the various surgical modifications in these 4 patients, who underwent to LDLT for BCS. Resection of right hepatic vein (RHV) with an adjacent fibrotic part of the IVC with direct anastomosis of the graft RHV to the IVC was performed in 2 patients. One patient underwent retrohepatic IVC excision and reconstruction with a cryopreserved autologous IVC graft. The fourth patient, with a preexisting mesoatrial shunt for BCS, underwent conversion of this to a RHV atrial shunt. Graft and patient survivals were 100%. There were few complications in either donors or recipients. LDLT for BCS can be performed safely with adequate venous drainage techniques and with anticoagulant therapy and good follow-up for early diagnosis and treatment of recurrence leading to excellent long-term results. ARIOUS TREATMENT modalities exist for BuddChiari syndrome (BCS), a veno-occlusive disorder resulting in hepatic outflow obstruction, hepatic congestion, liver failure, or portal hypertension.1,2 These options include surgical portocaval shunts,3 angioplasty or transjugular intrahepatic portosystemic shunt,4,5 and liver transplantation (OLT).6 – 8 Traditional teaching suggests that OLT should be offered only after failure of conventional therapy, such as anticoagulation, diuretics, or portosystemic shunts.9,10 Therapeutic strategies depend on the functional status of the liver. Patients with cirrhosis, malignancy, or hepatic encephalopathy warrant consideration for OLT. Available data for OLT performed for BCS show excellent long-term survival and patency.6 – 8 In cadaveric donor OLT, the inferior vena cava (IVC) is generally replaced
V
© 2010 Published by Elsevier Inc. 360 Park Avenue South, New York, NY 10010-1710 Transplantation Proceedings, 42, 839 – 842 (2010)
with the donor IVC, because the donor liver graft contains the segment of IVC and hepatic veins. In contrast, in living donor liver transplantation (LDLT), specific venous drainage procedures must be devised regarding the level and size of the stenotic or the obliterated retrohepatic IVC. In this report, we have discussed our results and presented a literature review of surgical techniques and outcomes in LDLT for BCS patients. From the Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. Address reprint requests to Suk-Koo Lee, MD, PhD, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 135-710, Korea. E-mail: [email protected] 0041-1345/10/$–see front matter doi:10.1016/j.transproceed.2010.02.045 839
840
CHOI, PARK, JUNG ET AL Table 1. Donor and Recipient Demographics Patient
Gender Age (y) MELD Donor age and gender Viral hepatitis Clinical presentation Previous intervention Hepatocellular carcinoma (largest tumor size, tumor number) Graft to body weight ratio Liver weight (g) Follow-up (mos)
#1
Female 44 7 32 M None Uncontrolled ascites TACE* Yes (1.5 cm, #1) 1.43% 884 24
#2
#3
#4
Male 45 12 17 M None Variceal bleeding EVL No
Female 50 11 23 M HBV Uncontrolled ascites TACE Yes (2 cm, #2)
Female 41 15 44 F None HCC TACE Yes (2.1 cm, #4)
0.78% 2400 18
1017 13
1.2%
1.1% 944 23
Abbreviations: TACE, transarterial chemoembolization; EVL, endoscopic variceal ligation.
PATIENTS AND METHODS During a 5-year period at our institution (January 2003 to December 2007), we performed LDLT in 377 patients including 4 (1.06%) with a primary diagnosis of BCS: 1 male and 3 females. Their mean age was 44 years (Table 1). Three patients were transplanted as a primary procedure. One patient had undergone surgical treatment using a mesoatrial shunt with a ringed PTFE graft 10 years before OLT (Fig 1A). Patients were approved for LDLT after complete evaluation by our liver transplant selection committee. All 4 patients received right lobe grafts. All donors underwent a complete preoperative evaluation, which involved a comprehensive medical examination including laboratory values, ultrasonography, computed tomography (CT) with reconstruction, magnetic resonace (MR) imaging with MR cholangiography, volumetry and psychiatric clearance. The donor hepatectomy was performed in standard fashion through a right subcostal incision with left and upper midline extensions. The recipient work-up included laboratory values, ultrasonography, and computed tomography (CECT with reconstruction). Recipient hepatectomy was performed through a bilateral subcostal incision with an upper midline extension. The portal high hilar dissection was described previously.11 The preservation of recipient IVC was achieved by meticulous dissection of the caudate lobe from the IVC, despite the presence of many short hepatic veins between the caudate lobe and the retrohepatic IVC. Portal venous and arterial anastomoses were completed in standard fashion. A duct- to-duct biliary anastomosis was done with a combination of a posterior running and an anterior interrupted row of absorbable sutures. Posttransplant immunosuppression included induction with basiliximab, and maintenance with tacrolimus, mycophenolate mofetil, and steroid. Steroid was weaned at 4 months after transplantation. In the postoperative period, color Doppler ultrasound was performed on days 1 and 5. 99m Tc-DISIDA scan and CT angiography were performed as per our routine OLT protocol on day 14.
