Publications by authors named "de Ville de Goyet Jean"

87 Publications

Inferior vena cava surgical cannulation for infants needing veno-venous extracorporeal membrane oxygenation.

Perfusion 2021 Jan 7:267659120987089. Epub 2021 Jan 7.

Department of Anesthesia and Intensive Care, IRCCS-ISMETT, Palermo, Italy.

Introduction: Femoral cannulation for veno-venous extracorporeal membrane oxygenation is challenging in infants because of the diameter of the vein.

Case Report: Prolonged ECMO support (67 days) was necessary for an 8-month-old (8 kg) girl with acute respiratory distress syndrome that was caused by H1N1 influenza. After 30 days on ECMO support and using a single 16 Fr double-lumen cannula (internal jugular vein), a second cannula was necessary to ensure adequate flow. This second 12 Fr single-lumen cannula was surgically placed through the right common iliac vein. An excellent flow profile was then achieved and ECMO continued successfully for 37 more days.

Discussion: As a lifesaving option, this double caval configuration successfully optimized the flow profile and oxygenation, outweighing the related risks.

Conclusion: In small children, a surgical approach to the inferior vena cava can be considered safe, especially in those cases where there is a shortage of adequate cannulas, or when central venous access is difficult.
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http://dx.doi.org/10.1177/0267659120987089DOI Listing
January 2021

Domino-liver transplantation: toward a safer and simpler technique in both donor and recipient.

Updates Surg 2021 Feb 23;73(1):223-232. Epub 2020 Sep 23.

University Pittsburgh Medical Center (UPMC-Italy) at the ISMETT (Istituto Mediterraneo for Trapianto e Terapie ad Alta Specializzazione), IRCCS, Palermo, Italy.

Domino-liver transplantation represents a rare chance to expand the donor liver pool. Fear of putting both donor and recipient at disadvantage has meant that the procedure has not been applied universally. A modification of the original technique which allows both safe procurement of the graft as well as safe implantation of the reconstructed graft in the domino-graft recipient using a 180° rotated, adequately trimmed, free iliaco-caval venous graft is described in detail.
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http://dx.doi.org/10.1007/s13304-020-00886-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7889565PMC
February 2021

Optimizing Liver Division Technique for Procuring Left Lateral Segment Grafts: New Anatomical Insights.

Liver Transpl 2021 02 29;27(2):281-285. Epub 2021 Jan 29.

Department of Pediatrics, Istituto di Ricovero e Cura a Carattere Scientifico-Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione, Palermo, Italy.

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http://dx.doi.org/10.1002/lt.25895DOI Listing
February 2021

Unusual Clinical Course for Untreated Malformative Biliary Atresia Infant: Is Portal Hypertension an Important Driver of Liver Fibrosis?

J Pediatr Gastroenterol Nutr 2021 02;72(2):216-219

Department of Translational Medical Science, Section of Paediatrics, University of Naples Federico II, Naples.

Abstract: In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years. Remarkably, she remained in satisfactory condition in absence of ascites or severe hepatic dysfunction, when 4 other similar patients-managed during the same period of time-all had the usual clinical deterioration and ascites, with the need for liver replacement. To our knowledge, there is no similar report in literature. Possible pathogenetic mechanisms and the role of portal hypertension as important factors are discussed.
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http://dx.doi.org/10.1097/MPG.0000000000002932DOI Listing
February 2021

Liver Transplantation for Pediatric Liver Cancer.

Cancers (Basel) 2020 Mar 19;12(3). Epub 2020 Mar 19.

Department of Pathology, Children's Hospital Medical Center of Cincinnati, Cincinnati, OH 45229, USA.

Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability. In the process, tumor histology, imaging protocols, and tumor staging have also matured to better assess response and LT candidacy. Significant trends include a steady increase in the incidence of and use of LT for hepatoblastoma, and a significant improvement in survival after LT for HCC with each decade. Although LT is curative for most unresectable primary liver sarcomas, such as embryonal sarcoma, the malignant rhabdoid tumor appears relapse-prone despite chemotherapy and LT. Pediatric liver tumors remain rare, and diagnostic uncertainty in some settings can potentially delay treatment or lead to the selection of less effective chemotherapy. We review the current knowledge relevant to diagnosis, LT candidacy, and post-transplant outcomes for these tumors, emphasizing recent observations made from large registries or larger series.
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http://dx.doi.org/10.3390/cancers12030720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7140094PMC
March 2020

A Right and a Left … With a Simple Needle….

Gastroenterology 2020 09 4;159(3):e3-e6. Epub 2020 Mar 4.

IRCCS ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Department of Diagnostic and Therapeutic Services, Radiology Unit, Palermo, Italy.

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http://dx.doi.org/10.1053/j.gastro.2020.02.053DOI Listing
September 2020

Novel Arterial Reconstruction With Donor Femoral Artery in Split-Liver Transplantation.

Liver Transpl 2020 05 9;26(5):729-730. Epub 2020 Apr 9.

Abdominal Surgery and Organ Transplantation Unit, Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, University of Pittsburgh Medical Center Italy, Palermo, Italy.

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http://dx.doi.org/10.1002/lt.25736DOI Listing
May 2020

Case report: horse or zebra, ascites or pseudo-ascites? Care for pictural details!

BMC Pediatr 2019 11 25;19(1):460. Epub 2019 Nov 25.

Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Via Sergio Pansini n. 5, 80131, Naples, Italy.

Background: Pseudo-ascites is a very rare condition in children and remains a challenging diagnosis. Targeted imaging may be helpful, but a high index of clinical suspicion is often necessary to guide the investigations, as pseudo-ascites may efficiently mimic true ascites. To date, still many cases of pseudo-ascites suffer diagnostic and therapeutic delay, and some are only diagnosed during surgical exploration. We report the case of a patient with a late laparoscopic diagnosis of pseudo-ascites. We retrospectively review our patient's imaging findings and suggest new characteristic features which may help differentiate pseudo-ascites from true ascites.

Case Presentation: A 7-month-old infant was referred for a progressive abdominal distention. Physical examination and initial ultra-sonographic findings evoked free ascites. An extensive diagnostic workup was then performed and was negative for hepatic, renal, cardiac, intestinal, pancreatic, inflammatory or infectious diseases, malignancy and congenital metabolic disorders. Pseudo-ascites was evoked and dedicated ultra-sonographic and magnetic resonance studies were repeated but could not confirm this diagnosis. Symptomatic diuretic treatment with spironolactone and furosemide was then started. A temporary and limited effect was noted but, with time, repeated paracenteses were necessary as the abdominal distention progressed causing discomfort and breathing difficulty. Last, because the patient's quality of life deteriorated, a peritoneal-venous shunting was proposed; as the operation started with a diagnostic laparoscopy, a benign giant cystic mesenteric lymphangioma was identified and totally excised. The resolution of symptoms was immediate and the patient remained symptom-free throughout the subsequent observation period that lasted more than 1 year.

Conclusions: Increased awareness about pseudo-ascites is necessary, as the diagnosis is often overlooked, and treatment delayed. Targeted imaging may be helpful, as some specific, although not pathognomonic, features exist which may aid in the diagnosis.
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http://dx.doi.org/10.1186/s12887-019-1826-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6876094PMC
November 2019

Cavoportal Hemitransposition in Liver Transplantation: Toward a More Safe and Efficient Technique.

Liver Transpl 2020 01 22;26(1):92-99. Epub 2019 Nov 22.

University Pittsburgh Medical Center-Italy, Istituto Mediterraneo for Trapianto e Terapie ad Alta Specializzazione, Istituto di Ricovero e Cura a Carattere Scientifico, Palermo, Italy.

