Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

Background: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis.

Dynamic Compression Therapy for Pectus Carinatum in Children and Adolescents: Factors for Success.

Background: Pectus carinatum (PC) is a congenital chest wall deformity. In childhood, it is increasingly treated with dynamic compression therapy. Factors for success for dynamic brace therapy are relatively unknown.

Determining the Correct Resection Level in Patients with Hirschsprung Disease Using Contrast Enema and Full Thickness Biopsies: Can the Diagnostic Accuracy be Improved by Examining Submucosal Nerve Fiber Thickness?

Background: Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter.

Methods: We retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection.

Familial Experience With Hirschsprung's Disease Improves the Patient's Ability to Cope.

Introduction: Familial occurrence of Hirschsprung's disease may have a positive effect on patients' ability to cope with the disease. The aim was to compare long-term bowel function and generic quality of life between patients with familial and non-familial Hirschsprung's disease.

Methods: This was a nationwide, cross-sectional study in which we included all 830 Hirschsprung patients of 8 years and older who had undergone surgery between 1957 and 2015.

Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease.

Objectives: Knowledge on long-term outcomes in patients with Hirschsprung disease is progressing. Nevertheless, differences in outcomes according to aganglionic lengths are unclear. We compared long-term bowel function and generic quality of life in Hirschsprung patients with total colonic or long-segment versus rectosigmoid aganglionosis.

Hypothermic Machine Perfusion as a National Standard Preservation Method for Deceased Donor Kidneys.

Background: Recently, continuous nonoxygenated hypothermic machine perfusion (HMP) has been implemented as standard preservation method for deceased donor kidneys in the Netherlands. This study was designed to assess the effect of the implementation of HMP on early outcomes after transplantation.

Methods: Kidneys donated in the Netherlands in 2016 and 2017 were intended to be preserved by HMP.

Comparison of intra- and extra-corporeal laparoscopic hernia repair in children: A systematic review and pooled data-analysis.

Background: Laparoscopic surgery is increasingly used to repair paediatric inguinal hernias and can be divided into intra- or extra-corporeal closing techniques. No statement regarding the superiority of one of the two techniques can be made. This study aims to provide evidence supporting the superiority of intra- or extra-corporeal suturing technique.

International Core Outcome Set for Acute Simple Appendicitis in Children: Results of a Systematic Review, Delphi Study, and Focus Croups With Young People.

Objective: To develop an international core outcome set (COS), a minimal collection of outcomes that should be measured and reported in all future clinical trials evaluating treatments of acute simple appendicitis in children.

Summary Of Background Data: A previous systematic review identified 115 outcomes in 60 trials and systematic reviews evaluating treatments for children with appendicitis, suggesting the need for a COS.

Methods: The development process consisted of 4 phases: (1) an updated systematic review identifying all previously reported outcomes, (2) a 2-stage international Delphi study in which parents with their children and surgeons rated these outcomes for inclusion in the COS, (3) focus groups with young people to identify missing outcomes, and (4) international expert meetings to ratify the final COS.

Left Ventricular Geometrical Changes in Severely Obese Adolescents: Prevalence, Determinants, and Clinical Implications.

Left ventricular hypertrophy (LVH) is independently associated with a higher risk of cardiovascular morbidity and mortality in adults. Adiposity is a risk factor for LVH, independent of blood pressure. Potential causes of this nonhemodynamic pathogenesis identified in adults include adverse body fat distribution, insulin resistance, dyslipidemia, and obstructive sleep apnea syndrome (OSA).

Obstructive sleep apnea in obese adolescents referred for bariatric surgery: association with metabolic and cardiovascular variables.

Background: obstructive sleep apnea syndrome (OSA) is a well-described disease entity in adults, with a higher prevalence in severely obese individuals, while at the same time associated with several comorbidities independently of BMI. Literature regarding OSA in severely obese adolescents is qualitatively and quantitatively limited, possibly resulting in suboptimal diagnosis and treatment.

Methods: polysomnographic, demographic, anthropometric, and comorbidity-related data were prospectively collected in 56 adolescents with morbid obesity refractory to conservative treatment who presented for surgical therapy.

Sternocostal instability after Ravitch repair in adolescents; 3 case-reports and a review of surgical techniques in the literature.

Unlabelled: Ravitch repair is a common surgical procedure to correct chest wall deformities. In this procedure, a subperichondreal cartilage resection of the deformed parasternal cartilage, and if necessary a repositioning of the sternum, is performed. Insufficient regeneration of the resected cartilage may result in sternocostal instability or even floating sternum.

Correlation of Preoperative State Anxiety and Pain Six Weeks After Surgical Correction of Pectus Excavatum.

