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Liver-transplanted, immunosuppressed pediatric patients undergoing repeated percutaneous transhepatic cholangiography (PTC) require optimized exposure to ionizing radiation. To establish local diagnostic reference levels (DRL) for pediatric PTC and investigate the routine use of X-ray equipment. 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 (PKA), air kerma at patient entrance reference point (Ka,r) 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. 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.cm2, 22 cGy.cm2, 68 cGy.cm2, 107 cGy.cm2 and 179 cGy.cm2 in PKA. 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. PKA correlated significantly with the number of acquisitions and tube-to-table distance. We established local DRLs for children undergoing PTC.

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.

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.

Hepatic venous outflow obstruction after paediatric liver transplantation is an unusual but critical complication. To review the incidence, diagnosis and therapeutic modalities of hepatic venous outflow obstruction from a large national liver transplant unit. 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. 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. 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.

Congenital portosystemic shunts (CPSS) are rare and underdiagnosed abdominal vascular malformations. Their relationship with cardiovascular (CV) disorders are poorly reported. Herein, we describe CV disorders associated with CPSS in a large cohort to propose adequate diagnosis and management. Among 168 children referred for a CPSS (1996–2019), those presenting at least one clinically significant CV disease including congenital heart diseases (CHD), heart failure (HF), pulmonary hypertension (PH) and/or hepatopulmonary syndrome (HPS) were included. CV disorders were detected using echocardiography complemented by right-sided heart catheterization and/or lung perfusion radionuclide when appropriate. 46/168 children with CPSS (27,4%) were included. In 36 patients, endovascular or surgical CPSS closure was required, including 24 for CV indications. CHD was present in 28 patients (16,6%); 7 had heterotaxy (4,1%). HF occurred in 21 neonates, diagnosed prenatally in 7, including 13 with CHD. HF decreased after cardiac surgery in 10 with CHD but also spontaneously before the age of 3 years in 8 except one without CHD. In 11 patients (mean age: 11 ± 5.2 years) CPSS were diagnosed as part of a work up for fixed PH (mean pressures 46 ± 15.1 mmHg, pulmonary vascular resistances 7 ± 2.5UW/m2). One of them died before CPSS closure, while PH remained stable despite CPSS closure in 10 patients, of which 2 then required lung transplantation. In 6 patients (median age: 5.5 years), hypoxia revealed HPS which resolved within 2 years of CPSS closure. CV disorders are frequently associated with CPSS including CHD with/without heterotaxy. HF is found in fetuses and neonates, regressing in most cases. Later, HPS and PH may develop. After CPSS closure, HPS is reversible while PH may only stabilize. Perinatal HF, complex CHD, heterotaxy, unusual course of CHD, HPS and PH should raise the possibility of CPSS. Conversely CV complications should be searched for in patients with CPSS.

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 (p Conclusion IR-guided techniques are promising in the treatment of pediatric solid tumors. Further prospective (randomized) trials are needed to clarify efficacy.

Copyright © 2019 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited, 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.

Liver diseases have been known to induce changes in systemic hemodynamics and to modify the liver-heart-lung balance. Severe or chronic liver disease may result in several heart or pulmonary complications independently of any other existing cardiac or lung disease. The main liver diseases causing

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

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.

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. 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 χ2 test were used to compare US signs between the two groups, followed by univariate regression analysis. The highest specificity and sensitivity for BA (p

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.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.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).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.

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.

Acute hepato-biliary disorders may affect previously healthy children or children with known liver disease or other diseases. The acute presentation is variable usually nonspecific, associating: jaundice, abdominal pain, fever, nausea and vomiting, neurological impairment in case of hepatic encephalopathy or an abdominal mass. Biological tests will provide information about the severity of the disorder, its mechanisms (biliary obstruction versus hepatocyte disorders), the presence of infection, raised tumoral markers, etc.

Liver and biliary disorders may present shortly after birth or even be suspected and diagnosed during fetal life. Emergency management may be required due to life-threatening conditions such as acute liver failure or cardiac failure related to hypervascularized tumors or vascular malformations.

