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Surgical Outcomes of Domino Liver Transplantation Using Grafts From Living Donors With Familial Amyloid Polyneuropathy
Abstract
Domino liver transplantation (DLT) using grafts from donors with familial amyloid polyneuropathy is an acceptable procedure for expanding the donor pool. The vascular and biliary reconstructions in living donor DLT (LDDLT) are technically demanding, and data on the short- and long-term surgical outcomes of domino donors and recipients in LDDLT are limited. In this study, we identified 25 domino recipients from our LT program (1999-2018), analyzed the vascular and biliary reconstructions performed, and evaluated the surgical outcomes, including graft survival. Piggyback technique was adopted in all 25 domino donors. The only surgical complication in domino donors was HV stenosis with an incidence rate of 4%. In 22 domino recipients, right hepatic vein (HV) and middle/left HV were reconstructed separately. Ten recipients had two arteries anastomosed, and 18 underwent duct-to-duct biliary anastomosis. HV stenosis and biliary stricture had incidence rates of 8% and 24%, respectively, in the recipients, but none of them developed hepatic artery thrombosis. The 1- and 5-year graft survival rates were 100% each in the domino donors, and 84.0% and 67.3% in the domino recipients, respectively. In conclusion, LDDLT has acceptable outcomes without increasing the operative risk in donors despite the demanding surgical technique involved.
... In much of the literature reporting on the long-term outcomes in DLT, the first recipient (or domino donor) was transplanted using a liver from a deceased donor (8). Data on adult living donor DLT (LDDLT) is limited to one case series (9). ...
Background
Liver transplantation (LT) is a therapeutic option in multiple inherited metabolic diseases (IMDs), including methylmalonic acidemia (MMA), as LT reduces the risk of acute metabolic decompensations and long-term complications associated with these diseases. In certain IMDs, such as maple syrup urine disease (MSUD), domino liver transplant (DLT) is an accepted and safe method which expands the donor pool. However, only one adult case of DLT using an MMA donor liver has been reported; outcome and safety are still unknown and questioned.
Case Description
In this case report, we describe our experience with DLT using MMA livers. Two adult MMA patients underwent living donor liver transplant (LDLT); their MMA livers were consecutively transplanted into two patients on the liver transplant waiting list who had limited chance of receiving a liver transplant in the short term due to their low model for end-stage liver disease (MELD) scores. No severe peri- or postoperative complications occurred, however the recipients of the MMA livers biochemically now have mild MMA.
Conclusions
DLT using MMA grafts is a feasible strategy to treat end-stage liver disease and expand the donor organ pool. However, the recipient of the MMA domino liver may develop mild MMA which could affect quality of life, and long-term safety remains unclear. Further long-term of outcomes for domino recipients of MMA livers, focusing on quality of life and any metabolic complications of transplantation are needed to better define the risks and benefits.
... A domino transplant occurs when a patient with a metabolic disease (e.g., familial amyloidosis) receives a deceased liver donor transplant and in turn donates their liver to a patient with liver disease or malignancy. The donor liver in the patient with metabolic disease is functionally and morphologically normal, although the recipient carries the risk of acquiring the systemic disease (e.g., amyloidosis), which occurs in approximately 30% of patients at a mean of 8 years after transplant [13]. Alternatively, living donor liver transplants are increasingly performed in patients for whom this is an option and allows liver transplant independent of MELD score, overcoming the shortcomings of organ shortage and long wait-list times. ...
The most challenging and time-consuming step of transjugular intrahepatic portosystemic shunt (TIPS) procedures is obtaining appropriate portal vein access. Given the lack of real-time direct target visualization, conventional fluoroscopic guidance requires multiple passes, contributing to complications. In comparison, intravascular ultrasound (IVUS) guidance during TIPS procedures provides direct visualization of hepatic structures and real-time guidance for portal vein puncture. IVUS guidance during TIPS creation improves procedural metrics such as radiation dose, contrast agent volume, procedure time, and technical success rate, and is particularly beneficial in technically challenging cases (e.g., in patients portal vein thrombosis, small or variant portal vein anatomy, Budd-Chiari syndrome, or liver masses). The purpose of this review is to summarize current IVUS technology, describe technical aspects of IVUS-guided TIPS creation, and discuss clinical indications for and benefits of using IVUS for TIPS creation, while presenting available evidence supporting the technique's use. Given the improved safety profile and overall success rate in comparison with conventional guidance methods, IVUS guidance has the future potential to become the standard practice for TIPS placement.
Background and Aim
Domino liver transplantation (DLT) utilizes otherwise discarded livers as donor grafts for another recipients. It is unclear whether DLT has less favorable outcomes compared to deceased donor liver transplantation (DDLT). We aimed to assess the outcomes of DLT compared to DDLT.
Methods
MEDLINE, Embase, and Web of Science database were searched to identify studies comparing outcomes after DLT with DDLT. Data were pooled using random‐effects modeling, evaluating odds ratios (OR) or mean difference (MD) for outcomes including waiting list time, severe hemorrhage, intensive care unit (ICU), length hospital stay (LOS), rejection, renal, vascular, and biliary events, and recipient survival at 1, 3, 5, and 10 years.
Results
Five studies were identified including 945 patients ( DLT = 409, DDLT = 536). The DLT recipients were older compared to the DDLT group ( P = 0.04), and both cohorts were comparable regarding lab MELD, hepatocellular carcinoma, and waitlist time. There were no differences in vascular (OR: 1.60, P = 0.39), renal (OR: 0.62, P = 0.24), biliary (OR: 1.51, P = 0.21), severe hemorrhage (OR: 1.09, P = 0.86), rejection (OR: 0.78, P = 0.51), ICU stay (MD: 0.50, P = 0.21), or LOS (MD: 1.68, P = 0.46) between DLT and DDLT. DLT and DDLT were associated with comparable 1‐year (78.9% vs 80.4%; OR: 1.03, P = 0.89), 3‐year (56.2% vs 54.1%; OR: 1.35, P = 0.07), and 10‐year survival (6.5% vs 8.5%; OR: 0.8, P = 0.67) rates. DLT was associated with higher 5‐year survival (41.6% vs 36.4%; OR: 1.70; P = 0.003) compared to DDLT, which was not confirmed at sensitivity analysis.
Conclusion
This meta‐analysis of the best available evidence (Level 2a) demonstrated that DLT and DDLT have comparable outcomes. As indications for liver transplantation expand, future high‐quality research is encouraged to increase the DLT numbers in clinical practice, serving the growing waiting list candidates, with the caveat of uncertain de novo disease transmission risks.
