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Insertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center's long-term experience.Retrospective single center review of the period (Jan 2000-Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range).Thirty one fetuses underwent 37 TAS insertions at a 25 (20-30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2-10) weeks following the 1st insertion. Twenty-eight survived to be born. Sixteen (9 male) infants were delivered in our center at 39 (36-41) weeks gestational age and birth weight of 3.1 (2.6-4.2) kg. All infants underwent surgery at 2 (0-535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1-70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4-20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC - 70% predicted).TAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity.

Congenital pulmonary airway malformation (CPAM) has an estimated prevalence in Europe of 1.06/10,000 live births with most being detected using maternal ultrasound screening. Malignant transformation is a possible complication though its prevalence is unknown and previous reports have usually been in older children. We reviewed our experience to identify those CPAM cases associated with malignancy.Single centre retrospective review of all surgically treated children with antenatally-detected CPAM, with detailed review of cases associated with malignancy.210 infants and children underwent resectional surgery for CPAM during the period 1994-2020, with 43(20.5%) undergoing surgery during the neonatal period. Of these, 3 infants, all males, had undergone surgical resection for respiratory distress (at 3, 4 and 8 days of life) with subsequent histological confirmation as Stocker type 1 CPAM with clear foci of mucinous adenocarcinoma. Subsequent genetic analysis showed somatic KRAS (Kirsten Rat Sarcoma Viral Oncogene) mutations in all three cases. No adjuvant treatment was required, and all are asymptomatic and disease-free at most recent follow-up (8 months, 2 and 6 years) CONCLUSIONS: This series highlights a clear association between type 1 CPAM and mucinous adenocarcinoma with KRAS point mutations, suggesting that the process of carcinogenesis has the potential to start in utero. This underlines the importance of discussing the risk of malignancy in prenatal and postnatal counselling.

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).

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p

IntroductionAs childhood mortality from infectious diseases falls across sub-Saharan Africa (SSA), the burden of disease attributed to surgical conditions is increasing. However, limited data exist on paediatric surgical outcomes in SSA. We compared the outcomes of five common paediatric surgical conditions in SSA with published benchmark data from high-income countries (HICs).MethodsA multicentre, international, prospective cohort study was undertaken in hospitals providing paediatric surgical care across SSA. Data were collected on consecutive children (birth to 16 years), presenting with gastroschisis, anorectal malformation, intussusception, appendicitis or inguinal hernia, over a minimum of 1 month, between October 2016 and April 2017. Participating hospitals completed a survey on their resources available for paediatric surgery.The primary outcome was all-cause in-hospital mortality. Mortality in SSA was compared with published benchmark mortality in HICs using χ2 analysis. Generalised linear mixed models were used to identify patient-level and hospital-level factors affecting mortality. A pResults1407 children from 51 hospitals in 19 countries across SSA were studied: 111 with gastroschisis, 188 anorectal malformation, 225 intussusception, 250 appendicitis and 633 inguinal hernia. Mortality was significantly higher in SSA compared with HICs for all conditions: gastroschisis (75.5% vs 2.0%), anorectal malformation (11.2% vs 2.9%), intussusception (9.4% vs 0.2%), appendicitis (0.4% vs 0.0%) and inguinal hernia (0.2% vs 0.0%), respectively. Mortality was 41.9% (112/267) among neonates, 5.0% (20/403) in infants and 1.0% (7/720) in children. Paediatric surgical condition, higher American Society of Anesthesiologists score at primary intervention, and needing/receiving a blood transfusion were significantly associated with mortality on multivariable analysis.ConclusionMortality from common paediatric surgical conditions is unacceptably high in SSA compared with HICs, particularly for neonates. Interventions to reduce mortality should focus on improving resuscitation and timely transfer at the district level, and preoperative resuscitation and perioperative care at paediatric surgical centres.

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30–100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.

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.

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.

