Auxiliary partial orthotopic living donor liver transplantation for a child with congenital absence of the portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the mesenteric vasculature in which visceral venous blood bypasses the liver, completely draining into the systemic circulation through a congenital porto-systemic shunt. Liver transplantation has rarely been indicated for patients with this disease. We present a child with CAPV who was managed successfully by living donor auxiliary partial orthotopic liver transplantation (APOLT), while preserving the right lobe of the native liver. In conclusion, APOLT for patients with CAPV is a feasible and ideal procedure because portal vein (PV) diversion is not necessary.     

End-to-side portocaval shunting for a small-for-size graft in living donor liver transplantation.

In the development of adult-to-adult living donor liver transplantation (LDLT), the small-for-size graft has been associated with poor clinical outcome. Persistent portal hypertension or portal venous overperfusion are considered to be causative factors, and partial diversion of portal flow to systemic circulation may be effective for avoiding injuries that occur in the small-for-size (SFS) graft. Recently, we constructed an end-to-side portocaval shunting using 1 of the portal branches and anastomosed the other branch with the portal vein of the graft in 2 cases of LDLT recipients transplanted with a SFS graft. With the suppression of portal hypertension, as well as sufficient portal flow to the graft, the recipients recovered successfully with favorable graft function. This new and simple technique may be able to be used as a feasible and effective method to attenuate the SFS syndrome.     

Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure

Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.     

Recanalized umbilico‐caval anastomosis as a temporary portosystemic shunt in pediatric living donor liver transplantation: the crossed fingers method

A temporary portocaval shunt (TPCS) associated with retrohepatic vena cava preservation prevents the edema caused by splanchnic congestion during liver transplantation (LT), especially for non‐cirrhotic cases. We herein report a modified TPCS technique using the recanalized umbilical vein and an end‐to‐side recanalized umbilico‐caval anastomosis for use during pediatric living donor liver transplantation (LDLT). This work evaluated a group of pediatric patients who underwent LDLT between 2001 and 2014 with the conventional TPCS (n=16) vs the recanalized umbilico‐caval shunt (the crossed fingers method, n=10). The crossed fingers method was performed by suturing an end‐to‐side anastomosis of the patent or recanalized umbilical vein to the vena cava using a continuous monofilament suture like “crossing the fingers,” that is, placing the left portal vein across the portal vein trunk next to it. The preoperative, surgical, and postoperative characteristics were similar in both groups except for the significantly shorter portal vein clamping time for the crossed fingers method. This method can allow the portal circulation to be totally decompressed before and after implanting the graft and while maintaining the hemodynamic stability throughout all stages of pediatric LDLT.     

Liver transplantation for fulminant hepatic failure without venovenous bypass and without portacaval shunting

Preservation of the caval vein during liver transplantation (OLT) has gained wide acceptance but portosystemic bypass or temporary portocaval shunt is still believed to be indicated in patients with fulminant hepatic failure. Herein we have described our initial experience with piggyback OLT without venovenous bypass and without portocaval shunting in five such patients. Division of the portal vein was always delayed until the native liver was completely dissected off the caval vein. The donor hepatic artery was anastomosed to the recipient aorta via an iliac interposition graft placed in the supraceliac position in two and at an infrarenal site in three patients. The ahepatic phase urinary output was low in the two patients in whom we applied supraceliac cross-clamping of the aorta. The mean ahepatic phase was 53 (45 to 67) minutes in four recipients who remained hemodynamically stable throughout surgery and prolonged to 5 hours in one patient due to a complicated supraceliac aortic anastomosis. Its repair resulted in hemodynamic instability, multiorgan failure, and death at 4 days following OLT. Four (80%) patients are alive in good condition with normal liver function after a mean of 12 (5 to 25) months of follow-up. In summary, liver transplantation for fulminant hepatic failure may be safely performed without venovenous bypass and without temporary portocaval shunting if the ahepatic phase is minimized and portal flow to the liver maintained up to the moment of hepatic excision. Arterial anastomosis with the supraceliac aorta prolongs the ahepatic phase and may impair kidney function: therefore, it should be avoided in these patients.     

Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein.

Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children, the majority of whom have no signs of encephalopathy and only slightly abnormal liver function tests. Additional anomalies common in CAPV are cardiovascular abnormalities and hepatic tumors. To date, only 5 adult patients (>18 years) with CAPV have been described, none of whom underwent liver transplantation. We describe a 45-year-old man with CAPV and end-stage renal insufficiency due to focal segmental glomerulopathy, who developed therapy-resistant encephalopathy with intermittently high ammonia levels. The patient underwent a combined liver and kidney transplantation and is doing well at 2.5 years of follow-up. Histopathological examination of the native liver showed no portal vein branches in the portal tracts. In conclusion, our experience suggests that, although children with CAPV usually have no symptoms of encephalopathy, this may still develop at a later stage in adult life. When encephalopathy becomes refractory to medical therapy, orthotopic liver transplantation (OLT) can be successfully performed with restoration of normal cerebral function.     

Renoportal anastomosis in right lobe living donor liver transplantation: report of a case

End-stage liver disease is often accompanied by thrombosis of the portal vein and the formation of splanchnic collateral vessels. Successful liver transplantation in such situations is more likely if the surgeon uses a strategy to establish a graft inflow. A 59-year-old male with a decompensated liver secondary to idiopathic portal hypertension underwent living donor liver transplantation (LDLT) using a right lobe liver graft donated from his son. His portal venous trunk was atrophied and a splenorenal shunt drained the mesenteric venous flow into the systemic circulation. LDLT was performed with renoportal anastomosis (RPA) using his right internal jugular vein as an interposed venous graft, without dissecting the collateral vessels. Although he developed temporary functional hyperbilirubinemia, he was discharged from the hospital 23 days after LDLT. This case suggests that RPA is a useful technique to manage patients with an obstructed portal vein and a splenorenal shunt.     

Left lobe adult-to-adult living donor liver transplantation: Should portal inflow modulation be added?

Recently, the successful application of portal inflow modulation has led to renewed interest in the use of left lobe grafts in adult-to-adult living donor liver transplantation (LDLT). However, data on the hepatic hemodynamics supporting portal inflow modulation are limited, and the optimal portal circulation for a liver graft is still unclear. We analyzed 42 consecutive adult-to-adult left lobe LDLT cases without splenectomy or a portocaval shunt. The mean actual graft volume (GV)/recipient standard liver volume (SLV) ratio was 39.8% ± 5.7% (median = 38.9%, range = 26.1%-54.0%). The actual GV/SLV ratio was less than 40% in 24 of the 42 cases, and the actual graft-to-recipient weight ratio was less than 0.8% in 17 of the 42 recipients. The mean portal vein pressure (PVP) was 23.9 ± 7.6 mm Hg (median = 23.5 mm Hg, range = 9-38 mm Hg) before transplantation and 21.5 ± 3.6 mm Hg (median = 22 mm Hg, range = 14-27 mm Hg) after graft implantation. The mean portal pressure gradient (PVP - central venous pressure) was 14.5 ± 6.8 mm Hg (median = 13.5 mm Hg, range = 3-26 mm Hg) before transplantation and 12.4 ± 4.4 mm Hg (median = 13 mm Hg, range = 1-21 mm Hg) after graft implantation. The mean posttransplant portal vein flow was 301 ± 167 mL/minute/100 g of liver in the 38 recipients for whom it was measured. None of the recipients developed small-for-size syndrome, and all were discharged from the hospital despite portal hyperperfusion. The overall 1-, 3-, and 5-year patient and graft survival rates were 100%, 97%, and 91%, respectively. In conclusion, LDLT with a left liver graft without splenectomy or a portocaval shunt yields good long-term results for adult patients with a minimal donor burden.     

Living Donor Liver Transplantation with Left Liver Graft

Small-for-size syndrome in LDLT is associated with graft exposure to excessive portal perfusion. Prevention of graft overperfusion in LDLT can be achieved through intraoperative modulation of portal graft inflow. We report a successful LDLT utilising the left lobe with a GV/SLV of only 20%. A 43 year-old patient underwent to LDLT at our institution. During the anhepatic phase a porto-systemic shunt utilizing an interposition vein graft anastomosed between the right portal branch and the right hepatic vein was performed. After graft reperfusion splenectomy was also performed. Portal vein pressure, portal vein flow and hepatic artery flow were recorded. A decrease of portal vein pressure and flow was achieved, and the shunt was left in place. The recipient post-operative course was characterized by good graft function. Small-for-size syndrome by graft overperfusion can be successfully prevented by utilizing inflow modulation of the transplanted graft. This strategy can permit the use of left lobe in adult-to-adult living donor liver transplantation.     

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.

