Publications by authors named "Daniele Pariente"

52 Publications

Tumor rupture in hepatoblastoma: A high risk factor?

Pediatr Blood Cancer 2020 09 3;67(9):e28549. Epub 2020 Jul 3.

Gustave Roussy, Department of Children and Adolescents Oncology, Université Paris-Saclay, Villejuif, France.

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.

Results: Twenty-two patients with available clinical data were included (nine PRETEXT-I/II, six PRETEXT-III, seven PRETEXT-IV, and five had lung metastases). Five patients had a subcapsular hematoma only, and 17 patients had intraperitoneal rupture (subcapsular hematoma and peritoneal effusion). A hepatic biopsy was performed in 19 patients. Intraperitoneal rupture occurred before biopsy in 12 and after biopsy in three (including one with prebiopsy subcapsular hematoma) (missing data: two). All patients were treated with chemotherapy, with high-risk regimens including cisplatin or carboplatin and doxorubicin in 19 and cisplatin or carboplatin alone in three. Liver surgery was performed in 20 patients (including three liver transplants). Fifteen patients (68%) achieved complete remission. With a median follow-up of 5.5 years, 11 events occurred (six progressions and three relapses, including three peritoneal progressions/relapses, one surgical complication, and one second cancer) and eight patients died. One of eight patients with no other high-risk criterion had a relapse. The three-year event-free survival and overall survival rates were 49.6% (95% CI = 30-69) and 68.2% (40-84), respectively.

Conclusions: Tumor rupture is predictive of poor prognosis with risk of peritoneal progression/relapse. However, it should not be a contraindication for liver transplantation.
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http://dx.doi.org/10.1002/pbc.28549DOI Listing
September 2020

Establishing local diagnostic reference levels for pediatric percutaneous transhepatic cholangiography interventions and optimizing the routine practice.

Pediatr Radiol 2020 05 18;50(6):827-832. Epub 2020 Feb 18.

Diagnostic Radiology Department, Bicetre University Hospital, 78 Rue Général Leclerc, 94270, Le Kremlin-Bicêtre, France.

Background: Liver-transplanted, immunosuppressed pediatric patients undergoing repeated percutaneous transhepatic cholangiography (PTC) require optimized exposure to ionizing radiation.

Objective: To establish local diagnostic reference levels (DRL) for pediatric PTC and investigate the routine use of X-ray equipment.

Materials And Methods: The study retrospectively analyzed data collected between October 2016 and June 2018 from a single center performing PTC. We collected exposure parameters including kerma area product (P), air kerma at patient entrance reference point (K) and fluoroscopy time via a dose archiving and communication system. Local diagnostic reference levels were derived as the 50th percentile of the distributions while considering published recommended weight groups. We investigated exposure variability with procedure complexity and with technical parameters recovered from the radiation dose structured report.

Results: The analysis included 162 PTC procedures performed in 64 children: 58% male, average age 6 years (range 39 days to 16 years) and weight 24 kg (range 3-60 kg). Local DRLs for weight groups 0-5 kg, 5-15 kg, 15-30 kg, 30-50 kg and 50-80 kg were, respectively, 6 cGy.cm, 22 cGy.cm, 68 cGy.cm, 107 cGy.cm and 179 cGy.cm in P. Local DRLs per weight group were also established for intermediate and complex procedures. Radiation dose structured report analysis highlighted good local practice with efficient collimation, low fluoroscopy pulse rate, no magnification and limited use of radiographic acquisitions. Meanwhile, table and detector positioning and tube projection could still be optimized. P correlated significantly with the number of acquisitions and tube-to-table distance.

Conclusion: We established local DRLs for children undergoing PTC.
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http://dx.doi.org/10.1007/s00247-020-04627-yDOI Listing
May 2020

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

Eur J Pediatr Surg 2020 Aug 23;30(4):317-325. Epub 2019 Jun 23.

Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Department of Pediatric Surgery and Urology, Descartes University, Université de Paris, Paris, France.

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.

Materials And Methods:  Using a defined search strategy, we searched for studies reporting the use of IR-techniques for pediatric solid tumors from 1980 to 2017. Reports with less than three patients, review, and opinion articles were excluded. The study was conducted under preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. We analyzed dichotomous and continuous variables by appropriate statistical methods.

Results:  Of 567 articles screened, 21 papers met the inclusion criteria (12 retrospective, 7 prospective, and 2 randomized-control trials). Many of the analyzed papers described relatively small cohorts of patients. IR-guided procedures were mainly rescue procedures to treat primarily unresectable tumors, local recurrences, or metastases. Inclusion/exclusion criteria and success definition were not specified in most reports. Major side effects were documented in 17/286 (6%) infants, while minor side effects were self-limiting in most patients. Six studies had a comparison between tumor embolization (127 infants) to surgery or chemotherapy without IR-procedures (113 controls). The meta-analysis showed lower mortality (16 vs. 47%) and surgical time for resection (206 vs. 250 m), higher 2-year tumor-free survival (82 vs. 36%), and favorable histology in IR group ( < 0.001 for all).

