Living-donor liver transplantation with hyperreduced left lateral segment grafts: a single-center experience.

BACKGROUND: In the setting of liver transplantation in small infants who receive left lateral segment (LLS) grafts, problems are encountered related to graft-size mismatching in the form of so-called "large-for-size" grafts. To address these problems, the feasibility of further reducing the size of LLS grafts to form hyperreduced LLS (HRLLS) grafts was investigated. METHODS: Of the 175 pediatric living-donor liver transplantations performed between November 2005 and December 2011 at our institute, 31 cases were performed using HRLLS grafts. The medical records were reviewed and data were collected retrospectively. RESULTS: The graft-to-recipient body weight ratio was successfully reduced from 5.2% ± 2.0% to 2.9% ± 0.5%. Portal vein thrombosis was observed in one case, and biliary stenosis was seen in two cases. No hepatic artery thrombosis was encountered. The graft and patient 2-year survival rate was 87%. When the results categorized according to the original disease were verified, patients with fulminant hepatic failure (FHF) weighed less and had smaller abdominal cavities compared with patients with cholestatic or metabolic disease. Patients with FHF frequently required skin or partial skin closure to avoid graft compression. For this reason, the anteroposterior diameters in the recipients' abdominal cavities were not adequately large to accommodate the graft thickness, especially in patients with FHF. CONCLUSIONS: In conclusion, living-donor liver transplantation using HRLLS grafts offers a safe and useful option for treating smaller infants.

Incidentally detected cholangiocarcinoma in an explanted liver with biliary atresia after Kasai operation.

This report presents the case of a 30-yr-old woman with BA who developed incidental cholangiocarcinoma following the Kasai operation. She showed progressive liver dysfunction and cirrhosis at the age of 30 yr and underwent LDLT. A 4-cm-diameter liver tumor in the anastomotic site of portoenterostomy was incidentally found as a result of a pathological examination of the explanted native liver. The tumor was pathologically diagnosed to be intrahepatic cholangiocarcinoma. Although cholangiocarcinoma in patients with BA has been previously reported in only three cases, it should be nevertheless always considered in the differential diagnosis of hepatic tumors during a long follow-up course in patients with BA.

Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein.

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. A 14-year-old male with CAPV underwent living donor liver transplantation with a left lobe graft from his father. The portal vein reconstruction was achieved with a renoportal anastomosis using an interpositional graft from the native collateral vein, because portal venous system directly drains to the left renal vein without constructing the confluence of superior mesenteric vein and splenic vein. The patient is doing well with a normal liver function and mild hypoxemia.

Living donor liver transplantation using grafts with hepatic cysts.

Cystic lesions in the liver are often found through the evaluation of liver donors. Multiple cysts are worrisome, and donor candidates with multiple cysts may be unacceptable as liver donors, especially when their recipients have fibrocystic disease (FCD), which is an inherited disorder. This study reviewed 183 cases of living donor liver transplantation. We collected clinical and radiological data associated with donors with cystic lesions and with donors without cystic lesions, and we evaluated the outcomes of these donors and their recipients. As part of the preoperative radiological assessment of grafts, magnetic resonance cholangiography (MRC) was performed to evaluate the biliary anatomy of donor candidates with multiple cysts. Thirty-four donors (18.6%) had 1 or more cystic lesions in the liver, and 6 of these donors had multiple cysts (ie, >10). Donors with multiple cysts were older and heavier, and there was a significant relationship between these donors and recipients whose original disease was FCD. During the follow-up (median = 3.1 years), all donors with cystic lesions were found to be doing well without any major postoperative complications. Fifteen recipients who received grafts with cystic lesions (12 left-sided lobes and 3 right-sided lobes) had no complications related to the cystic lesions. In conclusion, donors with cystic lesions may be acceptable as liver donors, although our data are limited mostly to left-sided lobe donation with a short follow-up period. MRC should be preoperatively performed to rule out any biliary anomalies, especially in donor candidates whose recipients have FCD.

Diaphragmatic hernia in infants following living donor liver transplantation: report of three cases and a review of the literature.

DH is a rare complication following LT. This report presents three cases of right-sided DH after LT using a left-sided graft. All of the patients were younger than one yr of age, and they were critically ill owing to their original disease, characterized by biliary atresia, progressive familiar intrahepatic cholestasis, and acute liver failure. DH occurred with sudden onset within three months after LT. All of the cases were promptly diagnosed and treated. A literature review of 24 cases of DH identified four factors associated with DH: left-sided graft, right-sided DH, relatively delayed onset of DH, and age-specific chief complaint. DH following LT should be considered as a potential surgical complication when a left-sided graft is used, especially in small infants with coagulopathy and malnutrition.

Glycemic management in living donor liver transplantation for patients with glycogen storage disease type 1b.

GSD type 1b is an autosomal recessive inborn error of carbohydrate metabolism caused by defects of the G6Pase translocase (G6PT). Patients with GSD1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, and neutropenia. LT has been indicated for severe glucose intolerance. This study retrospectively reviewed glycemic management of eight children with a diagnosis of GSD1b who underwent liver transplantation (LDLT). Between November 2005 and September 2011, 172 children underwent LDLT, of which eight (4.7%) were indicated for GSD1b. Glucose-rich solution was placed in all children when preoperative fasting started to prevent preoperative hypoglycemia. During the reperfusion of graft, the glucose administration could significantly be reduced to maintain the proper blood glucose level, while the dosage of glucose administration prior to reperfusion of graft was significantly higher in the patients with GSD1b in comparison with patients with BA. The current series also showed significantly high incidence of infectious complications in the patients with GSD1b owing to persistent neutropenia after LDLT. All patients are doing well with an excellent quality of life owing to the stabilization of glucose intolerance. This current study clearly documented drastic change in glycemic management in LDLT. Cautious perioperative management to prevent hypoglycemia and infection is crucial for successful LT.

