Combined Radial Incision and Steroid Injection for Refractory Colorectal Stenosis After Pull-Through Surgery in Hirschsprung Disease: An Innovative Conservative Treatment.

Background: Anastomotic stenosis after pull-through surgery remains a challenge in the management of Hirschsprung disease. Based on the management of esophageal stenosis, we evaluated the efficacy of combined radial incision and steroid injection for the treatment of refractory colorectal anastomotic stenosis after pull-through.

Impact Of Innovation: Combined radial incision and steroid injection is an alternative conservative treatment of refractory anastomotic stenosis after pull-though for Hirschsprung disease, avoiding a potential complicated redo pull-though surgery.

Intraperitoneal insufflation of carbon dioxide rescues intestinal damage in an experimental murine model of colitis.

Objectives: Necrotizing enterocolitis (NEC) is a severe neonatal surgical condition, associated with a prolonged pro-inflammatory state, leading to high mortality and morbidity rates. Carbon dioxide (CO ) insufflation during laparoscopy may have an anti-inflammatory effect. We aimed to evaluate the effects of CO -insufflation on experimental colitis.

Robotic-assisted colectomy in children: a comparative study with laparoscopic surgery.

The aim of this study was to compare outcomes of laparoscopic and robotic-assisted colectomy in children. All children who underwent a colectomy with a laparoscopic (LapC) or robotic-assisted (RobC) approach in our institution (January 2010-March 2023) were included. Demographics, surgical data, and post-operative outcomes within 30 days were collected.

Scarless laparoscopic incisions in Pfannenstiel (slip): the first 50 cases using an innovative approach in pediatric robotic surgery.

Incisions in the supra-pubic region have been described robotic-assisted surgery to improve cosmetic results in adults, but seldom in children. We aimed to present an innovative trocar placement in the Pfannenstiel line, named Scarless Laparoscopic Incisions in Pfannenstiel (SLIP), and evaluate its feasibility for various intra-abdominal procedures and its cosmetic results in pediatric robotic surgery. We performed a monocentric prospective study, including children undergoing robotic-assisted surgeries using a SLIP approach (July 2019-September 2021).

The role of irinotecan-bevacizumab as rescue regimen in children with low-grade gliomas: a retrospective nationwide study in 72 patients.

Introduction: At least half of children with low-grade glioma (LGG) treated with first line chemotherapy experience a relapse/progression and may therefore need a second-line chemotherapy. Irinotecan-bevacizumab has been recommended in this setting in France after encouraging results of pilot studies. We performed a retrospective analysis to define the efficacy, toxicity and predictors for response to the combination on a larger cohort.

How does magnetically controlled growing rods insertion affect sagittal alignment in ambulatory early onset scoliosis patients?

Purpose: The importance of sagittal alignment restoration in early onset scoliosis (EOS) management has rarely been investigated to date. The aim was to report the influence of magnetically controlled growing rods (MCGR) insertion on the sagittal alignment of EOS patients.

Methods: All consecutive ambulatory patients operated with MCGR rods between 2011 and 2018 were retrospectively included in four institutions.

Feeding disorders in children with oesophageal atresia: a cross-sectional study.

Introduction: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale.

Thoracoscopic Lobectomy for Congenital Pulmonary Airway Malformation: Where Are We in 2019?

Thoracoscopic surgery for congenital pulmonary airway malformation (CPAM) is still a matter of debate and used by approximately 50% of the surgeons in Europe. Several questions need to be addressed about CPAM. The adequate treatment, the surgical approach, and the follow-up are few of them.

Management of ureteropelvic junction obstruction in children-a roundtable discussion.

The investigation, management and follow-up of paediatric ureteropelvic junction obstruction is not standardized. The Young Pediatric Urology Committee of the European Society of Pediatric Urology interviewed five experts in the field on various aspects of management and compared this with published literature.

Congenital central hypoventilation syndrome and Hirschsprung disease: A retrospective review of the French National Registry Center on 33 cases.

Aim Of The Study: Congenital Central Hypoventilation Syndrome (CCHS) is a rare affection associated to Hirschsprung disease (HD) in 20% of the cases. Using the French CCHS registry, we described the population of patients suffering from both CCHS and HD reporting the outcome on these patients.

