Liver Transplant in Children with Hepatoblastoma.

OBJECTIVES: In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Sisli Memorial Hospital Transplantation Center are presented. MATERIALS AND METHODS: We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information. RESULTS: The median age of the 10 patients included in our study was 13.5 months (range, 8-120 mo), and the median weight was 10 kg (range, 6.5-30 kg). Two of the patients were twins. Five patients had pretreatment extent of disease III (centrally placed cases), and five had pretreatment extent of disease IV hepatoblastoma. Preoperative chemotherapy was given to 7 patients as cisplatin plus doxorubicin and to 3 patients per the International Childhood Liver Tumors Strategy Group 3 High-Risk Protocol at external centers. These protocols were administered according to treatment center preference. Nine patients received transplants from living donors. Two grafts were right lobes, and 7 were left lateral segments. In the remaining patient, a whole liver was received from a deceased donor. The histopathologic subgroups were epithelial in 5 patients, with others being of mixed type. Postoperative complications occurred in 3 patients as infection, intra-abdominal fluid collection, and acute rejection. The median follow-up was 32 months. One patient died because of lung metastasis within 9 months after transplant. CONCLUSIONS: Centers should offer liver transplant to patients with centrally located tumors. For centers that have an insufficient number of deceased donors, living-donor liver transplant with optimal planning and early treatment can be performed.

Lessons Learned From Review of a Single Center Experience With 500 Consecutive Liver Transplants in a Region With Insufficient Deceased-Donor Support.

OBJECTIVES: We present here the outcomes of our first 500 liver transplants and discuss the lessons learned during this time. MATERIALS AND METHODS: We retrospectively analyzed the first 500 consecutive transplants within the listing criteria of the United Network for Organ Sharing, with recipients and donors with minimum 1-year follow-up. Patient survival and donor complications were analyzed for 31 liver transplant recipients with hepatocellular carcinoma beyond the Milan criteria who had transplant performed during the same time. RESULTS: Between August 2006 and March 2013, there were 519 liver transplants performed in 500 patients (365 adult, 135 pediatric). There were 394 living-donor and 125 deceased-donor liver transplants. In addition, 31 adult liver transplants were performed in patients with hepatocellular carcinoma beyond Milan criteria (22 living-donor and 9 deceased-donor transplants). The main cause of chronic liver failure was biliary atresia in pediatric patients (30.4%) and chronic hepatitis B infection in adults (35.6%). The complication rate for primary nonfunction was 3.8%, overall biliary complications 24.0% (significantly higher after adult living-donor liver transplant, 30.3%), hepatic artery thrombosis 1.6%, portal vein thrombosis 3.0%, retransplant 3.8%, acute cellular rejection 29.6%, and bacterial infection 39.4%. Overall 1-, 3-, and 5-year patient survival rates in the first 500 consecutive transplants performed on recipients within United Network for Organ Sharing listing criteria were 87.8%, 85.0%, and 78.6%; for hepatocellular carcinoma patients beyond the Milan criteria, survival rates were 71.9%, 52.5%, and 38.2%. CONCLUSIONS: In regions without a sufficient number of deceased donors, living-donor liver transplant, with its associated problems, is the only alternative to deceased-donor liver transplant. Liver transplant requires teamwork, with all players working well together for a successful outcome. The important keys to success in liver transplant include decision-making, timing, surgical skills, experience, and close follow-up.

Calcification of Cryopreserved Arterial Graft Causing Delayed Obstruction of Portal Vein Flow After Liver Transplant.

In patients with biliary atresia, portal vein problems may cause challenges for liver transplant. Interposition grafts have been used for vascular anastomoses in transplant recipients with varied success. A cryopreserved iliac artery graft was used for the reconstruction of the portal vein in a 29-month-old infant with biliary atresia. At 17 months after transplant, she developed upper gastrointestinal bleeding that was caused by portal vein occlusion because of vascular calcifications in the graft. Upper gastrointestinal endoscopy showed esophageal varices with fresh bleeding, and the varices were band ligated. At 3 months after the bleeding episode, the patient was asymptomatic and biochemical tests were normal. In summary, liver transplant with cryopreserved iliac artery graft may be complicated by calcifications and portal vein occlusion, and caution is advised in using this graft material for portal vein anastomoses.

Preservation of the patent umbilical vein during a recipient hepatectomy: case report.

Clamping of the portal vein during a recipient hepatectomy during the anhepatic phase causes venous stagnation and hemodynamic instability. To prevent this, a temporary portocaval shunt is placed at some centers. This case report shows the patent umbilical vein of a patient undergoing a recipient hepatectomy, leading to a 20% reduction of pressure in the portal vein. Preservation of a patent umbilical vein may help prevent complications of high portal vein pressure during a recipient hepatectomy.

Current concepts in pediatric liver transplantation.

