Use of N-Acetylcysteine in Preterm Neonates with Enteral Feeding Intolerance and Intestinal Obstruction: A Case Series and Review of the Literature.

(1) Background: The use of N-acetylcysteine (NAC) to relieve meconium obstruction of prematurity in the first days of life has been reported, with NAC reducing the viscosity of luminal contents by cleaving the disulfide bonds of mucoproteins. However, its use in this population should be further explored since it has been associated with hypernatremia and transient increase in transaminases and bilirubin. (2) Methods: In this retrospective study, we included neonates admitted because of enteral feeding intolerance and intestinal obstruction from 2019 to 2021 who received NAC as a rescue therapy before explorative laparotomy.

Megacystis-microcolon-intestinal hypoperistalsis syndrome: don't forget the bladder.

Purpose: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS.

Methods: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years.

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

Background: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF.

A new variant in the GATA6 gene associated with tracheoesophageal fistula, pulmonary vein stenosis and neonatal diabetes.

Introduction: GATA6 is a gene that encodes a transcription factor with a key role in the development of several organ systems, including the development of the pancreas. It is associated with neonatal diabetes but also with other extra-pancreatic anomalies.

Case Presentation: This report describes the association of tracheoesophageal fistula (TEF), pulmonary vein stenosis (PVS), and neonatal diabetes caused by a novel mutation of the GATA6 gene in a small-for-gestational-age male neonate born at 32 weeks of gestation.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

Introduction: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations.

Methods: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects.

Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center.

Objective: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery.

European Paediatric Surgeons' Association Consensus Statement on the Management of Neonatal Ovarian Simple Cysts.

Introduction:  Neonatal ovarian simple cyst management from the pediatric surgical aspect is unclear on cyst size, follow-up, and preferred surgical approach. Therefore, this topic was selected for the 2022 Consensus Session meeting of the European Paediatric Surgeons' Association (EUPSA).

Methods:  The literature was reviewed on a predefined set of questions relating to the management of the neonatal ovarian simple cysts by a panel of 7 EUPSA members, on current evidence-based opinion and practice outlined.

Respiratory and Musculoskeletal Long-Term Outcomes after Surgical Resection of Congenital Cystic Adenomatoid Malformation of the Lung in Newborns, Infants, and Toddlers.

Introduction:  The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers.

Materials And Methods:  Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers).

Isoperistaltic gastric tube for long gap esophageal atresia (LGEA) in newborn, infants, and toddlers: a case-control study from a tertiary center.

Background: Limited evidence exists about outcomes after gastric tube formation as "rescue" technique to avoid esophageal replacement in long gap esophageal atresia (LGEA). The last ERNICA Consensus Conference on the Management of LGEA has placed the techniques of gastric tubulization among the priorities for future research.

Aims: Evaluate personal experience with Isoperistaltic Gastric Tube (IGT) and compare its outcomes with other more popular techniques for LGEA.

Early vasopressin infusion improves oxygenation in infants with congenital diaphragmatic hernia.

Objective: Congenital Diaphragmatic Hernia (CDH) is a complex disease including a diaphragmatic defect, lung hypoplasia, and pulmonary hypertension. Despite its increasing use in neonates, the literature on the use of vasopressin in neonates is limited. The aim of this work is to analyze the changes in clinical and hemodynamic variables in a cohort of CDH infants treated with vasopressin.

Lung Ultrasound Score in Neonates with Congenital Diaphragmatic Hernia (CDH-LUS): A Cross-Sectional Study.

The use of a lung ultrasound (LUS) score has been described in the early phases of neonatal respiratory distress syndrome; however, there is still no data regarding the application of the LUS score to neonates with a congenital diaphragmatic hernia (CDH). The objective of this observational cross-sectional study was to explore, for the first time, the postnatal changes in LUS score patterns in neonates with CDH, with the creation of a new specific CDH-LUS score. We included all consecutive neonates with a prenatal diagnosis of CDH admitted to our Neonatal Intensive Care Unit (NICU) from June 2022 to December 2022 who underwent lung ultrasonography.

