Long-term psychosexual adjustment of adults born with classic bladder exstrophy.

BACKGROUND: In literature studies exploring long-term psychosexual development and intimacy of adults living with the complex genitourinary conditions associated with classic bladder exstrophy (BE) are scarce, with small sample sizes and lacking in methodology. OBJECTIVE: This study aims to examine areas of potential psychosexual distress in adults born with classic BE to develop targeted clinical interventions. STUDY DESIGN: The validated Sexrelation Evaluation Schedule Assessment Monitoring (SESAMO) questionnaire was administered to all BE patients aged ≥18 years operated on in our tertiary referral center during infancy. Z-scores were calculated for each area of interest, considering i) gender (female vs male); ii) committed partnership status (singles vs couples); iii) the voiding technique adopted to empty the bladder. RESULTS: A total of 33 (F:M 12:21; singles: couples 11:22) adults with BE were enrolled in the study at a median age of 39 (32-47) years. Overall, BE adults performed the worst regarding psychosexual identity (z-score:-1.282), pleasure (z-score:-0.915) and desire (z-score:-0.583); singles regarding relational attitude (z-score:-1.751) and imaginative eroticism (z-score:-0.806); couples regarding extramarital sexuality (z-score:-1.175) and sexual communication (z-score:-0.255). When it came to gender, females significantly performed worse than males regarding psychosexual identity (-1.645 vs -1.282; p-value:<0.0001) and areas of pleasure (-1.126 vs -0.359; p-value:<0.001). Single females performed worse than males regarding actual masturbation (-0.763 vs 0.583; p-value:<0.05) and better regarding relational attitude (-1.226 vs -1.751; p-value:<0.05). Females in stable relationships performed worse than males regarding actual masturbation (-1.645 vs 0.306; p-value:<0.05) and better regarding sexual intercourse (1.866 vs -0.565; p-value:<0.01). The voiding techniques used to empty the bladder did not show any influence on these results. CONCLUSION: Adults with BE have a greater likelihood of experiencing a wide range of psychosexual difficulties. Identifying the specific areas of psychological distress can help them cope with their medical experience and actual clinical condition and clinicians plan adequate psychological interventions.

Robotic partial nephrectomy for complex kidney cyst in a 4-year old boy: Points of technique.

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.

Ovotesticular differences of sex development: male or female? Case series.

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.

Qualitative questionnaire on the psychosocial wellbeing of mothers of children with BEEC.

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.

Preputial reconstruction in hypospadias repair.

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.

Pediatric Ovarian Torsion and its Recurrence: A Multicenter Study.

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.

When a lymphatic malformation determines a bowel volvulus: Are clinical status and images always reliable?

INTRODUCTION: An acute abdomen in the form of small-bowel volvulus could be a presentation of a lymphatic malformation in childhood. CASE PRESENTATION: A 5year 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 50cm 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.

Does Preputial Reconstruction Increase Complication Rate of Hypospadias Repair? 20-Year Systematic Review and Meta-Analysis.

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. MATERIALS 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 seven studies comparing patients undergoing PR vs. circumcision showed no increased risk of urethral fistula formation associated with PR, odds ratio (OR) (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, OR (Mantel-Haenszel, Random effect, 95% CI), 1.27 (0.45-3.58). CONCLUSION: PR 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.

Informative nursery rhymes: a pilot study of children's satisfaction.

BACKGROUND: The aim of this study was to assess, by means of a questionnaire, the level of children's satisfaction relating to three informative charts, including a nursery rhyme, administered to the patient before a medical procedure. METHODS: We created three types of specific informative charts on three medical topics with the double function of informing the child before the medical procedure, and of distracting him/her by means of a nursery rhyme read aloud by the authors. To assess the level of children's satisfaction, we administered the patients a questionnaire. RESULTS: According to children's feedback, the charts were funny and useful. CONCLUSIONS: The charts conceived in this study seem to be an easily applicable and entertaining approach to provide information and distraction to children undergoing surgery.

Perforated appendix with abscess: Immediate or interval appendectomy? Some examples to explain our choice.

INTRODUCTION: There are no clear guidelines in the treatment of a perforated appendicitis associated with periappendiceal abscess without generalized peritonitis. PRESENTATION OF CASES: We retrospectively studied six examples of treated children in order to discuss the reasons of our team's therapeutic approach. Some children were treated with a conservative antibiotic therapy to solve acute abdomen pain, planning a routine interval appendectomy after some months. Others, instead, underwent an immediate appendectomy. DISCUSSION: By examining these examples we wanted to highlight how the first approach may be associated with shorter surgery time, fewer overall hospital days, faster refeeding and minor complications. CONCLUSION: Our team's therapeutic choice, in the case of a perforated appendicitis with an abscess and coprolith is an initial conservative case management followed by a routine interval appendectomy performed not later than 4 months after discharge.

[Fistulas of the lower urinary tract in children]. / Fistole del basso apparato urinario in età pediatrica.

