Does the distal ureteral diameter ratio (UDR) matter in the surgical management of vesicoureteral reflux in children?

PURPOSE: To evaluate UDR reliability, sensitivity, specificity and to identify the best treatment basing on UDR among single or double endoscopic injections and ureteral reimplantation. METHODS: Data of patients affected by primary VUR and treated by endoscopic injection over a 10 years period were retrospectively analyzed. Two radiologist attributed reflux grade and UDR on voiding cystourethrogram twice and blinded. Follow-up focused on resolution after 1 or 2 endoscopic injections. Relation between UDR, reflux grade and outcomes were analyzed. RESULTS: Patient enrolled were 198. Low grade VUR was present in 24.8%, grade 3 in 41.6%, grade 4-5 in 33.6%. Resolution after one injection was obtained in 88 patients; among 110 not resolved 104 cases had a second injection. Success after 2 injections was reported in 138 cases. UDR showed a higher reliability compared with reflux grade both in intra than inter-reader measurement (ICC > 90%). Success after 1 or 2 injections was reported for UDR < 0.33 and UDR < 0.47 respectively. CONCLUSION: UDR shows to be a more reliable measurement that allows for an objective estimation of VUR severity and prognosis. It represents a quantitative parameter that might be useful to identify patients who may benefit endoscopic or surgical treatment, avoiding unnecessary under or over-treatment.

An expert consensus on prevention, diagnosis, and management of hemorrhagic cystitis in pediatric hematopoietic cell transplantation, on behalf of the Infectious Disease and Hematopoietic Cell Transplant Working groups of Italian Pediatric Hematology Oncology Association (AIEOP).

The optimal management of hemorrhagic cystitis (HC) in hematopoietic stem cell transplantation (HCT) is debated, both for early onset HC (EOHC) secondary to chemotherapy toxicity and BK Polyomavirus (BKPyV)-related HC, due to the lack of controlled trials, particularly referred to pediatric setting. Actually, clinical practice is mainly based on guidelines of the European Conference on Infections in Leukemia, 6th edition, which considers both adult and pediatric populations but concludes that, despite much progress in understanding the pathogenesis, epidemiology, and risk factors, this complication still represents a disabling unmet clinical need with limited prophylactic and therapeutic options. Additionally, the Guidelines of the American Society of Clinical Oncology define the management of chemotherapeutic toxicity independently from the patients' population. A panel of experts belonging to the Hematopoietic Cell Transplant and Infectious Disease Working Group (WG) of Associazione Italiana di Emato-Oncologia Pediatrica (AIEOP) developed a consensus to define the best practices in prevention, diagnosis, and management of HC in pediatric HCT setting.

Robotic ureteral reimplantation and uretero-ureterostomy treating the ureterovesical junction pathologies in children: technical considerations and preliminary results.

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.

Near-miss events are really missed! Reflections on incident reporting in a department of pediatric surgery.

PURPOSE: 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. MATERIALS AND METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Functional Magnetic Resonance Urography in Ureteropelvic Junction Obstruction: Proposal for a Pediatric Quantitative Score.

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. Objective: This study aims to introduce a new radiological score based on fMRU findings to be able to differentiate surgical from non-surgical kidneys. Materials and Methods: We 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. Results: A 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. Conclusions: We propose a new fMRU score able to identify surgical vs. non-surgical kidneys with UPJO.

Surgical validation of functional magnetic resonance urography in the study of ureteral anomalies distal to the uretero-pelvic junction in a pediatric cohort.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Minimally Invasive Repair of Pectus Excavatum Without Bar Stabilizers Using Endo Close.

OBJECTIVES: 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. SUBJECTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series.

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.

Aortopexy for the treatment of tracheomalacia in children: review of the literature.

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.

Removal of pectus bar fixed with absorbable vs metallic stabilizers.

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.

Gaslini's tracheal team: preliminary experience after one year of paediatric airway reconstructive surgery.

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.