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.

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.

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.

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.

Preperitoneal repair of inguinal hernia.

From 2007 to date, fi ve boys with bladder exstrophy underwent this pre-emptive treatment of inguinal hernia at our institution. None has developed a recurrence after a median (range) follow-up of 29 (5 ­ 46) months. This approach avoids any manipulation of the inguinal canal, which is an advantage per se, but may be even more important in children with bladder exstrophy given the relatively high risk of hernia recurrence reported [ 1,2 ] . Furthermore, the presence of a pelvic diastasis makes the pelvic dissection of the cord easier than in normal children with a closed pelvic ring. The same procedure can also possibly be performed in children with a congenital inguinal hernia undergoing other procedures that require dissection of the perivesical space, e.g. ureteric re-implantation or ureterocoele repair.

Clinico-pathological correlation in duplex system ectopic ureters and ureteroceles: can preoperative work-up predict renal histology?

PURPOSE: Upper pole histology has been poorly investigated in duplex system ectopic ureters and ureteroceles. We aimed to determine the differences in histology between the conditions, and to identify clinical markers of renal damage. METHODS: Twenty-two patients undergoing partial nephrectomy between 2001 and 2007 for poorly functioning upper poles associated with ectopic ureters (n = 11) or ureteroceles (n = 11) were considered. Histology was classified into three groups: normal, chronic interstitial nephritis (CIN), and dysplasia. Clinical and radiological variables were compared between the two conditions and between cases with normal and abnormal histology. RESULTS: Of the 22 upper pole specimens, 9 had normal histology, 8 dysplasia, and 5 CIN. Statistical analysis failed to show any significant difference in preoperative variables or histology between ectopic ureters and ureteroceles, and in preoperative variables between cases with normal and abnormal histology. CONCLUSIONS: We did not find significant differences in the histology of upper poles associated with ectopic ureters and ureteroceles. Histology was normal in more than one-third of patients, although the poles were poorly functioning. We hypothesize that these poles were hypoplasic rather than dysplasic. We failed to identify predictors of histological damage. Hence, the latter cannot be considered a factor guiding our decision-making.

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.

Sexual function in men born with classic bladder exstrophy: a norm related study.

PURPOSE: We evaluated erectile function in men born with classic bladder exstrophy using a validated instrument and compared results with those in age matched controls. MATERIALS AND METHODS: A total of 28 patients born with bladder exstrophy were invited to self-administer an Italian version of the International Index of Erectile Function-15 to assess erectile and orgasmic function, sexual desire and satisfaction, and overall satisfaction. A score of 25 or less of 30 in the erectile function domain was considered diagnostic for erectile dysfunction. Scores in patients with bladder exstrophy were compared with scores in 38 normal controls who self-administered the same questionnaire. RESULTS: A total of 19 men (68%) with a median age of 27.1 years (range 18.3 to 41.2) returned the questionnaire, of whom 11 (58%) presented with erectile dysfunction compared to 9 (23%) age matched controls (p = 0.02). Erectile dysfunction was more common in patients with bladder exstrophy who underwent multiple continence surgeries. Orgasmic function was also significantly lower in patients with bladder exstrophy than in controls (p = 0.001). No difference was observed between the groups in the sexual desire, sexual satisfaction and overall satisfaction domains. CONCLUSIONS: Patients born with classic bladder exstrophy appear to have erectile dysfunction and decreased orgasmic function more commonly than normal controls, particularly when they underwent multiple continence surgeries. Sexual desire seems comparable to that of their peers. Eventually patients with bladder exstrophy seem to lead a sexual life that is as satisfactory as that of their peers.

Upper urinary tract infections are associated with RANTES promoter polymorphism.

We evaluated the association between MCP-1, CCR2, RANTES, and CCR5 gene polymorphisms and upper urinary tract infection in 273 children recruited in Northeast Italy. Statistical analysis of RANTES-403 G>A genotype frequencies showed that children carrying the RANTES-403 G allele are at higher risk for urinary tract infection, irrespective of vesicoureteral reflux.

Lower urinary tract symptoms (LUTS) after renal transplant in non-urologic anuric patients.

We assessed LUTS at least 12 months after RTx in patients without evidence of lower urinary tract dysfunction (non-urologic) that had been anuric for at least six months before RTx. No bladder recycling was performed before RTx. LUTS were evaluated using a questionnaire. Clinical records were also reviewed. LUTS in anuric patients were compared with those in non-anuric patients. Fourteen anuric patients fulfilled the inclusion criteria. Median age at RTx was 11 (5-21) yr, median duration of anuria before RTx 24 (7-46) months, and median post-RTx follow-up 2.7 (1.9-10.2) yr. Daytime symptoms were exceptional. Nocturia was the most common symptom (10 patients). Only one patient reported symptoms to affect her quality of life. One patient experienced a febrile UTI and none graft failure. LUTS (nocturia) proved unrelated to duration of anuria, length of follow-up, and presence of (nocturnal) polyuria. LUTS were not statistically different in patients anuric and non-anuric before RTx. Non-urologic patients suffer from long-term storage symptoms, particularly nocturia. LUTS, however, do not seem to increase the risks of urinary infections or graft failure and appear to occur irrespective of the presence of anuria before RTx. Bladder recycling before RTx seems unnecessary.

