The role of surgery in relieving calcified shunt site-related pain in patients with functioning VP shunt.

Purpose: Shunt calcification is a known late sequela of ventriculoperitoneal (VP) shunt insertion and is associated with shunt malfunction. However, in some patients, while shunt functionality is preserved despite calcification of the catheters, they experience nociceptive symptoms. In this paper, the authors present their surgical experience in managing patients with a functional VP shunt and experiencing pain secondary to shunt calcification.

Pediatric endoscopy training across Europe: a survey of the ESPGHAN National Societies Network 2016-2019.

The ability to perform endoscopy procedures safely and effectively is a key aspect of quality clinical care in Pediatric Gastroenterology, Hepatology and Nutrition (PGHN). The aim of this survey, which was part of a global survey on PGHN training in Europe, was to assess endoscopy training opportunities provided across Europe. Responses to standardized questions related to endoscopy training were collected from training centers across Europe through the presidents/representatives of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition National Societies from June 2016 to December 2019.

Cardiac care of children with dystrophinopathy and females carrying DMD-gene variations.

Objective: We provide succinct, evidence-based and/or consensus-based best practice guidance for the cardiac care of children living with Duchenne muscular dystrophy (DMD) as well as recommendations for screening and management of female carriers of mutations in the -gene.

Methods: Initiated by an expert working group of UK-based cardiologists, neuromuscular clinicians and DMD-patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK-cardiologists, consensus was reached on these best-practice recommendations for cardiac care in DMD.

Atlantoaxial limited dorsal myeloschisis: A report of two cases and review of literature.

Introduction: Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism that is characterised by a distinctive fibroneural stalk connecting the spinal cord to the overlying skin lesion. The skin lesions associated with LDM can appear benign clinically and careful evaluation with an MRI scan is essential for diagnosing LDM and to differentiate this entity from other forms of spinal dysraphism and benign causes of skin lesions.

Research Question: There is a lack of reported atlantoaxial LDM in the literature.

Renal outcome and plasma methylmalonic acid levels after isolated or combined liver or kidney transplantation in patients with methylmalonic acidemia: A multicenter analysis.

Background: Methylmalonic acidemia (MMAemia) is characterized by accumulation of methylmalonic acid (MMA) in all body tissues. To minimize disease-related complications, isolated kidney (KTx), liver (LTx) or combined liver-kidney transplantation (LKTx) have been suggested. However, the impact of these different transplant strategies on outcome are unclear.

A nationwide evaluation of bevacizumab-based treatments in pediatric low-grade glioma in the UK: Safety, efficacy, visual morbidity, and outcomes.

Background: Bevacizumab is increasingly used in children with pediatric low-grade glioma (PLGG) despite limited evidence. A nationwide UK service evaluation was conducted to provide larger cohort "real life" safety and efficacy data including functional visual outcomes.

Methods: Children receiving bevacizumab-based treatments (BBT) for PLGG (2009-2020) from 11 centers were included.

2022 European Thyroid Association Guidelines for the management of pediatric thyroid nodules and differentiated thyroid carcinoma.

At present, no European recommendations for the management of pediatric thyroid nodules and differentiated thyroid carcinoma (DTC) exist. Differences in clinical, molecular, and pathological characteristics between pediatric and adult DTC emphasize the need for specific recommendations for the pediatric population. An expert panel was instituted by the executive committee of the European Thyroid Association including an international community of experts from a variety of disciplines including pediatric and adult endocrinology, pathology, endocrine surgery, nuclear medicine, clinical genetics, and oncology.

measurement of ciliary beat frequency in 92 children with recurrent respiratory tract problems.

Background: Chronic or recurrent mucoid respiratory tract symptoms may be difficult to diagnose.

Method: Ninety-two children with chronic respiratory symptoms were divided into 4 groups: 18 children with refractory asthma, 10 with bronchiectasis without dextrocardia, 18 with dextrocardia and 46 with recurrent respiratory tract infections. Except for five neonates, cytology samples were taken under general anaesthesia.

ERF-related craniosynostosis and surgical management in the paediatric cohort.

Introduction: ERF mutation is one of the most recently identified genetic aberrations associated with syndromic craniosynostosis. Data on the pattern of craniosynostosis, surgical management of ERF-related craniosynostosis and outcomes is limited. We report on our single-centre experience in paediatric cohort of patients with syndromic craniosynostosis secondary to ERF mutation.

Surgical sequence, timing and volume, and variation in dento-facial outcome, speech and secondary surgery in children with unilateral cleft lip and palate: The Cleft Care UK Study.

