Living donor liver transplantation for Budd–Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures

Living donor liver transplantation (LDLT) for Budd–Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient’s suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5 years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation.     

Living Donor Liver Transplantation in Budd-Chiari Syndrome: A Single-Center Experience

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.     

活体肝移植治疗布加综合征并下腔静脉狭窄

目的 报道笔者利用尸体下腔静脉（IVC）替代受体肝后IVC施行成人间活体肝移植（LDLT）治疗布加综合征（BCS）并IVC狭窄的经验。方法 1例35岁男性BCS并肝后IVC狭窄的患者，曾接受内科治疗，并于9个月前经放射介入置入金属扩张器，但症状无缓解。最终患者被施行了成人间LDLT，术中采用了尸体IVC替代受体肝后IVC进行重建。结果 患者术后过程平稳，效果满意。结论 笔者认为采用LDLT及利用尸肝IVC重建受者肝后IVC治疗BCS并肝后IVC狭窄的术式可推荐作为一种新的术式。     

Surgical Techniques and Long-Term Outcomes of Living Donor Liver Transplantation for Budd-Chiari Syndrome

We have developed the surgical techniques of living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) and evaluated long-term outcomes including specific complications. BCS is characterized by hepatic outflow obstruction. Liver transplantation from living donors poses a unique challenge as liver replacement therapy does not replace the retrohepatic segment of inferior vena cava (IVC). We have performed 1105 LDLTs in 1055 patients from January 1990 to March 2005. Of these, nine patients (eight males and one female) underwent LDLT for BCS. Five out of nine patients underwent LDLT as a primary procedure and four patients had received other treatments before transplantation. Eight patients presented with chronic and one with fulminant liver failure. Predisposing factors were identified in three patients. IVC reconstruction without patch plasty was performed on four patients. Five patients needed cavoplasty using a replacement vein graft. Of the nine patients, seven are alive at a median follow-up of 58 months (range 1 month to 15.2 years) with two patients developing recurrent hepatic vein stenosis which were treated successfully with metallic stent placement. Two patients died: one from multiorgan failure and the other from pulmonary embolism secondary to disease recurrence. LDLT for BCS is highly effective by using modified cavoplasty and provides good long-term survival which may be obtained by life-long anticoagulant treatment and nonsurgical interventions.     

Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and associated antiphospholipid antibody syndrome

BACKGROUND: Recently, the antiphospholipid antibody syndrome (APLS) has been recognized as the cause of the Budd-Chiari syndrome (BCS) in adults. However, APLS-induced BCS has been seen rarely in children. The surgical strategy for BCS depends on the patency of the inferior vena cava (IVC) and on the primary disease. METHODS: A 10-year-old boy with a diagnosis of BCS complicated with IVC obstruction caused by APLS received medical treatment and radiologic intervention for 2 years. In spite of these treatments, no relief of the symptoms could be achieved. Finally, the patient underwent living donor liver transplantation (LDLT), which required cavoplasty. He has had an uneventful course since the LDLT. CONCLUSIONS: IVC-obstructed BCS associated with APLS seems to be a good indication for LDLT with cavoplasty. Because liver transplantation (LT) itself is not a cure for APLS, the risk of thrombosis cannot be eliminated entirely by LT. Although immunosuppression may influence antiphospholipid antibody production, long-term observation and life-long anticoagulant treatment is needed even after LT. J Pediatr Surg 36:659-662.     

New Left Lobe Transplantation Procedure with Caval Reconstruction Using an Inverted Composite Graft for Chronic Budd-Chiari Syndrome in Living-Donor Liver Transplantation—A Case Report

When the Budd-Chiari syndrome (BCS) lesion extends to the inferior vena cava (IVC) or the orifices of the hepatic vein, the thickened IVC and/or hepatic vein wall must be removed and IVC reconstruction is required in living-donor liver transplantation (LDLT). In various reports about IVC resection in LDLT for BCS, there are none about left lobe liver transplantation with reconstruction of the retrohepatic IVC (rhIVC). To overcome removal and reconstruction of the rhIVC in LDLT for BCS, we introduced a composite IVC graft that is applicable to both right and left lobe partial liver grafts for LDLT for BCS. Pathogenic IVC was removed together with the native liver between the lower edge of the right atrium and 5 cm above the renal vein junction with the use of venovenous bypass. The e-polytetrafluoroethylene graft was anastomosed to the suprarenal intact IVC. Then the native part was detached at the level of just above the renal junction. The composite graft was inverted and a half rim of the native part of the graft was anastomosed to the posterior wall of the right atrium. Next, the common venous orifice of the left lobe graft was anastomosed to the wall defect which was composed of the anterior wall of the right atrium and the distal end of the native part of the composite graft. In conclusion, our inverted composite graft technique will overcome the weak points of LDLT for BCS, such as incomplete removal of the pathogenic caval wall and reconstruction of the rhIVC.     