Conclusion:  IR-guided techniques are promising in the treatment of pediatric solid tumors. Further prospective (randomized) trials are needed to clarify efficacy.
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http://dx.doi.org/10.1055/s-0039-1692655DOI Listing
August 2020

Impact of Liver Diseases on Heart and Lungs.

JACC Cardiovasc Imaging 2019 10 15;12(10):2071-2075. Epub 2019 May 15.

Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France, and University Paris Diderot, Sorbonne Paris Cité, Paris, France; INSERM U1149, Centre de Recherche de L'inflammation, CRI, Paris, France.

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http://dx.doi.org/10.1016/j.jcmg.2019.03.020DOI Listing
October 2019

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.

Transplantation 2019 09;103(9):1903-1915

Department of Paediatrics, Paediatric Gastroenterology and Hepatology Unit, Cliniques Universitaires St Luc, Université Catholique de Louvain, Brussels, Belgium.

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.

Methods: Fourteen patients with UCDs and 6 with CN syndrome were divided into 3 cohorts by body weight and intraportally infused with 3 doses of HepaStem. Clinical status, portal vein hemodynamics, morphology of the liver, de novo detection of circulating anti-human leukocyte antigen antibodies, and clinically significant adverse events (AEs) and serious adverse events to infusion were evaluated by using an intent-to-treat analysis.

Results: The overall safety of HepaStem was confirmed. For the entire study period, patient-month incidence rate was 1.76 for the AEs and 0.21 for the serious adverse events, of which 38% occurred within 1 month postinfusion. There was a trend of higher events in UCD as compared with CN patients. Segmental left portal vein thrombosis occurred in 1 patient and intraluminal local transient thrombus in a second patient. The other AEs were in line with expectations for catheter placement, cell infusion, concomitant medications, age, and underlying diseases.

Conclusions: This study led to European clinical trial authorization for a phase II study in a homogeneous patient cohort, with repeated infusions and intermediate doses.
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http://dx.doi.org/10.1097/TP.0000000000002605DOI Listing
September 2019

Another point of view on 2017 PRETEXT.

Pediatr Radiol 2018 11 14;48(12):1817-1819. Epub 2018 Aug 14.

Department of Children and Adolescent Oncology, Gustave Roussy Cancer Campus, Villejuif, France.

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http://dx.doi.org/10.1007/s00247-018-4227-4DOI Listing
November 2018

Congenital portosystemic shunts: diagnosis and treatment.

Abdom Radiol (NY) 2018 08;43(8):2023-2036

Pediatric Surgery Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France.

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. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.
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http://dx.doi.org/10.1007/s00261-018-1619-8DOI Listing
August 2018

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

Pediatr Radiol 2018 05 21;48(5):667-679. Epub 2018 Feb 21.

AP-HP, Bicêtre Hospital, Pediatric Radiology Department, 78 rue du Gal Leclerc, 94270, Le Kremlin Bicêtre, France.

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. Transplants suspected to have early or delayed venous outflow obstruction were confirmed by percutaneous venography or surgical revision findings. Therapeutic intervention, recurrence and outcome were evaluated.

Results: Twenty-six of 792 children (3.3%) experienced post-transplant hepatic venous outflow obstruction. These patients had been diagnosed from 1 day to 8.75 years after transplantation. Six occurred during the early post-transplant period; in three of them, the graft was lost. Seventeen patients were initially treated by balloon angioplasty with success; 11 of these experienced recurrences. Four stents were implanted; one was complicated by definitive occlusion. Three of the five surgical revisions were successful. The initial stenosis involved the inferior vena cava in 10 grafts, in isolation or associated with hepatic vein involvement. Mean follow-up was 79 months after transplantation. Eight grafts were lost.

Conclusion: Acute postoperative hepatic venous outflow obstruction was associated with poor prognosis. Diagnostic venography should be performed if there is any suspicion of venous outflow obstruction, even if first-line examinations are normal. Stenosis frequently involved the inferior vena cava. Angioplasty was a safe and efficient treatment for venous outflow obstruction despite frequent recurrence.
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http://dx.doi.org/10.1007/s00247-018-4079-yDOI Listing
May 2018

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

J Pediatr Surg 2018 Mar 14;53(3):567-571. Epub 2017 Sep 14.

Pediatric Surgery Department-Hôpitaux universitaires Paris Sud, Hôpital Bicêtre Assistance Publique Hôpitaux de Paris, Université Paris XI, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.

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.

Patients And Methods: 6 cases of patients treated in our institution for RT of the liver between January 2007 and January 2015 are reported. Variables examined included age at diagnosis, tumor stage, treatment and long-term survival.

Results: Median age at diagnosis was 5months (range: 4-23). Normal for age serum AFP levels was observed in all patients. No patient presented with metastasis at diagnosis. The diagnosis of RT based on the loss of SMARCB1 was made early in 4 patients. The 2 others were initially diagnosed as nonsecreting hepatoblastomas. Median follow-up was 6years (range: 2-9). All patients received chemotherapy, with variable regimens depending on initial diagnosis, followed by surgical resection. Three patients (50%) died of disease. Two of them were mistaken for nonsecreting hepatoblastomas at diagnosis and had recurrence shortly after completion of treatment. The third one presented a cardiac right atrium thrombus. Three patients (50%) are long-term survivors; they received multimodal therapy including chemotherapy according to protocol EpSSG NRSTS consisting of doxorubicin and surgical removal of the tumor performed within 3months after diagnosis. One patient had adjuvant radiotherapy.

