Biliary and duodenal complications after « en bloc» liver-small bowel transplantation in children. A single center cohort study.

Background: The technique of « en bloc» liver and small bowel transplantation (L-BT) spares a biliary anastomosis, but does not protect against biliary complications. We analyze biliary and duodenal complications (BDC) in our pediatric series.

Methods: Between 1994 and 2020, 54 L-BT were performed in 53 children.

Tumor rupture in hepatoblastoma: A high risk factor?

Background: Hepatoblastoma tumor rupture is a high-risk criterion in the SIOPEL 3/4 protocol. Little is known about the outcome of these children.

Methods: Radiological signs of possible tumor rupture, defined as peritoneal effusion, peritoneal nodules, or hepatic subcapsular hematoma, were reported in 24 of 150 patients treated for hepatoblastoma in France from January 2000 to December 2014 after central radiological expert review.

Interventional Radiology-Guided Procedures in the Treatment of Pediatric Solid Tumors: A Systematic Review and Meta-Analysis.

Introduction:  The use of interventional radiology (IR) in the treatment of pediatric solid tumors has markedly increased over the last three decades. However, data on effectiveness of IR-techniques, such as embolization/ablation, are scarce. In this systematic review and meta-analysis, we examined the outcomes of IR-procedures in the treatment of solid tumors in children.

Phase I/II Trial of Liver-derived Mesenchymal Stem Cells in Pediatric Liver-based Metabolic Disorders: A Prospective, Open Label, Multicenter, Partially Randomized, Safety Study of One Cycle of Heterologous Human Adult Liver-derived Progenitor Cells (HepaStem) in Urea Cycle Disorders and Crigler-Najjar Syndrome Patients.

Background: Regenerative medicine using stem cell technology is an emerging field that is currently tested for inborn and acquired liver diseases.

Objective: This phase I/II prospective, open label, multicenter, randomized trial aimed primarily at evaluating the safety of Heterologous Human Adult Liver-derived Progenitor Cells (HepaStem) in pediatric patients with urea cycle disorders (UCDs) or Crigler-Najjar (CN) syndrome 6 months posttransplantation. The secondary objective included the assessment of safety up to 12 months postinfusion and of preliminary efficacy.

Congenital portosystemic shunts: diagnosis and treatment.

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia.

Diagnosis, treatment and outcome of hepatic venous outflow obstruction in paediatric liver transplantation: 24-year experience at a single centre.

Background: Hepatic venous outflow obstruction after paediatric liver transplantation is an unusual but critical complication.

Objectives: To review the incidence, diagnosis and therapeutic modalities of hepatic venous outflow obstruction from a large national liver transplant unit.

Materials And Methods: During the period from October 1992 to March 2016, 917 liver transplant procedures were performed with all types of grafts in 792 children.

Rhabdoid tumor of the liver: Report of 6 pediatric cases treated at a single institute.

Background: Rhabdoid tumors (RTs) of the liver are rare, aggressive and nonsecreting malignancies occurring mainly during the first year of life. Definition of RT relies on characteristic morphology and on the inactivation of the SMARCB1 tumor suppressor gene. The aim of this study was to analyze clinical data, treatments and outcomes in our patients.

Abdominal Arterial Anomalies in Children With Alagille Syndrome: Surgical Aspects and Outcomes of Liver Transplantation.

Objectives: Angiogenic defects secondary to gene mutations of JAG1 and NOTCH2, causing arterial anomalies in Alagille syndrome (AGS), are well described in the literature. The study analyzes the frequency of abdominal arterial anomalies in children with AGS with an emphasis on outcomes following liver transplantation (LT).

Methods: Between 1988 and 2013, 242 children with AGS were treated at our institution.

Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children.

Background: Undifferentiated embryonal sarcoma of the liver is a rare malignant mesenchymal tumour occurring mostly in children ages 6-10 years. The discrepancy between its solid appearance on US and cystic-like appearance on CT has been described.

Objective: To study the imaging particularities and similarities among our cases of undifferentiated embryonal sarcoma and to report the errors in initial diagnoses.

The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia.

Objectives: To describe and evaluate an additional sonographic sign in the diagnosis of biliary atresia (BA), the microcyst of the porta hepatis, in comparison with previously described signs.

Methods: Ultrasound performed in 321 infants (mean age 55 days) with cholestasis were retrospectively analyzed. BA was surgically confirmed in 193 patients and excluded in 128.

