SRY-negative 46,XX testicular/ovotesticular DSD: Long-term outcomes and early blockade of gonadotropic axis.

OBJECTIVE: SRY-negative 46,XX testicular and ovotesticular disorders/differences of sex development (T/OTDSD) represent a very rare and unique DSD condition where testicular tissue develops in the absence of a Y chromosome. To date, very few studies have described the phenotype, clinical and surgical management and long-term outcomes of these patients. Particularly, early blockade of the gonadotropic axis in patients raised in the female gender to minimize postnatal androgenization has never been reported. DESIGN: Retrospective description of sixteen 46,XX T/OTDSD patients. RESULTS: Sixteen 46,XX SRY-negative T/OTDSD were included. Most (12/16) were diagnosed in the neonatal period. Sex of rearing was male for six patients and female for ten, while the clinical presentation varied, with an external masculinization score from 1 to 10. Five patients raised as girl were successfully treated with GnRH analog to avoid virilization during minipuberty. Ovotestes/testes were found bilaterally for 54% of the patients and unilaterally for the others (with a contralateral ovary). Gonadal surgery preserved appropriate tissue in the majority of cases. Spontaneous puberty occurred in two girls and one boy, while two boys required hormonal induction of puberty. One of the girls conceived spontaneously and had an uneventful pregnancy. DNA analyses (SNP-array, next-generation sequencing and whole-exome sequencing) were performed. A heterozygous frameshit mutation in the NR2F2 gene was identified in one patient. CONCLUSIONS: This study presents a population of patients with 46,XX SRY-negative T/OTDSD. Early blockade of gonadotropic axis appears efficient to reduce and avoid further androgenization in patients raised as girls.

Double-face preputial island flap revisited: is it a reliable one-stage repair for severe hypospadias?

PURPOSE: We have studied outcome of double-face preputial island flap (DFPIF) technique in severe types of hypospadias: penoscrotal, scrotal and perineal. METHODS: We have used DFPIF in 75 boys at a median age of 1.1 years (1.0-1.5). The meatus was penoscrotal, scrotal or perineal after de-gloving the penis. The inner face of the foreskin was used for urethroplasty and the outer face for ventral skin covering. Modifications were added: proximal anastomosis was protected by a spongioplasty; in case of urethral plate transection, we anastomosed on onlay proximal and distal segments of the flap (onlay-tube-onlay) and the tubularized part was sutured to corpus cavernosa. FU was scheduled at one month then every 3 months for a year then annually. At each consultation, the surgeon filled out a detailed cosmetic and functional sheet including flowmeter. RESULTS: Thirty-four patients had onlay preputial flap repair with urethral plate preservation. Forty-one had the onlay-tube-onlay technique. All children had a curvature, 19 had a significant residual curvature after dissection, corrected by dorsal plication (n = 9) and ventral lengthening (n = 10). Median FU was 4.2 years (2.7-6.5). 36 children (48%) had complications and needed redo surgery: 12 fistulas, 11 diverticula, 7 meatal stenosis, 3 strictures and 2 residual curvatures. All children but three voided within the normal limits for their age. CONCLUSION: DFPIF remains a good option for a one-stage repair of severe hypospadias. After a median of 1.8 procedures, the final success rate was 96%. The healthy well-vascularized ventral skin allows safe redo surgery when needed.

Mast cell chymase protects against acute ischemic kidney injury by limiting neutrophil hyperactivation and recruitment.

Here we investigated the role of murine mast cell protease 4 (MCPT4), the functional counterpart of human mast cell chymase, in an experimental model of renal ischemia reperfusion injury, a major cause of acute kidney injury. MCPT4-deficient mice had worsened kidney function compared to wildtype mice. MCPT4 absence exacerbated pathologic neutrophil infiltration in the kidney and increased kidney myeloperoxidase expression, cell death and necrosis. In kidneys with ischemia reperfusion injury, when compared to wildtype mice, MCPT4-deficient mice showed increased surface expression of adhesion molecules necessary for leukocyte extravasation including neutrophil CD162 and endothelial cell CD54. In vitro, human chymase mediated the cleavage of neutrophil expressed CD162 and also CD54, P- and E-Selectin expressed on human glomerular endothelial cells. MCPT4 also dampened systemic neutrophil activation after renal ischemia reperfusion injury as neutrophils expressed more CD11b integrin and produced more reactive oxygen species in MCPT4-deficient mice. Accordingly, after renal injury, neutrophil migration to an inflammatory site distal from the kidney was increased in MCPT4-deficient versus wildtype mice. Thus, contrary to the described overall aggravating role of mast cells, one granule-released mediator, the MCPT4 chymase, exhibits a potent anti-inflammatory function in renal ischemia reperfusion injury by controlling neutrophil extravasation and activation thereby limiting associated damage.

