Search

Introduction Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. Materials & Methods: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. Results A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. Conclusions MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Background: Lymphangiomas represent 5% of all benign pediatric tumors. Abdominal lymphangiomas (ALs) are extremely rare. Therapy includes surgery, sclerotherapy, or pharmacological treatment. Lapar...

Purpose: We performed a retrospective study to determine the effectiveness and feasibility of articulating linear stapler in laparoscopic total splenectomy (LTS) and laparoscopic partial splenectomy (LPS), focusing on technical laparoscopic skills that could help pediatric surgeons to avoid intra- and postoperative complications. Methods: Retrospective cohort study of children younger than 18 years who underwent laparoscopic spleen surgery between January 2008 and March 2020. Age, sex, indication for surgery, operative time (OT), intra- and postoperative complications, and postoperative length of hospital stay (LHS) were analyzed. Data from parenchymal resection and vessels sealing techniques were obtained. Results: Thirty patients, 19 LTS and 11 LPS, were included. The mean age of the patients was 10.9 years, and 16 patients were male and 14 were female. For hematologic diseases, LTS was the elective surgery, associated with cholecystectomy in 5 cases. LPS was the common procedure for splenic cysts. The stapler was used in LTS to close the hilum vessels and in LPS for parenchymal resection. No statistically significant differences in OT were observed comparing LTS and LPS. Two conversions occurred in LTS; none in LPS. The mean LHS was 6 days in both groups. No recurrence or major complications appeared in both groups at 1-12 years of follow-up. In particular for LPS, there are no relapse of cyst neither reduction in splenic function. Conclusions: This study shows the effectiveness, feasibility, and safety of mechanic stapler in splenic surgery both for hilum vessels sealing and for parenchymal resection. The use of this device can reduce risk of hemorrhagic recurrences or major surgical complications improving the safety of the operation.

Eosinophilic gastrointestinal disorders are rare in children and present with a broad spectrum of non-specific symptoms. To date, no guidelines for diagnosis, therapy and follow-up are validated. Aim of our study is to focus on eosinophilic colitis (EC), to determine a possible correlation between associated disorders, macroscopic findings and treatment/follow up. Retrospective study from 2015 to 2019 including all colonoscopies performed at our Institution. Eosinophilic colitis was defined according to the threshold identified by Collins: > 100 Eo/Hpf: right colon, > 84 Eo/Hpf transverse and left colon, > 64 Eo/Hpf sigma and rectum. We excluded colonoscopy in patients with IBD or other diseases causing hypereosinophilia (i.e., parasite infection, GVHD). Among 399 colonoscopies performed in 355 patients, we made 50 diagnosis of EC, 36 males, 14 females, median age 8.5 (3–17). Symptoms leading to endoscopy were recurrent abdominal pain (66%), chronic diarrhea (64%), and chronic constipation (8%). Two patients presented with GI bleeding and one with weight loss. Macroscopic findings were mostly normal or lymphoid nodular hypertrophy presenting different endoscopic features. In seven children (14%) we found history of allergy and atopy. 22 children present a diagnosis of autistic spectrum disorder (ASD) with a prevalence higher than in the overall population (44% vs 28.5%, p = 0.03). According to symptoms, treatment consist variably of steroids, six food elimination diet, mesalamine. For patients with available follow-up, we found histological persistence of Eosinophils in 75%, even in patients with symptoms relief. This study focus attention on EC as a new challenging pathology. Multicentric randomized clinical trials are needed to understand physiopathological mechanisms to validate a possible endoscopic score and related histological threshold, and to standardize therapy according to clinical features and instrumental findings. The high prevalence of EC in ASD need further specific research.

Background: Over the last 30 years, the incidence of pediatric urolithiasis (PU) has been increasing and the surgical management has evolved toward a minimally invasive approach (MIA). We reported the experience of two Centers of Pediatric Surgery in the management of PU, focusing on MIA as first choice in treatment. Methods: Data were retrospectively analyzed from October 2009 to October 2019 in children with urolithiasis who were admitted to two referral Italian Centers of Pediatric Surgery. Demographic and clinical data of the patients, features of the urolithiasis, type of surgery were considered. Results: Seventy patients (7.3 ± 5.0 years) with normal renal function were treated for calculi in the pyelocaliceal system (45.7%), ureter (34.3%), bladder (4.3%), urethra (1.4%), and multiple locations (14.3%). Size of calculi was >10 mm in 55.7% of cases (kidney>bladder/urethra>multiple>ureter, p = 0.01). Symptoms were present in 75.7% of patients. Family history was positive in 16.9% of cases. MIA was performed in 59 patients (84.3%): 11.8% shockwave lithotripsy (kidney>ureter>multiple); 32.2% ureteral retrograde surgery (ureteral>other localizations); 30.5% retrograde intrarenal surgery (kidney>other localizations); and 25.4% other procedures including percutaneous nephrolithotomy, cystoscopic bladder stone removal or laser cystolithotripsy (kidney>bladder>multiple). Preoperative stenting was necessary in 52.8% of cases. Four MIA procedures (6.9%, kidney>ureter/multiple) were converted to open surgery. Open surgery was required as first approach in 15.7% of patients (kidney>ureter>multiple) who needed urgent surgery or had associated congenital renal anomalies. In 18/70 of children (25.7%), with prevalence of stones in kidney and multiple location (p < 0.01), a second procedure completed the treatment (88.8% MIA). Intraoperative difficulties were recorded in 8.5% of cases, without difference between location and size of calculi. Late complications (5.7%) were related to displacement and infection of the ureteral stent. Conclusions: MIA resulted to be feasible in more than 75% of primary surgery and in more than 85% of cases requiring a second procedure. Preoperative stent was mandatory in more than 50% of children. The technological evolution allowed to overcome many of the technical difficulties related to the approach to the papilla and lower calyxes. Open surgery is reserved for selected cases and endoscopic surgery represents the best choice of treatment for PU.

Aim:Video-assisted thoracoscopic surgery (VATS) has been widely used in the last decades. Nevertheless, the pros and cons of thoracoscopy vs. open surgery in pediatric oncology are still under debate. In literature, VATS has been applied for both diagnostic and ablative surgery to treat neurogenic tumors, thymic neoplasms, lung tumors and metastases, germ cell tumors, lymphoproliferative diseases, and other rare tumors. Recent reviews described excellent outcomes in pediatric oncology as well as in the treatment of adult lung cancer, with a significantly higher rate of mortality and complication in thoracotomy compared to VATS. We reviewed our experience on thoracoscopy in pediatric malignancy and compared it to the literature.Materials and Methods:This was a retrospective cohort-study of pediatric oncological patients who underwent VATS at our institution from 2007 to 2020, and a review of the recent literature on the topic.Results:A total of 43 procedures were performed on 38 oncological patients (18 males, 20 females). Median age was years 7.72 (0.35–18.6). Diagnosis: 10 neurogenic tumors, nine hematological diseases, five metastases, four lypoblastomas, three thymic pathologies, three germ cell tumors, two pleuropneumoblastomas, two myofibroblastic tumors, one myoepithelial carcinoma, one liposarcoma, and three suspected oncological mass. In three cases, a 3D model was elaborated to better plan the surgical approach. Diagnostic biopsies were 22 (51.1%), and ablative surgeries, 21 (48.9%). One neurogenic tumor was resected with the Da Vinci Robot. Median operative time was 120 min (30–420). A drain was left in place in 20 (46.5%) for a median of 4 days. Median length of hospitalization was 5 days (1–18). One case (2.3%) was converted (intraoperative bleeding). There were three post-operative complications (7.0%): one pneumonia, one pleural effusion, and one diaphragmatic paralysis (need for plication). Results were compared to recent literature, and morbidity and conversion rate were comparable to reviewed publications.Conclusion:VATS represents a valuable tool for diagnostic and therapeutic procedures in pediatric oncology. Nonetheless, it is a challenging technique that should be performed by expert surgeons on oncological and mini-invasive surgery. Three-dimensional reconstruction can optimize the pre-operative planning and guarantee a safer and more targeted treatment. Finally, the advent of robotics-assisted surgery represents a new challenge that may further implement the advantages of VATS.