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, especially 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.

Painful orgasm in an adolescent after seminal-sparing cystoprostatectomy: a puzzling symptom.

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.

Oncologic outcome and urinary function after radical cystectomy for rhabdomyosarcoma in children: role of the orthotopic ileal neobladder based on 15-year experience at a single center.

PURPOSE: We determined the oncologic and urological outcomes in patients with bladder/prostate rhabdomyosarcoma according to the type and timing of urinary tract surgery, with emphasis on the role of the Padua orthotopic ileal neobladder. MATERIALS AND METHODS: We retrospectively analyzed oncologic and urological outcomes of 11 consecutive patients treated at our institution between 1998 and 2012. RESULTS: Two patients underwent urethrectomy and placement of a heterotopic catheterizable ileal neobladder. The membranous urethra was preserved in 9 patients, 6 underwent primary Padua ileal neobladder at radical cystectomy, 2 underwent delayed Padua ileal neobladder and 1 underwent bilateral cutaneous ureterostomy. Four of these 9 patients experienced disease recurrence, including local recurrence in 2 despite negative intraoperative biopsies. Survivors undergoing heterotopic catheterizable ileal neobladder or primary Padua ileal neobladder learned to empty the bladder to completion without long-term upper tract deterioration. Both cases managed by delayed Padua ileal neobladder required clean intermittent catheterization eventually. Erections were reported in 5 of 6 surviving males. CONCLUSIONS: The Padua ileal neobladder allowed preservation of volitional urethral voiding in all survivors in whom it was placed at radical cystectomy. Nevertheless, local recurrence was noted in 2 of the 9 cases where the membranous urethra was preserved. By comparison, patients undergoing delayed Padua ileal neobladder after attaining disease-free status never achieved voiding per urethra. Therefore, a heterotopic reservoir might be a more reliable choice under these circumstances. Erectile function is preserved in the majority of cases.

Ureteral complications after renal transplant in children: timing of presentation, and their open and endoscopic management.

We retrospectively reviewed the records of 24 consecutive patients undergoing treatment for ureteral complications after RTx in the period 2001-2012 to determine the timing of presentation of the complications, and their open or endoscopic management. Three patients (12%) had a necrosis of the transplanted ureter soon after RTx. All required open urinary diversion in a native ureter. Ten cases (42%) developed ureteral obstruction. Time of presentation was variable mainly in relation to the underlying cause. Endoscopic treatment was successful in two cases with urinary stones and open surgery in two with mid-ureteral obstruction. Six patients had VUJ stenosis, three underwent open reimplantation, whereas temporary double-J stent placement was successfully performed in the remainder. Eleven patients (46%) had VUR. It seldom presented in the first year after RTx. Endoscopic treatment was attempted in all and was successful in all the six cases without vs. only one of the five with lower urinary tract pathology (p = 0.01). Endoscopic treatment is an option in patients with VUR in the absence of lower urinary tract pathology. It is an option also for the treatment of stones and can be attempted in case of VUJ stenosis. Ureteral necrosis always requires open treatment.

Failed hypospadias in paediatric patients.

Failed hypospadias refers to any hypospadias repair that leads to complications or causes patient dissatisfaction. The complication rate after hypospadias repairs ranges from 5-70%, but the actual incidence of failed hypospadias is unknown as complications can become apparent many years after surgery and series with lifelong follow-up data do not exist. Moreover, little is known about uncomplicated repairs that fail in terms of patient satisfaction. Risk factors for complications include factors related to the hypospadias (severity of the condition and characteristics of the urethral plate), the patient (age at surgery, endocrine environment, and wound healing impairment), the surgeon (technique selection and surgeon expertise), and the procedure (technical details and postoperative management). The most important factors for preventing complications are surgeon expertise (number of cases treated per year), interposition of a barrier layer between the urethroplasty and the skin, and postoperative urinary drainage. Major complications associated with failed hypospadias include residual curvature, healing complications (preputial dehiscence, glans dehiscence, fistula formation, and urethral breakdown), urethral obstruction (meatal stenosis, urethral stricture, and functional obstruction), urethral diverticula, hairy urethra, and penile skin deficiency.

Dismembered extravesical reimplantation of dilated upper pole ectopic ureters in duplex systems.