Portal vein reconstruction in pediatric living donor liver transplantation for patients younger than 1 year with biliary atresia.

BACKGROUND/PURPOSE: Infants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis. METHOD: Of 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique. The clinical characteristics of those who underwent the interposition reconstruction or the branch patch technique were compared, and the PV complications were assessed. RESULTS: Portal vein complications occurred in 5 patients (14.7%) in the branch patch group. There were 4 patient deaths, and all of them had received branch patch reconstruction. The branch patch reconstruction cases with a sclerotic small caliber (<4 mm) determined by using preoperative ultrasonography showed a significantly high mortality rate (44.4%). All patients with interposition vein graft reconstruction are still alive with excellent graft function without anticoagulation therapy. CONCLUSION: The interposition vein graft appears to be a feasible option with better graft survival and less PV complications when performing LDLT for biliary atresia in infants younger than 1.

Living-donor liver transplantation for propionic acidemia.

Propionic acidemia is a rare autosomal recessive disorder affecting the catabolism of branched-chain amino acids because of a genetic defect in PCC. Despite the improvements in medical treatment with protein restriction, sufficient caloric intake, supplementation of l-carnitine, and metronidazole, patients with the severe form of propionic acidemia have life-threatening metabolic acidosis, hyperammonemia, and cardiomyopathy, which results in serious neurologic sequelae and sometimes death. This study retrospectively reviewed three children with neonatal-onset propionic acidemia who received LDLT. Between November 2005 and December 2010, 148 children underwent LDLT, with an overall patient survival of 90.5%, in our center. Three patients were indicated for transplantation because of propionic acidemia. All recipients achieved a resolution of metabolic derangement and better quality of life with protein restriction and medication, although urine methylcitrate and serum propionylcarnitine levels did not decrease markedly. LT can reduce the magnitude of progressive cardiac/neurologic disability as a result of poor metabolic control. Further evaluation is therefore required to determine the long-term suitability of this treatment modality.

Pediatric liver-kidney transplantation for hepatorenal fibrocystic disease from a living donor.

The indications for and the timing of LT and/or KT for the patients with HRFCD are based on the severity of liver and kidney involvement. Most organs come from living donors, because the number of deceased donors is extremely low in Japan. Therefore, patients with HRFCD may need two organs from living donors. Four patients with HRFCD underwent living donor LT and KT from a single donor. The type of transplantation included combined LKT in one case, sequential LKT in two cases, and sequential KLT in one case. Although the case of combined LKT died because of sepsis, the other cases were doing well. Sequential LKT was successfully performed at the proper timing for each transplant; however, both of the donors suffered from a gastroduodenal ulcer after liver donation because of the psychological burden related to the relatively short period between two donations. In conclusion, living donation for LKT with cautious surgical procedures is not harmful for donors and recipients. However, changes in the allocation system established for deceased donors for HRFCD should be considered to avoid the need for two organ donations from the same living donor.

Micro-particle transporting system using galvanotactically stimulated apo-symbiotic cells of Paramecium bursaria.

It is well known that Paramecium species including green paramecia (Paramecium bursaria) migrate towards the anode when exposed to an electric field in a medium. This type of a cellular movement is known as galvanotaxis. Our previous study revealed that an electric stimulus given to P bursaria is converted to a galvanotactic cellular movement by involvement of T-type calcium channel on the plasma membrane [Aonuma et al. (2007), Z. Naturforsch. 62c, 93-102]. This phenomenon has attracted the attention of bioengineers in the fields of biorobotics or micro-robotics in order to develop electrically controllable micromachineries. Here, we demonstrate the galvanotactic controls of the cellular migration of P bursaria in capillary tubes (diameter, 1-2 mm; length, 30-240 mm). Since the Paramecium cells take up particles of various sizes, we attempted to use the electrically stimulated cells of P bursaria as the vehicle for transportation of micro-particles in the capillary system. By using apo-symbiotic cells of P bursaria obtained after forced removal of symbiotic algae, the uptake of the particles could be maximized and visualized. Then, electrically controlled transportations of particle-filled apo-symbiotic P bursaria cells were manifested. The particles transported by electrically controlled cells (varying in size from nm to /m levels) included re-introduced green algae, fluorescence-labeled polystyrene beads, magnetic microspheres, emerald green fluorescent protein (EmGFP)-labeled cells of E. coli, Indian ink, and crystals of zeolite (hydrated aluminosilicate minerals with a micro-porous structure) and some metal oxides. Since the above demonstrations were successful, we concluded that P bursaria has a potential to be employed as one of the micro-biorobotic devices used in BioMEMS (biological micro-electro-mechanical systems).

Liver transplantation in a patient with propionic acidemia requiring extra corporeal membrane oxygenation during severe metabolic decompensation.

LDLT is an effective treatment modality in patients with congenial metabolic liver disease. PA is a rare autosomal recessive disorder caused by deficiency in propionyl-CoA carboxylase. The present study demonstrates a two-yr-old girl with PA who was admitted for metabolic decompensation and immediately treated with CHD and protein intake restriction at 46 days of age. Two yr later, the patient was readmitted for severe metabolic decompensation with complete atrioventricular block and ventricular fibrillation. CHDF and ECMO were indicated because of progressive metabolic and cardiac deterioration. After full recovery of the ejection fraction, planned LDLT was performed to prevent further metabolic decompensation and fatal cardiac insufficiency. No significant events occurred after the operation and the condition of the patient is stable with continued protein restriction and carnitine supplementation.