Although traditionally only performed for hepatocellular carcinoma (HCC), the last decade has seen a resurgence in the use of liver transplant (LT) for non-HCC malignancies, likely due to improvements in neoadjuvant treatment regimens as well as the establishment of well-defined eligibility criteria. Given promising survival results, patients with perihilar cholangiocarcinoma, neuroendocrine liver metastases, and hepatic hemangioendothelioma are eligible to receive Model for End Stage Liver Disease (MELD) exception for tumors that meet well-defined criteria. Additional tumors such as colorectal cancer liver metastases, intrahepatic cholangiocarcinoma, and hepatocholangiocarcinoma may undergo transplant at specialized centers with well-defined protocols, although are not yet eligible for MELD exception. Transplant eligibility criteria commonly incorporate imaging findings, yet due to the relatively novel and evolving nature of LT for non-HCC malignancies, radiologists may be unaware of relevant criteria or of the implications of their imaging interpretations. Knowledge of the allocation process, background, and liver transplant selection criteria facilitates the radiologist' active participation in multidisciplinary discussion, leading to better and more equitable care for transplant candidates with non-HCC malignancy. This review provides an overview of transplant allocation and selection criteria in patients with non-HCC malignancy, with an emphasis on imaging features and the role of the radiologist.
Purpose
Occasionally, a recipient’s native hepatic arteries are not suitable for reconstruction in living donor liver transplantation (LDLT). The use of the great saphenous vein (GSV) conduits in such patients is seldom practiced since arterial conduits from deceased donors are available. Here, we share our experience with a significantly large group of LDLT recipients who underwent arterial reconstruction with GSV conduits.
Methods
We reviewed patients who underwent LDLT between 2012 and 2017. Patients who had arterial reconstruction using native hepatic arteries (group 1)(n = 452) were compared with those who had GSV interposition conduits for reconstruction (group 2)(n = 21). We compared hepatic artery thrombosis (HAT) rate, allograft dysfunction, morbidity, mortality, and actuarial 5-year survival in the two groups.
Results
HAT was seen in 0/452 (0%) versus 1/21(4.7%) patients (P = 0.04). Allograft dysfunction was seen in 89/423 (21%) versus 6/19(31.5%) (P = 0.2) patients. Overall mortality was 81/452 (17.9%) versus 8/21(38%) (P = 0.02). Death after a biliary complication was seen in 24/452 (5.3%) versus 4/21 (19%) patients (P = 0.02). Actuarial 1- and 5-year overall survival was 85% versus 67% and 79% versus 58% (P = 0.008).
Conclusion
GSV conduits are a suboptimal alternative for establishing hepatic arterial inflow in LDLT, but remain valuable in ominous situations.
Introduction
Domino liver transplantation (DLT) utilizes the explanted liver of one liver transplant recipient as a donor graft in another patient. While there may be unique risks associated with DLT, it is unclear if DLT has less favorable long term outcomes than deceased donor liver transplantation (DDLT). We used a propensity score matching approach to compare the outcomes of DLT recipients to DDLT recipients in a large national registry.
Methods
The United Network for Organ Sharing registry was queried for patients undergoing DLT or DDLT in 2002‐2016. Each DLT recipient was matched to a unique DDLT recipient to compare mortality and graft failure.
Results
There were 126 DLT and 62,835 DDLT recipients meeting inclusion criteria. After propensity score matching on recipient pre‐transplant characteristics, 123 DLT cases were matched to DDLT controls from the same region. On stratified Cox proportional hazards regression, DLT incurred no increase in the hazard of mortality (hazard ratio [HR] = 1.4; 95% confidence interval [CI]: 0.8, 2.7; p=0.265) or graft failure (HR = 1.2; 95% CI: 0.7, 2.1; p=0.556) compared to DDLT.
Conclusions
Using a large national registry, a propensity‐matched analysis found no increased risk of mortality or graft failure associated with DLT compared to DDLT.
This article is protected by copyright. All rights reserved.
Background:
The preservation of a graft's aberrant left hepatic artery (LHA) during liver transplantation (LT) ensures optimal vascularization of the left liver but can also be considered a risk factor for hepatic artery thrombosis. In contrast, ligation of an aberrant LHA may lead to hepatic ischaemia with the potential risk of graft dysfunction and biliary complications. The aim of this study was to prospectively analyse the impact on the surgical strategy for LT of 5 tests performed to establish whether an aberrant LHA was an accessory or a replaced artery, thus leading to the design of a decisional algorithm.
Methods:
From 8/2005 to 12/2016, 395 whole LTs were performed in 376 patients. Five parameters were evaluated to determine whether an aberrant LHA was an accessory or a replaced artery. Based on our decision algorithm, an aberrant LHA was ligated during surgery when assessed as accessory and preserved when assessed as replaced.
Results:
A total of 138 anatomical variants of hepatic arterial vascularization occurred in 120/395 (30.4%) grafts. Overall, the incidence of an aberrant LHA was 63/395 (15.9%). The LHA was ligated in 33 patients (52.4%) and preserved in 30 patients (47.6%). After a mean follow-up period of 46.2±35.9 months, the incidence of hepatic artery thrombosis, primary non-function, early allograft dysfunction, biliary stricture or leaks and overall survival was similar in the two groups.
Conclusions:
Once shown to be an accessory, an LHA can be safely ligated without clinical consequences on the outcome of LT. This article is protected by copyright. All rights reserved.
Although domino liver transplantation (LT) is an established procedure, data about the operative risks are limited. This study aimed at evaluating the operative risks of domino LT. Two retrospective analyses were conducted (comparison of familial amyloid polyneuropathy [FAP] liver donors [61 patients] vs. FAP nondonors [39 patients] and FAP liver recipients [61 patients] vs. deceased donor liver recipients [61 patients]). First analysis showed a 60-day mortality of 6.6% for FAP donors and 7.7% for FAP nondonors (p = 1.0). No patient developed primary graft nonfunction. Acute rejection was higher in FAP nondonors compared to FAP donors (38.5% vs. 13.1%). Both groups had similar vascular and biliary complication rates. ICU stay was similar, whereas total hospitalization was longer for FAP nondonors. Both groups had similar 1- and 5-year patient and graft survival rates (83.4% vs. 87.2%, and 79.8% vs. 71.8%, p = 0.7) and (83.3% vs. 87.2%, and 79.1% vs.71.8%, p = 0.7). The second analysis showed a 1.6% mortality for FAP liver recipients vs. 3.2% of the control group (p = 1). Both groups had similar morbidity and technical complication rates (18.0% vs. 13.1%, p = 0.45) and (0.18 vs. 0.15, p = 0.65). The domino procedure does not add any risk to FAP donor or recipient. It increases the organ pool allowing transplantation of marginal recipients who otherwise are denied deceased donor liver transplantation.
