Exstrophic bladder duplication in the sagittal plane: Surgical management of a rare case.

INTRODUCTION: The bladder-Exstrophy-Epispadias complex (BEEC) contains a wide spectrum of congenital malformations. A treatment naïve refugee was referred to our center with what was identified as BEEC. MATERIALS AND METHODS: A 27-year-old female patient was referred for total incontinence since birth by the general practitioner from the refugee center. An exstrophic bladder with blind ending ureteral orifices and a second non-exstrophic bladder with two orthotopic ureters was identified, demonstrating the bladder duplication in the sagittal plane. Laparotomy was performed, dissecting the exstrophic bladder plate caudally and using it as a ventral onlay to augment the non-exstrophic bladder. A Mitchell-type bladder neck reconstruction was performed with an autologous fascia sling around the bladder neck to obtain continence. As the patient had never voluntarily voided, chances of spontaneous voiding after surgery were low. Therefore creation of a continent Mitrofanoff-type vesicostomy was additionally realized and genital reconstruction was achieved. RESULTS: 12 months post operatively, the patient was completely continent, had a bladder capacity of 250 ml, and performed self-catheterization 5 times a day. No post-operative complications were observed. CONCLUSION: Admission of political refugees can implicate challenging surgeries for congenital malformations in adults, such as BEEC. This demonstrates the importance of multidisciplinary transitional care.

Patient information video: How to take care of your child after hypospadias surgery?

INTRODUCTION: Patient/parent education and participation helps improve post-operative care. Dressing and catheter care after hypospadias surgery varies widely and young parents are keen to use available media when seeking for help, especially if surgery is done in an outpatient setting. An information video about post-operative care after hypospadias is made available through a tertiary referral hospital's website. MATERIALS AND METHODS: Hypospadias surgery is an outpatient surgery in our setting. A double diaper system is used to prevent contamination of the urinary catheter and penile bandage with stools. A video explaining how the dressing works was made, helping parents maintaining the dressing and thereby solving possible questions/problems about dressing or medications while at home. Warning signs and symptoms are mentioned in which case parents should contact the urologist: fever, continuous blood loss and lack of urinary output. RESULTS: An information folder summarizing the latter, including a link to the video is given to all parents pre-operatively, providing reassurance according to parents' feedback. CONCLUSION: Parent participation can help improve post-operative care, especially in outpatient clinic setting. This video helps parents through post-operative care after hypospadias surgery. Its availability through informative folders and hospital's website is destined to educate and reassure parents.

Urethral duplication as cause of incontinence in a 5-years-old female: Surgical approach.

INTRODUCTION: Duplication of the urethra is a rare congenital anomaly in females which, if left unidentified, might cause refractory urinary incontinence. This video demonstrates its identification and treatment. PATIENT & METHODS: A 5-year-old female was referred for primary refractory diurnal incontinence without any dry moment since birth. Initial clinical examination showed permanent urine leakage from an opening cranial to the clitoris. Ultrasound did not show any bladder or kidney abnormalities. A diagnostic cystoscopy under anesthesia revealed an accessory meatus located cranially to the orthotopic meatus and to the clitoris. RESULTS: Lying in dorsal decubitus under general anesthesia, catheters were inserted in respectively the orthotopic and accessory meatus. The accessory meatus and urethra were dissected away into the bladder dome from a circumferential skin incision. Defects were closed and bladder drainage per urethram was left in situ for 10 days. P ostoperative recovery was uncomplicated. Complete resolution of the incontinence was immediately achieved at catheter removal. Daytime and night-time continence are observed with 3 years follow-up. CONCLUSION: Urethral duplication is a rare finding in females but should be excluded during the workup of primary incontinence. In girls with isolated urethral duplication, resection of the accessory urethra is the best surgical option.

Definitions, indications and practice of urotherapy in children and adolescents: - A standardization document of the International Children's Continence Society (ICCS).

