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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.

Background:Ureteropelvic junction obstruction (UPJO) is one of the most frequent causes of congenital hydronephrosis. It is essential to distinguish UPJO which needs surgical treatment. fMRU combines high quality morphological details of the kidney and excretory pathways with functional data.ObjectiveThis study aims to introduce a new radiological score based on fMRU findings to be able to differentiate surgical from non-surgical kidneys.Materials and MethodsWe retrospectively selected patients with hydronephrosis due to UPJO who underwent fMRU (January 2009–June 2018). A multidisciplinary team identified a list of fMRU morpho-functional predictive variables to be included in the analysis. To evaluate the role of different independent variables in predicting the outcome, a multivariable logistic regression model has been performed; the outcome variable was the surgical intervention. For each predictive variable, Odds Ratio and 95% Confidence Intervals were calculated. The likelihood ratio test was used to assess the significance of the variables. Using the regression model, we assigned a numerical value to each predictive variable, rounding up the beta-coefficients. The cut-off value of the total score was obtained from the ROC curve analysis.ResultsA total of 192 patients were enrolled, corresponding to 200 pathological kidneys. All of them underwent fMRU; 135 were surgically treated, while 65 underwent ultrasound or MRU follow-up. Predictive variables significantly associated with surgery resulted to be the urographic phase, the presence of abnormal vessels, and a baseline anterior-posterior pelvic diameter >23 mm. Beta coefficients of the logistic regression model were then converted in scores. The ROC curve of the score showed high sensitivity (84.3%) and specificity (81.3%) with a cut-off > 2.5.ConclusionsWe propose a new fMRU score able to identify surgical vs. non-surgical kidneys with UPJO.

Robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR) and robotic ureteroureterostomy (RUU) are two mini-invasive surgical techniques that have begun to be performed in pediatric urology in recent years. RALUR has been employed especially for VUR treatment, while RUU is considered principally in case of complex doubled ureteral systems. Our aim is to discuss the safety and feasibility of these approaches in children, focusing on technical considerations and supporting their use in different anomalies and pathologies of the ureterovesical junction. We retrospectively collected data about 58 patients who underwent 44 dismembered RALUR (D-RALUR), 28 non-dismembered RALUR (ND-RALUR) and 5 RUU between May 2020 and December 2021. Indications for surgery were primary or secondary vesicoureteral reflux, megaureter, secondary UVJ obstructions, complicated doubled ureteral systems. Mean age was 3.5 years (range 0.6-12.9) and mean weight 17.1 (range 7.2-80). No intraoperative complications occurred nor conversion to open approach were reported. Major postoperative complications were reported in 11.7% of cases with a higher incidence for ND-RALUR. Mean hospital stay was 2.14 days (range 1-8). Success rate at the short-term follow-up was 91.9% for D-RALUR, 96.3% for ND-RALUR and 100% for RUU. RALUR and RUU are two feasible and safe procedures to perform in children. RALUR represents the most required and adequate technique in the treatment of UVJ pathologies, however, in selected cases RUU could represent an effective alternative that has to be considered.

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.

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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).

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.

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.