Portal vein thrombosis (PVT) has been seen as an obstacle to liver transplantation (LTx). Recent data suggest that favorable results may be achieved in this group of patients but only limited information from small size series is available. The present study was conducted in an effort to review the surgical options in patients with PVT and to assess the impact of PVT on LTx outcome. Risk factors for PVT and the value of screening tools are also analyzed.
Adult LTx performed from 1987 through 1996 were reviewed. PVT was retrospectively graded according to the operative findings: grade 1: <50% PVT +/- minimal obstruction of the superior mesenteric vein (SMV); grade 2: grade 1 but >50% PVT; grade 3: complete PV and proximal SMV thrombosis; grade 4: complete PV and entire SMV thrombosis.
Of 779 LTx, 63 had operatively confirmed PVT (8.1%): 24 had grade 1, 23 grade 2, 6 grade 3, and 10 grade 4 PVT. Being male, treatment for portal hypertension, Child-Pugh class C, and alcoholic liver disease were associated with PVT. Sensitivity of ultrasound (US) in detecting PVT increased with PVT grade and was 100% in grades 3-4. In patients with US-diagnosed PVT, an angiogram was performed and ruled out a false positive US diagnosis in 13%. In contrast with US, angiograms differentiated grade 1 from grade 2, and grade 3 from grade 4 PVT. Grade 1 and 2 PVT were managed by low dissection and/or a thrombectomy; in grade 3 the distal SMV was directly used as an inflow vessel, usually through an interposition donor iliac vein; in grade 4 a splanchnic tributary was used or a thrombectomy was attempted. Transfusion requirements in PVT patients (10 U) were higher than in non-PVT patients (5 U) (P<0.01). In-hospital mortality for PVT patients was 30% versus 12.4% in controls (P<0.01). Patients with PVT had more postoperative complications, renal failure, primary nonfunction, and PV rethrombosis. The overall actuarial 5-year patient survival rate in PVT patients (65.6%) was lower than in controls (76.3%; P=0.04). Patients with grade 1 PVT, however, had a 5-year survival rate (86%) identical to that of controls, whereas patients with grades 2, 3, and 4 PVT had reduced survival rates. The 5-year patient survival rate improved from the 1st to the 2nd era in non-PVT patients (from 72% to 83%; P<0.01), in grade 1 PVT (from 53% to 100%; P<0.01), and in grades 2 to 4 PVT (from 38% to 62%; P=0.11).
The value of US diagnosis in patients with PVT depends on the PVT grade, and false negative diagnoses occur only in incomplete forms of PVT (grades 1-2). The degree of PVT dictates the surgical strategy to be used, thrombectomy/low dissection in grade 1-2, mesoportal jump graft in grade 3, and a splanchnic tributary in grade 4. Taken altogether, PVT patients undergo more difficult surgery, have more postoperative complications, have higher in-hospital mortality rates, and have reduced 5-year survival rates. Analysis by PVT grade, however, reveals that grade 1 PVT patients do as well as controls; only grades 2 to 4 PVT patients have poorer outcomes. With increased experience, results of LTx in PVT patients have improved and, even in severe forms of PVT, a 5-year survival rate >60% can now be achieved.
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.
Domino liver transplantation using a whole familial amyloid polyneuropathy (FAP) liver from a LDLT for a FAP patient presents satisfactory results, even though the transplant procedure is technically complicated.
Background
Domino liver transplantation aims to address the need to increase the liver donor supply. In a domino liver transplant, the domino recipient receives the explanted liver from the recipient of a traditional liver transplant. The domino donor typically requires liver transplant to correct a metabolic disorder; the explanted liver thus has a single gene defect but otherwise normal structure and function.
Methods
In this review, we detail the history of domino liver transplantation, appropriate domino donor indications, the technical advances to the surgical approach, current outcomes, and future opportunities.
Results
Development of de novo disease in the domino recipient has relegated adult domino liver transplant to be considered a source of marginal donor livers. However, pediatric domino liver transplant has leveraged certain metabolic disorders, especially maple syrup urine disease, in which the liver enzyme deficiency can be compensated by the systemic presence of sufficient enzyme. Advances in the surgical aspects of assuring adequate length of vasculature have improved the safety of the procedure in both domino donors and recipients.
Conclusions
Pediatric domino liver transplant utilizing domino donors with specific metabolic liver diseases should be considered a viable live donor option for children awaiting liver transplant.
Introduction:
The aim of this study was to clarify the impact of middle hepatic artery reconstruction on the outcomes of duct-to-duct biliary anastomosis after living donor liver transplantation (LDLT) using the left lobe.
Materials and methods:
Among 258 patients who underwent LDLT using the left lobe, 216 patients who underwent hepatic artery reconstruction and one hepatic duct reconstruction with duct-to-duct interrupted anastomosis were divided into three groups: Group A (n = 123), one arterial stump with left hepatic artery reconstruction; Group B (n = 32), two arterial stumps with only left hepatic artery reconstruction; Group C (n = 61), two arterial stumps with reconstruction of the left and middle hepatic arteries. The outcomes after LDLT were compared among the three groups.
Results:
No hepatic artery complications occurred. Group B had a significantly greater incidence of anastomotic biliary stricture than Group C. A multivariate analysis with Cox regression revealed that being in Group B was the only significant independent risk factor for postoperative anastomotic biliary stricture after LDLT.
Conclusions:
Middle and left hepatic artery reconstruction is safe in LDLT and may prevent biliary stricture caused by dual hepatic artery reconstruction when the graft has left and middle hepatic artery stumps.
Orthotopic liver transplantation is an established treatment for end-stage liver diseases and for some severe metabolic disorders and hepatic cancers, but an organ shortage is the limiting factor in meeting the need for the procedure. Explanted livers from patients with metabolic liver diseases who are undergoing liver transplantation, so-called domino livers, can be one of the solutions to diminish the organ shortage. Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disease associated with a mutation of the TTR gene. The liver produces variant transthyretin (TTR) amyloid fibrils, which accumulate in body connective tissues and various organs such as the heart, kidney, and small intestine. The result is dysfunction of these organs, leading to severe disability. The patients die 9–15 years after the onset of symptoms, due to malnutrition or heart complications. Until recently, the only potentially curative treatment has been liver transplantation. Because the FAP liver is entirely normal, apart from producing the mutated variant TTR, these explanted livers are well suited to be used for transplantation in selected patients. Obviously, there is a risk of transmitting the FAP disease by the explanted liver, but given the slow progression of FAP and the desperate situation of the potential domino liver recipient, we believe that the benefits outweigh the risks [1]. The first domino liver transplantation (DLT) using the explanted liver from an FAP patient was performed in Portugal in 1995. With time, the procedure has evolved strongly, and the technique is today used worldwide with good outcome [2, 3].
