Beyond image defined risk factors (IDRFs): a delphi survey highlighting definition of the surgical complexity index (SCI) in neuroblastoma.

PURPOSE: Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. METHODS: A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. RESULTS: After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. CONCLUSIONS: A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Koyanagi urethroplasty for proximal hypospadias: A stage procedure?

OBJECTIVE: To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias. METHODS: A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD). RESULTS: The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P<0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P>0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular. CONCLUSION: In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results. LEVEL OF EVIDENCE: III.

Repeated detrusor injection of botulinum toxin A for neurogenic bladder in children: A long term option?

AIMS: Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort. METHODS: Patients who underwent at least 4 IDI-TBA between 2005 and 2017 for neurogenic bladder related issues were included (detrusor overactivity and low compliance). Clinical and cystometric data were collected before and after the first injection and after the last injection. The primary endpoint was the proportion of patients with non-abnormal cystometry (no detrusor overactivity and normal compliance). Secondary outcomes were the evolution of the observed bladder capacity/expected ratio, surgical complications and acquired kidney impairment. RESULTS: From the 832 patients referred to our institution for neurogenic bladder, 48 underwent IDI-TBA, and 17 at least 4 injections. Among them, a total of 95 procedures were performed (median per patient 5 [4-8]). While the first injection had a significant effect for 82.3% patients, the last injection improved the medical condition for only 53.0% cases. The bladder capacity ratio, initially 36.1%, increased to 80.3% after the first injection but decreased to a level of 57.1% at last. After a median follow-up of 57 [34-102] months, no severe complications were reported but 11.8% of patients presented with repeated pyelonephritis. A bladder augmentation surgery was finally indicated for 35.3% cases. CONCLUSIONS: Despite a low complication rate and impressive cystometric results after the first injection, IDI-TBA efficacy decreased with time and repetition. These findings prone a long-term follow-up and a "à-la-carte" management of this specific population depending on the long-term response to IDI-TBA.

Emergency department visits for pediatric traumatic injuries during general confinement: A single-center study in an urban setting.

BACKGROUND: The first case of SARS-CoV-2 was detected in France in January 2020 and the government decided on national confinement from March 17 to May 11, 2020. Our aim was to analyze the incidence of pediatric emergency department (ED) visits and hospitalizations for traumatic injuries during this period. METHODS: Any visit with an ICD-10 discharge diagnosis code of burn, fracture, traumatic wound, or sprain/bruise contusion was recorded within the 2 weeks before (weeks 10 and 11) and during the confinement (weeks 12 and 19). The visits with the same ICD-10 discharge diagnosis code during similar weeks of the previous 2 years were also included. For each of those visits, the number of hospitalizations was counted. RESULTS: The number of recorded visits between week 10 and 19 in 2018, 2019, and 2020 was, respectively, 2657, 2625, and 1106 children. The average number of visits per day during the confinement (13±5) was significantly different from the average number of visits per day during the same weeks in 2018 and 2019 (38±8 vs. 39±9, P<0.0001). The average number of visits per day was significantly lower during confinement compared with 2018/2019 for three categories of diagnoses (P<0.0001) but not for burns (1.7 vs. 1.8, P=0.23). The average number of hospitalizations per day was significantly lower during the confinement than during 2018/2019 (1.6±1.3 vs. 2.6±1.8, P<0.0001). CONCLUSION: Confining children in an urban setting appears to decrease the incidence of injuries, except for burns. These data may be useful in reorganizing caregiver supervision and hospital units. These results will need to be consolidated in a multicenter study.

How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review.

INTRODUCTION: Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. METHODS: All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. RESULTS: Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1-2) resections-anastomoses (RA) and 6 (1-10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15-25 cm). Median time to full oral feed was 46 days (14-626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2-8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. CONCLUSION: Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.

Lateral endoscopic parathyroidectomy in children.

Endoscopic thyroid and parathyroid surgery was first described by Gagner in 1996, and Henry subsequently proposed a lateral endoscopic approach in 1999. Technical progress in the fields of optics, endoscopy, digital imaging and laparoscopy has gradually enhanced the feasibility and clinical utility of this technique for the treatment of benign and malignant lesions. To date, published paediatric cases have only concerned thyroid surgery. In the light of two clinical cases, this article describes our lateral endoscopic approach applied to paediatric parathyroid surgery.

Added value of MRI for the diagnosis of adnexal torsion in children and adolescents after inconclusive ultrasound examination.