Rat liver transplantation for total vascular reconstruction, using a suture method

We developed a novel protocol for rat orthotopic liver transplantation (OLT), using a suture method to establish hepatic artery flow. After determining that early inferior vena cava (IVC) unclamping maintained better circulation compared with the portal vein (PV) using porto-systemic shunted recipients, we developed a rat OLT model with total vascular reconstruction using a suture method. After connecting the suprahepatic IVC, the infrahepatic IVC was anastomosed, using a running suture method. IVC circulation was established immediately. The PV was anastomosed without intestinal congestion, using porto-systemic shunted recipients. The aortic conduit, including the donor celiac and hepatic artery, was anastomosed to the recipient abdominal aorta end-to-side. Eight of 11 OLT cases (72.7%) survived indefinitely. Biliary connection was achieved using a one-stent method. Three cases died 3-5 days postoperatively. Hepatic angiography showed good patency. The graft liver was histologically normal in long-surviving rats.     

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.     

The New Method of Time-Lag Ligation for Portosystemic Shunt Using Coronary Artery Bypass Graft Occluder for Adult Living Donor Liver Transplantation

We performed a living donor liver transplantation (LDLT) for a 57-year-old man who had end-stage liver failure with portal hypertension and an inferior mesenteric vein–left testicular vein (IMV-LTV) shunt. At operation, we did not clamp the shunt but encircled it with a coronary artery bypass graft (CABG) occluder (Sumitomo Bakelite K.K., Japan), which was passed outside the body through the abdominal wall to time-lag ligation (TLL). On postoperative day (POD) 5, we observed decreased portal flow. We performed TLL of the shunt using the CABG occluder without re-laparotomy. The portal flow increased, while the portal vein pressure increased slightly. In LDLT, portosystemic shunt has been reported to be a cause of portal thrombus formation or graft liver atrophy due to decreased PV flow in the mid postoperative period. However, perioperative ligation of a portosystemic shunt may prevent regeneration of the grafted liver because of excessive portal hypertension. Therefore the technique of time-lag ligation of a portosystemic shunt using a CABG occluder may be a minimally invasive, useful method to achieve physiological liver graft regeneration.     

The Use of Recipient Superficial Femoral Vein as a Venous Graft for Portal Vein Reconstruction in Right Lobe Living Donor Liver Transplantation

In living donor liver transplantation (LDLT), portal vein thrombosis (PVT) in the recipient is frequently regarded as a contraindication. To reconstruct the PV of a right-lobe liver graft (RLG) using an interposition or jump graft from the splenomesenteric junction, various vein grafts and technical modifications have been introduced. The internal jugular, external iliac, or great saphenous veins have been utilized in such reconstructive procedures. However, the superficial femoral vein (SFV) is preferable to the autologous vein grafts in terms of caliber, wall thickness, and length. We employed the recipient SFV to reconstruct PVT among 40 adult LDLT using RLG. Thirty-three were reconstructed by single end-to-end anastomosis with the right or left recipient PV. Three patients were transplanted with a RLG using 2 separated PVs reconstructed by double anastomoses with both the right and left PVs of the recipient. The remaining 4 patients required venous grafting for portal reconstruction. We used the recipient SFV as an interposition or jump graft from the splenomesenteric junction to the graft PV. There were 2 cases of anastomotic PV stenosis; 1 in portal reconstruction without a venous graft and the other with a SFV graft. Both were treated successfully by balloon angioplasty. The recipient SFV is an excellent size match for the PV reconstruction as a long interposition or jump conduit when the venous system from the deceased donor is not available. The indication for LDLT in patients with complete PVT should be carefully decided before transplantation in terms of portal reconstruction.     

Risk factors for portal vein complications in pediatric living donor liver transplantation

Shibasaki S, Taniguchi M, Shimamura T, Suzuki T, Yamashita K, Wakayama K, Hirokata G, Ohta M, Kamiyama T, Matsushita M, Furukawa H, Todo S. Risk factors for portal vein complications in pediatric living donor liver transplantation.
Clin Transplant 2010: 24: 550–556.
© 2009 John Wiley & Sons A/S. Abstract: Background: Portal vein (PV) complications in pediatric living donor liver transplantation (LDLT) are often asymptomatic in the early stages after transplantation and can be serious enough to lead to graft failure. There have been few reports on risk factors for PV complications in LDLT. The aim of this study is to investigate the influence of hepatic inflow upon PV complications and to predict patients at risk for these complications. Material/method: From 1997 to 2008, 46 pediatric patients underwent LDLT at our center. Portal venous and hepatic arterial flows and PV diameter were analyzed. Results: PV complications were identified in seven patients (15.2%) and occurred at a younger age and lower weight. As a result of appropriate treatment, none of the patients suffered graft failure. Analysis of the 46 patients and 27 patients under two yr of age indentified smaller PV diameter in recipient and larger discrepancy of PV diameter as risk factors. Portal venous flow tended to be low, in contrast to hepatic arterial flow, which tended to be high. Conclusion: PV size strongly influences PV complications. Other factors such as younger age, low portal venous flow, and high hepatic arterial flow may be risk factors for PV complications.     