Partial liver grafts used in living donor liver transplantation (LDLT) may have multiple hepatic artery (HA) stumps. This study was designed to validate the safety of partial reconstruction of multiple HAs in pediatric LDLT cases. From January 2000 to June 2014, 136 pediatric LDLT recipients were categorized into three groups: single HA group (Group 1, n = 74), multiple HAs with total reconstruction group (Group 2, n = 23), and multiple HAs with partial reconstruction group (Group 3, n = 39). Partial reconstruction was performed only when there was pulsatile back-bleeding after larger HA reconstruction and sufficient intrahepatic arterial flow was confirmed by Doppler ultrasound (DUS). There was no significant difference in biliary complication rate, artery complication rate, patient survival, and graft survival among these groups. Risk factor analysis revealed that the presence of multiple HAs and partial reconstruction of multiple HAs were not risk factors of biliary anastomosis stricture. In conclusion, partial reconstruction of HAs during pediatric LDLT using a left liver graft with multiple HA stumps does not increase the risk of biliary anastomosis stricture or affect graft survival when intrahepatic arterial communication is confirmed by pulsatile back-bleeding and DUS. This article is protected by copyright. All rights reserved.
Domino liver transplantation (DLT) with liver grafts from patients with hereditary transthyretin (TTR) amyloidosis has been performed throughout the world because of a severe liver graft shortage. Reports of acquired systemic TTR amyloidosis in domino liver recipients have been increasing; however, the precise pathogenesis and clinical course of acquired TTR amyloidosis remains unclear. We analyzed the relationship between the occurrence of acquired amyloidosis and clinical features in 22 consecutive domino liver donors with hereditary TTR amyloidosis (10 males and 12 females; mean age at DLT: 37.2 years; TTR mutations: V30M [n = 19], Y114C [n = 1], L55P [n = 1], and S50I [n = 1]) and 22 liver recipients (16 males and 6 females; mean age at DLT, 46.2 years). The mean times from DLT to amyloid first appearance and transplant recipient symptom onset were 8.2 years and 9.9 years, respectively. Kaplan-Meier analysis and quantification of the amyloid deposition revealed aging of recipients correlated with early de novo amyloid deposition. The sex of donors and recipients and the age, disease duration, and disease severity of donors had no significant effect on the latency of de novo amyloid deposition. In conclusion, our results demonstrate that recipient aging is associated with the early onset de novo amyloidosis. Because acquired amyloidosis will likely increase, careful follow-up for early amyloidosis detection and new treatments, including TTR stabilizers and gene-silencing therapies, are required.
Objectives:
The aim of this study was to evaluate the long-term outcome of liver transplantation (LT) for hepatocellular carcinoma (HCC) with Domino LT (DLT) using the "Double Piggy-back" technique.
Background data:
DLT using livers from familial amyloidotic polyneuropathy (FAP) patients is a well-described technique and useful for expanding the donor pool. However, data on long-term results for HCC are limited, particularly regarding the use of the "Double Piggy-back" technique.
Methods:
Between 2001 and 2014, a total of 260 patients undergoing LT for HCC were analyzed from a prospective database. Of those, 114 were submitted to DLT. Comparisons between groups were performed using propensity score matching.
Results:
Median follow-up was 34 months (1-152). Overall and disease-free 5-year survival rates for the whole population were 58% and 56%, respectively. There were 177 (68%) patients within Milan Criteria and an additional 26 (10%) within University of California San Francisco (UCSF) criteria. Patients older than 50 years were more likely to receive an FAP liver [odds ratio (OR) 1.94, confidence interval (CI) 1.02-3.69]. DLT patients had more major complications (23.7% vs 13.0%, P = 0.025). Only patients undergoing DLT presented with piggy-back syndrome (7% vs 0%, P = 0.001). After adjusting for potential confounders, DLT and cadaveric LT had a similar 5-year survival rate (59% vs 44%, respectively, P = 0.117). Thirteen patients (11.4%) evidenced FAP disease but not before 6 years after DLT.
Conclusions:
DLT for HCC is feasible and achieves equivalent results to cadaveric LT. The benefit of expanding the donor pool must be balanced against higher morbidity and a real risk of disease transmission.
Venous ouflow is critical to the success of liver transplantation(LT). In domino-LT the venous cuffs should be shared between donor and recipient and its length can be compromised. The aim of this study is to describe and compare the technical options for outflow reconstruction used in our institution.
Retrospective analysis of 39 consecutive domino-LT recipients between January 1997 and May 2013.
Twenty-seven men and 12 women(mean age 61.8±4.3 years), underwent LT and consented to receive a liver from a donor with familial amyloid polyneuropathy(FAP). The main indications were hepatocellular carcinoma and hepatitis C virus cirrhosis. All recipients were transplanted by piggy-back technique. Liver procurement in the FAP-donors was performed using the classical technique in 22 patients and the piggy-back technique in the last 17. In these latter cases, for vascular outflow reconstruction, a cadaveric venous graft was interposed between the hepatic veins(HV) stump of the FAP-liver and the recipient HV in 11 cases (28%). Since 2011, we have employed arterial grafts to be interposed between the vessels stumps, in 5 patients a tailored arterial graft and an aortic graft in one case. There was no postoperative mortality. Arterial and portal complications presented in 2(5.6%) and 4(11.1%) patients, respectively. Postoperative outflow complications(postLT subacute Budd-Chiari syndrome) occurred in 4 patients and all of them had received a venous interposed-graft for reconstruction. The incidence of outflow complications tended to be higher among patients with venous graft than with arterial graft interposition. Patient overall survival at 1,3,5 and 10 years was 97.1%,79.2%,76% and 67% respectively.
Arterial grafts constitute a feasible and safe option for vascular outflow reconstruction in domino-LT as they are associated to a relatively low incidence of complications. The recently proposed "Bellvitge" arterial graft technique should be added to the current range of available surgical modalities. This article is protected by copyright. All rights reserved.
