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.

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.

Ovarian tissue cryopreservation for fertility preservation before hematopoietic stem cell transplantation in patients with sickle cell disease: safety, ovarian function follow-up, and results of ovarian tissue transplantation.

PURPOSE: To describe the experience of performing ovarian tissue cryopreservation (OTC) before hematopoietic stem cell transplantation (HSCT), among girls/women with severe sickle cell disease (SCD)(SS or S/ß0-thalassemia) who are, besides the usual surgical risk, at risk of SCD-related complications during the fertility preservation procedure for improving their counseling and management. METHODS: This retrospective study included 75 patients (girls/women) with SCD who have had OTC before myeloablative conditioning regimen (MAC) for HSCT. Characteristics of patients and data on OTC, ovarian status follow-up, and results of ovarian tissue transplantation (OTT) were collected in medical records. RESULTS: At OTC, the median (IQR 25-75; range) age of the patients was 9.6 (6.9-14.1; 3.6-28.3) years, 56/75 were prepubertal, and no SCD or surgery-related complications occurred. The median follow-up post-HSCT was > 9 years. At the last follow-up, among prepubertal patients at HSCT, 26/56 were ≥ 15 years old and presented with a premature ovarian insufficiency (POI), except 2, including the patient who had received an OTT to induce puberty. Eight were 13-15 years old and presented for POI. The remaining 22 patients were under 13. Among the 19 patients who were menarche at HSCT, 2 died 6 months post-HSCT and we do not have ovarian function follow-up for the other 2 patients. All the remaining patients (n = 15) had POI. Five patients had OTT. All had a return of ovarian function. One patient gave birth to a healthy baby. CONCLUSION: OTC is a safe fertility preservation technique and could be offered before MAC independent of the patient's age.

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.

A 10-year experience in testicular tissue cryopreservation for boys under 18 years of age: What can be learned from 350 cases?

BACKGROUND: A growing number of centers worldwide are preserving testicular tissue (TT) of young boys at risk of fertility loss to preserve their fertility. Data in this regard are scarce and experience sharing is essential to the optimization of the process. OBJECTIVES: This report of our 10-year activity of pediatric fertility preservation (FP) has the objective to (1) improve knowledge regarding the feasibility, acceptability, safety, and potential usefulness of the procedure; (2) analyze the impact of chemotherapy on spermatogonia in the cryopreserved TT. MATERIALS AND METHODS: For this retrospective study of data prospectively recorded, we included all boys under 18 years of age referred to the FP consultation of our academic network between October 2009 and December 2019. Characteristics of patients and cryopreservation of testicular tissue (CTT) were extracted from the clinical database. Univariate and multivariate analyses were used to assess factors associated with the risk of absence of spermatogonia in the TT. RESULTS: Three hundred and sixty-nine patients (7.2 years; 0.5-17.0) were referred to the FP consultation for malignant (70%) or non-malignant (30%) disease, of whom 88% were candidates for CTT, after a previous chemotherapy exposure (78%). The rate of recorded immediate adverse events was 3.5%, with painful episodes dominating. Spermatogonia were detected in the majority of TTs: 91.1% of those exposed to chemotherapy and 92.3% of those not exposed (p = 0.962). In multivariate analysis, the risk of absence of spermatogonia was almost three-fold higher in boys > 10 years of age ([OR] 2.74, 95% CI 1.09-7.26, p = 0.035) and four-fold higher in boys exposed to alkylating agents prior to CTT ([OR] 4.09, 95% CI 1.32-17.94, p = 0.028). DISCUSSION/CONCLUSION: This large series of pediatric FP shows that this procedure is well accepted, feasible, and safe in the short term, strengthening its place in the clinical care pathway of young patients requiring a highly gonadotoxic treatment. Our results demonstrate that CTT post-chemotherapy does not impair the chance to preserve spermatogonia in the TT except when the treatment includes alkylating agents. More data on post-CTT follow-up are still required to ensure the long-term safety and usefulness of the procedure.

Day Surgery in Children Undergoing Retroperitoneal Robot-assisted Laparoscopic Pyeloplasty: Is It Safe and Feasible?