Objective This paper describes the fellowship programme of the International Affairs Committee of the British Association of Paediatric Surgeons. Patients and methods The selection of low-income and middle-income country (LMIC) fellows from 2005 to 2016, their funding, experience, and current roles are described. Qualitative and quantitative analysis was performed. Results Thirty-eight trainees from 21 LMIC were awarded fellowships over the 11-year period. Thirty-two have completed the fellowship at time of writing, all are now in consultant positions. Obtaining a visa was the single most cited barrier to starting the fellowship. Twenty completed the questionnaire. Hundred percent felt the fellowship had contributed to personal development and 71% had altered clinical practice subsequent to their experience. Thirty-three percent have gained research opportunities. Conclusion This evaluation supports LMIC-high-income country partnerships and highlights the benefits of fellowships to both the individual surgeon, their department and patient population. Keywords : BAPS, fellowship, low- and middle-income country, partnership

Summary We describe use of an improvised light source to perform cystoscopy and PUV resection while working in a resource poor setting. The light emitted from a mobile telephone LED (iPhone 6) was sufficient to perform the procedure and there was an excellent surgical outcome. We hope that this report may prove to be helpful to colleagues working in similar circumstances with limited resources.

Introduction The lack of suitable veins in children with critical central venous access requirements is a major obstacle to optimal care and is potentially life-threatening. We present outcomes following the use of vein-preserving (VP) surgical techniques, notably the sheath exchange for tunneled lines (SETL). Materials and Methods A retrospective, single observer analysis of a prospectively maintained departmental logbook as well as the medical records of patients. Two broad groups of central line replacements were identified; those inserted following removal of a previous line and a traditional “plastic-free” (PF) period and those exchanged without such an interval. Results Overall, 19 lines were directly exchanged during the study period and compared with 34 inserted after a PF period. Similar catheter life spans and infection rates were demonstrated in each group; 125 (range, 78–173) days in VP exchanges versus 122 (range, 70–175) days in PF replacements ( p = 0.41). Line Sepsis resulting in removal or change of line occurred at 103 (range, 60–147) days in VP group versus 104 (range, 45–164) days in PF ( p = 0.73). Conclusion For children with critical venous access requirements, direct line exchange procedures are a robust and reproducible means of vein preservation. The outcomes compare favorably with those following the more traditional removal, a PF period and reinsertion.

BackgroundThe pre-formed silo (PFS) is increasingly used in the management of gastroschisis, but its benefits remain unclear. We performed a systematic review and meta-analysis of the literature comparing use of a PFS with alternate treatment strategies.MethodsStudies comparing the use of a PFS with alternate strategies were identified and data extracted. The primary outcome measure was length of time on a ventilator. Mean difference (MD) between continuous variables and 95 % confidence intervals were calculated. Risk difference and 95 % CI were determined for dichotomous data.ResultsEighteen studies, including one randomised controlled trial, were included. Treatment strategy and outcome measures reported varied widely. Meta-analysis demonstrated no difference in days of ventilation, but a longer duration of parenteral nutrition (PN) requirement [MD 6.4 days (1.3, 11.5); p = 0.01] in infants who received a PFS. Subgroup analysis of studies reporting routine use of a PFS for all infants demonstrated a significantly shorter duration of ventilation with a PFS [MD 2.2 days (0.5, 3.9); p = 0.01] but no difference in duration of PN requirement. Other outcomes were similar between groups.ConclusionThe quality of evidence comparing PFS with alternate treatment strategies for gastroschisis is poor. Only routine use of PFS is associated with fewer days on a ventilator compared with other strategies. No strong evidence to support a preference for any strategy was demonstrated. Prospective studies are required to investigate the optimum management of gastroschisis. Standardised outcome measures for this population should be established to allow comparison of studies.

To measure and evaluate the effects of tracheal dimensions on survival and ventilation in a large series of infants with congenital diaphragmatic hernia (CDH) treated antenatally with fetal endoluminal tracheal occlusion (FETO).Tracheal dimensions on chest radiograph (CR) were measured by 2 blinded radiologists. Survival, day 1 best oxygenation index and duration of ventilation, continuous positive airway pressure, and hospital stay were recorded. Survivors with a minimum 12-month follow-up were longitudinally compared for incidence of gastroesophageal reflux, chest infections, chest deformities, and hernia recurrence.Seventy infants with CDH (41 who underwent FETO) were treated between 2004 and 2010. Hernia repair was performed in 26 infants without FETO (8 with patch repair) and 35 infants with FETO (26 with patch repair; P = .0015). Infants with FETO had a wider trachea than those without FETO at T1 (P.0001) and between T1 and the carina (P.0001). Tracheal diameter was similar in survivors and nonsurvivors in the FETO group. Tracheal size was not correlated with day 1 best oxygenation index in the FETO group (R2 = 0.17) or the non-FETO group (R2 = 0.07). There were no between-group differences in duration of mechanical ventilation (P = .30), continuous positive airway pressure (P = .20), or hospital stay (P = .30). In the longitudinal study, tracheal widths were larger on the last CR than on preoperative CR in patients without FETO (T1, P = .02; widest point, P = .001; carina, P = .0001), and for patients with FETO at the widest point (P.0001) and at the carina (P.0001), but not at T1 (P = .12). There were no differences in clinical variables between the FETO and non-FETO groups.FETO has a significant impact on tracheal size of infants with CDH; however, tracheal size does not affect survival or the requirement for early respiratory support.

