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.

Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01). CONCLUSIONS: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Evolution of fetal lung stiffness during gestation in two different congenital malformations.

This report is aimed at opening a new reflexion about exploration of fetal lungs malformations with 2-D ultrasound shear wave elastography (2D-SWE) in two fetuses. These preliminary results are drawn from the ELASTOMAP study, which was designed to explore feasibility of 2D-SWE on fetal lungs. Fetus A presented Congenital Pulmonary Airway Malformation and fetus B congenital obstructive emphysema. We estimated every 3 weeks the Congenital Pulmonary Airway Malformation Volume Ratio (CVR) of the malformation and we evaluated stiffness by 2D-SWE. Stiffness of the lesion and the normal lung were compared to liver stiffness using Lung to Liver Elastography (LLE) ratio. Interestingly, CVR and LLE ratios evolved differently in these two lesions caused by distinct physiopathological processes. In both cases, LLE ratio' variations preceded that of CVR. LLE ratio of the normal lung was constant through gestation. 2D-SWE could be a promising tool to describe stiffness evolution in fetal lungs malformations.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. CONCLUSIONS: Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

How to Explore Fetal Sacral Agenesis Without Open Dysraphism: Key Prenatal Imaging and Clinical Implications.

The estimated prevalence of fetal caudal dysgenesis is 1 per 100,000 births. The functional prognosis of sacral agenesis is dominated by the large spectrum of associated caudal malformations. Except for cases associated with hydrocephalus secondary to open spinal dysraphism or chromosomal anomalies, association with mental deficiency is rare. We propose a systematic prenatal approach to cases of fetal sacral agenesis based on 9 etiologic items: clinical context, type of sacral dysgenesis, associated spinal cord malformations, mobility of lower limbs, investigation of the presacral region, analysis of the gastrointestinal tract, analysis of the genitourinary tract, associated vertebral defects, and cytogenetic analysis.

Current perspectives in robot-assisted surgery.

From classical surgery to robot-assisted surgery (RAS), there has been a long way allowed by the improvements achieved in minimally invasive surgery (MIS). The last three decades have witnessed a prodigious development of MIS, and especially in the field of laparoscopic pediatric surgery but there are several limitations in the use of conventional laparoscopic surgery and RAS was developed to relieve these drawbacks. This new technology enables today the performance of a wide variety of procedures in children with a minimally invasive approach. As for all new technologies, an objective evaluation is essential with the need to respond to several questions: is the technology feasible?, is the technology safe?, is the technology efficient?, does it bring about benefits compared with current technology?, what are the procedures derived from most benefits of robotic assistance?, how to assume the transition from open surgery to minimally invasive access for RAS? In the first part of this article, some details are provided about technical concerns. Then, the implementation process with its organization, pitfalls, successes, and issues from human resources and financial standpoints is described. The learning curve is also analyzed, and a special focus on small children weighing less than 15 kg is developed. Finally, the concept of evaluation of this emerging technology is evocated and financial concerns are developed.

Automation of Wilms' tumor segmentation by artificial intelligence.

BACKGROUND: 3D reconstruction of Wilms' tumor provides several advantages but are not systematically performed because manual segmentation is extremely time-consuming. The objective of our study was to develop an artificial intelligence tool to automate the segmentation of tumors and kidneys in children. METHODS: A manual segmentation was carried out by two experts on 14 CT scans. Then, the segmentation of Wilms' tumor and neoplastic kidney was automatically performed using the CNN U-Net and the same CNN U-Net trained according to the OV2ASSION method. The time saving for the expert was estimated depending on the number of sections automatically segmented. RESULTS: When segmentations were performed manually by two experts, the inter-individual variability resulted in a Dice index of 0.95 for tumor and 0.87 for kidney. Fully automatic segmentation with the CNN U-Net yielded a poor Dice index of 0.69 for Wilms' tumor and 0.27 for kidney. With the OV2ASSION method, the Dice index varied depending on the number of manually segmented sections. For the segmentation of the Wilms' tumor and neoplastic kidney, it varied respectively from 0.97 to 0.94 for a gap of 1 (2 out of 3 sections performed manually) to 0.94 and 0.86 for a gap of 10 (1 section out of 6 performed manually). CONCLUSION: Fully automated segmentation remains a challenge in the field of medical image processing. Although it is possible to use already developed neural networks, such as U-Net, we found that the results obtained were not satisfactory for segmentation of neoplastic kidneys or Wilms' tumors in children. We developed an innovative CNN U-Net training method that makes it possible to segment the kidney and its tumor with the same precision as an expert while reducing their intervention time by 80%.

Clinical Effectiveness of Transversus Abdominis Plane (TAP) Block versus Local Anesthesia Wound Infiltration for Postoperative Pain Relief After Laparoscopic Appendicectomy in Children: A Study Protocol for a Multicenter Double-Blind Randomized Controlled Phase III Trial.

