Living Donor Liver Transplantation for Congenital Absence of the Portal Vein

The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.     

Outcomes of Surgical Strategies for Living Donor Liver Transplantation in Patients With Portal Vein Thrombosis: A Cohort Study

BackgroundAdequate portal flow to the liver graft is the requirement of a successful liver transplant (LT). Historically, portal vein thrombosis (PVT) was a contraindication for LT, especially for living donor LT (LDLT), demanding technically more difficult operations and advanced technique. In this study, the outcomes of patients with and without PVT after LDLT were compared.MethodsAdult LDLTs performed by 2 centers (n = 335) between 2013 and 2020 were included into this large cohort study. PVT was classified based on Yerdel classification grade 1 to 4.ResultsSixty-two patients with PVT constituted 19% of the study cohort of 335 recipients. While mean platelet count was found to be lower (P = .011) in the PVT group, patient age (P = .035), operation duration (P = .001), and amount of intraoperative blood transfusion (P = .010) were found to be higher. Incidence of PVT was higher in female patients than males (22.7% vs 16.1%, P = .037). There was no significant difference in survival between patients with and without PVT on 30-day (P = .285), 90-day (P = .565), 1-year (P = .777), and overall survival (P = .917). Early thrombosis did not show a better survival rate than Grades 2, 3, or 4 PVT. Thrombosis limited to portal vein was not found to bring a survival advantage compared with Grade 3 and 4 thromboses. Eversion thrombectomy was the most common procedure (66%) to overcome PVT intraoperatively.ConclusionAlthough technically more challenging, PVT is not a contraindication of LDLT. Similar outcomes can be achieved in LDLT in patients with PVT after proper restoration of portal flow, which eliminates the default survival disadvantage of patients with PVT.     

Long-Term Outcome of Portal Vein Stenting After Pediatric Living Donor Liver Transplantation

BackgroundPortal vein (PV) stenosis is sometimes seen in pediatric living donor liver transplantation (LDLT). PV stents have been attempted in adults with persistent stenosis. However, long-term usefulness of PV stenting is unknown because stents do not expand with growth. We investigated the effect and long-term outcome of PV stenting for stenosis after pediatric LDLT.MethodsWe included patients aged <18 years who underwent LDLT from 1998 to 2020 and who underwent PV stenting for stenosis. We assessed age at procedure, stent complications, and long-term outcomes.ResultsFive patients underwent PV stent placement. The median age at LDLT was 10 years (range, 0.8-18.1 years). The median interval between LDLT and stent placement was 25 months. The median age at stent placement was 16 years (range, 3-20 years). The median body weight was 38 kg (range, 13-63 kg). The median stent diameter was 8 mm. The median observation period after stent placement was 8 years. On average, body weight increased 1.6 times. One complication associated with stent placement was PV thrombosis, which resulted in stent failure, but we observed no portal hypertension. In the other 4 patients, the stent has remained functioning, and there was no clinical evidence of portal hypertension.ConclusionsPV stents are effective for intractable PV stenosis in children. PV stents were successfully placed in children as young as 3 years old and weighing 13 kg. Our data suggests that a stent placed in young children does not cause portal hypertension as patients grow.     

Feasibility and Outcomes of Direct Dual Portal Vein Anastomosis in Living Donor Liver Transplantation Using the Right Liver Graft With Anatomic Portal Vein Variations

Background

Portal vein (PV) reconstruction is a crucial factor in successful living donor liver transplantation (LDLT). In LDLT using the right liver grafts with anatomic PV variations, we sometimes encounter dual PV anastomosis. In this study we describe PV variations of donor liver in detail as well as our experiences with PV reconstruction in right liver grafts with PV variations.

Correlation Between Splenectomy and Portal Vein Complications in Living Donor Liver Transplantation

Objectives

In adults undergoing living donor liver transplantation (LDLT), the transplanted livers are partial grafts, and the portal venous pressure is higher than that observed with whole liver grafts. In patients undergoing LDLT concomitant with splenomegaly, portal venous flow is often diverted to collateral vessels, leading to a high risk of portal vein thrombosis. In such cases, occlusion of the collateral veins is important; however, complete occlusion of all collaterals without blocking the blood flow through the splenic artery causes portal hypertension and liver failure. We aimed to examine the effect of performing a splenectomy concomitant with LDLT to reduce portal vein complications.

