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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.

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.

The aim of this study was to evaluate the diagnostic accuracy of postnatal multidetector computed tomography (MDCT) compared with prenatal ultrasound (US), surgical findings, and histology, in 33 patients with congenital cystic lung disease.Thirty-three patients, 17 males and 16 females, were evaluated by MDCT. Twenty-seven of these patients underwent prenatal US between week 18 and 22, and between week 32 and 35 of gestation. Lung lobectomy, segmentectomy, atypical resection, lesion resection were performed in 31 patients and surgical specimens were analysed.Prenatal US and MDCT correctly diagnosed 76.9 and 94 % of the lesions, respectively. Disagreement occurred in six lesions with prenatal US and in two lesions with MDCT. No statistically significant differences were observed between the two techniques (P = 0.122).As most surgeons consider the surgical resection of these lesions mandatory, our study underscores the essential role of imaging, in particular CT, in providing invaluable preoperative information on congenital cystic lung diseases recognised in uterus.

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.

Esophageal atresia is a relatively common congenital malformation occurring in 1:3000-4500 live births. Improvement in surgical, anesthetic and neonatal care has achieved a survival rate near 100% in infants weighing over 1500 g and having no major cardiac problem. To achieve these outcomes, as well as a good surgical technique, careful preoperative management (early diagnosis, investigation of associated anomalies, suction of the upper pouch, prevention and treatment of gastric and bowel distension) and accurate postoperative care (postoperative analgesia and ventilation, management of trans-anastomotic and chest tube, prevention, early recognition and treatment of complications) are mandatory.

Preoperative tracheobronchoscopy (TBS) in the diagnostic assessment of newborns affected by esophageal atresia (EA) was described in 1981. Nevertheless, the value of the procedure is actually much debated; only a few studies have clearly explored the advantages of TBS and this procedure is not yet routinely included in the diagnostic and therapeutic assessment in many international pediatric surgery settings. Routine preoperative TBS is a safe procedure that enables the accurate examination of the tracheobronchial tree, the visualization of tracheoesophageal fistula and the diagnosis of tracheomalacia or associated respiratory anomalies. When a distal fistula is found, its occlusion with a Fogarty balloon catheter improves mechanical ventilation and facilitates surgical repair. This review provides a detailed overview on the use of TBS in newborns with EA, focusing on technical aspects, anesthesiological management, indications and limits. The benefits and risks of the procedure are also compared with alternative diagnostic tools, such as an esophageal contrast study, computed tomography scan and ultrasound.

Background Wandering spleen and splenic cyst are rare benign congenital conditions that can both cause severe complications related to torsion or trauma. Case Report A 14-year-old girl presented a mobile 10-cm-long abdominal mass in the left lower quadrant associated with mild abdominal pain. The diagnosis of an 8-cm-long nonparasitic cyst in a wandering spleen was confirmed by computerized tomography and negative serum indirect hemagglutination titer for hydatid disease. Laparoscopic unroofing of the cyst and splenopexy in a vycril mesh was performed. Results No problems were encountered during laparoscopic surgery. Postoperative course was uneventful, and at a 1-year follow-up, the spleen is viable and maintains a normal position in the phrenorenal angle. Conclusions In the child, treatment of wandering spleen associated with a cyst should aim at the prevention of vascular accidents and at conservation of the spleen. We achieved these goals with unroofing and splenopexy through laparoscopic surgery.

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.

Total parenteral nutrition is an important adjunct in the care of neonates with surgical disorders. Cholestasis is at present the most worrisome complication of this technique; it is difficult to treat and may progress to eventual cirrhosis and liver failure. This article reviews the pertinent clinical and nutritional data in a surgical patient with short bowel syndrome who developed parenteral nutrition-associated liver disease successfully treated with fish-oil based lipids.

Nowadays short bowel syndrome (SBS) is quite frequent, because of more aggressive surgical and medical approaches to the management of neonatal intra-addominal catastrophes. Intestinal rehabilitation can be reached in case of SBS with a strategy that merges nutritional, pharmacologic and surgical approaches to achieve the ultimate goal of enteral nutrition. Long-term clinical nutrition which combines total parenteral nutrition (TPN) and enteral nutrition is required for the adaptation process. Long-term TPN can, however, be associated with mechanical, septic and metabolic complications, most of which have been consistently reduced by a better understanding of the prerequisites for its application and by improvements in parenteral solutions. Parenteral nutrition associated cholestasis (PNAC) and liver disease (PNALD) remain indeed the most worrisome complications and bear with them a high mortality rate. Their prevention will further improve the role of TPN in patients with SBS. The etiology of PNAC and PNALD, although elusive, is thought to be multifactorial and proposed theories also include problems arising from lipid emulsions. Parenteral nutrition, that includes n-3 fatty acids, appear to diminish the extent of the inflammatory response thought to be responsible for PNAC and PNALD. This article will attempt to review the role of TPN in the rehabilitation process and discuss energy and macronutrients requirements.