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A major complication of feminizing genitoplasty in children is the loss of clitoral sensation with serious impact at adult life. We suggest a new method to evaluate the surgical results during childhood based on the bulbocavernosus or clitoro-perineal reflex (CPR). The afferent pathway of CPR implies the intact sensory receptors on the clitoral glans. Girls with congenital adrenal hyperplasia who were followed-up medically without surgery or who underwent feminizing genitoplasty with or without clitoroplasty were included (2002–2018). All clitoroplasties were standardized reduction clitoroplasty with preservation of neurovascular bundles associated with vaginoplasty and vestibuloplasty. Standardized examinations were prospectively performed including the CPR starting at one year postoperatively. The reflex was triggered by gentle touch of the glans by a cotton swab. Contraction of the perineal muscles was considered positive. Thirty-two children were operated at a median age of 8.6 months (5.8–12.1). Median follow-up (FU) was 3.9 years (1.3–6.4). Twenty-four patients had clitoroplasties: 17 were tested for CPR at one-year FU, and all had a positive test. Eight girls had genitoplasty without clitoral surgery, two of them were tested and were positive. Ten patients were managed without surgery, two of them were tested for the CPR and were positive. The reflex was always triggered easily and repeated at least twice during the FU. The clitoro-perineal reflex is a simple, non-invasive and reproducible test in early childhood and may serve as an early evaluation tool of clitoral innervation after feminizing genitoplasty. These results need to be confirmed at long term and completed at adult life.

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. 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. 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. 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.

Introduction The preservation of fertility is an integral part of care of children requiring gonadotoxic treatments for cancer or non-malignant diseases. In France, the cryopreservation of ovarian tissue has been considered and has been offered as a clinical treatment since its inception. The aim of this study is to review 20 years of activity in fertility preservation by ovarian tissue cryopreservation (OTC) for children and the feasibility of oocyte isolation and cryopreservation from the ovarian tissue at a single center. Material and methods Retrospective study including patients aged 15 years or younger who underwent OTC, combined for some with oocyte cryopreservation of isolated oocytes, before a highly gonadotoxic treatment for malignant or non-malignant disease was initiated. We describe the evolution of activities in our program for fertility preservation and patient characteristics at the time of OTC and follow up. Results From April 1998 to December 2018, 418 girls and adolescents younger than 15 years of age underwent OTC, representing 40.5% of all females who have had ovarian tissue cryopreserved at our center. In all, 313 patients had malignant diseases and 105 had benign conditions. Between November 2009 and July 2013, oocytes were isolated and also cryopreserved in 50 cases. The mean age of patients was 6.9 years (range 0.3-15). The most frequent diagnoses in this cohort included neuroblastoma, acute leukemia and hemoglobinopathies; neuroblastoma being the most common diagnosis in very young patients. During follow up, three patients requested the use of their cryopreserved ovarian tissue. All had undergone ovarian tissue transplantation, one for puberty induction and the two others for restoring fertility. So far, no pregnancies have been achieved. Eighty-four patients who had OTC died. Conclusions Ovarian tissue cryopreservation is the only available technique for preserving fertility of girls. To our knowledge this is the largest series of girls and adolescents younger than 15 years so far reported on procedures of OTC before highly gonadotoxic treatment in a single center.

International audience; 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.

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.

We have studied outcome of double-face preputial island flap (DFPIF) technique in severe types of hypospadias: penoscrotal, scrotal and perineal. 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. 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. 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.

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.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).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.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 remains controversial and is not included in most pediatric urology centers because of technical difficulties and lack of long-term results.To critically analyze our 10-yr experience with the retroperitoneal approach (RA), with a particular interest on the impact of the learning curve in a teaching center.Patients who underwent pyeloplasty between 1999 and 2010 at our institution were reviewed (n=390). The diagnosis of ureteropelvic junction obstruction was confirmed by ultrasound and technetium Tc 99m mercaptoacetyltriglycine-3 renal scan or magnetic resonance imaging; the same criteria were used to evaluate the outcome. The lateral RA was selected in children1 yr of age without abnormal migration or fusion of the kidney (n=104).Dismembered pyeloplasty and anastomosis were performed using running monofilament 5-0 or 6-0 absorbable suture. All were drained by double-J stent except 20 cases drained by external transanastomotic stent.We assessed intra- and postoperative morbidity and analyzed the teaching of technique and learning curve. Data are expressed as medians and interquartile range (25th, 75th percentiles) for quantitative variables.Median age was 6.2 yr (2.2-10.3). Thirty-three patients had crossing vessels. Median operative time was 185 min (160-235). Median hospital stay was 2 d (1-2). Redo pyeloplasty was needed in only two children (2%). Median follow-up was 2.1 yr (1.4-4.1). Operative time was3 h after 35 cases. After 30 cases performed by the same surgeon, standardization of the technique was feasible, which helped in the teaching process because 50% of the final 30 cases were done by trainees.Retroperitoneal dismembered laparoscopic pyeloplasty is a safe, reliable, and efficient procedure with an excellent outcome in selected children according to their indications and age, and the experience of the surgical team. Even if the transmission to trainees is successful, it is still a long learning process and remains a challenging task for a teaching center.

