Scandcleft randomized trials of primary surgery for unilateral cleft lip and palate: Speech proficiency at 10 years of age.

Background & Aim: To assess consonant proficiency and velopharyngeal function in 10-year-old children born with unilateral cleft lip and palate (UCLP) within the Scandcleft project.

Methods & Procedures: Three parallel group, randomized, clinical trials were undertaken as an international multicentre study by nine cleft teams in five countries. Three different surgical protocols for primary palate repair (Arm B-Lip and soft palate closure at 3-4 months, hard palate closure at 36 months, Arm C-Lip closure at 3-4 months, hard and soft palate closure at 12 months, and Arm D-Lip closure at 3-4 months combined with a single-layer closure of the hard palate using a vomer flap, soft palate closure at 12 months) were tested against a common procedure (Arm A-Lip and soft palate closure at 3-4 months followed by hard palate closure at 12 months) in the total cohort of 431 children born with a non-syndromic UCLP.

Benefits of Structured Pediatric to Adult Transition in Inflammatory Bowel Disease: The TRANSIT Observational Study.

Objective: To evaluate the impact of structured transition from pediatric to adult inflammatory bowel disease (IBD) services on objective patient outcomes, including disease flares, admission rates, and healthcare resource use.

Methods: A retrospective observational study in 11 United Kingdom gastroenterology centers. Transition patients attended ≥2 visits to the gastroenterology service with both pediatric and adult personnel jointly present; non-transition patients transferred to adult services without joint visits.

Transition Services for Paediatric Inflammatory Bowel Disease: A Multicentre Study of Practice in the United Kingdom.

Objectives: Patients with paediatric inflammatory bowel disease (IBD) constitute one of the largest cohorts requiring transition from paediatric to adult services. Standardised transition care improves short and long-term patient outcomes. This study aimed to detail the current state of transition services for IBD in the United Kingdom (UK).

Current understanding of febrile seizures and their long-term outcomes.

In this paper we reframe febrile seizures, which are viewed as a symptom of an underlying brain disorder. The general observation is that a small cohort of children will develop febrile seizures (2-5% in the West), while the greater majority will not. This suggests that the brain that generates a seizure, in an often-mild febrile context, differs in some ways from the brain that does not.

Impact of COVID-19 on diagnosis and management of paediatric inflammatory bowel disease during lockdown: a UK nationwide study.

Background: COVID-19 has impacted on healthcare provision. Anecdotally, investigations for children with inflammatory bowel disease (IBD) have been restricted, resulting in diagnosis with no histological confirmation and potential secondary morbidity. In this study, we detail practice across the UK to assess impact on services and document the impact of the pandemic.

Management of spontaneous intracranial hypotension: a series of 31 cases over 15-years with a challenging outlier.

Spontaneous intracranial hypotension (SIH) has been classified as a triad of postural headache, low CSF opening pressure (below 60mmH0) and diffuse pachymeningeal gadolinium enhancement on MRI. SIH is due to a non-iatrogenic defect in the dura somewhere along the neuraxis (usually in the spine). The resultant leak depressurizes the system and undermines the buoyancy-providing quality of CSF.

Corona Virus Disease 2019 and Paediatric Inflammatory Bowel Diseases: Global Experience and Provisional Guidance (March 2020) from the Paediatric IBD Porto Group of European Society of Paediatric Gastroenterology, Hepatology, and Nutrition.

Introduction: With the current coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised about the risk to children with inflammatory bowel diseases (IBD). We aimed to collate global experience and provide provisional guidance for managing paediatric IBD (PIBD) in the era of COVID-19.

Methods: An electronic reporting system of children with IBD infected with SARS-CoV-2 has been circulated among 102 PIBD centres affiliated with the Porto and Interest-group of ESPGHAN.

Scandcleft Project Trial 3: Comparison of Speech Outcomes in Relation to Sequence in 2-Stage Palatal Repair Procedures in 5-Year-Olds With Unilateral Cleft Lip and Palate.

Objective: To compare speech outcome following different sequencing of hard and soft palate closure between arms and centers within trial 3 and compare results to peers without cleft palate.

Design: A prospective randomized clinical trial.

Setting: Two Norwegian and 2 British centers.

Outcome of paediatric acute flaccid myelitis associated with enterovirus D68: a case series.