BACKGROUND: Urotherapy is an umbrella term for all non-surgical, non-pharmacological interventions for lower urinary tract disorders (LUTD) in children and adolescents. Urotherapy is a specialized practice, which has become mainstay therapy not only for daytime urinary incontinence, but also for nocturnal enuresis, functional constipation and fecal incontinence. The aim of urotherapy is to achieve the normalization of the micturition and bowel pattern and to prevent further functional disturbances by repeated training. It is well known that in the treatment of adult and childhood incontinence a team approach is best, where there are shared areas of expertise and also discipline-specific expertise available. AIM: We present a consensus view from a cross-professional team of experts affiliated with the International Children's Continence Society on definitions, indications and practice of urotherapy. This is a selective, non-systematic review with practical recommendations for the implementation and research on urotherapy. METHODS: The document uses the globally accepted ICCS terminology. Evidence-based literature serves as the basis, but in areas lacking in primary evidence, expert consensus is used. Before submission, a full draft was made available to all ICCS members for additional comments. RESULTS: Urotherapy uses non-pharmacological, non-surgical methods and focuses on behavioral interventions, largely based on cognitive-behavioral psychotherapy (CBT). Standard urotherapy comprises components such as provision of information, instructions, life-style advice, counselling and registration of symptoms. Specific urotherapy is tailored towards specific disorders and includes alarm treatment, biofeedback training, pelvic floor training, neurostimulation and other interventions. Fig. 1. Urotherapy is a treatment that addresses all aspects of incontinence, leading to the best clinical outcome. This includes somatic, psychosocial, and behavioral problems and quality of life. Therefore urotherapy is recommended by the ICCS as the first-line treatment for most types of LUTD. The document is intended to be clinically useful in primary, secondary and tertiary care.

The long-term added value of voiding school for children with refractory non-neurogenic overactive bladder: an inpatient bladder rehabilitation program.

INTRODUCTION & BACKGROUND: Despite adequate management, 20% of children with overactive bladder (OAB) syndrome fail to improve their bladder function. To approach the need for alternative strategies, an inpatient bladder rehabilitation 'voiding school' program was established. OBJECTIVE: The objective of this study was to evaluate the short- and long-term (1-year follow-up) outcome of this voiding school program in children with refractory OAB. In addition, the authors aimed to identify which children achieved the best outcomes with this voiding school program. STUDY DESIGN: The charts of all children (n = 357, mean age: 9.7 ± 2.0 years, 63.6% boys) with refractory OAB who attended voiding school between 2000 and 2010 were reviewed. A linear mixed model with random intercept was used to evaluate the incontinence (expressed by enuresis and daytime incontinence voiding scores) and maximal voiding volume (MVV). RESULTS & DISCUSSION: This study demonstrated an overall beneficial long-term effect of the inpatient program on day- and night-time incontinence, in which 36.6% of children achieved dryness during day- and night-time. In addition, the mean overall decline in the number of wet nights and days declined with 4 extra dry days and/or nights per week, in comparison with the level of continence before attending the voiding school program. In contrast, only a temporary increase in MVV was seen, however, without relapse incontinence. At last, the authors identified the negative impact of decreasing age, male sex, dysfunctional voiding and nocturnal polyuria on the overall outcome of the inpatient program. CONCLUSION: An inpatient rehabilitation 'voiding school' program is a successful and safe treatment modality for children with refractory OAB that results in long-term significant increase of continence, as well as amelioration in degree of severity. The worst outcomes of this voiding school program were detected in children with young age, who were boys, or had associated nocturnal polyuria, dysfunctional voiding, and/or faecal incontinence.

Androgen receptor expression in preputial dartos tissue correlates with physiological androgen exposure in congenital malformations of the penis and in controls.

