Pseudo-tunneling procedure for PICC-ports catheters insertion in patients with small veins at the middle third of the arm.

BACKGROUND: Axillary vein in the brachial tract or Basilic vein in the proximal third of the arm usually present a diameter wide enough to receive a catheter of 5 Fr. Therefore, a peripherally inserted central catheters with port (PICC-Ports) cannot be positioned in these areas. Pseudo-tunneling procedure allows the positioning of the Port in the middle part of the arm without using tunnelers during insertion of PICC with Port in patients who present deep veins of the arm too small to receive a PICC-Port catheter. The aim of this study was to present our experience with pseudo-tunneling procedure during the positioning of the PICC-Port, while using a particular variation of the technique already described for PICCs and midline. METHODS: From January to December 2023, 103 PICC-Ports were placed in adult patients. Ninety catheters were tunneled from the puncture of the axillary vein at the proximal arm point, while we used this new technique in 42 patients by the same trained team of Vascular Access Unit at ASST Spedali Civili Hospital of Brescia. RESULTS: All procedures were successfully performed at the first attempt. No insertion related complications were observed. CONCLUSIONS: Our data suggest pseudo-tunnelling is a safe and effective technique for PICC-Ports insertion avoiding central venous catheterization, even in patient with small vein at the arms.

Guidewire replacement of valved tunneled-cuffed silicone catheters with power injectable polyurethane tunneled-cuffed catheters or with ports.

BACKGROUND: Silicone Cuffed Centrally Inserted Central venous catheters (CICCs) were a type of catheters that have been used for a long time especially in cancer patients. Recently, thanks to biomedic research progresses, polyurethane catheters have resulted in higher surgical performances compared to classical silicone ones. Indeed, the inferior calibers of these new catheters lead to an extremely faster infusion rate. The presence of a valve at the tip of the catheter could suggest an impossible replacement procedure over a Seldinger guidewire. METHOD: The aim of this article is to explain our replacement technique over guidewire of silicone cuffed and valved tunneled CICCs with a power injectable polyurethane cuffed tunneled CICC. The casistic presented was evaluated at the Vascular Access Unit of ASST Spedali Civili in Brescia, Italy. The study involved 35 successful catheter replacement over guidewire, meanwhile cases where patients presented sepsis, exit site infection, or catheter damage were premeditatedly excluded. RESULTS: The maneuver was always conducted following the same procedure without noticing particular complications associated with CICC insertion. Indeed, the operation was quick, feasible, and safe. Septic, thromboembolic, or hemorrhagic complications also related to patients presenting dysfunctional coagulation cascade were not encountered. CONCLUSIONS: Our experience regarding the replacement technique of silicone cuffed and valved catheters over guidewire was considered feasible, accurate, and efficient for all patients treated, even in those presenting thrombocytopenia or a dysfunctional coagulation cascade.

Guidewire replacement of tunneled central venous catheters in children reusing the same subcutaneous route.

BACKGROUND: Tunneled centrally inserted central venous catheters (CICCs) are a type of catheters used especially in cancer pediatric patients or affected from short bowel disease and malabsorption. Recently, thanks to biomedical research progresses, polyurethane catheters have resulted in high surgical performances with a low caliber but extremely fast infusion rate. Sometimes the replacement procedure over a Seldinger guidewire could be an appreciable technique especially reusing the same vein and the same tunnel of the previous catheter, with local anesthesia. The aim of this article was to explain our replacement technique over guidewire of tunneled CICCs on pediatric patients. METHODS: The casuistry presented was evaluated at the Unit of Vascular Access of ASST Spedali Civili in Brescia, Italy. The study involved 7 successful catheter replacement over guidewire, meanwhile cases where patients presented sepsis, exit site infection or catheter damage were premeditatedly excluded. Three of them were tunneled and cuffed. Five catheters were in brachiocephalic vein and 2 in internal jugular vein. RESULTS: Every procedure was ended without complications. Of seven patients, five of them underwent to the procedure only with local anesthesia and 2 patients require premedication with Midazolam. No one required general anesthesia. The maneuver was always conducted following the same procedure without noticing particular complications associated with CICC insertion. Indeed, the operation was quick, feasible and safe. Septic, thromboembolic, or hemorrhagic complications also related to patients presenting dysfunctional coagulation cascade were not encountered. CONCLUSIONS: Our experience regarding the replacement technique of tunneled catheters over guidewire was considered feasible, accurate and efficient for all patients treated, even in those presenting thrombocytopenia or dysfunctional coagulation cascade.