Novel rescue procedure for inferior vena cava reconstruction in living‐donor liver transplantation using a vascular graft recovered 25 h after donors' circulatory death and systematic review

Liver transplantation is a lifesaving treatment for patients suffering from end‐stage liver disease. Rarely, acute congestion of the inferior vena cava (IVC) is being encountered because of tumor compression. MELD allocation does not reflect severity of this condition because of lack of organ failure. Herein, a patient is being presented undergoing urgent living‐donor liver transplantation (LDLT) with IVC reconstruction for a fast‐growing hepatic epithelioid hemangioendothelioma (HEH). IVC reconstruction using a venous graft recovered from a 25‐h after circulatory‐death prior transplantation became necessary to compensate severe venous congestion. Additionally, a systematic review of the literature searching MEDLINE/PubMed was performed. Protocol and eligibility criteria were specified in advance and registered at the PROSPERO registry (CRD42013004827). Published literature of IVC reconstruction in LDLT was selected. Two reports describing IVC reconstruction with cryopreserved IVC grafts and one IVC reconstruction using a deceased after‐circulatory‐death‐donor IVC graft were included. Follow‐up was at 12 and 13 months, respectively. Regarding the graft recovery in the setting of living‐related donation, this graft remained patent during the nine‐month follow‐up period. This is the first report on the use of a venous graft from a circulatory‐death‐donor, not eligible for whole organ recovery. We demonstrate in this study the feasibility of using a size and blood‐group‐compatible IVC graft from a cold‐stored donor, which can solve the problem of urgent IVC reconstruction in patients undergoing LDLT.     

Right lobe living-donor hepatectomy—the Toronto approach,tips and tricks

Living-donor liver transplantation (LDLT) is a well-established treatment for end-stage liver disease. Nevertheless, it has not been extensively accepted in North America or Europe as it has been in Asia. At the University of Toronto we initiated our LDLT program in 2000 and since then our program has grown each year, representing today the largest LDLT program in North America. Our right-lobe LDLT experience from 2000−2014 includes 474 right lobes. Only 30% of our grafts have included the middle hepatic vein. We present excellent outcomes in terms of graft and patient survival which is not different to that achieved with deceased donor liver transplantation. In the present study we will discuss the evolution, challenges and current practices of our LDLT program. We will discuss what is and has been the program philosophy. We will also discuss how we evaluate our donors and the extensive workup we do before a donor is accepted for live donation. Furthermore we will discuss some tips and tricks of how we perform the right hepatectomy for live donation.     

Using ileocolic artery for successful graft salvage in a recipient with hepatic artery thrombosis after living donor liver transplantation: case report

Hepatic artery (HA) occlusion is a sinister complication after liver transplantation. It frequently leads to graft loss if untreated. Urgent arterial reconstruction with thrombectomy may reduce the need for retransplantation. Living donor liver transplantation (LDLT) offers further challenges due to smaller-caliber vessels, shorter vascular stumps, and occasional multiple HA. Alternatives to the HA are needed when the native HA cannot be used or when HA complications develop. We describe the use of the recipient's ileocolic artery as an alternate HA in adult LDLT. Graft revascularization and timely salvage resulted in good patient recovery. A 6-month computed tomography angiography follow-up showed patency of the alternate vessels reconstructed.     

No-Touch En Bloc Right Lobe Living-Donor Liver Transplantation with Inferior Vena Cava Replacement for Hepatocellular Carcinoma Close to Retrohepatic Inferior Vena Cava: Case Report

Current studies have shown that living-donor liver transplantation (LDLT) for hepatocelluar carcinoma (HCC) satisfying the Milan criteria does not compromise patient survival or increase HCC recurrence compared with deceased-donor liver transplantation (DDLT). For patients with HCC beyond the Milan criteria, however, worse outcomes are expected after LDLT than after DDLT, despite insufficient data to reach a conclusion. Regarding operative technique, LDLT might be a less optimal cancer operation for HCC located at the hepatic vein confluence and/or paracaval portion. The closeness to the wall of the retrohepatic inferior vena cava (IVC) is greater than in conventional DDLT, rendering it difficult to perform a no-touch en bloc total hepatectomy. An LDLT, which must preserve the native IVC for the piggyback technique during engraftment, may lead to tumor remnants. To reduce recurrences after LDLT, we successfully performed a no-touch en bloc total hepatectomy including the retrohepatic IVC and all 3 hepatic veins. IVC replacement with an artificial vascular graft together with a modified right-lobe LDLT was performed for a patient having advanced HCC close to the hepatic vein confluence and paracaval portion. There was no artificial vascular graft-related complication, such as thrombosis or infection. Despite the limitations of LDLT, requiring the piggyback technique for graft implantation, IVC replacement using an artificial graft led us to perform a no-touch en bloc total hepatectomy as with a conventional DDLT.     

