The long-term outcome following thoraco-amniotic shunting for congenital lung malformations.

AIM OF THE STUDY: 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. METHODS: 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). MAIN RESULTS: 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). CONCLUSIONS: 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.

Neonatal congenital pulmonary airway malformation associated with mucinous adenocarcinoma and KRAS mutations.

AIM OF THE STUDY: 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. METHODS: Single centre retrospective review of all surgically treated children with antenatally-detected CPAM, with detailed review of cases associated with malignancy. MAIN RESULTS: 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.

Major abdominal wall defects in the low- and middle-income setting: current status and priorities.

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.

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.

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.

Gastroschisis: Bellwether for neonatal surgery capacity in low resource settings?

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.

3D printing to simulate laparoscopic choledochal surgery.

AIMS OF THE STUDY: Laparoscopic simulation has transformed skills acquisition for many procedures. However, realistic nonbiological 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 mold. This included a cuboid portal in which to slot disposable hybrid components representing hepatic and pancreatic ducts and choledochal cyst. The mold 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 centers, 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.

The role of preformed silos in the management of infants with gastroschisis: a systematic review and meta-analysis.

BACKGROUND: The 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. METHODS: Studies 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. RESULTS: Eighteen 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. CONCLUSION: The 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.

Interstitial cells of Cajal are decreased in patients with gastroschisis associated intestinal dysmotility.

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 >42days) 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 nonparametric tests. MAIN RESULTS: Twelve patients had samples available for histopathological evaluation. GAID patients had a significantly lower total number of ICCs 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.

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.

Is early delivery beneficial in gastroschisis?

PURPOSE: Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD). METHODS: 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. RESULTS: Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p<0.0001) and LOS (p<0.0001) 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 at <30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from <30weeks. CONCLUSION: Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at <37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis.

Tracheomegaly in infants with severe congenital diaphragmatic hernia treated with fetal endoluminal tracheal occlusion.

OBJECTIVE: 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). STUDY DESIGN: 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. RESULTS: 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. CONCLUSION: 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.

Paediatric laparoscopic hernia repair: Ex vivo skills in the reduced training era.

INTRODUCTION: Changes to surgical working hours have resulted in shorter training times and fewer learning opportunities. Tools that develop surgical skills ex-vivo are of particular interest in this era. Laparoscopic skills are regarded as essential by many for modern paediatric surgery practice. Several generic skills models have been reported and validated. However, there is limited evidence regarding the role of procedure specific models. Here, a laparoscopic paediatric hernia repair model is trialled with surgical trainees and their competence compared with consultant colleagues. PATIENTS AND METHODS: An ex-vivo paediatric inguinal hernia repair model was devised. Surgical trainees from 5 specialist centres were recruited and performed multiple standardised repairs. RESULTS: 23 trainees performed 192 repairs. Experts performed 10 repairs for comparison. Trainees were timed performing the repair and their accuracy measured. With repeated attempts trainee's timings and accuracy improved until by the 10 th repair they were no different from benchmark consultant scores. CONCLUSION: A simple, procedure specific ex-vivo training model has been evaluated for laparoscopic hernia training in paediatric surgery. The results suggest improvements in competence with repetition. Trainee and benchmark consultant scores are no different by the 10 th trainee attempt. We conclude that this model may have a valuable role in the training and assessment of future paediatric surgeons.

Is there unity in Europe? First survey of EUPSA delegates on the management of gastroschisis.

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 < 0.05 was considered significant. 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.

The "diamond port configuration": a standardised laparoscopic technique for adolescent intestinal resection and anastomosis.

BACKGROUND: Familiarity with technique and repetition enhance efficiency during laparoscopic surgery. This is particularly important when undertaking complex bowel resections. We report a standardised protocol that includes theatre layout, patient position and port insertion, which we believe facilitates excellent abdominal access and ergonomics and has the potential to shorten the duration of the team-learning curve. MATERIALS AND METHODS: A strategic unit development plan led to the commencement of a laparoscopic service for adolescents with bowel disorders. A standardised protocol for intestinal resections was agreed upon at a monthly Paediatric Minimal Access Group meeting. This covered patient position, port insertion, technical aspects of intestinal resection and perioperative management. In particular, a diamond configuration for ports was agreed upon. Data were prospectively collected, and included patient demographics, operative times, conversion rates and postoperative outcomes. Unless otherwise indicated, data are presented as medians with ranges. RESULTS: Seven procedures were carried out in six patients (three female) aged 14 (11-14) years. Access to the entire abdominal cavity, vision and ergonomics were excellent in all. There were no conversions to open surgery. In all procedures, the technique was considered safe and effective. The length of hospital stay was 6.5 (5.8-14) days. CONCLUSION: A standardised protocol including the use of the diamond port configuration has several putative advantages for laparoscopic bowel resections and anastomoses. These include efficiency, reproducibility, predictability, good visibility and excellent ergonomics. We recommend this approach as a means to shorten the procedure-specific learning curve of the laparoscopic team.

Gastroschisis: a multi-centre comparison of management and outcome.

BACKGROUND: Anecdotal evidence and a handful of literature reports suggest that the outcome for infants born with gastroschisis in many African countries is poor when compared to Western nations. We wished to evaluate current management strategies and outcomes in African and Western units that treat infants with gastroschisis. PATIENTS AND METHODS: We conducted a retrospective review of case-notes for infants with gastroschisis who presented to a hospital between 1 January 2004 and 31 December 2007. There were five participating centres, divided for analysis into an African cohort (three centres) and a Western cohort (two centres). RESULTS: Fewer infants presented to a hospital with gastroschisis in the African cohort when compared to the Western cohort, particularly when the size of catchment area of each hospital was taken into account. The physiological state of the infant on presentation and management strategy varied widely between centres. Primary closure, preformed silo and surgical silo with delayed closure were all utilised in the African cohort. Use of the preformed silo and delayed abdominal wall closure was the strategy of choice in the Western cohort. The 30-day mortality was 23% and 1% respectively. This primary outcome measure varied considerably in the African cohort but was the same in the two Western units. CONCLUSIONS: Gastroschisis in the African cohort was characterised by fewer infants presenting to a hospital and a more variable outcome when compared to the Western cohort. A detailed epidemiological study to determine the incidence of gastroschisis in African countries may provide valuable information. In addition, interventions such as prompt resuscitation, safe neonatal transfer, the use of the preformed silo and parenteral nutrition could improve outcomes in infants with gastroschisis.

GABBY: an ex vivo model for learning and refining the technique of preformed silo application in the management of gastroschisis.

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.