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.     

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.     

Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess

A 27-day-old boy had convulsion associated with brain abscesses and severe hypoxemia at the age of 3 months. Congenital absence of the portal vein (CAPV) and some associated anomalies were detected by radiological examinations. Brain abscess and hypoxemia were thought to be serious complications resulting from CAPV and were successfully corrected by living donor liver transplantation at the age of 4 months. This is the first report of a successful transplantation indicated for intrapulmonary shunt and brain abscess in an infant with CAPV.     

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.     

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.     

A rare case of complex vascular reconstruction in liver transplantation.

Abnormalities of recipient or donor vascular structures are associated with reconstructive difficulties in liver transplantation. A patient with thrombosis of the right hepatic vein and associated stricture of the inferior vena cava (IVC), portal vein thrombosis and multiple aberrant arteries underwent orthotopic liver transplantation. The donor's suprahepatic IVC was anastomosed to the recipient's intrathoracic IVC. The portal vein flow was restored by venous graft interposition, while the arterial flow was ensured by interposing an iliac arterial graft anastomosed to the infrarenal aorta. In conclusion, graft function remains excellent more than 5 years postoperatively.     

Living donor liver transplantation with replacement of vena cava for Echinococcus alveolaris: A case report

INTRODUCTION

There is no medical treatment for alveolar echinococceal disease (AED) of liver till now. Curative surgical resection is optimal treatment but in most advanced cases curative resection can’t be done. Liver transplantation is accepted treatment option for advanced AED. AED in some case invade surrounding tissue especially inferior vena cava (IVC). Advanced AED with invasion to IVC can be treated with deceased liver transplantation. Although living donor liver transplantation is very difficult to perform in patients with advanced AED with resected IVC, it come into consideration, since there is very few cadaveric liver.

PRESENTATION OF CASE

Here we present a case with advanced stage of AED of liver which cause portal hypertension and cholestasis. AED invaded surrounding tissue, right diaphragm, both lobes of liver and retrohepatic part of IVC. Invasion of IVC forced us to make resection of IVC and reconstruction with cryopreserved venous graft to reestablish blood flow. After that a living donor liver transplantation was done.

DISCUSSION

Curative surgery is the first-choice option in all operable patients with AED of liver. Advanced stage of AED like chronic jaundice, liver abscess, sepsis, repeated attacks of cholangitis, portal hypertension, and Budd-Chiari syndrome may be an indication for liver transplantation. In some advanced stage AED during transplantation replacement of retrohepatic part of IVC could be done with artificial vascular graft, cadaveric aortic and caval vein graft.

CONCLUSION

Although living donor liver transplantation with replacement of IVC is a very difficult operation, it should be considered in the management of advanced AED of liver with IVC invasion because of the rarity of deceased liver.     

在两个阶段开展临床肝移植的体会(附8例报告)

目的总结两个阶段开展临床肝移植的经验和体会。方法分析8例人体原位肝移植,包括1例肝肾联合移植的治疗结果。供肝保存第一阶段采用门静脉及肝动脉离体灌注,第二阶段采用腹主动脉及肠系膜上静脉UW液快速原位灌注。受体手术采用原位肝脏植入,吻合次序为肝上下腔静脉、门静脉、肝下下腔静脉、肝动脉和胆管,完成门静脉吻合后即开放门静脉结束无肝期。两个阶段均使用了免疫抑制剂但方法不同。结果第一阶段移植生存期最短39天,最长203天。除1例死于肿瘤复发外,全部死于败血症及严重感染。第二阶段移植病人1例半年后死于肿瘤转移,另3例仍健存,其中肝肾联合移植者生存已超过3年,在社会上正常生活工作。结论第二阶段取得了较好的效果。其中合理选择适应症,良好的供肝保存以及免疫抑制剂的使用等对肝移植的成功有重要影响。     

Liver Transplantation in an Adult With Adenomatosis and Congenital Absence of the Portal Vein: A Case Report

Congenital absence of the portal vein (CAPV) is a rare congenital anomaly in which the superior mesenteric veins (SMV) and splenic veins converge and bypass the liver, effectively draining directly into the systemic venous circulation via the inferior vena cava (IVC), or alternatively the renal or iliac vein, creating a native portosystemic shunt. Portosystemic shunting results in clinical manifestations of hepatic encephalopathy as well as a predisposition to focal nodular hyperplasia and tumors, including adenomas, hepatoblastoma, and hepatocellular carcinoma (HCC), by the disruption of enterohepatic blood flow. Historically, CAPV has been thought to be a rare condition found mainly at autopsy, however, in recent years due to advances in radiological techniques, CAPV detection has increased. Herein we describe a patient with known CAPV who initially underwent hepatic resection for HCC. During surveillance, additional masses were discovered and were identified as recurrent HCC. Unfortunately, this patient was not a candidate for further resection or locoregional therapy. We demonstrate that transplantation is a challenging but technically viable option for treatment of HCC complicating adenomatosis-associated CAPV.     

