Bladder triplication associated with exstrophy variant.

A one-day old full-term female neonate presented with a duplicate bladder and exstrophy variant including a patch of exstrophic or ectopic mucosa, duplicate vagina, uterus, and two complete bladders. We report on the surgical management performed in this case and functional urinary results based on a synchronous urodynamic study of the duplicate bladders. To our knowledge, the occurrence of duplicate bladder exstrophy variant with complete urinary continence has not been previously reported.

Long-term functional outcomes after penoscrotal hypospadias repair: A retrospective comparative study of proximal TIP, Onlay, and Duckett.

INTRODUCTION: A variety of techniques are available for proximal hypospadias repair. Onlay, proximal tubularized incised plate (TIP), and Duckett are among the popular choices because they can be performed as a one-step procedure. However, the decision to select a procedure often comes down to the surgeon's preference rather than that supported by evidence-based data. In particular, there is a paucity of literature on the long-term urinary outcomes after proximal hypospadias repair. OBJECTIVE: The aim of this study was to evaluate the evolution of long-term uroflowmetry parameters after proximal hypospadias surgery over a long-term follow-up including the adolescent period. STUDY DESIGN: Files from patients who underwent primary proximal hypospadias repair at our institution between 1997 and 2001 were reviewed. Only patients with documented serial postoperative uroflowmetry profiles at follow-up visits were included. Comparison between surgeries (pTIP vs. Onlay vs. Duckett) was performed according to the following postoperative time interval endpoints: 0-1 years, 1-2, >2-4, >4-6, >6-10, >10-12, and >12 years. Maximal urinary flow rate (Qmax) in relation to voiding volume (VV) adjusted for age or body surface area (BSA) were also evaluated in comparison to normal children using established Miskolc nomograms and compared between surgery techniques. RESULTS: Fifty-two patients met the inclusion criteria with a median follow-up of 10 years: 25 (59.6%) TIP, 18 (34/6%) Onlay, and nine (17.3%) Duckett. Overall, Qmax increased progressively according to time and age in particular during the period covering adolescence. At follow-up 12 years postoperatively, median Qmax values were 18.5 mL/second, 13.8 mL/second and 16.6 mL/second for TIP, Onlay, and Duckett, respectively, with no significant differenced detected between groups (p = 0.5) see figure). Compared with normal children when adjusted for voiding volume and BSA, the proportion of obstructive uroflowmetry patterns defined as Qmax<25th percentile of nomograms was more prevalent in patients aged 3-7 years old at 83.8% but decreased to less than 21.2% in patients aged >13 years for all procedures (see figure). DISCUSSION: These results are consistent with previous work showing frequently reduced Qmax after hypospadias surgery with great potential for improvement at puberty. CONCLUSION: These results suggest that the obstructive urinary flow pattern observed in patients early on is a frequent finding after proximal hypospadias surgery. However, because of the remarkable improvement observed at puberty a watchful waiting approach is proposed in order to avoid unnecessary intervention.

Prenatal vesico-allantoic cyst outcome - a spectrum from patent urachus to bladder exstrophy.

OBJECTIVE: The purpose of this study is to assess the prognostic significance of a cystic vesico-allantoic communication discovered on prenatal ultrasound in terms of its effect on the developing urinary tract. METHODS: Multi-institution review of prenatal screening ultrasound was performed between 2004 and 2015 to identify cases of in utero communication between the fetal urinary bladder and a cystic dilatation of the umbilical cord. Gestational age at diagnosis, perinatal evolution, and eventual urinary tract outcome are described and compared with existing literature. RESULTS: Five cases of cystic vesico-allantoic communication were identified on second-trimester screening ultrasound. Serial ultrasounds showed an increase in the size of the umbilical cystic component with gestational age, followed by its eventual rupture prior to delivery. All neonates had urinary leakage through the inferior portion of the umbilicus with bladder prolapse ranging from patent urachus to partial bladder exstrophy. CONCLUSIONS: An isolated prenatal ultrasound finding of an hourglass communication between the fetal bladder and a cyst of the umbilical cord should be considered predictive of a spectrum from patent urachus to bladder exstrophy. Given the significant ramications on the developing urinary tract, the prenatal finding of vesico-allantoic cyst warrants referral to a high-risk obstetrical center with urologic consultation.