© 2015 American Association for the Study of Liver Diseases.
Domino liver transplantation has been accepted as a safe procedure to further expand the organ donor pool. The most important technical challenge of the procedure resides in restoring a proper hepatic venous allograft outflow in the familial amyloidotic polyneuropathy-liver recipient. To overcome this issue, combined techniques were used to perform an innovative outflow reconstruction. A domino liver transplantation was successfully performed with reconstruction of complex venous outflow. The inferior vena cava sparing hepatectomy technique in the familial amyloidotic polyneuropathy-donor was used to cut the hepatic vein to the liver parenchyma. To overcome this issue the venous outflow tract was reconstructed using a longitudinally opened iliac vein graft from a post-mortem donor to create a new outflow tract using a diamond patch between the right and middle/left hepatic veins.
As a comment to Reichman et al., Professor Ohdan nicely reviewed the comparison of complications and outcome between living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) [1, 2]. However, one group of potential living donors was not commented on namely, transplantation using grafts from patients with metabolic liver diseases such as familial amyloidotic polyneuropathy (FAP)[3-5]. This domino liver transplantation (DLT) represents a significant relief to organ shortage in several regions in the world. The Familial Amyloidotic Polyneuropathy World Transplant Register (FAPWTR) (http://www.fapwtr.org) holds 1039 DLT performed in 21 countries until the end of 2011. This article is protected by copyright. All rights reserved.
A retrospective multicenter study has been conducted to evaluate domino liver transplantations (DLTs) in Germany. The study provides insight into survival and features having an impact on the assessment of neuropathy after DLT. In addition, a neurologic follow-up program with a scheme to estimate the likelihood of de novo amyloidosis is presented. A series of 61 DLTs at seven transplant centers in Germany was enrolled. The mean age of domino recipients at the time of transplantation was 58 years, 46 of them being men, and 15 being women. The median follow-up was 46 months. The overall 1-, 3-, and 5-year survival of domino recipients was 81.6%, 70.8% and 68.8%, respectively. Causes of death were primarily not related to familial amyloidosis. The main indication of DLT was hepatocellular carcinoma. Two of the reported domino recipients developed symptoms and signs of de novo amyloidosis within 10 years after transplantation. A total of 30 domino graft recipients (49.18%) presented with diabetes post transplantation. In conclusion, an advanced follow-up program is crucial to evaluate the risk of transmitting familial amyloidosis by DLT and to establish more strict selection criteria for domino recipients.
The status of domino liver transplantation (DLT) in Japan was evaluated. DLT and familial amyloidotic polyneuropathy (FAP) recipients who underwent living donor liver transplantation (LDLT) at Kumamoto University were reviewed with attention to surgical procedures. Thirty-nine DLTs were performed in Japan until 2010, and survival rates at 1 and 3 years were 82% and 63.6%, respectively. Six of 21 DLT recipients who survived for more than 3 years developed amyloid depositions within organs, and de novo amyloid neuropathy was reported in three patients. DLT from FAP recipients in Kumamoto was safely performed with preservation of the retrohepatic inferior vena cava in FAP recipients. All 20 FAP recipients who were DLT donors are alive with the exception of one who died of the original FAP 9 years after LDLT. The outcomes of DLT and FAP recipients in Kumamoto were satisfactory.
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.
Increasing precision of liver resection and use of reduced liver grafts stimulated this study of the blood supply of the intrahepatic bile ducts.
Corrosion casts of human liver were dissected to study the blood supply of the right and left hepatic ducts.
The dissected casts showed that the ducts are surrounded by a vascular plexus supplied from the main right and left hepatic arteries, segmental arteries, gastroduodenal artery and accessory hepatic arteries. This plexus is closely associated with the arteries supplying the caudate lobe. They arise from both right and left in two general patterns. This plexus links the arterial supplies of the right and left livers. Segment i.v. and the central portion of the left hepatic duct are often supplied by the right arterial system.
This study provides further insights into bile duct blood supply. The caudate lobe and biliary plexus provide collateral connections between the right and left livers.
This study sought to evaluate the potential impact of domino liver transplantation (DLT) on initial graft function and early postoperative outcome in patients with cirrhosis in a Portuguese liver transplantation center. A retrospective comparative analysis was performed between 77 domino recipients (from familial amyloidotic polyneuropathy donors) and 91 deceased donor recipients, all submitted to primary elective whole liver transplantation, using the piggyback technique, in a 42-month period. Outcome parameters included graft dysfunction (defined as either primary nonfunction or initial poor function, according to the Ploeg-Maring criteria) and Clavien II-IV complications in the first postoperative week. Domino and deceased donor recipients had similar preoperative severity indices (Child-Pugh classification and Model for End-Stage Liver Disease score) and immediate postoperative severity scores (APACHE II [Acute Physiology and Chronic Health Evaluation II] and SAPS II [Simplified Acute Physiology Score II]). In DLT, donors were younger, cold ischemia time was shorter, and intraoperative transfusion requirements of packed red blood cells and fresh-frozen plasma were significantly lower. Graft dysfunction incidence was 3.4-fold lower in DLT: 5.2% (only 4 cases of initial poor function) versus 18.0% (1 primary nonfunction and 15 cases of initial poor function), P = 0.010. Postoperative bleeding was the most frequent early Clavien II-IV complication (n = 29, 17.3%), with an incidence 2.2-fold lower in domino recipients. A statistically significant difference was not found in the other analyzed Clavien II-IV complications, intensive care unit stay, mechanical ventilation time, intensive care unit mortality, and 1-year survival rate. In conclusion, in this study the younger donors and shorter ischemic time associated with DLT may provide a protective role in regards to graft dysfunction and perioperative bleeding, which are 2 important determinants of early morbidity after liver transplantation.
A left liver graft in living donor liver transplantation often has 2 arterial stumps. The indication for multiple arterial reconstructions remains controversial. The aim of this retrospective study was to investigate whether single anastomosis of a left liver graft affects the outcome of living donor liver transplantation.
When a hepatic graft had 2 arterial stumps, the thicker (dominant) stump was reconstructed first. After the initial reconstruction, another reconstruction was performed only if no pulsating flow was observed from the remnant stump. A total of 134 left liver grafts were divided into 3 groups: Group 1 (n = 70), 1 arterial stump on a graft with 1 arterial reconstruction; Group 2 (n = 59), 2 stumps with 1 arterial reconstruction; Group 3 (n = 5), 2 stumps with 2 arterial reconstructions. The incidence of hepatic arterial thrombosis, biliary stenosis, and patient survival was compared between Groups 1 and 2.