Purpose: Postoperative pain relief after laparoscopic appendicectomy is a key determinant of early rehabilitation in children. Recent guidelines recommend performing either a transversus abdominis plane (TAP) block or local anesthesia (LA) wound infiltration as part of multimodal postoperative analgesia after appendectomy. To date, the clinical effectiveness of TAP block versus LA wound infiltration has never been compared. The hypothesis of this study is that the TAP block may provide a greater opioid-sparing effect after laparoscopic appendicectomy in children than LA wound infiltration. Study Design and Methods: We designed a multicenter double-blind randomized controlled phase III trial and aim to include 110 children who undergo laparoscopic appendicectomy. Children are randomized to receive either TAP block (TAP group) or LA wound infiltration (infiltration group). Multimodal analgesia is standardized in the two groups using the same protocol, which includes the stepwise prescription of paracetamol, phloroglucinol, ketoprofene, and nalbuphine according to the hetero-evaluation of pain performed by the nurses who were blinded to the treatment allocated using the validated FLACC scale. The primary outcome is the total dose of nalbuphine administered within 24 hours after surgery. Discussion: No study has specifically compared the clinical effectiveness of TAP block versus LA wound infiltration for postoperative pain relief after laparoscopic appendectomy in children. This paper describes the protocol for a randomized trial that addresses this issue. The results of this trial will be useful for editing guidelines with a higher level of evidence on this topic.

Financial and relational impact of having a boy with posterior urethral valves.

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care. Patients and method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the "impact on family scale" (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score. Results: We retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15-51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards. Conclusion: In conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible.

Risk factors for febrile urinary tract infection in boys with posterior urethral valves.

Objective: Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors could allow for tailoring UTI prevention. The aim of this study was to use the data from the CIRCUP randomized controlled trial data to identify patient characteristics associated with a higher risk of fUTI. Patients and methods: We performed a secondary analysis of the data from the CIRCUP randomized trial which included boys with PUV, randomized to circumcision and antibiotic prophylaxis vs. antibiotic prophylaxis alone and followed for 2 years. There was only 1 episode of fUTI in the circumcision group vs. 17 in the uncircumcised group. We therefore only studied the antibiotic prophylaxis alone group and compared age at prenatal diagnosis, size and weight at birth, presence of dilating VUR at diagnosis, abnormal DMSA scan at 2 months, and nadir creatinine between children who presented a fUTI and those who did not, as well as age at first episode of fUTI. Results: The study group consisted of 42 patients of which 17 presented at least on fUTI. Presence of dilating VUR was significantly associated with risk of fUTI (p = 0.03), OR: 6 [CI 95% = (1.13-27.52)]. None of the other parameters were associated with increased risk of fUTI. We observed three distinct time periods for presenting a fUTI with a decrease in infection rate after the first 40 days of life, then at 240 days of life. Conclusion: In boys with PUV, presence of high-grade VUR is associated with a higher risk of presenting a fUTI. The rate of febrile UTIs seems to decrease after 9 months.

Nutritional status at age 1 year in patients born with esophageal atresia: A population-based, prospective cohort study.

Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure. Study design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model. Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year (P < 0.05). Neither EA type nor surgical treatment was associated with growth failure. Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.

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.

Congenital Urethral Fistula: A Case Report and Literature Review.

Male congenital urethral fistula is an extremely rare condition. It is characterized by an abnormal opening of the ventral aspect of the penis. We report the case of a 1-month-old boy with congenital urethral fistula. We will describe the surgical technique, postoperative results, and literature review.

Fusion of multiple segmentations of medical images using OV2ASSION and Deep Learning methods: Application to CT-Scans for tumoral kidney.

Nephroblastoma is the most common kidney tumour in children. Its diagnosis is based on imagery. In the SAIAD project, we have designed a platform for optimizing the segmentation of deformed kidney and tumour with a small dataset, using Artificial Intelligence methods. These patient's structures segmented by separate tools and processes must then be fused to obtain a unique numerical 3D representation. However, when aggregating these structures into a final segmentation, conflicting pixels may appear. These conflicts can be solved by IA techniques. This paper presents a synthesis of our segmentation contribution in the SAIAD project and a new fusion method. The segmentation method uses the FCN-8s network with the OV2ASSION training method, which allows segmentation by patient and overcomes the limited dataset. This new fusion method combines the segmentations of the previously performed structures, using a simple and efficient network combined with the OV2ASSION training method as well, in order to manage eventual conflicting pixels. These segmentation and fusion methods were evaluated on pathological kidney and tumour structures of 14 patients affected by nephroblastoma, included in the final dataset of the SAIAD project. They are compared with other methods adapted from the literature. The results demonstrate the effectiveness of our training method coupled with the FCN-8s network in the segmentation process with more patients, and in the case of the fusion process, its effectiveness coupled with a common network, in resolving the conflicting pixels and its ability to improve the resulting segmentations.

