Bilateral Obturator Osteotomy: A Novel Osteotomy for Bladder Exstrophy Closure.

BACKGROUND: Abdominal and pelvic closure remains a challenge during bladder exstrophy initial repair. We aimed to report on the feasibility and results of a novel technique of bilateral obturator osteotomy. METHODS: Retrospective study of prospective collected data of children who underwent single-stage delayed bladder exstrophy closure combined with RSTM (Radical Soft Tissue Mobilization) for BEEC (Bladder Exstrophy Epispadias Complex) by the same team at different institutions between December 2017 and May 2021. When pubic approximation was not feasible at the end of the procedure, bilateral obturator osteotomy was performed through the same approach, consisting in bilateral divisions of the ilio-pubic rami, ischio-pubic rami, obturator membrane, and detachment of the internal obturator muscle. Pubic bone fragments were approximated together on the midline. Immobilization in a thermoformed posterior splint was indicated for 3 weeks. The main outcome criterion was the bladder dehiscence rate at 6 months, assessed by physical inspection. Secondary outcome criteria included neurovascular obturator pedicle injury, analyzed during orthopedic physical examination, wound or bone infections, gait acquisition, reported by parents and evaluated during medical examination, and vascular penile impairment, judged by penile and glans coloration. RESULTS: 17 children (11 males, 6 females) were included, at a median age of 2 months [1-33]; and representing 29% (17/58) of the children with bladder exstrophy who underwent the same surgical approach during the time of study. There was no postoperative bladder dehiscence with a median follow-up of 34 months [6-47]. No complication was observed. Pelvic X-rays showed bilateral normal ossification process. Neither gait abnormality, nor clinical indication of obturator nerve deficiency was observed during follow-up. CONCLUSION: When pubic bones approximation is not possible, bilateral obturator osteotomy is a useful adjunct in bladder exstrophy closure, feasible by the pediatric urologist through the same approach, and not requiring external fixator. LEVEL OF EVIDENCE: IV.

Financial and relational impact of having a boy with posterior urethral valves.

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care. Patients and method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the "impact on family scale" (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score. Results: We retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15-51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards. Conclusion: In conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible.

Indocyanine Green (ICG)-Guided One-Stage Delayed Bladder Closure and Radical Soft-Tissue Mobilization (Kelly Procedure) For Bladder Exstrophy Repair: The First Experience.

The vascular supply of the pelvic structures and the external genitalia can be easily injured during the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure) for bladder exstrophy surgical repair. Aiming to help surgeons assessing and confirming tissue perfusion and viability, indocyanine green (ICG)-based laser angiography was incorporated into the operative approach to reduce the risk of ischemic injuries. The EleVision IR system (Medtronic Ltd) was adopted to confirm the identification of the vascular pedicles and assess the tissue perfusion in real-time in a 5-month-old with bladder exstrophy undergoing the one-stage delayed bladder closure and radical soft-tissue mobilization (Kelly procedure). ICG (0.15 mg/kg) was intravenously administered at 6 key steps during surgery with the ability to be re-dosed every 15 minutes. ICG-based laser angiography helped to confirm the correct identification of the vascular structures during surgery and to assess tissue perfusion in real-time. Blood flow did not change considerably after initial dissection or upon approximating the pubis symphysis. At the end of the procedure, good penile perfusion was shown, proving that no direct injury or substantial compression of the pudendal vessels had occurred following the mobilization and the reconstructive phase. ICG-based laser angiography proved to be safe, effective, and easy to employ and should be considered as a reasonable adjunct for tissue perfusion assessment and operative decision-making in patients undergoing bladder exstrophy Kelly repair.

Assessment of Anorectal Function and Related Quality of Life of 27 Patients with Bladder Exstrophy or Epispadias After Kelly Radical Soft Tissue Mobilisation.

