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.

Early laparoscopic-assisted surgery is associated with decreased post-operative inflammation and intestinal strictures in infants with necrotizing enterocolitis.

INTRODUCTION: In 2015, a protocol including early laparoscopy-assisted surgery in the treatment of necrotizing enterocolitis (NEC) was implemented at our institution. Carbon dioxide insufflation during laparoscopy may have an anti-inflammatory effect. We aimed to compare post-operative outcome after early laparoscopy-assisted surgery and classical laparotomy for NEC. MATERIAL AND METHODS: Charts of premature infants undergoing surgery for NEC (2012-2021) were reviewed. Cases operated by early laparoscopy-assisted surgery (2015-2021) were compared to infants operated for NEC between 2012 and 2015 (laparotomy-NEC). Outcomes were post-operative CRP, need for reintervention, mortality, and the occurrence of post-NEC intestinal strictures. CRP was measured on the day of surgery (POD-0), 2 days (POD-2), and 7 days after surgery (POD-7). Data were compared using contingency tables for categorical variables and Student t-test or Mann-Whitney test for continuous variables. RESULTS: Infants with NEC operated by early laparoscopy (n = 48) and laparotomy (n = 29) were similar in terms of perforation (60% vs 58%, p = 0.99) and POD-0 CRP (139 vs 124 mg/L, p = 0.94). Delay between first signs of NEC and surgery was shorter in the laparoscopy group (3 vs 6 days, p = 0.004). Early laparoscopy was associated with a lower CRP on POD-2 (108 vs 170, p = 0.005) and POD-7 (37 vs 68, p = 0.002), as well as a lower rate of post-operative intestinal stricture (34% vs 61%, p = 0.04). CONCLUSIONS: In addition to being safe and feasible in premature infants, early laparoscopic-assisted surgery was associated with decreased NEC-related post-operative inflammation and strictures. A prospective, randomized study is needed in order to evaluate short and long-term effects of laparoscopy in infants with NEC. LEVEL OF EVIDENCE: Level III.

Lessons from a large nationwide cohort of 350 children with ovarian mature teratoma: A study in favor of ovarian-sparing surgery.

BACKGROUND: Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%-20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian-sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed. DESIGN: This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved. RESULTS: Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not. CONCLUSION: This study is a plea in favor of OSS as the first-choice treatment of OMT when possible. Close follow-up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors.

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.

A multicenter review of undescended testis torsion: A plea for early management.

INTRODUCTION: Torsion of an undescended testis (UT) is a surgical emergency, difficult to diagnose, whose prognosis depends on a quick management. AIM OF THE STUDY: To evaluate the management and outcome of these patients. STUDY DESIGN: We retrospectively analyzed all cases of UT torsion operated in nine French hospitals between 1997 and 2017. We divided patients in two groups: patients referred less than 6 h after the onset of symptoms (group A) or more than 6 h (group B). MAIN RESULTS: We collected 60 cases (17 in group A and 43 in group B). Median age was 2.2 years [IQR = 0.7-7.8] (2.3 y in group A and 2 y in group B, p = 0.76). Eleven patients (10 in group B) had neurological disorders (p = 0.15). The main reason for absence of UT treatment was the absence of surgical consultation in a normal delay (n = 44, 73%). Symptoms were pain (n = 58, 97%), inguinal mass (n = 55, 92%) and vomiting (n = 16, 27%). An inguinal mass with no palpable testis in the ipsilateral hemiscrotum was seen in 55 patients (92%). An ultrasound scan performed in 27 patients led to the diagnosis in 16 patients (59%). At surgery, an orchiectomy was performed in 4 patients (23%) of group A and 24 patients (56%) of group B (p = 0.04). After a median follow-up of 11 months [IQR = 4-23], 11 patients of group A (65%) and 7 patients of group B (16%) had a clinically normal testis (p = 0.03). The salvage rate among patients with conservative treatment was 85% for group A and 37% for group B (p = 0.01). DISCUSSION: Our study reveals that although UT torsion is an emergency, 72% of patients are referred more than 6 h after the onset of symptoms. We mostly found classic clinical presentation of UT torsion: a painful inguinal mass with an empty ipsilateral scrotum. Ultrasound was performed in half cases, and even if the result was not significant, it still seemed to be associated with a higher rate of orchiectomy especially in group B because of the delay in care. However, when ultrasound was realized early, it led to diagnosis in all cases. This dilemma poses the problem of the role of imaging in diagnostic management. CONCLUSIONS: Early clinical diagnosis in front of a painful inguinal mass with an empty scrotum is essential to improve the salvage rate of testis in UT torsion. Early management of UT should have avoided 68% of testis loss.

