Acquired colonic atresia in children: a report of three cases and review of the literature.

We describe cases of three infants who developed acquired colonic atresia presumed secondary to significant systemic cardiovascular compromise and in the absence of necrotizing enterocolitis. An acquired colonic atresia may present as feed intolerance and should be investigated with a lower gastrointestinal contrast study. We would also recommend routine lower gastrointestinal contrast study prior to stoma closure in an infant with history of significant cardiovascular compromise, even in the absence of significant widespread colonic inflammation such as necrotizing enterocolitis.

Methods of identifying surgical Necrotizing Enterocolitis-a systematic review and meta-analysis.

BACKGROUND: Current data suggests potential benefit of earlier surgery for necrotizing enterocolitis (NEC) however this requires accurate prognostication early in the disease course. This study aims to identify and determine the effectiveness of previously reported methods or tests for the identification of surgical NEC. METHODS: Systematic review and meta-analysis with registration on PROSPERO including articles describing a method of identifying surgical NEC. Outcomes of interest were effectiveness and repeatability of index test. RESULTS: Of the 190 full-text articles screened, 90 studies were included which contained 114 methods of identifying surgical NEC in 9546 infants. Of these methods, 44 were a scoring system, 37 a single biomarker, 24 an imaging method, and 9 an invasive method. Sensitivity and specificity ranged from 12.8-100% to 13-100%, respectively. Some methods (9.6%) provided insufficient methods for repeatability within clinical practice or research. Meta-analyses were possible for only 2 methods, the metabolic derangement 7 score and abdominal ultrasound. CONCLUSIONS: A range of methods for identifying surgical NEC have been identified with varying overall performance and uncertainties about reproducibility and superiority of any method. External validation in large multicentre datasets should allow direct comparison of accuracy and prospective study should evaluate impact on clinical outcomes. IMPACT: Earlier identification of need for surgery in necrotizing enterocolitis (NEC) has the potential to improve the unfavourable outcomes in this condition. As such, many methods have been developed and reported to allow earlier identification of surgical NEC. This study is the first synthesis of the literature which identifies previously reported methods and the effectiveness of these. Many methods, including scoring systems and biomarkers, appear effective for prognostication in NEC and external validation is now required in multicentre datasets prior to clinical utility.

Development Methodology, Availability, and Implementation of Core Outcome Sets in Pediatric Surgery.

Core outcome sets (COSs) provide a mechanism to guide researchers and clinicians when deciding which outcomes to report in research related to a specific clinical condition or intervention. The intention behind creating a COS for a specific condition is to improve the reporting of important and meaningful outcomes, thus enhancing the relevance of research. Additionally, a COS helps facilitate comparison of outcomes between different clinical studies and reduces research waste. In this paper, we discuss the availability of COSs in the field of pediatric general surgery. We provide an overview of the methodologies used to develop a COS, including common pitfalls, and finally, we discuss COS uptake and implementation. An understanding of all these aspects is important for researchers considering developing a new COS and for those reading research where a COS has been developed or used within a study. Failure to adequately appreciate the nuances of COS development, in particular, risks fundamental flaws that may jeopardize COS validity and subsequently hinder COS uptake and implementation.

Recent advances in our understanding of NEC diagnosis, prognosis and surgical approach.

Necrotising enterocolitis (NEC) remains a devasting condition that has seen limited improvement in outcomes in recent years. The incidence of the disease is increasing as more extremely premature infants survive. NEC is responsible for 1 in 10 neonatal deaths and up to 61% of survivors have significant neurodevelopmental delay. The aim of this review is to highlight recent advances in diagnosis, prognosis and surgical approach in this condition. Many recent studies have reported novel methods of diagnosis of NEC with the aim of earlier and more accurate identification. These include imaging and machine learning techniques. Prognostication of NEC is particularly important to allow earlier escalation of therapy. Around 25% of infants with NEC will require surgery and recent data has shown that time from disease onset to surgery is greater in infants whose indication for surgery is failed medical management, rather than pneumoperitoneum. This indication was also associated with worse outcomes compared to pneumoperitoneum. Ongoing research has highlighted several new methods of disease prognostication which includes differentiating surgical from medical NEC. Finally, recent randomised controlled trials in surgical technique are discussed along with the implications of these for practice. Further, high quality research utilising multi-centre collaborations and high fidelity data from electronic patient records is needed to address the issues discussed and ultimately improve outcomes in NEC.