INTRODUCTION: The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact of this procedure on subsequent anorectal function. METHODS: Monocentric controlled study of prospectively collected data of children who underwent RSTM for BEEC from 2010 to 2017. Patients born after 2017 were not included, as they were below the theoretical age of continence acquisition at the time of the study. Anorectal function was assessed using the Childhood Bladder and Bowel Dysfunction Questionnaire, and quality of life (QoL) related to fecal continence using the CINCY FIS questionnaire. The control group was paired on age and sex with a 1:3 patient/control ratio. Answers to questionnaires were collected from September 2021 to January 2022. Univariate statistical analysis comparing two groups and subgroup analysis following age were also performed. RESULTS: During the period of study, 55 children with BEEC underwent Kelly RSTM. Twenty-seven (49%) were included and paired with 81 healthy children on age and sex. Median age at surgery was 15 months [0.5-93] and median follow-up was 10 years [4-13]. Patient's group median age at evaluation was 11 years [5-19]. There was no difference between patients and control group in anorectal function for both incontinence and constipation items. No significant difference was found in QoL related to fecal incontinence assessment. Subgroup analysis did not show difference. CONCLUSION: This study suggests that the levator ani detachment during Kelly procedure, realised in a paediatric population under the age of 8, did not impact anorectal function with a mid-term follow-up. LEVEL OF EVIDENCE: III.

Outcome of long gap esophageal atresia at 6 years: A prospective case control cohort study.

BACKGROUND DATA: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years. METHODS: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed. RESULTS: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group. CONCLUSIONS: Long gap strongly influenced digestive morbidity at age 6 years.

Long-term results of artificial urinary sphincter implantation for urinary incontinence due to intrinsic sphincter deficiency in children.

PURPOSE: To report long-term results of artificial urinary sphincter implantation for urinary incontinence due to intrinsic sphincter deficiency in children. MATERIALS AND METHODS: This retrospective monocentric study included all patients who underwent artificial urinary sphincter (AMS 800) implantation before 18 years of age between 1986 and October 2018 for intrinsic sphincter deficiency. The primary outcome was the continence rate at the last follow-up, defined by the daily use of 0 pads. The secondary outcome was the overall survival of the device, defined as the absence of any repeated surgery (revision or explantation) during follow-up. Reoperation-free, revision-free, and explantation-free device survival rates were estimated using the Kaplan-Meier method. RESULTS: Thirty-six patients with a median age of 12 years (interquartile range [IQR]: 10-14) were included (15 females, 21 males). The median follow-up was 18.7 years (IQR: 9-26). The main underlying condition was spinal dysraphism (n = 24; 67%). The median time to the first reoperation was 9 years (IQR: 3.75-14.7). At the last follow-up, survival rates without revision were 84%, 71%, 55%, and 33% at 5, 10, 15, and 20 years, respectively. Survival rates without explantation were 91%, 84%, 80%, and 72% at 5, 10, 15, and 20 years, respectively. At the last follow-up, 29 patients had a functional device. The overall continence rate was 88%. All patients who had their device still in place were continent at the last follow-up. CONCLUSION: The artificial urinary sphincter is an effective long-term treatment for urinary incontinence related to intrinsic sphincter deficiency in children, providing a high rate of continence, even if associated with a high rate of reoperation.

Latitudes and attitudes: A multinational study of laparoscopic pyeloplasty in children.

PURPOSE: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty. MATERIALS AND METHODS: A descriptive, retrospective study was conducted evaluating patients undergoing Anderson-Hynes dismembered laparoscopic pyeloplasty. Centers from four different continents participated. Demographic data, perioperative management, results, and complications are described. RESULTS: Over a 9-year period, 744 laparoscopic pyeloplasties were performed in 743 patients. Mean follow-up was 31 months (6-120m). Mean age at surgery was 82 months (1 w-19 y). Median operative time was 177 min. An internal stent was placed in 648 patients (87%). A catheter was placed for bladder drainage in 702 patients (94%). Conversion to open pyeloplasty was necessary in seven patients. Average length of hospital stay was 2.8 days. Mean time of analgesic requirement was 3.2 days. Complications, according to Clavien-Dindo classification, were observed in 56 patients (7.5%); 10 (1%) were Clavien-Dindo IIIb. Treatment failure occurred in 35 cases with 30 requiring redo pyeloplasty (4%) and 5 cases requiring nephrectomy (0.6%). CONCLUSION: We have described the laparoscopic pyeloplasty experience of institutions with diverse cultural and economic backgrounds. They had very similar outcomes, in agreement with previously published data. Based on these findings, we conclude that laparoscopic pyeloplasty is safe and successful in diverse geographics areas of the world.

Risk factors for febrile urinary tract infection in boys with posterior urethral valves.