INTRODUCTION: The androgen receptor (AR) plays an important role in the development of male genitalia, and impaired androgen signalling has been hypothesised to underlie congenital penile malformations (CPM) such as hypospadias. Previous studies exploring the role of AR expression in the development of CPM have yielded conflicting results. OBJECTIVES: To assess AR expression in human foreskin of boys/men born with hypospadias, buried penis versus controls. STUDY DESIGN: Foreskin samples of 428 boys and men undergoing primary penile surgery (198 controls, 197 hypospadias, and 33 buried penis) were collected between October 2013 and July 2018. AR staining was performed in all samples and semi-quantitatively scored by two researchers independently, using a modified quick score (mQuicks) that assesses the proportion and intensity of AR staining in smooth muscle fibres. RESULTS: The interobserver variability of the mQuicks had a high level of agreement for the total score, as well as for the subscores. Two phases of high AR expression were observed in all groups, the first following the postnatal gonadotropin surge (i.e., mini-puberty) and the second in (pre-) puberty. No differences in AR expression were found in hypospadias or buried penis cases as compared to controls matched for age at time of surgery. DISCUSSION: This study describes the physiological evolution in AR expression in the human foreskin of boys with CPM and explains the cause of the previously reported, conflicting results. Despite the very large cohort, the limitations of this study are the low number of cases younger than six months at the time of surgery and the lack of Tanner stages to correlate with the mQuicks in adolescents. CONCLUSIONS: The mQuicks is a straightforward and informative tool to semi-quantitatively assess AR expression in the dartos tissue. In this study, AR expression in human foreskin shows a bimodal distribution in boys with CMP and controls, following physiological androgen exposure. No statistically significant difference in AR expression could be found between both groups. Whether other local mechanisms are affected by these physiological changes is currently unclear. However, strict age-matching should be considered when exploring the mechanisms underlying disturbed penile and urethral development in CMP.

Robot-assisted resection of ectopic kidney in children: An anatomical illustration.

INTRODUCTION: Failure of kidney migration during embryonic life results in an ectopic kidney, with an incidence varying from 1 in 500 to 1 in 1200. Pelvic kidney can be a rare cause of recurrent urinary tract infection (UTI), warranting nephrectomy in some cases. MATERIAL AND METHODS: A 7-year-old girl with a history of recurrent UTIs and vaginal discharge was diagnosed with a dysplastic afunctional ectopic kidney located in the pelvis, with ureter draining into the cervix or vaginal wall. RESULTS: A robot-assisted approach was chosen, with side docking of the robot to allow concurrent vaginoscopic/hysteroscopic exploration. A uterus bicornis was found. The ectopic ureter was dissected toward its drainage in the vaginal wall, where it was sutured and resected. The dysplastic kidney and ureter were removed. Compared to pure laparoscopic approach, dissection deep into the pelvis toward the vaginal wall is aided by the robotic dexterity and facilitates complete resection of the structure, which avoids leaving a ureteric stump into the vaginal wall. CONCLUSIONS: Robot-assisted surgery is a safe and effective option for resection of ectopic kidneys with ectopic ureter in children. Dissection up until the vaginal wall in children is aided by robotic dexterity, making this kind of surgery the ideal indication for robotic-assisted surgery.

Urethroplasty for urethral injuries and trauma-related strictures in children and adolescents: a single-institution experience.

INTRODUCTION: Urethral injuries and trauma-related strictures (UITSs) in children are rare. The treatment is challenging but crucial to avoid life-long urinary complications such as recurrent stricture formation, urinary incontinence, and impotence. OBJECTIVE: The aim was to report on the surgical and functional outcome of urethroplasty for UITSs and to provide data on patient-reported outcome measures (PROMs). MATERIAL AND METHODS: Between November 2001 and October 2017, 18 male children (≤18 years; median: 13 years) underwent urethroplasty for UITSs at a single tertiary referral center. Etiology was iatrogenic in five (27.8%), perineal straddle injury in six (33.3%) and pelvic fracture urethral injury (PFUI) in seven (38.8%) patients. PFUIs and short (≤3 cm) bulbar strictures were treated by transperineal anastomotic repair (n = 15; 83.3%), whereas a long bulbar stricture and a penile stricture were treated by, respectively, a preputial skin graft and flap urethroplasty. A penetrating penile urethral injury during circumcision underwent early exploration with primary repair of the laceration. Failure was defined as need for additional urethral instrumentation. PROMs were sent to patients ≥16 years at the latest evaluation. RESULTS: Median follow-up was 57 (range: 8-198) months. No complications and grade 1, 2, and 3 were present in, respectively, 13 (72.2%), two (11.1%), one (5.6%), and two (11.1%) patients. The success rate in a tertiary referral center was 94.4%. An immediate failure was observed in a patient with a PFUI and concomitant bladder neck injury. PROMs were available in 12 patients. Four patients (33.3%) reported erectile dysfunction. Post-void dribbling (25%) and urgency (50%) were the most frequently reported complaints. All patients were satisfied after urethroplasty and stated that they would undergo the surgery again. DISCUSSION: This series corroborates the recent trend in favor of transperineal anastomotic repair for PFUI, with combined abdominoperineal approach reserved for complex situations (e.g. bladder neck injury). For anterior UITSs, adaption of the technique to the characteristics of UITSs (etiology, location, length, and quality of graft bed) yielded excellent outcomes. Future systematic use of PROMs is also needed in children to elucidate the impact of urethroplasty on the urinary and sexual function. CONCLUSION: External trauma is the most important etiology of UITSs, but iatrogenic causes should not be neglected. Urethroplasty, mainly by anastomotic repair (AR) but with the technique adapted to local stricture characteristics if necessary, has an excellent long-term success rate in experienced hands. Functional disturbances are frequent, but despite this, patient satisfaction is high after urethroplasty.