Outcomes 1 year after non-operative management of uncomplicated appendicitis in children: Children with AppendicitiS during the CoronAvirus panDEmic (CASCADE) study.

BACKGROUND: A major shift in treatment of appendicitis occurred early in the SARS-CoV-2 pandemic with non-operative management used commonly outside research protocols and in units with limited previous experience. This study aims to compare real-world outcomes of surgery versus non-operative management of uncomplicated appendicitis in children with 1-year follow-up. METHOD: A prospective multicentre observational study of children treated for uncomplicated appendicitis at 74 hospitals in the UK and Ireland from 1 April to 31 July 2020 was performed. Propensity-score matched analysis was conducted using age, sex, C-reactive protein at diagnosis and duration of symptoms as covariates. Primary outcomes were success of non-operative management defined as achieving 1-year follow-up without undergoing appendicectomy due to recurrent appendicitis or ongoing symptoms, and occurrence of any predefined complication (intra-abdominal collection, wound infection, bowel obstruction or reintervention). RESULTS: Of 1464 children with presumed uncomplicated appendicitis, 1027 (70.2 per cent) underwent surgery and 437 (29.9 per cent) underwent non-operative management. Ninety-four children (21.5 per cent) treated by initial non-operative management required appendicectomy during the index hospital admission while recurrent appendicitis after discharge occurred in 25 (10.4 per cent) children within 1 year. The overall success rate of non-operative management at 1 year was 63.1 per cent (95 per cent c.i. 58.0 to 68.3 per cent). For propensity-score matched analyses, 688 children undergoing surgery and 307 undergoing non-operative management were included. Any predefined complication occurred in 50 (7.3 per cent) children undergoing surgery and in four (1.3 per cent) children undergoing non-operative management (OR 5.9 (95 per cent c.i. 2.1 to 16.6)) in the propensity-score matched cohort. There was no mortality or stoma formation. CONCLUSION: Non-operative management is a safe and valid alternative to appendicectomy in children with uncomplicated appendicitis.

Delayed finding of congenital duodenal obstruction following congenital diaphragmatic hernia repair.

Congenital diaphragmatic hernia (CDH) is encountered in just under 1 in 6000 live births, while congenital duodenal obstruction is seen once every 8000 live births. These congenital anomalies have only been reported together as part of the VACTERL syndrome and therefore in isolation represent an incredibly rare occurrence. This is a case report of a girl born at 34 weeks gestation who had an antenatal diagnosis of left CDH. Five days following operative repair of this, there was extensive pneumoperitoneum and pneumothorax. Upper gastrointestinal contrast study showed a perforation of the duodenum and at laparotomy, a duodenal web was found in the fourth part of the duodenum with perforation immediately proximal. Following duodenoduodenostomy, the postoperative recovery was good. She achieved full enteral feeds and was discharged home. This case highlights the importance of considering rare associations if postoperative recovery is not as expected to prevent delay in undergoing definitive treatment.

The Influence of the Introduction of Biologic Agents on Surgical Intervention in Paediatric Inflammatory Bowel Disease.

OBJECTIVES: To determine how the use of biological therapy is associated with surgical intervention for paediatric inflammatory bowel disease (PIBD) at a population level. METHODS: Hospital Episode Statistics data were obtained for all admissions within England (1997-2015), in children aged 0-18 years, with an ICD-10 code for diagnosis of Crohn disease (CD), ulcerative colitis (UC), or inflammatory bowel disease-unclassified (IBD-U). Office of Population Censuses and Surveys Classification of Surgical Operations and Procedures codes for major surgical resection associated with PIBD and for biological therapy were also obtained. Data are presented as median values (interquartile range). RESULTS: In total, 22,645 children had a diagnosis of PIBD of which 13,722 (61%) had CD, 7604 (34%) had UC, and 1319 (5.8%) cases had IBD-U. Biological therapy was used in 4054 (17.9%) cases. Surgical resection was undertaken in 3212 (14%) cases, more commonly for CD than UC (17.5 vs 10.3%, P < 0.0001). Time from diagnosis to major surgical resection was 8.3 (1.2-28.2) months in CD and 8.2 (0.8-21.3) months in UC. As the time-frame of the dataset progressed, there was a decreased rate of surgical intervention ( P = 0.04) and an increased use of biological therapy ( P < 0.0001). Additionally, the number of new diagnoses of PIBD increased. CONCLUSIONS: The introduction of biologic agents has been associated with a reduction in cases undergoing surgery in children with a known diagnosis of PIBD. As time progresses we will be able to determine whether biological therapies prevent the need for surgery altogether or just delay this until adulthood.

