Prevalence and outcomes of pediatric surgical conditions at Connaught Hospital in Freetown: a retrospective study.

Background: Sub-Saharan Africa experiences a disproportionate amount of pediatric surgical disease, with 80% of children lacking access to timely, affordable, and safe surgical care. This study aims to characterize the burden of disease and outcomes of pediatric surgical conditions at Connaught Hospital, the main pediatric referral hospital in Sierra Leone. Methods: This retrospective and hospital-based study included children up to 15 years old who were operated on between 2015 and June 2016 at Connaught Hospital in Freetown, Sierra Leone. Descriptive and inferential statistics were used to characterize the distribution of disease and compare all variables against age category and mortality. Findings: A total of 215 patients were included in this study of which 72.5% (n=132) were male and 27.5% (n=50) were female. Most of the patients were diagnosed with congenital anomalies (60.9%; n=131). However, infection was the leading diagnosis (60.5%; n=23) among patients aged 5-10 years (n=38). Inguinal hernia was the leading condition (65.0%; n=85) among patients presenting with a congenital anomaly. The condition with the highest mortality was infections (17.0%; n=8), followed by other conditions (9.1%; n=2) and congenital anomalies (3.1%; n=4). Based on the results of this study, over 7000 children with inguinal hernias remain untreated annually in Freetown, Sierra Leone. Conclusion: This study quantifies the burden of surgical disease among children, a foundational step toward the prioritization of pediatric surgical care in national health agendas, the development of evidence-based interventions, and the strategic allocation of resources in Sierra Leone.

Gastroschisis: A Successful, Prospectively Evaluated Treatment Model in a Middle-Income Country.

BACKGROUND: This research adopted a care protocol from high-income countries in a level II/III hospital in a middle-income country to decrease morbidity and mortality associated with gastroschisis. METHODS: We established a multidisciplinary protocol to treat patients with gastroschisis prospectively from November 2012 to November 2018. This included prenatal diagnosis, presence of a neonatologist and pediatric surgeon at birth, and either performing primary closure on the patients with an Apgar score of 8/9, mild serositis, and no breathing difficulty or placing a preformed silo, when unable to fulfill these criteria, under sedation and analgesia (no intubation) in the operating room or at the patients' bedside. The subsequent management took place in the neonatal intensive care unit. The data were analyzed through the Mann-Whitney and Student's t-distribution for the two independent samples; the categorical variables were analyzed through a chi-square distribution or Fisher's exact test. RESULTS: In total, 55 patients were included in the study: 33 patients (60%) were managed with a preformed silo, whereas 22 patients (40%) underwent primary closure. Prenatal diagnosis (P = 0.02), birth at the main hospital (P = 0.02), and the presence of a pediatric surgeon at birth (P = 0.04) were associated with successful primary closure. The primary closure group had fewer fasting days (P < 0.001) and a shorter neonatal intensive care unit length of stay (P = 0.025). The survival rate was 92.7% (51 patients). CONCLUSION: The treatment model modified to fit the means of our hospital proved successful.

A retrospective review of gastroschisis epidemiology and referral patterns in northern Ghana.

PURPOSE: To describe the epidemiology and referral patterns of gastroschisis patients in northern Ghana. METHODS: A hospital-based retrospective review was undertaken at Tamale Teaching Hospital (TTH) Neonatal Intensive Care Unit (NICU) between 2014 and 2019. Data from gastroschisis patients were compared to patients with other surgical diagnoses. Descriptive and inferential statistics were performed with SAS. Referral flow maps were made with ArcGIS. RESULTS: From a total of 360 neonates admitted with surgical conditions, 12 (3%) were diagnosed with gastroschisis. Around 91% (n = 10) of gastroschisis patients were referred from other hospitals, traveling 4 h, on average. Referral patterns showed gastroschisis patients were admitted from three regions, whereas patients with other surgical diagnoses were admitted from eight regions. Only 6% (12/201) of expected gastroschisis cases were reported during the 6-year period in all regions. All gastroschisis deaths occurred within the first week of life. CONCLUSIONS: Improving access to surgical care and reducing neonatal mortality related to gastroschisis in northern Ghana is critical. This study provides a baseline to inform future gastroschisis interventions at TTH. Priority areas may include special management of low birth weight newborns, better referral systems, empowerment of community health workers, and increasing access to timely, affordable, and safe neonatal transport.

Cost-effectiveness of neonatal surgery for congenital anomalies in low-income and middle-income countries: a systematic review protocol.

