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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.

The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years. We collected age at presentation, clinical features, histology, diagnostic process, management and outcome. Twenty seven patients (male/female 5/22), median age 13 days (1–1530) presented with hepatomegaly (24/27), cardiac failure (10/27), cutaneous hemangiomas (8/27), fever and anemia (6/27 each), vomiting (5/27), splenomegaly (4/27). The lesion was focal, multifocal, or diffuse in 9 patients of each group. The management included medical treatment (8/27), embolization (8/27), resection (3/27), observation (6/27), transplantation (2/27). After 16 months’ follow-up (30 days–11 years), 23/27 (85%) were alive. Diffuse lesions (4/4), cardiac failure (4/4), type II histology (4/4), age older than 6 months at diagnosis (3/4) predicted mortality (all p

Variants in the ACTG2 gene, encoding a protein crucial for correct enteric muscle contraction, have been found in patients affected with chronic intestinal pseudo-obstruction, either congenital or late-onset visceral myopathy, and megacystis-microcolon-intestinal hypoperistalsis syndrome. Here we report about ten pediatric and one adult patients, from nine families, carrying ACTG2 variants: four show novel still unpublished missense variants, including one that is apparently transmitted according to a recessive mode of inheritance. Four of the remaining five probands carry variants affecting arginine residues, that have already been associated with a severe phenotype. A de novo occurrence of the variants could be confirmed in six of these families. Since a genotype-phenotype correlation is affected by extrinsic factors, such as, diagnosis delay, quality of clinical management, and intra-familial variability, we have undertaken 3D molecular modeling to get further insights into the effects of the variants here described. The present findings and further ACTG2 testing of patients presenting with intestinal pseudo-obstruction, will improve our understanding of visceral myopathies, including implications in the prognosis and genetic counseling of this set of severe disorders.

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 (

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.

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.

Colostomy is considered by most authors the first step of treatment for neonates affected by high or intermediate anorectal malformations (ARMs). However, for this subset of patients, the debate between total diverting colostomy (TDC) and loop colostomy (LC) is still ongoing among the paediatric surgeons community. The aim of this paper is to present our experience consisting in applying the Brooke technique, until now used only for ileostomy, in order to perform a functionally diverting loop colostomy (FDLC) in patients with high or intermediate ARMs. At our institution, from January 2014 to December 2018, 12 patients (6 males and 6 females) with high or intermediate ARMs underwent colostomy according to the Brooke ileostomy technique. The only complication observed was mild proximal stoma prolapse in 1 patient. Creation and closure of the stomas were easy, and the final cosmetic result was very satisfying in all patients. We believe that this is a promising technique since it might overcome the drawbacks of a TDC as it is less invasive, easier, and quicker to perform. Furthermore, it guarantees final better cosmetic results, albeit functioning as a TDC.

S. typhi infection rarely involves the genitourinary system. We report the first described case of acute epididymo-orchitis due to S. typhi in a 14-year-old boy from Bangladesh. A high index of suspicion should be maintained when evaluating patients coming from endemic countries also in case of unusual sites of infection.

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.The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19.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.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 was6 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.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.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.

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.

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.

Introduction 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 requiring replacement due to displacement managed at our institution between January 2018 and January 2019. All procedures were performed at the patient's bed-side, 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 in order to preserve children's venous patrimony.

Study Objective In a pediatric setting, laparoscopic management of large cystic ovarian neoplasms with low malignancy probability is not suitable, because of the mass size; nevertheless, an effort should be made to be as minimally invasive as possible, without violating the principles of oncologic surgery. We describe our experience in managing these neoplasms with leak-proof extracorporeal drainage through mini laparotomy, followed by cyst excision or oophorectomy. Design Case series study, describing interventions and outcomes. Setting Department of pediatric surgery in a tertiary pediatric and adult university hospital. Participants Pediatric patients affected by large cystic ovarian mass. Interventions Hybrid minimally invasive approach using leak-proof extracorporeal drainage. Main Outcome Measures Data on demographic characteristics, tumor marker values, and imaging findings were collected and analyzed. Outcome of surgical technique was evaluated and reported. Results Between 2011 and 2018, 17 patients (mean age, 10.2 years; range, 2-14 years) affected by large cystic ovarian mass, were eligible for this technique. All patients had negative preoperative tumor markers. Of the seventeen subjects, 13/17 girls (76%) underwent pelvic magnetic resonance imaging. No sign of lymphadenopathy or metastasis was found. Surgery was successful in all patients, with ovarian preservation in 5/17 cases (29.4%). Mean surgical time was 98 minutes; no intra-abdominal leakage of neoplasm content or postoperative complications occurred. Mature cystic teratoma was the most frequent histopathological diagnosis (71%). Conclusion After a thorough patient selection, the management of large cystic ovarian neoplasms with leak-proof extracorporeal drainage performed through a mini laparotomy is a feasible and safe approach, with excellent cosmetic results. When achievable, ovarian-sparing surgery has to be considered.

