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Vesicoureteral reflux (VUR) is an abnormality frequently seen at a complete duplex system (DS). Operational correction is required and completed after the neonatal period when symptoms occur. This study aimed to evaluate the efficacy of Vantris and a need for additional surgery in children with DS VUR in a multicenter study. We performed retrospective analysis of prospectively acquired data, from 2009 to 2018, on 172 patients with a mean age of 3 years with VUR into either upper or lower moiety of the DS who underwent endoscopic correction utilizing Vantris at five centers worldwide. All patients were with primary VUR. The median follow-up was 7 years. Reflux was corrected in 122 patients (70%) after the first injection and 35 patients (20%) after the second injection. 15 patients (9%) failed endoscopic correction and required ureteral reimplantation. 13 patients suffered afebrile urinary tract infection, and 9 patients developed febrile urinary tract infection (UTI). 3 patients required partial nephrectomy of the poorly or non-functioning refluxing moiety following the failure of endoscopic correction. Our data shows that Vantris injection provides a high reflux resolution in a DS with VUR. Successful VUR resolution might spare some patients associated with VUR poorly functioning moieties further surgery.

Uretero-pelvic junction obstruction is the most common cause of prenatal hydronephrosis. It can be intrinsic, extrinsic due to crossing vessel (CV) or mixed. This paper aims to present the surgical outcome in a single pediatric third-level center. A retrospective analysis of all children operated between 2011 and 2018 was conducted. Demographic information, pre-operative pelvic antero-posterior diameter (APD), intra-operative parameters, hospital stay and follow-up were considered. Re-do operations and success rate for Vascular Hitch (VH), open and laparoscopic pyeloplasties were recorded. 128 patients were included. The mean pre-operative APD was 30 mm. The etiology was intrinsic in 71.9%, extrinsic in 25.0% and mixed in 3.1%. The median age between intrinsic and extrinsic groups was statistically different. Thirty-one VH, 88 dismembered and 9 non-dismembered pyeloplasties were performed. The median hospital stay was 2 days for VH and 6 for pyeloplasties. The median hospital stay was statistically longer in open pyeloplasties compared to laparoscopic. The mean post-operative APD was 14 mm. The success rate after VH was 90.3%, after open pyeloplasties 97.9% and after mini-invasive pyeloplasties 91.8%. VH could be a good option in the treatment of hydronephrosis due to CV, laparoscopic pyeloplasties have equivalent success rate and shorter hospital stay than open pyeloplasties.

Ureteral anomalies distal to the Uretero-Pelvic Junction (UPJ) belong to the wide spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). They can cause severe obstruction requiring a detailed anatomical depiction to define the surgical approach. Up to date, ultrasonography, voiding cystourethrography and scintigraphy are considered the gold-standard diagnostic tools to study obstructive anomalies of the urinary tract, however, they do not provide accurate ureteral anatomical details. The aim of our study was to evaluate the concordance between functional magnetic resonance urography (fMRU) and intraoperative findings to define ureteral anomalies distal to UPJ.Pediatric patients with ureteral anomalies distal to the UPJ who underwent surgery after performing fMRU were retrospectively collected. Surgical data were compared with radiological results. The concordance was assessed considering both pathological and non-pathological urinary tracts and was calculated by means of the Cohen's kappa coefficient. fMRU diagnostic accuracy was defined by sensitivity, specificity, and binomial exact confidence intervals.We included 46 patients. The sensitivity and specificity of fMRU were 98.0% and 83,3%; positive predictive value 90,4%, negative predictive value 96.2%. The concordance between surgical findings and fMRU was 92,3%, with a k Cohen's coefficient of 0.83 (excellent).Our study demonstrates the excellent agreement between fMRU and surgical findings in the definition of ureteral anomalies distal to the UPJ in children. Thus, it could be considered a valid imaging technique in the preoperative planning as it provides the surgeon with important information regarding the etiology and site of the obstruction.