Extended splanchnic venous thrombosis represents a challenge for the liver transplantation (LT) surgeon. In the absence of large venous tributaries, the cavoportal hemitransposition (CPHTr) and the combined liver-intestinal or multivisceral transplantation are the only technical solutions. Because of the reported high morbidity and mortality rates due to infrequent use and a lack of standardization, the former technique has been almost abandoned by the transplant community. A newly designed technique of CPHTr is presented that is based on the combination of an inferior vena cava (IVC)-sparing hepatectomy and large laterolateral cavocaval and end-to-side cavoportal anastomoses separated only by a double vascular stapler line. This technique allows the splanchnic blood to be completely diverted toward the allograft and to eliminate low-flow IVC areas, which possibly lead to complications. The modified CPHTr technique proposed here offers a valuable alternative to much more complex and invasive intestinal transplantation procedures.
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http://dx.doi.org/10.1002/lt.25635DOI Listing
January 2020

Preduodenal Portal Vein Reconstruction at Liver Transplantation: The Challenges and a Solution.

Liver Transpl 2019 12 12;25(12):1841-1844. Epub 2019 Oct 12.

Department of Pediatrics, Istituto di Ricovero e Cura a Carattere Scientifico, Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione, Palermo, Italy.

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http://dx.doi.org/10.1002/lt.25636DOI Listing
December 2019

Unusual venous collateral pathways allow for reperfusion of the intrahepatic portal venous system in children with portal vein thrombosis after split liver transplantation: Clinical relevance and management implications.

Pediatr Transplant 2019 09 22;23(6):e13539. Epub 2019 Jul 22.

Department for the Treatment and Study of Pediatric Abdominal Diseases and Abdominal Transplantation, IRCCS ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Palermo, Italy.

PVT is the most frequent vascular complication after LT in small children, and a higher incidence has been observed in those transplanted for biliary atresia or with a LLSG. Thrombosis of the PV causes extrahepatic portal hypertension and is associated with splenomegaly and the development of venous neo-collaterals, including gastro-oesophageal varices and splenorenal shunts. It has also been incidentally suggested in the literature that patients who have had a Roux-en-Y loop for a biliary reconstruction may present with a cavernomatous transformation of the distal portion of the loop. In this study, 13 children with CEPH caused by thrombosis of the PV after LT were analysed. The study evidenced the development of two types of hepatopetal venous networks: (a) a large cavernoma along the Roux loop and around the biliary anastomosis, and (b) a network of neo-collaterals in the gastro-duodeno-pancreatic area that connected to the intrahepatic portal branches directly through the liver capsule. These hepatopetal venous networks between the venous system of the surrounding organs or the omentum and the intrahepatic portal branches can be identified by radiologists. The relevance for the transplanting physician and the transplant surgeon is discussed.
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http://dx.doi.org/10.1111/petr.13539DOI Listing
September 2019

New Nutritional and Therapeutical Strategies of NEC.

Curr Pediatr Rev 2019 ;15(2):92-105

Pediatric Department for the Treatment and Study of abdominal Disease and Abdominal Transplants, ISMETT-UPMC, Palermo, Italy.

Necrotizing enterocolitis (NEC) is an acquired severe disease of the digestive system affecting mostly premature babies, possibly fatal and frequently associated to systemic complications. Because of the severity of this condition and the possible long-term consequences on the child's development, many studies have aimed at preventing the occurrence of the primary events at the level of the bowel wall (ischemia and necrosis followed by sepsis) by modifying or manipulating the diet (breast milk versus formula) and/or the feeding pattern (time for initiation after birth, continuous versus bolus feeding, modulation of intake according clinical events). Feeding have been investigated so far in order to prevent NEC. However, currently well-established and shared clinical nutritional practices are not available in preventing NEC. Nutritional and surgical treatments of NEC are instead well defined. In selected cases surgery is a therapeutic option of NEC, requiring sometimes partial intestinal resection responsible for short bowel syndrome. In this paper we will investigate the available options for treating NEC according to the Walsh and Kliegman classification, focusing on feeding practices in managing short bowel syndrome that can complicate NEC. We will also analyze the proposed ways of preventing NEC.
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http://dx.doi.org/10.2174/1573396315666190313164753DOI Listing
March 2020

A national mandatory-split liver policy: A report from the Italian experience.