PURPOSE: We report the results in patients with obstructed upper pole ectopic ureters in duplex systems undergoing dismembered extravesical reimplantation of the upper pole ureter alone. METHODS: Between 01/2007 and 03/2012, 11 patients with an upper pole ectopic ureter (1 bilateral) diagnosed following the antenatal detection of hydronephrosis and showing preserved function on renal scintigraphy in a dilated upper moiety, underwent a dismembered reimplantation of the ectopic upper pole ureter as follows. The ureter was identified, separated from the lower pole ureter, and divided just above the bladder. The distal stump was suture closed, while the proximal segment was mobilized, tapered as necessary, and reimplanted using an extravesical technique. RESULTS: Twelve ectopic ureters were reimplanted. Median (range) patient age at surgery was 8 (3-48)months. Ureteral tapering was performed in 11 ureters, by infolding in 9 and using an excisional tailoring in the single case undergoing bilateral reimplantation. After a median (range) follow-up of 17 (6-50)months, all patients were asymptomatic. Eleven reimplanted ureters showed improving hydroureteronephrosis, no obstruction on diuretic scintigraphy, and no evidence of reflux on indirect radionuclide cystography. One reimplanted ureter developed worsening hydroureteronephrosis after excisional tailoring of the ureter and partial nephrectomy was performed. CONCLUSIONS: Extravesical reimplantation of the upper pole ureter is an option in dilated upper pole ectopic ureters with good function. Separating the upper and lower pole ureters proximally to the bladder does not jeopardize the ureteral blood supply and allows leaving the lower pole ureter undisturbed. Excisional ureteral tailoring should be avoided.

Duplex system ureterocele in infants: should we reconsider the indications for secondary surgery after endoscopic puncture or partial nephrectomy?

OBJECTIVE: We assessed outcomes and need for secondary surgery after primary trans-urethral puncture (TUP) or upper pole partial nephrectomy (UPPN) in duplex system ureterocele (DSU) patients undergoing management that disregards vesicoureteral reflux and upper pole function. SUBJECTS AND METHODS: Between 2003 and 2010, 41 DSU <1 year underwent TUP (n = 32) or UPPN (n = 9). Postoperatively, additional investigations and surgery were limited to cases showing persistent hydroureteronephrosis or developing recurrent febrile urinary tract infections (UTI). Outcome parameters included upper tract decompression, UTI after decompression, continence status, and secondary surgery rate. Preoperative variables were compared between patients who required secondary surgery and those who did not. RESULTS: Additional surgery was required for persistent hydroureteronephrosis in 20% of cases after TUP vs none after UPPN. After decompression, 4 female patients developed recurrent febrile UTI and 2 required additional surgeries. No case suffered from urinary incontinence. After a median (range) follow-up of 46 (17-102) months, TUP or UPPN was the only surgery required in 32 (78%) cases irrespective of preoperative variables. CONCLUSION: UPPN seems more effective than TUP in decompressing severely dilated urinary tracts. After decompression, disregarding VUR status and upper pole function, TUP or UPPN is the only procedure required in 80% of DSU cases, regardless of preoperative variables.

Primary severe hypospadias: comparison of reoperation rates and parental perception of urinary symptoms and cosmetic outcomes among 4 repairs.

PURPOSE: We compared complication rates, urinary symptoms and cosmetic outcomes as perceived by parents of patients undergoing 1 of 4 repairs for proximal hypospadias associated with ventral curvature. MATERIALS AND METHODS: A total of 93 patients underwent hypospadias repair between 2004 and 2010. In patients requiring no urethral plate transection the repair consisted of tubularized incised plate urethroplasty (26 patients) or onlay island flap urethroplasty (31). In patients requiring urethral plate transection the repair consisted of onlay island flap on albuginea (18 patients) or 2-stage repair (18). Complications were assessed by chart review. A customized questionnaire and the Pediatric Penile Perception Score were administered to parents to evaluate their perception of urinary symptoms and cosmetic outcomes, respectively. RESULTS: After a median followup of 4.5 years (range 2.2 to 8.4) complications developed in 21 patients (23%) without any difference among procedures or between patients who did and did not require urethral plate transection. Parents of 75 patients (80%) participated in the survey without differences among repairs (p = 0.35). Reported urinary symptoms were not different among repairs. For Pediatric Penile Perception Score the only difference concerned the question about penile length (p = 0.03), with the score being significantly better for the techniques requiring urethral plate transection (p = 0.05). The 2-stage repair had a significantly better score for the question about penile length and overall Pediatric Penile Perception Score than all other techniques. CONCLUSIONS: Overall complication rates were comparable among repairs and did not increase after urethral plate transection. Urinary symptoms as reported by parents were comparable among the procedures. Perceived penile length was significantly better after urethral plate transection. The 2-stage repair yielded the best cosmetic results.

Scrotal dermoid extending to the posterior urethra through a corpus cavernosum in a child.

A 3-year-old boy presented with an asymptomatic intertesticular mass increasing in size. Plasma tumor markers (α-fetoprotein and ß-human chorionic gonadotrophin) were negative. The mass had a pattern similar to testicular parenchyma but no discernable blood flow on scrotal Doppler ultrasound. Magnetic resonance imaging scan showed that it reached the posterior urethra after passing through the right corpus cavernosum with a progressive tapering extending into the pelvis. The mass was excised surgically and histologically found to be consistent with a dermoid cyst. Such rare benign lesions should be considered in the differential diagnosis of painless scrotal masses in children. Its anatomy was accurately defined by magnetic resonance imaging and was essential to minimize the risk to adjacent structures.