We report the results of a study of survival, liver and kidney functions, and growth with a median follow-up of 24 years following liver transplantation in childhood. From 1988 to 1993, 128 children underwent deceased donor liver transplantation (median age: 2.5 years). Twenty-year patient and graft survival rates were 79% and 64%, respectively. Raised serum aminotransferase and/or γ-glutamyl transferase activities were present in 42% of survivors after a single transplantation. Graft histology (35 patients) showed signs of chronic rejection in 11 and biliary obstruction in 5. Mean total fibrosis scores were 4.5/9 and 3/9 in patients with abnormal and normal serum liver tests, respectively. Glomerular filtration rate was

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.

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.

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

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

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 (20 mg/m(2)/day intravenous (i.v.) infusion on days 1-5 and 8-12, every 21 days) 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 58.0 months; 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 1 year 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. (C) 2012 Elsevier Ltd. All rights reserved

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.

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 hyperc-holesterolemia 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.

The clinical presentation of foetal hepatic haemangioma (HH) is highly variable, from asymptomatic to life-threatening.The aim of this study was to describe foetal hepatic haemangioma and identify prognostic factors.Antenatal and postnatal imaging studies, clinical and biological records of infants with antenatally diagnosed HH (2001-2009) were reviewed.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.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.

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. Pediatr Blood Cancer 2011; 57: 1270–1275. © 2011 Wiley Periodicals, Inc.

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.

Resume Transrectal drainage of pediatric pouch of Douglas abscesses under suprapubic US guidance: technique and results J Radiol 2010;91:221-5 J Potet, S Franchi-Abella, M Al Issa, C Fayard et D Pariente Pelvic abscesses are not always amenable to percutaneous drainage. Transrectal drainage of pouch of Douglas abscesses, performed blindly by surgeons for many years, is not familiar to radiologists. The purpose of this article is to describe our technique for the drainage of pouch of Douglas abscesses under suprapubic US guidance and report our results in a series of 7 pediatric patients with deep pelvic abscess not amenable to percutaneous drainage after failure of antibiotics. The procedure was performed under general anesthesia. All procedures were successful and without complication. The drainage catheter was well tolerated in all cases with short post-drainage hospital stay. After failure of medical management, this technique can be used irrespective of patient age or sex using US transducers and drainage catheters routiney available in any radiology department. Les abces pelviens ne sont pas toujours accessibles au drainage percutane. Le drainage transrectal des abces du cul-de-sac de Douglas, pratique depuis longtemps par les chirurgiens a l’aveugle, est mal connu des radiologues. L’objectif de cet article est de decrire notre technique de drainage par voie transrectale des abces du cul-desac de Douglas sous controle echographique sus-pubien et de rapporter nos resultats sur une serie de 7 enfants, porteurs d’un abces sous tension non accessible a la ponction percutanee et apres echec d’une antibiotherapie adaptee, La procedure est faite sous anesthesie generale. Le drainage a ete un succes d’emblee dans tous les cas, sans complication, avec un drain bien tolere et une duree d’hospitalisation courte au decours Apres echec du traitement medical, cette technique est applicable quel que soit le sexe et l’âge, avec des sondes d’echographie et du materiel de drainage disponibles dans tout service de radiologie.

Background Liver nodules have been reported after portal systemic shunt surgery (PSSS) in animal experiments or in humans with liver cirrhosis. The aim of our study was to assess the incidence of liver nodules after surgery for extrahepatic portal vein obstruction (EHPVO) in children without associated liver disease. Methods We retrospectively reviewed the charts of 45 children who had surgery from 1979 to 2005 for EHPVO in our institution, consisting of 38 PSSS and 7 portal reperfusion procedures (PRPs). We assessed the presence of liver nodules on ultrasonography. Results Of 45 patients, 7 (15%) had liver nodules during a median of 80 months of follow-up. All the nodules occurred after PSSS. Five nodules were subjected to biopsy; we found 2 liver cell adenomas and 3 focal nodular hyperplasias. Conclusions In this study, liver nodules occurred in 18% of cases after PSSS for EHPVO in children and not after PRP. As many children have undergone PSSS throughout the world, the presence of liver nodules should be considered during the follow-up of those patients.