PURPOSE: The purpose of this study was to assess the performance of magnetic resonance imaging (MRI) in children and adolescents with suspected adnexal torsion (AT) after inconclusive initial ultrasound examination. MATERIALS AND METHODS: Twenty-eight girls with a mean age of 12±4 (SD) years (range: 1 month to 18years) were included. All had clinically suspected AT and inconclusive initial ultrasound findings followed by pelvic MRI as a second-line imaging modality. The final diagnosis was obtained by surgery or follow-up. Two radiologists blinded to the clinical, ultrasound and surgical data, retrospectively and independently reviewed MRI examinations. Clinical and MRI features associated with AT were searched for using univariate analyses. RESULT: Among the 28 patients, 10/28 patients (36%) had AT and 22/28 (79%) had an ovarian or tubal mass. AT was associated with an age<13years (OR: 10.7; 95% CI: 1.3-148.2) (P=0.022) and a whirlpool sign at MRI (OR: 61.0; median unbiased estimate, 7.2) (P<0.0001). When a mass was present, the best quantitative MRI criteria for AT were mass volume and ovary-corrected volume≥30cm3 (κ=0.72 and 0.61, respectively), mass axis length≥5cm (κ=0.90), and mass surface area≥14 cm2 (κ=0.58), with moderate to almost perfect interobserver agreement. The overall sensitivity, specificity and accuracy of MRI for the diagnosis of AT were 100% (10/10; 95% CI: 69-100), 94% (17/18; 95% CI: 73-100) and 96% (27/28; 95% CI: 82-100) respectively, with perfect interobserver agreement (κ=1). CONCLUSION: In pediatric patients with suspected AT and inconclusive initial ultrasound examination, a strategy including MRI as a second-line imaging modality should be considered if MRI does not delay a potential surgery.

Ingestion of neodymium magnet spheres: Three case studies.

We report three pediatric cases of two, five, and 12 neodymium magnet spheres ingested simultaneously. In two cases, endoscopic extraction failed because of the strength of the magnetic attraction and a gastrotomy was performed to remove all magnets. In the third case, the magnets were not accessible endoscopically and were removed by laparotomy. We wish to warn consumer groups and pediatricians about the danger of neodymium magnets.

Risk factors for local recurrence in Wilms tumour and the potential influence of biopsy - the United Kingdom experience.

RATIONALE: The UKW3 trial compared biopsy/pre-operative chemotherapy versus immediate nephrectomy and afforded the opportunity to examine the influence of percutaneous retroperitoneal biopsy and other factors on local and distant relapse of Wilms tumour (WT). METHODS: Patients with unilateral WT (stages I-IV) excluding metachronous relapse or early progressive disease were eligible. Metastatic and 'inoperable' tumours were biopsied electively. 'Local' was defined as relapse within the abdomen, except for liver metastases considered as 'distant' relapse, together with other haematogenous routes. Uni- and multivariable analyses estimated the risk factors for relapse. RESULTS: Overall, 285/635 (44.9%) patients had a biopsy. With a median follow-up of 10.1 years, 35 (5.5%) patients experienced a 'local', 15 a combined (2.4%) and 60 (9.4%) a 'distant' relapse. On univariate analysis, biopsy, anaplasia and tumour size were associated with an increased risk of local relapse. On multivariable analysis, anaplasia and tumour size remained significant for local relapse whereas the elevated risk of biopsy (hazards ratio (HR) = 1.80: 95% confidence interval (CI) 0.97-3.32, p = 0.060) was marginal. Age, anaplasia, tumour size, lymph nodes metastases and stage, but not biopsy, were individually associated with increased risk of distant relapse but only age and anaplasia remained significant following multivariable analysis. CONCLUSIONS: The UKW3 trial provides some reassurance that biopsy should not automatically lead to 'upstaging' of WT. Further assessment of this controversial area is required. Comparison of local relapse rates in a multinational trial in which the United Kingdom (UK) continued the practice of routinely biopsying all patients in contrast to the standard European approach will afford this opportunity and is planned.

[Pseudotumoral soft tissue masses in children and adolescents]. / Masses des tissus mous d'allure tumorale de l'enfant et de l'adolescent.

INTRODUCTION: Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics. MATERIALS AND METHODS: This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses. RESULTS: Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19 patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors). CONCLUSIONS: A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.

Mucormycosis as a rare cause of severe gastrointestinal bleeding after multivisceral transplantation.