Plexiform schwannoma (PS) is a benign tumour of the peripheral nerve sheath that is typically found in the skin. Fewer than 15 cases of visceral PS have been reported to date in both adults and children. We herein discuss a series of 3 patients (2 male and 1 female) with abdominal PS, aged 10-16 years (mean age, 12 years). All the patients had an acute presentation with abdominal pain, which was associated with rectal bleeding in 1 case and with walking difficulties in 1 case. Radiological investigations included abdominal magnetic resonance imaging (MRI) and computed tomography, along with neurofibromatosis screening (cerebral MRI and dermatological evaluation). Complete removal of the mass was possible in 2 of the patients (in 1 case by laparoscopically assisted surgery). Follow-up was uneventful. Abdominal PS is a rare occurrence. Due to its possible association with neurofibromatosis, the diagnosis of PS should prompt an investigation for other manifestations of this disorder.

Background To date, few studies have been conducted in Italy on pediatric eosinophilic gastrointestinal diseases (EGIDs). Aims To assess clinical features of pediatric patients with EGIDs who are followed in a tertiary pediatric center. Methods From January 2015 to December 2019, we retrospectively enrolled patients with EGIDs, and collected clinical, endoscopic, and histological data. Results We enrolled 112 patients, 75.8% were male. Mean age was 9.3 ± 4.8 years. Diagnosis of EGIDs has increased in the last two years, with non-esophageal EGIDs more prevalent than eosinophilic esophagitis (EoE) (5.1% vs. 4.4%). Approximately 30% of patients had allergic comorbidities, which prevailed in children with EoE. Autism spectrum disorders were common in patients with non-esophageal EGIDs (p = 0.007), a statistically significant finding. In addition, esophageal atresia was associated with EoE (p = 0.04). Most EGIDs patients had normal findings or an inflammatory endoscopic phenotype. Patients with EoE were mainly treated with proton pump inhibitors (PPIs) alone or in combination with swallowed steroids. PPIs, oral steroids, and food-elimination diets were prescribed to patients with non-esophageal EGIDs. Conclusion This is the first Italian study revealing an increased frequency of EGIDs in a pediatric population. Further studies are needed to characterize patients with these emerging diseases.

Background: Long-term negative sequelae of esophageal atresia (EA) may induce poor growth and impaired nutritional status in childhood. We describe the nutritional profile and energy metabolism of children with repaired EA to identify malnutrition risk factors and optimize growth management. Methods: Twenty-one children (&gt, 4 years) were included, and anthropometric measurements, nutritional assessment, and energy metabolism were considered. The subjects were defined as undernourished if they met BMI &lt, &minus, 2 standard deviation (SD). To grade undernutrition, we defined the prevalence of underweight, stunting, and wasting (cut-off level of &lt, 2 SD). Medical records were reviewed for the type of EA and surgery and perinatal data. Results: Malnutrition was detected in 28.6% of children. Underweight was detected in 23.8% of patients (all with undernutrition p &lt, 0.01). Wasting was noted in 28.6% of patients, of these 5 children were undernourished (p &lt, 0.001) and stunting was noticed in only one patient with malnutrition (p = 0.5). Resting expenditure energy (REE) was lower in undernourished subjects compared to subjects with adequate nutritional status (p &lt, 0.001). Malnutrition was associated to: type of EA (p = 0.003, particularly type A and C), intervention including deferred anastomosis due to long-gap repair (p = 0.04) with/or without jejunostomy (p = 0.02), gastric pull-up (p = 0.04), primary anastomosis (p = 0.04), pyloromyotomy in long-gap (p &lt, 0.01), small for gestational age condition (p = 0.001). Conclusions: undernutrition risk factors, beyond the type of malformation, surgery, and perinatal factors, must be early considered to personalize nutritional programming. Energy metabolism is important to monitor the nutritional requirements. The management of nutritional issues is surely a contributory factor able to counteract the poor growth of children with EA.

Primary Obstructive Megaureter (POM) is a common cause of hydronephrosis in children with spontaneous resolution in most cases. High-Pressure Balloon Dilatation (HPBD) has been proposed as a minimally invasive procedure for POM correction in selected patients. The aim of the paper is to review our experience with HPBD in patients with POM. We performed a retrospective study in a single Centre collecting data on patients’ demographics, diagnostic modalities, surgical details, results and follow-up. In particular, the endoscopic aspect of the orifice permitted the identification of 3 patterns: adynamic ureteral segment, stenotic ureteric ring and pseudoureterocelic orifice. We performed HPBD in 30 patients over 6 years. We had 23 patients with adynamic distal ureteral segment (type 1), 4 with stenotic ring (type 2) and 3 with ureterocelic orifice (type 3). In 3 patients (10%) the guidewire did not easily pass into the ureter requiring ureteral stenting or papillotomy. Post-operative course was uneventful. Five patients (3 pseudoureterocelic) required open surgery during follow-up. HPBD for the treatment of POM is a safe and feasible procedure and it can be a definitive treatment of POM. Complications are mainly due to double J stent and none of our patients had symptoms related to vescico-ureteral reflux. The aspect of the orifice, identified during cystoscopy, seems to correlate with the efficacy of the dilatation: type 1 and 2 are associated with good and excellent results respectively; type 3 do not permit dilatation in almost all cases requiring papillotomy. HPBD can be performed in selected patients of all paediatric ages as first therapeutic line. The presence of a pseudoureterocelic orifice or long stenosis might interfere with the ureteral stenting and seems associated with worse outcomes.