Unsolved problems in CDH follow-up.

In patients affected by CDH, survival beyond the neonatal period continues to increase thanks to technological and pharmacological improvements. Conversely, patients, families and caregivers are more and more frequently facing "new" complex late comorbidities, including chronic pulmonary and cardiac dysfunctions, neurodevelopmental challenges, and specific nutritional requirements, that often require ongoing long-term medical or surgical care. Therefore, late morbidity is now a key focus in clinical care of CDH.

Magnamosis for long gap esophageal atresia: Minimally invasive "fatal attraction".

Background: Aim of study is to report our preliminary experience with magnetic anastomosis (magnamosis) treating long-gap esophageal atresia (LGEA), the most challenging condition of esophageal atresia continuum. Magnamosis has been reported in 20 patients worldwide as an innovative and marginally invasive option.

Methods: Prospective evaluation of all LGEA patients treated with magnamosis was performed (study registration number: 2535/2021).

Hemodynamic Assessment of a Large Pulmonary Arteriovenous Malformation in a Neonate: Case Report and Review of Literature.

Herein we report the case of a neonate with a prenatally diagnosed large pulmonary arteriovenous malformation, managed with minimally invasive hemodynamic monitoring in our Neonatal Intensive Care Unit. The combination of Near-Infrared Spectroscopy and Pressure Recording Analytical Method could guide neonatal management of critical cases of vascular anomalies: immediate data are offered to clinicians, from which therapeutic decisions such as timing of surgical resection are made to achieve a positive outcome. We also systemically collected and summarized information on patients' characteristics of previous cases reported in literature to data, and we compared them to our case.

Vascular Birthmarks as a Clue for Complex and Syndromic Vascular Anomalies.

Vascular birthmarks are common in neonates (prevalence: 20-30%) and mostly incidental findings sometimes with spontaneous regression (salmon patch and nevus simplex). Capillary malformations are found in about 1% and infantile hemangiomas are found in 4% of mature newborns. Vascular malformations are classified according to their most prominent vessel type.

Guidelines of the Italian Society of Videosurgery (SIVI) in Infancy for the minimally invasive treatment of Hypertrophic Pyloric Stenosis in neonates and infants.

The most appropriate treatment for the infantile Hypertrophic Pyloric Stenosis (HPS) is still debated. The non-surgical conservative treatment with oral or intravenous administration of atropine does not enjoy a widespread appreciation for several factors (..

Guidelines of the Italian Society of Videosurgery in Infancy (SIVI) for the minimally invasive treatment of fetal and neonatal ovarian cysts.

In the last three decades, fetal ovarian cysts were diagnosed more frequently, due to technological improvement and the increasing use of prenatal screening ultrasound. Nonetheless, treatment uncertainties are still present, either prenatally or postnatally. Recently, significant innovations on diagnosis and treatment have been proposed and a more conservative, minimally invasive approach may be offered to the Pediatrician or the Surgeon who face with this condition during prenatal or neonatal age.

Neurodevelopmental outcome in infants with esophageal atresia: risk factors in the first year of life.

Data on neurodevelopmental outcomes of infants born with esophageal atresia (EA) are still scarce and controversial. The aims of our study were to evaluate motor and cognitive development during the first year of life, in patients operated on of EA and to investigate potential risk factors for motor and cognitive development both at 6 and 12 months. This is an observational prospective longitudinal study in a selected cohort of type C and D EA infants enrolled in our follow-up program from 2009 to 2017.

Diagnostic Workup of Neonates With Esophageal Atresia: Results From the EUPSA Esophageal Atresia Registry.

Controversies exist on the optimal diagnostic workup for neonates with esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). Aim of this study was to describe the current diagnostic policies in EA/TEF patients enrolled in an International multicenter registry. All patients consecutively registered from July 2014 to December 2017 in the EUPSA Esophageal Atresia Registry (EUPSA-EAR) were included in the study.