Background: In the last twenty-five years, Onabotulinum Toxin A (BTX-A) has gained increasing popularity for neurogenic lower urinary tract dysfunction (NLUTD) treatment. To maintain its efficacy, repeated BTX-A intradetrusor injections are required over time, with unknown effects on the bladder wall in children. The aim of this paper is to report long-term effects on the bladder wall in children treated with BTX-A. Methods: Children with NLUTD not responsive to anticholinergics were treated with BTX-A, according to our protocol, with bladder wall control using endoscopic cold-cup biopsy. Specimens were evaluated considering edema, chronic inflammation, and fibrosis. Results: Of the 230 patients treated from 1997 to 2022, we considered only specimens obtained in patients who had received ≥5 treatments (36 children), considered as the threshold to evaluate clinical effectiveness on long-term treatment with BTX-A. Most of them had congenital NLUTD (25 patients) and detrusor overactivity (27 patients). In all, increased edema and chronic inflammation with reduced fibrosis over time was reported; these data were not statistically significant. No difference was observed between patients with congenital and acquired diseases. Conclusions: Repeated intradetrusor BTX-A injections are not related to significant histological alterations in children, similarly with adults, and repeated injections could be considered safe.

Introduction Jeune's syndrome, or asphyxiating thoracic dystrophy (ATD), is a rare autosomal recessive disorder characterized by skeletal dysplasia. Ribs are typically short and horizontal resulting—in lethal variant—in severe lung hypoplasia, progressive respiratory failure, and death. Lateral thoracic expansion (LTE) consists in staggered bilateral ribs osteotomy leading to chest expansion and lung development. Studies on LTE in ATD patients report encouraging data, but the rarity of ATD implies the lack of a standardized surgical path. The aim of this report is to present our experience with LTE, the technical modification we adopted, and patients' clinical outcome. Materials and Methods We retrospectively reviewed data of 11 LTE performed in 7 ATD patients with lethal variant. Information regarding pre- and postoperative clinical conditions and surgical details was collected. We adopted a single-stage or a two-stage approach based on patient clinical condition. Computed tomography (CT) scan was performed before and after surgery and lung volume was calculated. Results Five patients are alive, while two died in intensive care unit for other than respiratory cause (sepsis). Most patients experienced clinical improvement in terms of decreased respiratory infections rate, need for ventilation, and improved exercise tolerance. Postoperative CT scan demonstrated a median lung volume increase of 88%. Conclusion Mortality in ADT patients is high. However, LTE is a feasible and safe surgical approach, which could improve clinical conditions and survival rate. Survived patients showed postoperatively less oxygen requirement and improved clinical conditions.

Objective: Thulium Fiber Laser (ThL) has become popular in Urology, due to its powerful action on tissue, achieving optimal ablation and hemostasis. Aim of our paper has been to evaluate efficacy of Thulium laser (ThL) ablation in infants affected by posterior urethral valve (PUV). Patients and Methods: Clinical charts of 25 infants (age  12 months) who underwent PUV ablation were retrospectively reviewed. According to our protocol all patients performed Voiding cystourethrography and cystoscopy 6-8 months after initial treatment. Several factors, including age and weight at surgery, operative time, postoperative bleeding, catheterization period, postoperative urinary retention, re-treatment for valve remnants and stricture at follow-up were evaluated. Preoperative, intraoperative and postoperative data were analyzed. Results: Mean age at primary surgery was 4.5 months (5 days – 10.5 months), mean weight at primary surgery was 5.7 Kg (2.5-10.3 Kg). Mean operative time was 29.5 minutes (15-50 minutes). None of the patients experienced intraoperative and post-operative bleeding. In all cases, post-operative catheterization period was one day. Residual valves were found in 6/25 (24%) patients. No cases of urethral stricture were registered during follow-up (48.4 months, range: 11 - 95). Analyzing literature data using other techniques, complication rate of ThL PUV ablation seems lower compared to standard treatments (electrofulguration, cold knife) and comparable to those reported with other laser techniques. Conclusion: PUV ablation with ThL has proven to be feasible and safe in infants. Further studies are needed to define the real effectiveness of this laser technology in PUV ablation. Miniaturized instruments and ThL technology make early PUV treatment feasible also in low body weight newborns.

Background: In pediatric age, the central airways are more flexible and mobile, with tracheal and bronchial walls easily tending to collapse, allowing partial or complete occlusion of the lumen: a situation described as tracheobronchomalacia (TBM). This is a condition that causes an increase in intrathoracic pressure that may accentuate airway collapse, and a biphasic or barking cough appears. Objectives: Although TBM is relatively frequent in pediatric age, the diagnostic criteria and subsequent treatment do not follow well-standardized criteria and often vary from pediatric center to center. Therefore, there is a need to standardize diagnostic procedures and the resulting medical or surgical treatments. Methods: We therefore organized a day of meetings to talk about TBM, inviting all Italian pediatricians and pediatric surgeons who diagnose and treat patients with this pathology on a daily basis. Results: This work, collecting all the meeting interventions, is a compendium that deals with all aspects of TBM, emphasizing the most correct criteria to diagnose and therefore best treat each pediatric patient with this clinical condition. We give particular emphasis to the need to perform static and dynamic videobronchoscopy (S/DVBS) to verify the patency of the tracheal lumen, so as to evaluate the severity of TBM. Conclusions: this work deals with TBM in all its diagnostic and treatment aspects and can be a valid help for all pediatricians who treat these patients.

Background: In pediatric age, the central airways are more flexible and mobile, with tracheal and bronchial walls easly tending to collapse, allowing partial or complete occlusion of the lumen: a situation described as tracheobronchomalacia (TBM). Any condition that causes an increase in intrathoracic pressure are situations in which airway collapse is accentuated and biphasic or barking cough appears. Objectives: Although the situation of TBM is relatively frequent in pediatric age, the diagnostic criteria and subsequent treatment do not follow well-standardized criteria, and often vary from pediatric center to center. Therefore, there is a real need to standardize diagnostic procedures and the resulting medical or surgical treatments. Methods: We therefore organized a day of meeting to talk about TBM, inviting from all Italy pediatricians and pediatric surgeons that diagnose and treat patients with this pathology on daily basis. Results: This work, collecting all the meeting interventions, is a compendium that truly deals with TBM in all its aspects, emphasizing above all the most correct criteria to diagnose and therefore best treat each individual pediatric patient with such clinical condition. We give particular emphasis to the need to perform static and dynamic videobronchoscopy (S/DVBS) to verify the patency of the tracheal lumen, so as to evaluate the severity of TBM, when the patient increases intrathoracic pressure. Conclusion: this work deals with TBM in all its diagnostic and treatment aspects and can be a valid help for all pediatricians who treat this pathology.