INTRODUCTION: Congenital anomalies are the fifth leading cause of death in children under 5 years old globally (591 000 deaths reported in 2016). Over 95% of deaths occur in low-income and middle-income countries (LMICs). It is estimated that two-thirds of the congenital anomaly health burden could be averted through surgical intervention and that such interventions can be cost-effective. This systematic review aims to evaluate current evidence regarding the cost-effectiveness of neonatal surgery for congenital anomalies in LMICs. METHODS AND ANALYSIS: A systematic literature review will be conducted in PubMed, MEDLINE, Embase, Cochrane Library, Scielo, Google Scholar, African Journals OnLine and Regional WHO's African Index Medicus databases for articles on the cost-effectiveness of neonatal surgery for congenital anomalies in LMICs. The following search strings will be used: (1) congenital anomalies; (2) LMICs; and (3) cost-effectiveness of surgical interventions. Articles will be uploaded to Covidence software, duplicates removed and the remaining articles screened by two independent reviewers. Cost information for interventions or procedures will be extracted by country and condition. Outcome measurements by reported unit and cost-effectiveness ratios will be extracted. Methodological quality of each article will be assessed using the Drummond checklist for economic evaluations. The Agency for Healthcare Research and Quality's Effective Health Care Program guidance will be followed to assess the grade of the studies. ETHICS AND DISSEMINATION: No ethical approval is required for conducting the systematic review. There will be no direct collection of data from individuals. The finalised article will be published in a scientific journal for dissemination. The protocol has been registered with PROSPERO (International Prospective Register of Systematic Reviews). CONCLUSION: Congenital anomalies form a large component of the global health burden that is amenable to surgical intervention. This study will systematically review the current literature on the cost-effectiveness of neonatal surgery for congenital anomalies in LMICs. PROSPERO REGISTRATION NUMBER: CRD42020172971.

Burden of Neonatal Surgical Conditions in Northern Ghana.

BACKGROUND: Congenital anomalies have risen to become the fifth leading cause of under-five mortality globally. The majority of deaths and disability occur in low- and middle-income countries including Ghana. This 3-year retrospective review aimed to define, for the first time, the characteristics and outcomes of neonatal surgical conditions in northern Ghana. METHODS: A retrospective study was conducted to include all admissions to the Tamale Teaching Hospital (TTH) neonatal intensive care unit (NICU) with surgical conditions between January 2014 and January 2017. Data were collected on demographics, diagnosis and outcomes. Descriptive analysis was performed on all data, and logistic regression was used to predict determinants of neonatal mortality. p < 0.05 was deemed significant. RESULTS: Three hundred and forty-seven neonates were included. Two hundred and sixty-one (75.2%) were aged 7 days or less at presentation, with males (n = 177, 52%) slightly higher than females (n = 165, 48%). The majority were delivered by spontaneous vaginal delivery (n = 247, 88%); 191 (58%) were born in hospital. Congenital anomalies accounted for 302 (87%) of the neonatal surgical cases and 45 (96%) deaths. The most common anomalies were omphalocele (n = 48, 13.8%), imperforate anus (n = 34, 9.8%), intestinal obstruction (n = 29, 8.4%), spina bifida (n = 26, 7.5%) and hydrocephalus (n = 19, 5.5%). The overall mortality rate was 13.5%. Two-thirds of the deaths (n = 30) from congenital anomalies were conditions involving the digestive system with gastroschisis having the highest mortality of 88%. Omphalocele (n = 11, 23.4%), gastroschisis (n = 7, 14.9%) and imperforate anus (n = 6, 12.8%) contributed to the most deaths. On multivariate analysis, low birthweight was significantly associated with mortality (OR 3.59, CI 1.4-9.5, p = 0.009). CONCLUSION: Congenital anomalies are a major global health problem associated with high neonatal mortality in Ghana. The highest burden in terms of both caseload and mortality is attributed to congenital anomalies involving the digestive system, which should be targeted to improve outcomes.

Investigating the use of ultrasonography for the antenatal diagnosis of structural congenital anomalies in low-income and middle-income countries: systematic review protocol.