Association of Maternal First Trimester Serum Levels of Free Beta Human Chorionic Gonadotropin and Hypospadias: A Population Based Study.

PURPOSE: Human chorionic gonadotropin stimulates fetal testosterone production and contributes to normal development of male genitalia. Using population based data we hypothesized that differences in maternal free beta human chorionic gonadotropin may be associated with hypospadias. MATERIALS AND METHODS: Data were obtained from the Paris Registry of Congenital Malformations (REMAPAR) (2011 to 2016). The initial study population included 3,172 pregnant women who gave birth to a singleton live born male infant with a congenital malformation. After exclusion of cases with unknown beta human chorionic gonadotropin and those with chromosomal or genetic abnormalities, the study population included 194 boys with isolated hypospadias and 1,075 controls. For cases with operative notes (125) we obtained data on type (proximal/distal) of hypospadias. Using quantile regression we compared median values of multiple of median beta human chorionic gonadotropin measured for first trimester Down syndrome screening (10th to 13th gestational weeks) for overall as well as by type of hypospadias vs controls. We also considered possible effects of placental dysfunction (maternal age, intrauterine growth retardation and preterm births) as potential confounding factors. RESULTS: Overall the median beta human chorionic gonadotropin multiple of median was comparable for women who had an infant with hypospadias vs controls (0.99 vs 0.95, p=0.3). However, proximal hypospadias was associated with a statistically significant higher median multiple of median than distal hypospadias or unspecified (1.49 vs 0.92 vs 1.05, p=0.02). The estimates were comparable after adjustment for placental dysfunction. CONCLUSIONS: Our findings support the hypothesis that an alteration in maternal beta human chorionic gonadotropin levels is associated with hypospadias. However, this association appears to be limited to proximal hypospadias.

Laparoscopic retroperitoneal approach for retrocaval ureter in children.

PURPOSE: Retrocaval ureter (RCU) is a rare congenital anomaly and published data on pediatric laparoscopic management are poor. The aim of this study was to report our experience of retroperitoneal laparoscopic approach for management of RCU in children. METHODS: A retrospective review of data from patients treated for RCU between 2002 and 2018 in our institution was performed. All patients were positioned in a flank position and underwent a three-port (5-mm optical trocar and two 3-mm trocars) laparoscopic retroperitoneal ureteroureterostomy. Anastomosis was made by 6/0 absorbable sutures. A JJ stent was always inserted. RESULTS: Five patients with a median age of 94 months (5-152) were operated on and followed up for a median time of 103 months (46-201). Median operating time was 200 min (160-270). No conversion and no transfusion occurred. Median hospital stay was 2 days (1-4). Ureteral stent was removed after 52 days (47-82). Complications included pyelonephretis (N = 1). In all cases, hydronephrosis decreased postoperatively. CONCLUSIONS: Retroperitoneal laparoscopic approach for RCU is safe and effective in children. Our video demonstrates different patients with specific surgical details to show how to manage these children. The global vision of the upper tract by laparoscopy leads to optimal management of these children even if the anomaly was not detected preoperatively.

Mast Cell Chymase and Kidney Disease.

A sizable part (~2%) of the human genome encodes for proteases. They are involved in many physiological processes, such as development, reproduction and inflammation, but also play a role in pathology. Mast cells (MC) contain a variety of MC specific proteases, the expression of which may differ between various MC subtypes. Amongst these proteases, chymase represents up to 25% of the total proteins in the MC and is released from cytoplasmic granules upon activation. Once secreted, it cleaves the targets in the local tissue environment, but may also act in lymph nodes infiltrated by MC, or systemically, when reaching the circulation during an inflammatory response. MC have been recognized as important components in the development of kidney disease. Based on this observation, MC chymase has gained interest following the discovery that it contributes to the angiotensin-converting enzyme's independent generation of angiotensin II, an important inflammatory mediator in the development of kidney disease. Hence, progress regarding its role has been made based on studies using inhibitors but also on mice deficient in MC protease 4 (mMCP-4), the functional murine counterpart of human chymase. In this review, we discuss the role and actions of chymase in kidney disease. While initially believed to contribute to pathogenesis, the accumulated data favor a more subtle view, indicating that chymase may also have beneficial actions.