Introduction: The management of primary spontaneous pneumothorax (PSP) in children still remains controversial. The current literature shows a lack of scientific evidence supporting a standardized management. Materials and Methods: A multicenter retrospective study was performed, patients admitted for PSP in five Pediatric Surgical Units over a 9-year period (from 2008 to 2017) were included. Patient data were reviewed for each case. Management and outcomes were statistically analyzed. Results: Overall 159 patients (135 males and 24 females) were included in this study. During the first hospital admission, 62/159 patients (39%) were conservatively managed with oxygen therapy; 95/159 patients (60%) required a chest drain insertion; 2/159 patients (1%) underwent surgery because of a bilateral pneumothorax. Thoracoscopy was performed in 75/159 (47%) patients after the second hospital admission or for persistent air leak during the first access. Postoperatively, 8/75 (11%) patients developed recurrent pneumothorax requiring chest drain insertion in 3/8 (37%) cases or aspiration in 1/8 (13%), whereas 4/8 (50%) patients were conservatively managed. All of the patients but one, were confirmed to be affected by emphysematous-like changes at histology. Conclusions: Despite the limitations of being a retrospective study, we suggest that the early surgical management in children with PSP is feasible and safe and it seems to significantly reduce the recurrence rate. To the best of our knowledge there are no pediatric guidelines for the management of PSP, therefore, we support the need for prospective studies to create the evidence-based pillars for correct and standardized management of this condition.

Minimally invasive surgery (MIS) is now common in the paediatric age group, again highlighting the relevance of ‘clean’ retrieval of surgical specimens. Since disposable tissue bags can be expensive, we have developed a low-cost method for specimen retrieval using a modified finger from a surgical glove. We have used this technique mostly for laparoscopic appendectomy. There was no spillage of contents or surrounding tissue contamination and all wounds healed well without any infection from exit wound. There was no break in the glove or dispersion of glove fragments within the peritoneum. We believe that the Reverse Glove-finger Technique is a safe, quick, easy and low-cost technique that is freely available to all, for retrieval of small specimens during MIS procedures.

AIM The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intrahepatic bile duct cilia (IHBC) in BA at diagnosis and its correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). METHODS Surgical liver biopsies taken at diagnosis from 22 BA infants (age range, 39-116 days) and from eight children with non-BA chronic cholestasis (age range, 162 days -16.8 years) were evaluated for IHBC by immunofluorescence (IF) and SEM. A minimum 18-month follow-up after surgery was available for all patients. RESULTS By IF, cilia were present in 6/8 (75%) non-BA but only in 3/22 (14%) BA cases, and cilia were reduced or absent in 19/22 (86%) BA and 2/8 (25%) non-BA livers (P 90% at 12 months, and >70% at 24 months post-surgery, were recorded regardless of cilia presence/absence at diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers. CONCLUSIONS The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis, although was not predictive of short-term survival with native liver. Scanning electron microscopy represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and/or disease progression warrants further investigation.

Introduction: The aim of our study was to present our experience with the use of peripherally inserted central catheters (PICCs) in pediatric patients receiving autologous or allogenic blood stem-cell transplantation. The insertion of the device in older children does not require general anesthesia and does not require a surgical procedure. Methods: From January 2014 to January 2017, 13 PICCs were inserted as a central venous device in 11 pediatric patients submitted to 14 autologous or allogeneic stem-cell transplantation, at the Bone Marrow Transplant Unit of the Children’s Hospital of Brescia. The mean age of patients at the time of the procedure was 11.3 years (range 3-18 years). PICCs remained in place for an overall period of 4104 days. All PICCs were positioned by the same specifically trained physician and utilized by nurses of our stem-cell transplant unit. Results: No insertion-related complications were observed. Late complications were catheter ruptures and line occlusions (1.2 per 1000 PICC days). No rupture or occlusion required removal of the device. No catheter-related venous thrombosis, catheter-related bloodstream infection (CRBSI), accidental removal or permanent lumen occlusion were observed. Indications for catheter removal were completion of therapy (8 patients) and death (2 patients). Three PICCs are currently being used for blood sampling in follow-up patients after transplantation. Conclusions: Our data suggest that PICCs are a safe and effective alternative to conventional central venous catheters even in pediatric patients with high risk of infectious and hemorrhagic complications such as patients receiving stem-cell transplantation.