The incidence of arterial thrombosis and biliary stenosis, and the patient survival curves, were equivalent between Groups 1 and 2.
When the criteria were satisfied, single arterial reconstruction in a left liver graft with 2 arterial stumps did not affect the patient survival or biliary complications.
Graft hepatic arteries (HAs) are usually reconstructed with a recipient HA branch (anatomical HA reconstruction) in living donor liver transplantation (LDLT). Surgeons often encounter difficulties in reconstructing HAs, particularly when a recipient artery other than an HA branch must be used; this is known as extra-anatomical HA reconstruction. The outcomes of LDLT recipients with extra-anatomical HA reconstruction were retrospectively reviewed. Between October 1996 and October 2009, we performed primary LDLT 335 times, re-LDLT 8 times, and HA re-reconstruction 5 times for patients with HA complications. Thirty-three extra-anatomical HA reconstructions were performed in 22 patients with primary LDLT (6.6%), 4 patients with re-LDLT (50%), and 4 patients with HA re-reconstructions for HA complications (80%). In extra-anatomical HA reconstructions, we used 12 right gastroepiploic arteries, 6 right gastric arteries, 5 gastroduodenal arteries, 2 left gastric arteries, 2 splenic arteries, 2 cystic arteries, and 4 interposition grafts as recipient inflow arteries. Only 1 HA-related complication, the formation of an aneurysm, occurred after extra-anatomical HA reconstruction. The overall graft and patient survival probabilities after primary LDLT with extra-anatomical HA reconstruction were comparable to those after LDLT with anatomical HA reconstruction, although approximately half of the patients with extra-anatomical HA reconstruction suffered anastomotic biliary strictures. Therefore, extra-anatomical HA reconstruction can be safely performed through the proper selection of recipient arteries and the use of interposition grafts. These procedures can save hepatic grafts, even when recipient HAs cannot be used as inflow arteries.
The aim of this study was to evaluate retrospectively the outcome of percutaneous transluminal venoplasty (PTV) after venous pressure measurement in patients with hepatic venous outflow obstruction following living donor liver transplantation (LDLT).
We studied 24 consecutive patients suspected of having hepatic venous outflow obstruction after LDLT. Pressure gradients were measured proximal and distal to the lesion, and gradient values >3 mmHg were considered hemodynamically significant. We evaluated the technical success, complications, outcome of venoplasty and recurrence, and the patency rate.
In all, 11 female patients manifested a pressure gradient >3 mmHg across the anastomotic site; they underwent subsequent PVT. The initial balloon venoplasty procedure was technically successful in 10 of the 11 patients (91%), and the pressure gradient was reduced from 5.8 to 1.1 mmHg (P < 0.01). Clinical improvement was observed in 9 of these 10 patients; one patient failed to improve and underwent retransplantation. Recurrent obstruction occurred in four patients; they underwent PTV with (n = 2) or without (n = 2) stent placement. There were no major procedural complications.
PTV following venous pressure measurement is an effective and safe treatment for venous outflow obstruction in patients subjected to LDLT. In patients with recurrent obstruction, re-venoplasty is recommended.
The incidence of clinically significant right hepatic vein (RHV) stenosis after adult living donor liver transplantation has been higher than expected. In this study, an assessment of the risk factors for the development of RHV stenosis in this context was undertaken. Hepatic anatomy, surgical techniques, and the incidence of RHV stenosis 1 year after transplantation were evaluated retrospectively in 225 recipients of right lobe grafts. These patients underwent independent RHV reconstruction, which was facilitated by the application of computed tomography morphometry and computational simulation analyses. Three types of preparation of the orifice of the graft RHV and 7 types of preparation for venoplasty of the recipient RHV were used. The frequency of high, middle, and low sites of RHV insertion into the inferior vena cava (IVC) was 56.0%, 36.4%, and 7.6%, respectively, for donors, and 26.7%, 58.7%, and 14.7%, respectively, for recipients. Nine patients (4%) developed RHV stenosis of early onset that required stent insertion during the first 2 postoperative weeks; in 12 patients (5.3%), RHV stenosis of delayed onset occurred. Inappropriate matching of RHV sites of insertion correlated with the incidence of stenosis of early onset (P = 0.039). Technical refinements to avoid adverse consequences of inappropriate ventrodorsal matching of RHV sites of insertion include making the recipient RHV orifice wide and enlarging the recipient IVC by a customized incision and patch venoplasty after anatomical assessment of the RHV and IVC of the graft and recipient.
Vascular complications (VC) after liver transplantation (OLT) are one of the most feared problems that frequently result in graft and patient loss. Herein we have reported our experience with VC after either deceased donor liver transplantation (DDLT) or living donor liver transplantation (LDLT).
Between April 2001 and September 2009, we performed 224 OLT: 155 DDLT and 69 LDLT. The overall male/female ratio was 136/88 and the adult/pediatric ratio was 208/16. We retrospectively identified and analyzed vascular complications in both groups.
In the DDLT group, 11/155 recipients (7%) suffered vascular complications; hepatic artery thrombosis (HAT; n=5; 3.2%), portal vein thrombosis occurred (n=4; 2.6%); hepatic vein stenosis (n=1; 0.6%), and severe postoperative bleeding due to a slipped splenic artery ligature (n=1, 0.6%). In the DDLT group, 4/11 (36.4%) patients died as a direct result of the vascular complications. In the LDLT group, 9/69 recipients (13%) suffered vascular complications: HAT (n=3; 4.3%), portal vein problems (n=5; 7.2%), and hepatic vein stenosis (n=1; 1.5%). Among LDLT, 3/9 (33.3%) patients died as a direct result of the vascular complications. In both groups vascular complications were associated with poorer patient and graft survival.
In our experience, the incidence of vascular complications was significantly higher among the LDLT group compared with the DDLT group. Vascular complications were associated with poorer graft and patient survival rates in both groups.
Domino liver transplantation (DLT) is a strategy to increase the donor pool. Explanted liver from patients with familial amyloidotic polyneuropathy (FAP) are often used as domino grafts, because the liver is normal apart from the production of the mutated transthyretin variant. We present the outcomes for both donors and recipients of DLT.
Retrospective analysis of initial DLT for 16 consecutive adult patients performed between July 2004 and July 2009. All cases of FAP donor to grafts were removed preserving the cava vein with reconstruction of the hepatic veins, except the first and seventh cases, where in we removed the retrohepatic vena cava with the liver without venovenous bypass. The postoperative follow-up period for surviving DLT recipients at the end of September 2009 was 2-62 months (mean, 26).