DCDC2 Mutations Cause Neonatal Sclerosing Cholangitis.

Neonatal sclerosing cholangitis (NSC) is a rare biliary disease leading to liver transplantation in childhood. Patients with NSC and ichtyosis have already been identified with a CLDN1 mutation, encoding a tight-junction protein. However, for the majority of patients, the molecular basis of NSC remains unknown.

Feasibility and Diagnostic Accuracy of Supersonic Shear-Wave Elastography for the Assessment of Liver Stiffness and Liver Fibrosis in Children: A Pilot Study of 96 Patients.

Purpose: To evaluate the feasibility of using supersonic shear-wave elastography (SSWE) in children and normal values of liver stiffness with the use of control patients of different ages (from neonates to teenagers) and the diagnostic accuracy of supersonic shear wave elastography for assessing liver fibrosis by using the histologic scoring system as the reference method in patients with liver disease, with a special concern for early stages of fibrosis.

Materials And Methods: The institutional review board approved this prospective study. Informed consent was obtained from parents and children older than 7 years.

Pitfalls in the surgical management of undifferentiated sarcoma of the liver and benefits of preoperative chemotherapy.

Aim: Undifferentiated sarcoma of the liver (USL) is the third commonest malignant liver tumor in children. The aim of our study is to evaluate the outcome of this rare entity according to the quality of the surgical resection and the compliance to the European pediatric soft tissue sarcoma group guidelines.

Patients And Methods: We conducted a monocentric review of patients referred to our department with a definitive pathologic diagnosis of USL between 1997 and 2013.

Obliterative portal venopathy: a study of 48 children.

Childhood obliterative portal venopathy presents at any age and may be genetic in origin. We report 48 children with obliterative portal venopathy, based on strict histologic criteria, investigated between 1972 and 2011. Diagnosis requires histology and is suggested by ultrasonography findings.

Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy.

Objective: To propose an anatomical classification of congenital portosystemic shunts (CPSs) correlating with conservative surgery.

Background: CPSs entail a risk of life-threatening complications because of poor portal inflow, which may be prevented or cured by their closure. Current classifications based on portal origin of the shunt are not helpful for planning conservative surgery.

Hepatobiliary intervention in children.

Various vascular and nonvascular hepatobiliary interventional radiology techniques are now commonly performed in children's hospitals. Although the procedures are broadly similar to interventional practice in adults, there are important differences in indications and technical aspects. This review describes the indications, techniques, and results of liver biopsy, hepatic and portal venous interventions and biliary interventions in children.

Dose-dense cisplatin-based chemotherapy and surgery for children with high-risk hepatoblastoma (SIOPEL-4): a prospective, single-arm, feasibility study.

Background: The objective of this study was to establish the efficacy and safety of a new treatment regimen consisting of dose-dense cisplatin-based chemotherapy and radical surgery in children with high-risk hepatoblastoma.

Methods: SIOPEL-4 was a prospective single-arm feasibility study. Patients aged 18 years or younger with newly diagnosed hepatoblastoma with either metastatic disease, tumour in all liver segments, abdominal extrahepatic disease, major vascular invasion, low α fetoprotein, or tumour rupture were eligible.

Efficacy of irinotecan single drug treatment in children with refractory or recurrent hepatoblastoma--a phase II trial of the childhood liver tumour strategy group (SIOPEL).

Purpose: To assess the clinical activity of irinotecan as single drug in children with refractory or recurrent hepatoblastoma.

Patients And Methods: Four cycles of irinotecan were administered (20mg/m(2)/day intravenous (i.v.

Improved Hepatocyte Engraftment After Portal Vein Occlusion in LDL Receptor-Deficient WHHL Rabbits and Lentiviral-Mediated Phenotypic Correction In Vitro.

Innovative cell-based therapies are considered as alternatives to liver transplantation. Recent progress in lentivirus-mediated hepatocyte transduction has renewed interest in cell therapy for the treatment of inherited liver diseases. However, hepatocyte transplantation is still hampered by inefficient hepatocyte engraftment.

Hepatic haemangioma-prenatal imaging findings, complications and perinatal outcome in a case series.

Background: The clinical presentation of foetal hepatic haemangioma (HH) is highly variable, from asymptomatic to life-threatening.

Objective: The aim of this study was to describe foetal hepatic haemangioma and identify prognostic factors.

Materials And Methods: Antenatal and postnatal imaging studies, clinical and biological records of infants with antenatally diagnosed HH (2001-2009) were reviewed.