Liver transplantation is the definitive treatment for end-stage liver disease in both children and adults. Advances over the last 2 decades have resulted in excellent patient and graft survival rates in what were previously cases of fatal disorders. These developments have been due to innovations in surgical technique, increased surgical experience, refinements in immunosuppressive regimens, quality improvements in intraoperative anesthetic management, better understanding of the pathophysiology of the liver diseases, and better preoperative and postoperative care. Remarkably, the use of split-liver and living-related liver transplantation surgical techniques has helped mitigate the well-recognized national organ shortage. This review will discuss the major aspects of pediatric liver transplantation as it pertains to indication for transplantation, recipient selection and listing for orthotopic liver transplantation, pre-orthotopic liver transplantation care of children, optimal timing of orthotopic liver transplantation, surgical technical considerations, postoperative care and complications, and patient and graft survival outcomes.

Treatment of adenovirus hepatitis with cidofovir in a pediatric liver transplant recipient.

AdV hepatitis is a rarely reported complication after pediatric liver transplantation that is associated with high rates of morbidity, mortality and graft failure. Successful treatment of AdV relies on early diagnosis of disease by quantitative PCR measurement of adenoviral DNA in blood and histological evidence in tissue biopsy. Pharmacologic treatment largely consists of antiviral therapy with CDV, an acyclic nucleoside phosphonate analog and reduction in immunosuppression. This report describes a case of AdV hepatitis in a pediatric liver transplant recipient successfully treated with a modified, renal sparing dosing of CDV.

A rare intraabdominal tumor: giant hepatic artery aneurysm.

A 55-year-old man was investigated for right upper abdominal quadrant pain. He had no history of abdominal trauma or surgery. Imaging studies showed a common hepatic artery aneurysm involving the gastroduodenal artery. Following aneurysmectomy, examination with a hand Doppler apparatus yielded clear arterial signals from the liver surface. Therefore, vascular reconstruction was not performed. He had an uneventful postoperative course.

Obstructive jaundice due to a textiloma mimicking a common bile duct stone.

A foreign body is a rare cause of obstructive jaundice. We report a 19-year-old woman with jaundice caused by a surgical gauze in the common bile duct (CBD). Four yours earlier, she had undergone a cholecystectomy and drainage for hydatid disease of the liver. Her postoperative course was complicated by a biliary fistula that healed after 50 days. She now presented with obstructive jaundice of 2 weeks' duration. Magnetic resonance cholangiopancreatography (MRCP) showed a signal-void mass, consistent with a CBD stone. Surgical exploration of the CBD revealed a surgical gauze as the cause of the obstruction. To the best of our knowledge, this is the first case of a surgical gauze obstructing the CBD requiring surgical removal.

Oxidative damage in an experimentally induced gastric and gastroduodenal reflux model.

The exact pathophysiologic mechanisms of esophageal cell damage and carcinogenesis by gastroesophageal reflux are not clearly understood. The aim of this study was to evaluate the damage to the esophageal epithelium that occurs after acid reflex and mixed acid and bile reflux by assessing histopathology, reactive oxygen species, and DNA damage. Eighty 10-week-old male Sprague-Dawley rats were divided into two groups, an acid reflux group and a mixed (acid/bile) reflux group. Acid reflux was achieved by esophagogastroplasty in which mixed reflux was encouraged via esophagoduodenal anastomosis. Each group contained a control subgroup that underwent sham laparotomy alone. The rats were killed 3, 6, 9, and 12 months after surgery. Malondialdehyde, protein carbonyl content, and DNA damage were determined in lymphocytes. Histopathologic analysis was performed according to the histologic activity index. Inflammation, ulcer, and regeneration in both reflux groups were significantly increased in the esophagus at 3, 6, 9, and 12 months compared with the control group. Mucosal damage was greater in the mixed reflux group compared with the gastric reflux group. Malondialdehyde and carbonyl content in the serum, and DNA damage in lymphocytes, were significantly increased in both reflux groups. At 9 and 12 months, oxidative damage was increased in the mixed reflux group compared with the acid reflux group. Oxygen-derived free radicals seem to be one of the important mediators in the evaluation and generation of reflux esophagitis. The impact of oxygen free radicals, as demonstrated in this study, can be evaluated by assessing the damage that they incur to lipid membranes, serum proteins, and circulating lymphocyte DNA. Serum malondialdehyde and carbonyl content as well as lymphocyte DNA damage were significantly increased in the setting of acid and mixed acid/bile reflux in these rodent models. Further, these serum and lymphocytic changes were associated with esophageal ulceration, inflammation, and regeneration. Evaluation of such markers as serum malondialdehyde and carbonyl content as well as evaluation of lymphocyte DNA might prove to be useful investigations in patients with precancerous and cancerous conditions in addition to conventional methods of diagnosis. Further studies, both animal and human are warranted.