Two patients out of 8 FAP donors died due to pulmonary thromboembolism on the 31st postransplant day, or sepsis at 35 days namely, an overall survival of 75%. One patient out of 8 recipients died namely, an early portal thrombosis on the 22nd postransplant day) with a crude survival of 87.5% in the recipient group (P = no significant [NS]). Four grafts from 8 FAP donors were lost-2 deaths and 2 retransplants due to thrombotic events on the first and second postransplant day-with a crude survival of 50%. Two of 8 recipients lost their grafts: 1 death and 1 retransplantation for an acute Budd-Chiari syndrome on the first postransplant day with a crude survival of 75% in the recipient group (P = not significant [NS]).
We believe that the FAP liver graft is an excellent option for selected patients. Special care must be taken with thrombotic events.
Although balloon angioplasty has been accepted as the safe and effective initial treatment to manage hepatic venous outflow abnormalities, it may induce rupture of the fresh anastomosis but also may be ineffective to eliminate various etiologies of venous outflow abnormalities in the early post-transplant period. Therefore, we performed primary stent placement in 108 patients to treat early-onset (≤4 weeks) post-transplant hepatic venous outflow abnormality. The following parameters were documented retrospectively: technical success and complications: clinical success; recurrence; and patency of stent-inserted hepatic veins.
Technical success was achieved in 166 (97.6%) of 170 anastomoses (107 patients). Major complications occurred in 5 (4.6%) patients: partial stent migration (n = 2) and stent malposition (n = 3). Clinical success was achieved in 83 (82.2%) of 101 patients who had abnormal liver enzymes or clinical symptoms. Seven patients without initial clinical symptoms have remained healthy. Restenosis or occlusion of the stent-inserted hepatic veins was documented in 22 patients at a mean of 9.6 ± 8.6 months after stent placement. Four of them underwent stent replacement or retransplantation due to liver function deterioration. Overall 1-, 3-, and 5-year primary patency rates were 82.3 ± 0.3%, 75.0 ± 0.4%, and 72.4 ± 0.5%, respectively. Multivariate Cox regression analysis showed that diameter of stents was an independent factor associated with patency of stents (p = 0.001).
Primary stent placement seems to be an effective treatment modality with an acceptable long-term patency to treat early post-transplant hepatic venous outflow obstruction. Liver Transpl 14:1505–1511, 2008.
Hepatic vein (HV) reconstruction is crucial in partial liver transplantation in which the inferior vena cava (IVC) is preserved.
We reviewed the medical records of 152 living-related donor liver transplantations (LRDLTs) in 150 children (45 left lobe grafts, 106 lateral segment grafts, and 1 right lobe graft) monitored for 12 months or longer.
A standard technique was a wide end-to-side anastomosis between the donor HV and cuffs, consisting of the recipient middle and left HV and an incision to the IVC. In 15 of 22 partial grafts with two separated HVs the two vessels were reformed by back table surgery to have a common anastomotic orifice, and two separate anastomoses of the individual vessels were made for the remaining seven grafts. Four patients with an absence of infrahepatic IVCs and two with completely obstructed IVCs had end-to-end anastomoses with recipient IVCs. Four patients with stenotic IVCs had end-to-side anastomoses with new orifices on the IVCs. Two patients had acute HV obstruction caused by twisting of the HV that required laparotomy, and six had late-onset HV obstruction that required radiologic intervention. A tissue expander was placed prophylactically in the right subphrenic space in 10 patients to prevent the dislocation of the graft into the right subphrenic space.
It is important in HV reconstruction in partial liver transplantation to make wide orifices and to adapt each graft to its orthotopic place, taking into consideration graft shape, size of the abdominal cavity of the recipient, and anatomic variations in vessels.
Familial amyloid polyneuropathy is an autosomal dominant disorder in which the liver produces a variant prealbumin that is deposited along nerves, leading to a progressive and fatal polyneuropathy that begins in the third decade of life. Liver transplantation has been the only successful treatment to date. Apart from the production of the variant protein, there are no other abnormalities in these amyloid livers. We describe two cases in which, at the time of transplantation, the amyloid livers were subsequently used for transplantation in another patient, and we discuss the implications.
Shortage of liver donors means that new methods of liver procurement must be explored. In domino transplantation, organs explanted during transplantation in one patient are transplanted into a second patient. Domino procedures can be performed with livers from patients having transplantation for hepatic metabolic disorders that cause systemic disease without affecting other liver functions. Familial amyloidotic polyneuropathy (FAP) type I is one of these.
We reviewed the Paul Brousse experience with a domino liver transplant program for FAP, hoping to extend the approach to other metabolic disorders.
Livers from 10 patients transplanted for FAP type 1 were used for domino transplants to patients with unresectable primary or metastatic liver cancers. There was no perioperative mortality. Neuropathy or cardiomyopathy did not increase the morbidity of the domino liver explant and transplant procedures. Morbidity for the domino recipients did not appear to be increased. Variant transthyretin was detected in the serum in FAP liver recipients, with no immediate clinical consequences.
The domino approach is feasible and requires careful planning of the surgical procedures for liver explantation, particularly for the nature and site of vascular anastomoses. Domino transplantation of metabolically dysfunctional livers creates new categories of potential donors and potential recipients. It raises new ethical, technical, and societal issues. The domino approach could be used in several genetic or biochemical disorders now treated by liver transplantation. It has the potential to increase the number of liver grafts available for transplantation.
Domino liver transplantation using FAP livers (DLTx/FAP) has become a worldwide strategy that is contributing significantly to the increase in liver grafts offered in Portugal. The safety of the procedure both in the FAP donors and in the recipients is emphasized in the series of 64 DLTx/FAP that has already been performed in the Hospital of the University of Coimbra (HUC). The occurrence of amyloidosis in the long-term survivors after DLTx/FAP is a serious possibility, although after 6 and 7 years post-transplant no symptoms have been reported by any of the patients. Indeed, amyloid deposits, although small, have been found in the skin and nerve biopsies of the first domino recipients (HUC unpublished data). Hopes that the disease will have a slow course in non-genetically affected patients still awaits the proof of time. Thus, DLTx/FAP should be considered an experimental procedure, although there is good reason to expect that the amyloidosis in the DLTx/FAP recipients will have a longer time course than in the genetically affected donors. The ethical implications of all these facts and expectations are discussed. A case by case approach is recommended. In such an approach, individual prognosis, urgency, graft shortage and the will of very well-informed patients or their legal representatives are determinants for judicious decisions when considering DLTx/FAP in patients under 50 years of age.