Long-term functional outcomes of distal hypospadias repair: a single center retrospective comparative study of TIPs, Mathieu and MAGPI.

INTRODUCTION: Tubularized Incised-Plate (TIP) urethroplasty is currently the preferred technique for distal hypospadias repair. Nevertheless, concerns have been raised on the long-term functionality of the reconstructed neourethra. OBJECTIVE: The aim of this study is to evaluate long-term uroflowmetry parameters' evolution after TIP surgery over a long-term follow-up including the adolescent period. TIP patients were compared to normal children using established Miskolc nomograms, as well as to patients who underwent Mathieu and Meatal Advancement and Glanuloplasty (MAGPI) surgery repairs for distal hypospadias. STUDY DESIGN: Files from patients who underwent primary distal hypospadias repair at our institution between January 1, 1997 and January 31, 2001 were reviewed. Only patients with documented serial postoperative uroflowmetry profiles at follow-up visits were included. Comparison between surgeries (TIP vs. Mathieu vs. MAGPI) was performed according to the following postoperative time interval endpoints: 0-6 months, 6-12 months, 12-24 months, 24-48 months, 4-6 years, 6-10 years and >10 years. Maximal urinary flow rate (Qmax) in relation to Voiding Volume (VV) adjusted for Age or Body Surface Area (BSA) were also evaluated in comparison to normal children using established Miskolc nomograms and compared between surgery techniques. RESULTS: 153 patients met the inclusion criteria: 70 (43%) TIP, 24 (17%) Mathieu and 59 (35%) MAGPI. Overall, Qmax increases progressively according to time and age and in particular during the period covering adolescence with a similar trend regardless of the type of surgery. Uroflowmetry profiles in terms of Qmax, VV and PVR were equivalent between surgeries at each examined time point. At 10 years of follow-up postopertively, mean Qmax were 17.2 ml/s, 18.8 ml/s and 21.6 ml/s respectively with no significant difference detected between groups (p = 0.344). Compared to normal children when adjusted for voiding volume and BSA, the proportion of obstructive uroflowmetry patterns defined as Qmax< 5th percentile of nomograms was more prevalent in patients aged 2-7 years old at 60% but decreased to less than 10%in patients aged >13 years for all procedures combined (see abstract figure) but without detected differences between surgery types (p = 0.276). DISCUSSION: After sub-optimal obstructive maximal urinary flows in the early postoperative period, hypospadias patients treated with TIP exhibit favourable long-term evolution with age and during adolescence in particular compared to normal children. In addition, a similar trend was found for patients treated with Mathieu and MAGPI with no significant differences detected between procedures. Nevertheless, because of the relatively small sample size we cannot exclude that a statistical difference between surgeries would have been detected if the study was adequately powered on every endpoint. Nevertheless and also as suggested by the values obtained, this potential difference may be quite small and not clinically relevant. CONCLUSION: These results suggest that the obstructive urinary flow pattern observed in patients early on is possibly an intrinsic feature associated to the malformation itself and may be less of a consequence of the surgical technique.

Establishing milestones in urology training: A survey of the Canadian Academy of Urological Surgeons.

BACKGROUND: : At the current time, technical skills are not directly evaluated by the Royal College of Physicians and Surgeons of Canada (RCPSC) as part of the certification process in urology. Rather, the RCPSC relies on the evaluation of Program Directors to ensure that trainees have acquired the necessary surgical skills. METHODS: : An electronic survey was sent out to the members of the Canadian Academy of Urological Surgeons (CAUS), including the 13 Canadian urology program directors, to assess the teaching and evaluation of technical skills of urology trainees. RESULTS: : The response rate was 37% (33/89), including 8 of the 13 (62%) Program Directors from across Canada. For the teaching of technical skills, most programs had access to live animal laboratories (69%), dedicated teaching time in simulation (59%) and physical training models (59%). Most relied on voluntary faculty. There was a wide variety of structured evaluations for technical skills used across programs, while 36% of respondents did not use structured evaluations. For trainees with deficiencies in technical skills, 67% of programs offered extra operative time with designated faculty, 26% offered additional simulation focused on the deficiency and 19% offered faculty tutorial sessions. CONCLUSION: : Among Canadian urology residency programs, there is considerable variability in the assessment of technical skills of trainees. Standardized objective assessment tools would help ensure that all trainees have acquired adequate surgical proficiency to operate independently.