Tracheal anomalies are rare entities and experience managing these anomalies is consequently limited and widely dispersed. TL can be divided into congenital and acquired anomalies. Among the first ones, tracheomalacia (TM) is the most frequent, and can be primary or associated with other conditions such as esophageal atresia. Although tracheomalacia is a cause of many respiratory symptoms, possibly severe up to death, indications, and types of treatment are not universally accepted. Among the treatments, aortopexy and posterior tracheopexy are the most effective. Internal stents are burdened by a significant rate of morbidity. Congenital tracheal stenosis (CTS), laryngotracheoesophageal clefts, tracheal webs, and tracheal agenesis are rare. Other congenital tracheal malformations such as tracheoesophageal fistula are described in other chapters of this book. Even if an embryological model explaining congenital tracheal anomalies remains elusive, it has been noted, as these are frequently associated with other mediastinal anomalies due to the intimate embryological development of the trachea, esophagus, and cardiovascular system. Acquired anomalies are usually represented by stenosis secondary to intubations and trauma or extrinsic compressions from mediastinal masses, as neoplasms or bronchogenic cysts.

Although children seem to be less affected than adults by COVID-19, Italianpaediatric hospitals have had to significantly reorganise services to deal with the pandemic. These include thetertiary-level Gaslini Children's Hospital in Genoa, which handles around 35,000 emergency room (ER) visits per year. The Government lockdown has dramatically reduced ER admissions and delayed programmed admissions (1).

The management of vesicoureteric junction (VUJ) diseases is a common and controversial problem in pediatric urology. Minimal invasive approaches were described for vesicoureteric reflux (VUR), obstructive megaureter (POM) and complicated duplex systems treatments. The aim of this paper is to describe our experience with minimal invasive approach focusing on technical considerations and preliminary outcomes. Data about patient aged between 0 and 18 years who underwent minimal invasive surgery for VUR, POM or complicated duplex system, were prospectively collected. Complications were classified according to Clavien–Dindo classification. One senior surgeon, trained in laparoscopy, performed surgeries (Lich Gregoir reimplantation, dismembered reimplantation and ureteroureterostomy). Seventeen patients were included in the study; thirteen had an extravesical reimplantation (nine non-dismembered, four dismembered) and four had an ureteroureterostomy. No complications were described. Median operating time was 98.5 min and median hospital stay was 4.1 days. Median follow-up was 7.5 months. Success rate for VUR was 78%; for dismembered reimplantation, one case showed post-operative VUR. Functional studies showed an improvement in split renal function on the pathologic moieties in patients treated by ureteroureterostomy for complicated ectopic ureters. This preliminary experience in minimal invasive surgery of the distal ureter shows that this approach is feasible and safe, reduces hospitalization and gives better cosmetic. Results may be impacted by the surgeon’s learning curve and technical modifications. As robotic procedures are described to be more effective and safe, less technical demanding and associated with good results, switching from laparoscopic to robot-assisted surgery could be useful to improve results.

To assess the efficacy of the vascular hitch procedure in extrinsic ureteropelvic junction obstruction (UPJO) by crossing vessels in children, both by open and mini-invasive approach. Retrospective analysis on patients treated by open or mini-invasive vascular hitch during the period 2006–2020. Preoperative imaging included renal ultrasound (US) and functional studies by magnetic resonance or renogram. Crossing vessels obstruction and UPJ anatomy were evaluated at surgery. Success was defined as the resolution of symptoms and improvement of hydronephrosis at US or functional studies. Forty-eight patients were included with mean age at surgery of 7.6 years and 25% had an antenatal diagnosis. Aberrant vessels were suspected preoperatively in 72.9%; mean hydronephrosis and renal function were 30.9 mm and 38% respectively. Seven cases were treated by open approach, 37 by laparoscopy and 4 were robotassisted. Mean operative time was 58.3 min and mean hospital stay was 2 days. Mean follow-up was 16.9 months; success rate was 91.6% and 5 patients required secondary surgery. Vascular hitch shows to be a safe, feasible and effective alternative to pyeloplasty. Careful pre and intra-operative patients’ selection is essential to exclude any intrinsic cause of UPJO. Mini-invasive surgery could be helpful for better evaluation of UPJ anatomy and its relationships with crossing vessels. Complications and secondary surgeries could be related to incorrect patients’ selection.

Thoracoscopic treatment is one of the options for the repair of Pectus Carinatum (PC). The ideal indication is a unilateral PC involving cartilages only, without sternal deformity or rotation. Very severe degree of PC represents a contraindication to thoracoscopic repair.