INTRODUCTION: Congenital anomalies are the fifth leading cause of mortality in children under 5 years globally. The greatest burden is faced by those in developing countries, where over 95% of deaths occur. Many of these deaths may have been preventable through antenatal diagnosis and early intervention. This study aims to conduct a systematic review that investigates the use of antenatal ultrasound to diagnose congenital anomalies and improve the health outcomes of infants in low-income and middle-income countries (LMICs). METHODS AND ANALYSIS: A systematic literature review will be conducted using three search strings: (1) structural congenital anomalies, (2) LMICs and (3) antenatal diagnosis. Four electronic databases will be searched: MEDLINE, Embase, PubMed and the Cochrane Library. Title, abstract and full-text screening will be undertaken in duplicate by two reviewers independently using Covidence. Consensus will be sought from the wider authorship for discrepancies. Data extraction will be undertaken by the principal investigator. The primary analysis will focus on the availability and effectiveness of antenatal ultrasound for structural congenital anomalies. Secondary outcomes will include neonatal morbidity and mortality, termination rates and referral rates for further antenatal care. Descriptive statistics and a narrative synthesis will be included in the final report. The methodological quality of the included studies will be evaluated using the Cochrane-approved Risk of Bias for Non-Randomised Studies of Intervention and Risk of Bias in Randomised Trials V.2.0 tools. ETHICS AND DISSEMINATION: Ethical approval is not required for conducting the systematic review as there will be no direct collection of data from individuals. The results will be submitted for publication in a scientific journal and presented internationally. CONCLUSION: This is the first study, to our knowledge, to systematically review current literature on the use of antenatal ultrasound for the detection of congenital anomalies in LMICs. This is vital to define current practice, highlight global disparities and evaluate effects on health outcomes for infants in low-resource settings. PROSPERO REGISTRATION NUMBER: CRD42019105620.

Management and outcomes of gastrointestinal congenital anomalies in low, middle and high income countries: protocol for a multicentre, international, prospective cohort study.

INTRODUCTION: Congenital anomalies are the fifth leading cause of death in children <5 years of age globally, contributing an estimated half a million deaths per year. Very limited literature exists from low and middle income countries (LMICs) where most of these deaths occur. The Global PaedSurg Research Collaboration aims to undertake the first multicentre, international, prospective cohort study of a selection of common congenital anomalies comparing management and outcomes between low, middle and high income countries (HICs) globally. METHODS AND ANALYSIS: The Global PaedSurg Research Collaboration consists of surgeons, paediatricians, anaesthetists and allied healthcare professionals involved in the surgical care of children globally. Collaborators will prospectively collect observational data on consecutive patients presenting for the first time, with one of seven common congenital anomalies (oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation and Hirschsprung's disease).Patient recruitment will be for a minimum of 1 month from October 2018 to April 2019 with a 30-day post-primary intervention follow-up period. Anonymous data will be collected on patient demographics, clinical status, interventions and outcomes using REDCap. Collaborators will complete a survey regarding the resources and facilities for neonatal and paediatric surgery at their centre.The primary outcome is all-cause in-hospital mortality. Secondary outcomes include the occurrence of post-operative complications. Chi-squared analysis will be used to compare mortality between LMICs and HICs. Multilevel, multivariate logistic regression analysis will be undertaken to identify patient-level and hospital-level factors affecting outcomes with adjustment for confounding factors. ETHICS AND DISSEMINATION: At the host centre, this study is classified as an audit not requiring ethical approval. All participating collaborators have gained local approval in accordance with their institutional ethical regulations. Collaborators will be encouraged to present the results locally, nationally and internationally. The results will be submitted for open access publication in a peer reviewed journal. TRIAL REGISTRATION NUMBER: NCT03666767.

Developing and implementing an interventional bundle to reduce mortality from gastroschisis in low-resource settings.

Background: Gastroschisis is associated with less than 4% mortality in high-income countries and over 90% mortality in many tertiary paediatric surgery centres across sub-Saharan Africa (SSA). The aim of this trial is to develop, implement and prospectively evaluate an interventional bundle to reduce mortality from gastroschisis in seven tertiary paediatric surgery centres across SSA. Methods: A hybrid type-2 effectiveness-implementation, pre-post study design will be utilised. Using current literature an evidence-based, low-technology interventional bundle has been developed. A systematic review, qualitative study and Delphi process will provide further evidence to optimise the interventional bundle and implementation strategy. The interventional bundle has core components, which will remain consistent across all sites, and adaptable components, which will be determined through in-country co-development meetings. Pre- and post-intervention data will be collected on clinical, service delivery and implementation outcomes for 2-years at each site. The primary clinical outcome will be all-cause, in-hospital mortality. Secondary outcomes include the occurrence of a major complication, length of hospital stay and time to full enteral feeds. Service delivery outcomes include time to hospital and primary intervention, and adherence to the pre-hospital and in-hospital protocols.  Implementation outcomes are acceptability, adoption, appropriateness, feasibility, fidelity, coverage, cost and sustainability. Pre- and post-intervention clinical outcomes will be compared using Chi-squared analysis, unpaired t-test and/or Mann-Whitney U test. Time-series analysis will be undertaken using Statistical Process Control to identify significant trends and shifts in outcome overtime. Multivariate logistic regression analysis will be used to identify clinical and implementation factors affecting outcome with adjustment for confounders. Outcome: This will be the first multi-centre interventional study to our knowledge aimed at reducing mortality from gastroschisis in low-resource settings. If successful, detailed evaluation of both the clinical and implementation components of the study will allow sustainability in the study sites and further scale-up. Registration: ClinicalTrials.gov Identifier NCT03724214.

