Laparoscopic Treatment of Wilms' Tumor: Criteria of SIOP-UMBRELLA Protocol may be Updated.

INTRODUCTION: Total nephrectomies for the treatment of Wilms' tumor (WT) are more and more performed by laparoscopy, although indications for this approach following the UMBRELLA guidelines are currently very restrictive. The purpose of this study was to assess the compliance to the criteria of the UMBRELLA protocol for minimally invasive approach of WT. METHODS: This retrospective multicenter study included children operated on by laparoscopic total nephrectomy for suspected WT before 2020. Imaging was reviewed centrally. RESULTS: Fifty-six patients (50 WT and 6 nephrogenic rests) were operated on at a median age of 3.3 ± 2.6 years. Thirteen (23%) patients had metastasis at diagnosis. The mean operative time was 213 ± 84 min. There were eight (14.3%) conversions and five peroperative complications. A local stage III was confirmed in seven (12.5%) cases, including two for tumor rupture. Only one (1.8%) of the procedures followed the SIOP-UMBRELLA indications for laparoscopy. The criterion "ring of normal parenchyma" was met only once. Conservative surgery seemed possible in ten (17.9%) cases. The extension of the tumor beyond the ipsilateral edge of the vertebra after chemotherapy and a volume over 200 mL were associated with an increased risk of conversion (p = 0.0004 and p = 0.001 respectively). After a mean follow-up of 5.2 ± 4.0 years, although there was no local recurrence, one death occurred due to metastatic progression at 15 months postoperatively. CONCLUSIONS: The laparoscopic approach of WT beyond the UMBRELLA recommendations was feasible with low risk of local recurrence. Its indications may be updated and validated.

Complications after Heller myotomy in children: a national multicenter study on the impact of prior endoscopic dilatation and identification of risk factors.

BACKGROUND: Although esophageal achalasia has been historically treated by Heller myotomy, endoscopic esophageal dilatations are nowadays often the first-line treatment in children. The aim was to assess whether performing an endoscopic dilatation before a Heller myotomy is associated with higher risks of esophageal perforation in children. METHODS: A retrospective multicentric study was performed, including children that underwent a Heller myotomy (2000-2022, 10 centers). Two groups were compared based on the history of previous dilatation before myotomy. Outcomes esophageal perforation (intra-operative or secondary) and post-operative complications requiring surgery (Clavien-Dindo III). Statistics Comparisons using contingency tables or Kruskal-Wallis when appropriate. Statistical significance: p-value < 0.05. RESULTS: A Heller myotomy was performed in 77 children (median age: 11.8 years), with prior endoscopic dilatation in 53% (n = 41). A laparoscopic approach was used in 90%, with associated fundoplication in 95%. Esophageal perforation occurred in 19% of children (n = 15), including 12 patients with intra-operative mucosal tear and 3 with post-operative complications related to an unnoticed esophageal perforation. Previous endoscopic dilatation did not increase the risk of esophageal perforation (22% vs 17%, OR: 1.4, 95%CI: 0.43-4.69). Post-operative complications occurred in 8% (n = 6), with similar rates regardless of prior endoscopic dilatation. Intra-operative mucosal tear was the only risk factor for post-operative complications, increasing the risk of complications from 5 to 25% (OR: 6.89, 95%CI: 1.38-31.87). CONCLUSIONS: Prior endoscopic dilatations did not increase the risk of esophageal perforation or postoperative complications of Heller myotomy in this cohort of children with achalasia. Mucosal tear was identified as a risk factor for post-operative complications.

Thoracoscopy for Pediatric Thoracic Neurogenic Tumors-A European Multi-Center Study.