To compare postnatal magnetic resonance imaging (MRI) with the reference standard computed tomography (CT) in the identification of the key features for diagnosing different types of congenital lung malformation (CLM). Respiratory-triggered T2-weighted single-shot turbo spin echo (ss-TSE), respiratory-triggered T1-weighted turbo field echo (TFE), balanced fast field echo (BFFE), and T2-weighted MultiVane sequences were performed at 1.5 T on 20 patients prospectively enrolled. Two independent radiologists examined the postnatal CT and MRI evaluating the presence of cysts, hyperinflation, solid component, abnormal arteries and/or venous drainage, and bronchocele. Diagnostic performance of MRI was calculated and the agreement between the findings was assessed using the McNemar-Bowker test. Interobserver agreement was measured with the kappa coefficient. CT reported five congenital pulmonary airway malformations (CPAMs), eight segmental bronchial atresias, five bronchopulmonary sequestrations (BPS), one congenital lobar overinflation, one bronchogenic cyst, and three hybrid lesions. MRI reported the correct diagnosis in 19/20 (95%) patients and the malformation was correctly classified in 22/23 cases (96%). MRI correctly identified all the key findings described on the CT except for the abnormal vascularization (85.7% sensitivity, 100% specificity, 100% PPV, 94.1% NPV, 95% accuracy for arterial vessels; 57.1% sensitivity, 100% specificity, 100% PPV, 84.2% NPV, 87% accuracy for venous drainage). MRI can represent an effective alternative to CT in the postnatal assessment of CLM. In order to further narrow the gap with CT, the use of contrast material and improvements in sequence design are needed to obtain detailed information on vascularization, which is essential for surgical planning. • Congenital lung malformations (CLMs) can be effectively studied by MRI avoiding radiation exposure. • Crucial features of CLM have similar appearance when comparing CT with MRI. • MRI performs very well in CLM except for aberrant vessel detection and characterization.

Purpose To evaluate clinical features at diagnosis, prognostic factors, and outcomes of malignant sacrococcygeal germ cell tumors (SC-GCTs) in patients enrolled in the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) TCG 2004 protocol. Patients and methods A prospective analysis was conducted on all consecutive patients diagnosed with malignant SC-GCTs between January 2004 and May 2017. Patients with stage I underwent surgery and subsequent surveillance, the others received pediatric cisplatinum-etoposide-bleomycin (pPEB) regimen and eventual deferred surgery. Results Of 45 patients, 35 were females. Age at diagnosis ranged from 1 day to 3.6 years (median 1.6 years); 26 were stage IV. Of 38 patients who underwent surgery, pathology revealed yolk sac tumor (YST) in 27 and teratoma + YST/embryonal carcinoma in 11, while seven patients were diagnosed based on imaging and elevated levels of alpha-fetoprotein (AFP). Of six patients approached with surgery, only one relapsed and was rescued with first-line chemotherapy. Overall, 38 out of 45 achieved complete remission, three a partial remission, and four were resistant. Ten out of 41 patients who entered remission later relapsed and nine were rescued with a second-line treatment. We observed a global failure percentage of 31% and a 5-year overall survival (OS) and event-free survival (EFS) of 95% and 69%, respectively. Conclusions Chemotherapyis generally effective in malignant SC-GCTs, even though almost one-third of our patients experienced events salvageable with second-line treatment. Most of the relapses occurred within 1 year from diagnosis. A close follow up with serial AFP level monitoring should be done for at least 2 years after diagnosis.

Monochorionic twins, resulting from a single fertilized egg giving rise to two separate embryos, are monozygotic and considered genetically identical. However, discordant phenotypes have been reported in monozygotic twins. We analyzed a retrospective cohort of 155 monochorionic pregnancies (312 twins) with major discordant structural anomalies coded by the ICD-10 system in order to describe the spectrum of anomalies, the management of the pregnancies, and the perinatal outcome. Treatment options included conservative management, selective feticide with bipolar cord coagulation, or complete termination. All survivors underwent at least 24 months of postnatal follow-up. Discordancy was complicated by twin-to-twin transfusion syndrome in eight pregnancies (5%) and by selective intrauterine growth restriction in 41 (26%). Major structural anomalies affected one system in 139 cases (90%) and multiple systems in 16 (10%). Median gestational age at diagnosis was 19.1 weeks (IQR 16.4–21.3). The most frequent single-system anomalies involved the nervous and circulatory systems. In total, 72 anomalous twins (46%) and 116 normal co-twins (74%) were delivered at a median gestational age of 34.6 weeks (IQR 31.0–36.3). Neonatal/infant death of the anomalous twin occurred in 22 cases (14%), with an overall survival rate of 32% (50/155). Surviving anomalous twins underwent major surgery in 22/50 cases (44%), four of whom (8%) now suffer from severe neurologic morbidity. This study shows that a wide spectrum of major discordant structural anomalies can be found in monochorionic pregnancies. The outcome for the anomalous twin is poor, while the survival rate for the normal co-twin was 71%, with a favorable overall prognosis.

Summary Objective To report a series of 5 patients with urothelial bladder cancer (UBC) three of them with a history of exposure to amines and only two with gross hematuria. Materials and methods After obtaining ethical and legal authorization, we performed a restrospective monocentric study. We collected information of patients with UBC over a period of 10 years. We recorded: age, sex, reason for presentation, familial history and risk factors, preoperative assessment, surgical details, histological type and grade, follow-up. Results 2 children came to our attention for hematuria and 3 for incidental bladder mass finding, at a median age of 11.8 years. We performed microscopically complete transurethral resection of the tumor (TURB). Median tumor size was 1.8 cm. No further therapy was required. All cancers belonged to NMIBC (Non-muscle-invasive Bladder Cancer) considering the 2004 WHO classification: 2 urothelial papillomas, 2 papillary tumors with low grade malignancy (PUN-LPM) and 1 papillary urothelial carcinoma of low histological grade (LG-PUC Ta, N0, M0). There was not any complications and no relapse occurred during follow-up (median 30 months). Conclusions In this study, UBCs presenting at a young age were low-grade and have not recurred in follow-up. This confirms the results of other series reported in Literature. Therefore there might be the space to perform a follow-up dedicated to children.

Introduction: Gastrointestinal infantile hemangiomas (IHs) are extremely rare and represent a diagnostic challenge, especially in newborns for the limited applicability of diagnostic approaches.We report two cases of neonates with gastrointestinal bleeding from intestinal hemangioma and a literature review of gastrointestinal IHs. Case 1: Full-term baby, asymptomatic at birth. On the seventh day of life she experienced failure to thrive, weight loss and hypotonus followed by melena and vomit. The imaging showed an intestinal vascular anomaly. We performed exploratory laparotomy and intestinal resection to remove the lesion. No complication occurred. Case 2: A 20-day old girl had melena and severe anaemia. The scintigraphic evaluation excluded gastric heterotopia. We opt for explorative surgery and we performed a laparoscopy that showed a congested and oedematous intestinal segment that was resected. Post-operative course was uneventful. Discussion: Gastrointestinal IHs may cause intractable bleeding, intussusception and intestinal perforations with bad prognosis. A prompt and accurate diagnosis is vital but not always easy. Colour-coded Duplex Scan are a good first-line imaging, MRI and CT give a better definition but the latter exposes to radiation. In the “era” of minimally invasive surgery, laparoscopy can have a role in the management of patients with gastrointestinal IHs. Conclusions: Gastrointestinal IHs should be considered in the differential diagnosis of neonates with anemia and gastro-intestinal bleeding. In selected cases, the minimally invasive approach permits diagnosis and treatment. Keywords: Gastrointestinal hemangioma, Neonates, Laparoscopy, Vascular anomalies, Gastrointestinal bleeding