Objectives: To estimate both the association of surgical variables in complete unilateral cleft lip and palate (cUCLP) in the UK with outcomes at age 5 years, and the association of secondary speech surgery, volume of surgery, and surgeon with the same outcomes.

Setting And Sample Population: The Cleft Care UK study, a cross-sectional study of 268 5-year-olds, born from 2005 to 2007, with cUCLP.

Materials And Methods: Information on surgical variables was extracted from a standardized questionnaire.

A Brief Clinical Study: The Use of a Custom Guide for Scapula Free Flap Harvest and Mandibular Reconstruction.

Head and neck cancer can leave patients with significant defects requiring major reconstruction. The scapula free flap remains a relatively underused flap choice. This article examines the novel use of a custom guide for a scapula free flap in mandibular reconstruction following resection.

Educational Review: Error traps in anesthesia for pediatric liver transplantation.

Anesthetic and surgical techniques for the liver transplantation have progressed considerably over the past sixty years; however, this procedure is still fraught with substantial morbidity. To increase the safety culture associated with the liver transplantation, we detail nine error traps associated with anesthesia for pediatric liver transplantation. These potential pitfalls are divided into the operative phases: pre-operative preparation (Failure to have a dedicated anesthesia team for pediatric liver transplantation); pre-anhepatic (Failure to prepare for massive blood loss, Failure to monitor for coagulation abnormalities); anhepatic including reperfusion (Failure to prepare for clamping of the inferior vena cava, Failure to recognize metabolic changes, Failure to maintain homeostasis for reperfusion, Failure to prepare for Post-reperfusion syndrome); and post-anhepatic (Failure to optimize liver perfusion, Failure to maintain hemostatic balance).

Heterozygous mutation causing hyperparathyroidism in children and adolescents: a report of 2 cases.

Objectives: Primary hyperparathyroidism (PHPT), whilst common in elderly populations, is rare in adolescents. Hereditary cases make up less than 10% of patients with PH. We report two patients with mutation presenting in early adolescence.

Dietary Liberalization in Tetrahydrobiopterin-Treated PKU Patients: Does It Improve Outcomes?

Purpose: this systematic review aimed to assess the effects of dietary liberalization following tetrahydrobiopterin (BH) treatment on anthropometric measurements, nutritional biomarkers, quality of life, bone density, mental health and psychosocial functioning, and burden of care in PKU patients.

Methods: the PubMed, Cochrane, and Embase databases were searched on 7 April 2022. We included studies that reported on the aforementioned domains before and after dietary liberalization as a result of BH treatment in PKU patients.

Educational impact of a novel cleft palate surgical simulator: Improvement in surgical trainees' knowledge and confidence.

Background: Trainees' experience in cleft surgery is limited due to the high-risk nature of the surgery and centralization of cleft care. Simulation training allows trainees to learn complex surgical tasks whilst ensuring patient safety. Existing cleft surgical simulators are over-simplified or prohibitively expensive.

Early mobilisation and rehabilitation in the PICU: a UK survey.

Objective: To understand the context and professional perspectives of delivering early rehabilitation and mobilisation (ERM) within UK paediatric intensive care units (PICUs).

Design: A web-based survey administered from May 2019 to August 2019.

Setting: UK PICUs.

Amplitude-reduction alert criteria and intervention during complex paediatric cervical spine surgery.

Objective: To determine the utility of widely used intraoperative neuromonitoring (IONM) alert criteria and intervention for predicting postoperative outcome following paediatric spinal surgery.

Methods: Retrospective analysis of somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) in consecutive cervical spine fixations. An intervention protocol followed amplitude-reductions in SSEPs (≥50 %) and/or MEPs (≥80 %).

Qualitative study exploring the well-being experiences of paediatric critical care consultants working in the UK during the COVID-19 pandemic.

Objectives: The aim of this study was to examine the well-being experiences of consultants working in paediatric critical care (PCC) settings in the UK during the COVID-19 pandemic.

Design: Qualitative design using individual interviews and thematic analysis.

Setting: PCC.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers.

Microsurgery in pediatric upper limb reconstructions: An overview.

The use of microsurgery has spread during the last decades, making resolvable many complex defects considered hitherto inapproachable. Although the small vessel diameter in children was initially considered a technical limitation, the increase in microsurgical expertise over the past three decades allowed us to manage many pediatric conditions by means of free tissue transfers. Pediatric microsurgery has been shown to be feasible, gaining a prominent place in the treatment of children affected by limb malformations, tumors, nerve injuries, and post-traumatic defects.