Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.

BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

Dermatological manifestations in Costello syndrome: A prospective multicentric study of 31 HRAS-positive variant patients.

BACKGROUND: Data on dermatological manifestations of Costello syndrome (CS) remain heterogeneous and lack in validated description. OBJECTIVES: To describe the dermatological manifestations of CS; compare them with the literature findings; assess those discriminating CS from other RASopathies, including cardiofaciocutaneous syndrome (CFCS) and the main types of Noonan syndrome (NS); and test for dermatological phenotype-genotype correlations. METHODS: We performed a 10-year, large, prospective, multicentric, collaborative dermatological and genetic study. RESULTS: Thirty-one patients were enrolled. Hair abnormalities were ubiquitous, including wavy or curly hair and excessive eyebrows, respectively in 68% and 56%. Acral excessive skin (AES), papillomas and keratotic papules (PKP), acanthosis nigricans (AN), palmoplantar hyperkeratosis (PPHK) and 'cobblestone' papillomatous papules of the upper lip (CPPUL), were noted respectively in 84%, 61%, 65%, 55% and 32%. Excessive eyebrows, PKP, AN, CCPUL and AES best differentiated CS from CFCS and NS. Multiple melanocytic naevi (>50) may constitute a new marker of attenuated CS associated with intragenic duplication in HRAS. Oral acitretin may be highly beneficial for therapeutic management of PPHK. No significant dermatological phenotype-genotype correlation was determined between patients with and without HRAS c.34G>A (p.G12S). CONCLUSIONS AND RELEVANCE: This validated phenotypic characterization of a large number of patients with CS will allow future researchers to make a positive diagnosis, and to differentiate CS from CFCS and NS.

Assessment of quality of life after laparoscopic GERD surgery in children: a prospective study.

The purpose of this study is to assess quality of life (QoL) after laparoscopic anti-reflux surgery (LARS) in children with gastroesophageal reflux disease (GERD) and to evaluate GERD symptoms and their impact on daily life and school. From June 2016 to June 2019, all children with GERD from 2 to 16 years of age, without neurologic impairment or malformation-related reflux, were prospectively included in a monocentric study. Patients (or their parents according to the age of the child) answered the Pediatric Questionnaire on Gastroesophageal Symptoms and QoL (PGSQ) before surgery and 3 and 12 months after surgery. Variables were compared by paired, bilateral Student t-test. Twenty-eight children (16 boys) were included. The median age at surgery was 77 months (IQR: 59.2-137) with median weight of 22 kg (IQR: 19.8-42.3). All had a laparoscopic Toupet fundoplication. Median duration of follow-up was 14.7 months (IQR: 12.3-22.5). One patient (4%) had a recurrence of GERD symptoms without abnormalities on follow-up examinations. Preoperative total PGSQ score was 1.42 (± 0.7) and decreased significantly 3 months (0.56 ± 0.6; p < 0.001) and 12 months after surgery (0.34 ± 0.4; p < 0.001). PGSQ subscale analysis revealed a significant decrease at 3 and 12 months for GERD symptoms (p < 0.001), impact on daily life (p < 0.001), and impact on school (p = 0.03). CONCLUSION: There was a significant improvement in symptoms and their frequency after LARS in children, as well as an improvement of QoL, in the short and medium term. The impact of GERD should be taken into consideration in the treatment decision, given that surgery clearly improves the QoL. WHAT IS KNOWN: • Laparoscopic anti-reflux surgery (LARS) is an established and effective treatment option in pediatric patients with severe GERD refractory to medical treatment. • Effect of LARS on the quality of life (QoL) has been mainly investigated in the adult population but there is very little data on the effect of LARS on the QoL in pediatric patients. WHAT IS NEW: • Our prospective study was the first to analyze the effect of LARS on QoL in pediatric patients without neurologic impairment using validated questionnaires at two postoperative time points with a significant improvement in postoperative QoL at 3 and 12 months. • Our study emphasizes the importance of evaluating QoL and impact of GERD on all the aspects of daily life and of taking these into consideration in the treatment decision.

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.

Unresectable thoracic neuroblastic tumors: Changes in image-defined risk factors after chemotherapy and impact on surgical management.

PURPOSE: Neuroblastoma management in children is multimodal and depends on multiple factors, including the possibility of complete surgical resection. Image-defined risk factors (IDRFs) are used to assess the feasibility of primary surgery. We studied the changes in IDRFs after neoadjuvant chemotherapy for thoracic neurogenic tumors. METHODS: We performed a multicenter review of 27 patients presenting with unresectable thoracic neurogenic tumors. Patients received neoadjuvant chemotherapy, according to their risk group. IDRF at diagnosis and before surgery were retrospectively analyzed by a radiologist and a surgeon, blind to the initial assessment. Surgical and oncologic outcomes were reviewed. RESULTS: None of the patients presented MYCN amplification, and 78 IDRFs were identified at diagnosis. Vascular IDRFs were the most frequent, with 28 vascular IDRFs detected in 18 patients, 22 of which disappeared after chemotherapy. Reductions of tumor volume were associated with a regression of IDRFs. Patients undergoing minimally invasive surgery had smaller tumor volumes than those undergoing open surgery, and no vascular IDRF. Two patients received two additional courses of chemotherapy to reduce tumor volume sufficiently for surgery. One patient with ganglioneuroblastoma underwent early surgery due to a lack of response to initial chemotherapy. CONCLUSION: Tumor volume reduction with neoadjuvant chemotherapy eliminates most IDRF in thoracic neurogenic tumors. Vascular IDRF are rapidly resolved at this site, making surgical resection and minimally invasive surgery possible.