How to remove a tunneled and cuffed central venous catheter quickly and safely? A little trick useful in children.

BACKGROUND: The aim of this study was to present our experience with a use of own variation of the traditional technique for tunneled and cuffed catheters removal. METHODS: From July 2021 to March 2023, 110 tunneled cuffed centrally inserted central catheters (CICCs) were removed at the Vascular Access Unit of ASST Spedali Civili of Brescia using our particular technique; pediatric patients were 15 admitted to "Children Hospital" of Brescia. The catheter is cut with its adhesions above the cuff and then pulled out. The cuff is then removed with all adhesions attached. RESULTS: Each procedure was successfully performed to the end with local anesthesia, also in younger children. The maneuver was very short and well tolerated. No related complications were observed. The maneuver has always been considered easy even by less experienced surgeons. The scars were aesthetically good and well accepted by the patients. CONCLUSIONS: The technique described has the advantage of not requiring the careful lysis of the adherence from the cuff. If the procedure is quick it can be performed without general anesthesia and without significant psychological trauma also in less compliant children. The skin cut very short leaves an almost invisible scar. It is another notable advantage especially for young girls.

Pseudo-tunneling procedure: An easy technique for insertion of PICCs and Midline catheters in patients with small veins of the arm.

BACKGROUND: Axillary vein in the brachial tract or Basilic vein in the proximal third of the arm has got usually an enough diameter to receive a catheter of 4 or 5 Fr. In this case the exit site should be too proximal to the axilla with an higher risk of infection. Pseudo-tunneling procedure can create an exit site at the middle of the arm without using tunnelers during insertion of Peripherally Inserted Central Catheters and Midlines in patients who present deep veins of the arm too small to receive a catheter to consent infusion and blood samples. AIM: The aim of this study is to present our experience with pseudo-tunneling procedure, also using a particular variation of the technique. METHODS: From January 2014 to August 2022 150 Peripherally Inserted Central Catheters and 221 Midlines were insert in pediatric and adults patients with too small deep veins at the middle third of the arm with this technique by the same trained team of Vascular Access Unit at ASST Spedali Civili Hospital of Brescia. RESULTS: All procedures were successfully performed at the first or at the second attempt. No insertion related complications were observed. CONCLUSIONS: Our data suggest pseudo-tunneling technique is a safe and effective procedure for of Peripherally Inserted Central Catheters and Midline insertion avoiding central venous catheterization even in patient with small vein at the arms.

Pseudo-tunneling procedure: an advantageous and safe technique for brachial catheters in younger children.

BACKGROUND: The aim of this study is to present our experience with the use of a particular tunneling technique called pseudo-tunnelling, during insertion of peripherally inserted central catheters (PICCs) and Midlines in younger patients. The children's brachial veins at the middle third of the arm are usually too small for cannulation. So the veins in the axilla are the best option for implantation of a four or five French catheter. A pseudo-tunneling procedure can create an exit site at the middle of the arm without using other procedural sets. METHODS: From January 2014 to August 2022, 60 PICCs and 113 midlines were inserted in children admitted to Children Hospital of Brescia. RESULTS: Every procedure was successfully performed during the first or the second time at latest. The time of procedure was not significantly different from non tunnelized procedures. No insertion related complications were observed. CONCLUSIONS: Our data suggests that pseudo-tunneling is a safe and effective procedure for brachial device implants to avoid central venous catheterization even in pediatric patients.

Nutrition and Intestinal Rehabilitation of Children With Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 2: Long-Term Follow-Up on Home Parenteral Nutrition.

Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The preferred treatment for IF is parenteral nutrition which may be required until adulthood. The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition. All members of the Nutrition Committee of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) contributed to this position paper. Some renowned experts in the field joined the team to guide with their expertise. A systematic literature search was performed from 2005 to May 2021 using PubMed, MEDLINE, and Cochrane Database of Systematic Reviews. In the absence of evidence, recommendations reflect the expert opinion of the authors. Literature on SBS mainly consists of retrospective single-center experience, thus most of the current papers and recommendations are based on expert opinion. All recommendations were voted on by the expert panel and reached >90% agreement. This second part of the position paper is dedicated to the long-term management of children with SBS-IF. The paper mainly focuses on how to achieve intestinal rehabilitation, treatment of complications, and on possible surgical and medical management to increase intestinal absorption.