Am J Transplant 2019 07 14;19(7):2029-2043. Epub 2019 Mar 14.

Hepatobiliary Surgery and Liver Transplantation Unit, University of Padova, Padova, Italy.

To implement split liver transplantation (SLT) a mandatory-split policy has been adopted in Italy since August 2015: donors aged 18-50 years at standard risk are offered for SLT, resulting in a left-lateral segment (LLS) graft for children and an extended-right graft (ERG) for adults. We aim to analyze the impact of the new mandatory-split policy on liver transplantation (LT)-waiting list and SLT outcomes, compared to old allocation policy. Between August 2015 and December 2016 out of 413 potentially "splittable" donors, 252 (61%) were proposed for SLT, of whom 53 (21%) donors were accepted for SLT whereas 101 (40.1%) were excluded because of donor characteristics and 98 (38.9%) for absence of suitable pediatric recipients. The SLT rate augmented from 6% to 8.4%. Children undergoing SLT increased from 49.3% to 65.8% (P = .009) and the pediatric LT-waiting list time dropped (229 [10-2121] vs 80 [12-2503] days [P = .045]). The pediatric (4.5% vs 2.5% [P = .398]) and adult (9.7% to 5.2% [P < .001]) LT-waiting list mortality reduced; SLT outcomes remained stable. Retransplantation (HR = 2.641, P = .035) and recipient weight >20 kg (HR = 5.113, P = .048) in LLS, and ischemic time >8 hours (HR = 2.475, P = .048) in ERG were identified as predictors of graft failure. A national mandatory-split policy maximizes the SLT donor resources, whose selection criteria can be safely expanded, providing favorable impact on the pediatric LT-waiting list and priority for adult sick LT candidates.
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http://dx.doi.org/10.1111/ajt.15300DOI Listing
July 2019

Microscopically positive resection margin after hepatoblastoma resection: what is the impact on prognosis? A Childhood Liver Tumours Strategy Group (SIOPEL) report.

Eur J Cancer 2019 01 5;106:126-132. Epub 2018 Dec 5.

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

Background: To evaluate the impact of a microscopically positive resection margin (microPRM) on the outcome of hepatoblastoma patients pretreated with chemotherapy.

Methods: Local recurrence and survival rates of 431 children treated in the SIOPEL 2 and 3 trials were analysed comparing 58 patients with microPRM with 371 who had a complete resection (CR) and who were then stratified by risk category. The tumour was standard-risk in 312 patients and high-risk (PRETEXT IV and/or extrahepatic disease and/or α-fetoprotein [AFP]<100 ng/ml) in 117 patients. All received cisplatinum-based neoadjuvant and postoperative chemotherapy as per protocol. Apart from one microPRM patient who went on to transplant, none received any additional local treatment.

Results: With a median follow-up of 67 months, local relapse occurred in 3/58 patients with microPRM (5%) and in 23/371 (6%) patients with CR. The 5-year overall survival (OS) was 91% (95% confidence interval [CI] 80%-96%) for the microPRM and 92% (95% CI 89%-95%) for the CR group. The 5-year event-free survival (EFS) was 86% (95% CI 74%-93%) for the microPRM and 86% (95% CI 82%-89%) for the CR group. Neither OS nor EFS was statistically significantly different between the two groups, neither overall nor when risk group stratified.

Conclusions: In the context of cisplatin-based chemotherapy, the presence of microPRM did not influence the outcome even without additional local treatment. Although CR remains the aim, microPRM does not necessitate mandatory second look surgery. A 'wait and see policy' is warranted if postoperative chemotherapy is administered and AFP levels and imaging become normal.
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http://dx.doi.org/10.1016/j.ejca.2018.10.013DOI Listing
January 2019

Multimodality imaging of the Meso-Rex bypass.