Resolution of Preoperative Portal Vein Thrombosis After Administration of Antithrombin III in Living Donor Liver Transplantation: Case Report

A 59-year-old man with hepatitis C virus-associated liver cirrhosis was transferred to our hospital to undergo living donor liver transplantation. Coagulation was impaired (prothrombin time [International Normalized Ratio], 3.27), and antithrombin III (AT-III) activity was 23% (normal, 87%-115%). Contrast-enhanced computed tomography scans revealed portal vein thrombosis (PVT) from the junction between the splenic and superior mesenteric vein to the porta hepatica; the portal vein was completely obstructed (PVT). To prevent further development of PVT, 1500 U of AT-III was administered for 3 days, elevating the AT-III activity to 50%. A contrast-enhanced computed tomography scan obtained 9 days after AT-III administration showed resolution of PVT. Living donor liver transplantation was safely performed without portal vein grafting. Thus, a low AT-III concentration may have an important role in the pathogenesis of PVT in patients with cirrhosis.     

Risk Factors for Portal Vein Complications After Pediatric Living Donor Liver Transplantation With Left-sided Grafts

Purpose

Portal vein complications (PVC) after pediatric living donor liver transplantation (LDLT) have rarely been reported. We evaluated the long-term incidence and of the risk factors for PVC after pediatric LDLT.

Methods

From April 1997 to November 2008, 96 pediatric patients underwent LDLT using left lateral segments or left lobes. We investigated recipient factors, donor factors, and operative factors through medical records. The portal vein sizes in 96 recipients ranged from 2.7 mm to 13.0 mm (median = 5.0 mm). Portal vein reconstruction was usually performed with the graft portal vein anastomosed to the bifurcation of the recipient right and left portal veins, the so-called “branch patch”.

Results

PVC occured in 11 patients (11.5%) including early PVC (n = 3), late PVC (n = 8). The disease-free survivals at 1, 5, and 10 years after LDLT were 94.7%, 88.7%, and 86.0%. Upon univariate analysis, a portal vein size < 5 mm graft-to-recipient weight ratio (GRWR) ≥ 4%, transfusion volume ≥ 270 mL were significant risk factors for PVC. Body weight < 8 kg and previous operative history tendes to be adverse for PVC. Upon multivariate analysis by Cox regression, portal vein size < 5 mm was a highly significant factor for PVC after pediatric LDLT (hazard ratio = 5.627, P = .027).

Conclusion

The disease-free survival at 10 years after LDLT was 86.0%. If the recipient's portal vein size < 5 mm received a large-for-size graft (GRWR ≥ 4%), it is important to observe by regular Doppler ultrasonography follow-up to detect PVC.     

Thrombosis Confined to the Portal Vein Is Not a Contraindication for Living Donor Liver Transplantation

BACKGROUND: There is a lack of agreement regarding preexisting portal vein thrombosis (PVT) in patients undergoing living donor liver transplantation (LDLT). We report the results of a single-center study to determine the impact of PVT on outcomes of adult LDLT recipients. METHODS: Of 133 cases of adult LDLT performed between January 2000 and December 2004, a thrombectomy was performed on 22 patients (16.5%) with PVT during the transplant procedure. One hundred eleven patients without PVT (group 1) were compared with those with a thrombosis confined to the portal vein (group 2; n = 15) and patients with the thrombosis beyond the portal vein (group 3; n = 7). RESULTS: The sensitivities of Doppler ultrasound and CT in detecting PVT were 50 and 63.6%. A prior history of variceal bleeding (OR = 10.6, p = 0.002) and surgical shunt surgery (OR = 28.1, p = 0.044) were found to be an independent risk factors for PVT. The rate of postoperative PVT was significantly higher in patients with PVT than in those without (18.2 vs. 2.7%; p = 0.014). In particular, the rethrombosis rate in group 3 was 28.6%. The actuarial 3-year patient survival rate in PVT patients (73.6%) was similar to that of the non-PVT patients (85.3%; p = 0.351). However, the actuarial 3-year patient survival rate in group 3 was 38.1%, which was significantly lower than that in groups 1 and 2 (p = 0.006). CONCLUSION: A thrombosis confined to the portal vein per se should not be considered a contraindication for LDLT.     

Doppler Ultrasound Evaluation of Postoperative Portal Vein Stenosis in Adult Living Donor Liver Transplantation

Aim

To evaluate the postoperative portal vein stenosis (PVS) and the diagnostic efficiency of Doppler ultrasound (DUS) in adult living donor liver transplantation (ALDLT).

Materials and Method

From January 2007 to December 2008, 103 ALDLTs were performed and postoperatively followed by routine DUS. The morphologic narrowing at the anastomotic site (AS) of the PVS was analyzed. We calculated the PV stenotic ratio (SR) using the following formula: SR (%) = PRE-AS/PRE (PRE = pre-stenotic caliber). An SR >50% was defined as the critical point for PVS. We also calculated the velocity ratio (VR) between the AS and PRE, and set the significant VR as >3:1. Statistical analyses were carried out to determine clinical significance.