Impact of the COVID-19 pandemic on management and outcomes of children with appendicitis: The Children with AppendicitiS during the CoronAvirus panDEmic (CASCADE) study.

AIM: To report the impact of the SARS-CoV-2 pandemic on management and outcomes of paediatric appendicitis in the UK and Ireland. METHODS: Prospective, multicentre observational cohort study at general surgical and specialist paediatric surgical centres in the United Kingdom and Ireland from 1st April to 31st July 2020. Primary outcome was treatment strategy used for acute appendicitis. RESULTS: This study includes 2002 children treated for acute appendicitis of a median age of 10 (range 1-15) years and 605 children from a similar data set pre pandemic from 2017. In the pandemic cohort 560/2002(28%) were initially treated non operatively of whom 125/560(22%) proceeded to appendicectomy within initial hospital admission. Non operative treatment wasn't used in the pre pandemic cohort. Diagnostic imaging use was greater during the pandemic compared to pre pandemic (54vs31%; p < 0.00001) but overall use of laparoscopy was similar during both time periods (62.4vs66.6%). Hospital readmission rate was lower (8.7vs13.9%; p = 0.0002) during the pandemic than pre pandemic and Re-intervention rate was similar (2.9vs2.6%;p = 0.42). In cases treated operatively negative appendicectomy rate was lower during the pandemic than pre pandemic (4.4vs15.4%; p =0.0001), and during the pandemic was amongst the lowest ever reported in the UK. CONCLUSION: COVID-19 has had a significant impact on the management of children with appendicitis in the UK and Ireland. The rate of imaging and the use of non operative management increased, whilst the negative appendicectomy rate reduced. Overall, patient outcomes have not been adversely impacted by change in management during the pandemic. CONCLUSION: Level I. TYPE OF STUDY: Prognosis study.

Surgical necrotizing enterocolitis: Association between surgical indication, timing, and outcomes.

BACKGROUND/PURPOSE: Despite improvements in neonatal care the outcomes of Necrotizing Enterocolitis (NEC) remain unchanged over previous decades. The study aims to explore whether different indications for surgical intervention in NEC are associated with timing of surgery and outcomes. METHODS: Population-based, prospective, observational study of all 27 paediatric surgical centres in the United Kingdom and Ireland identified using the British Association of Paediatric Surgeons Congenital Anomalies Surveillance System from 1st February 2013 to 28th February 2014. Infants were included if they had NEC and underwent first surgical intervention within 7 days of diagnosis. Primary outcomes were death, parenteral nutrition requirement or a composite outcome of death or PN requirement at 28 days post surgery. RESULTS: There were 133 infants meeting inclusion criteria. Indications for surgery were bowel perforation (n = 67), suspected necrotic bowel without bowel perforation and not deemed to have failed medical management (n = 20), those who had failed medical management (n = 42) and a palpable mass without any other indication (n = 4). Failed medical treatment as an indication for surgery was associated with an increased time to surgery of 30.28 (95% CI 13.46-47.10) hours from those whose indication was perforation and was also the strongest predictor of PN requirement or death at 28 days post-surgery (OR 4.54 [1.59-13.0]). CONCLUSIONS: Failed medical treatment as an indication for surgery for NEC is associated with poor outcome. Earlier intervention in these infants represents a potential opportunity to improve outcomes in this population.

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. METHODS: This was a prospective population-based study using the British Association of Paediatric Surgeons Congenital Anomaly Surveillance System. Cases were identified at specialist pediatric surgical centres in the United Kingdom during a 12-month period starting in March 2016. Outcomes were recorded at 1 year following surgical repair. RESULTS: There were 100 infants with possible follow-up at 1 year and follow-up was achieved in 80 of these (80%) of whom 76 were alive at 1 year. The remainder had been discharged home, although one remained on parenteral nutrition. Five (6.1%) infants underwent repeat surgery for reasons related to CDO and overall 23 (23%) experienced at least 1 central venous catheter-related complication within 1 year. Overall mortality either before repair or within 1 year following surgical repair was 8.4% (95% CI 2.5%-14.4%), no deaths were related to CDO. CONCLUSIONS: One year outcomes for CDO are generally very good with poor outcomes typically related to comorbidities. These data are useful for national benchmarking and parental counselling.