Laparoscopic orchidopexy: the easy way to go.

BACKGROUND/PURPOSE: Intraabdominal testes represent less than 10% of cryptorchid testicles, and yet, they are the most challenging to correct. In the last 15 years, the two-stage Fowler-Stephens orchidopexy has gained popularity. The traditional approach includes laparoscopic or open clipping of the testicular vessels (first stage) and open inguinal orchidopexy (second stage). We present our experience with 2-stage orchidopexy with both stages done through a laparoscopic approach. MATERIALS AND METHODS: Over a recent 5-year period, we reviewed patients operated for intraabdominal testis using a two-stage laparoscopic orchidopexy with a minimum of 1-year follow-up. In this study, success is defined as a nonatrophic, intrascrotal testis. Fifteen patients met the inclusion criteria, and none were lost to follow-up. RESULTS: In the 15 patients, 11 had a unilateral intraabdominal testis, and 4 had bilateral cryptorchidism, with one of the 2 testes intraabdominal. The first stage was done at a mean age of 32 months, and the average time between the two stages was 9.7 months. All procedures (31) were done on an outpatient basis. Only 2 complications occurred, one scrotal hematoma and one redo first stage because of unsuccessful clipping noted at the time of planned second stage. The success rate is 93.3% (14/15). All testicles are intrascrotal, and all but 1 have maintained preoperative volume. CONCLUSION: Two-stage laparoscopic orchidopexy is a fairly easy surgical procedure with minimum morbidity and high short term success rate. A larger cohort of patients with long-term follow-up is needed to substantiate these findings.

Topical triamcinolone for persistent phimosis.

PURPOSE: Between 2% and 5% of uncircumcised boys have persistent or pathological phimosis. Traditional treatment is usually circumcision. Recently medical treatment with topical corticosteroids has become more popular. We evaluated the efficacy of the topical steroid triamcinolone compared to foreskin retraction with an emollient cream and verified the long-term success rate of these treatments. MATERIALS AND METHODS: We performed a double-blind, randomized, placebo controlled study to compare 2-month twice daily treatment with emollient cream (placebo group 1) vs 0.1% triamcinolone (experimental group 2). Boys between ages 3 and 12 years with persistent or pathological phimosis were included in analysis. Study EXCLUSION criteria were previous treatment with topical corticosteroid, untreated balanitis and any known medical condition with immune system impairment. Patients were seen 2, 4 and 12 months after treatment initiation. Success was defined as complete, easy foreskin retraction at 4 and 12 months. Statistical analysis was done using Fisher's exact test. RESULTS: We enrolled 63 patients, of whom 43 completed the study. Despite multiple attempts 20 patients had incomplete followup and were excluded from study. Placebo group 1 included 25 patients and triamcinolone group 2 included 21. In group 1 the success rate was significantly lower than in group 2 (9 patients or 39% vs 16 or 76%, p = 0.0086). At 2 months 5 and 16 nonresponders in groups 2 and 1, respectively, were treated in nonblinded fashion with topical triamcinolone. In this subgroup 1 of 3 group 2 patients and 6 of 13 in group 1 achieved complete, easy retraction. Two and 1 patients were lost to followup in groups 1 and 2, respectively. Circumcision was required in only 5 patients (11.6%), including 4 (17.4%) initially in group 1. No complications were noted in either group. CONCLUSIONS: Triamcinolone is a highly effective and safe short-term treatment for persistent physiological or pathological phimosis. However, at long-term followup recurrence is frequent and not rare with triamcinolone and it may require re-treatment or circumcision.

RET Gly691Ser mutation is associated with primary vesicoureteral reflux in the French-Canadian population from Quebec.