Results

Using the definition of morphologic PVS by DUS, there were total 20 cases (19.4%) in this series with SR >50%, which included 17 cases with VR >3:1. Eight cases of severe PVS had a stenotic AS >5 mm and subsequently underwent interventional management. Doppler criteria of SR and VR values were elevated up to 75.8% and 7.5:1, respectively, in these treated cases. Two cases of severe PVS subsequently developed PV thrombosis. Intervention by balloon dilation and/or stenting was performed successfully in this PVS case.

Conclusion

DUS is the most convenient and efficient imaging modality to detect and follow postoperative PVS in ALDLT. The Doppler criteria of SR and VR are both sensitive but less specific. Cases of AS <5 mm require interventional management for good long-term graft survival.     

Unification Venoplasty to Cope With Recipient Portal Vein Anomaly During Living Donor Liver Transplantation

Purpose

To cope with recipient portal vein (PV) anomalies, such as early branching of the right posterior section (RPS), during living donor liver transplantation (LDLT) surgery, we performed a simulation study to standardize the surgical technique for unification portal venoplasty.

Methods

This study included an observational analysis of conventional methods utilizing RPS PV, simulation-based design of a new surgical technique, and clinical application of this new technique.

Results

In a case encountering RPS PV, a mild anastomotic PV stenosis was persistent over 6 months postsurgery, indicating the need for technical refinement. After computational simulation analysis, we found that simple suturing of the PV branch patch automatically resulted in a funnel-shaped elongation. A prospective recipient study (n = 30) indicated that usual PV reconstruction via the PV bifurcation method is feasible in the absence of unusual donor or recipient PV anomaly. Retrospective living donor PV anatomy analysis (n = 20) revealed that 20-mm-long limbs of the first-order PV branches are necessary to make a 10- to l5-mm-long funneled PV stump. This technique of unification venoplasty for an anomalous recipient PV was applied to an adult patient undergoing LDLT with a right liver graft, for which it was shown to be technically feasible and effective.

Conclusions

A simplified unification venoplasty technique was developed to cope with a recipient PV anomaly in adult LDLT.     

Portal Venous Pressure in Adult Living Donor Liver Transplantation

Objective

The relationship between portal pressure and small-for-size syndrome (SFSS) is unsettled. The purpose of this study was to evaluate the role of portal pressure in predicting SFSS.

Methods

Thirty-four patients with end-stage liver disease who received adult-to-adult living-donor liver transplantation (ALDLT) were included. Recipients were grouped based on whether they received portal flow modulation or not. The intraoperative portal vein flow volume (PVFV) and portal venous pressure (PVP) between the 2 groups were compared. The relationship of PVP to PVFV, graft weight-to-recipient weight ratio (GRWR), and graft weight-to-recipient spleen size ratio (GRSSR) were analyzed.

Results

Persistent portal hypertension was found after ALDLT. The PVP was linearly correlated with PVFV but not with GRWR or GRSSR. With the use of the following criteria, (1) PVFV >250 mL/min/100 g graft weight, (2) GRWR <0.8%, and (3) GRSSR <0.6, modulation of the portal flow was performed in 3 cases. The receiver operating characteristic analysis showed that 23 mm Hg was the cutoff point for PVP, with a sensitivity of 83% and specificity of 43%.

Conclusions

PVP is a weak parameter to use for portal flow modulation after ALDLT. It is sensitive but not specific to predict SFSS.     

Portal Vein Complications in Pediatric Living Donor Liver Transplantation Using Left‐Side Grafts

The aim of this report is to assess the rate of portal vein complications (PVCs), the success rate of treatment for PVCs and the prognosis of patients with PVCs for pediatric living donor liver transplantation (LDLT). Pre‐ and postoperative records of 521 pediatric LDLTs, using left‐side grafts were retrospectively reviewed. The overall rate of PVC was 9%, with early PVC occurring in nine patients (1.7%) with a mortality rate of 67% and late PVC in 38 patients (7.3%). Fifteen of these patients with late PVC showed complete portal vein occlusion despite various treatments, and in six of them the graft was lost. Histological examination revealed fibrosis in portal areas in 13 patients, around the central veins associated with cholestasis in the parenchyma in 10, and hepatocyte ballooning in 12. Correction of portal vein flow or retransplantation is necessary for the rescue of patients with early PVCs. Graft loss in the long term may be high with the occurrence of liver failure or portal hypertension related causes, such as hepatopulmonary syndrome and gastrointestinal bleeding in patients with late PVCs. For the rescue of these patients, especially for patients with body weight < 6 kg, regular monitoring of portal vein flow is essential.     