Conclusion: According to our results, search of SMARCB1 mutation or alternatively immunohistochemical assay for SMARCB1 in nonsecreting hepatoblastomas is mandatory to exclude RT. Chemotherapy according to EpSSG NRSTS protocol together with a surgical treatment seems justified to improve long-term survival.

Type Of Study: Retrospective study.

Level Of Evidence: Level IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2017.09.005DOI Listing
March 2018

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

J Pediatr Gastroenterol Nutr 2017 06;64(6):888-891

*Department of Pediatric Surgery †Department of Pediatric Radiology ‡Department of Pediatric Hepatology, Hôpitaux Universitaires Paris-Sud, Bicêtre Hospital, AP-HP, Université Paris XI, Le Kremlin-Bicêtre, France.

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. We performed a retrospective analysis of 55 who underwent LT during the study period. Preoperative abdominal arterial findings, operative reports, arterial reconstruction technique, and early as well as late complications following LT were reviewed specifically focusing on arterial thrombosis.

Results: Twenty-five patients had preoperative imaging available for analysis. Twelve of these patients showed celiac trunk stenosis (48.0%), 2, a superior mesenteric artery stenosis (8.0%) and one a stenosis of both renal arteries. Twenty patients (36.3%) underwent standard hepatic reconstruction using the native recipient hepatic artery. Thirty-five patients (63.7%) underwent aortic conduit reconstruction (ACR) from the infrarenal aorta using donor arterial conduits. Hepatic artery thrombosis occurred in 9 patients (16.3%). This number was higher in the standard arterial anastomosis group 7/20 (35.0%) than in those with ACR 2/35 (5.7%, P = 0.0079).

Conclusions: In this series, children with AGS pretransplant have a high prevalence of abdominal arterial anomalies. Preoperative abdominal vascular imaging makes it possible to anticipate whether or not a classical arterial revascularization can be performed or whether an ACR is required.
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http://dx.doi.org/10.1097/MPG.0000000000001538DOI Listing
June 2017

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

Pediatr Radiol 2016 Nov 26;46(12):1694-1704. Epub 2016 Aug 26.

Department of Pediatric Radiology, Bicêtre Hospital, 78, rue du Général Leclerc, Le Kremlin-Bicêtre, 94275, France.

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.

Materials And Methods: We conducted a retrospective study of 15 children with undifferentiated embryonal sarcoma diagnosed or referred to our hospital during 1997-2015 and analysed the clinical, biological and imaging data.

Results: We identified eight boys and seven girls ages 9 months to 14 years. Ten children presented with abdominal pain. Alpha-fetoprotein was slightly increased in one. Initial US and CT had been performed for all, while additional MRI had been done in two children. Initial CT demonstrated a hypoattenuated mass in all. Rupture was seen in five and intratumoural bleeding in seven children. Tumour volumes reduced during neoadjuvant chemotherapy in 10 children.

Conclusion: Undifferentiated embryonal sarcoma might be suggested in a non-secreting unifocal tumour with well-defined borders, fluid-filled spaces on US, hypoattenuation and serpiginous vessels on CT, and if there are signs of internal bleeding or rupture on CT or MRI.
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http://dx.doi.org/10.1007/s00247-016-3670-3DOI Listing
November 2016

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

Eur Radiol 2017 May 23;27(5):1812-1821. Epub 2016 Aug 23.

Pediatric Radiology, Bicêtre Hospital, 94270, Le Kremlin Bicêtre, Paris, France.

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. US evaluated gallbladder type (1: normal; 2: consistent with BA; 3: suspicious), triangular cord sign (TCS), microcyst and macrocyst, polysplenia syndrome, portal hypertension, and bile duct dilatation. T test and Pearson χ test were used to compare US signs between the two groups, followed by univariate regression analysis.

Results: The highest specificity and sensitivity for BA (p < 0.001) were respectively obtained with non-visible gallbladder (100 %-13 %), macrocyst (99 %-10 %), polysplenia (99 %-11 %), microcyst (98 %-20 %), type 2 gallbladder (98 %-34 %), and TCS (97 %-30 %). Combination of signs (macro or microcyst; cyst and no bile duct dilatation; microcyst and/or TCS; type 2 gallbladder and/or cyst) provided better sensitivities (25-49 %) with similar specificities (95-98 %) (p < 0.001). On univariate analysis, the single US signs most strongly associated with BA were polysplenia (odds ratio, OR 16.3), macrocyst (OR 14.7), TCS (OR 13.4) and microcyst (OR 8).

Conclusions: Porta hepatis microcyst is a reliable US sign for BA diagnosis.

Key Points: • The porta hepatis microcyst is a specific sign of biliary atresia. • It was found in 31 (16.1 %) of 193 patients with biliary atresia. • Its specificity was 98 % (p < 0.001). • High frequency transducer and color Doppler can show the porta hepatis microcyst.
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http://dx.doi.org/10.1007/s00330-016-4546-5DOI Listing
May 2017

DCDC2 Mutations Cause Neonatal Sclerosing Cholangitis.