Renal parenchyma impairment characterization in partial unilateral ureteral obstruction in mice with intravoxel incoherent motion-MRI.

Ureteropelvic junction obstruction constitutes a major cause of progressive pediatric renal disease. The biological mechanisms underlying the renal response to obstruction can be investigated using a clinically relevant mouse model of partial unilateral ureteral obstruction (pUUO). Renal function and kidney morphology data can be evaluated using renal ultrasound, scintigraphy and uro-magnetic resonance imaging (uro-MRI), but these methods are poorly linked to histological change and not all are quantitative. Here, we propose to investigate pUUO for the first time using an intravoxel incoherent motion diffusion sequence. The aim of this study was to quantitatively characterize impairment of the kidney parenchyma in the pUUO model. This quantitative MRI method was able to assess the perfusion and microstructure of the kidney without requiring the injection of a contrast agent. The results suggest that a perfusion fraction (f) reduction is associated with a decrease in the volume of the renal parenchyma, which could be related to decreased renal vascularization. The latter may occur before impairment by fibrosis and the findings are in accordance with the literature using the UUO mice model and, more specifically, on pUUO. Further investigation is required before this technique can be made available for the diagnosis and management of children with antenatal hydronephrosis and to select the optimal timing of surgery if required.

Urine biochemistry to predict long-term outcomes in fetuses with posterior urethral valves.

OBJECTIVE: Because the literature on the predictive value of fetal urinalysis is controversial in fetuses with lower urinary tract obstruction, we determined the best model of fetal urine biochemical markers correlated with long-term postnatal renal function based on glomerular filtration rate (GFR). METHOD: This retrospective study concerned 89 fetuses with lower urinary tract obstruction and their renal function after 10 years of age. We correlated fetal urine biochemical markers (total protein, ß2-microglobulin, sodium, chloride, glucose, calcium, and phosphorus) with GFR at 10 to 30 years of age in 89 patients with posterior urethral valves. We defined five stages of chronic kidney disease (CKD). RESULTS: Of the 89 patients, 18 (20%) are 20 years old or over. Postnatal renal function was good in 67.4% (GFR > 60 mL/min/1.73 m2 ) and poor in 17% (GFR < 30 mL/min/1.73 m2 ). All fetal urine markers differed between CKD stage 1 + 2 and CKD stage 4 + 5 (P < 0.001). ß2-microblobulin showed an 87% sensitivity for a 72% specificity. A combination of ß2-microglobulin and chloride gave the best results (93% sensitivity and 71% specificity) versus amniotic fluid volume (80% sensitivity and 73% specificity). CONCLUSION: Fetal urine biochemistry predicts long-term (10-30 years) postnatal renal function.

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.

Posterior urethral valves and vesicoureteral reflux: can prenatal ultrasonography distinguish between these two conditions in male fetuses?

OBJECTIVE: The objective of the study was to evaluate prenatal sonographic signs that distinguish male fetuses with posterior urethral valves (PUV) from those with vesicoureteral reflux (VUR). METHODS: Prenatal data were retrospectively retrieved from all consecutive women delivering between 2003 and 2012 of a male newborn with a postnatal diagnosis of PUV or VUR. Prenatal parameters included fetal bladder characteristics, identification of a dilated posterior urethra, and change in shape and size in the fetal renal pelvis or ureter. RESULTS: One hundred thirty-six women gave birth to a male newborn with a postnatal diagnosis of PUV (n = 49) or VUR (n = 87). The presence of posterior urethral dilatation [21 (42.9%) fetuses versus 0 (0%), p = 0.000], a thickened fetal bladder wall [37 (75.5%) vs 4 (4.6%), p = 0.000], and anhydramnios [14 (28.6%) vs 0, p = 0.000] were strongly associated with the postnatal diagnosis of PUV. Change in shape and size in the fetal renal pelvis or ureter was observed in 15 (17.2%) of 87 children with VUR versus 1 (2.0%) out of 49 with PUV (p = 0.010). CONCLUSIONS: Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR. © 2016 John Wiley & Sons, Ltd.

Long-term bladder function after ureterocele decompression in children.

