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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

Introduction: Appendicitis is the commonest and most frequently misdiagnosed acute abdominal surgical illness in the paediatric population worldwide. The aim of this study is to evaluate the role of coagulation profile in acute appendicitis (AA) in children. Materials and Methods: we retrospectively collected data of patients submitted to appendectomy from 2011 to 2017. According to histopathology, patients were divided into three groups: not histologically confirmed AA (NAA), simple AA (SAA) and complicated AA (CAA). White blood cell (WBC) count, relative neutrophilia (Neutr%), C-reactive protein (CRP), prothrombin time ratio (PTratio), activated partial thromboplastin time ratio (APTTratio) and fibrinogen (Fib) were compared among groups. Results: Three hundred and seven patients were included: 57 NAA, 184 SAA and 66 CAA. WBC was significantly different among groups: CAA (mean 16.67 × 103/ml), SAA (14.73 × 103/ml, P = 0.01) and NAA (10.85 × 103/ml, P < 0.0001). Significant differences were found for Neutr% (mean CAA 81.14 vs. SAA 77.03 P = 0.006, vs. NAA 63.86 P < 0.0001) and CRP (mean NAA 2.56, SAA 3.26, CAA 11.58, P < 0.0001). PTratio and Fib increased with the severity of AA receiver operator characteristic curves were similar for CRP (0.739), Fib (0.726), WBC (0.746) and Neutr% (0.754), while for PTratio and aPTTratio were 0.634 and 0.441, respectively. Conclusions: extrinsic coagulation pathway is altered in AA, especially in CAA. Coagulation can be useful in the diagnostic and perioperative anaesthetic management of AA in children. Fib seems to have the highest accuracy.

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.

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.

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.

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.

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.

This chapter is based upon an invited lecture for the Symposium of Neonatal Surgery on 24th–25th October 2016 in Bologna, Italy. Biliary atresia is a “congenital” disorder of the biliary tree characterized by progressive fibrosing obstruction and presenting as conjugated jaundice and pale stools in the first weeks of life. The true aetiology is largely unknown, although embryological and infective hypotheses have been suggested. Treatment is entirely surgical being an attempt to restore bile flow and salvage of the native liver in the first instance (Kasai portoenterostomy) reserving liver transplantation for those where this approach is unsuccessful.

Aim of study CMV-IgM + ve associated biliary atresia (CMV-BA) is a distinct etiological subgroup characterized by older age at presentation and a greater degree of inflammation and hepatic fibrosis, leading to a worse outcome. We report our experience with adjuvant antiviral therapy after Kasai portoenterostomy (KPE). Methods Single-center prospective database identification of CMV-IgM + ve associated BA managed between 2003 and 2017. Since 2011, IV ganciclovir (5 mg/kg b.d.) and/or oral valganciclovir (520 mg/m2 b.d.) were started in the early postoperative period in selected cases and continued until negativity of CMV DNA load [Anti-Viral Therapy (AVT) Group 1]. Clearance of jaundice was defined as achieving a total bilirubin ≤ 20 μmol/L in post-KPE period and tested with a Fisher test; native liver survival (NLS) and overall actuarial survival (OS) were compared with untreated BA CMV IgM + ve patients (Group 2) using a Log-Rank test. A P value of Results During the 14-year period, 376 infants with histologically confirmed BA were treated; of those 38(10%) were CMV IgM + ve at presentation. One child was considered too late at presentation for KPE and underwent primary liver transplantation while another only started AVT one month after KPE. Both were excluded from survival analysis. Therefore 36 underwent KPE [AVT Group 1 (n = 8) and Control Group 2 (n = 28)]. Overall age at surgery was 67(53–77) days. There was no difference in age at surgery (P = 0.26); bilirubin (P = 0.12); or AST (P = 0.15) between Group 1 and Group 2. Viral load data were available in 16 with a trend towards higher counts in the AVT group 1 [4935 (2668–18,817) vs. 1296 (253–10,471) c/ml; P = 0.06]. Clearance of jaundice was higher in AVT Group 1 (75% vs 21%, P = 0.009). There was no difference in OS (P = 0.24) but NLS was improved in the AVT Group 1 (75% vs. 25% at 2 years; P = 0.04). Conclusions Although this finding may be regarded as preliminary, adjuvant antiviral therapy appeared to improve outcome in infants with CMV IgM + ve BA. Level of evidence III.

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.

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.

Choledochal malformation (CM) may be defined as morphological abnormality of the biliary tract characterised by dilatation in the absence of acute mechanical obstruction. Bile duct maljunction with the incoming pancreatic duct and formation of a long common channel is a recognised element of CM. This allows free intermixing of pancreatic juice and bile, although the how dynamic this relationship is remains unknown. We have established that there is an inverse relationship of choledochal pressure and bile amylase (as a surrogate of reflux) and that the key component of damage to the biliary epithelium is pressure generated.

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 original version of this article unfortunately contained a mistake. Arnaldo Caruso was not listed among the authors.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

To identify risk factors that can predict prevalence of anastomotic strictures (AS) following esophageal atresia (EA) repair.Of 46 consecutive patients with EA managed at our institution between 2004 and 2012, 35 underwent esophageal anastomosis and were included in this retrospective longitudinal study. Routine endoscopy was performed 1 month after surgical repair. According to stricture index (SI), endoscopically calculated as SI = (D - d)/D, where D is the diameter of the esophageal pouch and d the stricture diameter, population was divided into Group 1, SI ≤ 0.1 (no evidence of stricture); Group 2, 0.3 SI 0.1 (mild stricture); Group 3, SI ≥ 0.3 (high-grade stricture). Trends of subsequent endoscopic esophageal dilatations were compared between the groups using Wilcoxon-Mann-Whitney or Pearson's tests. Cox regression analysis was performed to estimate the hazard ratio.Gastro-esophageal reflux disease (P = 0.04), tension on the anastomosis (P = 0.02) and long-gap form (P = 0.008) have an increased risk of developing AS. SI at 1 month after surgery correlates with the average number of future dilatations: Group 2 and 3 compared to Group 1 required more dilatations (hazard ratio 2.291 and 12.765).AS remain frequent complications of esophageal surgery, especially in specific subgroups of patients. SI at 1 month after surgery could already predict the severity of the stricture and the need for subsequent endoscopic esophageal dilatations.