Hum Mutat 2016 10 24;37(10):1025-9. Epub 2016 Aug 24.

Paris Descartes Sorbonne Paris Cité University, Imagine institute, Paris, France.

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. We identified biallelic missense mutations or in-frame deletion in DCDC2 in four affected children. Mutations involve highly conserved amino acids in the doublecortin domains of the protein. In cholangiocytes, DCDC2 protein is normally located in the cytoplasm and cilia, whereas in patients the mutated protein is accumulated in the cytoplasm, absent from cilia, and associated with ciliogenesis defect. This is the first report of DCDC2 mutations in NSC. This data expands the molecular spectrum of NSC, that can be considered as a ciliopathy and also expands the clinical spectrum of the DCDC2 mutations, previously reported in dyslexia, deafness, and nephronophtisis.
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http://dx.doi.org/10.1002/humu.23031DOI Listing
October 2016

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.

Radiology 2016 Feb 21;278(2):554-62. Epub 2015 Aug 21.

From the Departments of Pediatric Radiology (S.F.A., L.C., D.P.), Pediatric Hepatology (E.G.), and Epidemiology (G.A., B.D.), Hôpital Bicêtre, Hôpitaux Universitaires Paris Sud, Assistance Publique Hôpitaux de Paris, Université Paris Sud, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France; Departments of Pathology (M.F.) and Adult Radiology (J.M.C.), Hôpital Necker, Assistance Publique Hôpitaux de Paris, Université Paris V, Assistance Publique Hôpitaux de Paris, Paris, France; and Institut Langevin, ESPCI ParisTech, CNRS UMR7587, INSERM U979, Paris, France (J.L.G., M.T.).

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. First, 51 healthy children (from neonate to 15 years) were analyzed as the control group, and univariate and multivariate comparisons were performed to study the effect of age, transducer, breathing condition, probe, and position on elasticity values. Next, 45 children (from 1 month to 17.2 years old) who underwent liver biopsy were analyzed. SSWE measurements were obtained in the same region of the liver as the biopsy specimens. Biopsy specimens were reviewed in a blinded manner by a pathologist with the use of METAVIR criteria. The areas under the receiver operating characteristics curve (AUCs) were calculated for patients with fibrosis stage F0 versus those with stage F1-F2, F2 or higher, F3 or higher, and F4 or higher.

Results: A successful rate of SSWE measurement was 100% in 96 patients, including neonates. Liver stiffness values were significantly higher when an SC6-1 probe (Aixplorer; SuperSonic Imagine SA, Aix-enProvence, France) was used than when an SL15-4 probe (Aixplorer) was used (mean ± standard deviation, 6.94 kPa ± 1.42 vs 5.96 kPa ± 1.31; P = .006). There was no influence of sex, the location of measurement, or respiratory status on liver elasticity values (P = .41-.93), although the power to detect such a difference was low. According to the degree of liver fibrosis at liver biopsy, 88.5%-96.8% of patients were correctly classified, with AUCs of 0.90-0.98 (95% confidence interval [CI]: 0.8, 1.0). The AUC for patients with stage F0 versus stage F1-F2 was 0.93 (95% CI: 0.87, 0.99).

Conclusion: SSWE allows accurate assessment of liver fibrosis, even in children with early stage (F1-F2) disease, and the choice of transducer influences liver stiffness values.
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http://dx.doi.org/10.1148/radiol.2015142815DOI Listing
February 2016

Primary Hepatic Ewing Sarcoma With EWS-FLI1 RNA Transcript in a Child.

J Pediatr Hematol Oncol 2015 Jul;37(5):411-3

*Pediatric and Adolescent Oncology Department, Nantes University Hospital ∥Physiopathology of Bone Resorption and Therapy of Primitive Bone Tumors Laboratory, Nantes University UMR 957 ¶Pathology Department, Centre Hospitalier Universitaire, Nantes Departments of †Pediatric Surgery ‡Pediatric Pathology §Pediatric Radiology, Bicêtre Hospital Le Kremlin-Bicêtre, France.

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http://dx.doi.org/10.1097/MPH.0000000000000289DOI Listing
July 2015

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

Eur J Pediatr Surg 2015 Feb 26;25(1):132-7. Epub 2014 Sep 26.

Department of Pediatric Surgery, Bicêtre Hospital, Le Kremlin-Bicêtre, France.

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. We looked at the diagnosis and management pitfalls, surgical technique, and outcomes. Results are expressed as median (range).

