Megacystis-microcolon-intestinal hypoperistalsis syndrome: don't forget the bladder.

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.

Lower Urinary Tract Dysfunction in Pediatric Patients with Multiple Sclerosis: Diagnostic and Management Concerns.

BACKGROUND: Multiple sclerosis (MS) is increasing in the pediatric population and, as in adults, symptoms vary among patients. In children the first manifestations can sometimes overlap with acute neurological symptoms. Urological symptoms have not been much studied in childhood. We shared our experience with MS urological manifestation in children. METHODS: This article is a retrospective evaluation of all children with MS, according to the Krupp criteria, who also present with urological symptoms. We collected demographic and clinical history, the MR localization of demyelinating lesions, urological symptoms, and exams. RESULTS: We report on six MS pediatric cases with urological manifestation. Urinary symptoms, characterized by urinary incontinence in five patients and urinary retention in one patient, appeared in a different time frame from MS diagnosis. Urodynamic exams showed both overactive and underactive bladder patterns. Treatment was defined according to lower urinary tract dysfunction, using clean intermittent catheterization, oxybutynin, and intradetrusor Onabotulinum Toxin-A injection. A low acceptance rate of invasive evaluation and urological management was observed. CONCLUSIONS: The MS diagnosis was traumatic for all our patients. We believe it is important to address urological care in young people from the time of diagnosis for prompt management; it could be useful to include a pediatric urologist in multidisciplinary teams.

Thulium Laser for the Treatment of Posterior Urethral Valves in Infants.

Objective: Thulium laser (ThL) has become popular in urology, because of its powerful action on tissue, achieving optimal ablation and hemostasis. Aim of our article was to evaluate efficacy of ThL in infants affected by posterior urethral valve (PUV) ablation. 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 to 8 months after initial treatment. Several factors, including age and weight at surgery, operative time, postoperative bleeding, catheterization period, postoperative urinary retention, retreatment 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) and mean weight at primary surgery was 5.7 kg (2.5-10.3 kg). Mean operative time was 29.5 minutes (range 15-50 minutes). None of the patients experienced intraoperative and postoperative bleeding. In all cases, postoperative catheterization period was 1 day. Residual valves were found in 6 of 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 than standard treatments (electrofulguration, cold knife) and comparable with 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.

Hypospadias management in children with anorectal malformation: a multidisciplinary single center experience.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.

Onabotulinum Toxin A Intradetrusor Injections in Children with Neurogenic Lower Urinary Tract Dysfunction: Long-Term Histological Effects on the Bladder Wall.

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.

Magnamosis for long gap esophageal atresia: Minimally invasive "fatal attraction".

BACKGROUND: Aim of study is to report our preliminary experience with magnetic anastomosis (magnamosis) treating long-gap esophageal atresia (LGEA), the most challenging condition of esophageal atresia continuum. Magnamosis has been reported in 20 patients worldwide as an innovative and marginally invasive option. METHODS: Prospective evaluation of all LGEA patients treated with magnamosis was performed (study registration number: 2535/2021). Main outcomes considered were demographic and surgical features, postoperative complications and feeding within 6-month of follow-up. RESULTS: Between June 2020 and January 2021, 5 LGEA patients (Type A, Gross classification) were treated. Median preoperative gap was 5 vertebral bodies. Magnetic bullets were placed at an average age of 81 days of life, leading to successful magnamosis in all cases: 4 infants had primary magnetic repair (one after thoracoscopic mobilization of the pouches), 1 patient had a delayed magnamosis after Foker's procedure. Esophageal anastomosis was achieved after an average of 8 days. No anastomotic leak was found. All patients developed anastomotic stenosis at 6-month follow-up, requiring a mean of 6 dilations each. Full oral feeding was achieved in 3 patients, while 2 were still on oral-gastrostomy feeding. One patient experienced small esophageal perforation after dilation (3 months after magnamosis), distal to the anastomotic stricture and subsequently developed oral aversion. CONCLUSIONS: Our preliminary results suggest magnamosis a safe and effective minimally invasive option in patients with LGEA. Absence of postoperative esophageal leaks may represent a major advantage of magnamosis over conventional surgery, although possible high rate of esophageal stenosis should be further evaluated. LEVELS OF EVIDENCE: IV (Case series with no comparison group).