PURPOSE: There is limited knowledge of long-term bladder function after ureterocele decompression. We studied bladder function in patients who underwent surgery in childhood for duplex system ureteroceles. MATERIALS AND METHODS: Toilet trained children treated for duplex system ureteroceles between 1990 and 2010 were included in study. We evaluated voiding dysfunction by the valid DVSS questionnaire and noninvasive studies, including uroflowmetry, electromyogram and post-void residual urine measurement. Urodynamics were done only in patients with abnormal DVSS or abnormal noninvasive studies. Patients were divided into group 1-ureterocele decompression (endoscopic incision), upper pole partial nephrectomy and ureteropelvic anastomosis, and group 2-primary or secondary bladder surgery (ureterocelectomy, ureterovesical reimplantation and bladder floor reconstruction). RESULTS: Of 62 operated patients 17 were lost to followup and 45 were fully studied at a mean followup of 9.5 years (range 3 to 20). Initial surgery was done at mean age of 5.1 months (range 6 days to 48 months). In the 33 group 1 patients, of whom 70% underwent endoscopic incision, the mean DVSS score was 1.5 (range 0 to 6), 7 patients (22%) had abnormal uroflowmetry or significant post-void residual urine and none had abnormal DVSS results. In the 12 patients in group 2 the mean DVSS score was 4 (range 0 to 11), 8 patients (66%) had abnormal uroflowmetry and significant post-void residual urine, and 3 had abnormal DVSS findings (p = 0.036). All group 2 patients underwent bladder surgery after decompression, including endoscopic incision in 2 and upper pole partial nephrectomy in 1. Only 1 child needed clean intermittent catheterization at age 3 years for hypocontractile megacystis and repeat febrile urinary tract infections. CONCLUSIONS: Ureterocele decompression alone in early childhood does not lead to major bladder dysfunction at long-term evaluation. Even if secondary bladder surgery is needed, significant bladder dysfunction is rare.

Laparoscopic pyeloplasty in children with horseshoe kidney.

PURPOSE: Laparoscopic pyeloplasty for ureteropelvic junction obstruction associated with horseshoe kidney has been described in adults but seldom in young children. We describe our experience in 10 children treated successfully with laparoscopic dismembered pyeloplasty. MATERIALS AND METHODS: Eight boys and 2 girls with a mean age of 8 years (range 0.7 to 16.5) underwent laparoscopic dismembered pyeloplasty for ureteropelvic junction obstruction associated with horseshoe kidney between 2002 and 2012. Presenting symptoms were abdominal pain (3 patients), urinary tract infection (2), abdominal mass (1) and hematuria (1). Horseshoe kidney was diagnosed preoperatively in all cases but 1. The anastomosis was done by running or interrupted 5-zero or 6-zero resorbable sutures and drained by a Double-J® stent. RESULTS: Laparoscopic dismembered pyeloplasty was feasible in all cases. Mean operating time was 220 minutes (range 180 to 260). Anatomical abnormalities included anteriorly crossing vessels at the ureteropelvic junction in 6 cases (polar vessels in 4, ureteropelvic junction posterior to an abnormal branch of vena cava in 1 and renal vein in 1) and high ureteral insertion in 4. Two children underwent an extensive reduction pyeloplasty. Mean hospital stay was 3.2 days (range 1 to 8). Mean followup was 50 months (range 4 to 132). All patients were asymptomatic with significant improvement of dilatation. CONCLUSIONS: The transperitoneal laparoscopic approach is adapted for pyeloplasty in children with horseshoe kidney. This procedure allows global exploration of the upper tract and efficient identification of anatomical anomalies, especially crossing vessels. Although our series is small, it is the first known description specific to horseshoe kidney in children and demonstrates that this approach has lasting effectiveness in young children.

Genito-urinary Reconstruction in Female Children With Congenital Adrenal Hyperplasia: Favorable Surgical Outcomes can be Achieved by Contemporary Techniques and a Dedicated Multidisciplinary Management.