The basics of transition in congenital lifelong urology.

PURPOSE: Transition in urology is defined by the process that allows an adolescent or a young adult with a congenital or acquired urogenital anomaly to assume increasing responsibility for their own health care and to become the primary decision maker in their care. METHODS: A review of the literature regarding transitional care for lifelong urologic congenital anomalies was performed with the aim of reporting expert opinion when data are non-existent. This review focuses on special considerations for adolescents and young adults with spina bifida, bladder exstrophy, anorectal malformations and differences of sexual development. RESULTS: Urologic goals during the transition from childhood to adulthood continue to include attention to the preservation of renal function and optimization of lower urinary tract function. Additional concerns include care to decrease long-term surgical complications (especially after augmentation cystoplasty), to monitor for malignancy, to prepare for sex activity and fertility, and to help the adult patient in decision making. Transition aims to maximize quality of life and independence by ensuring uninterrupted appropriate care through a multidisciplinary approach which varies by geographical location and healthcare setting. Barriers include patient and family factors as well as provider and system related factors. A dedicated team is an important element of successful transition.

Concomitant gastrostomy tube insertion during laparoscopic Nissen fundoplication for gastro-esophageal reflux disease: analysis of risk factors for fundoplication failure.

BACKGROUND: Laparoscopic Nissen fundoplication (LNF) and gastrostomy tube (GT) placement may be performed concomitantly in children with gastro-esophageal reflux disease (GERD) and failure to thrive. We aimed to evaluate the rate and risk factors for LNF failure in children undergoing concomitant LNF/GT. METHODS: A retrospective multi-institutional cohort study was conducted, reviewing patients that underwent LNF (2005-2014). Data collected included patient demographics, comorbidities, and type of GT (laparoscopy- or endoscopy-assisted). The primary outcome measure was LNF failure. Data was compared using contingency tables or Mann-Whitney tests, when appropriate. An exploratory analysis by Kaplan-Meier survival and Cox proportional hazards analysis was performed to determine predictors of time to LNF failure after LNF/GT. RESULTS: Of 189 children that underwent LNF, 99 (52%) had a concomitant GT (55% laparoscopy-, 45% endoscopy-assisted). LNF failed in 15% after LNF/GT and in 17% after LNF alone (p = 0.84), at a median age of 23 months (IQR 8-41). Using univariate analysis, we found that a younger age at the time of surgery (p = 0.05), prematurity (p = 0.0018), esophageal atresia (p = 0.01), and endoscopy-assisted GT (p = 0.02) were potential predictors of LNF failure after LNF/GT. After multivariate regression analysis, prematurity (p = 0.007) remained significantly associated with LNF failure after LNF/GT. No predictive factors for LNF failure after LNF alone were identified. CONCLUSIONS: Concomitant GT insertion and LNF is a common practice, as half of the children that undergo LNF also received GT insertion. Children born preterm or with esophageal atresia comprise a fragile population at high-risk of LNF failure after LNF/GT. Prospective, multicentric studies are needed to evaluate the best GT technique to use in children undergoing LNF.

Management of lower urinary tract fibroepithelial polyps in children.