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Posterior urethral valves: Role of prenatal diagnosis and long-term management of bladder function; a single center point of view and review of literature.

Posterior Urethral Valves (PUV) are the most common cause of lower urinary tract obstruction. More severe forms are detected early in pregnancy (mainly type I), while other forms are usually discovered later in childhood when investigating lower urinary tract symptoms. Bladder dysfunction is common and is associated with urinary incontinence in about 55% (0%-72%). Despite the removal of the obstruction by urethral valve ablation, pathological changes of the urinary tract can occur with progressive bladder dysfunction, which can cause deterioration of the upper urinary tract as well. For this reason, all children with PUV require long-term follow-up, always until puberty, and in many cases life-long. Therefore, management of PUV is not only limited to obstruction relief, but prevention and treatment of bladder dysfunction, based on urodynamic observations, is paramount. During time, urodynamic patterns may change from detrusor overactivity to decreased compliance/small capacity bladder, to myogenic failure (valve bladder). In the past, an aggressive surgical approach was performed in all patients, and valve resection was considered an emergency procedure. With the development of fetal surgery, vesico-amniotic shunting has been performed as well. Due to improvements of prenatal ultrasound, the presence of PUV is usually already suspected during pregnancy, and subsequent treatment should be performed in high-volume centers, with a multidisciplinary, more conservative approach. This is considered to be more effective and safer. Primary valve ablation is performed after clinical stability and is no longer considered an emergency procedure after birth. During childhood, a multidisciplinary approach (pediatric urologist, nephrologist, urotherapist) is recommended as well in all patients, to improve toilet training, using an advanced urotherapy program with medical treatments and urodynamic evaluations. The aim of this paper is to present our single center experience over 30 years.

Appendicitis in COVID19 Pandemic Era. Early experience of an International Referral Center of Pediatric Surgery.

AIM: We describe treatments of acute appendicitis at "Bambino Gesù" Children's Hospital during the peak of the Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) pandemic in Italy. MATERIAL AND METHODS: From March 1st to May 31st, all suspected appendicitis admitted to hospital entered this study. Following Institutional COVID19-protocol, between March 1st-21st, only patients with respiratory symptoms and/or history of recent travel to risk areas received nasopharyngeal swab. From March 22nd to May 31st, protocol was adapted to worsening epidemic conditions and a pre-triage area has been arranged to accommodate all patients undergoing the swab. RESULTS: 14 out of 53 patients were hospitalized between march 1st-21st, 39 from march 22nd to may 31st. swab was performed in 2 patient of first group and in all of second. DISCUSSION: During the study period, no covid19-contagion occurred in hospital staff by covid19-patients. CONCLUSION: Our covid19-protocol protected staff and patients allowing the maintenance of our standard of treatment. KEY WORDS: Appendicitis, Children, Covid19, Sars-cov-2.

Uptake and Intracellular Trafficking Studies of Multiple Dye-Doped Core-Shell Silica Nanoparticles in Lymphoid and Myeloid Cells.