Global Surgery Symposium.

This article describes the Global Surgery Symposium held within the 65th British Association of Paediatric Surgeons (BAPS) Annual Congress in 2018. Global surgery is a rapidly expanding and developing field and is of particular importance in paediatrics since children account for up to 50% of the population in low- and middle-income countries (LMICs). It is estimated that up to a third of childhood deaths in LMICs are the result of a surgical condition, and congenital anomalies have risen to become the 5th leading cause of death in children less than 5-years of age globally. Trainees in high-income countries (HICs) are increasingly interested in global surgery engagement through clinical placements, research, or education, or a combination of these. There is considerable controversy regarding the ethics, practicalities, usefulness, safety, and sustainability of these initiatives. In addition, there is debate as to whether such placements should occur within the paediatric surgery training pathway. LEVEL OF EVIDENCE: 5 (Expert Opinion).

Care of infants with gastroschisis in low-resource settings.

There is great global disparity in the outcome of infants born with gastroschisis. Mortality approaches 100% in many low income countries. Barriers to better outcomes include lack of antenatal diagnosis, deficient pre-hospital care, ineffective neonatal resuscitation and venous access, limited intensive care facilities, poor access to the operating theatre and safe neonatal anesthesia, and lack of neonatal parenteral nutrition. However, lessons can be learned from the evolution in management of gastroschisis in high-income countries, generic efforts to improve neonatal survival in low- and middle-income countries as well as specific gastroschisis management initiatives in low-resource settings. Micro and meso-level interventions include educational outreach programs, and pre and in hospital management protocols that focus on resuscitation and include the delay or avoidance of early neonatal anesthesia by using a preformed silo or equivalent. Furthermore, multidisciplinary team training, nurse empowerment, and the intentional involvement of mothers in monitoring and care provision may contribute to improving survival. Macro level interventions include the incorporation of ultrasound into World Health Organisation antenatal care guidelines to improve antenatal detection and the establishment of the infrastructure to enable parenteral nutrition provision for neonates in low- and middle-income countries. On a global level, gastroschisis has been suggested as a bellwether condition for evaluating access to and outcomes of neonatal surgical care provision.

Improving outcomes for neonates with gastroschisis in low-income and middle-income countries: a systematic review protocol.

INTRODUCTION: There is a significant disparity in outcomes for neonates with gastroschisis in high-income countries (HICs) compared with low-income and middle-income countries (LMICs). Many LMICs report mortality rates between 75% and 100% compared with <4% in HICs. AIM: To undertake a systematic review identifying postnatal interventions associated with improved outcomes for gastroschisis in LMICs. METHODS AND ANALYSIS: Three search strings will be combined: (1) neonates; (2) gastroschisis and other gastrointestinal congenital anomalies requiring similar surgical care; (3) LMICs. Databases to be searched include MEDLINE, EMBASE, Scopus, Web of Science, ProQuest Dissertations and Thesis Global, and the Cochrane Library. Grey literature will be identified through Open-Grey, ClinicalTrials.gov, WHO International Clinical Trials Registry and ISRCTN registry (Springer Nature). Additional studies will be sought from reference lists of included studies. Study screening, selection, data extraction and assessment of methodological quality will be undertaken by two reviewers independently and team consensus sought on discrepancies. The primary outcome of interest is mortality. Secondary outcomes include complications, requirement for ventilation, parenteral nutrition duration and length of hospital stay. Tertiary outcomes include service delivery and implementation outcomes. The methodology of the studies will be appraised. Descriptive statistics and outcomes will be summarised and discussed. ETHICS AND DISSEMINATION: Ethical approval is not required since no new data are being collected. Dissemination will be via open access publication in a peer-reviewed medical journal and distribution among global health, global surgery and children's surgical collaborations and international conferences. CONCLUSION: This study will systematically review literature focused on postnatal interventions to improve outcomes from gastroschisis in LMICs. Findings can be used to help inform quality improvement projects in low-resource settings for patients with gastroschisis. In the first instance, results will be used to inform a Wellcome Trust-funded multicentre clinical interventional study aimed at improving outcomes for gastroschisis across sub-Saharan Africa. PROSPERO REGISTRATION NUMBER: CRD42018095349.

