Successful reconstruction of late portal vein stenosis after hepatic transplantation

Stenosis of the portal vein anastomosis occurred in three pediatric patients seven to 42 months after transplantation. Dominant symptoms were those of portal hypertension and hypersplenism. Diagnosis was made by angiography. Successful surgical reconstruction was possible in all three patients.     

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.     

Pharmacokinetic analysis of cyclosporine in a renal transplant recipient with congenital absence of the portal vein

Here we report therapeutic drug monitoring of cyclosporine in a kidney transplant recipient lacking enterohepatic circulation. The patient developed steroid‐resistant nephrotic syndrome at age 14 years, and was medicated with an oral cyclosporine microemulsion. However, her cyclosporine trough level was unexpectedly elevated, and subsequent investigations showed that she was deficient in drug metabolism as a result of the congenital absence of the portal vein. Her renal function gradually decreased and she became dialysis‐dependent at the age of 21 years, and kidney transplantation was planned. Based on pretransplant therapeutic drug monitoring, we started cyclosporine microemulsion at half of the conventional dosage. After transplantation, the dosage was successfully adjusted to achieve a target trough level. The post‐transplant course was stable with no symptoms of rejection or cyclosporine‐associated nephrotoxicity.     

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.     

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.     

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.     

Explanted portal vein grafts for middle hepatic vein tributaries in living-donor liver transplantation

BACKGROUND: The availability of a venous graft is limited in the setting of living donor liver transplantation (LDLT), and the management of the middle hepatic vein middle hepatic vein tributaries in right lobe LDLT still remains controversial. METHODS: Twenty-three right lobe LDLT grafts, with the reconstruction of middle hepatic vein tributaries using the explanted portal veins from the explanted livers, were evaluated for the patency, postLDLT liver function tests, and graft survival. RESULTS: The methods of outflow reconstruction were classified into three types: the interposition of the graft to the middle/left hepatic vein (n=12), to the vena cava (n=9), and to the vena cava as a co-orifice with the graft right hepatic vein (n=2). The 1- and 3-year patency rates were 76.7% and 76.7% respectively, with the graft occlusion in five cases. The occluded cases (n=5) had significantly higher aspartate aminotransferase and alanine transaminase levels as compared with those of patent cases (n=18) at 4 weeks after transplantation (P<0.01). However, there was no significant difference in the total bilirubin and prothrombin time in either group during the observation periods. The 1- and 3-year graft survival rates were 91.1% and 91.1%, respectively. In addition, there was no graft loss due to occlusion. CONCLUSION: The use of the recipient's explanted full-length hilar portal vein for the reconstruction of the middle hepatic vein tributaries is thus considered to be a feasible and valuable strategy in the setting of a right lobe LDLT, where appropriate vascular grafts are not always available.     

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.     

Acute portal vein thrombosis secondary to donor/recipient portal vein diameter mismatch after orthotopic liver transplantation: a case report

The incidence of portal vein thrombosis in end-stage liver disease is estimated as varying between 5% and 21%, whereas in candidates undergoing liver transplantation, this is 3-13%. Portal vein thrombosis occurring after liver transplantation can be managed surgically by thrombectomy, retransplantation, splenorenal shunt, or Wall-stent placement, or nonsurgically by angioplasty, local high-dose infusion of thrombolytic agents, combination of portal thrombolysis, or embolization of a pre-existing spontaneous splenorenal shunt. We report a case of portal vein thrombosis after liver transplantation diagnosed on postoperative day 1 in a 57-year-old patient who received a liver from an 8-year-old donor. The patient was successfully treated surgically with portal vein thrombectomy and systemic anticoagulation. Portal vein thrombosis, in this case, was considered to be secondary to size discrepancy between the donor and the recipient portal veins. Routine use of daily Doppler ultrasound was the key factor in early diagnosis.     

Congenital absence of the portal vein and role of liver transplantation in children.

BACKGROUND/PURPOSE: Congenital absence of the portal vein (CAPV) is a subtype of congenital portosystemic shunt, which can cause a broad spectrum of clinical manifestations. The authors report on 4 patients with CAPV including a boy with CAPV-associated encephalopathy, which was resolved effectively by liver transplantation (LT). METHODS: The case records of 4 pediatric patients with CAPV who were referred to the author's institution between 1984 and 1999 were reviewed. RESULTS: The patients (3 boys and 1 girl) ranged in age at diagnosis from 0.8 to 14 years. Two patients had growth retardation or disturbed consciousness, and the other 2 had no specific manifestations. Not only high serum levels of bile acids, ammonia, and transaminases but also low plasma levels of branched-chain amino acids were common laboratory findings. The absent portal vein was replaced by a large portosystemic shunt, which connected the splanchnic vein to the inferior vena cava or the left renal vein. Two patients survived without any symptoms, but 1 with growth retardation died of hepatic failure. The other with encephalopathy did not respond to medical therapy and underwent LT, which resolved symptoms and metabolic disorders effectively. CONCLUSIONS: Patients with CAPV do not always have a good prognosis. They should be followed up with careful observation of their symptoms, hepatic function, and metabolic abnormalities. LT might be indicated for patients with symptomatic CAPV unresponsive to medical therapy.     