Nutrition and Intestinal Rehabilitation of Children With Short Bowel Syndrome: A Position Paper of the ESPGHAN Committee on Nutrition. Part 1: From Intestinal Resection to Home Discharge.

Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The mainstay of treatment for IF is parenteral nutrition (PN). The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition. All members of the Nutrition Committee of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) contributed to this position paper. Some renowned experts in the field joined the team to guide with their experience. A systematic literature search was performed from 2005 to May 2021 using PubMed, MEDLINE, and Cochrane Database of Systematic Reviews. In the absence of evidence, recommendations reflect the expert opinion of the authors. Literature on SBS mainly consists of retrospective single-center experience, thus most of the current papers and recommendations are based on expert opinion. All recommendations were voted on by the expert panel and reached >90% agreement. The first part of this position paper focuses on the physiological mechanism of intestinal adaptation after surgical resection. It subsequently provides some clinical practice recommendations for the primary management of children with SBS from surgical resection until discharged home on PN.

Testicular torsion during the COVID-19 pandemic: Results of a multicenter study in northern Italy.

INTRODUCTION: The literature reported an increased avoidance of the Emergency Department (ED) during COrona VIrus Disease 19 (COVID-19) pandemic, causing a subsequent increase of morbidity and mortality for acute conditions. Testicular torsion is a surgical emergency, which can lead to the loss of the affected testicle if a delayed treatment occurs. As testicular loss is time-related, outcome was hypothesized to be negatively affected by the pandemic. OBJECTIVE: The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19. STUDY DESIGN: Medical records of pediatric patients operated for testicular torsion of six Paediatric Surgical Units in Northern Italy between January 2019 and December 2020 were retrospectively reviewed. Patients were divided as for ones treated during (dC) or before the pandemic (pC). To reflect possible seasonality, related to lockdown restrictions, winter and summer calendar blocks were also analysed. For all cohorts, demographic data, pre-operative evaluation, operative notes and post-operative outcomes were reviewed. Primary outcomes were referral time, time from diagnosis to surgery and ischemic time, while secondary outcomes were orchiectomy and atrophy rates. Statistic was conducted as appropriate. RESULTS: A total of 188 patients with acute testicular torsion were included in the study period, 89 in the pre-COVID-19 (pC) period and 99 during COVID-19 (dC). Time from symptom onset to the access to the Emergency Department (T1) was not different among the two populations (pC: 5,5 h, dC: 6 h, p 0.374), and similarly time from diagnosis to surgery (pC: 2,5 h, dC: 2,5 h, p 0.970) and ischemic time (pC: 8,2 h, dC: 10 h, p 0.655). T1 was <6 h in 46/99 patients (46%) pC and 45/89 patients (51%) dC (p = 0.88, Fisher's exact test). Subgroup analysis accounting for different lockdown measures, confirm the absence of any difference. Orchiectomies rate was 23% (23/99) dC and 21% (19/89) pC (p = 0.861, Fisher's exact test) and rate of post-operative atrophy was 9% dC (7/76) and 14% pC (10/70), p = 0,44, Fisher's exact test. DISCUSSION: Despite worldwide pediatric ED accesses reduction, we reported that neither ischemic time nor the long-term outcomes in children with testicular torsion increased during the COVID-19 pandemic. In the available literature, few studies investigated the topic and are controversial on the results. Similarly to our findings, some studies found that timing and orchiectomy rates were not significantly different during the pandemic, while others reported a correlation to pandemic seasonality. Furthermore, in the recent pediatric literature it has been reported a delayed testicular torsion diagnosis due to shame in informing parents. Strengths of this study are the large numerosity, its multicentric design and a long study period. Its main limitation is being retrospective. CONCLUSIONS: We reported our large cohort from one of the most heavily COVID-19-affected regions, finding that referral, intra-hospital protocols and ischemic time in testicular torsion were not increased during to the pandemic, as well as orchiectomy rate and atrophy.

Autologous Intestinal Reconstruction Surgery in Short Bowel Syndrome: Which, When, and Why.