Living domino liver transplantation in an adult with congenital absence of portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.     

Optimal portal venous circulation for liver graft function after living-donor liver transplantation

BACKGROUND: Previous studies have shown poor outcome after living-donor liver transplantation (LDLT) as a result of excessive portal venous pressure (PVP), excessive portal venous flow (PVF), or inadequate PVF. We investigated optimal portal venous circulation for liver graft function after LDLT in adult recipients retrospectively. METHODS: Between June 2003 and November 2004, 28 adult patients underwent LDLT in our institution. We modulated PVP under 20 mmHg in these 28 cases by performing a splenectomy (n=4) or splenorenal shunt (n=1). The PVF and PVP were measured at the end of the operation. Compliance was calculated by dividing PVF by PVP. RESULTS: PVF and compliance showed a significant inverse correlation with peak billirubin levels after LDLT (r = -0.63: r=-0.60, P<0.01), and with peak international normalized ratio after LDLT (r=-0.41: r=-0.51, P<0.05). Compliance was higher in right-lobe graft with middle hepatic vein cases (148+/-27 ml/min/mmHg), and lower in left-lobe graft cases (119+/-50 ml/min/mmHg). CONCLUSIONS: Liver graft function was better when PVF and graft compliance were higher and PVP was maintained under 20 mmHg.     

Portal vein arterialization for liver transplantation with extensive portomesenteric vein thrombosis: a case report

We report herein a case of extensive thrombosis of portal venous system including mesenteric vein in a 70-year-old man who suffered from end-stage post-hepatitis C cirrhosis and who underwent orthotopic liver transplantation. There was no way to divert portal blood flow to the new liver because such an extensive thrombosis of portomesenteric venous system. There are some case reports of portocaval hemitransposition with some success but high mortality. We decided to arterialize the portal vein of the liver allograft with the recipient hepatic artery and the donor hepatic artery was anastomosed to the supraceliac aorta. He recovered slowly from the operation. At 1 year after the transplantation, he is doing well with perfect liver function tests. This case challenges our belief that portal blood flow is essential for the liver because of hepatotrophic factors.     

Prospective Evaluation of Intraoperative Hemodynamics in Liver Transplantation with Whole,Partial and DCD Grafts

The interaction of systemic hemodynamics with hepatic flows at the time of liver transplantation (LT) has not been studied in a prospective uniform way for different types of grafts. We prospectively evaluated intraoperative hemodynamics of 103 whole and partial LT. Liver graft hemodynamics were measured using the ultrasound transit time method to obtain portal (PVF) and arterial (HAF) hepatic flow. Measurements were recorded on the native liver, the portocaval shunt, following reperfusion and after biliary anastomosis. After LT HAF and PVF do not immediately return to normal values. Increased PVF was observed after graft implantation. Living donor LT showed the highest compliance to portal hyperperfusion. The amount of liver perfusion seemed to be related to the quality of the graft. A positive correlation for HAF, PVF and total hepatic blood flow with cardiac output was found (p = 0.001). Portal hypertension, macrosteatosis >30%, warm ischemia time and cardiac output, independently influence the hepatic flows. These results highlight the role of systemic hemodynamic management in LT to optimize hepatic perfusion, particularly in LDLT and split LT, where the highest flows were registered.     

Mesorenal shunt using inferior mesenteric vein and left renal vein in a case of LDLT

Adult-to-adult living donor liver transplantation (LDLT) has become an established treatment option around the world. However, small-for-size graft syndrome remains one of the most serious complications affecting transplant outcomes. Excessive portal hypertension and overperfusion have been shown to play a causative role in this graft injury. Recently, portal hypertension per se has been considered detrimental to graft function, and thus to be avoided for successful outcomes after LDLT. We constructed a mesorenal shunt with anastomosis of the inferior mesenteric vein and left renal vein in the case of an LDLT recipient who showed high portal vein pressure after graft reperfusion. The inferior mesenteric vein is close to the left renal vein, and the anastomosis was obtained with relative ease. The shunt was effective in decreasing portal vein pressure, and postoperative graft function was satisfactory. This new method represents an option for attenuating portal hypertension when elevated portal vein pressure is observed in adult LDLT after graft reperfusion.