A Population-Based Cohort Study on Diagnosis and Early Management of Anorectal Malformation in the UK and Ireland.

Background: This study describes the presentation and initial management of anorectal malformation (ARM); evaluating the frequency, causes and consequences of late diagnosis.

Methods: A prospective, population cohort study was undertaken for newly diagnosed ARMs in the UK and Ireland from 01/10/2015 and 30/09/2016. Follow-up was completed at one year.

Developing a core outcome set for the health outcomes for children and adults with congenital oesophageal atresia and/or tracheo-oesophageal fistula: OCELOT task group study protocol.

Introduction: Heterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions to standardise outcome reporting, facilitate meta-analysis and improve the relevance of research for patients and families. Our aim is to develop an internationally-agreed, comprehensive COS for OA-TOF, relevant from birth through to transition and adulthood.

Wilms Tumor With Raised Serum Alpha-Fetoprotein: Highlighting the Need for Novel Circulating Biomarkers.

Wilms tumor (WT) is the commonest cause of renal cancer in children. In Europe, a diagnosis is made for most cases on typical clinical and radiological findings, prior to pre-operative chemotherapy. Here, we describe a case of a young boy presenting with a large abdominal tumor, associated with raised serum alpha-fetoprotein (AFP) levels at diagnosis.

Frenotomy with breastfeeding support versus breastfeeding support alone for infants with tongue-tie and breastfeeding difficulties: the FROSTTIE RCT.

Background: Tongue-tie can be diagnosed in 3-11% of babies, with some studies reporting almost universal breastfeeding difficulties, and others reporting very few feeding difficulties that relate to the tongue-tie itself, instead noting that incorrect positioning and attachment are the primary reasons behind the observed breastfeeding difficulties and not the tongue-tie itself. The only existing trials of frenotomy are small and underpowered and/or include only very short-term or subjective outcomes.

Objective: To investigate whether frenotomy is clinically and cost-effective to promote continuation of breastfeeding at 3 months in infants with breastfeeding difficulties diagnosed with tongue-tie.

The use of temozolomide in paediatric metastatic phaeochromocytoma/paraganglioma: A case report and literature review.

There is increasing evidence to support the use of temozolomide therapy for the treatment of metastatic phaeochromocytoma/paraganglioma (PPGL) in adults, particularly in patients with mutations. In children however, very little data is available. In this report, we present the case of a 12-year-old female with a SDHB-related metastatic paraganglioma treated with surgery followed by temozolomide therapy.

Retained intravascular catheter fragment at removal of implantable vascular access device: Incidence, risk factors, and outcomes.

Background: Retained intravascular catheter fragments (RICF) are a rare complication of implantable vascular access device (IVAD) removal for which there is limited understanding of aetiology. There is a varied approach to management among the currently published literature.

Aims: The aim of this study was to establish incidence, risk factors, and outcomes for RICF after attempted removal of IVADs.

Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study.

Background: Contemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management.

Methods: A prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012-September 2014.

One-year Outcomes of Congenital Duodenal Obstruction: A Population-based Study.

Objectives: Congenital duodenal obstruction (CDO) occurs in 1.2 per 10,000 live births and is frequently associated with other anomalies, most commonly cardiac. The aim of this study was to report important outcomes to 1 year following surgical repair.

The impact of trisomy 21 on epidemiology, management, and outcomes of congenital duodenal obstruction: a population-based study.

Purpose: Congenital duodenal obstruction (CDO) is associated with trisomy 21 (T21), or Down's syndrome, in around a third of infants. The aim of this study was to explore the impact of T21 on the epidemiology, management, and outcomes of infants with CDO.

Methods: Data were prospectively collected from specialist neonatal surgical centres in the United Kingdom over a 12 month period from March 2016 using established population-based methodology for all babies with CDO.

Congenital duodenal obstruction in the UK: a population-based study.

Objective: Congenital duodenal obstruction (CDO) comprising duodenal atresia or stenosis is a rare congenital anomaly requiring surgical correction in early life. Identification of variation in surgical and postoperative practice in previous studies has been limited by small sample sizes. This study aimed to prospectively estimate the incidence of CDO in the UK, and report current management strategies and short-term outcomes.

One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study.

Objective: To report outcomes to 1 year, in infants born with congenital diaphragmatic hernia (CDH), explore factors associated with infant mortality and examine the relationship between surgical techniques and postoperative morbidity.

Design: Prospective national population cohort study.

Setting: Paediatric surgical centres in the UK and Ireland.

Variations in the Detection of Anorectal Anomalies at Birth among European Cities.

Introduction:  The diagnosis of anorectal malformations (ARMs) is made at birth by perineal examination of the newborn, yet small series reported late diagnosis in almost 13%. No large series to date have looked into the magnitude of missed ARM cases in the neonatal period across Europe. This study aimed to define the rate of missed ARM at birth across four United Kingdom and European Union centers.

What can make things better for parents when babies need abdominal surgery in their first year of life? A qualitative interview study in the UK.

Objectives: To understand the experiences of parents of infants who required surgery early in life. To identify messages and training needs for the extended clinical teams caring for these families-including paediatric surgeons, neonatologists, nurses, obstetricians, midwives and sonographers.

Setting: UK-wide interview study, including England, Wales and Scotland.

One-year outcomes following surgery for necrotising enterocolitis: a UK-wide cohort study.

Objective: The objective was to describe outcomes and investigate factors affecting prognosis at 1 year post intervention for infants with surgical necrotising enterocolitis (NEC).

Design: Using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System, we conducted a prospective, multicentre cohort study of every infant reported to require surgical intervention for NEC in the UK and Ireland between 1 March 2013 and 28 February 2014. Association of independent variables with 1-year mortality was investigated using multivariable logistic regression analysis.

Corrigendum: A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

This corrects the article DOI: 10.1038/srep41149.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14.

Oesophageal atresia with no distal tracheoesophageal fistula: Management and outcomes from a population-based cohort.

Purpose: To describe the incidence and outcomes to one-year in infants born with oesophageal atresia (OA) with no distal tracheoesophageal fistula within a population cohort.

Methods: A subgroup analysis of a prospective multicentre population cohort study was undertaken describing the outcomes of infants with OA and no tracheoesophageal fistula, (type A) and those with only an upper pouch fistula, (type B).

Main Results: Twenty-one of 151 infants in the whole cohort were diagnosed with type A or B oesophageal atresia (14%).

'Less may be best'-Pediatric parapneumonic effusion and empyema management: Lessons from a UK center.

Background: Children with empyema are managed at our center using a protocol-driven clinical care pathway. Chemical fibrinolysis is deployed as first-line management for significant pleural disease. We therefore examined clinical outcome(s) to benchmark standards of care while analyzing disease severity with introduction of the pneumococcal conjugate vaccine.

Laparoscopic assistance for primary transanal pull-through in Hirschsprung's disease: a systematic review and meta-analysis.

Objective: To compare outcomes following totally transanal endorectal pull-through (TTERPT) versus pull-through with any form of laparoscopic assistance (LAPT) for infants with uncomplicated Hirschsprung's disease.

Design: Systematic review and meta-analysis.

Setting: Five hospitals with a paediatric surgical service.

Towards evidence-based emergency medicine: best BETs from the Manchester Royal Infirmary. BET 3: Super calprotectin will not expedite your discharge.

A short-cut review was carried out to establish whether measurement of the plasma calprotectin (S100A8/A9) level can be used to enable safe exclusion of acute appendicitis in children presenting to the emergency department with abdominal pain. Four studies were directly relevant to the question. The author, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses of these papers are shown in table 3.