Short bowel syndrome (SBS), secondary to any natural loss or after any extensive bowel resection for congenital malformations or acquired disease, is the most common cause of intestinal failure in children. Extensive introduction of parenteral nutrition (PN) has dramatically changed the outcome of these patients, allowing for long-term survival. The main goal in children with SBS remains to be increasing enteral tolerance and weaning from PN support. Post resection intestinal adaptation allows for achievement of enteral autonomy in a subset of these patients, but the inability to progress in enteral tolerance exposes others to long-term complications of PN. Autologous intestinal reconstruction surgery (AIRS) can facilitate the fulfilment of enteral autonomy, maximizing the absorptive potential of the remaining gut. All the different intestinal reconstruction techniques, from simple procedures like tapering, reversed segments, and colon interposition, to more complex lengthening procedures (LILT: longitudinal intestinal lengthening and tailoring, STEP: serial transverse enteroplasty, and SILT: spiral intestinal lengthening and tailoring) and techniques designed for peculiar problems like controlled intestinal tissue expansion or duodenal lengthening are presented. AIRS indications, clinical applications, and results reported in the literature are reviewed.

Robotic surgery in emergency setting: 2021 WSES position paper.

BACKGROUND: Robotics represents the most technologically advanced approach in minimally invasive surgery (MIS). Its application in general surgery has increased progressively, with some early experience reported in emergency settings. The present position paper, supported by the World Society of Emergency Surgery (WSES), aims to provide a systematic review of the literature to develop consensus statements about the potential use of robotics in emergency general surgery. METHODS: This position paper was conducted according to the WSES methodology. A steering committee was constituted to draft the position paper according to the literature review. An international expert panel then critically revised the manuscript. Each statement was voted through a web survey to reach a consensus. RESULTS: Ten studies (3 case reports, 3 case series, and 4 retrospective comparative cohort studies) have been published regarding the applications of robotics for emergency general surgery procedures. Due to the paucity and overall low quality of evidence, 6 statements are proposed as expert opinions. In general, the experts claim for a strict patient selection while approaching emergent general surgery procedures with robotics, eventually considering it for hemodynamically stable patients only. An emergency setting should not be seen as an absolute contraindication for robotic surgery if an adequate training of the operating surgical team is available. In such conditions, robotic surgery can be considered safe, feasible, and associated with surgical outcomes related to an MIS approach. However, there are some concerns regarding the adoption of robotic surgery for emergency surgeries associated with the following: (i) the availability and accessibility of the robotic platform for emergency units and during night shifts, (ii) expected longer operative times, and (iii) increased costs. Further research is necessary to investigate the role of robotic surgery in emergency settings and to explore the possibility of performing telementoring and telesurgery, which are particularly valuable in emergency situations. CONCLUSIONS: Many hospitals are currently equipped with a robotic surgical platform which needs to be implemented efficiently. The role of robotic surgery for emergency procedures remains under investigation. However, its use is expanding with a careful assessment of costs and timeliness of operations. The proposed statements should be seen as a preliminary guide for the surgical community stressing the need for reevaluation and update processes as evidence expands in the relevant literature.

Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia.

BACKGROUND: Kasai portoenterostomy (KPE) is the preferred treatment for biliary atresia (BA) patients. It has been shown that the center caseload of KPE impacts on native liver survival. We aimed to define the impact of KPE caseload on complications at the time of liver transplantation (LT). METHODS: Retrospective data collection of LT for BA performed in our tertiary center between 2010 and 2018. The patients were grouped according to the caseload of the center that performed KPE: Group A (≥5 KPE/year) and Group B (<5 KPE/year). We analyzed total transplant time (TTT), hepatectomy time, amount of plasma and red blood cell (RBC) transfusions, occurrence of bowel perforations at LT. RESULTS: Among 115 patients, Group A (n 44) and Group B (n 71) were comparable for age, sex, PELD score, TTT. The groups differed for: median hepatectomy time (57 min, IQR = 50-67; vs 65, IQR 55-89, p = 0.045); RBC transfusions (95 ml, IQR 0-250; vs 200 ml, IQR 70-500, p = 0.017); bowel perforations (0/44 vs 15/71, p = 0.001). One-year graft loss in Group A vs Group B was 1/44 vs 7/71 (p = 0.239), whereas deaths were 0/44 vs 5/71 respectively (p = 0.183); 5/15 patients who had a perforation eventually lost the graft. CONCLUSIONS: This study found an association between KPE performed in low caseload center and the incidence of complications at LT. These patients tend to have a worse outcome. The centralization of KPE to referral center represents an advantage at the time of LT. MINI ABSTRACT: We studied the impact of Kasai portoenterostomy (KPE) caseload on complications at the time of liver transplantation (LT), in 115 patients. We found an association between KPE performed in low caseload center and increased bowel perforations and blood transfusions. We suggest to centralize to experienced center all children requiring KPE.