Primary vesicoureteral reflux (pVUR) is a common, genetically heterogeneous congenital urinary tract abnormality in children. It causes urine to flow backward from the bladder to the ureter due to a developmental defect at the vesicoureteral junction, whose formation requires rearrangement during transformation (Ret)-mediated signaling pathways. To study the genetic causes of pVUR in Quebec patients, we used a sequencing-based candidate gene approach to screen the RET gene and found that 83 out of 118 pVUR patients are carriers of the rare A allele of single nucleotide polymorphism (SNP) rs1799939:G>A that results in a Gly691Ser mutation, a statistically significant increase in allelic frequency, that is absent at six flanking RET SNPs tested. Ser691 is a predicted phosphorylation site and our analysis of transfected cells showed that the Gly691Ser Ret mutant can efficiently interact and associate with a 75-80-kD tyrosine phosphorylated cellular protein, an event not seen with wild-type Ret. This interaction and/or the steric or electric hindrance created by phospho-Ser691 may interfere with the known regulatory functions of the normally phosphorylated phospho-Tyr687 and phospho-Ser696 on the cytoskeleton actin reorganization that are responsible for cell motility and morphology, which consequently may lead to the deficiency in ureteral development observed in pVUR. Our study demonstrates that the Ret Gly691Ser mutation is associated with pVUR and may be one of the genetic causes of this condition in the French-Canadian population in Quebec.

Lost and found testes: the importance of the hCG stimulation test and other testicular markers to confirm a surgical declaration of anorchia.

BACKGROUND: In patients with impalpable testes,laparoscopy or open surgery is considered conclusive in establishing the absence of testicular tissue. METHODS: Retrospective chart review. RESULTS: Over a 22-year period, 4 out of 82 patients with a diagnosis of bilateral anorchia by laparoscopy or laparotomy had persistent testicular tissue suggested by endocrine evaluations. The clue to the presence of testicular tissue was: (1) a pubertal rise in plasma testosterone (2 patients); (2) the presence of possible Müllerian structures and of a detectable plasma anti-Müllerian hormone (1 patient), and (3) the fact that one of the gonads had not been seen at surgery (1 patient who still had a testosterone response to hCG postoperatively). Testes were localized by venography (3 patients) and laparotomy (1 patient). CONCLUSION: A surgical diagnosis of bilateral anorchia needs to be confirmed by hCG stimulation, gonadotropin levels, or other markers of testicular function.

Impact of early screening for reflux in siblings on the detection of renal damage.

OBJECTIVE: To assess the impact of screening siblings after detecting significant vesico-ureteric reflux (VUR) and renal scarring, as such screening might identify patients with VUR before urinary tract infections develop, but might also detect clinically insignificant VUR. PATIENTS AND METHODS: We used a previously reported screening protocol to assess the clinical characteristics of patients, including the incidence of renal scarring, and their siblings, and compared the results. In all, 123 children were screened and 44 (36%) had VUR on voiding cystography. The median (range) age at screening was 9 (1-90) months. RESULTS: The grades of VUR detected were < III in 61% and > or = III in 39%; VUR was bilateral in 48%. In all, 37 siblings with VUR were assessed by ultrasonography; 70% were normal, including 12 (32%) children with VUR of grade > or = III. When used, renal scintigraphy was normal in 74% of siblings, vs 18% of index patients. However, when screened after 2 years old, siblings had twice the risk of already having renal damage on renal scintigraphy (P = 0.04). CONCLUSION: Early screening (< or = 2 years) appears to be more protective for avoiding renal damage than screening older patients. Thus we propose early screening in asymptomatic siblings to detect VUR before it becomes clinically significant.

Retroperitoneoscopic left nephrectomy in a horseshoe kidney with the use of the harmonic scalpel.

Laparoscopy has greatly evolved over the years. More recently, retroperitoneoscopy has gained wide acceptance as the preferred approach for simple and complicated nephrectomies. We report on a 12 year-old girl who underwent a left nephrectomy on a horseshoe kidney through a retroperitoneoscopic approach using the harmonic scalpel for dissection and isthmus division. The operation was successful, recovery uneventful, and the patient was discharged 24 hrs post operatively, with excellent cosmesis. Retroperitoneoscopy can be considered if ablative surgery is contemplated in a horseshoe kidney, with adequate preoperative evaluation and solid retroperitoneoscopic surgical experience. The use the harmonic scalpel was greatly beneficial for the overall success of this complicated intervention.