Highlights • Hypothermic cardiopolmunary bypass is safe for prolonged total vascular exclusion. • Ante situm liver resection is feasible for hepatoblastoma considered unresectable. • Inferior vena cava replacement with aortic graft from cadaveric donor is feasible. • Preoperative and intraoperative assessment are essential to achieve good outcome., Introduction Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear. Presentation of case A 11-months-old boy was referred for a right hepatic lobe mass(90 × 78 mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795 IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61 × 64 mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft. Conclusions We report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.

Omphalomesenteric duct and urachus are two transitory structures, normally encountered during the embryonic development. The first usually disappears by the fifth to seventh week of gestation, while the urachus progressively narrows and is obliterated by fibrous proliferation during the fetal life. Remnants of these embryonic structures account for a wide variety of abnormalities that may require surgical correction.

BACKGROUND Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. METHODS A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (

Bowel dilatation is a common adaptive mechanism after intestinal resection. The symptomatic dilated dysmotile duodenum is difficult to manage, since conventional bowel tailoring and lengthening techniques are potentially hazardous because of the anatomy of the duodenal blood supply, the proximity to the pancreas, and the risk of injury to the common bile duct. A 2-month-old child with short bowel and a symptomatic massively dilated duodenum was treated with a Transverse Flap Duodenoplasty (TFD). The duodenum was opened longitudinally along its antimesenteric border preserving an intact strip of tissue overlying the pancreatic head. Three full thickness vascularized pedicle flaps were cut on both the anterior and posterior walls and were spirally rotated and sutured to create a uniform propulsive duodenum without diverticulae. Healing was complicated by a soft anastomotic duodeno-ileal stenosis that resolved after three elective balloon dilatations. Oral feeding established rapidly. The child is growing, does not vomit, and passes 1–2 semiformed motions daily. TFD is a safe and versatile technique that preserves all duodenal absorptive mucosa and that removes any risk to the pancreas, bile duct, and ampulla of Vater. Our experience, although limited, has been encouraging and leads us to suggest TFD for the management of the difficult symptomatic dysmotile dilated duodenum.

The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intrahepatic bile duct cilia (IHBC) in BA at diagnosis and its correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM).Surgical liver biopsies taken at diagnosis from 22 BA infants (age range, 39-116 days) and from eight children with non-BA chronic cholestasis (age range, 162 days -16.8 years) were evaluated for IHBC by immunofluorescence (IF) and SEM. A minimum 18-month follow-up after surgery was available for all patients.By IF, cilia were present in 6/8 (75%) non-BA but only in 3/22 (14%) BA cases, and cilia were reduced or absent in 19/22 (86%) BA and 2/8 (25%) non-BA livers (P 0.01). In BA, cilia presence was found to be associated with clearance of jaundice at 6-month follow-up (P 0.05). However, high overall survival rates with native liver,90% at 12 months, and70% at 24 months post-surgery, were recorded regardless of cilia presence/absence at diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers.The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis, although was not predictive of short-term survival with native liver. Scanning electron microscopy represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and/or disease progression warrants further investigation.

Wandering spleen is a condition in which an incomplete fusion of the splenic ligaments allows the spleen to move within the abdomen, predisposing it to splenic torsion along its vascular pedicle. Torsion of a wandering spleen is an uncommon occurrence, especially in children, and associated torsion of the distal pancreas is even more unusual, with only four cases having been reported in adults. Non-specific clinical presentation makes radiologic evaluation essential in order to obtain a diagnosis and to send the patient for early surgery before life-threatening complications arise. Here we present a rare case of torsion of wandering spleen together with volvulus of the distal pancreas in a 13-year-old girl. In this case, prompt radiological assessment allowed for an early diagnosis, and the patient was successfully treated with urgent laparoscopic derotation of both the spleen and the distal pancreas as well as mesh splenopexy. To the best of our knowledge, this procedure has never been described in a pediatric setting.