Results: There were 13 patients (M/F=7:6=1.1). Age at presentation was 8 years (range, 11 months-16 years). Of the 13 patients, 10 patients (69%) presented with abdominal pain, 5 (38%) with tumoral bleeding, and 2 (15%) with peritoneal rupture. All lesions were unique, nonmetastatic, and heterogeneous with cystic components measuring 14 (6-19) cm. Six (46%) patients had an upfront surgery: five because of wrong clinical diagnosis (three query mesenchymal hamartoma, one spontaneous peritoneal bleeding, and one cystic lymphangioma), and one because of rapid enlargement of the mass. Seven (54%) patients (including one with tumoral bleeding) received neoadjuvant chemotherapy, and had their tumor diameter decreasing by 40% (range, 0-60%). Final surgery consisted of seven right hepatectomies; one right extended hepatectomy; three mesohepatectomies; two left hepatectomies. There were three incomplete resection in the upfront surgery group versus none in the neoadjuvant chemotherapy group. The degree of tumor necrosis after chemotherapy ranged from 95 to 100%. Surgical complications included the following: liver transplantation (LT) following a Budd-Chiari syndrome after a mesohepatectomy, one biliary ducts injury treated by Roux-en-Y loop. All patients received the postoperative chemotherapy according to the European protocol. One of seven patients (14%) with neoadjuvant chemotherapy underwent radiotherapy for rupture at diagnosis versus three of six patients (50%) with upfront surgery: one for rupture at diagnosis and two for rupture during upfront surgery. One patient (17%) with upfront surgery had local recurrence at 2 years after initial surgery, and is in second complete remission 1 year after a redo surgery. All patients are alive at a median 34 months (range, 5-134) follow-up.

Conclusion: USL presents with painful mixed cystic and solid liver mass. If misdiagnosed and mistreated (enucleation or unroofing), the usual good outcome of this malignancy could be impaired. Preoperative chemotherapy is recommended.
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http://dx.doi.org/10.1055/s-0034-1387937DOI Listing
February 2015

Treatment of Erdheim-Chester disease with canakinumab.

Rheumatology (Oxford) 2014 Dec 17;53(12):2312-4. Epub 2014 Sep 17.

Department of Pediatrics, Nîmes University Hospital, Nîmes, INSERM U 844, University of Montpelier 1, Montpellier, Department of Pediatric Radiology, Department of Pediatrics, Assistance Publique Hôpitaux de Paris, Bicêtre University Hospital, Le Kremlin Bicêtre, University of Poitiers, Laboratoire Inflammation, Tissus Epithéliaux et Cytokines (LITEC) Poitiers, Laboratory of Genetics, Rare and Autoinflammatory Diseases, Department of Genetics, LBM - Montpellier University Hospital, INSERM U844, Montpellier and Department of General Pediatrics and Internal Medicine, Assistance Publique Hôpitaux de Paris, Robert Debré University Hospital, University of Paris, Paris, France.

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http://dx.doi.org/10.1093/rheumatology/keu344DOI Listing
December 2014

Obliterative portal venopathy: a study of 48 children.

J Pediatr 2014 Jul 25;165(1):190-193.e2. Epub 2014 Apr 25.

Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. Electronic address:

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. Portal hypertension is the main complication but is absent in some cases. Prognosis is relatively good, but the detection of cardiopulmonary complications is essential.
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http://dx.doi.org/10.1016/j.jpeds.2014.03.025DOI Listing
July 2014

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

Ann Surg 2014 Jul;260(1):188-98

*Department of Pediatric Surgery, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France †Department of Pediatric Surgery, Univ Paris Descartes, Sorbonne Paris Cité, Hôpital Necker Enfants Malades, APHP, Paris, France ‡Department of Pediatric Radiology, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France §Department of Pediatric Hepatology, Univ Paris Sud, Hôpitaux Universitaires Paris Sud, APHP, Le Kremlin Bicêtre France ¶Department of Hepato-biliary Surgery and Liver Transplantation, Univ Pierre et Marie Curie, Hôpital Saint Antoine, APHP, Paris, France.

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.

Methods: Twenty-three patients who underwent at least 1 surgical procedure to close the CPSs were included in this retrospective study (1997-2012). We designed a classification according to the ending of the shunt in the caval system. We analyzed the results and outcomes of surgery according to this classification.

Results: Two patients had an extrahepatic portosystemic shunt, 17 had a portacaval shunt [subdivided in 5 end-to-side-like portal-caval, 7 side-to-side-like portal-caval, and 5 H-shaped (H-type portal-caval)], 2 had portal-to-hepatic vein shunts (portohepatic), and 2 had a persistent ductus venosus. All extrahepatic portosystemic shunts, H-type portal-caval, portohepatic, and patent ductus venosus patients had a successful 1-stage ligation. All 5 end-to-side-like portal-caval patients had a threadlike intrahepatic portal venous system; a 2-stage complete closure was successfully achieved for 4 and a partial closure for 1. The first 2 side-to-side-like portal-caval patients had a successful 2-stage closure whereas the 5 others had a 1-stage longitudinal caval partition. All patients are alive and none needed a liver transplantation.

Conclusions: Our classification correlates the anatomy of CPSs and the surgical strategy: outcomes are good provided end-to-side-like portal-caval shunts patients have a 2-stage closure, side-to-side portal-caval shunts patients have a 1-stage caval partition, and the others have a 1-stage ligation.
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http://dx.doi.org/10.1097/SLA.0000000000000266DOI Listing
July 2014

Hepatobiliary intervention in children.

Cardiovasc Intervent Radiol 2014 Feb 2;37(1):37-54. Epub 2013 Aug 2.

Le Centre Hospitalier Universitaire du Kremlin-Bicêtre, 78 rue du Général Leclerc, 94270, Le Kremlin-Bicêtre, France.