Abdominal pain in children is common and sometimes results from serious pathology amenable to surgical intervention. Though the true cause of many symptoms is not always determinable a diagnostic label is often assigned. The final diagnosis for many children is ‘non-specific abdominal pain’ – an ill-understood group of conditions. Acute appendicitis is common in children and can be life threatening; the history is short in the majority and the diagnosis can be obvious, but when the diagnosis is in doubt, active observation and judicious investigation are appropriate. For children in whom appendicitis is strongly suspected, surgery is the standard treatment though the child with an appendix mass may be managed conservatively. Adequate resuscitation and preoperative broad-spectrum antibiotics are important for successful outcomes. Appendicectomy can be carried out open or laparoscopically. Laparoscopic appendicectomy is the intervention of choice where facilities and expertise are available. Operating late at night is not recommended. Complications of the disease include rupture with localized or generalized peritonitis leading to intra-abdominal and pelvic abscesses, and wound infections. Rarely there may be an iatrogenic injury to the bowel or an appendix stump rupture resulting in a faecal fistula. While acute appendicitis remains a cause of morbidity and occasionally mortality, with timely presentation the majority of children have a good outcome.

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 3 months 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 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 45 weeks (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 3 months of age.

Aims of the Study Laparoscopic simulation has transformed skills acquisition for many procedures. However, realistic non-biological simulators for complex reconstructive surgery are rare. Life-like tactile feedback is particularly difficult to reproduce. Technological innovations may contribute novel solutions to these shortages. We describe a hybrid model, harnessing 3D technology to simulate laparoscopic choledochal surgery for the first time. Methods Digital hepatic anatomy images and standard laparoscopic trainer dimensions were employed to create an entry level laparoscopic choledochal surgery model. The information was fed into a 3D systems project 660pro with visijet pxl core powder to create a free standing liver mould. This included a cuboid portal in which to slot disposable hybrid components representing hepatic and pancreatic ducts and choledochal cyst. The mould was used to create soft silicone replicas with T28 resin and T5 fast catalyst. The model was assessed at a national pediatric surgery training day. Results The 10 delegates that trialed the simulation felt that the tactile likeness was good (5.6/10 +/- 1.71, 10 = like the real thing), was not too complex (6.2/10 +/- 1.35; where 1 = too simple, 10 = too complicated), and generally very useful (7.36/10 +/- 1.57, 10 = invaluable). 100% stated that they felt they could reproduce this in their own centres, and 100% would recommend this simulation to colleagues. Conclusion Though this first phase choledochal cyst excision simulation requires further development, 3D printing provides a useful means of creating specific and detailed simulations for rare and complex operations with huge potential for development.