Thoraco-pelvic orthosis: a portable and removable bassinet for secure patient immobilization after primary bladder exstrophy closure.

A portable and removable thoraco-pelvic orthosis for patient immobilization after neonatal primary bladder exstrophy closure is described. The device is made of a polyethylene shell, moulded at 170°C, coated inside with a 5 mm pad of plastazote and works applying a constant gentle pressure on the hips.

Urinary function after Snodgrass repair of distal hypospadias: comparison with the Mathieu repair.

PURPOSE: To evaluate urinary function in patients with distal hypospadias undergoing repair by the tubularized incised-plate urethroplasty (TIP or Snodgrass), compare the results with those in patients treated by the Mathieu technique, and show the potential issues inherent to the evaluation of such results. PATIENTS AND METHODS: A cross-sectional assessment was performed of uncomplicated distal hypospadias operated on during a 3-year period, already toilet trained, and able to void volitionally. Evaluation included clinical assessment urinary symptoms and urinary stream, and uroflowmetry. RESULTS: Out of 83 patients operated on during the study period, 10 (12%) developed complication and 32 were not toilet trained or refused to participate in the study. Median follow-up in the remaining 41 patients included in the study was 20 (3-36) months. None of these patients presented voiding symptoms or urinary stream abnormalities. Uroflowmetry was normal in 30 cases and obstructive in 11 (27%). An obstructive flow pattern was more common in patients undergoing TIP versus Mathieu repair, 8 of 19 (42%) versus 3 of 22 (14%), respectively (P = 0.07). Four TIP cases with an obstructive uroflow pattern were managed conservatively. CONCLUSIONS: Although both the TIP and the Mathieu repair allow good results in terms of urinary function after distal hypospadias repairs, the TIP technique seems more likely to be associated with urine flow pattern abnormalities. The actual clinical relevance of this finding remains ill defined.

Complications of trans-anastomotic externalised stents in open pyeloplasty: influence of the method of placement, the duration of stenting, and the associated bladder drainage.

PURPOSE: To assess the influence of the method for stent placement, the duration of stenting, and the presence of bladder drainage on the complication rate of open pyeloplasty. PATIENTS AND METHODS: Complications were, retrospectively, compared in 228 consecutive open pyeloplasties performed at institution A using a trans-pyelostomic 6-Fr splint/stent for 5 days and no bladder drainage, and 150 consecutive open pyeloplasties performed at institution B using a trans-nephrostomic 6-Fr splint/stent for 9 days plus bladder drainage. RESULTS: Median age at surgery was comparable between groups. The overall complication rate was 13% and was comparable at the two institutions, but for the presence of perioperative bleeding that was more common when the stent was placed trans-nephrostomically (institution B) and the rate of stent dislodgements, which was lower at institution A, perhaps due to some technical details aiming to prevent any inadvertent traction on the stent. Additional procedures, such as double J internal stent insertion, were required in <2% of cases. CONCLUSIONS: A 5-day period of stenting after open pyeloplasty is generally enough. Trans-nephrostomic and trans-pyelostomic stent placement is equally effective. However, the former can be associated with a slightly higher bleeding rate. Details during stent placement are keys to avoid postoperative dislodgement and malfunctioning. Systematic bladder drainage seems unnecessary.

Aptness and complications of labial mucosa grafts for the repair of anterior urethral defects in children and adults: single centre experience with 115 cases.

PURPOSE: We report a comprehensive review of our experience with labial mucosa graft urethroplasty to evaluate the indications for this kind of substitution urethroplasty, and the possible complications and risk factors for an untoward recipient site outcome both in children and adults. MATERIALS AND METHODS: Complications were retrospectively assessed by chart review. Patients with and without complications were compared with respect to age at surgery, indication for surgery (epispadias vs. hypospadias vs. urethral stricture), graft length and configuration (tube vs. patch). Moreover, in cases with urethral stricture a comparison was made according to the level of the stricture (penile vs. bulbar). RESULTS: After a median follow-up of 36 (6­90) months, the overall success rate was 66%. Complications were observed in 39 (34%) patients, of whom 18 (16%) required additional surgical procedures. Complications appeared to be unrelated to patient age at surgery, indication for surgery, graft length, and the urethral level of the stricture. Use of the graft as a tube was the single factor significantly more frequent in patients experiencing complications, particularly in the urethral defects associated with hypospadias where use of a tube corresponded to an odd ratio of 5.86 (95% CI 1.5­23.4). CONCLUSIONS: Oral grafts harvested from the lower lip are a versatile tissue for the repair of urethral defects associated with either urethral malformations or strictures, both in children and adults. Use of the graft as an on-lay seems preferable particularly in hypospadias repairs.