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.

Safety of laparoscopic fundoplication in children under 5 kg: a comparative study.

BACKGROUND: Laparoscopic fundoplication in children under 5 kg is still debated. Our objective was to evaluate the safety and efficacy of laparoscopic fundoplication (LF) in children under 5 kg. METHODS: We reviewed the cases of 96 children treated by laparoscopic fundoplication between 2005 and 2014. Thirty-five patients had a weight of 5 kg or less at the time of LF (Low Weight Group) and 61 patients had a weight between 5.1 and 10 kg (High Weight Group). The pre-operative, peri-operative, post-operative data regarding surgery and anesthesia were compared between groups. RESULTS: Mean weight was 3.9 ± 0.8 kg in the LWG and 7.8 ± 1.5 kg in the HWG. Children in the LWG were more prone to pre-operative respiratory management (40% mechanical ventilation and 42.9% oxygen therapy). The operating times (82 ± 28 min for LWG and 85 ± 31 min for HWG) and respiratory parameters during the procedure (PCO2) were comparable between groups. Post-operative complications were 1 gastric perforation with peritonitis and 1 small bowel obstruction in the LWG, 2 cases of gastric perforation with peritonitis in the HWG. Mean follow-up was 67 ± 44 months. Significant recurrence of GERD requiring a redo fundoplication was noted in 3 patients in the LWG and 1 patient in the HWG. CONCLUSION: Laparoscopic fundoplication is a safe procedure in infants ≤ 5 kg without increase of post-operative complications, recurrence, or mean operative time.

Q-island flap urethroplasty: 1-stage procedure for reconstruction of Y-type urethral duplications in children.

PURPOSE: We review our experience with Q-flap urethroplasty in children with Y-type urethral duplication. MATERIALS AND METHODS: Six children with Y-type urethral duplication underwent 1-stage urethral reconstruction using Q-flap urethroplasty between 1988 and 2012. The ventral hypospadiac urethra was always the functional one, and, therefore, was used for urethroplasty. RESULTS: Mean age at surgery was 5 years (range 1 to 13). All 6 patients had an excellent cosmetic result, with a normal appearing penis, a large apical meatus and no skin chordee. All 6 voided normally. None of the 6 children required reoperation. The only complications were urethral stenosis in 2 patients and lower urinary tract infection in 3. Cosmetic and functional results were analyzed during a mean followup of 8 years (range 1 to 12). CONCLUSIONS: Q-flap urethroplasty provides a long penile skin flap that allows 1-stage reconstruction in children with Y-type urethral duplication, with good cosmetic and functional results.

Adrenocortical tumor with precocious puberty in a 2-month-old girl.

Adrenocortical tumor is a rare childhood tumor with a median age at onset of 3.2 years. Virilization is the most common sign. Laparotomy is the reference treatment and has a favorable course. The diagnosis of adrenal tumor can be difficult. The main parameters of malignant tumors are size and metastasis. Analysis of TP53 mutation can facilitate final diagnosis. We report a case of virilizing adrenal tumor that developed in a 2-month-old girl, and which was treated with laparoscopic adrenalectomy.

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.

Nodular lesions of self-healing juvenile cutaneous mucinosis: a pitfall!

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.

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.

[Long-term occupational situation after cancer: A French registry-based study]. / Situation professionnelle à long terme après un cancer : étude réalisée à partir de registres de population.

INTRODUCTION: Few studies have explored the long-term occupational situation after cancer. The aim of our study were to study the employment status among long-term cancer survivors and to compare it to cancer-free controls from the general population at 5, 10 or 15 years after cancer diagnosis. METHODS: From data of a registry-based study, long-term survivors from breast,cervical and colorectal cancer, randomly selected from three tumor registries in France, were compared to cancer-free controls randomly selected from electoral lists. We selected active cancer survivors and cancer-free controls aged less than 60 at the time of the survey. We have studied the employment status of cases vs. controls and the factors associated with employment status. RESULTS: At 5, 10 or 15 years after diagnosis, we did not observe any significant difference in employment status between cases and controls. Among cases, 17% had lost their jobs. Older age, lower incomes, lower education, a short-term employment contract, the presence of co-morbidities, fatigue and a worse quality of life were associated with job loss. DISCUSSION: Although the employment status of the cases was comparable to that of the controls, efforts should be intensified to make it easier for patients diagnosed with cancer to return to work.

[DICER1 constitutional pathogenic variant syndrome: Where are we in 2019?] / Syndrome associé aux variants pathogènes constitutionnels de DICER1 : Où en sommes-nous en 2019 ?