OBJECTIVES: To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. METHODS: We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. RESULTS: We identified 119 patients with a median age of 4 years old (3 months-17 years). The diameter was 5.7 cm (1.1-15). INRG stage was L1 n = 46, L2 n = 56, MS n = 5, M n = 12. Of 69 patients with image-defined risk factors (IDRF), 29 had only (T9-T12) locations. Twenty-three out of 34 patients with preoperative chemotherapy had an 18 mm (7-24) decrease in diameter. Seven out of 31 patients lost their IDRF after chemotherapy. Fourteen had a conversion to thoracotomy. The length of the hospital stay was 4 days (0-46). The main complications included chylothorax (n = 7) and pneumothorax (n = 5). Long-term complications included Horner's syndrome (n = 5), back pain, and scoliosis (n = 5). Pathology was 53 neuroblastomas, 36 ganglioneuromas, and 30 ganglioneuroblastomas. Fourteen had a postoperative residue. With a median follow-up of 21 months (4-195), 9 patients had a recurrence, and 5 died of disease. Relapses were associated with tumor biology, histology, and the need for chemotherapy (p = 0.034, <0.001, and 0.015, respectively). Residues were associated with preoperative IDRF (excluding T9-T12 only) and the need for preoperative chemotherapy (p = 0.04 and 0.020). CONCLUSION: Our results show that thoracoscopy is safe, with good outcomes for thoracic neurogenic tumors in selected cases. Surgical outcomes are related to the IDRFs, whereas oncologic outcomes are related to tumor histology and biology.

Pectus Arcuatum: A Pectus Unlike Any Other.

BACKGROUND: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers. METHODS: Multicenter retrospective study of young patients' files diagnosed with pectus arcuatum. RESULTS: The clinical diagnosis of pectus arcuatum was made in 34 patients with a mean age at diagnosis of 10.3 years (4-23 years). A chest profile X-ray or a CT scan was performed in 16 patients (47%) and confirmed the diagnosis of PA by the presence of a sternal fusion. It was complete in 12 patients. A malformation was associated in 35% of cases (Noonan syndrome 33%, scoliosis 25% or cardiopathy 16%). 11 patients (32%) had a family history of skeletal malformation. Orthopedic treatment was initiated in 3 patients without any success. 11 patients underwent surgical correction, which was completed in 7 of them. CONCLUSION: The diagnosis of pectus arcuatum is based on clinical experience and if necessary, on a profile chest X-ray showing the fusion of the sternal pieces. It implies the search for any associated malformations (musculoskeletal, cardiac, syndromic). Bracing treatment is useless for pectus arcuatum. Corrective surgery, based on a sternotomy associated with a partial chondro-costal resection, can be performed at the end of growth. LEVEL OF EVIDENCE: IV.

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

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

Pediatric endoscopic subcutaneous mastectomy (pesma) with liposuction in adolescents with gynecomastia.

BACKGROUND: Surgical techniques for treatment of gynecomastia are increasingly less invasive. We described technical standardization of pediatric endoscopic subcutaneous mastectomy (PESMA) with liposuction. METHODS: All adolescents with primary gynecomastia, operated using PESMA with liposuction over the period June 2014-July 2021, were included. The video recording of procedures was analyzed to standardize the operative technique. After patient installation, 3 trocars were placed on the mid-axillary line. The technique included 5 steps: (1) subcutaneous injection of lipolysis solution and liposuction; (2) creation of working space using an inflated balloon; (3) gland dissection using 5-mm sealing device; (4) specimen extraction through the largest trocar orifice; and (5) placement of suction drainage tube. RESULTS: Twenty-four male adolescents, operated for Simon's grade 2B and 3 gynecomastia using PESMA with liposuction over the study period, were included. Mean patient age was 16 years (range 15-18). Gynecomastia was bilateral in 19/24 (79.2%) and unilateral in 5/24 (20.8%). One (4.1%) conversion to open was reported. The mean operative time was 87 min (range 98-160) for unilateral and 160 min (range 140-250) for bilateral procedure. The mean length of stay was 2.2 days (range 1-4). Patients wore a thoracic belt for 15 up to 30 days postoperatively. Post-operative complications occurred in 5/24 (20.8%): 2- or 3 mm second-degree burns in 4 (16.7%) and subcutaneous seroma in 1 (4.1%). All complications were Clavien 2 grade and did not require further treatment. Aesthetic outcomes were very good in 21/24 (87.5%). Three (12.5%) boys had persistent minimal breast asymmetry but did never perceive it negatively. CONCLUSION: PESMA combined with liposuction was feasible and safe for surgical treatment of gynecomastia in this selected cohort of patients. Although challenging, this procedure provided good aesthetic results, with no scars on the anterior thoracic wall. Standardization of the operative technique was a key point for successful outcome.