Technique for replacement of Groshong® peripherally inserted central venous catheters (PICCs) in children.

BACKGROUND: The use of peripherally inserted central catheters (PICCs) in children is increasingly common due to their certain advantages and low rate of adverse events. Valved PICCs, when displaced, must be removed and new a PICC must be placed in another vein. We present our PICC replacement technique over a Seldinger guidewire using the same entrance vein. METHODS: This prospective study involved all consecutive patients with a previously inserted Groshong PICC (Bard Access Systems Inc., Salt Lake City, UT, USA) requiring replacement due to displacement managed at our institution between January 2018 and January 2019. All procedures were performed at the patients' bedside, under local anesthesia. All the steps of the procedure were described. RESULTS: The procedure was performed in 19 patients (mean 10.4 years, range 5-18 years) in a mean time of 12 minutes (range 10-35). In all cases, the maneuver was quick and painless. No complications were encountered during the procedure and at follow-up. CONCLUSIONS: Our preliminary experience suggests that the PICC replacement technique using the same entrance vein is simple, safe, and feasible, and provides clear benefits to preserve children's venous patrimony.

Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group.

Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.

Prediction of complex gastroschisis: The evolution of therapeutic techniques and their relation with fetal sonographic features.

BACKGROUND: To analyze prenatal ultrasound (US) markers to predict treatment and adverse neonatal outcome in fetal gastroschisis. METHODS: It was conducted a retrospective single-center study considering all pregnancies with isolated gastroschisis that were treated in our department between 2008 and 2020. 17 US markers were analyzed. Moreover, the association between prenatal ultrasound signs and neonatal outcomes was analyzed: need of bowel resection, techniques of reduction, type of closure, adverse neonatal outcomes, time to full enteral feeding, length of total parenteral nutrition and length of hospitalization. RESULTS: The analysis included 21 cases. We found significant associations between intestinal dilation (≥10 mm) appeared before 30 weeks of gestation and the need of bowel resection (p = 0.001), the length of total parenteral nutrition (p = 0,0013) and the length of hospitalization (p = 0,0017). Intrauterine growth restriction (IUGR) is a risk factor for serial reduction (p = 0,035). There were no signs significantly associated with the type of closure. Hyperbilirubinemia is related with gestational age (GA) at the diagnosis of intra-abdominal bowel dilation (IABD) (p = 0.0376) and maximum IABD (p = 0.05). All newborns with sepsis had echogenic loops in uterus (p = 0.026). The relation between the GA at delivery and the GA at the extra-abdominal bowel dilation (EABD)≥10 mm was r = 0.70. CONCLUSION: We showed the significant role of the early presence of bowel dilation in predicting intestinal resection and adverse outcomes. All IUGR fetuses needed staged reduction through the silo-bag technique. The echogenic bowel was related to neonatal sepsis, while IABD was associated with hyperbilirubinemia.

Endoscopic treatment of periampullary duodenal duplication cysts in children: Four case reports and review of the literature.

BACKGROUND: Duodenal duplications are rare congenital anomalies of the gastrointestinal tract. As the periampullary variant is much rarer, literature is scant and only few authors have reported their experience in diagnosis and treatment, particularly with operative endoscopy. CASE SUMARY: To report our experience with the endoscopic treatment in a series of children with periampullary duodenal duplication cysts, focusing on the importance of obtaining an accurate preoperative anatomic assessment of the malformations. The pediatric periampullary duodenal duplication cyst literature is reviewed. We conducted a systematic review according to the PRISMA guidelines. The PubMed database was searched for original studies on "duodenal duplication", "periampullary duplication" or "endoscopic management" published since 1990, involving patients younger than 18 years of age. Eligible study designs were case report, case series and reviews. We analyzed the data and reported the results in table and text. Fifteen eligible articles met the inclusion criteria with 16 patients, and analysis was extended to our additional 4 cases. Median age at diagnosis was 13.5 years. Endoscopic treatment was performed in 10 (50%) patients, with only 2 registered complications. CONCLUSION: Periampullary duodenal duplication cysts in pediatric patients are very rare. Our experience suggests that an accurate preoperative assessment is critical. In the presence of sludge or stones inside the duplication, endoscopic retrograde cholangio-pancreatography is mandatory to demonstrate a communication with the biliary tree. Endoscopic treatment resulted in a safe, minimally invasive and effective treatment. In periampullary duodenal duplication cyst endoscopically treated children, long-term follow-up is still necessary considering the potential malignant transformation at the duplication site.