Methods: Medical records were reviewed.

Phenotypic spectrum associated with SPECC1L pathogenic variants: new families and critical review of the nosology of Teebi, Opitz GBBB, and Baraitser-Winter syndromes.

The SPECC1L protein plays a role in adherens junctions involved in cell adhesion, actin cytoskeleton organization, microtubule stabilization, spindle organization and cytokinesis. It modulates PI3K-AKT signaling and controls cranial neural crest cell delamination during facial morphogenesis. SPECC1L causative variants were first identified in individuals with oblique facial clefts.

Patient and transplant outcome in infants starting renal replacement therapy before 2 years of age.

Background: Despite major technical improvements in the care of children requiring renal replacement therapy (RRT) before 2 years of age, the management of those patients remains challenging and transplantation is generally delayed until the child weighs 10 kg or is 2 years old. In this national cohort study, we studied patient and graft survival in children starting RRT before 2 years of age to help clinicians and parents when deciding on RRT initiation and transplantation management.

Methods: All children starting RRT before 24 months of age between 1992 and 2012 in France were included through the national Renal Epidemiology and Information Network (REIN) registry.

Cytoreductive surgery and hyperthermic intraperitoneal perfusion with chemotherapy in children with peritoneal tumor spread: A French nationwide study over 14 years.

Background: Efficacy and role of cytoreductive surgery (CRS) and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) remain poorly documented in pediatric tumors.

Methods: This retrospective national study analyzed all pediatric patients with peritoneal tumor spread treated by CRS and HIPEC as part of a multimodal therapy in France from 2001 to 2015.

Results: Twenty-two patients (nine males and 13 females) were selected.

Standardization of pediatric uroradiological terms: A multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.

Standardization of pediatric uroradiological terms: a multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.

Neurocognitive profiles in MSUD school-age patients.

Maple syrup urine disease (MSUD), an inborn error of amino acids catabolism is characterized by accumulation of branched chain amino acids (BCAAs) leucine, isoleucine, valine and their corresponding alpha-ketoacids. Impact on the cognitive development has been reported historically, with developmental delays of varying degree. Currently, earlier diagnosis and improved management allow a better neurodevelopment, without requirement of special education.

Relocation stress induces short-term fecal cortisol increase in Tonkean macaques (Macaca tonkeana).

The level of glucocorticoids, especially if obtained from noninvasive sampling, can be used as an index of animal well-being, allowing evaluation of the animal's response to environmental modifications. Despite evidence that these hormones play a relevant role in energy metabolism regulation in perceived or real stress events, little is known regarding the factors that could modify the capability of animals to cope with relocation events. The aim of this research was to assess fecal cortisol metabolite concentrations before, during and after acute stress (transfer and relocation event) in two well-established social groups of Tonkean macaques (Macaca tonkeana).

Quantitative Shear-Wave Elastography of the Liver in Preterm Neonates with Intra-Uterine Growth Restriction.

The feasibility and reproducibility of liver stiffness measurements using Supersonic Shear-wave Imaging (SSI) in preterm neonate have not been reported. Our aim was to determine if liver stiffness differs between intra-uterine growth restriction (IUGR) and appropriate for gestational age (AGA) preterm infants with/without cholestasis. We measured liver stiffness (in kPa) in 45 AGA and 18 IUGR preterm infants, and assessed reproducibility in 26 preterms using Intraclass Correlation Coefficients (ICC) and Bland-Altman tests.

Nijmegen Breakage Syndrome: Clinical and Immunological Features, Long-Term Outcome and Treatment Options - a Retrospective Analysis.

Purpose: Nijmegen Breakage Syndrome (NBS) is a rare inherited condition, characterized by microcephaly, chromosomal instability, immunodeficiency, and predisposition to malignancy. This retrospective study, characterizing the clinical and immunological status of patients with NBS at time of diagnosis, was designed to assess whether any parameters were useful in disease prognosis, and could help determine patients qualified for hematopoietic stem cell transplantation.

Methods: The clinical and immunological characteristics of 149 NBS patients registered in the online database of the European Society for Immune Deficiencies were analyzed.