Aim To report the first European survey on the current management of gastroschisis and ascertain the degree of variability between centers. Methods A 10-question survey was administered at the 2011 European Paediatric Surgeons' Association (EUPSA) Congress. Questionnaires were completed by 205 delegates from 39 countries. A total of 21 responses (10%) were incomplete and voided. The remaining 184 were divided on the basis of following region of practice: Western Europe (WE, n = 102), Eastern Europe (EE, n = 59), and non-European countries (n = 23). Differences between WE and EE were analyzed using contingency tests. p Results A total of 15% WE and 2% EE responders work in centers where antenatal magnetic resonance imaging scans are routinely used. Nonplanned delivery is the most popular approach (WE 46%, EE 58%). Primary closure is the preferred choice (WE 92%, EE 86%), and it is achieved by operative fascial closure in the majority (WE 80%, EE 75%) rather than by Bianchi technique (WE 20%, EE 25%). Staged reduction and closure is less popular (WE 8%, EE 14%), and it is achieved by custom-made silo (WE 25%, EE 12.5%), preformed silo (PFS) followed by surgical closure (WE 63%, EE 75%), or PFS followed by sutureless closure (WE 12%, EE 12.5%). Objection to PFS in WE is mainly related to surgeons' lack of confidence in the technique (40%), whereas in EE it is due to unavailability and high cost (62%, p = 0.01). In case of associated intestinal atresia, immediate resection and anastomosis is preferred by 60% of WE surgeons versus 35% of EE surgeons (p = 0.03), who equally favor primary closure and delayed surgery (33%). Nutrition is preferably delivered by peripheral long line in WE (64%) and by central line inserted in the first week of life in EE (62%, p = 0.003). Conclusions Primary fascial closure is currently the preferred method of gastroschisis closure across Europe. Aspects of care such as strategy for intestinal atresia and delivery of parenteral nutrition differ significantly between WE and EE. Economic considerations appear to influence management strategy particularly in EE. A Europe-wide audit appears warranted to identify whether this survey reflects actual practice.

Among children presenting to hospital with abdominal pain, acute appendicitis is common. The history is short in the majority. Where the diagnosis is in doubt, active observation and judicious investigation are appropriate. For confirmed cases and children in whom appendicitis is strongly suspected, surgery is the standard treatment though the child with an appendix mass can initially be managed conservatively. Adequate resuscitation and broad-spectrum antibiotics are important pre-operatively to ensure successful outcomes. Appendicectomy can be carried out open or laparoscopically. Laparoscopic appendicectomy is becoming the intervention of choice where facilities and expertise are available. Operating late at night and in the early hours of the morning is not recommended. Complications of the disease include rupture with localized or generalized peritonitis. Surgical and post-operative complications include injury to the bowel or the ureter which are both uncommon, but abscess formation is frequently seen. While acute appendicitis remains a cause of morbidity and occasionally mortality, with timely presentation the majority of children have a good outcome.

Introduction: In mother-infant pairs experiencing breastfeeding difficulties, frenulotomy for tongue-tie may improve breastfeeding. We tested the hypothesis that those experiencing nipple pain are most likely to benefit from the procedure in a prospective cohort study. Materials and Methods: Mother-infant pairs attending a dedicated clinic for the assessment and treatment of tongue-tie completed a standardised, structured symptom questionnaire. Three months later outcome was assessed by questionnaire. Multivariate logistic regression analysis was used to determine preoperative predictors of successful outcome. Results: Sixty-two infants < 90 days old underwent frenulotomy and completed follow-up. At presentation, 52 mothers (84%) reported nipple pain, and 32 mothers (52 %) nipple trauma. Three months after frenulotomy, 78 % of respondents were still breastfeeding. Feed lengths (mean reduction: 17 mins ; p

Background: Antenatally detected asymptomatic congenital cystic lung lesions may be managed conservatively or by surgical resection. We undertook a systematic review and meta-analysis to quantify the risks of elective surgery, emergency surgery, and observation. Methods: All series published between 1996 and 2008, where the postnatal management of congenital cystic lung lesions was described, were reviewed. A meta-analysis was performed to determine whether elective or emergency surgery was associated with a higher risk of adverse outcomes. Results: There were 41 reports describing 1070 patients (of whom 79% were antenatally detected). Five hundred five neonates survived without surgery into infancy, of whom only 16 (3.2%) became symptomatic. For all ages, elective surgery was associated with significantly less complications than emergency surgery. The risk ratio was 2.8 (95% confidence interval, 1.4-5.5; P < .005) when comparing complications after elective surgery with emergency surgery. Conclusions: The risk of asymptomatic cases developing symptoms is small. However, elective surgery is associated with a better outcome than emergency surgery. If elective surgery is undertaken, it should be performed before 10 months. Although no prognostic indicators have so far been identified in the literature, a conservative approach may be appropriate for small lesions.