Intrahepatic Segmental Portal Vein Thrombosis After Living-Related Donor Liver Transplantation

Background

Intrahepatic segmental portal vein thrombosis after living-related liver transplantation (LRLT) is uncommon. The cause remains unclear.

Methods

After providing written informed consent, 25 recipients receiving LRLT at our institution from January 2011 to September 2013 were enrolled in this study. We performed triphase computerized tomographic (CT) study of the liver graft of each recipient 1 month after LRLT. The patencies of hepatic artery, portal vein, and hepatic vein were evaluated in detail. The triphase CT scans of the liver of each donor before transplantation also were reviewed. Thrombosis of the intrahepatic segmental portal vein was defined as the occlusion site of the portal vein being intrahepatic. Extrahepatic portal vein thrombosis was excluded in this study.

Results

Among the 25 patients, 2 (8%) developed thrombosis of intrahepatic segmental portal vein. One 47-year-old man received LRLT for hepatitis B viral infection–related liver cirrhosis (Child-Pugh class C) with 3 hepatocellular carcinomas (total tumor volume <8 cm). Another 53-year-old man received LRLT for alcoholic liver cirrhosis (Child-Pugh class C). Both had developed progressive jaundice and cholangitis 1 month after surgery. Intrahepatic biliary stricture was found on the follow-up magnetic resonance images. However, liver triphase CT study demonstrated occlusion of intrahepatic portal vein of segment 8 in each patient. Radiologic interventions and balloon dilatation therapy via percutaneous transhepatic biliary drainage route improved the symptoms and signs of cholangitis and obstructive jaundice for both.

Conclusions

Thrombosis of intrahepatic segmental portal vein is not common but is usually associated with complications of intrahepatic bile duct. Early detection is important, and follow-up CT study of liver is suggested.     

Selective Hemi-Portocaval Shunt Based on Portal Vein Pressure for Small-for-Size Graft in Adult Living Donor Liver Transplantation

We developed an algorithm of graft selection in which left lobe donation is considered primarily if the graft-to-recipient weight ratio (GRWR) is estimated to be greater than 0.6% in preoperative volumetry with utilization of a hemi-portocaval shunt (HPCS) based on portal vein pressure (PVP) more than 20 mmHg at the time of laparotomy. A total of 11 consecutive adult living donor liver transplantations with small-for-size graft according to our graft selection algorithm were performed between December 2005 and August 2007. Ten patients required HPCS using a vein graft all survived without small-for-size syndrome (SFSS) and shunt complications with a median follow-up of 296 days. One patient without HPCS died of chronic vascular rejection. In all cases, PVP were regulated successfully under 20 mmHg by HPCS. Graft volume reached in mean 84.3% of standard liver volume in right lobe grafts and mean 95.4% in left lobe grafts at 3 months after liver transplantation. Actuarial rate of shunt patency at 1, 3, 6 months and 1 year were 80%, 55%, 26% and 20%, respectively. Selective HPCS based on PVP is an effective procedure and results in excellent patient and graft survival with avoidance of SFSS in grafts greater than 0.6% of GRWR.     

术中监测及调整门静脉血流预防活体肝移植术后小肝综合征

目的探讨活体肝移植术中门静脉血流量及门静脉压力的监测及调整对小肝综合征的预防作用。方法回顾性分析我院移植一科2007年10月至2008年10月期间行活体肝移植并进行术中门静脉血流监测的44例患者的临床资料,包括实测供肝重量占受者体重比(GRWR)、门静脉血流量及压力、术后是否出现小肝综合征表现等。对于实测GRWR偏小(GRWR1%)的病例,如果术中门静脉压力12 mm Hg(1 mm Hg=0.133 kPa)或者门静脉血流量250 ml/(min.100 g),在术中加行脾切除或脾动脉结扎,分析监测和调整门静脉血流(脾切除和脾动脉结扎)是否可以预防小肝综合征的发生。结果行脾切除6例,切脾后门静脉血流量及压力均较切除前明显降低(P0.05);脾动脉结扎7例,结扎后门静脉压力较结扎前明显降低(P0.05),而门静脉血流量无明显变化(P0.05)。术后44例患者均未出现小肝综合征表现。结论活体肝移植术中通过切脾或行脾动脉结扎降低移植肝门静脉血流量及压力对术后小肝综合征有预防作用     

Donor Outcomes After Liver Donation in Adult to Adult Living Donor Liver Transplantation

Objectives

This study aims to investigate postdonation outcomes of adult living donor liver transplantation donors and remnant liver regeneration in different graft types.