Current trends in 2021 in surgical management of vesico-ureteral reflux in pediatric patients: results of a multicenter international survey on 552 patients.

BACKGROUND: The selection of best surgical approach for treatment of vesico-ureteral reflux (VUR) in the pediatric population remains debated. This study aimed to report the results of a multicenter survey about the current trends in surgical management of pediatric VUR. METHODS: An online questionnaire-based survey was performed, with participation of six international institutions. All children (age <18 years) affected by primary III-V grade VUR, who were operated over the last 5 years, were included. The incidence of each VUR intervention, patients' demographics and outcomes were analyzed. RESULTS: A total of 552 patients (331 girls), with a median age of 4.6 years (range 0.5-17.6), were included. Deflux® injection (STING) was the most common technique (70.1%). The multicenter success rate after single treatment was significantly lower after STING (74.4%) compared with the other treatments (P=0.001). Persistent VUR rate was significantly higher after STING (10.8%) compared with the other treatments (P=0.03). Choosing endoscopy over surgery mean reducing Clavien Dindo grade 2 complications by 5% but increasing redo procedure rate by 7%. STING was the most cost-effective option. CONCLUSIONS: This survey confirmed that the choice of the technique remains based on surgeon's preference. Deflux® injection currently represents the first line therapy for primary VUR in children and the role of surgical ureteral reimplantation is significantly reduced. STING reported acceptable success rate, less postoperative complications and lower costs but higher failure and re-operation rates and related costs compared with the other surgical approaches. The adoption of laparoscopy and robotics over open reimplantation remains still limited.

Early detection of ureteropelvic junction obstruction in neonates with prenatal diagnosis of renal pelvis dilatation using 1H NMR urinary metabolomics.

Renal pelvis dilatation (RPD) is diagnosed in utero on prenatal ultrasonography (US) and can resolve spontaneously. However, isolated RPD can also reflect ureteropelvic junction obstruction (UPJO), which requires surgical treatment to prevent progressive renal deterioration. The diagnosis of UPJO can only be confirmed after birth with repeat US and renal isotope studies. 1H Nuclear Magnetic Resonance spectroscopy (NMR) was performed on urine of newborns with prenatally diagnosed unilateral RPD and healthy controls to identify specific urinary biomarkers for UPJO. The original combination of EigenMS normalization and sparse partial-least-squares discriminant analysis improved selectivity and sensitivity. In total, 140 urine samples from newborns were processed and 100 metabolites were identified. Correlation network identified discriminant metabolites in lower concentrations in UPJO patients. Two main metabolic pathways appeared to be impaired in patients with UPJO i.e. amino acid and betaine metabolism. In this prospective study, metabolic profiling of urine samples by NMR clearly distinguishes patients who required surgery for UPJO from patients with transient dilatations and controls. This study will pave the way for the use of metabolomics for the diagnosis of prenatal hydronephrosis in clinical routine.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion. RESULTS: Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03). CONCLUSIONS: Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children. LEVEL OF EVIDENCE: II - Prospective Study.

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.

Esophageal Atresia and Respiratory Morbidity.

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.

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.

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.

Outcomes of laparoscopic and open surgical treatment of intestinal malrotation in children.