Introduction A parent is often present during anesthetic induction of their child. Some ask to see surgery. We sought views regarding the prospect of a parent in theater during surgery. Methods A questionnaire survey of parents, theater staff, and surgeons was used. Visual analog scales were also used. A standard error of the mean was calculated for each parameter. Statistical analysis was by Student's t test. Comparisons were made between groups, and a P value of less than .05 was considered significant. Results Three hundred seven respondents—204 parents, 75 theater personnel, and 28 surgeons. Parents favored the option to be present in theater. Across groups, support declined with intensity of intervention; minor surgery under local anesthetic, parental score of 8.43, declining to 6.5 for minor elective surgery under general anesthetic, and 5.1 for emergency surgery. There were also declines for theater personnel (2.7, 1.1, and 0.9) and surgeons (4.29, 1.5, and 0.6). Scores for theater personnel and surgeons were significantly lower than the parents ( P Conclusion This study confirms a desire by parents to be present in theater during surgery on their child but demonstrates the concerns of professionals. We propose a randomized study to test the hypothesis that having a parent in theater has measurable benefits.

Background The “clip and drop” (CD) has been proposed as a useful strategy in the management of severe multifocal necrotizing enterocolitis (NEC). There is little published data on clinical outcomes after this intervention. We report a 2-center experience with this technique. Methods A retrospective review of infants who underwent CD between 1998 and 2006 at 2 tertiary pediatric surgery centers. Data recorded included intestinal resections, interval between laparotomies, anastomoses at subsequent surgery, time to full feeds, and complications including mortality. Data are reported as median with ranges. Results Thirteen infants (7 male, 6 female) with a birth weight of 811 (514-2110) g underwent CD of up to 5 bowel segments. In 8 of 9 early survivors, all CD segments were viable. Six patients (46%) were alive at 29 (9-96) months. Survivors underwent 4 (3-4) laparotomies and 4 (2-6) bowel anastomoses and had intestinal continuity restored at 67 (51-162) days. Conclusions With multiple interventions, half the infants in this high-risk group survived and achieved full enteral feeds. The CD is a valuable technique in a selected group of infants with fulminant NEC.

Background: Gastroschisis (GS) is a congenital full-thickness defect of the anterior abdominal wall, whose incidence is increasing. Traditional postnatal management options include primary reduction and closure under general anaesthetic or operative silo construction for defects judged to be unsuitable for immediate repair. The cot-side application of the preformed silo (PFS) with delayed abdominal wall closure has recently been advocated as the management method of choice for infants with GS. We report a novel trainer designed to facilitate acquisition and refinement of the skills to apply the PFS. Materials and Methods: A model of an infant with GS was constructed to allow application of a PFS. Each step of the clinical application of a PFS could be simulated. Paediatric surgeons at a regional meeting participated in evaluating the model. This cohort was surveyed with regards to previous clinical experience applying the PFS, invited to apply the silo on the model and then resurveyed with regard to the technique, ease of the application of the PFS on the model, its robustness and potential use as a training tool. Results: Seventeen paediatric surgeons completed the surveys and applications of the PFS on the model. Under supervision, each step of the procedure was completed by all participants. Feedback was enthusiastic and positive and participants judged the model to be realistic and potentially very useful as a training tool (median score 8 out of 10). Conclusions: We have developed and evaluated a reproducible, low-cost model of an infant with GS. This ex vivo trainer may be a useful adjunct in the acquisition and refinement of the skills of surgeons who manage GS using a PFS.

Purpose We report our experience of the management of arterial occlusion in the newborn. Methods A case note review was carried out after ethical approval. Doppler ultrasonography confirmed the occlusion. Thrombolysis was the primary intervention. Surgery was used selectively. A good outcome was one without tissue loss or functional impairment or minimal tissue loss without functional impairment. Data are presented as medians with ranges. Results Ten patients (9 male; median gestational age, 35.5 weeks [range, 28-39 weeks]) presented on day 1 (range, 1-8 days). Initial management included systemic tissue plasminogen activator (8 patients) and surgery (2 infants in whom thrombolysis was contraindicated). Improvement was noted in 7 of 8 infants treated medically and in both who underwent surgery. Three infants had significant tissue loss. Outcome at 29 months (range, 1.3-95.4 months) was good in the remaining 7. Conclusions A multidisciplinary approach, thrombolysis and selective surgery achieved tissue preservation and function in the majority while minimizing complications. Early referral to centers with multidisciplinary teams is recommended.