The aim of this study was to evaluate the frequency of surgical and organizational events that occurred in the whole Department of Paediatric Surgery at Gaslini Children’s Hospital through an incident-reporting system in order to identify the vulnerabilities of this system and improve it. This is a 6-month prospective observational study (1st January–1st July 2010) of all events (including surgical and organizational events, and near misses) that occurred in our department of surgery (pediatric surgery, orthopedics and neurosurgery units). Over a 6-month study period, 3,635 children were admitted: 1,904 out of 3,635 (52.4%) children underwent a surgical procedure. A total number of 111 adverse events and 4 near misses were recorded in 100 patients. A total of 108 (97.3%) adverse events occurred following a surgical procedure. Of 111 adverse events, 34 (30.6%) required re-intervention. Eighteen of 100 patients (18%) required a re-admission, and 18 of 111 adverse events (16.2%) were classified as organizational. Infection represented the most common event. An electronic physician-reported event tracking system should be incorporated into all surgery departments to report more accurately adverse events and near misses. In this system, all definitions must be standardized and near misses should be considered as important as the other events, being a rich source of learning.

Background/Purpose The removal of a pectus bar fixed with a metallic stabilizer can be time consuming and tedious, because in some cases, fibrous or new bone tissue covers the metallic devices. Our study aims to evaluate bar removal in 2 groups of patients with metallic and absorbable stabilizers, respectively. Methods A total of 162 patients underwent mini-invasive repair of pectus excavatum. In all the cases, the bar was stabilized with at least 1 stabilizer on the left side. We used both metallic and absorbable stabilizers. Absorbable stabilizers were preferred when they were available in the market. The bar was removed in 30 patients. We compared removal of the bar in 17 absorbable stabilizers with those bars fixed with 18 metallic stabilizers. Length of incision, operative time, postoperative pain, and complications were studied. Results No differences between metallic and absorbable stabilizers were found in terms of postoperative pain and complications. However, removal of the bar fixed with an absorbable stabilizer required a significantly smaller incision and shorter operative time. Conclusions Removal of the pectus bar fixed with an absorbable stabilizer was simpler and faster.

Sternal cleft is a chest wall malformation that can expose mediastinal viscera and vessels to injuries. It can be classified into two forms, complete and partial. Its etiology and incidence are unknown and it is often associated with other defects. The aim of this article is to review the literature and report our experience with this rare anomaly, focusing on clinical presentation and management. We reviewed the English written literature about sternal cleft and collected the clinical data of all the published series. We present seven new cases that we have observed and treated since 1999. Literature reports 51 series including 86 patients, more frequently female (62%) and affected with partial superior form (67%). Sternal cleft is often asymptomatic (74%) and associated with other defects (72%). Surgical treatments include primary closure (73%), bone graft interposition (10%), prosthetic closure (7%), and muscle flap interposition (3%). In our series, primary closure was possible in four cases, while in three cases we placed a prosthesis. Five patients had associated defects and two were affected with PHACES (posterior fossa abnormalities, hemangiomas, arterial lesions, cardiac abnormalities/aortic coarctation, abnormalities of the eye, and sternum defects) syndrome. We report for the first time the association of sternal cleft with connectival nevi in three of our patients. At follow-up, we observed no major complication or recurrences. Although primary closure is the preferred option and should be performed in the neonatal period, the use of prostheses warrants good results as well. Prior to treatment, associated defects and syndromes should be excluded.

Since the introduction of the Nuss technique for pectus excavatum (PE) repair, stabilization of the bar has been a matter of debate and a crucial point for the outcome, as bar dislocation remains one of the most frequent complications. Several techniques have been described, most of them including the use of a metal stabilizer, which, however, can increase morbidity and be difficult to remove. Our study compares bar stabilization techniques in two groups of patients, respectively, with and without the metal stabilizer.A retrospective study on patients affected by PE and treated by the Nuss technique from January 2012 to June 2013 at our institution was performed in order to evaluate the efficacy of metal stabilizers. Group 1 included patients who did not have the metal stabilizer inserted; stabilization was achieved with multiple (at least four) bilateral pericostal Endo Close™ (Auto Suture, US Surgical; Tyco Healthcare Group, Norwalk, CT) sutures. Group 2 included patients who had a metal stabilizer placed because pericostal sutures could not be used bilaterally. We compared the two groups in terms of bar dislocation rate, surgical operative time, and other complications. Statistical analysis was performed with the Mann-Whitney U test and Fisher's exact test.Fifty-seven patients were included in the study: 37 in Group 1 and 20 in Group 2. Two patients from Group 2 had a bar dislocation. Statistical analysis showed no difference between the two groups in dislocation rate or other complications.In our experience, the placement of a metal stabilizer did not reduce the rate of bar dislocation. Bar stabilization by the pericostal Endo Close suture technique appears to have no increase in morbidity or migration compared with the metal lateral stabilizer technique.