Clinical outcomes of living donor liver transplantation for hepatitis C virus (HCV)-positive patients

BACKGROUND: Whether hepatitis C virus recurrence occurs earlier and with greater severity for living donor liver transplantation (LDLT) than for deceased donor liver transplantation (DDLT) has recently become a subject of debate. METHODS: We retrospectively evaluated clinical outcomes for a cohort of 91 HCV-positive patients who underwent LDLT at Kyoto University with a median follow-up period of 25 months. RESULTS: Overall 5-year patient survival for HCV patients was similar to that for non-HCV patients (n=209) who underwent right-lobe LDLT at our institute (69% vs. 71%). Survival rate of patients without HCC (n=34) tended to be better than that of patients with HCC (n=57) (82% vs. 60%, P=0.069). According to annual liver biopsy, rate of fibrosis progression to stage 2 or more (representing significant fibrosis) was 39% at 2 years after LDLT. Univariate analysis showed that female recipient and male donor represented significant risk factors for significant fibrosis. Progression to severe recurrence (defined as the presence of liver cirrhosis (F4) in a liver biopsy and/or the development of clinical decompensation) was observed in five patients. CONCLUSIONS: Postoperative patient survival was similar for HCV-positive and -negative recipients in our adult LDLT series. Rates of progression to severe disease due to HCV recurrence seemed comparable between our LDLT recipients and DDLT recipients described in the literature. Although longer-term follow-up is required, our results suggest that LDLT can produce acceptable outcomes also for patients suffering from HCV-related cirrhosis.     

Outflow block secondary to stenosis of the inferior vena cava following living-donor liver transplantation?

Although it is well known that outflow block is caused by stenosis or occlusion of hepatic vein anastomoses following living donor liver transplantation (LDLT), there have been few reports on inferior vena cava (IVC) stenosis following LDLT. In this paper, we report two cases of IVC stenosis and hepatic vein outflow block following right hepatic LDLT in the absence of stenosis of any of the anastomoses. Both patients presented with liver dysfunction, an ascitic fluid volume of approximately 2000 mL, and congestion in their biopsy specimens, and venocavography demonstrated IVC stenosis with gradients of more than 10 mmHg in patients with a dominant inferior right hepatic vein (IRHV) anastomosis. After a Gianturco expandable metallic stent successfully implanted in the IVC, the patient's liver function recovered and the volume of ascitic fluid decreased. The pathogenesis of hepatic vein outflow block secondary to IVC stenosis following LDLT may involve the anastomosis with the IRHV, which is the dominant draining vein of the graft and larger than the RHV, caudal to the IVC stenosis and a significant IVC pressure gradient that results in increased IRHV pressure. In conclusion, it is important to include hepatic vein outflow block in the differential diagnosis when patients who have undergone right hepatic LDLT in which anastomosis of the large IRHV has been performed develop manifestations of liver dysfunction.     