Background/purpose Currarino syndrome (CS) phenotype, initially described as the triad of hemisacrum, anorectal malformation (ARM) and presacral mass, can be extremely variable. The triad is often incomplete and 3 main CS phenotypical subtypes have been described: Complete, Mild and Minimal. Various associated malformations are often present. Mutations in the MNX1 gene are the main genetic background of CS, although they are not present in almost half of the cases. Aim of our study is to analyze the distribution of the 3 CS subtypes and the incidence of associated malformations in a large sample of patients and to add information about the role of the genetic testing in guiding the diagnostic and prognostic evaluation of CS patients. Methods A multicentre retrospective data collection was performed. CS patients' phenotype was accurately analyzed according to a diagnostic-therapeutic standardized data collection sheet. The distribution of the three CS types and the frequency of each associated malformation were calculated. The phenotype of the patients with a known genetic anomaly was compared to the phenotype of the population with no genetic diagnosis, in order to determine whether the presence of a known genetic defect could correlate with a more severe CS phenotype. Results Data from 45 patients were analyzed. Twenty patients (44.5%) presented a Complete CS type, 19 (42.2%) a Mild CS and 6 (13.3%) a Minimal CS. In addition to the classical triad elements, 38 (84.5%) patients showed associated anomalies. The group of patients who resulted positive for a MNX1 mutation comprised a higher number (56.5%) of Complete CS cases than the group of patients that did not carry any MNX1 mutation (13%) ( p = 0.0085). We could not find any relationship between CS subtype and the number of associated anomalies ( p = 0.5102). Conclusions The presence of a MNX1 mutation seems to correlate with a more severe CS phenotype. MNX1 seems the main responsible for the expression and the severity of the CS triad, while the associated anomalies appear to be prevalently determined by genes sited on different loci. A thorough multidisciplinary diagnostic overview of CS patients should always include genetic counseling and analysis, both in postnatal and prenatal settings. Type of study Retrospective Study. Level of evidence II.

Introduction The clinical manifestation of a stretched low-lying cone (LLC) is represented by the tethered cord syndrome (TCS) with cutaneous, urologic, neurologic, and orthopaedic dysfunctions. TC is frequently found in patients with anorectal malformations (ARMs). The aim of our article is to report a series of patients affected by LLC and ARMs and evaluate their management and long-term follow-up results. Materials and Methods We performed a retrospective analysis over a period of 15 years including patients with ARM and TC and excluding those with severe polymalformations. We collected information related to the types of malformations and demographic data. We analyzed their management (perinatal protocol, radiological investigations, surgery, and multidisciplinary follow-up). QOL was assessed through the International Classification of Functioning, Disability, and Health for Children and Youth (ICF-CY). Results Thirty-three patients among 210 ARM cases had TC (16%). Eleven of them underwent neurosurgery. One patient had retethering; out of 11 patients, 4 remained stable and 6 improved after surgery (UDS normalization and resolution of symptoms). At a mean follow-up of 10 years, four patients were on clean intermittent catheterization and five on the Peristeen transanal irrigation. The majority of patients were defined as 3 (from 1-bad to 5-excellent) for their physical and mental state. Conclusion The use of MRI is considered to complete the ARM screening in detecting TC. The multidisciplinary approach is crucial and helps in defining the management of patients. In fact, it is not clear how the features of ARM and TC affect each other. The selection of cases for surgery should take into account the critical elements.

Introduction: Situs ambiguous (SA) is a rare clinical entity that includes a spectrum of abnormalities in which organs and vessels are opposed to their normal arrangement. SA is frequently associated with other malformations such as splenic alterations, biliary atresia and vascular abnormalities. We report two cases of patients with SA, polysplenia and choledochal cyst. This association has never been described to date. Case reports: Two patients, both males, came to our attention at the ages of 7 and 8 months after ultrasound identification of choledochal cysts. One patient, who had a prenatal suspicion of situs ambiguous and dextrocardia, presented with failure to thrive and increased hepatic enzymes. The other patient was asymptomatic, but prenatal evaluation showed gastric malposition. Post-natal laboratory tests were normal. Preoperative radiological diagnostic work-up showed polysplenia, intestinal anomaly of rotation and fixation, pre-duodenal portal vein in one baby. Both patients underwent surgical correction by cyst excision and Roux-en-Y hepaticojejunostomy. Follow-up (3 months and 3.5 years respectively) was uneventful. Biochemical hepatic parameters normalized soon after surgery. Discussion: Situs anomalies in children are often associated with other malformations. The most common are splenic and cardiac defects but intestinal malformations and hepatico-biliary abnormalities have been reported too. Above all, the BASM (biliary atresia splenic malformation) syndrome has been extensively examined, obtaining important information on the etiology of the cystic form of biliary atresia. Conclusions: A careful evaluation of patients with situs ambiguous is important to exclude associated anomalies and to plan the most appropriate surgical approach. The association with choledochal cyst should be taken into account. Keywords: Choledochal cyst, Situs ambiguous, Polysplenia

Aim of the study: We would like to report an unusual case of mature cystic teratoma (MCT) of the left colon presenting with intestinal obstruction in a syndromic patient with low-anorectal malformation (ARM). Case report: The boy had multiple malformations: low ARM with perineal fistula, situs ambiguous, dextrocardia, double outlet right ventricle with pulmonary stenosis and anterior aorta.At two years he required cardiac surgery but he developed intestinal obstruction. The intestinal condition put us in a diagnostic challenge: we thought about intestinal stenosis with fecal impaction but radiological investigations were inconclusive. During surgery we identified an abdominal mass that was resected together with the adjacent descending colon. Histology showed MCT. Follow-up was uneventful. Conclusions: MCT rarely occurs outside the sacrococcygeal–gonadal location. They are frequently associated with ARM in Currarino Syndrome: the mass is located in front of the sacrum and causes anal stenosis. Nevertheless, in the reported patient, MCT did not arise from the spine but from the left colon.Paediatric cases of colonic MCT have never been reported. Affected adults can develop constipation and/or intestinal obstruction. Surgery permits to remove the mass in both symptomatic and asymptomatic patients. Keywords: Mature cystic teratoma, Dermoid cyst, Anorectal malformation, Intestinal obstruction, Paediatric surgery

Primary myoepithelial carcinoma of the lung (PMC-L) arising from the bronchial glands in lower respiratory tract is exceedingly rare. Thus far, few cases in adults and only one in a pediatric patient have been recorded. To our knowledge, this is the first report of PMC-L successfully removed in a child, focusing on the importance of multidisciplinary primary surgery for the treatment of this tumor. A 7-year-old girl was admitted for persistent cough and fever; she was unresponsive to oral antibiotics. Chest radiography showed loss of volume of left lung sustained by almost total atelectasis. After routine clinical investigations, she was referred for computed tomography scan and magnetic resonance imaging that documented the presence of a mass occupying the entire left upper lobe, infiltrating the pulmonary hilum (main bronchus, pulmonary artery, superior pulmonary vein, and pericardium). After multidisciplinary evaluation, the histopathologic diagnosis of PMC-L was established using ultrasonography-guided transthoracic core needle biopsy and bronchoscopic biopsies. She was then subjected to left pneumonectomy under extracorporeal circulation and positioning of a thoracic expander filled with 200 mL of saline solution. The postoperative course was uneventful. With TREP (very Rare Tumor in Pediatric Age) consent radiotherapy was performed (61.2 Gy). At the 10-month follow-up, the patient was alive, breathing normally without any oxygen support, without recurrence of PMC-L or metastasis, and without any chest deformity. To our knowledge, this is the first case where a pediatric patient was successfully operated for PMC-L involving the whole lung. Extracorporeal circulation enabled us to perform radical primary surgery. Prosthesis implant not only maintained normal chest expansion but also allowed focused radiotherapy, thus enabling us to prevent damage to vital organs.