Inactivating germline pathogenic variants of the DICER1 gene are responsible for a spectrum of rare diseases, which expanded a lot in recent years. The constitution of an U.S. registry with these patients and their families as well as the registration of patients in European databases of rare tumors helped to better identify diseases encountered in this syndrome but also to study its pathophysiology (major role in miRNA maturation and recently discovered functions, e.g. in genome integrity maintenance). Most encountered disorders are pediatric malignancies, mainly the pulmonary pneumoblastoma and Sertoli-Leydig tumours. However, benign pathologies such as thyroid goiters, cystic nephromas or pulmonary cystic lesions are also frequently reported. Homogeneous guidelines regimens written by the European groups working on very rare pediatric tumors are proposed but it is important to underscore that they rely on rare scientific data; therefore overall consensus remains precarious. The genetic counseling to families is still difficult due to the large observed spectrum of tumors and the incomplete penetrance. In this article, the authors update current knowledge on the DICER1 syndrome.

The National Pediatric Surgery Simulation Program in France: A tool to develop resident training in pediatric surgery.

BACKGROUND/PURPOSE: To implement resident curriculum in France based on theoretical teaching and bed side training, the national council known as the "Collège Hospitalier et Universitaire de Chirurgie Pédiatrique" examined the relevance and feasibility of systematically introducing simulation program in the pediatric surgery resident training. MATERIAL AND METHODS: A national simulation training program was developed and took place in a 2-day session organized in 7 simulation centers in France. The program included technical (laparoscopic/suturing technique on low-fidelity models) and nontechnical (6 scenarios for standardized consultation, and a team work scenario based on errors prevention in the operative room) skills. Evaluation of the program (Likert scale from 1 (bad) to 5 (excellent) and notation on 20 points) concerned trainees and trainers. RESULTS: 40 residents (95% of all pediatric surgery French residents) attended with a ratio of trainees/trainer of ½. The training objectives earned a score of 4.46/5. The pedagogical value of the seminar scored 4.7/5, teaching quality 17.95/20, and the overall seminar score was 17.35/20. CONCLUSION: This program, unique nationally, was assessed very favorably by the participating residents and by the involved trainers. To our knowledge, it represents the first mandatory national simulation training program included within a surgical training model. LEVEL OF EVIDENCE: Level IV.

Low-Cost Training Simulator for Open Dismembered Pyeloplasty: Development and Face Validation.

PURPOSE: Surgical simulation has benefited from a surge in interest over the last decade because of the increasing need for a change in the traditional apprenticeship model of teaching surgery. Open surgery for ureteropelvic junction (UPJ) poses unique training challenges owing to smaller workspaces, and finer sutures used that require increased surgical dexterity when compared with adult analogues. We describe the development and face validation of a low-cost training simulator for open dismembered pyeloplasty. MATERIALS AND METHODS: The simulator is built with A4 Kraft envelopes, catheter tip syringe filled with 30mL of air, tape, 260 modeling balloon, and 11-in party balloon. Evaluation of the device is based on an evaluation form including 11 items on a 5-point Likert-type scale. Thirty-one departments of pediatric surgery in France were contacted and received a pack containing 4 to 10 devices, already set up and ready for use, a tutorial and an evaluation form. Candidates were stratified according to their level of expertise. RESULTS: A total of 180 devices were sent. Procedures on the device were performed 118 times (65%) by expert surgeons (n = 44), fellows (n = 25), and residents (n = 49). Statistically significant difference was noted for 4 items (anatomy, model exposition, UPJ resection, and difficulty) for the 3 levels of expertise. The global score evaluation for realistic items, face validity, and usability was 4.2 (range: 1-5). CONCLUSION: This low-cost model is evaluated as an efficient tool for UPJ teaching and training. It shows promise as an educational tool.

Thoracoscopic Rib Resection in Children.

OBJECTIVE: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy. MATERIALS AND METHODS: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted. Inclusion criteria were benign tumors or rib anomalies such as synostosis, in children younger than the age of 18 years, and thoracoscopy. Open surgery and malignant tumors were excluded. Thoracoscopy was put forward using one optical port as well as one or two operative ports. RESULTS: Ten patients presented with chest pain, dyspnea, or unexplained cough. Six tumors were incidentally diagnosed. One patient presented with a chest wall deformation. Single-lung ventilation was required in 2 cases. In 1 case of endochondroma, a segmental rib resection was performed, leaving a part of the periosteum and the intercostal vessels and nerve. In this case, rib resection was performed using an endoscopic shaver drill system. As for the other cases, a simple resection of the tumor or of the bridge between two ribs (synostosis) was performed. In these cases, a Codman Kerrison laminectomy rongeur was used. There was no complication during or after surgery. Nevertheless, 2 years after surgery, pain did not disappear in 1 case, probably due to a definitive intercostal nerve damage. CONCLUSION: Benign rib lesions in children are rare. Thoracoscopy may be offered to reduce the functional deleterious consequences of an open surgery. It may be put forward especially in case of hereditary multiple exostoses where redo procedures may be required.