Gonadal dysgenesis in disorders of sex development: Diagnosis and surgical management.

Recent studies on gonadal histology have improved the understanding of germ cell malignancy risk in patients with disorders of sex development (DSD), and evidence-based gonadal management strategies are gradually emerging. Especially in 46,XY DSD and 45,X/46,XY DSD, which are characterized by gonadal dysgenesis, the risk of germ cell malignancy is significantly increased. This paper summarized the progress over the past 10 years in malignancy risk assessment in patients with DSD, and its implications for optimal surgical handling of the involved gonads.

Global minimally invasive pyeloplasty study in children: Results from the Pediatric Urology Expert Group of the European Association of Urology Young Academic Urologists working party.

INTRODUCTION: Minimally invasive pyeloplasty (MIP) for ureteropelvic junction (UPJ) obstruction in children has gained popularity over the past decade as an alternative to open surgery. The present study aimed to identify the factors affecting complication rates of MIP in children, and to compare the outcomes of laparoscopic (LP) and robotic-assisted laparoscopic pyeloplasty (RALP). MATERIALS AND METHODS: The perioperative data of 783 pediatric patients (<18 years old) from 15 academic centers who underwent either LP or RALP with an Anderson Hynes dismembered pyeloplasty technique were retrospectively evaluated. Redo cases and patients with anatomic renal abnormalities were excluded. Demographics and operative data, including procedural factors, were collected. Complications were classified according to the Satava and modified Clavien systems. Failure was defined as any of the following: obstructive parameters on diuretic renal scintigraphy, decline in renal function, progressive hydronephrosis, or symptom relapse. Univariate and multivariate analysis were applied to identify factors affecting the complication rates. All parameters were compared between LP and RALP. RESULTS: A total of 575 children met the inclusion criteria. Laparoscopy, increased operative time, prolonged hospital stay, ureteral stenting technique, and time required for stenting were factors influencing complication rates on univariate analysis. None of those factors remained significant on multivariate analysis. Mean follow-up was 12.8 ± 9.8 months for RALP and 45.2 ± 33.8 months for LP (P = 0.001). Hospital stay and time for stenting were shorter for robotic pyeloplasty (P < 0.05 for both). Success rates were similar between RALP and LP (99.5% vs 97.3%, P = 0.11). The intraoperative complication rate was comparable between RALP and LP (3.8% vs 7.4%, P = 0.06). However, the postoperative complication rate was significantly higher in the LP group (3.2% for RALP and 7.7% for LP, P = 0.02). All complications were of no greater severity than Satava Grade IIa and Clavien Grade IIIb. DISCUSSION: This was the largest multicenter series of LP and RALP in the pediatric population. Limitations of the study included the retrospective design and lack of surgical experience as a confounder. CONCLUSIONS: Both minimally invasive approaches that were studied were safe and highly effective in treating UPJ obstruction in children in many centers globally. However, shorter hospitalization time and lower postoperative complication rates with RALP were noted. The aims of the study were met.