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PurposeEndoscopic treatment with high-pressure balloon dilatation (HPBD) has been proposed as a feasible, safe and minimally invasive procedure for primary obstructive megaureter, but the level of evidence is still low. This systematic review aims to evaluate the efficacy and safety of HPBD and its long-term results in paediatric population.MethodsThe review was conducted following the PRISMA guidelines. Systematic research of available literature in the English language from 1995 until June 2022 was conducted through EMBASE, MEDLINE, Cochrane Library and NIH Registry of Clinical Trials. For each relevant study, information was gathered regarding the study design, the inclusion/exclusion criteria, the indications for treatment, the success rate and the complications.ResultsThe final set included 13 studies. Median age of the patients ranged from 4 months to 7 years (full range 15 days-15 years). The indications for HPBD varied in different studies, however most authors included patients with increasing dilation, obstructive patterns found at diuretic renogram scan, decreased renal function or symptoms. All the studies analysed reported a success rate ranging from 69 to 100% for the treatment of POM with HPBD. This approach may avoid surgery in up to 77% of cases and is thought to be a less invasive, definitive and safe treatment for this disorder. The complication rate ranged from 0 to 50% and it was mostly infectious or associated to the stent placement or both.ConclusionHPBD demonstrated to be safe for the treatment of POM in infants, with effective long-term results and can be a definitive treatment in a considerable subset of patients. Nevertheless, the overall level of evidence for HPBD is still low and further comparative studies or randomized clinical trials are needed.

Complex kidney cysts are rarely observed in childhood. In adult patients, when radiological studies found a suspicious renal lesion, the gold standard is surgical asportation. The robotic surgery is well known as a secure procedure for treatment these patients, and is nowadays a real alternative also for pediatric patients. The challenges in children surgery are linked to anesthesiologic gestion, smaller operative fields, the need of specific instruments and more delicate tissue handling. We present a step-by-step video description of a robotic partial nephrectomy for a renal multicystic mass in a 4 year-old child.

Background The choice of the sex of rearing in patients with ovotesticular differences of sex development (OT-DSD) is difficult. The final decision should be given by the patient himself or herself, but families’ opinion is not to neglect especially when the diagnosis is precocious and the patient can’t give the consent to medical or surgical procedures. How should we behave if the parents refuse to raise a child with genital ambiguity? Case presentation We describe and comment on our multidisciplinary approach in three patients with neonatal diagnosis of OT-DSD. The families expressed a strong desire for that which concerned the sex of rearing of their babies in contrast to the International trend of “wait and see”. A specific counselling and a constant psychological support were given. Conclusions Recent trends suggest of postponing surgery to involve the patient in the decision. Child’s well-being is the goal of therapy. When medical and psychological support is not able to force parents to accept a child suffering from genital ambiguity, we think that it is better to opt for reversible medical/surgical treatments rather than allowing patients to grow up within a family that does not accept them.

Highlights • Small-bowel volvulus could be a presentation of a cystic mesenteric LM. • Diagnosis could be intraoperative. • Complete excision including, in some selected cases, a bowel resection, should be the treatment of choice., Introduction An acute abdomen in the form of small-bowel volvulus could be a presentation of a lymphatic malformation in childhood. Case presentation A 5 year old male was admitted to our Institute for an acute abdomen. Clinical aspects and radiological images were not specific for a certain diagnosis. Laparotomy revealed a big soft mass, with a milky content, completely involving about 50 cm of ileus with a partial volvulus of the intestinal loop. A complete mass excision and also a bowel involved resection were performed. After a histological examination, a lymphatic malformation was diagnosed. Discussion The diagnosis of a mesenteric lymphatic malformation could be intraoperative and a complete resection should be the treatment of choice. Sometimes it could be necessary to perform an involved bowel tract resection in the case of volvolus with ischemia. Conclusions Paediatricians and surgeons should bare in mind that an intrabdominal lymphatic malformation may present as a nonspecific an acute abdomen caused by a bowel volvolus and diagnosis may not be so simple preoperatively.