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.
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http://dx.doi.org/10.1007/s00270-013-0712-1DOI Listing
February 2014

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

Lancet Oncol 2013 Aug 4;14(9):834-42. Epub 2013 Jul 4.

Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Amsterdam, Netherlands.

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. Treatment consisted of preoperative chemotherapy (cycles A1-A3: cisplatin 80 mg/m(2) per day intravenous in 24 h on day 1; cisplatin 70 mg/m(2) per day intravenous in 24 h on days 8, 15, 29, 36, 43, 57, and 64; and doxorubicin 30 mg/m(2) per day intravenous in 24 h on days 8, 9, 36, 37, 57, and 58) followed by surgical removal of all remaining tumour lesions if feasible (including liver transplantation and metastasectomy, if needed). Patients whose tumour remained unresectable received additional preoperative chemotherapy (cycle B: doxorubicin 25 mg/m(2) per day in 24 h on days 1-3 and 22-24, and carboplatin area under the curve [AUC] 10·6 mg/mL per min per day intravenous in 1 h on days 1 and 22) before surgery was attempted. After surgery, postoperative chemotherapy was given (cycle C: doxorubicin 20 mg/m(2) per day in 24 h on days 1, 2, 22, 23, 43, and 44, and carboplatin AUC 6·6 mg/mL per min per day in 1 h on days 1, 22, and 43) to patients who did not receive cycle B. The primary endpoint was the proportion of patients with complete remission at the end of treatment. Analysis was by intention to treat. This trial is registered at ClinicalTrials.gov, NCT00077389.

Findings: We report the final analysis of the trial. 62 eligible patients (39 with lung metastases) were included and analysed. 60 (98%, 95% CI 91-100) of 61 evaluable patients (one child underwent primary hepatectomy) had a partial response to preoperative chemotherapy. Complete resection of all tumour lesions was achieved in 46 patients (74%). At the end of therapy, 49 (79%, 95% CI 67-88) of 62 patients were in complete remission. With a median follow-up of 52 months, 3-year event-free survival was 76% (95% CI 65-87) and 3-year overall survival was 83% (73-93). 60 (97%) patients had grade 3-4 haematological toxicity (anaemia, neutropenia, or thrombocytopenia) and 44 (71%) had at least one episode of febrile neutropenia. Other main grade 3 or 4 toxicities were documented infections (17 patients, 27%), anorexia (22, 35%), and mucositis (seven, 11%). One child died of fungal infection in neutropenia. Moderate-to-severe ototoxicity was documented in 31 (50%) patients. 18 serious adverse events (including two deaths) reflecting the observed side-effects were reported in the trial (the most common was ototoxicity in five patients).

Interpretation: The SIOPEL-4 treatment regimen is feasible and efficacious for complete remission at the end of treatment for patients with high-risk hepatoblastoma.

Funding: Cancer Research UK and Cancer Research Switzerland/Oncosuisse.
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http://dx.doi.org/10.1016/S1470-2045(13)70272-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3730732PMC
August 2013

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).

Eur J Cancer 2012 Dec 24;48(18):3456-64. Epub 2012 Jul 24.

Academic Medical Center, Amsterdam, The Netherlands.

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.) infusion on days 1-5 and 8-12, every 21days) unless tumour progression occurred or resectability was achieved earlier. Tumour response was assessed according to modified SIOPEL and Response Evaluation Criteria In Solid Tumours (RECIST) criteria. Main end-points were best overall response rate (RR), early progression rate (EPR) and progression free survival (PFS).

Results: Twenty-four eligible patients (median age 58.0months; 19 boys) were enrolled in the study (11 relapses, 13 refractory diseases). Of the 23 evaluable patients six had an overall partial response, 11 stable disease and six progressive disease, of which four were early progression (RR: 26%, EPR: 17%). In eight patients the residual tumour could be completely resected; seven patients became tumour free. At last follow-up 12 patients were alive (six with no evidence of disease, six with disease). PFS at 1year was 24%. Patients with relapse had a higher RR than patients with refractory disease (46% versus 8%) and patients with isolated lung lesions showed a better response than patients with other tumour localisations (50% versus 13%). The main grade 3-4 toxicities, diarrhoea and neutropenia, occurred in half of the patients.

Conclusion: Irinotecan has a significant anti-tumour activity and acceptable toxicity in patients with relapsed hepatoblastoma and therefore should be considered for the treatment of these patients. Exploration of the role of irinotecan in the initial treatment of hepatoblastoma is warranted.
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http://dx.doi.org/10.1016/j.ejca.2012.06.023DOI Listing
December 2012

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

Cell Med 2012 Feb 8;4(2):85-98. Epub 2012 May 8.

‡‡ INSERM U1064, CHU Hôtel Dieu, Université de Nantes , Nantes , France.