2 showed venous outlet obstruction at the level of the major hepatic veins but the IVC was patent. The orifice of the obliterated right hepatic vein was resected with freshening
RESULTS
The demographic features of the recipients and donors are listed in Table 1. There were no significant donor complications. Three patients had hepatocellular carcinoma and 1, a history of variceal bleeding with endoscopic treatment. No patient had evidence of hypercoagulopathy. Patients 1 and
Fig 1. (A) Pretransplant CT angiography reconstruction of patient with previous mesoatrial shunt. (B) Posttransplant CT follow-up focusing to hepatic venous drainage.
LDLT AND BUDD-CHIARI SYNDROME
of the adjacent IVC for anastomosis of the right hepatic vein of the graft to the IVC in end to side fashion using the piggy-back method without venovenous bypass. In the case of patient 3, we detected a 2.5-cm obliteration of the retrohepatic IVC around the orifice of hepatic veins. After caval sparing during total hepatectomy, the obliterated IVC was dissected, removed and interposed with a cryopreserved cadaveric IVC graft. Due to the presence of extensive collateral vessels a venovenous bypass was not needed (Fig 2). Patient 4 had a history of surgical treatment using a mesoatrial shunt from the superior mesenteric vein to the right atrium using a ringed PTFE graft 10 years prior as primary treatment for BCS (Fig 1A). The liver and the retrohepatic IVC, which was totally obliterated, were removed and the previous mesoatrial shunt resected near the mesenteric vein, and then closed. The hepatic vein of the right lobe graft was anastomosed to the PTFE graft to established drainage to the atrium through the previous mesoatrial shunt graft (Fig 1B). Immediate postoperative anticoagulation utilized low-molecular-weight heparin, which was later converted to long term therapy with anti-platelet agents only. In all cases, follow-up Doppler and CT scans revealed that all vascular structures were patent. Graft and patient survival was 100%. Postoperatively, 1 patient displayed a biliary stricture, which was managed with endoscopic retrograde cholangiopancreatographic dilatation and stent placement. Another patient display cytomegalovirus infection requiring readmission for ganicclovir treatment.
Fig 2.
841
DISCUSSION
Patients with BCS, who display a poor quality of life secondary to venous obstruction, hepatomegaly, decompensated liver function, fulminant hepatic failure or hepatocellular carcinoma qualify for OLT in the current Model for End-Stage Liver Disease system. OLT for BCS was first performed in 1974. Since then, several series and registry analyses including 2 recent large series have documented the European and US experiences of liver transplantation for BCS.6 – 8 In patients with certain synthetic deficiencies, such as antithrombin III or protein C, liver transplantation offers the additional advantage of curing the genetic defects. Patients with BCS have multiple therapeutic options that may not be available to other patients on the transplant waiting list. Although liver transplantation has emerged as an increasingly safe therapeutic option for patients with BCS, its application should not be used liberally. Most literature on OLT for BCS concerns DDLT. Surgical techniques and long-term results of DDLT have reported 5-year survival rates of 45%–95%; BCS has been reported to recur in 0%–27% of cases.6 – 8 The deceased donor graft has an IVC segment with hepatic veins. Consequently one can apply techniques involving removal of the retrohepatic IVC as part of the donor hepatectomy and the cavocaval anastomosis in the recipient. Depending on the status of the collaterals, the standard operative technique of DDLT in BCS was used with or without a venovenous bypass procedure.
Interposed cyroperserved cadaveric vena caval graft and anastomosis of graft.