Background and Aims: Recent epidemiological studies showed an increase in ulcerative colitis among children, especially in its aggressive form, requiring surgical treatment. Although medical therapeutic strategies are standardized, there is still no consensus regarding indications, timing and kind of surgery. This study aimed to define the surgical management of paediatric ulcerative colitis and describe attitudes to it among paediatric surgeons. Methods: This was a retrospective cohort study. All national gastroenterology units were invited to participate. From January 2009 to December 2013, data on paediatric patients diagnosed with ulcerative colitis that required surgery were collected. Results: Seven units participated in the study. Seventy-one colectomies were performed (77.3% laparoscopically). Main surgical indications were a severe ulcerative colitis attack (33.8%) and no response to medical therapies (56.3%). A three-stage strategy was chosen in 71% of cases. Straight anastomosis was performed in 14% and J-pouch anastomosis in 86% of cases. A reconstructive laparoscopic approach was used in 58% of patients. Ileo-anal anastomosis was performed by the Knight–Griffen technique in 85.4% and by the pull-through technique in 9.1% of patients. Complications after colectomy, after reconstruction and after stoma closure were reported in 12.7, 19.3 and 35% of cases, respectively. Conclusions: This study shows that there is general consensus regarding indications for surgery. The ideal surgical technique remains under debate. Laparoscopy is a procedure widely adopted for colectomy but its use in reconstructive surgery remains limited. Longer follow-up must be planned to define the quality of life of these patients.

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.

We describe a severe injury occurred after Nuss bar removal. Two years after Nuss procedure, a bar flipping was noted. The bar was easily removed. A massive right hemothorax occurred: a middle lobe laceration was noted, and lobectomy was performed. A review of the literature showed some case-reports of major complications during or after bar removal operation, occurred after bar flipping or in patients with lung or heart related morbidities. Aim of this article is to report our experience, to focus on risk factors for complication during bar removal and to discuss the introduction of thoracoscopy to reduce the risks.