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. We previously showed that partial portal vein embolization (PVE) improved hepatocyte engraftment in a nonhuman primate model. We developed here an ex vivo approach based on PVE and lentiviral-mediated transduction of hepatocytes from normal (New Zealand White, NZW) and Watanabe heritable hyperlipidemic (WHHL) rabbits: the large animal model of familial hypercholesterolemia type IIa (FH). FH is a life-threatening human inherited autosomal disease caused by a mutation in the low-density lipoprotein receptor (LDLR) gene, which leads to severe hypercholesterolemia and premature coronary heart disease. Rabbit hepatocytes were isolated from the resected left liver lobe, and the portal branches of the median lobes were embolized with Histoacryl® glue under radiologic guidance. NZW and WHHL hepatocytes were each labeled with Hoechst dye or transduced with lentivirus expressing GFP under the control of a liver-specific promoter (mTTR, a modified murine transthyretin promoter) and were then immediately transplanted back into donor animals. In our conditions, 65-70% of the NZW and WHHL hepatocytes were transduced. Liver repopulation after transplantation with the Hoechst-labeled hepatocytes was 3.5 ± 2%. It was 1.4 ± 0.6% after transplantation with either the transduced NZW hepatocytes or the transduced WHHL hepatocytes, which was close to that obtained with Hoechst-labeled cells, given the mean transduction efficacy. Transgene expression persisted for at least 8 weeks posttransplantation. Transduction of WHHL hepatocytes with an LDLR-encoding vector resulted in phenotypic correction in vitro as assessed by internalization of fluorescent LDL ligands. In conclusion, our results have applications for the treatment of inherited metabolic liver diseases, such as FH, by transplantation of lentivirally transduced hepatocytes.
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http://dx.doi.org/10.3727/215517912X647136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4733829PMC
February 2012

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

Pediatr Radiol 2012 Mar 18;42(3):298-307. Epub 2011 Sep 18.

Pediatric Radiology, Hopital Bicêtre, 78 Rue du Général Leclerc, Le Kremlin Bicêtre 94275, France.

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.

Results: Sixteen foetuses had one focal lesion, with a mean volume of 75 ml (5-240 ml). One had multifocal HH. Most presented as a focal well-delimited heterogeneous vascular mass. Four had associated cardiomegaly, five had cardiac failure. Eight of the nine foetuses with cardiac disorders were symptomatic at birth: cardiac failure with pulmonary hypertension (9), consumptive coagulopathy (8), compartmental syndrome (2). All received supportive medical treatment, four embolisation. Five of these died. The remaining eight had a normal cardiac status. Two became symptomatic after birth: one with a large porto-hepatic shunt and one with significant mass effect. Prenatal cardiac abnormality (univariate, P = 0.031), enlargement of more than one hepatic vein (P = 0.0351) and large volume (P = 0.0372) were associated with symptomatic disease.

Conclusion: Hepatic haemangioma associated with prenatal cardiac disorders, large volume and more than one enlarged hepatic vein have poorer outcome and require specific perinatal multidisciplinary management.
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http://dx.doi.org/10.1007/s00247-011-2214-0DOI Listing
March 2012

Initial liver transplantation for unresectable hepatoblastoma after chemotherapy.

Pediatr Blood Cancer 2011 Dec 9;57(7):1270-5. Epub 2011 Sep 9.

Paediatric Surgery APHP-Bicêtre Hospital University of Paris Sud XI, France.

Background: SIOPEL protocols have recommended liver transplantation for unresectable hepatoblastoma (HBL) after chemotherapy in absence of visible extrahepatic disease.

Methods: This retrospective single center study includes 13 children treated following SIOPEL 3 or 4 protocols who underwent orthotopic liver transplantation (OLT) for HBL between February 2001 and May 2009.

Results: Twelve patients had PRETEXT IV HBL, one had PRETEXT II P + HBL, two had pulmonary metastasis at diagnosis. Extra hepatic vascular involvement was present in seven patients (two vena cava, four main portal vein). Twelve patients received a deceased donor organ graft; wait time to OLT was 16 days (1-50 days). One patient received a living donor graft. Four patients did not undergo post-OLT chemotherapy because of major post-OLT surgical complications. Mean follow up was 3.1 years (1-5 years). Ten patients are alive, eight in first complete remission (CR), one is in second CR after two surgical pulmonary metastasis were removed, the latter is in second CR after a surgery for excision of two local recurrences and re-OLT for a secondary HBL in the first graft. Three patients died (two from tumor recurrence, one from cardiac failure after second OLT). Overall survival at 1 and 4 years was 100% and 83.3%.

Conclusions: Application of SIOPEL protocols for treatment of HBL in a specialized multidisciplinary team with access to liver transplantation has resulted in excellent survival. Initial extrahepatic disease should not be considered a contraindication. Future refinements of the protocol need to be considered to reduce toxicity.
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http://dx.doi.org/10.1002/pbc.23301DOI Listing
December 2011

Widening spectrum of liver angiosarcoma in children.

J Pediatr Gastroenterol Nutr 2011 Dec;53(6):615-9

Service d'Hépatologie Pédiatrique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, and Université Paris Sud 11, Paris, France.

Objectives: Liver hemangiomas are vascular tumors, which occur in the first months of life and carry risks of initial complications, but are considered to be benign histologically and to regress with time. Histologic studies suggest that a subtype, type 2 hemangioendothelioma, is akin to angiosarcoma and may have a severe long-term prognosis. We report 5 girls with type 2 hemangioendothelioma of the liver.