842
However, surgical techniques and long-term follow-up are lacking for LDLT in BCS; experiences have been mostly restricted to case reports only. From a technical perspective, LDLT provides substantial challenges in the setting of BCS. Several technical considerations like caudate lobe enlargement; diffuse fibrotic reactions in the retroperitoneum, which increase the complexity of steps requiring control of the IVC; portal vein thickening and thrombosis; as well as increased bleeding and subsequent hemostasis make LDLT challenging in these patients. In LDLT, the piggy-back technique is used with preservation of the recipient IVC. The anastomosis between the recipient hepatic vein cuff and the graft hepatic vein is performed using a partial liver graft in the absence of a donor IVC segment. Stenosis associated with BCS has been classified by Yamada et al into 2 types;8 partial or complete occlusion. In the latter, there is an absence of pressure gradient differences across the area of stenosis due to well-developed hemi-azygous veins, and the other is partial occlusion with the presence of pressure gradient differences across the area of stenosis. The latter is distinguished by the existence of a clinical symptom, such as ascites, lower extremity edema, and renal dysfunction. This classification correlates with surgical procedures. The key considerations in the surgical procedure for LDLT among patients with BCS is the management of a stenosed or occluded IVC, and the choice of techniques to reconstruct the graft hepatic veins.8 In situations where large pressure differences exist before and after the stenosis, lower extremity edema, distended abdominal flank, and back veins and albuminuria may occur. Patch plasty for IVC stenosis was recommended by Yamada et al.8 They described the necessity for the IVC patch plasty depending on the site and extension of the IVC defects after thrombectomy and resection of the dense fibrotic wall of the IVC. We were able to resect the hepatic vein cuff and adjacent fibrotic area, then performing a direct anastomosis to the IVC in patients 1 and 2. We did not need a venous patch plasty, due to partial resection of the IVC with a subsequent wide anastomosis hence minimizing the chances of a recurrence. IVC replacement becomes necessary when the degree of IVC stenosis or obstruction is extensive or when a metallic stent has been implanted into the diseased IVC during a previous intervention.12 In cases of complete occlusion of the IVC, resection of the segment with placement of a cryopreserved autologous graft is an option. In patient 3 the strictured fibrosed IVC was replaced by an autologous graft. If the retroperitoneum is severely scarred and fibrosed, and resection of the IVC is technically risky, a possible option could be a direct RHV-left atrial shunt. We realized this option in patient 4, who had a preexistent meso-atrial shunt. Here, the mesenteric end of the previous meso-atrial shunt was disconnected, and the RHV of the graft was anastomosed to the PTFE shunt, hence achieving direct drainage to the left atrium. This RHV-atrial PTFE graft
CHOI, PARK, JUNG ET AL
showed good long-term patency at 2 years. A direct RHVleft atrial shunt can be an option for patients with preexisting meso-atrial shunts or when IVC excision is technically risky due to local conditions. Kuang et al13 reported unfavorable long-term results of using cryopreserved vein grafts for portal vein and hepatic artery replacement due to complications including aneurysm, thrombosis, and stricture. However, the long-term patency of autologous grafts has not been reported for vein replacement. Yamada et al8 presented 9 patients with BCS who were treated with LDLT. This series reported 88% 1- and 71% 3-year survivals, with 2 patients suffering recurrent BCS. We have had similar results with no recurrences at a mean follow-up of 20 months; these results are comparable to DDLT in BCS. In conclusion, LDLT may be performed safely for patients with BCS with suitable modifications in the surgical technique depending on the type of BCS and the local conditions. Long-term anticoagulant therapy and good follow-up for early diagnosis and interventional radiologic treatment of recurrent BCS can yield excellent outcomes after LDLT for BCS. REFERENCES 1. Menon KV, Shah V, Kamath PS: The Budd-Chiari syndrome. N Engl J Med 350:578, 2004 2. Klein AS: Management of Budd-Chiari syndrome. Liver Transpl 12:S23, 2006 3. Murad SD, Valla DC, de Groen PC, et al: Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 39:500, 2004 4. Molmenti EP, Segev DL, Arepally A, et al: The utility of TIPS in the management of Budd-Chiari syndrome. Ann Surg 241:978, 2005 5. Eapen CE, Velissaris D, Heydtmann M, et al: Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut 55:878, 2006 6. Segev DL, Nguyen GC, Locke JE, et al: Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl 13:1285, 2007 7. Mentha G, Giostra E, Majno PE, et al: Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol 44:520, 2006 8. Yamada T, Tanaka K, Ogura Y, et al: Surgical techniques and long-term outcomes of living donor liver transplantation for BuddChiari syndrome. Am J Transplant 6:2463, 2006 9. Campbell DA, Rolles K, Jamieson N, et al: Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome. Surg Gynecol Obstet 166: 511, 1988 10. Shaked A, Goldstein RM, Klintmalm GB, et al: Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. SGO 174:453, 1992 11. Lee KW, Joh JW, Kim SJ, et al: High hilar dissection: new technique to reduce biliary complication in living donor liver transplantation. Liver Transpl 10:1158, 2004 12. Shimoda M, Marubashi S, Dono K, et al: Utilization of autologous vein graft for replacement of the inferior vena cava in living-donor liver transplantation for obliterative hepatocavopathy. Transpl Int 20:804, 2007 13. Kuang AA, Renz JF, Ferrell LD, et al: Failure patterns of cryopreserved vein grafts in liver transplantation. Transplantation 62:742, 1996