Successful hepatic transplantation in congenital absence of recipient portal vein

The first case of hepatic transplantation in a patient with congenital absence of the portal vein (CAPV) is reported. A 10-year-old girl with biliary atresia and CAPV underwent successful hepatic transplantation and has normal liver function 9 months after transplantation. This case is only the seventh reported case of CAPV. Patients with CAPV commonly have additional liver anomalies, cardiac and inferior vena cava anomalies, and polysplenia. Surprisingly, hepatic encephalopathy is not a prominent feature in patients with CAPV despite systemic drainage of mesenteric venous blood. CAPV should not be considered a contraindication to hepatic transplantation.     

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

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

保留门静脉-腔静脉血流的肝移植实验研究

为探讨犬肝移植的新术式，以及不采取静脉转流的可行性，施行了犬保留门静脉－腔静脉（门－腔）血流的背驮式肝移植术。受体在切除自体肝之前先将肠系膜下静脉与右肾平面以下的下腔静脉行侧侧吻合，建立门－腔血流通路，然后再行原位肝移植术；测定受体切肝及植肝等５个时限的血流动力学参数。结果５只犬有４只存活，在下腔静脉及门静脉吻合后５～１０分钟即有胆汁分泌。血流动力学的有关指标表明，造成心血管系统不平衡、肾瘀血、胃肠瘀血等危险因素被解除。认为本术式更符合人的内环境及生理，避免了静脉转流术的各种弊端，为无转流泵的中心施行肝移植找到了新的途径。     

Successful Urgent Transplantation of an Adult Kidney into a Child with Inferior Vena Cava Thrombosis

Poor venous drainage options following inferior vena cava (IVC) thrombosis have been considered to complicate or preclude renal transplantation of adult kidneys into pediatric patients. We describe urgent renal transplantation in a 5-year-old (15.3 kg) male with IVC thrombosis using an adult living donor. Preoperative magnetic resonance venography revealed a patent infrahepatic/suprarenal vena cava and portal system. In surgery, the right liver lobe was mobilized sufficiently to anastomose the graft renal vein to the native IVC at the confluence of the native left renal vein and proximal vena cava. Graft function has remained excellent with serum creatinine of 0.5 mg/dL at 36 months. IVC thrombosis need not preclude successful transplantation of adult-sized kidneys into children.     

Successful Living Donor Liver Transplantation in Portomesenteric Thrombosis

Chronic portal vein thrombosis (PVT) is often considered a relative contraindication for living donor liver transplantation due to the risks involved and higher morbidity. In this report, we describe a surgical strategy for living donor liver transplant in patient with complete PVT using venovenous bypass from the inferior mesenteric vein (IMV) and then using a jump graft from the IMV for portal inflow into the graft. IMV is a potential source for portal inflow in orthotopic liver transplant.     

Right posterior portal vein stenosis developed after living donor liver transplantation using a modified right lobe graft with a type 2 portal vein: a case report

Portal vein complications after liver transplantation (LT) can lead to graft liver failure. In this living donor liver transplantation case a stenosis developed in the right posterior branch of the portal vein of the graft liver from a living donor with type 2 portal vein variation. A 61-year-old woman diagnosed with hepatocellular carcinoma due to hepatitis B received a liver graft revealing a single lumen divided by a septum. The portal vein was anastomosed to the recipient portal vein without venoplasty. Postoperative Doppler sonogram revealed poor flow in the right posterior portal vein with compensatory arterial hyperperfusion. The postoperative computed tomography (CT) scan revealed narrowing of the proximal part of the right posterior portal vein with periportal tracking. Without intervention, the liver enzyme and bilirubin levels decreased to normal and the follow-up CT scan showed decreased periportal tracking. The patient was discharged without major complications. We believe that the posterior portal vein stenosis resulted from the direct anastomosis of the portal vein without a further venoplasty. Although there was no major complication due to the posterior portal vein stenosis in our patient, we suggest a venoplasty to prevent portal vein stenosis when using right lobe grafts with a type 2 portal vein, even if a single lumen is present and there is a margin for a direct anastomosis.     