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. Infants with T21 were compared to those without any chromosomal anomaly. RESULTS: Of 102 infants with CDO that underwent operative repair, T21 was present in 33 [32% (95% CI 23-41%)] babies. Cardiac anomalies were more common in those with T21 compared to those without a chromosomal anomaly (91 vs 17%, p < 0.001), whereas associated gastrointestinal anomalies were less common in infants with T21 (3 vs 12%, p = 0.03). Surgical management was not influenced by T21. Time to achieve full enteral feed, need for repeat related surgery, and mortality were similar between groups. Infants with T21 had a longer median initial inpatient stay (23 vs 16.5 days, p = 0.02). CONCLUSIONS: Infants with T21 have a higher incidence of cardiac anomalies and a longer initial inpatient stay; however, it does not change CDO management or outcomes. This information is important for prenatal and postnatal counselling of parents of infants with CDO and T21.

Management and early outcomes of children with appendicitis in the UK and Ireland during the COVID-19 pandemic: a survey of surgeons and observational study.

OBJECTIVES: Acute appendicitis is the most common surgical condition in children. In the UK, appendicectomy is the most common treatment with non-operative management unusual. Due to concerns about the risk of SARS-CoV-2 transmission during surgical procedures, surgeons were advised to consider non-operative treatment and avoid laparoscopy where possible. This study aims to report management and outcomes, to date, of children with appendicitis in the UK and Ireland during the COVID-19 pandemic. DESIGN: Survey of consultant surgeons who treat children with appendicitis that informed a prospective multicentre observational cohort study. SETTING: Data were collected from centres in the UK and Ireland for cases admitted between 1 April and 31 May 2020 (first 2 months of the COVID-19 pandemic) at both general surgical and specialist paediatric surgical centres. PARTICIPANTS: The study cohort includes 838 children with a clinical and/or radiological diagnosis of acute appendicitis of which 527 (63%) were male. MAIN OUTCOMES MEASURED: Primary outcome was treatment strategy used for acute appendicitis. Other outcomes reported include change in treatment strategy over time, use of diagnostic imaging and important patient outcomes to 30 days following hospital admission. RESULTS: From very early in the pandemic surgeons experienced a change in their management of children with appendicitis and almost all surgeons who responded to the survey anticipated further changes during the pandemic. Overall, 326/838 (39%) were initially treated non-operatively of whom 81/326 (25%) proceeded to appendicectomy within the initial hospital admission. Of cases treated initially surgically 243/512 (48%) were performed laparoscopically. Diagnostic imaging was used in 445/838 (53%) children. Cases treated non-operatively had a shorter hospital stay than those treated surgically but hospital readmissions within 30 days were similar between groups. In cases treated surgically the negative appendicectomy rate was 4.5%. There was a trend towards increased use of surgical treatment and from open to laparoscopic appendicectomy as the pandemic progressed. CONCLUSION: Non-operative treatment of appendicitis has been widely used for the first time in children in the UK and Ireland and is safe and effective in selected patients. Overall patient outcomes do not appear to have been adversely impacted by change in management during the pandemic thus far.

Surgical management of genitourinary lichen sclerosus et atrophicus in boys in England: A 10-year review of practices and outcomes.