BACKGROUND: The safety of the laparoscopic treatment of intestinal malrotation remains controversial. This study compared the outcomes of laparoscopic and open surgical treatment of intestinal malrotation. METHODS: A multicentric retrospective study included pediatric cases of intestinal malrotation operated on between 2005 and 2016. RESULTS: This study included 227 children with a median age of 17 days (0-17.2 years), including 161 with a midgut volvulus. Forty-six(20.3%) procedures were started by laparoscopy and 181(79.7%) by laparotomy. Laparoscopy was more frequent for elective surgery (45.9%) than for emergency procedures (10.8%, p < 0.001). Conversions were significantly more frequent during emergency procedures (66.7% vs 17.9%)(p = 0.001). Considering only 61 elective surgeries, the mean hospital stay was significantly shorter after laparoscopy (5.3 days +/-5.2 vs 10.1 days +/-13, p = 0.01), the overall complication rate was comparable (15.8% vs 21.7%, p = 0.7) but post-operative volvulus was significantly more frequent after laparoscopy (13% vs 0%, p = 0.04). Outcomes of the two approaches were not significantly different after 166 emergency procedures. CONCLUSION: Laparoscopy can be performed by experienced team for the treatment of selected cases of intestinal malrotation. Conversion to open surgery should be done with a low threshold, as the rate of volvulus recurrence is concerning. LEVEL OF EVIDENCE: Level III.

Multicystic and diffuse malignant peritoneal mesothelioma in children.

BACKGROUND: Malignant and multicystic peritoneal mesotheliomas are extremely rare tumors in children, developing from mesothelial cells. No specific guidelines are available at this age. METHODS: We performed a retrospective analysis of all identified children (< 18-year-old) treated in France from 1987 to 2017 for a diffuse malignant peritoneal mesothelioma (DMPM) or a multicystic peritoneal mesothelioma (MCPM). RESULTS: Fourteen patients (5 males and nine females), aged 2.2 to 17.5 years, were included. The most frequent presenting symptoms were abdominal pain, ascitis, and alteration in the general condition. Eight patients had epithelioid mesothelioma, three had biphasic mesothelioma, and three had MCPM. Eight patients with DMPM diagnosis received cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). Among them, six patients had neoadjuvant systemic chemotherapy, one patient, post-operative chemotherapy, and one patient CRS and HIPEC only. Three patients received only systemic chemotherapy. All patients with MCPM had only surgery. After a median follow-up of seven years (2-15), six patients (6/11; one death) with DMPM and two patients (two/three) with MCPM had a local and distant recurrences. CONCLUSION: Peritoneal mesothelioma in children is a rare condition with difficult diagnosis and high risk of recurrence. Worldwide interdisciplinary collaboration and networking are mandatory to help diagnosis and provide harmonious treatment guidelines.

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

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

Utility of radiation-free imaging for initial evaluation of pectus excavatum.

OBJECTIVES: The OrtenBodyOne scanner is a radiation-free, 3-dimensional imaging system recently developed for evaluation of the severity of pectus excavatum (PE). The goal of this study was to evaluate the utility of this new imaging system compared with that of computed tomography (CT) for the evaluation of the severity of PE. METHODS: Patients treated for PE from April 2015 to January 2017 with available CT and OrtenBodyOne data were included. Correlations between indexes calculated from CT and from OrtenBodyOne were determined by applying the non-parametric Spearman correlation procedure with a Bonferroni correction to adjust for multiple comparisons. RESULTS: Forty men (90.9%) and 4 women (9.1%), 20 with symmetrical (45.5%) and 24 with asymmetrical PE (54.5%), were included. The median age was 16.1 years (range 4.3-63.5 years). The following measures and indexes acquired using OrtenBodyOne and CT were significantly correlated: pectus depth (r = 0.84; P = 0.002), anthropometric index (r = 0.81; P = 0.002) and asymmetry index (r = 0.67; P = 0.002). The correlation between the CT Haller index and the external Haller index was only significant for symmetrical PE (r = 0.57; P = 0.008). CONCLUSIONS: The OrtenBodyOne imaging system can be used to evaluate the severity of symmetrical PE using the external Haller index. Asymmetry and anthropometric indexes are more reliable for the evaluation of asymmetrical PE. Measures can be repeated throughout treatment while avoiding unnecessary irradiation.

Treatment of Gynecomastia by Endoscopic Subcutaneous Mastectomy in Adolescents.

