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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.

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.

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.

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.

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.

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.

To investigate the diagnostic and therapeutic assessment in children with adenomyomatosis of the gallbladder (AMG).AMG is a degenerative disease characterized by a proliferation of the mucosal epithelium which deeply invaginates and extends into the thickened muscular layer of the gallbladder, causing intramural diverticula. Although AMG is found in up to 5% of cholecystectomy specimens in adult populations, this condition in childhood is extremely uncommon. Authors provide a detailed systematic review of the pediatric literature according to PRISMA guidelines, focusing on diagnostic and therapeutic assessment. An additional case of AMG is also presented.Five studies were finally enclosed, encompassing 5 children with AMG. Analysis was extended to our additional 11-year-old patient, who presented diffuse AMG and pancreatic acinar metaplasia of the gallbladder mucosa and was successfully managed with laparoscopic cholecystectomy. Mean age at presentation was 7.2 years. Unspecific abdominal pain was the commonest symptom. Abdominal ultrasound was performed on all patients, with a diagnostic accuracy of 100%. Five patients underwent cholecystectomy, and at follow-up were asymptomatic. In the remaining patient, completely asymptomatic at diagnosis, a conservative approach with monthly monitoring via ultrasonography was undertaken.Considering the remote but possible degeneration leading to cancer and the feasibility of laparoscopic cholecystectomy even in small children, evidence suggests that elective laparoscopic cholecystectomy represent the treatment of choice. Pre-operative evaluation of the extrahepatic biliary tree anatomy with cholangio-MRI is strongly recommended.

Aim of the study The management of newborns with esophageal atresia (EA) and right aortic arch (RAA) is still an unsolved problem. This study provides a systematic review of epidemiology, diagnosis, management and short-term results of children with EA and RAA. Materials and methods The PubMed database was searched for original studies on children with EA and RAA. In each study, data were extracted for the following outcomes: number of patients, associated anomalies, type of surgical repair, morbidity and mortality rate. Results Eight studies were selected, including 54 patients with EA and RAA. RAA was encountered in 3.6% of infants. Preoperative detection of RAA was reported in 7 of them. In these patients, primary anastomosis was achieved through the right approach in 3 (thoracotomy in 2 and thoracoscopy in 1) while the left approach was the primary choice in 4 (thoracotomy in 2 and thoracoscopy in 2). No significant differences were found between the right and left approaches with regard to leaks (P = 0.89), strictures (P = 1) or mortality (P = 1). In 47/54 patients (87%) RAA was noted during right thoracotomy, and primary anastomosis was achieved through the same approach in 29 (61.7%); conversion to other approaches (left thoracotomy or esophageal substitution) was performed in 15 children (38.3%). No significant differences were found between primary left thoracotomy (LT) and LT after RT with regard to leaks (P = 0.89), strictures (P = 1) or mortality (P = 1). Conclusions Skills and preferences of the surgeon still guide the choice of surgical approach even when preoperatively faced with RAA. A multicenter, prospective randomized study is strongly required.

To study that inflammatory fibroid polyps (IFPs) in children are extremely uncommon tumors that may occur throughout the gastrointestinal tract.A systematic review of the pediatric literature and a report of a new case of IFP is also presented. The PubMed database was searched for original studies on pediatric IFPs since 1960, according to "Preferred reporting items for systematic reviews and meta-analyses" guidelines for systematic reviews.Five studies were finally enclosed, encompassing 6 children with IFPs (mean age 64 mo). Tumors were located in the stomach (2 patients), in the small bowel (2 patients), in the rectum (1 patient) and in the colon (1 patient). Open surgery was performed in all patients and complete excision of the mass was achieved in all cases. All patients are alive and free of symptom. Authors described a further case of a 3-year-old boy with a large duodenal IFP, in whom the tumor was removed by "en block resection". The presence of IFP throughout the gastrointestinal tract and its variable clinical appearances make it difficult to diagnose. An accurate pre-operative assessment is fundamental in order to differentiate IFP from other more aggressive gastrointestinal tumor, enabling unnecessary demolitive surgery.When complete resection of the IFP is achieved, the prognosis is excellent.

Sigmoid volvulus (SV) is extremely uncommon in children and is usually associated with a long-standing history of constipation or pseudo-obstruction. An early diagnosis and management are crucial in order to prevent the appearance of hemorrhagic infarction of the twisted loop, avoiding further complications such as necrosis, perforation and sepsis. In patients with no evidence of peritonitis or ischemic bowel, treatment starts with resuscitation and detorsion of the SV, accomplished by means of sigmoidoscopy and concomitant rectal tube placement. The bowel is then prepared and surgery is undertaken electively during the same hospitalization. We report a detailed review of the literature focusing on technical details, risks and benefits of endoscopic management of SV in childhood.