Background: Pain is a major concern in the early postoperative phase after correction of pectus excavatum. Most studies only focus on pain management in the first days after surgery and describe methods to alleviate the pain immediately postoperatively. The severity of postoperative pain may be influenced by anxiety.

Impact of Cold Ischemia Time on Outcomes of Deceased Donor Kidney Transplantation: An Analysis of a National Registry.

Background: Cold ischemia time (CIT) is known to impact kidney graft survival rates. We compare the impact of CIT on graft failure and mortality in circulatory death versus brain death donor kidneys and how it relates to donor age.

Methods: We used the prospective Dutch Organ Transplantation Registry to include 2153 adult recipients of brain death (n = 1266) and circulatory death (n = 887) donor kidneys after static cold storage from transplants performed between 2005 and 2012.

Establishing a core outcome set for treatment of uncomplicated appendicitis in children: study protocol for an international Delphi survey.

Introduction: Appendicitis is a global disease affecting roughly 1 in every 12 people in the world, with the highest incidence between ages 10 and 19 years. To date, a wide variety of health outcomes have been reported in randomised controlled trials and meta-analyses evaluating treatments for appendicitis. This is especially the case in studies comparing non-operative treatment with operative treatment.

Abdominal Wall Pain or Irritable Bowel Syndrome: Validation of a Pediatric Questionnaire.

Objectives: A questionnaire study demonstrated that some adult patients who were diagnosed with irritable bowel syndrome (IBS) were in fact having an abdominal wall pain syndrome, such as anterior cutaneous nerve entrapment syndrome (ACNES). The aim of the present study was to investigate whether a pediatric version of this questionnaire was useful in diagnosing abdominal wall pain syndromes in children with chronic abdominal pain (CAP).

Methods: An 18-item questionnaire was tested in 3 groups of children with CAP: group 1, children who underwent surgery for ACNES (n = 42); group 2, children who were found to have ACNES after an outpatient analysis (n = 57); and group 3, children diagnosed with IBS (n = 53).

Clinical recovery in children with uncomplicated appendicitis undergoing non-operative treatment: secondary analysis of a prospective cohort study.

Non-operative treatment of uncomplicated appendicitis in children is gaining ground. Pending definitive evidence regarding its effectiveness, there is a call to evaluate clinical recovery after non-operative treatment. In this study, we analyzed data collected during initial admission of a multicenter prospective cohort in which children, 7-17 year, were treated non-operatively for uncomplicated appendicitis.

Early cost-utility estimation of the surgical correction of pectus excavatum with the Nuss bar.

Objectives: The surgical correction of pectus excavatum (PE) with a Nuss bar provides satisfactory outcomes, but its cost-effectiveness is yet unproven. We prospectively analysed early outcomes and costs for Nuss bar placement.

Methods: Fifty-four patients aged 16 years or older (6 females and 48 males; mean age, 17.

Euthanasia through living organ donation: Ethical, legal, and medical challenges.

Euthanasia is categorically prohibited in almost all countries throughout the world. In Belgium and the Netherlands, combining euthanasia and subsequent organ donation in a so-called donation after circulatory-death (DCD) procedure is feasible on legal and medical grounds, and is increasingly gaining social and ethical acceptance. However, heart transplantation after DCD is currently not performed in Belgium and the Netherlands after euthanasia due to concerns surrounding the prolonged warm ischemia time associated with DCD and its effect on subsequent heart function.

Diagnostic characteristics of anterior cutaneous nerve entrapment syndrome in childhood.

Unlabelled: Some children suffering from chronic abdominal pain may have an abdominal wall entity such as anterior cutaneous nerve entrapment syndrome. This syndrome is largely suspected on a combination of findings at history and physical examination. The aim is to obtain clues in history and physical examinations in a selected population of children with anterior cutaneous nerve entrapment syndrome.

Initial non-operative management of uncomplicated appendicitis in children: a protocol for a multicentre randomised controlled trial (APAC trial).

Introduction: Based on epidemiological, immunological and pathology data, the idea that appendicitis is not necessarily a progressive disease is gaining ground. Two types are distinguished: simple and complicated appendicitis. Non-operative treatment (NOT) of children with simple appendicitis has been investigated in several small studies.

Anterior rectus sheath blocks in children with abdominal wall pain due to anterior cutaneous nerve entrapment syndrome: a prospective case series of 85 children.

Background: Chronic abdominal pain in children may be caused by the anterior cutaneous nerve entrapment syndrome. Local nerve blocks are recommended as an initial treatment in adults. Evidence on effectiveness and safety of such a treatment in children is lacking.