INTRODUCTION: Since most biologically active macromolecules are natural nanostructures, operating in the same scale of biomolecules gives the great advantage to enhance the interaction with cellular components. Noteworthy efforts in nanotechnology, particularly in biomedical and pharmaceutical fields, have propelled a high number of studies on the biological effects of nanomaterials. Moreover, the determination of specific physicochemical properties of nanomaterials is crucial for the evaluation and design of novel safe and efficient therapeutics and diagnostic tools. In this in vitro study, we report a physicochemical characterisation of fluorescent silica nanoparticles (NPs), interacting with biological models (U937 and PBMC cells), describing the specific triggered biologic response. METHODS: Flow Cytometric and Confocal analyses are the main method platforms. However TEM, NTA, DLS, and chemical procedures to synthesize NPs were employed. RESULTS: NTB700 NPs, employed in this study, are fluorescent core-shell silica nanoparticles, synthesized through a micelle-assisted method, where the fluorescence energy transfer process, known as FRET, occurs at a high efficiency rate. Using flow cytometry and confocal microscopy, we observed that NTB700 NP uptake seemed to be a rapid, concentration-, energy- and cell type-dependent process, which did not induce significant cytotoxic effects. We did not observe a preferred route of internalization, although their size and the possible aggregated state could influence their extrusion. At this level of analysis, our investigation focuses on lysosome and mitochondria pathways, highlighting that both are involved in NP co-localization. Despite the main mitochondria localization, NPs did not induce a significant increase of intracellular ROS, known inductors of apoptosis, during the time course of analyses. Finally, both lymphoid and myeloid cells are able to release NPs, essential to their biosafety. DISCUSSION: These data allow to consider NTB700 NPs a promising platform for future development of a multifunctional system, by combining imaging and localized therapeutic applications in a unique tool.

Urethral duplication in male epispadias: a very uncommon association.

BACKGROUND: Urethral duplication associated with epispadias is a rare malformation. Few cases are described in Literature. We report the experience of two centers to add to the literature. METHODS: A retrospective study was conducted in two Italian Centers. All patients with urethral duplication associated with epispadias, treated from 1997 to 2017 were included. The preoperative work-up included renal-urinary ultrasonography and voiding cystourethrogram. All patients underwent surgery according to the Mitchell-Caione technique. Cosmetic result, urinary continence and satisfaction degree of patients at the last follow-up were evaluated as outcomes. Six male patients with urethral duplication in epispadias were included. Two patients presented penile epispadias and four penopubic epispadias. Only one patient had urinary incontinence as presenting symptomatology. The diagnosis of urethral duplication was accidental during preoperative evaluation in the remaining five patients. RESULTS: At last follow-up (mean 8.3 years) all patients but one presented good cosmetic result, one patient presented mild stress urinary incontinence, one presented nocturnal enuresis. The physical genital appearance was improved in all patients. Urethral duplication in association with epispadias is a rare urogenital abnormality. No classification is universally accepted. CONCLUSIONS: Based on our experience, we believe that the presence of any duplication should be carefully searched during surgery for male epispadias.

A rare cause of acute scrotum in a child: torsion of an epididymal cyst. Case report and review of the literature.

Torsion of an epididymal cyst is an extremely rare cause of acute scrotum in children and in young boys. Its reported incidence is 5-20%. The treatment is usually conservative. Many cases (up to 60%) regress spontaneously, especially if below 3 cm. Few cases have been reported in Literature (7 cases/2018). We add our patient as eighth case. He was a 13-year-old boy who was admitted for acute scrotum. Ultrasound excluded torsion of the testis and he was managed for 5 day conservatively. Based on clinical history and physical exam, we decided to perform a prompt surgery that revealed a torsion of epididymal cyst that was confirmed by histological exam.

Low Weight Child: Can It Be Considered a Limit of Robotic Surgery? Experience of Two Centers.

Purpose: Robotic surgery should be considered a technical opportunity for many procedures in pediatric age. The purpose of this study is to verify the correlation between robotic procedures and low weight. Materials and Methods: It is a retrospective study (2013-2017). We included all patients who underwent robotic assisted surgery for major procedures in two Italian centers for pediatric surgery. All patients were divided into two groups (group A ≤15 kg and group B > 15 kg). Parameters considered were as follows: time to console, average time procedures, time of hospitalization, and minor/major complications. Statistical test used was Wilcoxon-Mann-Whitney test. Results: We enrolled 83 patients. Group A: 28 patients, medium weight 11.2 kg (range: 7-15 kg). Group B: 55 patients, average weight 40.8 kg (range: 15.2-120 kg). In relation to the outcomes considered, we found statistical significance in relation to surgical time and time at the console. Conclusions: The retrospective analysis of our case found that body weight cannot be considered an absolute contraindication for the execution of surgical procedures in robotics. The improvement of instruments permits to perform complex surgical procedures in low-weight children without additional difficulties. However, it is important to point out that the youngest child of our case had a weight of 7 kg.

Computational Assessment of Facial Expression Production in ASD Children.

In this paper, a computational approach is proposed and put into practice to assess the capability of children having had diagnosed Autism Spectrum Disorders (ASD) to produce facial expressions. The proposed approach is based on computer vision components working on sequence of images acquired by an off-the-shelf camera in unconstrained conditions. Action unit intensities are estimated by analyzing local appearance and then both temporal and geometrical relationships, learned by Convolutional Neural Networks, are exploited to regularize gathered estimates. To cope with stereotyped movements and to highlight even subtle voluntary movements of facial muscles, a personalized and contextual statistical modeling of non-emotional face is formulated and used as a reference. Experimental results demonstrate how the proposed pipeline can improve the analysis of facial expressions produced by ASD children. A comparison of system's outputs with the evaluations performed by psychologists, on the same group of ASD children, makes evident how the performed quantitative analysis of children's abilities helps to go beyond the traditional qualitative ASD assessment/diagnosis protocols, whose outcomes are affected by human limitations in observing and understanding multi-cues behaviors such as facial expressions.

Preputialplasty: can be considered an alternative to circumcision? When, how, why? Experience of Italian centre.

BACKGROUND: Phimosis is a condition in which the foreskin cannot be portrayed on the glans. It is a physiological and common condition in the pediatric age. The pathological form derives from an inflammatory or traumatic lesion. Circumcision is the most common surgical treatment of phimosis but it is a controversial practice, especially in occidental world. METHODS: We enrolled 61 patients with pathological phimosis (22/balanoposthitis, 18/painful erection, 21/urinary discomfort) between 2015-2017. RESULTS: All patients underwent preputialplasty. CONCLUSIONS: Various alternatives to circumcision have been described, as manual retraction therapy, topical steroid therapy, and many types of preputialplasty. We report our technique.

Comparison of primary total hip replacements performed with a direct anterior approach versus the standard lateral approach: perioperative findings.

BACKGROUND: Given the increasing demand for tissue-sparing surgery, the surgical approach is the subject of lively debate in total hip replacement. The aim of this paper is to compare the efficacy of the minimally invasive direct anterior approach and the standard lateral approach to total hip replacement surgery by observing intra- and perioperative outcomes. MATERIALS AND METHODS: The authors conducted a retrospective study on a group of 419 consecutive patients undergoing total hip replacement for coxarthrosis. The patients were divided into a first group (A) of 198 patients who had surgery with the standard lateral approach, and a second control group (B) of 221 patients who had the same procedure via the minimally invasive direct anterior approach. Assessment of the two groups considered the following perioperative parameters: length of the surgical procedure, intraoperative complications, intra- and postoperative blood loss, postoperative pain, postoperative nausea and vomiting, length of stay, and type of discharge. RESULTS: The two groups were homogeneous when compared in relation to mean age, sex and body weight. The minimally invasive direct anterior approach was performed within an acceptable time (89 ± 19 min vs. 81 ± 15 min) and with modest blood loss (3.1 ± 0.9 g/dL vs. 3,5 ± 1 g/dL). Patients experienced less pain (1.4 ± 1.5 NRS score vs. 2.5 ± 2 NRS score), and PONV affected only 5% versus 10% of cases. Times to discharge were shorter (7 ± 2 days vs. 10 ± 3.5 days), and 58.4% versus 11.6% of patients were discharged to home. CONCLUSIONS: In our study, patients treated with a minimally invasive direct anterior approach had a better perioperative outcome than patients treated with the lateral approach. The longer time of surgery for the minimally invasive direct anterior approach may be attributed to the learning curve. Further studies are necessary to investigate the advantages of a minimally invasive direct anterior approach in terms of clinical results in the short and long run.