Mucormycosis, an emerging fungal infection in solid organ transplant patients, is mostly located in rhino-orbito-cerebral, pulmonary, and cutaneous areas, or disseminated with poor prognosis. A 4-year-old girl with chronic intestinal pseudo-obstruction syndrome underwent a modified multivisceral transplantation, including half of the stomach, the duodeno-pancreas, the small bowel, and the right colon. On postoperative day 5, a digestive perforation was suspected. Surgical exploration found a small necrotic area on the native stomach, which was externally drained. The next day, massive gastric bleeding occurred. During the emergency laparotomy, 2 hemorrhagic ulcers were found and resected from the transplanted stomach. Pathology and fungal culture showed mucormycosis caused by Lichtheimia (formerly Absidia) ramosa in both the transplanted and native stomach. High-dose intravenous liposomal amphotericin B was immediately started. No other site of fungal infection was found. The child recovered, and 3 years after transplantation, is alive and well, off parenteral nutrition. The originality of this case is the very early presentation after transplantation, the unusual site, and the complete recovery after rapid medico-surgical management. The origin of the fungus and treatment are discussed.

[Anorectal malformations]. / Malformations ano-rectales.

Anorectal malformations (ARM) are the result of an abnormal development of the terminal part of the digestive tract interesting anus and/or rectum that occur early between the sixth and tenth week of embryonic development. They carry a malformation spectrum of severity depending on the level of disruption of the anorectal canal and of the associated caudal malformations (sacrum and spine). ARM are associated in over half the cases with other malformations that can be integrated in some cases in known syndromes. If surgical treatment to restore anatomy as normal as possible is indispensable, post-operative care is essential for these patients whose defecation mechanisms are altered, to reach if not continence, at least a socially acceptable cleanliness.

Renal transplantation in children: critical analysis of age related surgical complications.

To determine age-related risk factors of urological and vascular complications. We performed a retrospective analysis of the data of 202 renal transplantations in 193 children between 1989 and 2007 at a single institution. Out of 193 grafts (combined renal and liver grafts were excluded), we observed urological complications in 42 cases (21.7%) leading to graft loss in one case and vascular complications in 27 cases (13.9%) leading to graft loss in seven. The urological complications were VUR (n=25, 12.4%), ureteral stricture (n=10, 5%), anastomotic leak (n=4, 2%), ureteral necrosis (n=2, 1%), and incrustative pyelitis (n=1, 0.5%). Vascular complications were arterial stricture (n=14, 7.2%), arterial thrombosis (n=4, 2%), venous thrombosis (n=2, 1%), and others (n=7). Donors aged less than six yr were a risk factor of vascular complications leading to graft loss (p=0.0001), whereas patients with PUV had more urological complications (p=0.001). Overall patient and graft survival is 93.1% and 84% at five yr, respectively. Surgical complications remain a major cause of graft loss (12%) and morbidity in children's kidney transplantation (38.9%). Young age of donors is the major risk factor of early graft loss as a result of vascular complication. However, donor selection based on age is limited by the shortage of organs.

Population pharmacokinetics and bayesian estimator of cyclosporine in pediatric renal transplant patients.

Cyclosporine A (CsA) is an immunosuppressive drug widely used in pediatric renal graft recipients. Its large interindividual pharmacokinetic variability and narrow therapeutic index render therapeutic drug monitoring necessary. However, information about CsA pharmacokinetics is scarce and no population pharmacokinetic (popPK) studies in these populations have been reported so far. to the objectives of this study were 1) to develop a PKpop model and identify the individual factors influencing the variability of CsA pharmacokinetics in pediatric kidney recipients; and 2) to build a Bayesian estimator allowing the estimation of the main PK parameters and exposure indices to CsA on the basis of a limited sampling strategy (LSS). The popPK analysis was performed using the NONMEM program. A total of 256 PK profiles of CsA collected in 98 pediatric renal transplant patients (mean age 9.7 +/- 4.5 years old) within the first year posttransplantation were studied. A 2-compartment model with first-order elimination, and Erlang distribution to describe the absorption phase, fitted the data adequately. For Bayesian estimation, the best LSS was determined based on its performance in estimating area under the concentration-time curve (AUC0-12h) and validated in an independent group of 20 patients. The popPK analysis identified body weight and posttransplant delay as individual factors influencing the apparent central volume of distribution and the apparent clearance, respectively. Bayesian estimation allowed accurate prediction of AUC0-12h using predose, C1h, and C3h blood samples with a mean bias between observed and estimated AUC of 0.5% +/- 11% and good precision (root mean square error = 10.9%). This article reports the first popPK study of CsA in pediatric renal transplant patients. It confirms the reliability and feasibility of CsA AUC estimation in this population. The body weight and the posttransplantation delay were identified to influence PK interindividual variability of CsA and were included in the Bayesian estimator developed, which could be helpful in further clinical trials.