INTRODUCTION: Circumcision has long been the mainstay of management for genitourinary lichen sclerosus et atrophicus (LS); however, there has been growing interest in surgical techniques that preserve the foreskin. OBJECTIVE: The aim of this study was to assess population-based surgical management of LS in England and determine surgical outcomes. STUDY DESIGN: Cases of LS treated in English NHS trusts (2002-2011) were extracted from the Hospital Episode Statistics (HES) Database. Cases were identified by both an ICD-10 code for LS and either an OPCS4.6 code for circumcision or preputioplasty (with/without injection of steroid). Subsequent admissions were analysed for related complications/procedures. Data are presented as median (interquartile range) unless otherwise stated. RESULTS: 7893 patients had surgery for LS, of whom 7567 (95.8%) underwent circumcision (Table). Primary preputioplasty was performed in 326 (4.1%) in 44/130 centres; of these 151/326 had concomitant injection of steroid. Age at surgical intervention was 9 (6-11) years. There were no postoperative bleeds following preputioplasty. Of those treated with preputioplasty, 74 (22%) had subsequent circumcision at a median of 677 (277-1203) days post operation. Concomitant steroid injection reduced the risk of subsequent circumcision (21/151 (14%) vs. 53/175 (30%), p < 0.001). More children underwent a second operative procedure following preputioplasty than those having had a primary circumcision (27.9% vs. 7.9%, p < 0.001). CONCLUSION: Although circumcision is the predominant treatment for LS, these data suggest that preputioplasty is a valid option in management, albeit with a higher re-intervention rate. Selection bias may play a role and a randomized controlled trial is needed. Preputioplasty combined with steroid injection appears to reduce the chance of completion circumcision.

A Case of Embryonal Rhabdomyosarcoma Presenting as a Lobulated Protrusion From the Urethral Meatus at Birth.

This is the first reported case of rhabdomyosarcoma presenting as a mass protruding from the urethral meatus present at birth. A male neonate was transferred to a tertiary pediatric urology center on day 4 of life where the mass was surgically excised. Histology confirmed an embryonal rhabdomyosarcoma and chemotherapy commenced. The patient completed chemotherapy at the age of 8 months. The child is now 3.5 years old and well with no recurrence of disease.

Isolation of Enteric Nervous System Progenitor Cells from the Aganglionic Gut of Patients with Hirschsprung's Disease.

Enteric nervous system progenitor cells isolated from postnatal human gut and cultured as neurospheres can then be transplanted into aganglionic gut to restore normal patterns of contractility. These progenitor cells may be of future use to treat patients with Hirschprung's disease, a congenital condition characterized by hindgut dysmotility due to the lack of enteric nervous system ganglia. Here we demonstrate that progenitor cells can also be isolated from aganglionic gut removed during corrective surgery for Hirschsprung's disease. Although the enteric nervous system marker calretinin is not expressed in the aganglionic gut region, de novo expression is initiated in cultured neurosphere cells isolated from aganglionic Hirschsprung bowel. Furthermore, expression of the neural markers NOS, VIP and GFAP also increased during culture of aganglionic gut neurospheres which we show can be transplantation into cultured embryonic mouse gut explants to restore a normal frequency of contractility. To determine the origin of the progenitor cells in aganglionic region, we used fluorescence-activated cell sorting to demonstrate that only p75-positive neural crest-derived cells present in the thickened nerve trunks characteristic of the aganglionic region of Hirschsprung gut gave rise to neurons in culture. The derivation of enteric nervous system progenitors in the aganglionic gut region of Hirschprung's patients not only means that this tissue is a potential source of cells for future autologous transplantation, but it also raises the possibility of inducing the differentiation of these endogenous cells in situ to compensate for the aganglionosis.

Features of educational interventions that lead to compliance with hand hygiene in healthcare professionals within a hospital care setting. A BEME systematic review: BEME Guide No. 22.

BACKGROUND: In the United Kingdom, there are approximately 300,000 healthcare-associated infections (HCAI) annually, costing an estimated £1 billion. Up to 30% of all HCAI are potentially preventable by better application of knowledge and adherence to infection prevention procedures. Implementation of Department of Health guidelines through educational interventions has resulted in significant and sustained improvements in hand hygiene compliance and reductions in HCAI. AIM: To determine the features of structured educational interventions that impact on compliance with hand hygiene in healthcare professionals within a hospital care setting. METHODS: Sixteen electronic databases were searched. Outcomes were assessed using Kirkpatrick's hierarchy and included changes in hand hygiene compliance of healthcare professionals, in service delivery and in the clinical welfare of patients involved. RESULTS: A total of 8845 articles were reviewed, of which 30 articles met the inclusion criteria. Delivery of education was separated into six groups. CONCLUSIONS: It was not possible to identify individual features of educational interventions due to each study reporting multicomponent interventions. However, multiple, continuous interventions were better than single interventions in terms of eliciting and sustaining behaviour change. Data were not available to determine the time, nature and type of booster sessions with feedback needed for a permanent change in compliance.