Aiming for a holistic integrated service for men diagnosed with prostate cancer - Definitions of standards and skill sets for nurses and allied healthcare professionals.

PURPOSE: To establish a comprehensive set of recommendations for the service structure and skill set of nurses and allied healthcare professionals in prostate cancer care. METHODS: Using components of formal consensus methodology, a 30-member multidisciplinary panel produced 53 items for discussion relating to the provision of care for prostate cancer patients by specialist nurses and allied healthcare professionals. Items were developed by two rounds of email correspondence in which, first, items were generated and, second, items refined to form the basis of a consensus meeting which constituted the third round of review. The fourth and final round was an email review of the consensus output. RESULTS: The panel agreed on 33 items that were appropriate for recommendations to be made. These items were grouped under categories of "Environment" and "Patient Pathway" and included comments on training, leadership, communication and quality assessment as well as specific items related to prostate diagnosis clinics, radical treatment clinics and follow-up survivor groups. CONCLUSIONS: Specialist nurses and allied healthcare professionals play a vital role alongside urologists and oncologists to provide care to men with prostate cancer and their families. We present a set of standards and consensus recommendations for the roles and skill-set required for these practitioners to provide gold-standard prostate cancer care. These recommendations could form the basis for development of comprehensive integrated prostate cancer pathways in prostate cancer centres as well as providing guidance for any units treating men with prostate cancer.

The management of boys under 3 months of age with an inguinal hernia and ipsilateral palpable undescended testis.

AIMS: The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies. METHODOLOGY: We undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005-2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p<0.05 was considered significant. RESULTS: Survey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic. Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36-65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred. CONCLUSION: This study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age.

Urethral Duplication with Two Hypospadic Meati-An Unusual Variant.

Duplication of the urethra is a rare congenital anomaly, with approximately 300 cases reported in the literature. We report a unique case of this condition in a male infant. This case differs from the classical Effman type II-A2 duplication because of the presence of two hypospadic urethral meati, as opposed to a ventral or dorsal accessory meatus with a normally positioned distal urethra. The patient underwent a single-stage repair consisting of a proximal urethra-urethral anastomosis and distal urethral tubularization at 21 months of age with excellent results in terms of both function and cosmesis.

Differential diagnosis of double site intussusception in childhood: a 15-year-old girl presenting with bowel obstruction.

Abdominal pain in a teenage girl is common; however, thorough history and examination can often distinguish a more rare or sinister diagnosis. We present a case of a 15-year-old girl presenting with abdominal pain, who was subsequently diagnosed with double intussusception secondary to her previously unrecognised Peutz-Jeghers syndrome (PJS).

Epidemiology, management and outcome of gastroschisis in Sub-Saharan Africa: Results of an international survey.

BACKGROUND: The aim was to compare gastroschisis (GS) epidemiology, management and outcome in low-income countries (LIC) in Sub-Saharan Africa (SSA) with middle- (MIC) and high-income countries (HIC). MATERIALS AND METHODS: A 10-question survey was administered at the 2012 Pan-African Paediatric Surgery Association Congress. RESULTS are presented as median (range); differences were analysed using contingency tests. RESULTS: A total of 82 delegates (28 countries [66 institutions]) were divided into LIC (n = 11), MIC (n = 6) and HIC (n = 11). In LIC, there were fewer surgeons and more patients. LIC reported 22 cases (1-184) GS/institution/year, compared to 12 cases (3-23)/institution/year in MICs and 15 cases (1-100)/institution/year in HICs. Antenatal screening was less readily available in LIC. Access to parenteral nutrition and neonatal intensive care in LIC was 36% and 19%, compared to 100% in HIC. Primary closure rates were similar in LIC and HIC at 58% and 54%, respectively; however, the majority of staged closure utilised custom silos in LIC and preformed silos in HIC. In LIC, mortality was reported as >75% by 61% delegates and 50-75% by 33%, compared to <25% by 100% of HIC delegates (P < 0.0001). CONCLUSIONS: Gastroschisis is a problem encountered by surgeons in SSA. Mortality is high and resources in many centres inadequate. We propose the implementation of a combined epidemiological research, service delivery training and resource provision programme to help improve our understanding of GS in SSA whilst attempting to improve outcome.

Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia.

AIM OF THE STUDY: Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH. The aim of this study was to evaluate the predictive value of BOI-d1 in four disparate high volume centers in Europe. METHODS: A retrospective, multicenter study of infants with CDH born between 2000 and 2009 in four European tertiary institutions was conducted. Ethical approval was obtained from institutional review boards. Centers no. 1 and. 4 offered extracorporeal membrane oxygenation (ECMO), whereas center no. 3 offered fetal endoluminal tracheal occlusion (FETO) in fetuses defined as poor prognosis (lung-to-head ratio [LHR]≤ 1.0 and "liver-up" position). Prenatal LHR and perinatal variables, including gestational age, birth weight, defect side, liver position, BOI-d1, and patch requirement, were analyzed. Receiver operating characteristic curves were used to determine cutoff values for continuous variables. Comparison was made between survivors and nonsurvivors using univariate analysis and logistic regression analysis, p<0.05 was considered significant. RESULTS: A total of 235 infants (center no. 1, n=29; no. 2, n=64; no. 3, n=113; and no. 4, n=29) were included. One infant required (2%) ECMO and 66 (28%) had FETO. LHR was available in 83 patients (36%). Overall survival (discharge from hospital) and 28-day survival were 67.6% (n=159) and 72.3% (n=170), respectively. Univariate analysis showed that significant categorical predictors of 28-day survival were liver-down position (p<0.0001), LHR >1 (p=0.003), and primary repair (p=0.02) but not defect side (p=0.83). Area under the receiver operating characteristic (AUROC) curve for continuous variables; gestational age, birth weight, and BOI-d1 were 0.70, 0.68, and 0.88, respectively. AUROC for BOI-d1 (28-day survival) was 0.91 and had sensitivities (73 and 91%) and specificities (92 and 80%) for cutoffs of 40 and 82, respectively. CONCLUSION: This multicenter study showed, that except from the defect side, all the prenatal variables studied have predictive value but the most useful is BOI-d1. This is simple to calculate and represents an excellent marker for lung function and a reliable early postnatal predictor of survival.

Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble.

The 'double bubble' sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. We present a case of cystic biliary atresia presenting with a "double bubble" at 36-weeks gestation. Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy. Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome. This case highlights the need to be mindful of other important anomalies that can give this appearance and that may require early intervention.

The outcome of critically ill neonates undergoing laparotomy for necrotising enterocolitis in the neonatal intensive care unit: a 10-year review.

PURPOSE: To evaluate outcomes in critically ill neonates with necrotising enterocolitis (NEC) undergoing a laparotomy in the neonatal intensive care unit (NICU). METHODS: This is a retrospective review of neonates diagnosed with NEC who underwent a laparotomy on NICU between 2001 and 2011. Demographic, diagnostic, operative and outcome data were analysed. Nonparametric comparison was used. Data are reported as median (range). RESULTS: 221 infants with NEC were referred for surgical evaluation; 182 (82%) underwent surgery; 15 (8%) required a laparotomy on NICU. Five had NEC totalis, 4 multifocal disease and 6 focal disease. Five had an open and close laparotomy, 8 stoma with/without bowel resection and 2 bowel resection and primary anastomosis. Ten (67%) died at a median of 6.5-hours (2-72) postoperatively; 2 died at 72 and 264-days. The 30-day mortality rate was higher (p=0.01) among infants undergoing a laparotomy on NICU (10/15; 67%) than in theatre (54/167; 32%). There was no significant difference in mean Paediatric Index of Mortality 2 Scores between survivors and nonsurvivors (p=0.55). Three (20%) infants remain alive with no or minimal disability at 1.4 (0.5-7.5) years. CONCLUSION: Laparotomy for NEC on NICU is a treatment option for neonates who are too unstable to transfer to theatre. However, with 67% dying within 6.5-hours and a further 13% after months in hospital, we must consider whether surgery is always in their best interests. Development of a prediction model to help distinguish those at highest risk of long-term morbidity and mortality could help with decision making in this difficult situation.