INTRODUCTION: Fibroepithelial polyps (FEP) of the lower urinary tract are relatively common in adults but rare in children, with fewer than 250 cases reported in the literature to date. OBJECTIVE: The aim of this study was to address the experience of FEP management in children. STUDY DESIGN: A retrospective multicenter review was undertaken in children with defined FEP of the lower urinary tract managed between 2008 and 2018. The data at 18 pediatric surgery centers were collected. Their demographic, radiological, surgical, and pathological information were reviewed. RESULTS: A total of 33 children (26 boys; 7 girls) were treated for FEP of the lower urinary tract at 13 centers. The most common presentation was urinary outflow as hematuria (41%), acute urinary retention (25%), dysuria (19%), or urinary infections (28%). A prenatal diagnosis was made for three patients with hydronephrosis. Almost all of the children (94%) underwent ultrasound imaging of the urinary tract as the first diagnostic examination, 23 (70%) of them also either had an MRI (15%), cystourethrography (25%), computerized tomography (6%), or cystoscopy (45%). Two of these children (6%) had a biopsy prior to the surgery. The median preoperative delay was 7.52 (range: 1-48) months. Most of the patients were treated endoscopically, although four (12.1%) had open surgery and two (6.1%) had an additional incision for specimen extraction. The median hospital stay was 1.5 (range: 1-10) days. There were no recurrences and no complications after a median follow-up of 13 (range: 1-34) months. DISCUSSION: The main limitation of our study is the retrospective design, although it is the largest one for this pathology. CONCLUSION: This series supports sonography as the most suitable diagnosis tool before endoscopy to confirm the diagnosis and to perform the resection for most FEP in children. This report confirms the recognized benign nature in the absence of recurrences. LEVEL OF EVIDENCE: Level V.

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.

Realization of Open Software Chain for 3D Modeling and Printing of Organs in Simulation Centers: Example of Renal Pelvis Reconstruction.

OBJECTIVE: Three-dimensional (3D) printing has many uses in healthcare such as in surgical training. It is becoming an interesting tool finding new pedagogical purposes in medical simulation. In this study, using a process consisting of 3D modeling, a simulator dedicated to pyeloplasty was designed, manufactured, and evaluated by experts. DESIGN: With the aid of open-source software and computer-aided design software, 3D models of a renal parenchyma, a renal pelvis and a ureter were created. This renal apparatus was processed and crafted with additive manufacturing using soft polymer materials. Polyvinyl alcohol material was used to print the components in order to make them dissectible and to evaluate their use in surgical teaching. SETTING AND PARTICIPANTS: Seven expert surgeons evaluated the model by performing a pyeloplasty sequence established in a previous work. An evaluation grid with 8 items related to surgical movement was rated on a 5-point Likert scale to assess how similar working with the model was to actual surgery. RESULTS: Three items were rated with a score greater than or equal to 4 (Needle penetration, Thread-sliding, and Cutting Strength). Suture strength was rated with a score above 3.5 for both renal pelvis and ureter, whereas elasticity was rated below 3. Handling and mobility properties were rated above 3 for the renal pelvis and below 3 for the ureter. The cost of the unit was $0.30 per renal unit. The primary difference identified was a difference in elongation between polyvinyl alcohol material and real biological tissue. CONCLUSIONS: It is feasible to generate and print a low cost upper urinary tract model from patient data imagery using environmentally friendly products that can be used effectively in surgical training. The simulator has been able to reproduce sensations related to surgical movements for a low cost. Hereafter, research into the pedagogical benefits provided to students, and through them, patients, should be performed. 3D printing models can offer new opportunities for healthcare simulation specific to different surgical fields.

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.

The role of a pediatric tertiary care center in avoiding collateral damage for children with acute appendicitis during the COVID-19 outbreak.