Objective: Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors could allow for tailoring UTI prevention. The aim of this study was to use the data from the CIRCUP randomized controlled trial data to identify patient characteristics associated with a higher risk of fUTI. Patients and methods: We performed a secondary analysis of the data from the CIRCUP randomized trial which included boys with PUV, randomized to circumcision and antibiotic prophylaxis vs. antibiotic prophylaxis alone and followed for 2 years. There was only 1 episode of fUTI in the circumcision group vs. 17 in the uncircumcised group. We therefore only studied the antibiotic prophylaxis alone group and compared age at prenatal diagnosis, size and weight at birth, presence of dilating VUR at diagnosis, abnormal DMSA scan at 2 months, and nadir creatinine between children who presented a fUTI and those who did not, as well as age at first episode of fUTI. Results: The study group consisted of 42 patients of which 17 presented at least on fUTI. Presence of dilating VUR was significantly associated with risk of fUTI (p = 0.03), OR: 6 [CI 95% = (1.13-27.52)]. None of the other parameters were associated with increased risk of fUTI. We observed three distinct time periods for presenting a fUTI with a decrease in infection rate after the first 40 days of life, then at 240 days of life. Conclusion: In boys with PUV, presence of high-grade VUR is associated with a higher risk of presenting a fUTI. The rate of febrile UTIs seems to decrease after 9 months.

Postoperative JJ stent is not necessary after balloon high-pressure endoscopic dilatation of primary obstructive megaureter.

INTRODUCTION: A wide range of surgical interventions have been described for the management of primary obstructive megaureter (POM). Endoscopic balloon dilatation has been developed through last decades as a minimally invasive alternative to classic surgery. OBJECTIVE: To assess the need for placement of a double J stent after endoscopic balloon dilatation procedure, by comparing the post-operative related outcomes with and without double J placement. Secondary outcome was the success rate, considering the need for further procedure after endoscopic balloon dilatation and the improvement of the ureteral diameter in the two groups. STUDY DESIGN: Historical retrospective comparison of children treated by endoscopic dilatation for POM, with post-operative JJ stent left in place (2012-2014) or without ureteral JJ stent (since 2015). Post-operative complications were reported following Clavien-Dindo grading system and compared between the two groups. Success rate was defined as absence of need for further surgical reimplantation. Ureteral diameters on preoperative and postoperative renal ultrasounds were compared. RESULTS: Endoscopic dilatations were performed in 42 patients for 46 renal units during the study period. There was a significantly higher rate of post-operative complications in the group with JJ stenting compared to the group without double J stenting regarding all Clavien-Dindo grades (56% vs 15%, p = 0.014) and Clavien-Dindo grade III only (31% vs 0%, p = 0,0051) (Figure). The success rate was similar in the JJ group (75%, F-up: 70 months [13-101]) and the no JJ group (81%, F-up: 26 months [12-95]). There was a significant improvement of US renal pelvis and ureter dilatation in both groups, with a median follow-up of 35.5 months [12-101]. DISCUSSION: The overall rate of complications was slightly higher than in other reports and higher in the JJ group regarding Clavien-Dindo grade III complications. The success rate was comparable to previous studies reviewing endoscopic dilatations and equivalent in the two groups. CONCLUSION: In our study, the omission of postoperative ureteral drainage by a JJ stent after endoscopic balloon dilatation of POM did not increase post-operative complications rate without demonstrable impact on the success rate.

Adults with previous hypospadias surgery during childhood: Beware of bulbar strictures.

BACKGROUND: Urethral strictures represent the most frequent long-term complication in men after history of hypospadias surgery. OBJECTIVES: To better define the spectrum of men previously treated for hypospadias during childhood, presenting at an adult urology clinic with persistent urethral complications. STUDY DESIGN: Retrospective review of the records of 42 consecutive adult patients with a personal history of hypospadias repair during childhood referred at one adult tertiary urology center between 2004 and 2017. Those with persistent urethral strictures were included: 28 patients (median age 28yr [17-81]). Residual chordee, aesthetic concerns, isolated fistulas, and motives of consultation unrelated to hypospadias were excluded. Early initial success rate and location of urethral stricture were studied. Statistical Analysis was done by non-parametric tests. RESULTS: On the 42 consecutive adults eligible, a total of 28 patients had a persistent urethral stricture. During childhood, 13/28 boys had required multiple surgical revision procedures, including six of them for postoperative urethral strictures. During adulthood, initial urethral assessment revealed 29 urethral strictures in 28 patients (penile urethra 23/29, bulbar urethra 8/29). The early initial success rate of stricture treatment was 50% (median follow-up: 4 years [1-7]). Additional surgical procedures were needed and performed in 11 patients. Eight patients were eventually diagnosed with a bulbar stricture, either isolated (n = 5) or combined with a distal stricture (n = 3), without significant relationship with initial position of meatus. DISCUSSION: Predictive factors for bulbar strictures locations could not be identified. It was however observed that symptomatic bulbar strictures do exist in adults, in the long term after penile hypospadias repair during childhood, independently of the initial site of hypospadias, initial success of primary repair, and the type of surgical reconstructions performed during childhood. CONCLUSION: Bulbar strictures represent more than 25% of the overall strictures diagnosed in adult patients treated for hypospadias during childhood, independently of the original site of urethral reconstruction.