We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results.

Imaging is a crucial part of the evaluation of children with primary liver malignancies. In addition to staging the intrahepatic extent of the tumour, the 2005 revision of the PRETEXT system specifies certain criteria for assessment of extrahepatic disease. This article reviews the imaging findings of two of these criteria: direct extrahepatic extension of the tumour (E1, E1a, E2, E2a) and tumour rupture at diagnosis (H1).

Over the last 15 years, various oncology groups throughout the world have used the PRETEXT system for staging malignant primary liver tumours of childhood. This paper, written by members of the radiology and surgery committees of the International Childhood Liver Tumor Strategy Group (SIOPEL), presents various clarifications and revisions to the original PRETEXT system.

Background Prenatal management of abnormal abdominal situs is challenging since prognosis is highly variable depending on the associated malformations. Methods The authors report on two cases of ambiguous abdominal situs. Prenatal management included specialized ultrasound examination of fetal anatomy and heart, amniocentesis for determination of the fetal karyotype and fetal abdominal MR. Results In both cases, abnormal situs was associated with other abdominal anomalies such as azygous continuation, biliary atresia, bowel obstruction and polysplenia. Fetal MR was a useful additional tool in the assessment of fetal abdominal anatomy in both cases. Conclusions On the basis of these reports, the literature is reviewed and a prenatal management scheme is proposed. Copyright © 2006 John Wiley & Sons, Ltd.

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Resume L'invagination intestinale aigue (IIA) du nourrisson et de l’enfant est definie par la penetration d'un segment intestinal dans un segment sous-jacent. Elle est responsable d’un syndrome occlusif avec une compression veineuse et un œdeme qui peuvent rapidement evoluer vers la necrose intestinale. C'est donc une urgence therapeutique. Il existe deux types d'IIA : l'IIA idiopathique du nourrisson representant 90 a 95 % des invaginations de l'enfant ; l'IIA secondaire a une lesion locale isolee (diverticule de Meckel, polype…) ou s'integrant dans une pathologie plus generale du tube digestif (purpura de Henoch-Schoenlein, mucoviscidose…) ou survenant dans un contexte particulier (IIA postoperatoire). L’echographie abdominale est l’examen cle permettant d’affirmer le diagnostic d’IIA, chez un nourrisson presentant des crises douloureuses abdominales paroxystiques et des vomissements. Le traitement par reduction pneumatique ou hydrostatique, propose en l’absence de contre-indication, est efficace dans 80 a 90 % des cas. Le traitement chirurgical n’est realise qu’en cas d’echec de la reduction non operatoire ou dans certaines circonstances particulieres. Le pronostic de cette affection benigne est excellent, au prix d’une etroite collaboration entre les differentes equipes, pediatres, chirurgiens, radiologues et anesthesistes.

La invaginacion intestinal aguda (IIA) en el lactante y en el nino se define por la penetracion de un segmento intestinal en un segmento subyacente. Provoca un sindrome oclusivo con compresion venosa y edema que pueden evolucionar rapidamente hacia la necrosis intestinal. Por tanto, se trata de una urgencia terapeutica. Existen dos tipos de IIA: IIA idiopatica del lactante, que representa el 90-95% de las invaginaciones en el nino; IIA secundaria a una lesion local aislada (diverticulo de Meckel, polipo, etc.) que o forma parte de una afeccion mas general del aparato digestivo (purpura de Henoch-Schoenlein, mucoviscidosis, etc.) o aparece en un contexto concreto (IIA postoperatoria). La ecografia abdominal es la exploracion clave para confirmar el diagnostico de IIA en un lactante que presenta crisis dolorosas abdominales paroxisticas y vomitos. El tratamiento por reduccion neumatica o hidrostatica, que se propone cuando no existen contraindicaciones, resulta eficaz en el 80-90% de los casos. Solo se recurre al tratamiento quirurgico cuando la reduccion no operatoria fracasa, o en determinadas circunstancias. El pronostico de esta afeccion benigna es excelente, siempre que exista una estrecha colaboracion entre los distintos equipos de pediatras, cirujanos, radiologos y anestesistas.

Background & Aims: Germline mutations in hepatocyte nuclear factor 1α (TCF1/HNF-1α) are associated with maturity-onset diabetes of the young type 3 (MODY3), and somatic biallelic inactivations of the gene are found in hepatocellular adenomas and liver adenomatosis. This study investigated cosegregation of HNF-1α germline mutations with diabetes and liver adenomatosis in 2 families. Methods: Two unrelated patients with liver adenomatosis and harboring HNF-1α germline and somatic mutations were studied. Subsequently, we screened 9 relatives in the 2 independent families for diabetes, hepatocellular adenomas, and HNF-1α germline mutations. Results: In family A, a father and his son presented with an intraperitoneal hemorrhagic rupture of a liver adenomatosis without diabetes. A heterozygous R229X germline mutation was identified in HNF-1α in the father and his son and also in his second 27-year-old son without hepatocellular adenomas. In family B, a diagnosis of liver adenomatosis was made fortuitously in a 14-year-old girl. A heterozygous G55fsX57 germline mutation in HNF-1α was identified in this patient, her diabetic father, and her 2 sisters. Systematic exploration showed liver adenomatosis in the 2 sisters. Somatic inactivation of the second HNF-1α allele was found in liver tumors in both families. Conclusions: This study describes familial liver adenomatosis and shows the association with germline HNF-1α mutations in adults and children. It also highlights the importance of screening for hepatocellular adenomas, diabetes, and HNF-1α germline mutations in relatives of patients with liver adenomatosis. Finally, prevalence of liver adenomatosis remains to be evaluated in MODY3 subjects.

The aim of this report is to describe a rare complication of clostridium difficile (CD) disease, the occurrence of an inflammatory pseudotumor that caused intestinal obstruction in a liver transplant recipient. A 9-month-old girl underwent liver transplantation for biliary atresia. She was given tacrolimus as primary immunosuppressive therapy. Three months after liver transplantation, she presented with febrile protracted bloody diarrhea and failure to thrive. A diagnosis of post-transplant lymphoproliferative disease associated with Epstein-Barr virus infection was initially made on histological examination of duodenal biopsies. Tacrolimus was discontinued. Despite treatment with anti-CD20 monoclonal antibodies the child's condition deteriorated and she presented with intestinal occlusion. A mass at the ascending colon was seen on the computed tomography scan mimicking lymphoma and the child underwent surgical laparotomy. Histological examination of the mass showed typical pathological lesions of inflammatory pseudotumor and CD pseudomembranous colitis. Diagnosis of CD disease was confirmed upon the identification of CD toxin A in stools. She was successfully treated by metronidazole and gamma-globulin perfusions. Delayed diagnosis and anti-CD20 monoclonal antibodies therapy (associated with hypogammaglobulinemia) possibly played a major role in the severity of CD pseudomembranous colitis and in the occurrence of an inflammatory pseudotumor.