Background The management of children with main pancreatic duct injuries is controversial. We report a series of patients with pancreatic trauma who were treated using minimally invasive techniques. Methods Retrospective review of children with pancreatic trauma treated at a UK tertiary referral institution between 1999 and 2004. Results Fifteen children (11 boys) were admitted with pancreatic trauma. Twelve (80%) were less than 50th centile for body weight. Contrast-enhanced computed tomography (CT) scans were used to define organ injury, supplemented by magnetic resonance cholangiopancreatography (MRCP) in 7. Twelve (80%) underwent diagnostic endoscopic retrograde cholangiopancreatography (ERCP) with a median time after injury of 11 (range, 6-29) days. The degree of pancreatic injury was defined by ERCP and CT/MRCP as grade II (n = 2), grade III (n = 4), grade IV (n = 9) (American Association for the Surgery of Trauma grades). Nine children had a transductal pancreatic stent inserted endoscopically. Computed tomography/ultrasound-guided drainage was performed in 4 children for acute fluid collections. Two children later underwent endoscopic cyst-gastrostomy, one of whom later required conversion to an open cyst-gastrostomy. Conclusion Body habitus may predispose to pancreatic duct trauma. Contrast-enhanced CT scan (and MRCP) should dictate the need for ERCP. Transductal pancreatic stenting allows internal drainage of peripancreatic collections and may reestablish duct continuity, although a proportion still requires percutaneous or endoscopic cyst-gastrostomy drainage. Open surgery for pancreatic trauma should now be an exception.

Introduction Economic disadvantage may adversely influence the outcomes of infants with gastroschisis (GS). Gastroschisis International (GiT) is a network of seven paediatric surgical centres, spanning two continents, evaluating GS treatment and outcomes. Material and methods A 2-year retrospective review of GS infants at GiT centres. Primary outcome was mortality. Sites were classified into high, middle and low income country (HIC, MIC, and LIC). MIC and LIC were sometimes combined for analysis (LMIC). Disability adjusted life years (DALYs) were calculated and centres with the highest mortality underwent a needs assessment. Results Mortality was higher in the LICs and LMICs: 100% in Uganda and Cote d'Ivoire, 75% in Nigeria and 60% in Malawi. 29% and 0% mortality was reported in South Africa and the UK, respectively. Septicaemia was the commonest cause of death. Averted and non-avertable DALYs were nil in Uganda and Cote d'Ivoire (no survivors). In the UK (100% survival) averted DALYs (met need) was highest, representing death and disability prevented by surgical intervention. Performance improvement measures were agreed: a prospectively maintained GS register; clarification of the key team members of a GS team and management pathway. Conclusions We propose the use of GS as a bellwether condition for assessing institutional capacity to deliver newborn surgical care. Early access to care, efficient multidisciplinary team working, appropriate resuscitation, avoidance of abdominal compartment syndrome, stabilization prior to formal closure and proactive nutritional interventions may reduce GS-associated burden of disease in low resource settings.

Background Gastroschisis associated intestinal dysmotility (GAID) is poorly understood. Animal experiments suggest that interstitial cells of Cajal (ICC), play an important role. Methods Infants with gastroschisis (GS) and GAID (time to full feed > 42 days) were selected. Age matched GS and control (NEC, ileal atresia, malrotation, and volvulus) samples from primary (T1) and secondary (T2) time points underwent standard histopathology and immunohistochemistry for identification of ICC, followed by evaluation of ICC numbers, distribution, morphology, relation to ganglion cells, and myenteric plexus architecture. Groups were compared using parametric and non-parametric tests. Main Results Twelve patients had samples available for histopathological evaluation. GAID patients had a significantly lower total number of ICC than controls (3 vs. 8, P < 0.0029). ICC number at T1 was 2.5 vs. 6, P=0.0629 and significantly lower at T2. (3.5 vs. 11, P=0.0124). GAID patients did not show a significant increase of ICC from T1 to T2. Controls showed a significant increase of ICC over time (6 vs. 11, P=0.0408). Conclusion Intestinal samples from infants with GAID who underwent stoma formation demonstrated fewer ICC than controls. There was no improvement or cell recovery during the study period. The ability to modulate ICC may have significant implications for the management of GAID.