Summary Objective In principle, the prepuce can be reconstructed during hypospadias repair, but the procedure has not gained wide acceptance and preputial reconstruction (PR) is surrounded by several controversies. Material and methods A review is provided of the technique for PR, how PR combines with the other steps of hypospadias repair, the risks of complications related to the urethroplasty and specific to PR, and the results of PR with particular regard to the relevance for the patient and his family. Results PR can be important for patients requiring hypospadias repair and their parents. It can be performed in almost all patients with distal hypospadias except perhaps those with the most asymmetrical prepuces or severe ventral skin deficiency. PR does not seem to increase urethroplasty complications, but combination of PR with tubularisation of the urethral plate urethroplasty seems to offer the best chance of success. Specific complications occur in around 8% of patients and include partial or complete dehiscence of the prepuce and secondary phimosis. To prevent the latter, the reconstructed prepuce should be easily retractile at the end of surgery. Technical modifications can help to achieve this goal. Cosmetically, reconstructed prepuces are not fully normal, but the abnormality could be less important for a patient and his parents that the complete absence of the prepuce. Conclusion On the basis of the evidence summarised above, an algorithm for PR in patients with distal hypospadias is proposed. PR can be offered to the vast majority of distal hypospadias patients, although some modification of the technique for hypospadias repair can be required. Retractility of the reconstructed prepuce at the end of surgery seems paramount for final success.

Study Objective To report results of a retrospective multicentric Italian survey concerning the management of pediatric ovarian torsion (OT) and its recurrence. Design Multicenter retrospective cohort study. Setting Italian Units of Pediatric Surgery. Participants Participants were female aged 1-14 years of age with surgically diagnosed OT between 2004 and 2014. Interventions Adnexal detorsion, adnexectomy, mass excision using laparoscopy or laparotomy. Different kinds of oophoropexy (OPY) for OT or recurrence, respectively. Main Outcome Measures A total of 124 questionnaires were returned and analyzed to understand the current management of pediatric OT and its recurrence. The questionnaires concerned patient age, presence of menarche, OT site, presence and type of mass, performed procedure, OPY technique adopted, intra- and postoperative complications, recurrence and site, procedure performed for recurrence, OPY technique for recurrence, and 1 year follow-up of detorsed ovaries. Results Mean age at surgery was 9.79 ± 3.54 years. Performed procedures were open adnexectomy (52 of 125; 41.6%), laparoscopic adnexectomy (25 of 125; 20%), open detorsion (10 of 125; 8%), and laparoscopic detorsion (38 of 125; 30.4%). Recurrence occurred in 15 of 125 cases (12%) and resulted as significant ( P = .012) if associated with a normal ovary at the first episode of torsion. Recurrence occurred only in 1 of 19 cases after OPY (5.2%). Ultrasonographic results of detorsed ovaries were not significant whether an OPY was performed or not ( P = 1.00). Conclusion Unfortunately, oophorectomy and open technique are still widely adopted even if not advised. Recurrence is not rare and the risk is greater in patients without ovarian masses. OPY does not adversely affect ultrasonographic results at 1 year. When possible OPY should be performed at the first episode of OT.

Summary Introduction Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. Objective The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality. Study design 13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO). Results The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas ( Summary Table ). Discussion This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this research showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program. Conclusion This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people. Table . Areas Median percentile rank-pre pre-intervention Median percentile rank-post postintervention M F M F Related psycho-environmental situations 84* 89* 37 37 Body experience 98* 98* 46 38 Areas of pleasure 90* 90* 37 37 Medical and sexual history 99* 98* 40 40 Motivation and conflict 95* 95* 31 31

Introduction: We performed a systematic review of the literature on preputial reconstruction (PR) during hypospadias repair to determine the cumulative risk of preputial skin complications and the influence of PR on urethroplasty complications, namely fistula formation and overall reoperation rate of the repair. Material and Methods: A systematic search of the literature published after 06/1995 was performed in 06/2015 using the keyword “hypospadias”. Only studies on the outcome of PR in children, defined as dehiscence of the reconstructed prepuce or secondary phimosis needing circumcision, were selected. A meta-analysis of studies comparing PR vs. circumcision was performed for the outcomes “hypospadias fistula formation” and “reoperation rate”. Results: Twenty studies were identified. Nineteen reported the outcome of PR in 2115 patients. Overall, 95% (2016/2115) of patients undergoing PR had distal hypospadias. The cumulative rate of PR complications was 7.7% (163/2115 patients), including 5.7% (121/2115 patients) preputial dehiscences and 1.5% (35/2117 reported patients) secondary phimoses needing circumcision. A meta-analysis of 7 studies comparing patients undergoing PR vs. circumcision, showed no increased risk of urethral fistula formation associated with PR, Odds Ratio (Mantel-Haenszel, Fixed effect, 95% CI), 1.25 (0.80-1.97). Likewise, two studies comparing the overall reoperation rate did not show an increased risk of reoperation associated with PR, Odds Ratio (Mantel-Haenszel, Random effect, 95% CI), 1.27 (0.45-3.58). Conclusions: Preputial reconstruction carries an 8% risk of specific complications (dehiscence of reconstructed prepuce or secondary phimosis needing circumcision), but does not seem to increase the risk of urethroplasty complications, and the overall reoperation rate of hypospadias repair.

