Search

Hepatoblastoma (HB) is the most common malignant liver tumor in children. Although the development of treatment strategies with advances in chemotherapy has greatly improved the prognosis of HB, surgical resection and liver transplantation still play a vital role in the treatment of HB. In recent years, technological innovations have led to the development of new surgical approaches for HB. In this review, we describe the latest research on the surgical management of HB, including new imaging technologies, minimally invasive approaches, and the application of associating liver partition portal vein ligation for staged hepatectomy. We also discuss the current role of liver transplantation, use of ante-situm or ex-situ liver resection with auto-transplantation, and management of metastatic HB.

The impact of pediatric liver transplantation on intellectual development has yet to be determined. We investigated the intellectual outcomes of school-aged patients after living donor liver transplantation for biliary atresia in infancy. The Wechsler Intelligence Scale for Children—fourth edition test was administered to 20 patients who survived $$\ge $$ 5 years after living donor liver transplantation. Borderline full scale intelligence quotient was defined as ≤ 85. Pre-, peri-, and postoperative data were compared between patients with > 85 and ≤ 85 to identify predictive factors of borderline performance. The one-sample t test demonstrated that the mean full scale intelligence quotient of patients after transplantation for biliary atresia was significantly lower than that of the general population (91.8 vs. 100.0, p = 0.026) and 7 (35%) were classified as intellectual borderline functioning. Multivariable logistic regression models were unable to identify any factors predictive of full scale intelligence quotients of ≤ 85. This is the first study to indicate that the mean full scale intelligence quotient among school-aged patients who underwent living donor liver transplantation for biliary atresia in infancy is significantly lower than that of the general population.

Portal vein complications (PVCs) after adult living donor liver transplantation (LDLT) are potentially lethal. We categorized PVCs by the time of onset (early versus late

Introduction Portal vein thrombosis (PVT) and portal vein stenosis (PVS) are rare complications after liver transplantation that can lead to graft failure and patient death. Material and methods The aim of this study was to evaluate the effect of interventional treatment for PVT and PVS occlusion after liver transplantation. Follow-up data of 7 patients who underwent stent replacement for PVT and/or PVS were analyzed. The clinical success, complications, and portal vein patency were analyzed. Results Clinical success was obtained in 6 of the 7 patients. No portal hypertension-related symptoms reoccurred in the 6 patients during the follow-up. Conclusions Interventional radiologic treatment produced a high success rate and a favorable long-term outcome.

Hypogenesis or agenesis of right hepatic lobe is a rare abnormality and is generally associated with gallbladder and biliary tract abnormalities. Cases of biliary injury following cholecystectomy have been reported in patients with agenesis of right hepatic lobe because the anatomical anomalies complicate the surgical approach. We report a case of laparoscopic cholecystectomy in a patient with hypogenesis of right hepatic lobe. A 92-year-old male patient was admitted to our hospital with fever and right lower abdominal pain with suspected acute appendicitis. Abdominal computed tomography revealed gallstones with acute cholecystitis and hypogenesis of right hepatic lobe. He underwent laparoscopic cholecystectomy with the left semilateral decubitus position. The patient's postoperative course was uneventful. In conclusions, some patients with liver lobe hypoplasia do not present with the typical symptoms of acute cholecystitis due to dislocation of the gallbladder. The left semilateral decubitus position with modified placement of port sites is useful for laparoscopic cholecystectomy in patients with hypogenesis of right hepatic lobe.

Adhesions due to previous upper abdominal surgery may complicate later liver transplantation. Here we report successful living donor liver transplantation (LDLT) in a patient with a history of total gastrectomy. A 32-year-old Japanese woman developed end-stage liver failure due to alcoholic cirrhosis. She had undergone total gastrectomy, pancreato-splenectomy, and partial colectomy due to rupture of a pancreatic cyst. LDLT was performed using a right lobe graft from her sister. To minimize blood loss and injury to the jejunum, adhesions between the left lobe and nearby organs were dissected without blood flow in or out of the liver. The right liver graft was implanted uneventfully. She was extubated on postoperative day (POD) 1, but then developed septic shock due to aspiration pneumonia on POD 2. She was reintubated and antibiotics and antifungal agents were administered. Administration of tacrolimus was changed to an intravenous route on POD 3. Her condition improved and she was re-extubated on POD 9. On POD 14, tacrolimus was administered orally. She was discharged from our hospital on POD 30 without any other events and is doing well 6 months after LDLT. We believe that careful planning, such as mobilizing the left lobe with the blood flow blocked just before liver explantation, elevating the head of the bed during tube-feeding, and calculating the area under the curve after drug administration will enable liver transplantation for patients with a history of total gastrectomy.

We herein present the case of a four-yr-old boy with PA who developed AMR after ABO-incompatible LDLT despite undergoing B cell desensitization using rituximab. Although the CD19+ lymphocyte count decreased to 0.1% nine days after the administration of rituximab, he developed a high fever which was accompanied by arthralgia due to a streptococcal infection 13 days after rituximab prophylaxis. After the clearance of the infection, he underwent ABO-incompatible LDLT 36 days after the administration of rituximab. The CD19+ lymphocyte count just prior to LDLT was 1.2%. He developed AMR five days after LDLT, and the antidonor-type IgM and IgG antibody titers increased to 1:1024 and 1:1024, respectively. He was treated by plasma exchange, IVIG, steroid pulse therapy, and rituximab re-administration; however, his liver dysfunction continued. Despite intensive treatment, he died due to complicated abdominal hernia, acute renal failure, and ARDS. This case suggests that a streptococcal infection may induce the activation of innate immune responses; thus, additional desensitization therapy should be considered prior to ABO-incompatible LDLT if B cell reactivation is suspected.

Background For most patients with liver failure receiving maintenance renal replacement therapy (RRT), treatment with living-donor liver transplantation (LDLT) alone is indicated in Japan. Material and Methods We retrospectively reviewed patients who underwent LDLT while receiving RRT in our hospital. Results Three of the 5 patients who underwent LDLT while on RRT died during the first year after transplantation. Conclusions The indications for liver transplantation in patients on RRT require careful examination.