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

The aim of this study was to evaluate whether a training programme involving an assessment exercise performed on a laparoscopic trainer model leads to an improvement in the acquisition of laparoscopic skills in surgical trainees. Subjects were recruited from a cross-section of surgical trainees at the Great Ormond Street Hospital, Department of Surgery and the Institute of Child Health. All subjects completed both a baseline laparoscopic surgical skills questionnaire and three exercises on a new laparoscopic trainer model. Thirteen subjects completed both the baseline questionnaire and all three assessment exercises. These subjects exhibited a wide range of previous experience in laparoscopic surgery. Sixty-nine percent of subjects showed a significant improvement in the assessment exercise score with training (ANOVA; P = 0.01). Sixty-two percent of subjects showed a greater improvement between exercises 2 and 3 than between exercises 1 and 2. The difference in score between exercises 1 and 2 was not statistically significant (P = 0.597), whereas the difference in score between both exercises 2 and 3 and exercises 1 and 3 was statistically significant (P = 0.018 and P = 0.005, respectively). The double glove training model is thus a simple, inexpensive, and easily reproducible tool that elicits a significant improvement in laparoscopic surgical skills in surgical trainees with a broad range of previous laparoscopic experience. It can therefore be used as part of a training programme to facilitate the acquisition of laparoscopic skills in a paediatric surgery setting.

The aim of this study was to evaluate whether an assessment exercise performed on a laparoscopic trainer model reliably reflects previous laparoscopic experience and can therefore be used to accurately assess laparoscopic skills in surgical trainees. Subjects were recruited from a cross-section of surgical trainees and students at the Great Ormond Street Hospital for Children and the Institute of Child Health. Subjects were required to complete a baseline laparoscopic surgical skills questionnaire and an exercise on a new laparoscopic trainer model. Nine subjects completed both the baseline questionnaire and the exercise. These subjects exhibited a wide range of previous experience in laparoscopic surgery. Subjects with higher self-assessment scores had the lowest exercise scores (i.e. better scores; P=0.003). Furthermore, the exercise score was strongly negatively correlated with the baseline number of training modalities received (P=0.007) and the laparoscopic experience score (P=0.027). The assessment exercise on a novel laparoscopic trainer was capable of differentiating between subjects with little laparoscopic experience and those with more extensive previous laparoscopic training. The correlation between the exercise score and measured baseline variables suggests that the scoring system used in this model is sensitive and specific to measuring skills relevant to laparoscopic surgery.

Aims Sacrococcygeal teratomas (SCTs) are the commonest neonatal tumors with an incidence of approximately 1:30,000. There are few large single-center series and even fewer describing both their antenatal and postnatal course. We report the outcome of all fetuses investigated at a tertiary fetal medicine center with this diagnosis. Method Demographic details were obtained from a prospectively maintained database. Patient records were examined for additional data including antenatal and postnatal interventions. Data were described as median (range). Results Forty-one SCTs were diagnosed antenatally during the period 1993 to 2004. Twelve were excluded from subsequent analysis (single antenatal visit or attending for second opinion [n = 6] and termination of pregnancy [n = 6]). Twelve underwent fetal intervention (laser vessel ablation [n = 4], alcohol sclerosis [n = 3], cyst drainage [n = 2], amniodrainage [n = 2], vesicoamniotic shunt [n = 1]) for fetal hydrops and polyhydramnios to aid in delivery and to prevent obstructive uropathy developing in the fetus. Of these, 3 died in utero and 9 survived to be born (median gestational age, 33 weeks [27-37 weeks]). A further 3 died in the neonatal period. There are 6 long-term survivors (50%) from this group. Seventeen infants, without intervention, were born at median gestational age 38 weeks (26-40 weeks). One infant with severe cardiac anomalies died on the day of birth. All surviving infants had definitive excisional surgery at a median of 2 days (1-16 days). Current median follow-up of survivors is 39 months (8-86 months). There have been no recurrences. One child has mild constipation, and 3 are awaiting cosmetic revision of their scars. Conclusions The overall survival of antenatally diagnosed SCT is approximately 77%, with the development of hydrops and others requiring in utero intervention carrying a poor prognosis. Otherwise, the outcome after surgical excision is excellent.

