Riccardo A. Superina

BACKGROUND: Liver transplantation is increasingly advocated as primary surgical therapy for children with hepatoblastoma involving 3 or 4 sectors of the liver after neoadjuvant chemotherapy. This study evaluated the results of nontransplant hepatectomy in children who might otherwise have been considered for liver transplantation.METHODS: All children who underwent resection at a single institution from 1998 to 2009 for POST‐TEXT IV or centrally located POST‐TEXT III hepatoblastoma after neoadjuvant chemotherapy were reviewed.RESULTS: Fourteen children (7 boys) with a median age of 8 months at diagnosis met study criteria. Pulmonary metastases in 3 patients were resected in 2 and resolved with chemotherapy in 1 patient. Preoperative grouping after neoadjuvant chemotherapy was POST‐TEXT IV in 3 patients and POST‐TEXT III in 11 patients. Thirteen of 14 (93%) children who underwent aggressive resection despite being potential candidates for primary transplantation were alive and tumor‐free with a median follow‐up of 57 months. Observed survival rates at 1, 2, and 5 years were 93%, 91%, and 88% respectively. Event‐free survival rates at 1, 2, and 5 years were 93%, 91%, and 75%, respectively.CONCLUSIONS: Excellent survival (93%) was obtained with aggressive resection in children with POST‐TEXT III and IV hepatoblastoma meeting criteria for transplant referral. The 1 death occurred in a patient with unfavorable small cell histology. These children should be managed at institutions experienced in both advanced pediatric hepatobiliary surgery and transplantation. Operative exploration was frequently required to ultimately determine which tumors can be resected and which require transplantation. Cancer 2011. © 2010 American Cancer Society.

Choledochal cyst is a relatively uncommon entity in Western countries. No reports of choledochal cyst in heart transplant patients have been reported to date. We report two cases of choledochal cyst in pediatric heart transplant recipients, one with post-transplant lymphoproliferative disorder (PTLD) within the cyst. The first patient had abdominal pain, increased liver enzymes and was seropositve for Epstein-Barr virus. A choledochal cyst with PTLD was removed 4 years after heart transplantation. The second patient presented 14 years after heart transplantation with a choledochal cyst that was excised for severe abdominal pain. This previously unreported association between choledochal cysts in conjunction with PTLD and heart transplantation is interesting and a possible common pathogenesis is proposed. The management and alternative treatments were briefly noted. We recommend an aggressive treatment for patients with suspected choledochal cyst after heart transplantation because of the increased potential for malignant transformation.

The treatment of portal hypertension in children has undergone considerable evolution in the past decade. The treatment offered depends on the cause of the hypertension and the underlying health of the liver. The diagnosis of portal hypertension often can be made by the history and physical examination. Upper gastrointestinal bleeding in the presence of splenic enlargement is pathognomonic for portal hypertension. Bleeding and hypersplenism are the principal symptoms. Treatment of bleeding starts with confirming the diagnosis with esophageal and gastric endoscopy. The patient is admitted to an intensive care unit and started on intravenous octreotide. Banding or sclerosis of esophageal varices will result in cessation of the bleeding but not a permanent cure. A careful investigation for the cause of the portal hypertension should be done. This includes imaging studies of intra-abdominal arteries and veins, a liver biopsy, and liver function tests, including coagulation studies. For patients with extrahepatic portal vein thrombosis, early consideration should be given to surgical treatment with a meso-Rex bypass. Patients with Liver disease should be treated For the underlying disorder and undergo regular endoscopic monitoring for recurrence of varices. Patients with well-compensated cirrhosis should be considered for selective surgical shunting, and those with advanced disease for liver transplantation. The benefit of long-term beta blockers in children has not been proven by clinical trials.

Purpose of review Hepatoblastoma is the commonest primary liver tumor in children and may pose a significant treatment dilemma. Determination of the resectability can be challenging. For those tumors deemed unresectable, liver transplantation now offers real hope for cure. Recent findings Modern chemotherapy has improved long-term survival, but complete resection of the tumor is still essential for cure. Liver transplantation, as a primary surgical intervention, has emerged as a viable treatment option for unresectable hepatoblastoma, rivaling the results obtained after resection of lesser stage lesions. In contrast, rescue transplantation for disease relapse after partial hepatectomy has been associated with less favorable long-term results. Pretransplant chemotherapy given to reduce tumor bulk in hopes of making a tumor resectable should be limited so that posttransplant chemotherapy can be given if possible. Summary Posttransplant chemotherapy should be entertained in all children undergoing transplantation. The treatment of patients with central lesions adjacent to the main hepatic/portal veins, or multifocal and solitary tumors involving all four sectors should be undertaken in a transplant center. The choice of resection versus transplantation may not always be obvious and the decision should be made by an experienced team taking into consideration all factors in order to obtain the best chance for cure.

The goal of this study was to determine the effectiveness of mesenteric vein to left portal vein bypass operation (MLPVB) in correcting extrahepatic portal vein thrombosis (EHPVT) in children. The treatment of idiopathic EHPVT has been primarily palliative, whereas MLPVB restores hepatic portal flow in patients with EHPVT. Thirty-four children with symptomatic EHPVT underwent surgery with intent to perform MLPVB and were followed for up to 7 years. MLPVB was successful in 31 patients (91%), all of whom maintain patent vein grafts and have symptomatic relief of EHPVT in follow-up. All patients had complete relief from gastrointestinal bleeding. Patients with hypersplenism had significant increases in platelet and leukocyte counts and reduction in spleen size. Superior mesenteric vein flow increased from 119 +/- 66 mL/min before bypass to 447 +/- 225 mL/min (P < 0.0001) after surgery. Postoperative blood flow in the bypass graft expressed as a fraction of calculated ideal portal flow for size correlated inversely with age (P < 0.001). Left-portal vein diameter increased from 2.6 +/- 1.6 mm to 7.3 +/- 2.4 mm 2 years after surgery (P < 0.002). Liver volume increased from 703 +/- 349 cm3 to 799 +/- 351 cm3 1 week after surgery (P < 0.001). Prothrombin time improved to normal in all patients 1 year after surgery. MLPVB provides excellent relief of symptoms in children with idiopathic EHPVT and results in liver growth and normalization of coagulation parameters. This surgery is corrective and should be done at as early an age as possible.

We studied the impact of multiple bile duct anastomosis on the development of biliary complications after liver transplantation in children. A total of 101 patients received a primary liver transplant and were divided into 2 groups: those with a single bile duct (n = 77) and those with multiple bile ducts (n = 24). Mean follow-up was 39.8 ± 20.8 months. A total of 27 patients presented with biliary complications (26.7%), 18 patients (18.7%) presented with early complications (12 leaks and 6 strictures), and 9 patients (8.9%) had late strictures. Hepatic artery thrombosis (HAT) and multiple bile ducts were significant risk factors for the development of biliary complications, and the presence of multiple bile ducts was an independent risk factor. Patients with multiple bile ducts had a significantly greater incidence of total biliary complications compared to those with single ducts. Patients with multiple ducts had a higher incidence of leaks when compared to those in the single duct group, but the incidence of strictures, both early and late, was similar in both groups. One-year patient and graft survivals were not statistically different in the 2 groups. In conclusion, the presence of more than one bile duct in the graft is an independent risk factor for the development of biliary complications after pediatric liver transplantation. (Liver Transpl 2005;11:161–166.)