Abdom Radiol (NY) 2019 04;44(4):1379-1394

Diagnostic and Therapeutic Services Department, IRCCS-ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Via Ernesto Tricomi 5, 90127, Palermo, Italy.

Extrahepatic portal vein obstruction (EHPVO) is the most common cause of upper gastrointestinal bleeding in children. It is defined as thrombosis of the extrahepatic portal vein with or without extension to the intrahepatic portal veins. The Meso-Rex shunt is the gold standard treatment in children with favorable anatomy since it restores physiological portal liver reperfusion. This is achieved by rerouting the splanchnic venous blood through an autologous graft from the superior mesenteric vein (SMV) into the Rex recess of the left portal vein, curing portal hypertension by doing so. General and hepatobiliary radiologists must be familiar with multimodality imaging appearances of EHPVO and with the role of imaging in identifying suitable candidates for Meso-Rex bypass surgery. Imaging might also detect complications of this procedure, some of which might be treated via interventional radiology.
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http://dx.doi.org/10.1007/s00261-018-1836-1DOI Listing
April 2019

Aggressive prevention and preemptive management of vascular complications after pediatric liver transplantation: A major impact on graft survival and long-term outcome.

Pediatr Transplant 2018 12 1;22(8):e13288. Epub 2018 Sep 1.

Department of Pediatrics and Pediatric Transplantation, ISMETT, UPMC, Palermo, Italy.

Vascular complications are a major cause of patient and graft loss after LTs. The aim of this study was to evaluate the effect of a multimodal perioperative strategy aimed at reducing the incidence of vascular complications. A total of 126 first isolated LTs-performed between November 2008 and December 2015-were retrospectively analyzed. A minimum follow-up period of 24 months was analyzable for 124/126 patients (98.4%). The aggressive preemptive strategy consisted of identifying and immediately managing any problem and any abnormality in the vascular flow, in any of the hepatic vessels, and at any time after the liver graft revascularization. As a result, with a median follow-up of 57 months (3-112 months), not a single graft has been lost from vascular or biliary problems. The actuarial 8-year graft survival is 96.5%. These results have shown that a combination of technical attention, medical prevention, an early diagnosis, and rapid interventions reduced the negative impact of vascular problems on the outcome of both grafts and patients.
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http://dx.doi.org/10.1111/petr.13288DOI Listing
December 2018

Hepatic vein stenting in a 7 week/old infant with Budd-Chiari syndrome using an anterograde approach from the inferior accessory hepatic vein.

Dig Liver Dis 2018 11 18;50(11):1246. Epub 2018 Jun 18.

Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IRCCS-ISMETT), Palermo, Italy.

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http://dx.doi.org/10.1016/j.dld.2018.06.004DOI Listing
November 2018

Caval replacement strategy in pediatric retroperitoneal tumors encasing the vena cava: a single-center experience and review of literature.

J Pediatr Surg 2019 Mar 10;54(3):557-561. Epub 2018 Jun 10.

Department of Pediatric Surgery, Bambino Gesù Children's Hospital, IRCCS, Piazza San Onofrio 4, 00165, Rome, Italy.

Background: Complete encasement of the inferior vena cava by retroperitoneal tumors is rare. Although replacement of the vena cava has been considered for various conditions in adults, it is rarely used in children except for challenging resections and as a last chance approach - often aiming more at debulking than cure.

Materials And Methods: From January 2009 to February 2017, 4 patients (2 adrenal neuroblastomas, 1 renal cell carcinoma, 1 infantile fibrosarcoma) underwent elective en-bloc resection of tumor and of the infrahepatic portion of the inferior vena cava (IVC), with planned IVC prosthetic replacement. In three cases a portion of the left renal vein had to be resected as well, with the vein reanastomosed onto the prosthesis, and a concomitant auto-transplantation of the right kidney was associated in one neuroblastoma patient.

Results: All patients had an uncomplicated postoperative course. In one patient, the prosthetic conduit is patent at long-term (43 months), while the middle portion of the prosthesis did eventually thrombose at mid-term after surgery in the three others - with no related symptoms. Interestingly, all renal venous reconstructions remain patent. Three patients (2 neuroblastomas and 1 infantile fibrosarcoma) are alive and disease-free at 43, 74 and 108 months after surgery, respectively. One patient with renal cell carcinoma died of recurrence of the disease 21 months after surgery.

Conclusion: Resection and reconstruction of the vena cava, including the renal vein portion, can be considered and planned electively in case of tumoral encasement. This strategy is associated with good tolerance of the operation, low morbidity and satisfactory long-term function, even in cases with progressive and/or secondary partial thrombosis.

Level Of Evidence: IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2018.06.008DOI Listing
March 2019

Pediatric Chronic Intestinal Failure in Italy: Report from the 2016 Survey on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Nutrients 2017 Nov 5;9(11). Epub 2017 Nov 5.

Pediatric Surgery Unit, Women's and Children's Health Department, University of Padua, 35122 Padua, Italy.

Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy.

Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family's place of residence and underlying diagnosis determining IF.

Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20-18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53-2.20).

Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.
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http://dx.doi.org/10.3390/nu9111217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5707689PMC
November 2017

More is less: Calling for joining forces for rare pediatric liver tumors research.

Liver Transpl 2017 12;23(12):1501-1504

Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland.

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http://dx.doi.org/10.1002/lt.24968DOI Listing
December 2017

Beyond the Milan criteria for liver transplantation in children with hepatic tumours.

Lancet Gastroenterol Hepatol 2017 06;2(6):456-462

Department of Oncology, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.

The Milan criteria were originally defined in the context of adult liver transplantation for patients with hepatocellular carcinoma and cirrhotic livers. The aim of the criteria was to select patients with small tumours and no disease spread who had a good chance of success, thus avoiding futile transplants. This objective was reached successfully. For the management of selected children with unresectable hepatoblastoma, an almost opposite strategy was proposed and has been implemented in the past two decades, in which transplantation is indicated on the basis of large tumour size and anatomy that precludes the possibility of safe and radical resection. This approach has also had great success. Although both strategies are well established for these two different age groups and diseases, a grey area exists with regard to hepatocellular carcinoma or other tumour types in children. In this Viewpoint, we aim to review the existing literature about the indications, selection process, and results of liver transplantation for liver tumours in children, and discuss evidence that supports the implementation of either of the two strategies in the context of managing selected children with liver tumours using transplantation.
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http://dx.doi.org/10.1016/S2468-1253(17)30084-5DOI Listing
June 2017

Bevacizumab Combined with Chemotherapy in Children Affected by Hepatocellular Carcinoma: a Single-center Experience.

Anticancer Res 2017 03;37(3):1489-1493

Department of Pediatric Hematology/Oncology and Stem Cell Transplantation, Bambino Gesù Pediatric Hospital, Rome, Italy.

Background: Hepatocellular carcinoma (HCC) is very rare in children and is traditionally associated with a poor prognosis because many patients are not amenable to conventional resection, even in the absence of metastatic disease. For patients with locally advanced or metastatic tumors, conventional chemotherapy appears to offer limited survival benefits.

Patients And Methods: We report a case series of five consecutive pediatric patients with HCC who were treated with bevacizumab along with conventional platinum-based chemotherapy.

Results: Overall, all five patients presented an objective response to neoadjuvant chemotherapy. Four patients remain alive and free of disease 54 months after diagnosis (range=20-85 months) after treatment with bevacizumab in combination with chemotherapy and surgery that consisted of partial liver resection in two patients and liver transplantation in three.