Static electricity within sterile packaging may result in bacterial contamination of central venous catheters (CVCs) prior to insertion. To prevent this, some surgeons inject saline into the pack before opening it. This trial was designed to determine the effect of this procedure. A double blind randomised controlled trial of 47 CVCs comparing injection of 2 ml of sterile saline into the pack prior to opening with no injection was performed. Five centimetre lengths cut from the tip of the catheter before and after subcutaneous tunnelling were sent for microbiological culture. Eight catheters (17%) showed evidence of bacterial contamination prior to insertion into the vein. Two (4.2%) were contaminated prior to tunnelling and seven (14.9%) afterwards. One catheter was contaminated before and after tunnelling. All but one of the contaminating bacteria were coagulase negative staphylococci. There was no significant difference in the contamination rate between catheters from packs that had been injected (5/25) and those that had not (3/22), P = 0.56. Just under one-fifth of the catheters were contaminated with bacteria prior to insertion into the vein but this was not influenced by prior injection of saline into the pack. We conclude that there is no evidence to support the practice of injecting the catheter pack prior to opening.

BACKGROUND: Intralesional injection of OK-432 has been proposed as an effective treatment of lymphangioma. The aim of this study was to review our experience with OK-432 injection of lymphangioma and to identify factors associated with successful outcome. METHODS: We made a case note review of 19 children who received OK-432 injection. Median duration of follow-up was 17 months. RESULTS: Lesions were diagnosed antenatally in 4 children, at birth in 4 children, and between 1 month and 11 years in the remainder. Anatomic locations were head/neck in 14, axilla in 1, and multiple locations in 4. Median number of injections per child was 2 (range, 1 to 5). Disappearance of the lesion was achieved after OK-432 injection in 2 patients (11%) and a marked reduction in 5 (26%); all these lesions were in the head and neck. Lesions larger than 5 cm and those outside the head and neck region did not respond well to OK-432 injection. Fourteen children (74%) required surgical excision after injection. Complications of OK-432 injection included partial tracheal obstruction, fever, local inflammatory response, and abscess formation. CONCLUSIONS: OK-432 injection was effective in approximately one third of children with lymphangioma. Lesions outside the head and neck and those larger than 5 cm are unlikely to respond to this therapy. Injection of lymphangioma surrounding the airways may be hazardous

Single port surgery (SPS) has been demonstrated to have some advantages over conventional laparoscopy. However, currently available port sizes may limit the application in younger children or those with a small umbilicus. Moreover, the consultant learning curve required to master single port surgery may have a negative impact on surgical training. We report the first series of children who were treated with a reduced incision technique for appendicectomy using flexible-tip laparo-endoscopic surgery (FLES). FLES was set up using one 11-mm and 2×5-mm bladeless ports (Ethicon XCEL™) via umbilical and low left iliac fossa incisions. A 10-mm flexible-tip laparo-endoscope was utilized. Tip angulation ensured visibility while minimising instrument clashing. A database of children undergoing FLES was kept prospectively. Demographic and peri-operative information and complications were recorded. Data are presented as medians with ranges. Between March and June 2010, 5 children (4 females) aged 9 (4–13) years underwent FLES for right iliac fossa pain. 2 procedures were performed by the admitting consultant, 3 by a supervised inexperienced laparoscopic trainee. 4 children had acute appendicitis including 1 with an inflammatory mass. Another had a haemorrhagic ovarian cyst. Appendicectomy was performed in all. The duration of surgery was 104 (93–130) min, and postoperative hospital stay was 2 (1–6) days. At 7 (5–8) months’ follow-up no complications have been recorded. At follow-up, the cosmetic results were judged to be excellent in all by the children, their parents and the reviewing surgeon. FLES is an alternative to standard laparoscopy and SPS in children, and be performed effectively and safely by junior trainees. Cosmetic results are excellent. It may represent a bridge technology, particularly for younger children, until single port products and techniques more suitable for appendicectomy in this age group are available. Finally, flexible-tip technology may play a useful role as SPS evolves.

Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD).A retrospective review of gastroschisis (1992-2012) linked fetal/neonatal data at 2 tertiary referral centers was performed. Primary outcomes were ENT and length of hospital stay (LOS). Data (median [range]) were analyzed using parametric/non-parametric tests, positive/negative predictive values, and regression analysis.Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p0.0001) and LOS (p0.0001) were reduced with increasing gestational age. IABD persisted to last scan in 92 patients, 68 (74%) simple (intact/uncompromised bowel), 24 (26%) complex. IABD or EABD diameter in complex patients was not significantly greater than simple gastroschisis. Combined IABD/EABD was present in 22 patients (14 simple, 8 complex). When present at30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from30weeks.Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis.