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.

The digital era of pediatric urological clinical care: telemedicine for management of lower urinary tract symptoms and nocturnal enuresis - a comprehensive tertiary center insight.

BACKGROUND: Lower urinary tract symptoms (LUTS) and nocturnal enuresis (NE) are complex conditions requiring a long-term follow-up. Telemedicine is an emerging technological tool in the surgical field, and its availability exponentially grew during the COVID-19 pandemic, expanding its application fields, optimizing technical aspects, reducing costs, and ensuring high-quality standards. This work describes our experience with telemedicine in a Division of Pediatric Urology for the follow-up of enuresis and LUTS. METHODS: A retrospective analysis of our telemedicine preliminary experience was conducted at Regina Margherita Children's Hospital in Turin, Italy. This study included all the patients aged <18 years who were monitored for enuresis and LUTS through telemedicine between September 1, 2021 and July 31, 2023. Clinical data and outcomes were analyzed, and patients and families were asked to voluntary fill an evaluation questionnaire on their satisfaction. Additionally, we focused on the post COVID-19 period, between September 2022 and July 2023, analyzing the data of two different patients' populations: the first one (G1) of patients choosing telemedicine outpatients visits (TOVs) and the second one (G2) of those choosing a face-to-face visit. RESULTS: One hundred five patients were enrolled. One hundred sixty-two TOVs were performed. The median age at first visit was 9.7±0.66 years (range 7-16 years). Diagnosis were: 77/105 (67%) NE and 28/105 (33%) LUTS. The average referred distance between the patients' residence and the hospital was 46.35±129.37 km (range 2-1300 kilometers) and the time taken to overcome it was 44.21±77.29 minutes (range 10-780 minutes). In 64/105 cases (61%) the follow-up was interrupted for total healing or symptoms resumption. Only two cases (2%) required the conversion to an in-person ambulatory consult, due to a social-linguistic barrier. 146/162 families (90%) filled the survey questionnaire at the end of each TOV, reporting in 94% of cases a high satisfaction level. In the comparative statistical analysis of the two patient groups, G1 (52 telemedicine office visits, [TOVs]) vs. G2 (25 face-to-face visits), the average referred distance was 17.78±7.98 km (range: 5-35 km) for G1, contrasting with 7.04±3.35 km (range: 2.5-14 km) for G2 (P=0.00001). Additionally, the waiting time before the visit was 3.96±2.90 minutes (range: 0-10 minutes) for G1, in contrast to 26.52±11.22 minutes (range: 5-44 minutes) for G2 (P=0.00001). Furthermore, a higher compliance with behavioral or pharmacological indications was observed in the G1 group, exhibiting lower adherence in only 12 out of 52 cases compared to 14 out of 25 cases in G2 (P=0.0091). CONCLUSIONS: Telemedicine is a proper solution and an effective tool to manage the therapeutic follow-up of NE and LUTS, ensuring suitable quality standards and reducing social costs, such as the loss of working days and transport costs. The implementation and complete integration of its use into the healthcare system should be the goals to pursue in order to take full advantage of all its potentials.

Retrograde Intrarenal Surgery for Renal Stones: Is It a Safe and Effective Option in Preschool Children?

INTRODUCTION: Few studies in the literature describe the Retrograde Intra-Renal Surgery (RIRS) outcome in preschool children. We evaluated the feasibility, stone-free rate and complications of RIRS in preschool children at two European tertiary care centres of Pediatric Urology. MATERIAL AND METHODS: The retrospective study includes all children undergone RIRS for stones <25 mm from 2017 to 2022. Patients were divided into Group 1 <5 years (G1) and Group 2 >5 years (G2). Semirigid ureterorenoscope 4.5-6.5 Ch and a 7.5 Fr flexible ureteroscope with a 9.5/11 Ch ureteral access sheath (UAS) were used. Stone-free rate (SFR) was evaluated at 3 months. Fischer/Chi-square test for qualitative data and Mann-Whitney for quantitative data were used for statistical analysis. RESULTS: 63 patients underwent RIRS, 19 G1-patients, median age 3.55 ± 1.06 years (range 1.5-5 years), and 44 G2-patients, median age 11.25 ± 2.95 (range 6-17 years) (p < 0.00001). Intraoperative complications occurred in 1 case in G1(5%) and 3 in G2(7%) (p = 1): two minor ureteric injuries in G2 were treated by a prolonged JJ-stent. Postoperative fever was reported in 3 cases in G1 (16%) and 4 in G2(9%) (p = 0.42), while post-operative hematuria in 4 G1-patients (21%) and in 7 G2-patients (16%) (p = 0.72). SFR was 84.2% in G1 and 88.6% in G2. At an average follow-up of 15.05 ± 4.83 months in G1 and 19.95 ± 10.36 months in G2, reintervention for residual stones was necessary in 3 cases in G1(16%) and in 6 cases in G2(14%) (p = 1). CONCLUSIONS: In a European country with low-volume pediatric stone centers, RIRS is a promising therapeutic option in young children as it offers acceptable stone-free rate and a low incidence of high-grade complications. LEVEL OF EVIDENCE: III.

JJ stent dislodgement in the distal ureter: how to recover it in preschool children?

Double-J ureteral stents are usually placed after various urological procedures. The dislodgement of their distal ringlet is a rare complication, whose retrieval is arduous in younger children, due to the small ureteral caliber. We propose our innovative endoscopic approach to recover the dislodged JJ stent. Under 8-9.8 Ch cystoscopy, the ureteral meatus is gently cannulated with a 00.18″ guidewire, then a balloon catheter Passeo 18 3-4 mm (Biotronik, Lake Oswego, OR, USA) is coaxially inserted. A pneumatic dilatation of the vesical-ureteral junction is performed up to 8 atmospheres for 5 minutes under direct vision. Consequently, the ureteral meatus allows the cystoscope passage, and the JJ-stent can be recovered thanks to endoscopic grasping forceps. A mono-J stent is then left in place for 24 hours. Four patients aged 8 months - 4 years have been successfully treated with this approach after that JJ migration was found intraoperatively or during ultrasonography. No intra- or postoperative complications occurred. Postoperative hospital stay was prolonged for one day. During 29.5 medium follow-up no clinical or ultrasonographic signs of vesical-ureteral reflux ensued. Our cystoscopic approach is effective and safe to ensure a prompt endoscopic JJ retrieval without changing neither surgical approach nor the anesthesiological support. We believe that all the pediatric urology centers should know the procedure and have small size balloon catheter available.

Robot-assisted laparoscopic pyeloplasty in a pediatric patient with horseshoe kidney: surgical technique and review of the literature.

OBJECTIVE: The aim of this study was to describe the technical aspects of a robotic pyeloplasty in pediatric patients with uretero-pelvic junction obstruction (UPJO) in horseshoe kidney (HSK) through the report of our recent case, and to outline the state of the art of minimally invasive pyeloplasty (MIP) with a systematic review of the literature. METHODS: We describe all the steps of our surgery performed on an 11-year-old patient with left UPJO in HSK in detail. All the anatomic landmarks are clearly showed, with particular attention to trocar placement in a pediatric patient. A systematic review of the literature on the outcomes of MIP in HSK patients has been performed, including 16 articles. RESULTS: Our surgery was successful following a standardized approach. However, we recorded a rare complication, the herniation of a small portion of omentum through the 8 - mm defect used for the caudal robotic port. The evidence synthesis shows excellent postoperative outcomes for both laparoscopic and robot-assisted laparoscopic pyeloplasties in HSK patients. Probably, MIP should be preferred to the traditional open approach in these patients, when feasible. CONCLUSIONS: Transperitoneal robotic pyeloplasty is an excellent minimally invasive choice for the treatment of UPJO in HSK pediatric patients. The pediatric setting should prompt attention to every detail to avoid unfortunate complications.

Original Koyanagi urethroplasty versus modified Hayashi technique: outcome in 57 patients.

OBJECTIVE: To compare outcomes of the original Koyanagi technique with the Hayashi modification in severe hypospadias, i.e. hypospadias with a division of the corpus spongiosum located behind the penile midshaft associated with marked hypoplasia of the ventral aspect of the penis and a non-preservable urethral plate. Choice of technique for treating severe hypospadias is discussed. PATIENTS AND METHODS: Twenty-six patients (group I) were operated in Lyon using the original Koyanagi procedure and 31 (group II) underwent a modified Hayashi procedure in Paris. Most patients received preoperative androgenic stimulation. Mean follow up was 25 months (group I) and 34 months (group II). RESULTS: Sixteen patients (61.5%) in group I and 19 (61.3%) in group II developed complications leading to 21 additional procedures in each group. Urethral dehiscence was found in 11/26 patients in group I (42.3%), and in 6/31 patients in group II (19.3%); fistulae were respectively found in 5/26 (19.2%) and 12/31 (38.7%), and stenosis in 9/26 (34.6%) and 5/31 (16.1%) patients. Urethrocele was found in 7/26 (26.9%) and 5/31 (16%) patients, respectively. Twelve (group I) and 7 (group II) patients are awaiting re-operation. CONCLUSION: Although the complication rate is quite similar between the original and the modified Koyanagi procedure, the Hayashi technique seems to provide a better distal blood supply to the reconstructed urethra.

Outcome of severe hypospadias repair using three different techniques.

OBJECTIVE: To compare the outcomes of three different urethroplasty techniques (onlay, buccal mucosa, Koyanagi type I) used in the reconstruction of severe hypospadias. PATIENTS AND METHODS: Over 10 years (1997-2007), 300 severe hypospadias cases were treated with a mean follow up of 2 years (1-105 months); 203 were operated by the same surgeon of whom 184 completed follow up. Three main techniques were used according to the quality of the urethral plate: onlay urethroplasty (133), buccal graft urethroplasty (25) and Koyanagi type I (26). The mean age at surgery was 36 months (8-298); 76 required preoperative androgen stimulation (onlay 37, buccal 11, Koyanagi 26); 18 required a corporoplasty to straighten the penis (onlay 13, buccal 3, Koyanagi 2). RESULTS: Thirty-eight onlay (28.5%); 14 buccal (56%); 16 Koyanagi (61.5%) urethroplasties had a complication. The fistula rate was 15% for the onlay group; 32% for the buccal mucosa group; 19.2% for the Koyanagi cases. The dehiscence rate was, respectively, 11.3%, 20% and 42.3%. The stricture rate was, respectively, 1.5%, 20% and 34.6%. Urethrocele was found in seven Koyanagi patients. Final functional and cosmetic results were satisfactory in 126/133 (94.7%) onlay, 20/25 (80%) buccal and 14/26 Koyanagi (53.8%) urethroplasties. Primary cases had better results (89%) than redo cases (75.9%). Patients submitted to preoperative androgen therapy developed more complications (onlay: 40.5% vs 23.9%; buccal: 70% vs 43.7%). CONCLUSION: Two striking results are the low number of severe hypospadias cases requiring an additional corporoplasty, and the increased complication rate found in androgen-stimulated patients. The excellent results of the onlay procedure could be related to the use of dorsal preputial tissue, which in hypospadias is characterized by a well-balanced protein platform compared to the ventral tissues.

Management of severe hypospadias.

Many classifications of hypospadias have been published, mainly based on the position of the ectopic meatus, which is an insufficient criterion to define the severity of this malformation. What really marks the proximal landmark of this malformation is the level of division of the corpus spongiosum, which is always proximal to the ectopic meatus. In this article, we will focus on the most severe forms of hypospadias which include those with a proximal division of corpus spongiosum (below the midshaft), important chordee and a poor development of the ventral radius, reflecting a marked hypovirilization of the genital tubercle, and cripple hypospadias resulting from several previous failed surgical procedures. The principle of hypospadias surgery will be reviewed together with the outcome of the current surgical techniques. Furthermore, common complications will be outlined. There is no minor or major hypospadias and all forms require a solid experience of the surgeon, as minor looking hypospadias may turn out to be far more complex to repair than they appear once the ventral radius of the penis has been dissected.

Quality of life for adult women born with bladder and cloacal exstrophy: a long-term follow up.

OBJECTIVE: To assess the quality of life of 23 female patients over 18 years of age treated and followed in the same institution. METHODS: Fifty questions were sent to this group of patients covering their uro-gynaecological history, education and profession, social life and related psychological issues, sexual life and fertility. All replies were kept anonymous. RESULTS: Seventeen patients (74%) replied. Urine incontinence at various degrees was found in 65%. Genital prolapse was reported in 29.5%. Students accounted for 41%; 59% were actively working with a satisfactory professional life in 82% of cases. Seventy-six per cent did not consider that exstrophy affected their professional life, and 76% were happy with their social life. Psychological distress was reported by 41%, and the symptoms are detailed. Sixty-four per cent were married or had a stable partner. Seventy-six per cent were active sexually, all declaring heterosexual relations. Eighty-eight per cent had periods. Four pregnancies were reported and morbidity during gestation is described. All children were born by Caesarean section and were normal. These results are discussed and compared with the literature. CONCLUSION: Despite many hurdles, this group of patients can expect a subnormal social, family and professional life. Urinary incontinence and poor body image were the most important factors altering quality of life.

Potential determinant factors of sexual identity in ambiguous genitalia.

This is a review of literature scanning the potential factors which may affect Sexual Identity (S.I.) and Gender Identity (G.I.) in patients with ambiguous genitalia. Definitions of these concepts are outlined. Genetic, gonadal, hormonal, social and cultural pressures are reviewed as well as lessons to learn from clinical experiences and outcomes. Current criteriae used to assign gender in a child with ambiguous genitalia are discussed including medical and surgical criteriae as well as cultural disruptors. At the dawn of the third millennium, it is remarkable how little we know about the establishment of our individual and social identities.