Severe tracheomalacia presents a significant challenge for Paediatricians, Intensivists, Respiratory Physicians, Otolaryngologists and Paediatric Surgeons. The treatment of tracheomacia remains controversial, but aortopexy is considered by most to be one of the best options. We conducted a review of the English literature relating to aortopexy. Among 125 papers, 40 have been included in this review. Among 758 patients (62% males) affected with tracheomalacia, 581 underwent aortopexy. Associated co-morbidities were reported in 659 patients. The most frequent association was with oesophageal atresia (44%), vascular ring or large vessel anomalies (18%) and innominate artery compression (16%); in 9% tracheomalacia was idiopathic. The symptoms reported were various, but the most important indication for aortopexy was an acute life-threatening event (ALTE), observed in 43% of patients. The main preoperative investigation was bronchoscopy. Surgical approach was through a left anterior thoracotomy in 72% of patients, while median approach was chosen in 14% and in 1.3% a thoracoscopic aortopexy was performed. At follow-up (median 47 months) more than 80% of the patients improved significantly, but 8% showed no improvement, 4% had a worsening of their symptoms and 6% died. Complications were observed in 15% of patients, in 1% a redo aortopexy was deemed necessary. In our review, we found a lack of general consensus about symptom description and evaluation, indications for surgery, though ALTE and bronchoscopy were considered by all an absolute indication to aortopexy and the gold standard for the diagnosis of tracheomalacia, respectively. Differences were reported also in surgical approaches and technical details, so that the same term “aortopexy” was used to describe different types of procedures. Whatever approach or technique was used, the efficacy of aortopexy was reported as high in the majority of cases (more than 80%). A subgroup of patients particularly delicate is represented by those with associated gastro-esophageal reflux, in whom a fundoplication should be performed. Other treatments of tracheomalacia, particularly tracheal stenting, were associated with a higher rate of failure, severe morbidity and mortality. Non english abstract La tracheomalacia severa rappresenta una sfida per Pediatri, Intensivisti, Pneumologi, Otorinolaringoiatri, Chirurghi Pediatri. Il trattamento della tracheomalacia è tuttora controverso. L’aortopessi è considerata da molti la migliore opzione terapeutica. Abbiamo condotto una revisione della letteratura di lingua inglese su tale argomento. Di 125 lavori, 40 sono stati inclusi nella revisione. Tra 758 pazienti (62% maschi) affetti da tracheomalacia, 581 sono stati sottoposti ad aortopessi tra il 1968 e il 2008. In 659 pazienti alcune comorbidità erano presenti. L’associazione più frequente era con l’atresia esofagea (44%), l’anello vascolare o un’anomalia dei grossi vasi (18%), la compressione da parte dell’arteria innominata (16%); nel 9% la tracheomalacia era idiopatica. I sintomi riportati sono stati variabili, ma l’indicazione più importante all’aortopessi sono stati eventi di ALTE, osservati nel 43% dei pazienti. Lo studio diagnostico preoperatorio principale è stato la broncoscopia. L’approccio chirurgico è avvenuto attraverso una toracotomia anteriore sinistra nel 72% dei pazienti, mentre un approccio mediano è stato scelto nel 14% e nell’1.3% dei casi è stato eseguito un approccio toracoscopico. Al follow-up (mediana di 47 mesi) la maggioranza dei pazienti sono migliorati significativamente, ma l’8% di essi non è migliorato, il 4% è peggiorato e il 6% è morto. Complicazioni sono state riportate nel 15% dei pazienti, nell’1% un nuovo intervento di aortopessi è stato necessario. In questa revisione abbiamo trovato che non c’è un consenso generale sulla valutazione e sulla descrizione dei sintomi, sulle indicazioni chirurgiche ed esami preoperatori, anche se le ALTE e la broncoscopia venivano considerate rispettivamente un’indicazione assoluta all’aortopessi e il “gold standard” diagnostico per la tracheomalacia. Venivano riportate differenze negli approcci chirurgici e nei dettagli tecnici, e lo stesso termine di aortopessi veniva usato per indicare diverse procedure chirurgiche. In ogni caso, indipendentemente dall’approccio o tecnica utilizzati, l’efficacia dell’aortopessi veniva riportata come elevata nella maggioranza dei casi (più dell’80%). Un sottogruppo di pazienti particolarmente delicato è rappresentato da quelli con reflusso gastroesofageo associato, nei quali sarebbe indicata una fundoplicatio. Altri trattamenti della tracheomalacia, quali stent tracheale, sembrano gravati da una maggiore percentuale di insuccessi, morbidità severa e mortalità.

Background congenital and acquired airway anomalies represent a relatively common albeit challenging problem in a national tertiary care hospital. In the past, most of these patients were sent to foreign Centres because of the lack of local experience in reconstructive surgery of the paediatric airway. In 2009, a dedicated team was established at our Institute. Gaslini's Tracheal Team includes different professionals, namely anaesthetists, intensive care specialists, neonatologists, pulmonologists, radiologists, and ENT, paediatric, and cardiovascular surgeons. The aim of this project was to provide these multidisciplinary patients, at any time, with intensive care, radiological investigations, diagnostic and operative endoscopy, reconstructive surgery, ECMO or cardiopulmonary bypass. Aim of this study is to present the results of the first year of airway reconstructive surgery activity of the Tracheal Team. Methods between September 2009 and December 2010, 97 patients were evaluated or treated by our Gaslini Tracheal Team. Most of them were evaluated by both rigid and flexible endoscopy. In this study we included 8 patients who underwent reconstructive surgery of the airways. Four of them were referred to our centre or previously treated surgically or endoscopically without success in other Centres. Results Eight patients required 9 surgical procedures on the airway: 4 cricotracheal resections, 2 laryngotracheoplasties, 1 tracheal resection, 1 repair of laryngeal cleft and 1 foreign body removal with cardiopulmonary bypass through anterior tracheal opening. Moreover, in 1 case secondary aortopexy was performed. All patients achieved finally good results, but two of them required two surgeries and most required endoscopic manoeuvres after surgery. The most complex cases were the ones who had already been previously treated. Conclusions The treatment of paediatric airway anomalies requires a dedicated multidisciplinary approach and a single tertiary care Centre providing rapid access to endoscopic and surgical manoeuvres on upper and lower airways and the possibility to start immediately cardiopulmonary bypass or ECMO. The preliminary experience of the Tracheal Team shows that good results can be obtained with this multidisciplinary approach in the treatment of complicated cases. The centralization of all the cases in one or few national Centres should be considered.