Spinal muscular atrophy (SMA) includes genetic heterogeneous diseases characterized by muscle weakness and atrophy. There are at least two risk factors for SMA patients to develop urinary dysfunctions: 1) the neural degeneration, that influences the pelvic floor and sphincters; 2) the disabilities that predispose to constipation and urinary incontinence and symptoms. Nevertheless, the true incidence of urological disorders remains unknown. The evaluation of urinary symptoms in SMA patients remains scarcely investigated. The aim of our paper is to assess the association between urological disorders and SMA. We worked out a questionnaire to assess the voiding habits of patients with SMA followed at a single Italian Centre. We collected demographic data and information regarding respiratory, orthopedic and gastrointestinal involvement. We excluded patients

Microgastria is a rare congenital condition often associated with other anomalies. In the present report we describe the case of a 6-year-old girl with isolated CM who presented with dumping syndrome successfully treated by a Hunt-Lawrence pouch.

Background The aim of the study was to assess current international practice in the long term follow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM). Methods An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care. Results 96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that >30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13–16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition. Conclusions International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners.

Introduction The use of prosthetic patches of non-absorbable materials represents a valid tool in the treatment of abdominal wall and diaphragmatic defects in pediatric age. In recent years research has developed biological dermal scaffolds made from a sheet of acellular matrix that can provide the desired support and reduce the occurrence of complications from non-absorbable implant. We present our experience and a systematic review to evaluate the use of biologic prosthesis for abdominal wall closure in pediatric patients. Methods The study from January 2009 to January 2015 involved 20 patients treated with Permacol™ implant. We observed postoperative complications only in patients treated for abdominal wall closure, which is the major indication for the use of Permacol™. We conducted a systematic review and meta-analysis (according to PRISMA) on PubMed/Medline, Scopus and EMBASE regarding the use of biological prosthesis in pediatric population considering the incidence of complications as the primary outcome. Results 3/20 patients experienced complications: 2 patients with skin necrosis healed conservatively and 1 of them developed laparocele. Thus only 1 patient with incisional hernia had significant surgery complication. In patients who were permanently implanted with Permacol™ it has not determined adverse reactions with optimal functional outcome. Conclusions In accordance with the few data (case reports and case series) reported in literature about pediatric patients, our experience in different pathologies and applications has shown the effectiveness of Permacol™, in particular for the non-occurrence of infections, that often affect the use of prosthesis., Highlights • Use of biological prosthesis is well known for the treatment of complicated abdominal defects in adults surgery but not in pediatric surgery. • We analyzed the largest case series present in the literature in pediatric surgery. • Looking at our results the use of Permacol™ seems to be safe and feasible in pediatric complicated abdominal wall closure.

One-trocar laparoscopic appendectomy (OTA) is routinely adopted in children with acute appendicitis. In case of a difficult appendectomy, it is necessary to add additional trocar/s to safely complete the procedure. This technique is called multiport hybrid laparoscopic appendectomy (HLA). We aimed to compare the outcome of multiport HLA versus OTA. METHODS: We retrospectively reviewed the data of 1,092 patients underwent LA in 5 European centers of pediatric surgery in the last 5 years. We compared 2 groups: G1 of 575 patients (52.6 %) (average age 10 years) underwent OTA and G2 of 517 patients (47.4 %) (average age 8.2 years) underwent multiport HLA. RESULTS: No intra-operative complications occurred in both groups. An additional pathology was treated in 12 cases (8 Meckel's diverticulum, 2 carcinoids, 2 ovarian cysts) in G2. Operative time was significantly shorter in G2 compared to G1 (47.8 vs 58.6 min; p < .001). The average analgesic requirement was significantly shorter in G2 compared to G1 (44 vs 56 h; p < .001). As for postoperative complications, the incidence of port-site infections was similar between the two groups, while the incidence of postoperative abdominal abscesses (PAA) was significantly higher in G1 compared to G2 (4.7 vs 0.2 %; p < .001). The cosmetic outcome was excellent in all patients of both groups. A subgroup analysis between complicated and uncomplicated appendicitis showed that only in complicated cases, the average operative time, the average VAS pain score, the average analgesic requirements and the incidence of PAA were significantly higher in OTA group compared to multiport HLA group (p < .001). CONCLUSIONS: Our results suggest that OTA is a valid and safe procedure for the uncomplicated cases, while additional trocars are required in case of complicated appendicitis. Multiport HLA significantly reduces the operative time, the incidence of abdominal abscesses and the analgesic requirements compared to OTA.

Congenital deformities of the chest wall or chest wall malformations (CWMs) comprise a complex spectrum of anomalies which range from those that do not impact on the health and quality of life of the patient to those which are life-threatening.

Esophageal achalasia is a primary motility disorder that is thought to be due to a dysfunction of the enteric nervous system [1]. It is characterized by loss of ganglion cells with degeneration of inhibitory myenteric plexus that innervates the esophageal body and the region of the lower esophageal sphincter (LOS) of unknown origin. It is hypothesized that decreased nitric-oxide synthase leads to a neuronal excitatory/inhibitory imbalance with esophageal aperistalsis, incomplete LOS relaxation in response to swallowing, and increased LOS resting pressures.

Very limited informations are currently available about the best approach to perform retroperitoneoscopic surgery. This multicentric international study aimed to compare the outcome of lateral versus prone approach for retroperitoneoscopic partial nephrectomy (RPN) in children. METHODS: The records of 164 patients underwent RPN in 7 international centers of pediatric surgery over the last 5 years were retrospectively reviewed. Sixty-one patients (42 girls and 19 boys, average age 3.8 years) were operated using lateral approach (G1), whereas 103 patients (66 girls and 37 boys, average age 3.0 years) underwent prone RPN (G2). The two groups were compared in regard to operative time, postoperative outcome, postoperative complications, and re-operations. RESULTS: The average operative time was significantly shorter in G2 (99 min) compared to G1 (160 min) (p = 0.001). Only 2 lateral RPN required conversion to open surgery. There was no significant difference between the two groups as for intraoperative complications (G1:2/61, 3.3%; G2:6/103, 5.8%; p = 0.48), postoperative complications (G1:9/61, 14.7%; G2:17/103, 16.5%; p = 0.80), and re-operations (G1:2/61, 3.3%; G2:4/103, 3.8%; p = 0.85). Regarding postoperative complications, the incidence of symptomatic residual distal ureteric stumps (RDUS) was significantly higher in G2 (7/103, 6.8%) compared to G1 (1/61, 1.6%) (p = 0.001). Most re-operations (4/6, 66.6%) were performed to remove a RDUS . CONCLUSIONS: Both lateral and prone approach are feasible and reasonably safe to perform RPN in children but the superiority of one approach over another is not still confirmed. Although prone technique resulted faster compared to lateral approach, the choice of the technique remains dependent on the surgeon's personal preference and experience. Our results would suggest that the lateral approach should be preferred to the prone technique when a longer ureterectomy is required, for example in cases of vesico-ureteral reflux into the affected kidney moiety, in order to avoid to leave a long ureteric stump that could become symptomatic and require a re-intervention.