Methods And Results: Three children initially presented with classical infantile multinodular hemangioma, including cardiac and pulmonary complications and regression of tumors at age 1½ to 2½ years. All 3 experienced tumor relapse at ages 2½ to 3, leading to death at ages 2½ to 5. Tumor histology showed type 2 hemangioendothelioma. The other 2 children presented with liver tumors at ages 2 and 3 years. In 1, initial biopsy of a single tumor showed benign type 1 hemangioendothelioma, but surgical resection was followed by relapse in the remaining liver, lung metastases, and death. Whole tumor histology showed both type 1 and 2 lesions. In the other child, tumor biopsy showed type 2 lesions. She underwent liver transplantation and is alive without tumor recurrence 3 years later.

Conclusions: Careful follow-up is necessary to detect late recurrence in infants with multinodular liver hemangiomas. Vascular liver tumors occurring after infancy are likely to be malignant. The high risk of relapse in the remaining liver suggests that if no metastases are detected, liver transplantation is preferable to surgical tumor resection in both situations.
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http://dx.doi.org/10.1097/MPG.0b013e318230146cDOI Listing
December 2011

Renal safety in pediatric imaging: randomized, double-blind phase IV clinical trial of iobitridol 300 versus iodixanol 270 in multidetector CT.

Pediatr Radiol 2011 Nov 29;41(11):1393-400. Epub 2011 Jun 29.

Radiology Department, CHU Charles Nicolle, Rouen, France.

Background: It is debated whether iso-osmolar and low-osmolar contrast media are associated with different incidences of contrast medium-induced nephropathy (CIN) in patients with renal insufficiency.

Objective: To compare the incidence of CIN in children undergoing contrast-enhanced multidetector computer tomography (MDCT) with intravenous injection of low-osmolar (iobitridol, Xenetix® 300) or an iso-osmolar (iodixanol, Visipaque® 270) iodinated contrast medium.

Materials And Methods: One hundred forty-six children with normal renal function were included in this multicenter trial and underwent contrast-enhanced MDCT. The primary endpoint was the relative change in creatinine clearance from 48 h pre- to 72 h postcontrast medium administration using a noninferiority analysis in the intent-to-treat (ITT, n = 128) and per protocol (n = 68) populations. Secondary endpoints were incidence of CIN, global image quality, diagnostic efficacy and clinical safety.

Results: In the ITT population, the noninferiority of iobitridol over iodixanol was demonstrated. CIN incidence was 4.8% (three cases) with iobitridol and 10.6% (seven cases) with iodixanol (not significant). No statistically significant differences were observed for the secondary endpoints.

Conclusion: Comparable satisfactory safety profiles were confirmed for both contrast media, with no significant difference in the incidence of CIN in children with normal renal function.
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http://dx.doi.org/10.1007/s00247-011-2164-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3195264PMC
November 2011

Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.

J Pediatr Gastroenterol Nutr 2010 Sep;51(3):322-30

Radiologie Pédiatrique, Hôpital Bicêtre, APHP, Le Kremlin-Bicêtre, France.

Background And Objective: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature.

Patients And Methods: Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods.

Results: Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications.

Conclusions: Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.
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http://dx.doi.org/10.1097/MPG.0b013e3181d9cb92DOI Listing
September 2010

Paediatric chronic liver diseases: how to investigate and follow up? Role of imaging in the diagnosis of fibrosis.

Pediatr Radiol 2010 Jun 30;40(6):906-19. Epub 2010 Apr 30.

Pediatric Radiology Department, Bicêtre Hospital, Assistance Publique Hôpitaux de Paris, University Paris XI, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.

Chronic liver diseases are rare in children, but encompass a wide spectrum of disorders that may all be complicated by liver fibrosis and therefore by portal hypertension. They may be classified according to the level of portal flow obstruction: prehepatic, intrahepatic or suprahepatic. Most of them, except presinusoidal diseases, may progress to cirrhosis that carries additional risks of impaired liver function and development of hepatocellular carcinoma. Imaging plays an important role in guiding the diagnosis and biopsy and for follow-up during treatment. US, with high-frequency transducers and Doppler, is the first modality of choice, directs the rest of the investigations and guides interventional radiology. MDCT has made great progress and has replaced angiography for diagnostic purposes. MRI is indicated for parenchyma and nodule characterization and for biliary tract evaluation. To avoid liver biopsy, several elasticity imaging techniques have been developed and have to be evaluated for accuracy and convenience in children. The role of each modality with main imaging findings is described in extrahepatic portal vein obstruction, hepatoportal sclerosis, congenital hepatic fibrosis, cirrhosis and Budd-Chiari syndrome.
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http://dx.doi.org/10.1007/s00247-010-1600-3DOI Listing
June 2010

Prenatal and postnatal Ciliated Hepatic Foregut Cysts in infants.

J Pediatr Surg 2010 Mar;45(3):E9-14

Department of Pediatric Surgery, Bicêtre Hospital, Assistance Publique Hôpitaux de Paris, University Paris XI, Le Kremlin-Bicêtre, France.

Purpose: Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC.

Methods: We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis.

Results: Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery. Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia.

Conclusion: In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.
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http://dx.doi.org/10.1016/j.jpedsurg.2009.12.009DOI Listing
March 2010