Radical soft tissue mobilization and reconstruction (Kelly procedure) for bladder extrophy [correction of exstrophy] repair in males: initial experience with nine cases.

PURPOSE: to report the early experience with the Kelly procedure for the treatment of bladder exstrophy (BE) in males. MATERIALS AND METHODS: Nine boys with BE were treated at our institute. One had an untouched BE, four had epispadias after neonatal bladder closure, and four were secondary phalloplasties. Data on surgical complications, continence status, presence of erections and parental satisfaction with penile appearance and length are reported. RESULTS: Mean patient age was 4.7 (1-8.9) years. No intra-operative complications occurred. Two secondary cases experienced formation of a bladder-neck fistula and glans ischemia, respectively. The latter led to glans loss. All the patients had some residual degree of hypospadias after surgery. After a median follow-up of 18.1 (10-22) months, one patient developed chronic bladder outlet obstruction. Overall, five patients are dry (including two on clear intermittent catheterization and one with a Minz II pouch). The other four are still younger than 3 years, all have spontaneous micturitions and dry interval between 30 and 120 min. Of the eight patients without phalloplasty complications, all had erections, and parents judged the penile length and appearance as being satisfactory in six cases. CONCLUSION: The Kelly procedure is feasible in a vast array of BE patients, but may be formidable, especially in secondary phalloplasties. It allows for complete reconfiguration and lightening of the penis, but exposes to potentially catastrophic complications, such as partial or complete penile loss. Longer follow-up is needed to assess the results in terms of continence.

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.

Donor site outcome after oral mucosa harvest for urethroplasty in children and adults.

PURPOSE: We report short and long-term donor site outcomes after oral mucosa graft harvesting for urological reconstruction in a large series of patients including children, and identify possible risk factors for an untoward long-term outcome. MATERIALS AND METHODS: A total of 78 patients were evaluated. Short-term outcomes included time to restore normal oral diet, perioral sensory defect/discomfort and jaw opening impairment occurring within 4 weeks of surgery. Long-term outcomes included donor site scarring, perioral sensory defect and jaw opening impairment occurring more than 1 year postoperatively. Long-term outcomes were assessed via a questionnaire administered to patients and on clinical examination by an oral surgeon. Outcomes were compared in children (younger than 12 years at surgery) and adults, and with regard to harvesting site, graft length, length of followup and other variables. RESULTS: Two-thirds of the patients returned to a normal oral diet within 3 days postoperatively (range 1 to 8). All patients complained of perioral sensory defect/discomfort postoperatively, and 26% had jaw opening impairment. After a median followup of 7.6 years (range 1 to 13.2) perioral sensory defect was the most common complication observed (28%) in cases formally evaluated by an oral surgeon. The sensory defect was seldom perceived by the patients and never required treatment. It was statistically more common in patients undergoing surgery as adults, whereas none of the other variables proved significant. CONCLUSIONS: Oral mucosa graft harvesting is safe irrespective of age. About a quarter of patients, more commonly adults, will have a long-term perioral sensory defect. However, the defect is never perceived as bothersome.

Scintigraphic renal function after unilateral pyeloplasty in children: a systematic review.

OBJECTIVE: To systematically review previous reports and thus determine the functional outcome after pyeloplasty in children with unilateral hydronephrosis due to pelvi-ureteric junction obstruction, and the possible variables that could affect it. METHODS: We searched Medline/PubMed, Embase, and Web of Science for articles in English published from 1966 to 2007, using both 'medical subject headings' and 'free text' protocols. Abstracts, full texts, and bibliographies of pertinent papers were reviewed to select studies of scintigraphic renal function before and after pyeloplasty. The postoperative change in scintigraphic renal function was assessed in relation to presentation (antenatal vs postnatal), timing of surgery (early vs delayed), age at surgery, and preoperative ultrasonography (US) and scintigraphic findings. RESULTS: Thirty-six studies (2.1% of the initial search) were eventually selected for review. Studies were generally of poor scientific quality and very heterogeneous in their indications for surgery and follow-up protocols. Postoperative function showed a wide variability. Symptomatic patients diagnosed postnatally seemed to have a greater chance of functional improvement after surgery than asymptomatic patients diagnosed antenatally. The chance of improvement seemed also to be greater in patients with moderately rather than severely impaired preoperative function. Otherwise, the improvement seemed unrelated to the age at surgery, the preoperative US findings, or the excretion pattern on renal scintigraphy. Of patients having delayed surgery >97.5% had preserved function afterward. CONCLUSIONS; Patients with moderately impaired preoperative function and those diagnosed postnatally because of symptoms are those with the greatest likelihood of having a functional improvement after surgery.