Determining oncogenic patterns and cancer predisposition through the transcriptomic profile in Mitchell-Riley syndrome with heterotopic gastric mucosa and duodenal atresia: a case report.

BACKGROUND: Homozygous mutations in the transcription factor RFX6 are the cause of the Mitchell-Riley syndrome (MRS) associating neonatal diabetes, congenital digestive system, such as biliary atresia, pancreatic hypoplasia, duodenal and/or jejunal atresia, intestinal malrotation, gallbladder aplasia, cholestasis. A constitutive inactivation of RFX6 leads also to gastric heterotopia. Application of RNA-seq in human diseases may help to better understand pathogenic mechanism of diseases and to predict the risk of developing chronic disorders and personalizing their prevention and treatment. We evaluated oncogenic patterns and cancer predisposition using the transcriptomic profile in a case of MRS with neonatal diabetes, duodenal atresia, and extensive intestinal tract gastric heterotopia. RESULTS: We signalled the interactors of RFX6 with other up and downregulated genes, that may be interested in severity of diabetic condition, in multi-organs impairment and cancer predisposition. Furthermore, several dysregulated genes are involved in biological processes that can lead to promote cancer including "Evading apoptosis" (BAD, BBC3, EGF, FGFR2, FLT3LG, HMOX1, HRAS, IFNAR2, IGF1R, IL12RB1, IL13RA1, IL15, IL2RB, IL2RG, IL6R, KEAP1, MGST1, PDGFA, PDGFRB, PIK3R3, RALB, RALGDS, RASSF1, SOS1, TGFA, TXNRD3), "Proliferation" (APC, BRAF, CCND2, CCND3, CCNE2, FGFR2, FLT3LG, FZD1, FZD6, HMOX1, HRAS, IGF1R, KEAP1, LRP6, MAPK3, MGST1, PDGFA, PDGFB, PDGFRB, RB1, SOS1, TGFA, TXNRD3, WNT10B), "Sustained angiogenesis" (BRAF, FGFR2, FLT3LG, HRAS, IGF1R, JAG1, MAPK3, NOTCH2, PDGFA, PDGFB, PDGFRB, SOS1, TGFA, TGFB1), "Genomic instability" (BAD, BBC3) and "Insensitivity to anti-growth signals" (SMAD2, TGFB1). We also inspected the signalings and their related genes in cancer, such as "PI3K signaling", "ERK signaling", "JAK-STAT signaling", "Calcium signaling", "Other RAS signaling", "WNT signaling". CONCLUSIONS: In our MRS patient, we signaled the interactors of RFX6 with other up- and downregulated genes that may be related to severe diabetic condition, multi-organ impairment, and cancer predisposition. Notably, many dysregulated genes may lead to triggering carcinogenesis. The possibility of the patient developing cancer degeneration in heterotopic gastric mucosa and/or additional long-term tumoral sequelae is not excluded. Personalized prevention and treatment strategies should be proposed.

Extended Adhesion-Sparing Liver Eversion during Kasai Portoenterostomy for Infants with Biliary Atresia.

BACKGROUND: Despite the fact that Kasai portoenterostomy (KPE) is the primary treatment for biliary atresia (BA), liver transplantation (LT) remains the ultimate surgery for two-thirds of these patients. Their true survival rate with the native liver reflects the original KPE and the burden of post-operative complications. We report an original modification of the adhesion-sparing liver eversion (ASLE) technique during KPE that facilitates the total native hepatectomy at time of transplantation. METHODS: All consecutive patients with BA who underwent KPE at our department and subsequent LT at Paediatric Liver Transplant Centre at Papa Giovanni XXIII Hospital between 2010-2018 were retrospectively enrolled. All patients underwent ASLE during KPE. Patients' demographic data, type of KPE, total transplant time (TTT), hepatectomy time (HT), intra-operative packed red blood cells and plasma transfusions, intra- and post-operative complications were noted. RESULTS: 44 patients were enrolled. Median TTT and HT were 337 and 57 min, respectively. The median volume of packed red blood cell transfusion was 95 mL. No patients presented bowel perforation during the procedure or in the short post-operative course. No mortality after LT was recorded. CONCLUSIONS: In addition to the well-known advantages of the standard liver eversion technique, ASLE reduces the formation of intra-abdominal adhesions, lowering significantly the risk of bowel perforation and bleeding when liver transplantation is performed for failure of KPE.