Long-term outcomes of transmesenteric portal vein recanalization for the treatment of chronic portal vein thrombosis after pediatric liver transplantation.

Portal vein thrombosis (PVT) may occur at any time following liver transplantation. We describe our experience with portal vein recanalization in cases of thrombosis after liver transplantation. Twenty-eight children (5%) out of 566 liver transplant recipients underwent portal vein recanalization using a transmesenteric approach. All children received left hepatic segments, developed PVT, and had symptoms or signs of portal hypertension. Portal vein recanalization was performed via the transmesenteric route in all cases. Twenty-two (78.6%) patients underwent successful recanalization and stent placement. They received oral anticoagulants after the procedure, and clinical symptoms subsided. Symptoms recurred due to portal vein restenosis/thrombosis in seven patients. On an intention-to-treat basis, the success rate of the proposed treatment was 60.7%. Only 17 out of 28 children with posttransplant chronic PVT retained stent patency (primary + assisted) at the end of the study period. In cases of portal vein obstruction, the transmesenteric approach via minilaparotomy is technically feasible with good clinical and hemodynamic results. It is an alternative procedure to reestablish the portal flow to the liver graft that can be performed in selected cases and a therapeutic addition to other treatment strategies currently used to treat chronic PVT.

Successful domino liver transplantation in maple syrup urine disease using a related living donor

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient's mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD.

Successful domino liver transplantation in maple syrup urine disease using a related living donor.

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient's mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD.

Orthotopic liver transplantation without venovenous bypass using the conventional and piggyback techniques.

INTRODUCTION: Orthotopic liver transplantation is a widely used procedure for the treatment of irreversible liver diseases for which there is no possibility of medical treatment. When this procedure is performed by the conventional technique, the retrohepatic vena cava is removed along with the native liver. The inferior vena cava (IVC) remains clamped until the revascularization of the graft, and in this period there is a reduction in the venous return, which may induce a fall by up to 50% in the cardiac output with hemodynamic instability and a fall in renal perfusion pressure. The use of a portal-femoral-axillary venovenous bypass system, in which the blood from the femoral and portal veins returns to the heart via the axillary vein propelled by a centrifugal pump, is intended to minimize the effects of the IVC clamping. In the piggyback (PB) technique, the native liver is removed and the IVC of the recipient is preserved and only partially clamped. We have employed both techniques without the use of venovenous bypass for 10 years. The objective of this study was to compare the results obtained from the use of the two techniques. PATIENTS AND METHODS: A retrospective analysis was performed of 195 patients transplanted between 1999 and 2008: 125 by the conventional technique and 70, the PB technique. The intraoperative parameters were analyzed (surgical time, ischemia time, use of blood products, and diuresis), as well as intensive care support (duration of stay in intensive care unit and use of vasoactive drugs), period of intubation, length of hospital stay, renal function, graft function, postoperative complications, retransplantation, and patient survival. RESULTS: The PB group showed a reduction in surgical time, warm ischemia time, the use of packed red blood cells concentrates, and fresh frozen plasma, as well as mortality at 30 days (P<.05). There were no differences in relation to cold ischemia time, intraoperative diuresis; length of stay and use of vasoactive drugs in the intensive care unit; the period of intubation; the duration of hospital stay; the renal function; the graft function; the need for reoperation; the incidence of sepsis, biliary complications, vascular complications; need for retransplantation; and 1-year mortality. The cumulative survival rate at 1 year was significantly better among the PB patients. CONCLUSION: Orthotopic liver transplantation can be performed without venovenous bypass with good results, using either the conventional technique or the PB technique. Provided that there is no technical contraindication and a long ischemia period is not foreseen, the PB technique should be the technique of choice.