Unresectable thoracic neuroblastic tumors: Changes in image-defined risk factors after chemotherapy and impact on surgical management.

PURPOSE: Neuroblastoma management in children is multimodal and depends on multiple factors, including the possibility of complete surgical resection. Image-defined risk factors (IDRFs) are used to assess the feasibility of primary surgery. We studied the changes in IDRFs after neoadjuvant chemotherapy for thoracic neurogenic tumors. METHODS: We performed a multicenter review of 27 patients presenting with unresectable thoracic neurogenic tumors. Patients received neoadjuvant chemotherapy, according to their risk group. IDRF at diagnosis and before surgery were retrospectively analyzed by a radiologist and a surgeon, blind to the initial assessment. Surgical and oncologic outcomes were reviewed. RESULTS: None of the patients presented MYCN amplification, and 78 IDRFs were identified at diagnosis. Vascular IDRFs were the most frequent, with 28 vascular IDRFs detected in 18 patients, 22 of which disappeared after chemotherapy. Reductions of tumor volume were associated with a regression of IDRFs. Patients undergoing minimally invasive surgery had smaller tumor volumes than those undergoing open surgery, and no vascular IDRF. Two patients received two additional courses of chemotherapy to reduce tumor volume sufficiently for surgery. One patient with ganglioneuroblastoma underwent early surgery due to a lack of response to initial chemotherapy. CONCLUSION: Tumor volume reduction with neoadjuvant chemotherapy eliminates most IDRF in thoracic neurogenic tumors. Vascular IDRF are rapidly resolved at this site, making surgical resection and minimally invasive surgery possible.

Comparison of the outcomes of the pediatric kidney transplantation between recipients below and above 15 kg: a single center retrospective study.

BACKGROUND: The renal transplantation is the best treatment for end-stage renal disease in children. We present the findings of an analysis of our institution's paediatric transplant outcomes comparing recipients under 15 kg, who represent this potentially higher risk group, to those above 15 kg. METHODS: We retrospectively identified consecutive paediatric kidney transplants from a prospectively collected database for analysis. We included all recipients under the age of 18 years at the time of transplant between 2006 and 2018 without any exclusion criteria. The primary outcome was death-censored graft survival at 1 year, 5 years and 10 years. RESULTS: 109 paediatric kidney transplants were performed in 100 children. Graft survival in the all population was 98%, 96% and 76% at 1 year, 5 years and 10 years, respectively. Recipient weight below 15 kg was not found to be a risk factor of graft loss. Overall, we found no individual factor to be statistically significantly associated with renal graft lost. The overall complication rate was 16% (18/109) with 12 early complications (11%) and 6 late ones (5%). CONCLUSION: Kidney transplantation in children weighing < 15 kg seems safe and offers the same patient and graft survival outcomes as in other (> 15 kg) pediatric recipients with equally low complication rates.

Analysis of enteric nervous system and intestinal epithelial barrier to predict complications in Hirschsprung's disease.