Conclusion: In our experience, bevacizumab combined with chemotherapy was an effective and safe option for the treatment of children affected by HCC.
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http://dx.doi.org/10.21873/anticanres.11475DOI Listing
March 2017

Liver transplant in ethylmalonic encephalopathy: a new treatment for an otherwise fatal disease.

Brain 2016 Apr 25;139(Pt 4):1045-51. Epub 2016 Feb 25.

Department of Surgery and Transplantation, Bambino Gesù Children's Research Hospital IRCCS, Rome, Italy.

Ethylmalonic encephalopathy is a fatal, rapidly progressive mitochondrial disorder caused by ETHE1 mutations, whose peculiar clinical and biochemical features are due to the toxic accumulation of hydrogen sulphide and of its metabolites, including thiosulphate. In mice with ethylmalonic encephalopathy, liver-targeted adeno-associated virus-mediated ETHE1 gene transfer dramatically improved both clinical course and metabolic abnormalities. Reasoning that the same achievement could be accomplished by liver transplantation, we performed living donor-liver transplantation in an infant with ethylmalonic encephalopathy. Unlike the invariably progressive deterioration of the disease, 8 months after liver transplantation, we observed striking neurological improvement with remarkable achievements in psychomotor development, along with dramatic reversion of biochemical abnormalities. These results clearly indicate that liver transplantation is a viable therapeutic option for ETHE1 disease.
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http://dx.doi.org/10.1093/brain/aww013DOI Listing
April 2016

Genetics and Molecular Modeling of New Mutations of Familial Intrahepatic Cholestasis in a Single Italian Center.

PLoS One 2015 17;10(12):e0145021. Epub 2015 Dec 17.

Dept. Pathology and Molecular Histopathology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Familial intrahepatic cholestases (FICs) are a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. Three distinct forms are described: FIC1 and FIC2, associated with low/normal GGT level in serum, which are caused by impaired bile salt secretion due to defects in ATP8B1 encoding the FIC1 protein and defects in ABCB11 encoding bile salt export pump protein, respectively; FIC3, linked to high GGT level, involves impaired biliary phospholipid secretion due to defects in ABCB4, encoding multidrug resistance 3 protein. Different mutations in these genes may cause either a progressive familial intrahepatic cholestasis (PFIC) or a benign recurrent intrahepatic cholestasis (BRIC). For the purposes of the present study we genotyped 27 children with intrahepatic cholestasis, diagnosed on either a clinical or histological basis. Two BRIC, 23 PFIC and 2 BRIC/PFIC were identified. Thirty-four different mutations were found of which 11 were novel. One was a 2Mb deletion (5'UTR- exon 18) in ATP8B1. In another case microsatellite analysis of chromosome 2, including ABCB11, showed uniparental disomy. Two cases were compound heterozygous for BRIC/PFIC2 mutations. Our results highlight the importance of the pathogenic role of novel mutations in the three genes and unusual modes of their transmission.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0145021PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4683058PMC
June 2016

Reducing left liver lobe grafts, more or less? Don't throw out the baby with the bath water....

Pediatr Transplant 2015 Dec 4;19(8):815-7. Epub 2015 Nov 4.

Paediatric Surgery, Università di Roma Tor Vergata, Rome, Italy.

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http://dx.doi.org/10.1111/petr.12635DOI Listing
December 2015

Congenital double intrahepatic portosystemic shunt: Imaging findings and endovascular closure.

Ann Hepatol 2015 Nov-Dec;14(6):919-23

Interventional Radiology Unit, Department of Surgery. Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.5604/16652681.1171784DOI Listing
July 2016

Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium.

Hepatology 2016 Apr 23;63(4):1368-80. Epub 2015 Oct 23.

Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.

Unlabelled: Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized.