3D reconstruction of Wilms' tumor and kidneys in children: Variability, usefulness and constraints.

INTRODUCTION: Wilms' tumor (WT) is the most common type of malignant kidney tumor in children. Three-dimensional reconstructions can be performed pre-operatively to help surgeons in the planning phase. OBJECTIVES: The main objective of this study was to determine the variability of WT segmentation and 3D reconstruction. The secondary objectives were to assess the usefulness of these 3D reconstructions in the surgical planning phase and in the selection of patients for nephron-sparing surgery (NSS). METHODS: 14 scans from 12 patients were manually or semi-automatically segmented by 2 teams using 3D Slicer software. Inter-individual variability of 3D reconstructions was measured based on the Dice index. The utility of 3D reconstructions for the surgical planning was evaluated by 4 pediatric surgeons using a 5-point Likert scale. The possibility of undertaking NSS was evaluated according to the criteria defined in the Umbrella SIOP-RTSG 2016 protocol. RESULTS: Segmentation of the WT, healthy kidney, pathological kidney, arterial and venous vascularization could be performed for all of the patients in this study. Urinary cavities segmentation could only be performed for 5 out of 14 scans that had a delayed acquisition phase. The mean time required to carry out these segmentations was 8.6 h [3-15 h]. The mean Dice index for all of the scans was good (mean: 0.87; range [0.83-0.91]). Considering each anatomical structure, the Dice index was very good for the WT (mean: 0.95; range [0.91-0.97]) and the healthy kidney (mean: 0.95; range [0.93-0.96]), good for the pathological kidney (mean: 0.87; range [0.69-0.96]) and arterial vascularization (mean: 0.84; range [0.74-0.91]). The Dice index was lower than 0.8 for venous vascularization only (mean: 0.77; range [0.58-0.86]). All the surgeons who were interviewed agreed that the 3D reconstructions were realistic representations and useful for the surgical planning phase. The images reconstructed in 3D allowed most of the criteria defined by the Umbrella SIOP-RTSG 2016 protocol to be evaluated regarding the selection of patients who could benefit from NSS. CONCLUSION: The inter-individual variability of 3D reconstructions of WT is acceptable. Three-dimensional representation appears to assist surgeons with the surgical planning phase by allowing them to better anticipate the operative risks. 3D reconstructions can also be an additional tool to better select patients for NSS. However, the manual or semi-automatic method used is very time-consuming, making it difficult for a routinely use. Developing techniques to automate this segmentation process, therefore, appears to be essential if surgeons and radiologists are to use it in daily practice.

Dual intestinal anomalies in dizygotic twins.

We report on the case of two digestive malformations in dizygotic/dichorionic/diamniotic twins born at 31 weeks of gestation. The mother (gravida 1 para 0) was treated by hydroxychloroquine for systemic lupus erythematosus during pregnancy. Twin A presented an arch-like dilatation on antenatal ultrasounds, characteristic of segmental volvulus. After birth, twin B presented repeated vomiting on feeding, leading us to diagnose ileal atresia despite normal antenatal ultrasounds. Both twins underwent surgery and the postoperative period was uneventful. After 1 year of follow-up, the twins are in excellent health without digestive sequelae. Genetic testing for cystic fibrosis was negative. The placenta showed diffuse signs of hypoxia and ischaemia, indicating that the root cause was vascular. The pathophysiology of intestinal atresia is hypothesised to derive from a vascular incident during fetal development. We are therefore led to believe that an intrauterine vascular event is the most likely cause of the dual malformation.

High Pressure Balloon Dilatation of Primary Obstructive Megaureter in Children: A Multicenter Study.

Aim of the Study: We described the initial experience of four referral centers in the treatment of primary obstructive megaureter (POM) in children, by high-pressure balloon dilatation (HPBD) of the ureterovesical junction with double JJ stenting. We managed a retrospective multicenter study to assess its effectiveness in long-term. Methods: We reviewed the medical records of all children who underwent HPBD for POM that require surgical treatment from May 2012 to December 2017 in four different institutions. The primary outcome measured was ureterohydronephrosis (UHN) and its degree of improvement after the procedure. Secondary outcomes were postoperative complications and resolution of preoperative symptomatology. Main Results: Forty-two ureters underwent HPBD for POM in 33 children, with a median age of 14.7 months - (range: 3 months -15 years). Ureterohydronephrosis improves in 86% of ureters after one endoscopic treatment. Three cases required a second HPBD. Four patients required surgical treatment for worsening of UHN after endoscopic treatment. The post-operative complication rate was 50% (21 ureters). In 13 cases (61%), they were related to double J stent. The median follow-up was 24 months (2 months -5 years) and all patients were symptom-free. Conclusion: We reported the first multicenter study and the largest series of children treated with HPBD, with an overall success rate of 92%. Endoscopic treatment can be a definitive treatment of POM since it avoided reimplantation in 90% of cases. Complications are mainly due to double J stent.