Toward 300 liver transplants a year

The technical success of cadaveric whole-size liver transplantation and better immunosuppressive drugs has extended the application of this life-saving procedure to include patients with irreversible acute and chronic liver diseases. However, because of the scarcity of cadaveric liver grafts, living-donor liver transplantation (LDLT) has emerged as an alternative to cadaveric-donor liver transplantation (CDLT), especially in Asia. In Korea, 8% of the population are hepatitis B virus (HBV) carriers, and the resultant HBV cirrhosis, with or without hepatocellular carcinoma (HCC), is common in the 40- to 60-year-old generation. Accordingly, many patients require orthotopic liver transplantation (OLT). In 1992, we started performing CDLTs in the Asan Medical Center. In 1994, the first successful pediatric LDLT was performed in Korea, on a 9-monthold infant with biliary atresia. In 1997, the first successful adult LDLT was performed in our department, using a left lobe, on a 37-year-old patient with HBV cirrhosis associated with HCC. Even after the first successful right-lobe LDLT, we faced the obstacle of anterior segment congestion of a right-lobe graft, and initiated reconstruction of the middle hepatic venous tributaries of a right-lobe graft in 1998. In 1999, we performed more than 100 OLTs a year. Insufficient graft size has hindered the expansion of adult LDLT, when the remaining left-lobe of potential donors is too small to assure donor safety. Dual two-left-lobe graft LDLT (transplanting from two donors into one recipient) was developed in 2000 to solve graft-size insufficiency and minimize donor risk. More than 200 OLTs a year have been performed since 2004, while broadening the indications for adult LDLT to near complete obstruction of the portal vein, with the application of intraoperative portography (IOP) and portal vein stenting. In 2007, 320 LTs were performed, including 276 adult LDLTs, 10 pediatric LDLTs, and 34 CDLTs (including 7 adult and 1 pediatric split-liver transplant). There has been no donor mortality in LDLT. With technical refinement and advanced perioperative care, the in-hospital mortality of recipients has dropped to 4%: attributed to the dedication of our liver transplantation team members.     

肝细胞癌合并下腔静脉癌栓的手术治疗

目的 探讨肝细胞癌(简称肝癌)合并下腔静脉癌栓的手术治疗方法。方法 采用肝切除 腔静脉取栓治疗4例肝癌合并下腔静脉癌栓患者,取栓方法包括经荷栓肝静脉取栓(1例)和下腔静脉切开取栓(3例),后者又分在全肝血流阻断下取栓(2例)和在萨氏钳局部血管阻断下取栓(1例)。结果 4例肝癌及下腔静脉癌栓均得到成功切除,术中无明显并发症发生;术后除l例发生中等量胸水外,无其他并发症发生;随访中3例已死亡,分别生存30、10和14个月;1例尚存活,已生存7个月。结论 肝癌合并下腔静脉癌栓的手术治疗安全可行,其基本术式为肝切除 下腔静脉切开取栓。     

Successful Living-Donor Liver Transplantation With Intraoperative Endovascular Recanalization via Transsplenic Access in a Recipient With Grade III Portal Vein Thrombosis: A Case Report

Extensive portosplenomesenteric thrombosis is regarded as a relative contraindication to liver transplantation because of the complexity of the surgical procedure. This report describes a case of living-donor liver transplantation (LDLT) for a patient with extensive portosplenomesenteric thrombosis, in whom portal flow was successfully restored by intraoperative transplenic portal vein and superior mesenteric vein stenting after surgical thrombectomy. The patient’s liver function remained normal with a patent portal vein stent 6 months after LDLT, and Doppler ultrasonography demonstrated a normal wave form for portal flow. To the best of our knowledge, this is the world’s first case of endovascular management of the portal vein via percutaneous transsplenic access during LDLT, demonstrating that transsplenic access can be an alternative approach without liver graft injury when the superior mesenteric vein branch and inferior mesenteric vein cannot be used as access routes.     

Endovascular interventions for hepatic artery complications immediately after pediatric liver transplantation

Hepatic artery complications after living donor liver transplantation (LDLT) can directly affect both graft and recipient outcomes. For this reason, early diagnosis and treatment are essential. In the past, relaparotomy was generally employed to treat them. Following recent advances in interventional radiology, favorable outcomes have been reported with endovascular treatment. However, there is ongoing discussion regarding the best and safe time for definitive endovascular interventions. We herein report a retrospective analysis for six children with early hepatic artery complication after pediatric LDLT who underwent endovascular treatment as primary therapy at our institution. We evaluate the usefulness of endovascular treatment for hepatic artery complication and its optimal timing. The mean patient age was 11.9 months and mean body weight at LDLT was 6.7 kg. The mean duration between the transplantation and first endovascular treatment was 5.3 days. Five of the six patients were technically successful treated by only endovascular treatment. Of these five patients, two developed biliary complications. Endovascular procedures were performed 10 times in six patients without any complications and nine of the 10 procedures were successful. By selecting optimal devices, our findings suggest that endovascular treatment can be feasible and safe in the earliest time period after pediatric LDLT.     

Impact of human leukocyte antigen mismatching on outcomes of living donor liver transplantation for primary biliary cirrhosis.