Conclusion: There are few pediatric data to indicate the efficacy and safety of pharmacologic or endoscopic therapies as primary prophylaxis or that prevention of a sentinel variceal bleed will ultimately improve survival; therefore, no recommendation for primary prophylaxis with endoscopic variceal ligation, sclerotherapy, or nonspecific beta-blockade in children was proposed.
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http://dx.doi.org/10.1002/hep.28153DOI Listing
April 2016

Optimization and planning of operating theatre activities: an original definition of pathways and process modeling.

BMC Med Inform Decis Mak 2015 May 17;15:38. Epub 2015 May 17.

Department of Economics and Business Studies, University of Genoa, Genoa, Italy.

Background: The Operating Room (OR) is a key resource of all major hospitals, but it also accounts for up 40% of resource costs. Improving cost effectiveness, while maintaining a quality of care, is a universal objective. These goals imply an optimization of planning and a scheduling of the activities involved. This is highly challenging due to the inherent variable and unpredictable nature of surgery.

Methods: A Business Process Modeling Notation (BPMN 2.0) was used for the representation of the "OR Process" (being defined as the sequence of all of the elementary steps between "patient ready for surgery" to "patient operated upon") as a general pathway ("path"). The path was then both further standardized as much as possible and, at the same time, keeping all of the key-elements that would allow one to address or define the other steps of planning, and the inherent and wide variability in terms of patient specificity. The path was used to schedule OR activity, room-by-room, and day-by-day, feeding the process from a "waiting list database" and using a mathematical optimization model with the objective of ending up in an optimized planning.

Results: The OR process was defined with special attention paid to flows, timing and resource involvement. Standardization involved a dynamics operation and defined an expected operating time for each operation. The optimization model has been implemented and tested on real clinical data. The comparison of the results reported with the real data, shows that by using the optimization model, allows for the scheduling of about 30% more patients than in actual practice, as well as to better exploit the OR efficiency, increasing the average operating room utilization rate up to 20%.

Conclusions: The optimization of OR activity planning is essential in order to manage the hospital's waiting list. Optimal planning is facilitated by defining the operation as a standard pathway where all variables are taken into account. By allowing a precise scheduling, it feeds the process of planning and, further up-stream, the management of a waiting list in an interactive and bi-directional dynamic process.
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http://dx.doi.org/10.1186/s12911-015-0161-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4436841PMC
May 2015

Pancreatitis and portal vein thrombosis in children: the chicken or the egg causality dilemma.

J Pediatr Surg 2015 Apr 21;50(4):565-9. Epub 2014 Nov 21.

Hepatobiliary Surgery Unit, Department of Surgery, Bambino Gesù Children's Hospital, Roma, Italy; Pediatric Surgery, University of Roma Tor Vergata, Roma, Italy.

Background: The association of pancreatitis and portal vein thrombosis (PVT) is extremely rare in children. We report on 3 cases which suggest that there may be a causal relation between the two.

Methods: Clinical characteristics and evolution of 3 children with this particular condition were analyzed retrospectively. In this group of patients, the strategy consisted in opting for early surgical decompression of the portal hypertension, which was followed by a favorable outcome, not only in terms of complications related to the portal hypertension but also of a contemporaneous spontaneous regression of the concurrent pancreatic disease, in absence of any other specific management of the latter problem.

Results And Conclusions: Combined PVT and pancreatitis is exceptional in children. Although this series is small, it provides insight and some evidence that the pancreatic disease might be secondary to the cavernomatous transformation of the regional venous system. More interestingly, it suggests that the appropriate management strategy should be to rapidly relieve portal hypertension after resolution of the acute phase of pancreatitis.
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http://dx.doi.org/10.1016/j.jpedsurg.2014.11.039DOI Listing
April 2015

Unusual Tract and Complication of a Percutaneous Gastrostomy Tube.

J Pediatr Gastroenterol Nutr 2017 03;64(3):e71

*Pediatric Surgery Unit, Passoscuro †Hepato-Biliary Surgery Unit, Department of Pediatric Surgery and Transplantation Center, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy.

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http://dx.doi.org/10.1097/MPG.0000000000000779DOI Listing
March 2017