PURPOSE: To evaluate the impact of the COVID-19 pandemic-related lockdown on management and outcomes of children with acute appendicitis. METHODS: A retrospective cohort study was conducted, including children treated for acute appendicitis (January 20th-May 11th, 2020). The data regarding the severity of appendicitis and outcome were collected and compared for two time periods, before and after the nationwide lockdown (March 17th, 2020). RESULTS: The number of cases of acute appendicitis increased by 77% during the lockdown (n = 39 vs. n = 69, p = 0.03). During the lockdown, children treated for appendicitis were older (11.1 vs. 8.9 years, p = 0.003), and were more likely to live more than 5 km away from our institution (77% vs. 52%, p = 0.017). Less children had previously consulted a general practitioner (15% vs. 33%, p = 0.028), whereas more children were transferred from other hospitals (52% vs. 31%, p = 0.043). There was no difference in terms of length of hospital stay, rate of postoperative intra-abdominal abscess, ER visits, and readmissions between both periods. Three children (4%) were diagnosed with COVID-19 and appendicitis. CONCLUSIONS: Despite an increase in the number of children with appendicitis managed at our hospital during the COVID-19-related lockdown, management, and outcome remained similar. Although our pediatric center was strongly affected by this pandemic, maintaining our prior practice strategies for acute appendicitis avoided the occurrence of collateral damage for those children.

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.

Adherence with bladder irrigation following augmentation.

BACKGROUND: Patients with bladder augmentation (BA) are routinely counseled to irrigate their bladders daily. However, reports of adherence with this regimen are lacking. OBJECTIVE: To evaluate adherence to a bladder irrigation protocol and identify risk factors associated with adherence among adults with spina bifida (SB) and BA. STUDY DESIGN: Adults with SB after BA followed in a multidisciplinary clinic were identified (2017-2019). All patients or caregivers were taught the importance of and the technique for the bladder irrigation protocol prior to and after BA. Patient demographics (age, gender, ambulatory status, and presence of a caregiver in clinics) and surgical details (type of BA, age at surgery, length of follow-up, presence of a catheterizable channel, position of stoma, bladder neck surgery, presence of Malone antegrade colonic enema or ventriculo-peritoneal shunt, and number of stone surgeries) were obtained from the medical record. Patients reported other variables in a standardized clinic questionnaire. Answers were confirmed by health care providers. The variables included who performs clean intermittent catheterization (CIC), size of catheter, frequency of CIC, use of overnight catheterization, difficulties with CIC, number of UTIs, and continence per urethra and per catheterizable channel. Adherence to bladder irrigation was also assessed in the questionnaire. ?Strict adherence' was defined as bladder irrigation performed ≥6 times/week with ≥120 mL of saline. For statistical analysis, a more lenient definition of ?higher adherence' was used: bladder irrigation ≥2 times/week with at least 60 mL. ?Lower adherence' was defined as ≤1 time/week or with less than 60 mL. Factors associated with ?higher adherence' were assessed with non-parametric tests (Bonferroni-corrected p-value: 0.002). RESULTS: Adherence was assessed in 87 eligible patients (60.9% females; mean age of 28.8 ± 8.2 years). No patient (0.0%) reported ?strict adherence', and 62 and 25 patients (71.3% and 28.7%) reported ?higher' and ?lower' adherence' to bladder irrigation, respectively. Nine patients (10.3%) in the ?lower adherence' group did not irrigate at all. No variables were statistically significant on univariate analysis, including previous bladder stone surgery or having a channel (p ≥ 0.01). On exploratory analysis, higher adherence was only associated with self-catheterizations versus those performed by caregivers (76.7% vs 33.3%, p = 0.01). CONCLUSIONS: Adherence to a bladder irrigation protocol in adults with SB and BA is poor. A history of bladder stones requiring surgery and the presence of a catheterizable channel do not appear to affect adherence. It remains unclear why some patients are more likely than others to irrigate their bladders. Future work will focus on methods to improve adherence.

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.