Summary Introduction The bladder exstrophy–epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life. Objective The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC. Study design Fourteen mothers of children aged 6–10 years and affected by BEEC (9 boys and 5 girls) were interviewed. Data on the mothers' experiences were collected through semi-structured interviews (Table). Results The qualitative analysis of the interviews showed that participants described experiences that were characterised by emotions such as fear and anger. Each mother had implemented a different and, sometimes, dysfunctional strategy in order to cope with the complex situation of the son/daughter. The aspects that most clearly emerged from mothers' descriptions were (1) the traumatic situation at the birth of the baby, (2) the sense of embarrassment concerning the pathological condition as the child was growing and the consequent sense of isolation of the mother, and (3) the fluctuation of feelings towards the multidisciplinary staff, which was sometimes seen as an important source of help and some other times as too destabilising and not helpful at all. Discussion The study provided some insight into the psychological and social conditions experienced by mothers of children with BEEC, which could serve as a basis for developing multidisciplinary teams with greater awareness about families living with this condition and better timing in addressing their needs. Conclusions Mothers of children with BEEC show emotional and social difficulties. This is a crucial aspect to consider when planning a multidisciplinary approach to the treatment/therapy, especially considering that children examined in this study are approaching adolescence. Table . Semi-structured interview topic. Number of questions 1 Impact of the diagnosis 4 2 Relationship with the medical team 2 3 Relationship with the partner 4 4 Social life: relationship with colleagues at work, relatives and friends 4

A lower urinary tract fistula consist in an abnormal connection between bladder, urethra and adjacent abdominal organs or skin. There are several types of urinary fistulas in paediatric age and they may be congenital or acquired. Etiology may be due to embriological defects, infectious processes, malignant tumours, pelvic irradiation as well as complications following surgical procedures, expecially postsurgical repair of hypospadia or epispadia. Clinical presentation depends on the type of fistula and diagnosis is based on signs, symptoms and radiological or endoscopic examinations. We performed PubMed research using terms such as lower urinary fistulae, urology and paediatrics and we consulted medical texts. We reviewed selected articles and used the relevant ones to perform our study concentrating on classification, diagnosis and treatment of different types of fistulas. Paediatric lower urinary fistulas are an uncommon pathology, but the knowledge of their etiology and classification is important to recognise them and lead the physician to an appropriate treatment, which is surgical in most cases.

An 18-year-old boy, followed up after seminal-sparing cystectomy for bladder rhabdomyosarcoma, presented complaining of recurrent episodes of left scrotal/inguinal pain arising after orgasms. Full work-up ruled out disease recurrence, but showed enlarged seminal vesicles. Ligation of the vas deferens was unsuccessful. The patient was started on α-blockers to reduce vas contractions with improvement of symptoms. The possible pathophysiology and treatments of this symptom are discussed.

Objective: The EEC exstrophy-epispadias complex represents a spectrum of malformations with an incidence of 1/35,000 live births occurring at varying degrees of bladder exstrophy and anatomical-functional alterations of the external genitalia. The appearance of the psycho sexual development has a significant role on the growth of an independent adult self. To compare the presence of alexithymia or depressive traits, difficulties in sexual assertiveness and / or in sex-lives and the life quality in people with EEC with a cohort consisting of unaffected peers. Design and Method: The retrospective case-control observational study included a cohort of EEC cases and a cohort of control groups of subjects not suffering from any urogenital malformation. The criteria for inclusion of cases: confirmed diagnosis of EEC, age>16 years; sex M-F; completion of informed consent and privacy forms. The inclusion criteria of the controls: the absence of any urogenital malformation, age>16; sex M-F; completion of informed consent and privacy forms. The following questionnaires were administered to 35 cases and 35 controls: BDI-II, TAS-20, SF-36, SHF, SAQ. Results: A significant presence of alexitmia and depressive traits has been highlighted in the case cohort. The life quality in the cases score lower in both physical health and mental health compared to the control cohort; while the sex life experiences of the EEC cases significantly differ from the controls also in terms of sexual assertiveness in all four sub-cases Conclusions: The findings raise awareness on the implementation of interventions aimed at improving the verbalization of emotions, the support of depressive discomfort, and sexual assertiveness.