Background and aims Duodenal atresia is a relatively common anomaly and its frequency ranges from 1 in 3000 to 5000 live births. Association of duodenal atresia with situs viscerum inversus is rare and fewer than 20 patients have been reported in literature. Methods We present a sub-Saharan 1 years old female baby, born at 31 weeks with prenatal diagnosis of situs viscerum inversus with dextrocardia, ventricular septal defect, ductus venous agenesis, duodenal atresia and intestinal malrotation. We considered risk factors, clinical and surgical management, complications and evolution of this rare association. Results A 31 weeks female baby was born by emergency caesarean section for cardiotocographic changes. Her mother history was positive for pre-gestational diabetes on insulin therapy and for Streptococcus beta-haemolytic infection. Incomplete intrapartum amplicillin prophylaxis was made. The baby weighed 2300 g and her Apgar scores were 5 and 5, needing non-invasive ventilation with nCPAP. Because of suspected intestinal obstruction, parental nutrition was started one day after birth and a radiological examination was performed showing the mirror image of a double-bubble configuration of duodenal atresia. She underwent laparotomy at 6 days of life, confirming situs inversus and intestinal necrosis secondary to volvulus on common mesentery (apple peel type 3b): duodeno-cecal anastomosis with saving the ileocecal valve was performed. Histology of the surgical specimen confirmed the transmural necrosis. Six days after, enteral nutrition was progressively introduced with good tolerance and weight gain; parental nutrition was continued with subsequent cholestasis needing the modification of the lipid composition. At 3 months of age, she underwent a Bianchi intestinal lengthening procedure and cholecystectomy. The onset of post-surgical duodenal stenosis two months after required one endoscopic pneumatic dilation (8–10 mm) and a gastrostomy button was placed too. Upper endoscopies performed 1 month and 6 months later were normal. Enteral and parental nutrition was continued and the oral feeding was gradually introduced. Conclusions Duodenal atresia and situs inversus are rare combinations. Outcome is good with appropriate multidisciplinary management and follow-up. Maternal diabetes could be factor risk of laterality, as suggested in literature.

A 40-day-old infant presented with pallor and poor food intake, and was found to have normochromic normocytic anaemia and mild neutrophil leucocytosis. As late-onset sepsis was suspected, antibiotic therapy was started, with clinical improvement within 7 days, but haemoglobin levels remained below 8 g/dL. There was no evidence of bleeding or haemolysis, and no response to iron supplementation and a 4-week cow's milk protein elimination diet, and she was referred to our centre at 2.5 months of age. Abdominal MRI showed a vast pseudonodular mass involving and …

Fig 1. Abdominal radiograph showing massive dilatation of the whole colon, without signs of intestinal perforation. A 10-YEAR-OLD GIRL was evaluated at the Emergency Department because of a 2-day history of abdominal distension with pain and nonbilious vomiting. She had a 5-year history of chronic constipation, previously diagnosed as functional constipation. On physical examination, her abdomen was massively distended with voluntary guarding. Laboratory evaluation showed an elevated C-reactive protein (24 mg/dL, normal range 0–5 mg/dL). Abdominal radiograph showed a massively dilated colon (Fig 1). A nasogastric tube was placed, and a water-soluble contrast enema was obtained (Fig 2). The enema showed the “twisted taper” or “bird’s beak” configuration of the twisted colon, in which the dilated proximal segment represents the bird’s head, and the tapered distal end its beak. These findings were consistent with the diagnosis of sigmoid volvulus. After an unsuccessful attempt at endoscopic decompression and derotation, the patient underwent an emergency exploratory operation. At laparotomy, 1808 counterclockwise torsion of the sigmoid colon was confirmed. The dilated sigma had a maximal diameter of 17 cm, without sign of ischemia or necrosis. Colonic irrigation with saline solution was performed, and the patient underwent derotation and resection of 25 cm of dilated sigma and primary end-to-end colocolonic anastomosis with sigmoidopexy. Sigmoid volvulus is extremely rare in children and is usually associated with a long-standing history of constipation or pseudo-obstruction. In these patients, redundant sigmoid colon could

none 34 no 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. open Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; D’antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dall’oglio, Luigi; Spada, Marco; Grandi, Francesca Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; Dâ antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dallâ oglio, Luigi; Spada, Marco; Grandi, Francesca

Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. In recent years, because of the advances in prenatal diagnosis, neonatal critical care, and surgical procedures, overall outcomes have improved substantially, including for premature children. Nowadays, most of the research is focused on medium- and long-term morbidity, with particular reference to respiratory and gastroesophageal problems; the high frequency of late sequelae in esophageal atresia warrants regular and multidisciplinary checkups throughout adulthood. Surprisingly, there are few studies on the impact of prenatal diagnosis and there is continuing debate over the prenatal and preoperative management of these complex patients. In this review, we analyze the literature surrounding current knowledge on the management of newborns affected by esophageal atresia, focusing on prenatal management and preoperative assessment.

We report a child with post-surgical short bowel state who underwent bowel expansion followed by spiral intestinal lengthening and tailoring (SILT) at 10 months of age. Growth at 1-year follow-up is along the 15-25th centile on 82 % oral calories as normal diet and 18 % as parenteral nutrition, and he is passing 2-3 semisolid motions daily. SILT is a versatile technique for reconstructing dilated bowel towards improved propulsion and absorption, and has a role in the management of the short bowel state.