In Hirschsprung's disease (HSCR), postoperative course remains unpredictable. Our aim was to define predictive factors of the main postoperative complications: obstructive symptoms (OS) and Hirschsprung-associated enterocolitis (HAEC). In this prospective multicentre cohort study, samples of resected bowel were collected at time of surgery in 18 neonates with short-segment HSCR in tertiary care hospitals. OS and HAEC were noted during postoperative follow-up. We assessed the enteric nervous system and the intestinal epithelial barrier (IEB) in ganglionic segments by combining immunohistochemical, proteomic and transcriptomic approaches, with functional ex vivo analysis of motility and para/transcellular permeability. Ten HSCR patients presented postoperative complications (median follow-up 23.5 months): 6 OS, 4 HAEC (2 with OS), 2 diarrhoea (without OS/HAEC). Immunohistochemical analysis showed a significant 41% and 60% decrease in median number of nNOS-IR myenteric neurons per ganglion in HSCR with OS as compared to HSCR with HAEC/diarrhoea (without OS) and HSCR without complications (p = 0.0095; p = 0.002, respectively). Paracellular and transcellular permeability was significantly increased in HSCR with HAEC as compared to HSCR with OS/diarrhoea without HAEC (p = 0.016; p = 0.009) and HSCR without complications (p = 0.029; p = 0.017). This pilot study supports the hypothesis that modulating neuronal phenotype and enhancing IEB permeability may treat or prevent postoperative complications in HSCR.

Preoperative Topical Estrogen Treatment vs Placebo in 244 Children With Midshaft and Posterior Hypospadias.

PURPOSE: Urethral fistula and dehiscence are common after hypospadias surgery. Preoperative androgens have been considered to reduce these complications although this consideration is not evidence-based. Dermatologists have reported the benefits of topical estrogens on skin healing. We investigated whether the preoperative use of topical promestriene could reduce healing complications in hypospadias surgery. Our primary objective was to demonstrate a reduction of healing complications with promestriene vs placebo. Impact on reoperations and other complications, clinical tolerance, bone growth, and biological systemic effects of the treatment were also considered. METHODS: We conducted a prospective, randomized, placebo-controlled, double-blind, parallel group trial between 2011 and 2015 in 4 French centers. One-stage transverse preputial island flap urethroplasty (onlay urethroplasty) was selected for severe hypospadias. Promestriene or placebo was applied on the penis for 2 months prior to surgery. The primary outcome was the presence of postoperative urethral fistula or dehiscence in the first year postsurgery. For safety reasons, hormonal and anatomical screenings were performed. RESULTS: Out of 241 patients who received surgery, 122 patients were randomized to receive placebo, and 119 patients received promestriene. The primary outcome was unavailable for 11 patients. Healing complications were assessed at 16.4% (19/116) in the placebo vs 14.9% (17/114) in the promestriene arm, and the odds ratio adjusted on center was 0.93 (95% confidence interval 0.45-1.94), P = 0.86. CONCLUSIONS AND RELEVANCE: Although we observed an overall lower risk of complications compared to previous publications, postsurgery complications were not different between promestriene and placebo, because of a lack of power of the study or the inefficacy of promestriene.

Minimally invasive surgery for unilateral Wilms tumors: Multicenter retrospective analysis of 50 transperitoneal laparoscopic total nephrectomies.

PURPOSE: To evaluate feasibility and outcomes of minimally invasive surgery (MIS) in Wilms tumor (WT). METHODS: International multicenter review of MIS total nephrectomies for WT between 2006 and 2018. Medical records of confirmed WT were retrospectively assessed for demographic, imaging, treatment, pathology, and oncological outcome data. RESULTS: Fifty patients, with a median age of 38 months (6-181), were included in 10 centers. All patients received neoadjuvant chemotherapy, as per SIOP protocol. Median tumor volume post-chemotherapy was 673 mL (18-3331), 16 tumors crossed the lateral border of the spine, and three crossed the midline. Six patients with tumors that crossed the lateral border of the spine (tumor volumes 1560 mL [299-2480]) were converted to an open approach. There was no intraoperative tumor rupture. Overall, MIS was completed in 19% of the 195 nephrectomies for WT presenting during the study period. Tumor was stage I in 29, II in 16, and III in 5, and histology was reported as low in three, intermediate in 42, and high risk in five. Three patients had positive tumor margins. After a median follow-up of 34 months (2-138), there were two local recurrences (both stage I, intermediate risk, 7 and 9 months after surgery) and one metastatic relapse (stage III, high risk, four months after surgery). The three-year event-free survival was 94%. CONCLUSION: MIS is feasible in 20% of WT, with oncological outcomes comparable with open surgery, no intraoperative rupture, and a low rate of local relapse. Ongoing surveillance is, however, needed to evaluate this technique as it becomes widespread.

Primary female epispadias: Perineal approach or Kelly repair?