Outcomes of Donor Evaluations for Adult-to-Adult Right Hepatic Lobe Living Donor Liver Transplantation

The purpose of this study is to determine the role of liver biopsy and outcome of patients undergoing donor evaluation for adult-to-adult right hepatic lobe living donor liver transplantation (LDLT). Records of patients presenting for a comprehensive donor evaluation between 1997 and February 2005 were reviewed. Liver biopsy was performed only in patients with risk factors for abnormal histology. Two hundred and sixty patients underwent a comprehensive donor evaluation and 116 of 260 (45%) were suitable for donation, 14 of 260 (5.4%) did not complete evaluation and 130 of 260 (50%) were rejected. Four patients underwent unsuccessful hepatectomy surgery due to discovery of intraoperative abnormalities. Between 1997 and 2001, the acceptance rate of donor candidates (63%) was higher than 2002-2005 (36%), p < 0.0001. Sixty-six of the 150 eligible patients (44%) fulfilled criteria for liver biopsy and 28 of 66 (42%) had an abnormal finding. Less than half of the patients undergoing donor evaluation were suitable donors and the donor acceptance rate has declined over time. A large proportion of the patients undergoing liver biopsy have abnormal findings. Our evaluation process failed to identify 4 of 103 who had aborted donor surgeries.     

Living Donor Liver Transplantation for Pediatric Patients with Inheritable Metabolic Disorders

Forty-six pediatric patients who underwent living donor liver transplantation (LDLT) using parental liver grafts for inheritable metabolic disorders (IMD) were evaluated to determine the outcomes of the surgery, decisive factors for post-transplant patient survival and the impact of using donors who were heterozygous for the particular disorder. Disorders included Wilson disease (WD, n = 21), ornithine transcarbamylase deficiency (OTCD, n = 6), tyrosinemia type I (TTI, n = 6), glycogen storage disease (GSD, n = 4), propionic acidemia (PPA, n = 3), methylmalonic acidemia (MMA, n = 2), Crigler-Najjar syndrome type I (CNSI, n = 2), bile acid synthetic defect (BASD, n = 1) and erythropoietic protoporphyria (EPP, n = 1). The post-transplant cumulative patient survival rates were 86.8 and 81.2% at 1 and 5 years, respectively. Post-transplant patient survival and recovery of the growth retardation were significantly better in the liver-oriented diseases (WD, OTCD, TTI, CNSI and BASD) than in the non-liver-oriented diseases (GSD, PPA, MMA and EPP) and pre-transplant growth retardation disadvantageously affected post-transplant outcomes. Although 40 of 46 donors were considered heterozygous for each disorder, neither mortality nor morbidity related to the heterozygosis has been observed. LDLT using parental donors can be recommended as an effective treatment for pediatric patients with IMD. In the non-liver-oriented diseases, however, satisfactory outcomes were not obtained by hepatic replacement alone.     

Simplified Unification Patch Venoplasty for Anomalous Portal Vein Branching in Living Donor Liver Transplantation With Right Lobe Graft

Double portal vein (PV) branches during living donor liver transplantation (LDLT) with right lobe grafts have been considered challenging both in terms of donor safety and the complexity of vascular reconstruction in the recipient. Herein, we describe our experience with 24 adult LDLT recipients during which we employed unification patch venoplasty to reconstruct right lobe grafts with double PV orifices. We retrospectively reviewed the outcomes of 195 adult LDLT recipients receiving right lobe grafts, including 24 cases of adult LDLT recipients in which unification patch venoplasty was used to treat double PVs from January 2010 to June 2015. The anomalous portal vein branches of the donors were of type II in 7 cases (29.2%), type III in 15 cases (62.5%), and type IV in 2 cases (8.3%). We used propensity score matching analysis to compare the clinical outcomes of these recipients with those of 59 recipients who underwent adult LDLT using right lobe grafts with normal PVs in the same period. Intraoperative PV stenting was necessary in 2 (8.3%) of the 24 recipients undergoing unification patch venoplasty. During the follow-up period, all PVs remained patent until death or censoring. No significant difference in terms of postoperative vascular complications was evident between the 2 groups. Moreover, no major complications requiring reoperation or endoscopic and/or radiologic intervention developed in any of the 24 living donors with double PVs. In conclusion, our simplified unification patch venoplasty could be safe and feasible when used to reconstruct double PV orifices in right lobe LDLT from donors with complex PV anomalies.