Resume Objectifs. – Preciser les differentes causes des syndromes inflammatoires febriles de l’enfant, et analyser la place des differents examens complementaires a realiser. Patients et methodes. – Nous avons mene une etude retrospective des hospitalisations, entre 1990 et 2000, pour syndrome inflammatoire febrile defini par une temperature superieure a 38 °C associee a une augmentation de la vitesse de sedimentation (VS) superieure a 20 mm/h et/ou de la proteine C reactive (CRP) superieure a 20 mg/L et ne relevant pas d’une cause infectieuse simple. Resultats. – Les dossiers de 62 enfants âges de deux mois a 15 ans (âge median de quatre ans) ont ete analyses. Les differents diagnostics se repartissaient en maladie inflammatoire systemique, hemopathie maligne et maladie sans diagnostic precis dans respectivement 79 % (49 enfants), 3,2 % (deux enfants) et 17,8 % (11 enfants) des cas. La maladie de Kawasaki etait la cause preponderante (22 enfants), surtout avant l’âge de deux ans. Une VS superieure a 100 mm/h et une CRP superieure a 100 mg/L ont ete observees dans 59 % des maladies de Kawasaki, 71 % des arthrites juveniles idiopathiques non compliquees de syndrome d’activation lympho-histiocytaire (SAL), 100 % des hemopathies malignes, et 7 % des diagnostics imprecis. Une VS inferieure a 50 mm/h et une CRP inferieure a 50 mg/L ont ete observees dans 75 % des SAL et 46 % des diagnostics imprecis. Le taux de polynucleaires neutrophiles, le bilan hepatique, la triglyceridemie, de la ferritine et de sa fraction glycosylee, l’echographie et le scanner abdominopelviens, l’echographie cardiaque, les analyses histologiques de lesions cliniques ou radiologiques ont ete egalement informatifs. En revanche, les examens scintigraphiques se sont averes peu utiles, en l’absence d’orientation clinique. Conclusion. – Le diagnostic de maladie de Kawasaki doit etre facilement evoque devant un syndrome febrile non infectieux chez le nourrisson. Les valeurs de la VS, de la CRP, du nombre de polynucleaires neutrophiles, des transaminases, des γGT et des triglycerides, les echographies abdominale et cardiaque permettent souvent d’orienter le diagnostic.

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.

Peliosis hepatis, a condition characterized by the presence of blood-filled lacunar spaces in the liver, usually has a chronic presentation pattern and is mainly reported in adult patients in association with chronic wasting disorders and after administration of various drugs. The present report concerns two previously healthy young children in whom peliosis hepatis initially presented as acute hepatic failure and who had Escherichia coli pyelonephritis. Both patients had active intraperitoneal hemorrhage from the peliotic liver lesions, and liver ultrasonography showed multiple hypoechoic areas of different sizes, which in this context should suggest the diagnosis. One child died from hypovolemic shock and the other recovered. This study indicates that acute peliosis hepatis can be a serious life-threatening disease in children.

The objective of this report is to review portal complications (PC) after pediatric liver transplantation (LT) for biliary atresia (BA) in the Bicêtre surgical series. From January 1, 1988, to February 28, 1995, 96 children with BA underwent 115 LTs Portal anastomosis was done on either the recipient portal vein (n = 85) or superior mesenteric vein (n = 11). No antiaggregative agents were administered postoperatively. Median follow-up was 50 months (range, 12 to 97). Nineteen PC (16.5%) occurred in 17 recipients: 16 portal thrombosis (PT) and 3 portal stenosis (PS). Fifteen instances of early PT occurred between days 0 and 17 (median, day 2). Emergency thrombectomy was performed in 9 cases (successful in 5). Three children underwent a secondary portosystemic shunt (successful in 2). Three PS were cured by either surgery or balloon dilatation. Four children died, 3 are alive with portal hypertension (PHT), and 10 are alive without PHT. Three-year patient actuarial survival is 82.4% in PC cases and 82% in others (NS). Significant risk factors of PC are young age and weight at the time of LT, small portal vein, and emergency LT. Analysis of our own results and review of the literature suggest that prevention of PC depends primarily on appropriate surgical technique. Reduction of postoperative hypercoagulability may also play an important role: a meta-analysis of 1,257 published pediatric LT show an overall risk of PT of 2.2% in teams using aspirin with or without dipyridamole compared with 7.8% when no antiaggregative agents are given (P = .0001).