Prenatal magnetic resonance imaging is the best tool to visualize foetal airway. To evaluate the performance of MRI in the assessment of foetal airway status in the presence of a neck mass. Two paediatric radiologists with 12- and 2-year experience in foetal imaging retrospectively analysed 23 foetal MRI examinations, performed between 2001 and 2016, after a second-level ultrasound suspicious for presence of a neck mass. Postnatal imaging, postoperative report, histology, autopsy, and clinical outcomes were the reference standard to calculate sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of prenatal MRI in detecting airway patency. We used the Cohen к statistics to estimate the interobserver agreement. We also assessed MRI performance in the diagnosis of the mass nature. We obtained data about postnatal airway status in 19 of 23 patients; prenatal MRI demonstrated a sensitivity of 9/9 [100%, 95% confidence interval (CI) 66–100%], specificity 8/10 (80%, 44–98%), accuracy 17/19 (89%, 67–99%), PPV 9/11 (82%, 48–98%), and NPV 8/8 (100%, 63–100%); the interobserver agreement was perfect. Prenatal MRI correctly identified 21 of 23 masses (к = 0.858); the interobserver agreement was almost perfect (к = 0.851). Prenatal MRI demonstrated high accuracy in assessing foetal airway status and diagnosing mass nature, allowing proper delivery planning.

Introduction: Peripheral neuroblastic tumors (PNTs) account for 8%–10% of all pediatric tumors. Adrenal glands and sympathetic ganglia are the commonest site of tumor growth. In the clinicopathologic spectrum of PNTs, neuroblastoma and ganglioneuroma are the most primitive and the most mature tumor form, while ganglioneuroblastoma represents an intermediate state of maturation. Surgical resection is the therapy of choice in localized disease, but can lead to serious complications when performed in the presence of certain imaging-defined risk factors. Case presentation: We present a rare case of primary intrarenal ganglioneuroblastoma diagnosed in a teenager who underwent conservative surgery and, despite this, developed upper pole renal ischemia without loss of parenchymal function. Conclusion: We underline the complex management of these extremely rare cases of neuroblastic tumors, which require a dedicated multidisciplinary team.

Introduction Being able to have children could have an important positive effect on the future lives of pediatric cancer survivors. Working at a cancer institute makes us responsible for filling the gaps in our knowledge in this area of patient care. Methods We describe our activities in a series of young females diagnosed with cancer and evaluated for fertility preservation options. We discuss the developed skills and organization as well as the practical difficulties encountered in managing fertility preservation. Results Since September 2012, laparoscopy and cryopreservation of cortical ovarian tissue has been performed in 16 girls (with ovary transposition in 3, and after several cycles of chemotherapy in 5) and egg banking in 4 young women (before chemotherapy in 2 and several years after treatment in 2). Conclusions Recommendations on fertility preservation indicate that discussing the problems early on is crucial to future success. It is unthinkable to simply provide information and offer the opportunity to choose a fertility preserving technique without helping and accompanying patients and their families in their decisions and choices on the matter.

To compare the outcome of laparoscopic and retroperitoneoscopic approach for partial nephrectomy in infants and children with duplex kidneys. Data of 102 patients underwent partial nephrectomy in a 5-year period using MIS procedures were analyzed. Fifty-two children underwent laparoscopic partial nephrectomy (LPN), and 50 children underwent retroperitoneoscopic partial nephrectomy (RPN). Median age at surgery was 4.2 years. Statistical analysis was performed using χ 2 test and Student’s t test. The overall complications rate was significantly higher after RPN (15/50, 30 %) than after LPN (10/52, 19 %) [χ 2 = 0.05]. In LPN group, complications [4 urinomas, 2 symptomatic refluxing distal ureteral stumps (RDUS) and 4 urinary leakages] were conservatively managed. In RPN group, complications (6 urinomas, 8 RDUS, 1 opening of remaining calyxes) required a re-operation in 2 patients. In both groups no conversion to open surgery was reported. Operative time (LPN:166.2 min vs RPN: 255 min; p

PURPOSE: Retroperitoneoscopic partial nephrectomy (RPN) in children is considered a complex technique with limited diffusion among pediatric surgeons and urologists. We aimed to report the outcome of this technique in infants and children with duplex kidney in a 5-year retrospective multicentric international survey. MATERIALS AND METHODS: Data on 50 children who underwent RPN (41 upper-pole nephrectomies and 9 lower-pole nephrectomies) were retrospectively collected in this six-institution survey. Median age at surgery was 3.3 years. There were 35 girls and 15 boys. The left side was affected in 28 patients, versus the right side in 22 patients. We assessed intraoperative and postoperative morbidity. Follow-up (median, 2.5 years; range, 12 months-5 years) was based on clinical controls and echo color Doppler renal ultrasound scans. RESULTS: Median duration of surgery was 255 minutes. Surgery was always performed with the patient in a lateral position. Special hemostatic devices were used for dissection and parenchymal section in all centers. Three patients from two centers (6%) required conversion to open surgery. We recorded seven complications (six peritoneal perforations, one opening of the remaining calyxes) in the 50 cases. Re-operation rate was 0%. Average length of hospital stay was 4.1 days. CONCLUSIONS: Our survey shows that RPN remains a challenging procedure with a long learning curve, performed only in pediatric centers with huge experience in this field. In our survey operative time was longer than 4 hours. The complication rate remains high (7/50, or 14%), with complications classified as Grade II according to the Clavien-Dindo classification. They did not require further surgery, but they were associated with a prolonged hospital stay.

Moving from the study conducted in 2004 on adult surgeons we want to analyze the ergonomics applied in pediatric minimally invasive surgery. An online survey was conducted among the members of the European Paediatric Surgeons' Association that included 14 questions pertaining to demographic, surgical glove size, double glove use, prior hand surgery, and the ease or difficulty in using different types of laparoscopic instruments. A total of 138 pediatric surgeons completed the survey. The difficulty score (DS) was similar between the 3.5- and 5-mm instruments. Other specialized instruments such as Ligasure, Ultracision, Clip applicators, endobags, and staplers were found to have higher DS. The needle holder was the only instrument that is part of the normal 5-mm operating sets, which was found to have a higher DS. Our survey found increased DS with the endobag and stapler, but this was not significant. Also prior hand surgery or double glove use was not associated with difficulty in usage of minimally invasive instruments when compared with normal hands in this survey.

The neoplasms arising from the neural crest-derived cells in the adrenal medulla or extra-adrenal paraganglia include peripheral neuroblastic tumours, pheochromocytoma and paraganglioma.

Background Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. Procedure Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. Results The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. Conclusions Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses. Pediatr Blood Cancer 2015;62:1202–1208. © 2015 Wiley Periodicals, Inc.