INTRODUCTION: Congenital adrenal hyperplasia (CAH) is the most common cause of genital atypia in females. A dedicated multidisciplinary team (MDT) should be included for an optimal management. Here, we aimed to review our surgical experience and to assess long-term urinary, gynecological and endocrine outcomes after primary genitoplasty in this specific cohort. METHODS: Patients born with CAH and who underwent feminizing genitoplasty in our institution were retrospectively identified (2001-2021). We analyzed patients' characteristics, intraoperative details, and postoperative urinary, gynecological, and endocrine outcomes. RESULTS: Forty patients were included and followed-up for a median (IQR) time of 7 (1-19) years. Thirty-eight (95%) had 21-hydroxylase deficiency. After multidisciplinary decision and written consent from patient and/or family, a single-stage reconstructive surgery was performed at a median age of 10 (3-165) months. Median length of hospital stay was 5 (1-7) days. Procedures were: PUM (N = 35 (87.5%)), TUM (N = 3 (7.5%)), urogenital mobilization was unnecessary in 2 (5%). Reduction clitoroplasty was done in 33 (82.5%) patients. Only 3 (7.5%) experienced significant Clavien-Dindo complications requiring additional surgery during the follow-up period. Recurrent urinary tract infections (UTI) occurred in 6 (15%), one required ureteric reimplantation for symptomatic high-grade vesicoureteric reflux. All patients over 3 years were toilet-trained without incontinence. Severe vaginal stenosis occurred in 1 (2.5%) patient. In patients who achieved puberty, 6/9 had vaginal calibration at a median age of 17.3 (16-21) years without detected stenosis. One (2.5%) had major hypertrophy of the right labia minora requiring labiaplasty. Nine (22.5%) reached puberty. Two (5%) patients developed acne/hirsutism. Short stature was noted in 11 (27.5%) and obesity in 18 (45%). CONCLUSION: Based on our contemporary series, genitourinary reconstructive surgery for female patients born with CAH is technically feasible and safe with a low complication rate. A regular follow-up with a MDT to assess long-term complications is necessary, and it is vital to inform patients and families about the different management options with all the risks and benefits of surgery. TYPE OF THE STUDY: original research, clinical research. LEVEL OF EVIDENCE: Level 3 retrospective study.

A catheterizable serous-lined urinary outlet associated with the ileal bladder augmentation Abol-Enein and Ghoneim procedure: a safe and reliable procedure in children.

Purpose: This study aims to evaluate the long-term outcome of the serous-lined extramural continent catheterizable outlet procedure (SLECCOP) associated with ileal bladder augmentation in children. Methods: This was a monocentric and retrospective study (2002-2021) that included children (<18 years) undergoing the SLECCOP associated with W-shaped ileocystoplasty with a catheterizable channel (Abol-Enein and Ghoneim procedure). Patients who received other types of bladder augmentation or W-shaped ileocystoplasty without a catheterizable channel were excluded. Patient records were reviewed for demographic information, surgical data, and long-term outcomes. Results: This study included 52 children [33 boys, median age: 8.5 (0.8-18) years]. Pathological conditions included 28 children with the bladder exstrophy and epispadias complex (BEEC), 11 with neurogenic bladders, and 13 with other pathologies. Two patients underwent total bladder substitution. Thirty-four (65%) patients had bladder neck reconstruction (BNR), with 23 undergoing the SLECCOP and ileocystoplasty and 11 having prior BNR. All stomas, except for two, were umbilical, and were associated with omphaloplasty in 28 patients with the BEEC. A total of 40 stomas were created using the appendix (77%) and 12 with a Monti tube (23%). Stoma-related complications included cutaneous strictures (n = 2, 4%) and leaks (n = 10, 19%), all treated by dextranomer/hyaluronic acid copolymer injection (n = 10). A redo surgery was required in three patients: extraserosal wrapping was performed for persistent leakage (n = 2, 4%), and surgical revision was required for the Monti tube procedure (n = 1, 2%). Three patients (6%) underwent dilatation for transient stoma stenosis. Leakage occurred in 20% of appendix channels (n = 8/40) and 17% of Monti tubes (n = 2/12). Strictures were reported in 3% of appendix channels (n = 1/40) and 8% of Monti tubes (n = 1/12). Bladder stones developed in four patients (8%). Channel leakage persisted in one patient (2%) at a median follow-up of 4.4 years (IQR 1.4-9.7). Conclusion: W-Ileal bladder augmentation with the SLECCOP is an efficient technique for treating children with incontinence caused by different etiologies. The rate of channel complication is very low, specifically for strictures, in this complex population of patients.

Staged laparoscopic orchiopexy of intra-abdominal testis: Spermatic vessels division versus traction? A multicentric comparative study.