Enterovirus D68 (EV-D68) is an emerging infection associated with acute flaccid myelitis (AFM). Cases of AFM associated with EV-D68 infection have increased in recent years and the evidence for a causal link is growing. However, our understanding of the epidemiology, clinical features, prognosis, and neurological sequelae of EV-D68 requires ongoing surveillance and investigation.

Management of cervical spine trauma in children.

Purpose: Paediatric cervical spine injuries are fortunately a rare entity. However, they do have the potential for devastating neurological sequelae with lifelong impact on the patient and their family. Thus, management ought to be exceptional from the initial evaluation at the scene of the injury, through to definitive management and rehabilitation.

Long-Term Outcomes Following Achievement of Clinically Inactive Disease in Juvenile Idiopathic Arthritis: The Importance of Definition.

Objective: Potential targets for treat-to-target strategies in juvenile idiopathic arthritis are minimal disease activity (MDA) and clinically inactive disease (CID). We undertook this study to compare short- and long-term outcomes following achievement of MDA and CID on the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10) and following achievement of CID on Wallace et al's preliminary criteria.

Methods: Children recruited to the Childhood Arthritis Prospective Study, a UK multicenter inception cohort, were selected if they were recruited prior to January 2011 and diagnosed as having oligoarthritis or rheumatoid factor-negative or -positive polyarthritis.

How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition.

Objectives: Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at 1 year following presentation.

A Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 1. Planning and management.

Background And Aims: Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 5. Speech outcomes in 5-year-olds - consonant proficiency and errors.

Background And Aim: Normal articulation before school start is a main objective in cleft palate treatment. The aim was to investigate if differences exist in consonant proficiency at age 5 years between children with unilateral cleft lip and palate (UCLP) randomised to different surgical protocols for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 2. Surgical results.

Background: Longstanding uncertainty surrounds the selection of surgical protocols for unilateral cleft lip and palate, and randomised trials have only rarely been performed. The Scandcleft Project consists of three trials commenced in 1997 involving ten centres in Denmark, Finland, Norway, Sweden, and the UK. Three groups of centres tested a newly-defined common technique for palatal repair (Arm A) against their local protocols (Arms B, C, D).

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 4. Speech outcomes in 5-year-olds - velopharyngeal competency and hypernasality.

Background And Aim: Adequate velopharyngeal function and speech are main goals in the treatment of cleft palate. The objective was to investigate if there were differences in velopharyngeal competency (VPC) and hypernasality at age 5 years in children with unilateral cleft lip and palate (UCLP) operated on with different surgical methods for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy.

Contemporary practice of testicular prosthesis insertion.

Objectives: To assess the practice of testicular prosthesis insertion (TPI) related to orchidectomy in one geographical region and to identify the difference in the rates of insertion among different age groups.

Patients And Methods: Males who underwent orchidectomy between 1989 and 2009 were identified from data collected from Scottish Morbidity Records. Patients were classified into six age groups.

Changing indications for paediatric tracheostomy and the role of a multidisciplinary tracheostomy clinic.

Objective: This paper presents our experience of managing children with a tracheostomy in a multidisciplinary team clinic consisting of an ENT consultant, paediatric respiratory consultant, a nurse specialist, and speech and language therapist.

Method: A retrospective case note review was conducted of all children seen in the multidisciplinary team tracheostomy clinic (at a tertiary paediatric hospital) between February 2009 and September 2014.

Results: Ninety-seven patients were examined.

An interventional airway delivery service for congenital high airway obstruction.

Background: Congenital airway obstruction is rare but potentially fatal. We developed a complex airways interventional delivery team to manage such cases. Antenatal imaging detects airway compromise at an early stage and facilitates the planning of delivery procedures ('ex utero intrapartum treatment' and 'operation on placental support') which maintain feto-placental circulation whilst an airway is secured.

Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.

Background: The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease.

Methods: Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed.

Trends in 30-day mortality rate and case mix for paediatric cardiac surgery in the UK between 2000 and 2010.

Objectives: To explore changes over time in the 30-day mortality rate for paediatric cardiac surgery and to understand the role of attendant changes in the case mix.

Methods Setting And Participants: Included were: all mandatory submissions to the National Institute of Cardiovascular Outcomes Research (NICOR) relating to UK cardiac surgery in patients aged <16 years. The χ(2) test for trend was used to retrospectively analyse the proportion of surgical episodes ending in 30-day mortality and with various case mix indicators, in 10 consecutive time periods, from 2000 to 2010.