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).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.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).Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR. © 2016 John WileySons, Ltd.

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.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.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.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.

Hypospadias is a malformation of the penis which can be benign or severe. Surgical treatment is carried out from the age of 9 months, in order to straighten the penis, reconstruct the urethra and the glans and to secure skin cover.These children need to be followed-up until puberty, in order to prevent complications and to put in place any necessary psychosocial support.

Objective Ultrasound prenatal evaluation of pelvic cystic mass can be challenging. After having ruled out a cloaca anterior to a large hydrocolpos, it is important to differentiate between combined urogenital anomalies such as urogenital sinus and isolated genital anomalies. Patients and methods We reviewed the charts of 13 women referred for a third trimester pelvic MRI for cystic pelvic mass discovered in second trimester ultrasound. We evaluated MRI compared with postnatal surgical findings in order to determine clues for improving prenatal diagnoses. Results MRI excluded the diagnosis of cloacal malformation in nine cases with no false negative. Once a cloaca is ruled out, a different signal between the bladder and the hydrocolpos on T2 sequences is in favor of an isolated genital obstruction. In contrast, in case of urogenital sinus, the vagina is filled with a mixture of genital secretions and urine, which gives it an MRI signal similar to the bladder on T2 sequences. Conclusion Third trimester fetal MRI is an essential exam for characterization of pelvic cystic mass diagnosed by ultrasound. This exam appears valuable for invalidating the diagnosis of cloacal malformation and for differentiating between isolated genital obstruction and urogenital sinus.

The absence of a testis occurs for various reasons in children, but testicular prosthesis implantation in children is uncommon. The optimal time for prosthesis placement is still unclear, and its complication rate has been poorly studied in children.The aim of this study was to determine the risk factors of complications in cases of testicular prosthesis implantation in children.A monocentric, retrospective review was performed of children implanted with a testicular prosthesis between 2008 and 2014. All implantations were performed through an inguinal incision with a standardized procedure. Children were divided into two groups depending on the interval after orchiectomy: (A) early implantation (delay between surgeries1 year); and (B) delayed surgeries (delay ≥1 year). Statistical analysis was performed with Student and Fisher tests.Twenty-six patients (A, 15; B, 11) had a total of 38 testicular prostheses placements. Mean follow-up was 36.2 months. First surgery was performed at the mean age of 11.8 years (range 0-17.9) (A, 14.1; B, 8.1; P = 0.01) and testicular prosthesis implantation at the mean age of 14.7 years (range 9-18) (A, 14.3; B, 14.6) with a mean delay of 36.1 months (A, 1.3; B, 80.3). Indications were mainly spermatic cord torsion (27%), bilateral anorchia (27%), and testicular atrophy after cryptorchidism surgery (19.2%). Complications (10.5%) included two cases of extrusion, one infection and one migration. Patient 1 had a history of acute lymphoblastic leukemia with testicle relapse 2 years after induction therapy. High-dose chemotherapy, total body irradiation and bilateral orchiectomies were performed, and bilateral prostheses were implanted 12 years after the end of chemotherapy. Complications happened 85 days after surgery. Patient 2 was followed-up for a proximal hypospadias. The tunica vaginalis flap, which was used during a redo urethroplasty, lead to testicular atrophy. Thirteen years after the last penile surgery, a testicular prosthesis was placed through an inguinal incision, and extrusion occurred 203 days after surgery. Bacterial cultures of the prostheses were sterile and histological review showed no sign of granuloma or graft rejection. The complication rate was significantly higher if the delay between the two surgeries exceeded 1 year (P = 0.01). Indications of orchiectomy, prior scrotal incision, and prosthesis size were not risk factors.Testicular prosthesis implantation was relatively safe in a pediatric cohort. The complication rate was significantly higher if the delay between the orchiectomy and the prosthetic placement exceeded 1 year. These results suggest that reducing the delay between orchiectomy and prosthesis implantation may lead to fewer complications.