Background: Robot-assisted pyeloplasty is the most frequently performed robotic procedure in children. A retroperitoneal approach limits surgical trauma and avoids peritoneal irritation. This led to the establishment of the criteria for day surgery (DS) and a related clinical care pathway. Objective: To assess the feasibility and safety of DS in children undergoing retroperitoneal robot-assisted laparoscopic pyeloplasty (R-RALP). Design setting and participants: We performed a bicentric prospective study (NCT03274050) over 2 yr involving the two major paediatric urology teaching hospitals in Paris. A clinical pathway and a prospective research protocol were specifically established. Intervention: DS in selected children undergoing R-RALP. Outcome measurements and statistical analysis: The primary outcomes were DS failure, 30-d complications, and readmission rates. The secondary outcomes included preoperative characteristics, perioperative parameters, and surgical outcomes. Quantitative variables were expressed as medians with interquartile ranges. Results and limitations: Thirty-two children fulfilled specific inclusion criteria and were consecutively selected for DS following R-RALP. The median patient age was 7.6 yr (4.1-11.8) and weight 25 kg (14-45). The median console time was 137 min (108-167). There were no intraoperative complications or conversions. Six children were kept under observation overnight and discharged the following day due to persistent pain (n = 3), parental anxiety (n = 2), or a prolonged procedure (n = 1). The median duration of hospital stay of the 26 children in the DS setting was 12.7 h (12.2-13.2). During the 30-d period, there were four emergency room visits (15%) resulting in two patients requiring readmission (8%): one for febrile urinary tract infection (Clavien-Dindo II) and one child with no JJ stent for urinoma (Clavien-Dindo IIIb). Radiological studies confirmed improvement in dilatation for all cases with no recurrence (median follow-up: 15 mo). Conclusions: This prospective case series is the first to demonstrate the feasibility and safety of DS in children undergoing R-RALP, obviating the need for routine inpatient care. Excellent results can be achieved by careful patient selection, a clear clinical pathway, and a dedicated team. Further evaluation is warranted to assess the cost effectiveness. Patient summary: This study shows that day surgery after robotic pyeloplasty is both safe and effective in selected children.

New method for early evaluation of clitoris innervation using clitoro-perineal reflex after feminizing genitoplasty in early childhood: a pilot-study.

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.

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.

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.

Redo Laparoscopic Pyeloplasty in Infants and Children: Feasible and Effective.

Purpose: To determine the feasibility and effectiveness of redo laparoscopic pyeloplasty among patients with failed previous pyeloplasty, specifically examining rates of success and complications. Materials and Methods: We retrospectively reviewed the charts of all patients, who underwent redo laparoscopic pyeloplasty from 2006 to 2017. This included patients who underwent primary pyeloplasty at our institution and those referred for failures. Analysis included demographics, operative time, complications, length of hospital stay, complications, and success. Success was defined as improvement of symptoms and hydronephrosis and/or improvement in drainage demonstrated by diuretic renogram, especially in those with persistent hydronephrosis. Descriptive statistics are presented. Results: We identified 22 patients who underwent redo laparoscopic pyeloplasty. All had Anderson-Hynes technique except two cases in which ureterocalicostomy was performed. Median (IQR) follow-up was 29 (2-120) months, median time between primary pyeloplasty and redo laparoscopic pyeloplasty was 12 (7-49) months. The median operative time was 200 (50-250) min, and median length of hospital stay was 3 (2-10) days. The procedure was feasible in all cases without conversion. During follow-up, all but two patients demonstrated an improvement in the symptoms and the degree of hydronephrosis. Ninety-one percent of patients experienced success and no major complications were noted. Conclusions: Redo laparoscopic pyeloplasty is feasible and effective with a high success rate and low complication rate.

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.

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.

Reflective Practice About Retroperitoneal Laparoscopy in Comparison to Open Surgery for Ureteropelvic Junction Obstruction Repair in Children Less Than 1 Year of Age.

Introduction: The interest in laparoscopy in the treatment of ureteropelvic junction obstruction (UPJO) in children under 12 months of age remains controversial. The aim of this study is to evaluate feasibility and benefits of retroperitoneal laparoscopy (RL) compared to open surgery in this age group. Materials and Methods: Between January 2012 and May 2017, we performed 222 pyeloplasties: 144 by laparoscopy and 78 by open surgery. From 2012, the choice of operative technique was decided according to the laparoscopic experience of the surgeon; two surgeons operated laparoscopically on all children <12 months of age, while others operated using posterior lumbotomy (PL). The RL is standardized and performed by 3 trocars (5, 3, 3). Pre, per and postoperative parameters were analyzed retrospectively. Statistical tests: Pearson, Fisher, Student and Mann-Whitney. Results: During this 5-year period, 24 RL and 53 PL were included with a median follow-up of 27 months (5-63). In the LR group, postoperative drainage was performed by JJ (13 cases) and external stent (11 cases). No conversion has been listed in this group. In each group there was one failure that needed redo pyeloplasty. Duration of hospitalization and intravenous acetaminophen use were significantly lower in the RL group (2.8 vs. 2.3 days, p = 0.02, respectively) while operating time was significantly longer (163 vs. 85.8 min, p = 0.001). The postoperative complication rate was statistically identical in each group (urinary tract infection, wall hematoma, hematuria…). Conclusion: RL is feasible in children under 1 year of age in the hands of well-experienced surgeons with longer operative time but without added morbidity. Subject to the retrospective nature of our study, the RL seems to offer a benefit regarding duration of hospitalization and analgesics consumption.

Ovarian tissue cryopreservation for fertility preservation in 418 girls and adolescents up to 15 years of age facing highly gonadotoxic treatment. Twenty years of experience at a single center.

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.

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 Mitrofanoff procedure in children: critical analysis of difficulties and benefits.

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.