Background: Morbidity of traditional open surgery for the treatment of gynecomastia includes asymmetry, retraction of the nipple, and poor scarring. Other approaches were described to improve the results. Endoscopic subcutaneous mastectomy (ESCM) was reported in adult series, including a few adolescents. This technique was considered as safe with good aesthetic results. The aim of this study was to evaluate the results of ESCM in an adolescent series. Patient and Methods: We treated 19 adolescents with Simon's grade IIB and III gynecomastia between June 2014 and July 2018. They could choose open surgery through the nipple or endoscopic surgery by axillary approach. To perform ESCM, three trocars were placed on the midaxillary line to dissect the gland. The resection was performed leaving 1 cm thickness of gland behind the nipple. The gland was extracted after morcellation through the 10 mm trocar. A drainage tube was placed in the cavity. The patients wore a thoracic belt smoothly compressing the operative areas for 15 days. The result was considered as good when there was no remaining gland, good symmetry, and no nipple retraction or nipple lateral displacement. Results: During the study period, 12 adolescents were treated by ESCM and 7 preferred open surgery. Among the 12 ESCM patients, 7 had bilateral and 5 unilateral gynecomastia. A subcutaneous injection of serum was done in the last 6 patients to facilitate the creation of the work space. A 2- or 3-mm second-degree burn occurred in 4 cases, 2 on the nipple and 2 just above the nipple, with a 2 mm remaining scar above the nipple in 1 case. The postoperative course was uneventful in 11 adolescents. One subcutaneous seroma expanded at 15 days postoperative and resolved after 3 weeks of prolonged compression by thoracic belt. The adolescents had 11 good results and 1 persistent asymmetry; 2 other asymmetries had a spontaneous improvement after 1 or 2 years. Conclusion: ESCM is feasible and safe for the treatment of gynecomastia in adolescents. This technique is challenging but permits to reach good aesthetic results and avoids scars on the anterior wall of the thorax.

Postoperative bladder dysfunction and outcomes after minimally invasive extravesical ureteric reimplantation in children using a laparoscopic and a robot-assisted approach: results of a multicentre international survey.

OBJECTIVES: To assess and compare postoperative bladder dysfunction rates and outcomes after laparoscopic and robot-assisted extravesical ureteric reimplantation in children and to identify risk factors associated with bladder dysfunction. PATIENTS AND METHODS: A total of 151 children underwent minimally invasive extravesical ureteric reimplantation in five international centres of paediatric urology over a 5-year period (January 2013-January 2018). The children were divided in two groups according to surgical approach: group 1 underwent laporoscopic reimplantation and included 116 children (92 girls and 24 boys with a median age of 4.5 years), while group 2 underwent robot-assisted reimplantation and included 35 children (29 girls and six boys with a median age of 7.5 years). The two groups were compared with regard to: procedure length; success rate; postoperative complication rate; and postoperative bladder dysfunction rate (acute urinary retention [AUR] and voiding dysfunction). Univariate and multivariate logistic regression analyses were performed to assess predictors of postoperative bladder dysfunction. Factors assessed included age, gender, laterality, duration of procedure, pre-existing bladder and bowel dysfunction (BBD) and pain control. RESULTS: The mean operating time was significantly longer in group 2 compared with group 1, for both unilateral (159.5 vs 109.5 min) and bilateral procedures (202 vs 132 min; P = 0.001). The success rate was significantly higher in group 2 than in group 1 (100% vs 95.6%; P = 0.001). The overall postoperative bladder dysfunction rate was 8.6% and no significant difference was found between group 1 (6.9%) and group 2 (14.3%; P = 0.17). All AUR cases were managed with short-term bladder catheterization except for two cases (1.3%) in group 1 that required short-term suprapubic catheterization. Univariate and multivariate analyses showed that bilateral pathology, pre-existing BBD and duration of procedure were predictors of postoperative bladder dysfunction (P = 0.001). CONCLUSION: Our results confirmed that short-term bladder dysfunction is a possible complication of extravesical ureteric reimplantation, with no significant difference between the laparoscopic and robot-assisted approaches. Bladder dysfunction occurred more often after bilateral repairs, but required suprapubic catheterization in only 1.3% of cases. Bilaterality, pre-existing BBD and duration of surgery were confirmed on univariate and multivariate analyses as predictors of postoperative bladder dysfunction in this series.