Improved Quality of Life and Unchanged Magnetic Resonance Brain Imaging After Living Donor Liver Transplantation for Late-Onset Ornithine Transcarbamylase Deficiency: Report of a Case

We report the case of a 7-year-old girl with ornithine transcarbamylase deficiency whose quality of life (QOL) improved greatly after a living donor liver transplantation (LDLT). Ornithine transcarbamylase deficiency had been diagnosed when she was 2 years old and she finally underwent LDLT, with her father as the donor, when she was 7 years old. The patient had suffered episodes of hyperammonemic encephalopathy ranging from lethargy to coma, treated by hemodialysis twice before LDLT, and her intelligence quotient was borderline for her age. Preoperative magnetic resonance imaging (MRI) showed an atrophic area in the subcortical white matter of the frontal lobe. After LDLT, the patient suffered acute rejection with hyperamylasemia, but not hyperammonemia. Postoperative MRI and quantitative MR spectroscopy showed no changes in the subcortical lesion. She has been followed up carefully for 16 months and has had no further complications or any sign of hyperammonemia.     

Application of cryopreserved vein grafts as a conduit between the coronary vein and liver graft to reconstruct portal flow in adult living liver transplantation

Abstract:  Adult-to-adult living donor liver transplantation is an alternative to donation from a deceased individual, and can help relieve the shortage of liver donations available for adult patients in Asian countries. When transplant candidates have thrombosis and deterioration of the portal vein, living donor liver transplantation is relatively contraindicated because portal veins in the grafts are short and vein grafts may not be available to reconstruct the portal vein. From June 2003 to May 2007, 82 adult living donor liver transplantations were performed at Chang-Gung Memorial Hospital. Three patients had portal vein thrombosis and marked fibrosis of the portal vein and cryopreserved vein grafts were used to reconstruct portal flow from the engorged coronary vein to the graft portal vein. All vein grafts are patent and all patients have normal liver function at 21–36 months after transplantation. When cryopreserved vein grafts are available, adult living donor liver transplantation can be successfully performed in patients with marked deterioration of the portal vein. The short distance from the engorged coronary vein to the graft portal vein may decrease the incidence of re-thrombosis of the venous conduit.     

Functional portal flow competition after auxiliary partial orthotopic living donor liver transplantation in noncirrhotic metabolic liver disease

Auxilliary partial orthotopic liver transplantation (APOLT) was introduced initially as a tentative or permanent support for patients with potentially reversible fulminant hepatic failure and has extended its indication to congenital metabolic disorder of the liver that has otherwise normal functional integrity. Postoperative management of APOLT is complicated because of functional portal flow competition between the native and graft liver. The native portal vein diversion to the graft is sometimes indicated to prevent functional competition; however, it is still an open question whether this technique can be theoretically indicated for APOLT patients. The authors report a on patient with ornithine transcarbamylase deficiency who received APOLT from a living donor without native portal vein diversion. Because of functional portal vein competition between the native and graft liver, the patient had to have portal vein diversion, portal vein embolization, and finally native hepatectomy to induce the graft regeneration after APOLT. After the experience of the current case, primary portal vein diversion for APOLT with noncirrhotic metabolic liver disease patients to prevent functional portal flow competition is recommended.     

Posttransplant bilioportal fistula with portal vein thrombosis: a case report

An 8-year-old female patient, known to have post-Kasai biliary atresia with mild intrapulmonary shunting, underwent living donor liver transplantation because of recurrent cholangitis. After the treatment of postoperative biliary stricture with percutaneous transhepatic biliary drainage, the patient subsequently developed hematochezia with portal vein thrombosis. The intraoperative findings showed portal vein thrombosis with a bilioportal fistula. We performed closure of the bilioportal fistula and reconstruction of the portal vein with a native internal jugular vein interposition graft. A bilioportal fistula due to percutaneous hepatobiliary procedures is a reportedly a rare complication following liver transplantation. The patient is currently doing well after a successful surgical intervention.     

Portal vein stenosis: a rare yet clinically important cause of delayed-onset ascites after adult deceased donor liver transplantation: two case reports

Vascular complications following liver transplantation are well documented. While complications involving the portal vein are less common than the hepatic artery, portal vein complications can lead to potentially life-threatening sequelae including graft loss. Portal vein stenosis is an infrequent complication following liver transplant. The majority of these complications are seen in living donor liver transplants and pediatric liver transplants. We present 2 cases of delayed onset portal vein stenosis in adult deceased donor liver transplantation (ADDLT). The first patient presented with refractory ascites twelve months after ADDLT. He was diagnosed and successfully treated with percutaneous transhepatic portovenography and venoplasty. The second patient had a history of irradiation to his portal bed in the setting of cholangiocarcinoma. He developed refractory ascites and esophageal variceal bleeding >2 years after ADDLT. He underwent percutaneous transhepatic portovenoplasty, but eventually required placement of a portal stent due to continued problems with recurrent ascites. These 2 cases highlight the importance of considering portal vein stenosis in the differential diagnosis of late-onset ascites following liver transplantation, especially if there have been any predisposing risk factors such as portal bed irradiation or prior splenectomy.