Ano-rectal trauma is common in motor vehicle accidents involving children. Inadequate initial assessment of the extent of lesions may be life threatening. We describe two cases where children were struck by buses that subsequently rolled over them in the prone position, resulting in ano-rectal and gluteal muscle wrenching. The first patient was inadequately assessed. Initial management did not include a diverting stoma, leading to life-threatening necrosis and septic shock. The second benefitted from our previous experience and recovery was uneventful. The distinctive mechanism of trauma in true gluteal muscle and anal canal wrenching is discussed. Gluteal muscle, anal canal and rectal wrenching as a result of rolling force from a motor vehicle is a very serious condition requiring immediate intestinal diversion with a stoma. Immediate repair may be attempted at the same time as stoma creation if the patient is stable. Broad-spectrum antibiotics and close wound monitoring are necessary to avoid muscle necrosis and serious complications.

We assessed whether an external ureteropelvic stent was a feasible and safe alternative to Double-J® stent after laparoscopic pyeloplasty in children, thus avoiding a second general anesthesia.Our study included 22 concurrent age matched children who underwent retroperitoneal laparoscopic pyeloplasty between 2000 and 2008. In group 1 an external ureteropelvic stent was inserted through the renal pelvis, then clamped on postoperative day 2. In group 2 a Double-J stent was antegradely inserted. The ureteropelvic stent was removed at the outpatient clinic on day 10, while the Double-J stent was removed under general anesthesia at 1 month. We retrospectively compared operative time, hospital stay, intraoperative and postoperative complications, and followup.Mean±SD age at surgery was 31±9 months in group 1 and 37±12 months in group 2. Mean±SD operative time was slightly shorter in group 1 (190±40 minutes) than in group 2 (205±23 minutes). No intraoperative complications were encountered during placement of stent. Mean±SD hospital stay was 2.18±1.20 days in group 1 and 2.45±0.54 days in group 2. No postoperative complications were reported in group 1. The Double-J stent was noted in the posterior urethra in 1 patient in group 2, requiring cystoscopic repositioning. No patient had urinary tract infection. Median followup was 34 months (range 22 to 56) in group 1 and 35 months (16 to 72) in group 2.The feasibility of external ureteropelvic stenting after laparoscopic pyeloplasty will pave the way to minimizing the use of Double-J stenting and eliminating a second general anesthesia for catheter removal.

Summary Objectives The Mitrofanoff principle is an accepted continent urinary diversion. We studied the feasibility and the possible benefits of using a laparoscopic approach in children with significant bladder dysfunction associated with difficulty doing efficient urethral catheterization. Patients and methods A fully laparoscopic Mitrofanoff continent cystostomy was attempted in 15 children with a median age of 9 years (IQR 6), between 2003 and 2013. Before the Mitrofanoff procedure was considered, urodynamic evaluation was done for each patient, to study bladder compliance, detrusor activity, and bladder capacity. The procedure was performed using a transperitoneal four-port approach. A 30-degree down camera angle was optimal for viewing the appendix and the posterior wall of the bladder. The operative steps of the open procedure were replicated laparoscopically. The proximal end of the appendix was spatulated and anastomosed to the posterior wall of the bladder, providing an antireflux mechanism by an extramucosal tunnel. The distal end of the appendix was brought out as the cutaneous umbilical stoma. Some modifications were done because of the high rate of conversion due to early opening of the mucosa (harmonic hook) or difficult anastomosis: (a) use of 5-mm trocars to change the laparoscope position from the left to right subcostal area to better visualize the anastomosis, (b) the anastomosis was suspended at its two ends during suturing; a trans-abdominal traction suture of the bladder was inserted for better exposure of the anastomosis (hitch stitch) and to stabilize the anastomotic line during suturing, (c) use of a monopolar hook to cut the detrusor muscle fibers, to avoid incidental opening of the mucosa, and (d) the window between the appendix and the peritoneum was closed to avoid internal hernia. Results The procedure was totally completed by laparoscopy in 12 cases. Three were converted to an open procedure due to tearing of bladder mucosa (n = 2) or appendix ischemia (n = 1). Median operative time for fully laparoscopic Mitrofanoff was 255 min (IQR 52). Median follow-up was 18 months (IQR 35). No patient required stomal revision. Seven patients were continent, five experienced urinary leakage from urethra n = 1 and/or stoma n = 5. Three patients with stomal urinary leakage were successfully managed by Deflux (dextranomer-based implants) injection in the catheterizable channel. Two patients required an open revision of the appendicovesical anastomosis. The patient with both stomal and urethral urinary leakage also required the implantation of an artificial urinary sphincter 1.5 years after Mitrofanoff. One patient had bladder augmentation. Conclusion Although our results of laparoscopic Mitrofanoff procedure in children are unsatisfying in cases of high-pressure bladders in terms of incontinent stoma, we still believe that it is justified to develop this challenging technique with more refinement and improvement, to provide a minimal invasive procedure that may postpone or even avoid bladder augmentation in pediatric age.