BACKGROUND: Staged laparoscopic management of intra-abdominal testes using pedicular section is recognized as gold standard technique, successful in 85 % of cases for scrotal testicular position with less than 10 % testicular atrophy. Recently, Shehata proposed a new technique without pedicular division for these testes, using spermatic vessels traction, but did not provide a comparative study of the two techniques. OBJECTIVE: To evaluate the laparoscopic spermatic pedicular traction (Shehata technique, ST) for the treatment of intra-abdominal testis, as an alternative to gold standard pedicular section (2-stage Fowler-Stephens, FS). STUDY DESIGN: Intra-abdominal testes of 129 patients in two tertiary pediatric urology centers were managed laparoscopically (2011-2019) either by 2-stage FS orchidopexy or ST according to the surgeon preference. Testicular position and size were statistically compared. RESULTS: A total of 147 testes were pulled down by 80 ST and 67 FS, including 18 bilateral cases. Median (IQR) age at surgery was 24.2 (15.6-46.4) months (ST) and 18.3 (13.1-38.2) months (FS) (p = 0.094). Scrotal pulling-down of the testis was performed after a median (IQR) period of 2.3 (1.6-3.4) months (ST) and 6.1 (4.7-8.3) months (FS), respectively (p < 0.005). Although ST had collapsed in 17 cases (21.3 %), only one (1.3 %) redo procedure was required. After a median (IQR) follow-up of 22 (12-40) and 19 (8.75-37) months (p = 0.59), the testis was in the scrotum in 85 % and 81 % of ST and FS cases, respectively (p = 0.51). Testicular atrophy occurred in 10 % of ST and 13.4 % of FS (p = 0.61). Multivariate analysis using the propensity score analysis did not identify any difference between the two techniques. DISCUSSION: Our results seem to confirm that FS and ST achieve the same results regarding final testicular position and testicular atrophy rate, with a long-term follow-up. Our study supports pediatric surgeons to favor laparoscopic spermatic pedicular traction (ST) which preserves the testicular vascularization and may ensure better spermatogenesis after puberty. More details on the size and position of the testicle at the beginning of the first laparoscopy seem however essential to assess more accurately the outcomes of each surgical technique. Our outcomes will also be re-evaluated when our patients have reached puberty, from an exocrine and endocrine points of view. CONCLUSIONS: This study showed similar results after laparoscopic traction or section of spermatic vessels for intra-abdominal testis in a long-term follow-up, providing more evidence for the use of ST as a valuable alternative to FS.

Evaluating pediatric ureteropelvic junction obstruction: Dynamic magnetic resonance urography vs renal scintigraphy 99m-technetium mercaptoacetyltriglycine.

BACKGROUND: Ureteropelvic junction obstruction (UPJO) is a common congenital urinary tract disorder in children. It can be diagnosed as early as in utero due to the presence of hydronephrosis or later in life due to symptomatic occurrence. AIM: To evaluate the discrepancy between dynamic contrast-enhanced magnetic resonance urography (dMRU) and scintigraphy 99m-technetium mercaptoacetyltriglycine (MAG-3) for the functional evaluation of UPJO. METHODS: Between 2016 and 2020, 126 patients with UPJO underwent surgery at Robert Debré Hospital. Of these, 83 received a prenatal diagnosis, and 43 were diagnosed during childhood. Four of the 126 patients underwent surgery based on the clinical situation and postnatal ultrasound findings without undergoing functional imaging evaluation. Split renal function was evaluated preoperatively using scintigraphy MAG-3 (n = 28), dMRU (n = 53), or both (n = 40). In this study, we included patients who underwent surgery for UPJO and scintigraphy MAG-3 + dMRU but excluded those who underwent only scintigraphy MAG-3 or dMRU. The patients were divided into groups A (< 10% discrepancy) and B (> 10% discrepancy). We examined the discrepancy in split renal function between the two modalities and investigated the possible risk factors. RESULTS: The split renal function between the two kidneys was compared in 40 patients (28 boys and 12 girls) using scintigraphy MAG-3 and dMRU. Differential renal function, as determined using both modalities, showed a difference of < 10% in 31 children and > 10% in 9 children. Calculation of the relative renal function using dMRU revealed an excellent correlation coefficient with renal scintigraphy MAG-3 for both kidneys. CONCLUSION: Our findings demonstrated that dMRU is equivalent to scintigraphy MAG-3 for evaluating split renal function in patients with UPJO.