Auxiliary rat liver transplantation with portal vein arterialization in acute hepatic failure

BACKGROUND: The aim of our work was to study the effect of the portal vein arterialization of an auxiliary liver graft on survival, liver function, and regeneration of the native liver suffering from surgically induced acute liver failure (ALF). METHODS: In Lewis rats (control group: n=10), ALF was induced by resection of about 85% of liver tissue. The auxiliary liver graft (reduced size of 30%) was transplanted into the right upper quadrant of the abdomen (trial group: n=12). The portal vein was arterialized via the renal artery. The infrahepatic vena cava was anastomosed end-to-side, and the bile duct was implanted into the duodenum. RESULTS: Survival rate over a 3-month period was 10/12 in the trial group vs. 2/10 in the controls. In the trial group, the prothrombin time rose up to 38+/-2 sec on day 1 after surgery (control group: 66+/-6 sec); on day 5 after surgery, it returned to values of 30+/-1 sec. On day 1 after surgery, serum albumin fell to 25+/-1 g/L (preoperative value: 32+/-1 g/L). Within 3 weeks, it returned to normal. The hepatobiliary scan on day 7 after surgery showed normal uptake in the liver graft, whereas the uptake of the native liver was distinctly reduced. After 3 months, the transplanted liver had atrophied (0.6% of body weight), the native liver hypertrophied (2.5% of body weight), with a normal total weight for both livers of 3.1% of body weight. CONCLUSIONS: Thus, auxiliary liver transplantation with arterialized portal vein allows maintenance of liver function at the time of ALF and regeneration of the native liver.     

Reconstruction of middle hepatic vein of a living-donor right lobe liver graft with recipient left portal vein.

Venous outflow problems in right lobe, living-donor liver transplantation are uncommon, but devastating when they occur. We describe the successful use of the recipient's left portal vein as an interposition graft to drain a dominant middle hepatic vein in a right lobe liver transplant. Two weeks after transplantation, the vein graft accounted for 56% of the total venous outflow of the liver.     

超声引导下经门静脉穿刺干细胞移植治疗肝纤维化

Liver cirrhosis;Mesenchymal stem cell transplantation     

Congenital absence of the portal vein.

A 14-year-old girl presented at the hospital after discovering an abdominal tumor. CT scan and ultrasonography indicated a hepatic tumor and also revealed the absence of the portal vein. The patient was admitted to excise the hepatic tumor. It was found that the venous blood from the small intestines flowed into the left renal vein and then emptied directly into the inferior vena cava. A tumor extending from the right lobe through the middle portion of the liver was excised. The postoperative course was satisfactory and marked regeneration of the residual hepatic tissue was observed. Also the blood level of ammonia in the superior mesenteric vein was low, approximately 120 micrograms/dl, compared to the normal value of 350 micrograms/dl in the portal vein. This low blood level may indicate the presence of some homeostatic control mechanism.     

Simultaneous hepatic artery and portal vein thrombosis after living donor liver transplantation

Simultaneous hepatic artery and portal vein thrombosis rarely occurs after liver transplantation. The etiology is unknown. Of 213 patients (72 children and 141 adults) that underwent living donor liver transplantation (LDLT) from January 1996 to March 2003, 4 (2%) developed simultaneous thrombosis at 3 hours to 7 days (median, 4 days) after the operation. Emergent thrombectomy was performed in three patients; the remaining patient was registered in the Japan organ transplant network. All of the patients died due to hepatic failure (range, 18 hours to 6 days after the diagnosis; median, 2 days). Portal vein, hepatic artery, and hepatic vein velocity in the liver graft were measured every 12 hours by Doppler ultrasonography for 2 weeks after liver transplantation. These parameters were stable until just before the simultaneous thrombosis. These findings indicate that protocol Doppler ultrasonography can diagnose, but not predict, this fatal complication.     

Left lobe living related liver transplantation in the absence of an extrahepatic left portal vein

Absence of a normal left extrahepatic portal vein is considered to be a contraindication to left lobe living-related liver transplantation. This report is of a successful case of living- related liver transplantation using a left lobe procured in a patient presenting with an absent horizontal segment of the left extrahepatic vein.