Italian pediatric surgery has a long history, rich in events, places, and personalities that make it unique. Our first pediatric surgical unit dates back to the first half of the 19th century and its number has grown exponentially during the following decades, with a maximum flowering in the years after the second world war.The rich history of pediatric surgery has not prevented it from facing important problems, such as the demographic crisis, underemployment of surgeons, and care of pediatric patients by adult specialists.The attempt to find solutions to these problems has led to a great deal of efforts not only by pediatric surgeons, but also by the Italian government and institutions, with the implementation of ministerial plans that favor the specificity and growth of our discipline through thematic and territorial networks, through more effective training and specialization plans, the cooperation between the Directors of Pediatric Surgical Units for a better coordination of care, the participation in European networks, such as the recently created ERNs (European Reference Networks).Many Italian pediatric surgeons are active in International Societies, as members or coordinators, in the design and management of multicenter studies and surveys and even in the creation of new international networks.The Italian response to the demand for specialization and centralization involves knowledge sharing, large-scale cooperation, transversal between specialists and vertical with governmental authorities for a better and more effective care of pediatric patients.

Aim: To report the case of a patient with total colonic tubular duplication pointing out the diagnostic and therapeutic difficulties and reviewing the Literature concerning this rare malformation. Case report: The patient was a female girl born at term with vertebral and spinal anomalies. At 6 months she underwent emergent left upper lobectomy for a symptomatic, previously undiagnosed left congenital lobar emphysema. She also had intractable coprostasis and abdominal distension. The clinical picture and the results of the contrast enema made us think of congenital megacolon but suction rectal biopsies were inconclusive. Therefore we obtained full thickness colonic and terminal ileum biopsies and we performed an ileostomy for faecal diversion and bowel management. A few months later, the anterograde contrast study from the distal ileostomy showed two colons with proximal communication and a distal pouch. On laparotomy, we confirmed the diagnosis of complete colonic tubular duplication. We divided the common wall between the two colons, we performed a mucosectomy and we removed the duplicated segment. Post-operative course was uneventful. Conclusions: Duplications of the colon are rare and the diagnosis is not easy. Two main problems occurred during surgery: differentiating between normal and duplicated colon and removing the duplication avoiding extensive colonic resections. Resection of the common wall, mucosectomy and removal of the duplicated colon represented a feasible and safe procedure. Keywords: Colon duplication, Constipation, Paediatric surgery, Congenital malformation

Background Gastrointestinal (GI) carcinomas are very rare in the pediatric and adolescent age range. We report the clinical features, treatment, and outcome of a series of children and adolescents with GI carcinoma prospectively registered in the Italian Tumori Rari in Eta Pediatrica (TREP) project. Methods The TREP project developed diagnostic and therapeutic guidelines based on recommendations currently in use for adults. Clinical data were centrally registered and reviewed. Results Fifteen patients were registered over the years 2000–2016. Most of the tumors were colorectal carcinomas (12 cases). All but one patient had advanced-stage disease (American Joint Committee on Cancer stages III–IV), and the majority of patients had aggressive histological subtypes, i.e. poorly differentiated (G3) (five patients), mucinous (four patients), and signet ring (two patients) adenocarcinomas. Surgery was performed in 13 of 15 patients, and was radical in nine of 13 patients. Only one patient received postoperative radiotherapy. All patients received chemotherapy, with the addition of bevacizumab in two cases. Nine patients were still alive at the time of the present report, but two of them had only just completed their treatment program and one patient is still on treatment. Six patients died due to disease progression. Conclusions This prospective report on pediatric GI tract carcinomas confirms the rarity and biological aggressiveness of these diseases in pediatric and adolescent age. Further prospective studies are needed to explore the distinct biology of tumor in this age group in order to find new therapeutic targeted agents.

Background Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperative medical treatment in such patients were reviewed to 1) ascertain whether a shared treatment strategy exists among centers and 2) if possible, propose some recommendations for the perioperative management of HT in NB patients. Methods A retrospective multicenter survey was conducted on patients affected by NB who presented HT symptoms. Results From 2006 to 2014, 1126 children were registered in the Italian Registry of Neuroblastoma (RINB). Of these, 21 with HT (1.8%) were included in our analysis. Pre- and intraoperative HT management was somewhat dissimilar among the participating centers, apart from a certain consistency in the intraoperative use of the alpha-1 blocker urapidil. Six of the 21 patients (28%) needed persistent antihypertensive treatment at a median follow-up of 36months (range 4–96months) despite tumor removal. Involvement of the renal pedicle was the only risk factor constantly associated to HT persistency following surgery. A correlation between the presence of HT and the secretion of specific catecholamines and/or compression of the renal vascular pedicle could not be demonstrated. Conclusion Based on this retrospective review of NB patients with HT, no definite therapeutic protocol can be recommended owing to heterogeneity of adopted treatments in different centers. A proposal of perioperative HT management in NB patients is however presented. Level of evidence IV.

Leydig cell tumors (LCTs) are rare cord-stromal tumors that may occur in testis or ovaries and may produce androgens or estrogens. The majority has been found in men between the ages of 20 and 60 years. Adults with androgen-secreting LCTs are usually asymptomatic; feminizing syndromes may result from the production of estradiol or the peripheral aromatization of testosterone. In children, LCTs usually present between 5 and 10 years of age with isosexual precocious pseudopuberty or gynecomastia. We report 2 cases of LCT in prepubertal boys presenting with advanced unilateral pubarche and testicular volume asymmetry. Both subjects had normal penis size for age; no axillary hair or other signs of puberty were present. Height velocity was normal, and bone age was coincident with chronological age. Androgen levels were normal, as well as estrogen, corticotropin, and cortisol concentration. Testicular ultrasound demonstrated a testicular mass. Histology examination revealed a well-differentiated LCT. This is the first report of 2 pediatric patients with LCT presenting with advanced pubarche in absence of systemic hyperandrogenism. We hypothesize that the neoplastic cells may locally produce high levels of androgens or androgen-like bioactivity molecules that are responsible for the clinical manifestation. We suggest that a testicular ultrasound should be obtained in all children presenting with unilateral pubarche, with or without hyperandrogenism.

Background: Magnetic resonance imaging (MRI) is a possible alternative to the computed tomography (CT) in the evaluation of pediatric lung diseases (1). Aim of the study: to determine the diagnostic utility of MRI as compared with contrast CT for the postnatal diagnosis of congenital lung malformations (CLM). Materials and methods: two experienced pediatric radiologist prospectively assessed MRI and CT in 12 patients (6 male, 6 female, age range: 0-6 months) with prenatal diagnosis of CLM. For each patient9s lung malformation were evaluated site (side, lobe and segment), appearance (solid, cystic, mixed, aerial), number of cysts ( Results: Observers found 15 lung malformations (3 CPAM type II, 1 CPAM type I, 3 hybrid lesions, 5 segmental bronchial atresias, 1 intralobar bronchopulmonary sequestration, 2 extralobar bronchopulmonary sequestrations) in 12 patients and MRI diagnosis was correct in all cases. MRI showed 100% accuracy, sensitivity, specificity, PPV and NPV in the assessment of cysts, anomalous systemic arterial vessels, and air-trapping. MRI found lower values of sensitivity, accuracy and NPV in the assessment of the global extension of the malformation, number of cysts, abnormal venous drainage, bronchial atresia. Conclusions: MRI can be an effective alternative to CT in the postnatal assessment of lung malformations.