Outcome and etiologies of fetal megacystis according to the gestational age at diagnosis.

OBJECTIVE: To investigate the gestational age-specific outcomes and the different etiologies of megacystis diagnosed at screening ultrasound. METHODS: A retrospective single-center study was conducted between 1989 and 2009. We identified all consecutive cases of megacystis prenatally diagnosed during routine ultrasound screening. Outcome, final diagnosis, and renal function were recorded. RESULTS: Eighty-four patients were included. An isolated lower urinary tract obstruction was observed in 38/84 (45.2%), ureterovesical reflux in 9/84 (10.7%), an associated congenital abnormality in 32/84 (38.1%) and a normal bladder in 5/84 (6%). Increased gestational age at diagnosis was correlated with an increased rate of live born children (P < 0.01). No cases of megacystis diagnosed in the first trimester were born alive. When diagnosis of posterior urethral valves (PUV) was made in the third trimester, the ultimate survival rate was 11/13 (84.6%) compared with 3/12 (25%) for a diagnosis made in the second trimester (P = 0.02). CONCLUSION: Lower urinary tract obstruction is the main etiology of megacystis. Megacystis can also be part of more complex malformations. Outcome of megacystis detected in the first trimester is poor. PUV detected in the third trimester had a better overall survival rate than PUV detected in the second trimester.

[Advances in pediatric urology]. / Les progrès de l'urologie pediatrique.

Urogenital disorders in children include congenital malformations affecting the urinary system and the genital organs, as well as disorders acquired during childhood. They require medical and surgical treatment by a dedicated team, notably a paediatric urologist, nurse and psychologist. Significant advances have been made in this discipline over the last ten years.

Pediatric robot-assisted extravesical ureteral reimplantation (revur) in simple and complex ureter anatomy: Report of a multicenter experience.

BACKGROUND: Robot-assisted extravesical ureteral reimplantation (REVUR) is a well established approach for surgical treatment of pediatric vesicoureteral reflux (VUR). However, further evidence is needed to confirm its efficacy even in case of complex anatomy. OBJECTIVE: This study aimed to further confirm the evidence that REVUR is safe and effective in both simple and complex ureter anatomy. STUDY DESIGN: The charts of all patients with VUR, who received REVUR in 6 different institutions over a 5-year period, were retrospectively reviewed. Patients with both simple and complex ureter anatomy were included. Patient demographics, surgical variables, and post-operative results were assessed. VUR resolution was defined as either being resolved VUR on voiding cystourethrogram (VCUG) or clinically without symptoms during the follow-up. RESULTS: Fifty-seven patients with median age of 6.9 years (range 4.5-12), receiving REVUR in the study period, were included. Eighteen (31.6%) patients had complex anatomy and included prior failed endoscopic injection (n = 13), complete ureteral duplication (n = 2), periureteral diverticulum (n = 2), ectopic megaureter requiring dismembering (n = 1). The median operative time was 155 min for unilateral and 211.5 min for bilateral repairs. The clinical + radiographic VUR resolution rate was 96.5%. Post-operative complications (Clavien 2) included urinary retention following bilateral repair (n = 5, 8.7%), febrile urinary tract infection (UTI) (n = 6, 10.5%) and gross hematuria (n = 3, 5.2%). Comparative analysis between simple and complex cases showed that REVUR was faster in simple cases in both unilateral [p = 0.002] and bilateral repair [p = 0.001] and post-operative urinary retention was more frequent in simple cases [p = 0.004] and in patients with pre-operative bowel and bladder dysfunction (BBD) [p = 0.001] (Table). DISCUSSION: This series confirmed that the robot-assisted technique was feasible even in cases with complex anatomy using some technical refinements, that justified the longer operative times in both unilateral and bilateral cases. An interesting finding of this study was the correlation emerged between BBD and risk of post-operative urinary retention and VUR persistence. Our results also excluded any significant correlation between complex cases and risk of post-operative urinary retention. The main study limitations included the retrospective and nonrandomized design, the small number of cases and the arbitrary definition of complex anatomy. CONCLUSION: REVUR was safe and effective for management of VUR in both simple and complex ureter anatomy. Complex REVUR required slightly longer operative times, without significant differences in post-operative mordidity and success rates. Aside from complex anatomy, BBD emerged as the main risk factor associated with surgical failure and post-operative morbidity.