Patient selection criteria of deceased donor liver transplantation for primary biliary cirrhosis (PBC) are almost completely established. The aim of this study was to establish selection criteria for both patients and donors of living donor liver transplantation (LDLT) for PBC. We used univariate and multivariate analyses to examine patient and donor characteristics of our first 50 cases of LDLT for PBC to elucidate factors that significantly impacted patient survival or disease recurrence after LDLT in the univariate and/or multivariate analyses. Multivariate analysis demonstrated that the presence of persistent ascites before LDLT, a higher number of human leukocyte antigen (HLA)-A, -B, and -DR mismatches between donor and recipient, and donor age >or=50 years were factors significantly associated with early posttransplant death. Independent risk factors for PBC recurrence after LDLT were a lower number of HLA mismatches between donor and recipient, and a lower average trough level of tacrolimus within 1 year after LDLT. Specifically, the lower the number of HLA-A, -B, and -DR mismatches or the average trough level of tacrolimus within 1 year after LDLT, the higher the possibility of developing a recurrence of PBC. In conclusion, the absence of persistent ascites before LDLT, a lower number of HLA-A, -B, and -DR mismatches between donor and recipient, and a younger donor (<50 years) are preferred for gaining acceptable survival outcomes for the transplant. However, a lower number of HLA-A, -B, and -DR mismatches between donor and recipient may be a risk factor for PBC recurrence.     

Utilization of autologous vein graft for replacement of the inferior vena cava in living-donor liver transplantation for obliterative hepatocavopathy

Obliterative hepatocavopathy (OHC) is a subtype of Budd-Chiari syndrome in which stenosis or obstruction of the retrohepatic inferior vena cava (IVC) is observed. Although IVC replacement is necessary in OHC patients, there are hardly any graft vessels available for IVC reconstruction during living-donor liver transplantation (LDLT). Here, we describe a novel technique of IVC reconstruction using only the autologous blood vessels in an OHC patient during LDLT. In this case, sufficient drainage of the hepatic outflow and reconstruction of the venous return from the lower half of the body were simultaneously required. Therefore, we substituted the retrohepatic IVC with the suprarenal IVC of the recipient, and we reconstructed the IVC continuity by using the autologous internal jugular vein and external iliac vein. The operation was safe, and the postoperative venous drainage from the hepatic tributaries was in good condition. This procedure might be an option for IVC replacement during LDLT.     

The impact of meticulous management for hepatic artery thrombosis on long‐term outcome after pediatric living donor liver transplantation*

Abstract: To analyze the risk factors in the development of hepatic artery thrombosis (HAT) and assess the impact of our perioperative management for HAT on the long‐term outcome after pediatric living donor liver transplantation (LDLT), we reviewed 382 patients under 12 yr of age who underwent 403 LDLT from January 1996 to December 2005. One‐ and 10‐yr patient survival rates were 78% and 78% in the patients with HAT (27 patients; 6.7%), and 84% and 76% in the patients without HAT, respectively (p = n.s.). Univariate analysis showed gender (female), body weight (lower), and graft‐to‐recipient weight ratio (higher) were significant risk factors in the patients with HAT (p < 0.05). Patients with Doppler ultrasound signal loss of the hepatic artery (HA) accompanied by an increase of liver enzymes underwent thrombectomy and reanastomosis (S‐group, n = 13), and patients with a weak HA signal underwent anticoagulant therapy (M‐group, n = 13). One patient underwent re‐LDLT. One‐ and five‐yr patient survival rates were 83% and 83% in the S‐group, and 77% and 77% in the M‐group (p = n.s.). The incidence of biliary complications in the S‐group (58%) was significantly higher than that of the M‐group (15%). For a successful long‐term outcome, the early detection of HAT and prompt medical and surgical intervention are crucial to minimize the insult of HAT.     

Whole-Liver Graft Without the Retrohepatic Inferior Vena Cava for Sequential (Domino) Living Donor Liver Transplantation

Grafts used in Domino liver transplantation (LT) obtained from living donor liver transplantation (LDLT) for familial amyloid polyneuropathy (FAP) patients have been mainly used as reduced grafts. Because of small-for-size problems seen in LDLT, using whole liver grafts could improve post-LT outcome. Eight consecutive Domino LDLT using whole livers without retrohepatic inferior vena cava (IVC) from FAP patients were retrospectively analyzed. The graft weight/recipient's body weight ratio (GWRW) in the domino recipients ranged from 1.28% to 2.4% (mean: 1.52). Multiple vascular reconstructions in the whole-liver domino LT resulted in longer than usual warm ischemia time (mean: 64 min); however immediate post-operative recovery of hepatic function was uneventful. At 8-40 months after the transplant, all the FAP patients are well and all of the domino recipients are alive. Domino LT using a whole FAP liver from a LDLT for a FAP patient presents satisfactory results, even though the transplant procedure is technically complicated.