Background: Asymptomatic Congenital Lung Malformations (CLM) are often electively resected on the assumption that lobectomy has low impact on respiratory health and function. A recent meta-analysis showed that elective resection is safe and prevents the risk of symptoms development, which may result in a higher rate of postoperative complications. Aim of the study: To assess respiratory symptoms and lung function at school age in children who underwent lobectomy for asymptomatic CLM. Materials and Methods: We retrospectively reviewed performance, symptoms (open interview) and spirometric values of FEV1, FVC, FEV1/FVC and FEF75-25 with comparison to z-scores (Stanojevic S. et al., Am J Crit Care Med 2008;177(3):253-60) of 18 patients between 4-9 years of age. Results: Between 2000-2009, 18 patients (9 female, 9 male) underwent elective lobectomy (8 upper left, 3 lower left, 6 lower right, 1 middle lobe) in our hospital, 13 patients within the first year of life and 5 after . The pathologic diagnoses were: 6 congenital lobar emphysema, 2 pulmonary sequestration and 10 Congenital Pulmonary Airway Malformation (CPAM). At follow-up observation at 4-9 years of age, 14 patients were symptoms free and their effort tolerance was comparable to peers; 3 patients had effort dyspnea and reduced effort tolerance, 1 had occasional wheeze controlled with inhaled corticosteroids therapy. Mean and median of FEV1, FVC and FEV1/FVC were within normal range if compared to z-scores, while FEF75-25 was lower (mean 1%, median 3.2%). Conclusions: The majority of our 18 patients were symptoms free, 4 had mild symptoms and limitations. Lung function was essentially within predicted values.

Study Objective To report results of a retrospective multicentric Italian survey concerning the management of pediatric ovarian torsion (OT) and its recurrence. Design Multicenter retrospective cohort study. Setting Italian Units of Pediatric Surgery. Participants Participants were female aged 1-14 years of age with surgically diagnosed OT between 2004 and 2014. Interventions Adnexal detorsion, adnexectomy, mass excision using laparoscopy or laparotomy. Different kinds of oophoropexy (OPY) for OT or recurrence, respectively. Main Outcome Measures A total of 124 questionnaires were returned and analyzed to understand the current management of pediatric OT and its recurrence. The questionnaires concerned patient age, presence of menarche, OT site, presence and type of mass, performed procedure, OPY technique adopted, intra- and postoperative complications, recurrence and site, procedure performed for recurrence, OPY technique for recurrence, and 1 year follow-up of detorsed ovaries. Results Mean age at surgery was 9.79 ± 3.54 years. Performed procedures were open adnexectomy (52 of 125; 41.6%), laparoscopic adnexectomy (25 of 125; 20%), open detorsion (10 of 125; 8%), and laparoscopic detorsion (38 of 125; 30.4%). Recurrence occurred in 15 of 125 cases (12%) and resulted as significant ( P = .012) if associated with a normal ovary at the first episode of torsion. Recurrence occurred only in 1 of 19 cases after OPY (5.2%). Ultrasonographic results of detorsed ovaries were not significant whether an OPY was performed or not ( P = 1.00). Conclusion Unfortunately, oophorectomy and open technique are still widely adopted even if not advised. Recurrence is not rare and the risk is greater in patients without ovarian masses. OPY does not adversely affect ultrasonographic results at 1 year. When possible OPY should be performed at the first episode of OT.

Background Peripheral neuroblastic tumors are the most common extracranial solid neoplasms in children. Early and adequate tissue sampling may speed up the diagnostic process and ensure a prompt start of optimal treatment whenever needed. Different biopsy techniques have been described. The purpose of this multi-center study is to evaluate the accuracy and safety of the various examined techniques and to determine whether a preferential procedure exists. Methods All children who underwent a biopsy, from January 2010 to December 2014, as a result of being diagnosed with a peripheral neuroblastic tumor, were retrospectively reviewed. Data collected included patients’ demographics, clinical presentation, intraoperative technical details, postoperative parameters, complications, and histology reports. The Mann–Whitney U and Fisher's exact tests were used for statistical analysis. Results The cohort included 100 patients, 32 of whom underwent an incisional biopsy (performed through open or minimally invasive access) (Group A), and the remaining 68 underwent multiple needle-core biopsies (either imaging-guided or laparoscopy/thoracoscopy-assisted) (Group B). Comparing the two groups revealed that Group A patients had a higher rate of complications, a greater need for postoperative analgesia, and required red blood cell transfusion more often. Overall adequacy rate was 94%, without significant differences between the two groups (100% vs. 91.2% for Group A and Group B, respectively, P = 0.0933). Conclusions Both incision and needle-core biopsying methods provided sub-optimal to optimal sampling adequacy rates in children affected by peripheral neuroblastic tumors. However, the former method was associated with a higher risk of both intraoperative and postoperative complications compared with the latter.

The pain management in pediatric surgery is a very complex and relevant problem with many clinical implications. Pain in children requires special considerations due to physical and psychological immaturity. The enhancement in pain management results in a reduction of recovery time and complications. Few studies are available in the literature focused on postoperative pain and analgesia requirements in children following surgical procedures. In the present brief review we summarize the current strategies of pain management in pediatric surgery and we envision the new hypothetical horizons in this field.

Background: This report describes three Italian centers’ experience in the treatment of children with esophageal achalasia. Patients and Methods: Between June 2000 and June 2012, 31 children (13 girls and 18 boys, with a median age of 8.4 years) affected by esophageal achalasia were treated in three different institutions with an esophagomyotomy according to Heller’s procedure via laparoscopy associated with a Dor antireflux procedure. Between 2000 and 2005 (for 14 patients) we used mono- or bipolar coagulation to perform myotomy; after 2005 (for 17 patients) we used the new hemostatic devices to perform it. Results: Median length of surgery was 120 minutes. Median hospital stay was 4 days. We recorded eight complications in our series: 3 patients (9.6%) had a mucosal perforation, and 5 children (16.1%) presented dysphagia after surgery. When comparing the data before and after 2005, it seems that the new hemostatic devices statistically shortened the length of surgery (P < .01, Student’s t test). Conclusions: On the basis of our experience, laparoscopic Heller’s myotomy associated with an antireflux procedure is a safe and effective method for the treatment of achalasia in the pediatric population. Intraoperative complications were < 10%, and they occurred mostly at the beginning of our experience. Residual dysphagia occurred in about 16% of cases. The use of the new hemostatic devices seems to reduce the length of surgery and intraoperative bleeding. Considering the rarity of this pathology, we believe that patients with achalasia have to be treated only at centers with a strong experience in the treatment of this pathology.

Müllerian derivatives are a frequent finding in patients with external genital ambiguity. In cases in which their removal is indicated, traditional surgical approaches are both invasive and associated with risks. We report a case of mixed gonadal dysgenesis in which a large prostatic utricle was successfully removed via laparoscopy.

Background Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. Methods A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). Results A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. Conclusions This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.