Familial amyloidotic polyneuropathy (FAP) is a fatal disease, belonging to a group of systemic disorders caused by an amyloidogenic transthyretin (TTR) variant. Orthotopic liver transplantation (OLT) eliminates the source of the variant TTR molecule, and is presently the only known curative treatment. A fascinating consequence of this treatment is the possibility of retransplanting the removed FAP liver into another non-FAP patient, which created the so-called domino liver transplantation (DLT) procedure. The Familial Amyloidotic Polyneuropathy World Transplant Registry (FAPWTR) was initiated in 1995, and in 1999 a Domino Liver Transplantation Registry (DLTR) was created. Herein data from these Registries are presented. A total number of 56 centers in 16 countries have performed OLT for FAP, and, today, approximately 65-70 OLTs are performed annually worldwide. During the last decade, a total of 623 patients have undergone 660 OLTs. Patient survival is excellent and comparable to the survival with OLT performed for other chronic liver disorders. Twenty-six centers in 12 countries have reported recipients of DLT grafts and presently 30-35 DLTs are performed annually. The FAPWTR and DLTR have become useful tools in evaluating the potential risks and benefits of these relatively new therapeutic options, in addition to encouraging a rewarding collaboration between centers involved in the management of these patients.
This report describes two rescued cases with rare complications of the hepatic artery in living-donor liver transplantation (LDLT). In both cases a segment of the autologous inferior mesenteric artery (IMA) was successfully used as an arterial graft for re-vascularization under microsurgery. The first case was that of a pseudoaneurysm of the hepatic artery, which caused massive gastrointestinal bleeding. The hepatic arteries of the pre- and post-aneurysm were divided, and the arterial graft from the recipient's IMA was interposed for reconstruction. The second case was that of an intimal dissection of the recipient's hepatic artery. Because the dissection extended to the root of the common hepatic artery, the autologous IMA was interposed between the donor's hepatic artery and the proximal stump of the recipient's splenic artery. Reconstruction using the arterial graft of the autologous IMA is feasible for re-vascularization of the hepatic artery in liver transplantation.
To evaluate prospectively the relationship between the arterial collateral system at the hepatic hilum and the blood supply to the hilar bile duct by using computed tomography (CT) and angiography during temporary balloon occlusion of the right or left hepatic artery.
Institutional review board approval and informed consent were obtained. The study included 13 patients with no lesions at the hepatic hilum (eight men and five women; age range, 41-78 years; mean, 65.8 years). After serial angiographic studies were performed for preoperative evaluation or transcatheter arterial chemoembolization, a 5.5-F catheter with an occlusion balloon was positioned in the right or left hepatic artery. Eleven patients underwent angiography of the left hepatic artery with temporary occlusion of the right hepatic artery, and two patients underwent angiography of the right hepatic artery with temporary occlusion of the left hepatic artery. In addition, 11 patients underwent single-level dynamic CT during hepatic arteriography (CTHA) with temporary occlusion of the right or left hepatic artery. The images from angiography and CTHA were interpreted by two authors who assessed the existence of the arterial communication and its branching points, location, and relationship to the hilar bile duct and caudate lobe.
During temporary occlusion of the right or left hepatic artery, the communicating arcade (CA) between the right and left hepatic arteries was immediately evident in all patients. On the left side, the CA originated from the segment IV artery in eight patients (62%) and from the left hepatic artery in five (38%). On the right side, the CA originated from the right anterior hepatic artery in six patients (46%), the right hepatic artery in two (15%), and both arteries in five (38%). The CA was extrahepatically located close to the hilar bile duct and forked into a few branches to the caudate lobe.
The CA may play an important role not only in the interlobar arterial collateral system but also in the blood supplies to the caudate lobe and hilar bile duct.
Domino liver transplantation (DLT) using grafts from patients with familial amyloidotic polyneuropathy (FAP) is an established procedure at many transplantation centers. However, data evaluating the long-term outcome of DLT are limited. The aim of the present study was to analyze the risk of de novo polyneuropathy, possibly because of amyloidosis, and the patient survival after DLT. At our department, 28 DLT using FAP grafts were conducted between January 1997 and December 2005. One patient was twice subjected to DLT. Postoperative neurological monitoring of peripheral nerve function was performed with electroneurography (ENeG) in 20 cases. An ENeG index based on 12 parameters was calculated and correlated to age and/or height. Three patients developed ENeG signs of polyneuropathy 2-5 years after the DLT, but with no clinical symptoms. The 1-, 3- and 5-year actuarial patient survival in hepatocellular carcinoma (HCC) patients (n = 12) and non-HCC patients (n = 15) was 67%, 15%, 15% and 93%, 93%, 80%, respectively (P = 0.001). Development of impaired nerve conduction in a proportion of patients may indicate that de novo amyloidosis occurs earlier than previously expected. Survival after DLT was excellent except in patients with advanced HCC.
The double piggyback technique has been proposed for domino liver transplantation. To make this possible, it is necessary to reconstruct the venous outflow of the domino liver graft on the back table. The authors describe the technical details of this procedure in three consecutive cases. A deceased donor cava-iliac bifurcation segment was used. The iliac veins were anastomosed to the ostia of the right and middle-left hepatic veins, and the graft cava vein was anastomosed to the ostium of the three hepatic veins of the recipient. In all cases anatomic compatibility was observed; the outcome of the patients was satisfactory.
Domino liver transplantation, wherein a patient who himself undergoes liver transplantation in turn donates his liver to another recipient, has been performed since the mid-1990 s. Although livers from a handful of metabolic disorders cured by liver transplantation have been used for domino transplantation, familial amyloidotic polyneuropathy (FAP) livers are by far the most common source. FAP is an inherited disorder never presenting its clinical manifestation before the age of 15. In many carriers, the genetic disorder never manifests during lifetime. Thus, only a proportion of patients with FAP develop disease symptoms, which has been the rationale for using such livers for other patients on the waiting list for liver transplantation. According to the Familial Amyloidotic Polyneuropathy World Transplant Registry (FAPWTR), only 2 out of more than 500 patients so far have developed symptoms after domino liver transplantation using an FAP liver. Domino recipients with nonmalignant indications for liver transplantation show excellent long-term survivals. With careful selection of recipients, the procedure helps to reduce the organ shortage and the time on the waiting list for patients with malignant disorders.
Domino liver transplantation as a valuable option