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

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.

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.

Summary Objective To evaluate the functional and cosmetic result of modified-triple incision preputial plasty for surgical management of phimosis in children. Materials and methods This prospective study involved all consecutive children (age 3–15) with phimosis, managed at our Institution in a one–year period. All patients unable to retract the foreskin and symptomatic with episodes of posthitis or balanoposthitis or ballooning of the foreskin with voiding were initially included. After a two-month trial of topic corticosteroids treatment, for patients refractory to conservative ointment treatment, “trident” preputial plasty was offered. This procedure combines two lateral Z-plasty and one middle Y–V plasty. All the procedures were performed as a day care, in general anaesthesia by two senior surgeons. Outpatient follow-up controls were scheduled at 1 and 2 week, 1, 6 and 12 months after surgery, respectively. Results A total of 41 patient were enrolled. Mean operative time was 24 minutes (range 15–43). At 12-month follow-up, all but one patients (97.6%) was able to retract the prepuce. Cosmetic and functional results were satisfactory. Conclusion In selected group of children with phimosis, the “trident” preputial plasty provided excellent cosmetic and functional result. Adequate retraction of the prepuce by the patients soon after surgery is mandatory.

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Purpose Aim of this systematic review is to investigate the thoracic and cervical surgical approaches of H-type tracheo-esophageal fistula (TEF) according to the position of the fistula. Methods The PubMed database was searched for original studies on H-type TEF treatment published between 1977 and 2012. Manuscripts finally included were divided into open and thoracoscopic surgery groups. Results Seventeen studies were selected for open surgery group, and most of them agree on the importance of pre-operative diagnosis of the fistula by preliminary tracheoscopy. Right cervicotomy was used in 70 cases (76.9%), left cervicotomy in 12 (13.2%), and thoracotomy only in 9 (9.9%). Five studies were included in thoracoscopic group (6 patients). Indications for the surgical approach (cervical vs thoracic) according to the position of the TEF were clearly described in 10 manuscripts, and all stated differences in surgical technique details. Complications and mortality rates were not statistically correlated to the different surgical approaches. Conclusions The evidence base in regard to the treatment of H-type fistula in children is poor and the skills and preferences of the surgeons guide the choice of the procedure. Surgical division of the fistula is curative, and the key to a successful repair is the pre-operatively identification of the level of the fistula with tracheoscopy. Right cervicotomy seems to be the approach of choice in the majority of case, with the thoracic approach appropriate only for fistulae opening below T2. Further well-designed prospective studies which take into account of selection and performance bias are strongly required.

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.

Scrotal swelling suggesting testicular torsion is a rare urological emergency which requires a clinical urgent evaluation and most of the times must be managed surgically. In newborns it can occur in the postnatal period, usually within the twenty-eighth day of life, or more frequently in utero, during the descent of the testis into the scrotum. Usually its poor fixedness allows the testis an abnormal mobility inside the scrotum, configuring the framework of extravaginal torsion. On the contrary during the perinatal period a twist that takes place inside the tunica vaginalis, known as intravaginal torsion, is extremely uncommon and only few cases are well documented in the literature. Authors present a rare case of intravaginal testicular torsion occurred in perinatal period. In this situation only the early surgical exploration of the scrotum may allow the rescue of the gonad, although in rare cases. Timing of surgical treatment and need for contralateral testicular fixation remain controversial. However since the anatomical defect of the tunica vaginalis can be bilateral the surgical fixation even of the contralateral testis is important, now or later, in order to prevent any future torsion of this gonad. The authors also present a brief review of recent literature on the subject.

This retrospective study was performed to compare the relative incidence of esophageal atresia (EA) with proximal tracheoesophageal fistula (PTEF) at our institution with those reported in literature and to test the hypothesis that our higher relative incidence is caused by the routine use of tracheoscopy.A total of 204 children with EA were managed at our institution from 1981 to 2012. The type of EA and the diagnostic assessment were noted, and the relative incidence of PTEF was calculated. For patients managed from 1981 to 2003 (Group 1), the PTEF was diagnosed by contrast esophagogram or during surgical repair. For those born after 2004 (Group 2), the final diagnosis was made by routine rigid tracheoscopy performed preoperatively. The relative incidence of PTEF was compared between these two groups and with those reported in 15 selected published large series, encompassing 4197 patients with EA.Of 204 patients with EA, 10 had PTEF, with a relative incidence of 4.9%, statistically higher than those reported in reference group (1.14%, P0.001). The routine employ of tracheoscopy involved a higher relative incidence of PTEF (Group 2=11.11%, Group 1=3.14%, P=0.038). The age of diagnosis of PTEF was 2.8 days for children of Group 2 and 4.2 days for Group 1 (P=0.038).The presence of the proximal TEF should be always ruled out before surgery. Routine employ of rigid tracheoscopy avoids delay of the diagnosis, improves diagnostic accuracy, and involves a higher relative incidence of proximal fistul. This procedure should be recommended in children undergoing EA repair.

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.