Eleven patients presenting with spontaneous perforation of the biliary tract were treated at Bicêtre Hospital between 1971 and 1993. Three groups were individualised, each with a different pattern of local presentation: generalised biliary peritonitis (n = 2), localised biliary peritonitis (n = 4), secondary biliary stenosis (n = 5). In each case, cholestatic jaundice developed after a postnatal symptom-free interval. Ten patients were operated on. Perforation was located in the cystic duct (n = 2), at the junction of the cystic and hepatic ducts (n = 4), in the common hepatic duct (n = 1) or common bile duct (n = 1). The site of perforation was no longer identifiable in two cases with stenosis. A cholecystectomy was performed in the 2 cases with cystic duct perforation; in the cases of lesions of the main duct, either simple external biliary drainage (n = 3) or biliary reconstruction (n = 5) was carried out. Postoperative complications included bile leak (n = 2), ascending cholangitis (n = 1), portal vein thrombosis (n = 2). Five patients were submitted to further surgery including biliary revision (n = 3), porto-systemic shunt (n = 1), and other procedures (n = 2). One infant died from postoperative sepsis; 2 were lost to follow-up, one of which probably did not survive; 4 are alive and well. Late sequelae are present in 4 children: portal hypertension (n = 1), mild residual bile duct dilatation without cholestasis (n = 1), and mild to moderate liver fibrosis (n = 2). Prompt diagnosis and appropriate treatment should improve the prognosis of this rare condition.

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.

Pancreatic haemangiomas in infants are rare. Seven cases have been reported in the literature to date. We report two new cases. One of our cases had antenatal diagnosis and was associated with atypical portal vein thrombosis. The diagnosis was obtained by percutaneous biopsy without complication. For both cases, regression was observed without treatment.

Hepatic artery thrombosis (HAT), one of the most severe complications of pediatric orthotopic liver transplantation (OLT), often compromises graft and/or child survival. Of 590 OLT performed in 516 children over a 20-year period, 45 were complicated by early HAT, during the first 2 weeks after transplantation. Systematic Doppler ultrasonographic detection of HAT allowed successful surgical revascularization in 19 instances, resulting in a 20-year graft survival rate of 77% versus 24% of cases when revascularization was not attempted or failed. A combination of surgical emergency revascularization, biliary interventional radiology, biliary surgery and/or retransplantation resulted in an 80% 20-year patient survival rate, identical to that of transplanted children who did not experience early HAT. The majority of long-term survivors with their initial graft had normal liver tests, no biliary dilation on ultrasonography and minimal or moderate fibrosis on liver histology. A failed attempt at revascularization did not significantly alter patient survival. Despite these encouraging results, for the children and their parents to overcome the entire process in terms of reoperations, repeated radiological interventions, number of hospitalizations and emotional stress, remains an ordeal of such magnitude that it justifies renewed efforts to progress in the prevention of this complication.

Diaphragmatic paralysis, a difficult diagnosis in the pediatric age group, has classically been made by fluoroscopy or B-mode ultrasound. We report our experience with TM-mode exploration. Twenty-seven patients suspected to have diaphragmatic paralysis were examined by means of inspiratory and expiratory chest radiography, fluoroscopy and B-mode ultrasound. The diaphragmatic echo was recorded on TM-tracing during spontaneous breathing using coronal oblique scans. Direction, excursion and the pattern of the transition between inspiration and expiration were analysed. In 7 patients examination was normal and TM mode demonstrated movement of normal direction and excursion with a sharp aspect of the transition zone. Diaphragmatic paralysis was present in 11 patients: unilateral in 9 and bilateral in 2 cases. TM mode demonstrated paradoxical movement, reduced excursion and a smooth transition zone. In 9 patients with diaphragmatic dysfunction TM mode demonstrated movement in the normal direction but with reduced excursion and a smooth transition zone. Compared to other imaging modalities, TM-mode records diaphragmatic movements more objectively. It can identify direction of the movement even if they are fast and of weak amplitude and in the case of bilateral paralysis. TM can differentiate paralysis from dysfunction. Moreover, this low-cost, non-irradiating made of imaging can be performed at the bedside and is available on all basic devices.