OBJECTIVES: Primary female epispadias encompasses a spectrum of disease, presenting with a variable degree of incontinence. We hypothesized that although perineal urethrocervicoplasty can be a successful first-line procedure in patients with normal bladder, a more radical reconstruction was necessary to achieve continence in cases lying at the most severe end of the spectrum. Our aim was to assess the results of a surgical management using perineal approach in girls with normal bladder capacity, and Kelly radical soft-tissue mobilization (RSTM) in patients with inadequate bladder, based on the assumption that bladder capacity (BC) is a reliable marker of epispadias severity. STUDY DESIGN: Prospective inclusion of incontinent girls with female epispadias referred to a single institution. Patients with normal BC were treated with perineal urethrocervicoplasty (PUCP, group 1). Patients with small bladder underwent RSTM (group 2). Follow-up was at 1, 3, 6, and 12 months postoperatively, then annually, including physical examination, renal ultrasound at each visit, continence status, and estimation of functional/maximal BC. The main study outcome was continence status at the age of 5 years or later, if postoperative follow-up was >12 months. RESULTS: From 2006 to 2017, 16 consecutive children were prospectively included in this study, at a median age of 39 months (5-102 months). Seven girls were included in group 1 and underwent PUCP; at the last follow-up, five out of seven were dry by day (4/5 day and night), although three out of five required bladder-neck injection after perineal reconstruction due to stress incontinence. Two patients with persistent incontinence and absence of BC increase after PUCP subsequently underwent RSTM. Eleven patients with low BC (56% [10-94%] of expected BC) were included in group 2 (9 without prior surgery, 2 after PUCP failure). Among the eight evaluable patients, eight out of eight achieved diurnal continence, and 3/8 were fully continent. One girl with obstructive micturition required clean intermittent catheterization. DISCUSSION: The traditional approach of female epispadias based on staged reconstruction (urethroplasty followed by bladder-neck reconstruction) raised concerns regarding the risk of non-physiological obstructive micturition. The perineal approach was suggested as an alternative, with reported diurnal continence rates of 60-80%, but less than 50% of nocturnal continence, presumably in relation with limited bladder capacity. In cases selected within the most severe end of the epispadias spectrum, the Kelly RSTM seems to offer excellent continence rates. CONCLUSION: A tailored approach to female epispadias, based on perineal reconstruction in favorable cases, and radical soft-tissue mobilization in severe cases, seems to yield good continence outcomes in the long term.

Cytoreductive surgery and hyperthermic intraperitoneal perfusion with chemotherapy in children with peritoneal tumor spread: A French nationwide study over 14 years.

BACKGROUND: Efficacy and role of cytoreductive surgery (CRS) and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) remain poorly documented in pediatric tumors. METHODS: This retrospective national study analyzed all pediatric patients with peritoneal tumor spread treated by CRS and HIPEC as part of a multimodal therapy in France from 2001 to 2015. RESULTS: Twenty-two patients (nine males and 13 females) were selected. The median age at diagnosis was 14.8 years (4.2-17.6). Seven had peritoneal mesotheliomas; seven, desmoplastic small round cells tumors (DSRCT); and eight, other histologic types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 (73%) cases. Incomplete resections were classified as CC-1 in four (18%) cases and CC-2 in two (9%) cases. Fourteen (64%) patients had complications within 30 days from HIPEC, requiring an urgent laparotomy in eight (36%) cases. Thirteen (59%) patients received adjuvant chemotherapy and four (18%) received total abdominal radiotherapy after surgery. Sixteen (72%) patients had relapse after a median time of 9.6 months (1.4-86.4) and nine (41%) eventually died after a median time of 5.3 months (0.1-36.1) from relapse. Six (27%) patients (four mesotheliomas, one pseudopapillary pancreatic tumor, and one DSRCT) were alive and in complete remission after a median follow-up of 25.0 months (5.3-78.2). The mean overall survival (OS) and disease-free survival (DFS) were 57.5 months (95% CI [38.59-76.32]) and 30.9 months (95% CI [14.96-46.77]). Patients with a peritoneal mesothelioma had a significantly better OS (p = 0.015) and DFS (p = 0.028) than other histologic type. CONCLUSIONS: In this national series, outcomes of HIPEC are encouraging for the treatment of peritoneal mesothelioma in children.

Standardization of pediatric uroradiological terms: a multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.

Standardization of pediatric uroradiological terms: A multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.