Short and Medium Term Outcomes of Open and Laparoscopic Assisted Oesophageal Replacement Procedures.

AIM OF THE STUDY: We describe the short- and medium-term outcomes following open and laparoscopic assisted oesophageal replacement surgery in a single tertiary paediatric surgical centre. METHODS: A retrospective review (institutional audit approval no. 3213) on patients who underwent open or laparoscopic-assisted oesophageal replacement (OAR vs. LAR) at our centre between 2002 and 2021 was completed. Data collected (demographics, early complications, stricture formation, need for oesophageal dilatations, and mortality) were analysed using GraphPad Prism v 9.50 and are presented as median (IQR). RESULTS: 71 children (37 male) had oesophageal replacement surgery at a median age of 2.3 years (IQR 4.7 years). 51 were LAR (6 conversions). Replacement conduit was stomach (n = 67), colon (n = 3), or jejunum (n = 1). Most gastric transpositions had a pyloroplasty (46/67) or pyloromyotomy (14/67). Most common pathology was oesophageal atresia (n = 50 including 2 failed transpositions), caustic injury (n = 19 including 3 due to button battery), stricture of unknown cause (n = 1), and megaoesophagus (n = 1). There were 2 (2.8 %) early postoperative deaths at 2 days (major vessel thrombosis), 1 month (systemic sepsis), and one death at 5 years in the community. The rate of postoperative complications were comparable across LAR and OAR including anastomotic leak, pleural effusions, or early strictures. More patients with caustic pathology needed dilatations (60 % vs 30 % in OA, p = 0.05). CONCLUSIONS: Outcomes of open and laparoscopic-assisted oesophageal replacement procedures are comparable in the short and medium term. Anastomotic stricture is higher in those with caustic injury. LEVEL OF EVIDENCE: IV.

Laparoscopic inguinal hernia repair (LIHR): the benefit of the double stitch in the largest single-center experience.

AIM: To review our experience of laparoscopic inguinal hernia repair (LIHR) regarding complication rates, the practice of closing the asymptomatic patent processes vaginalis (PPV), and comparison of complication rates between pre-term (< 37 week gestation) and term infants. METHODS: Retrospective review of LIHR performed between 2009 and 2021. Repair was performed by intracorporal single or double purse string/purse string + Z-stitch using a non-absorbable suture. Data were analyzed using Chi-squared/Mann-Whitney and are quoted as median (range). RESULTS: 1855 inguinal rings were closed in 1195 patients (943 (79%) male). 1378 rings (74%) were symptomatic. 492 (41%) patients were pre-term. Corrected gestational age at surgery was 55 weeks (31 weeks-14.6 years) and weight 5.9 kg (1-65.5). Closure of contralateral PPV was higher in the premature group (210/397 [53%] vs. 265/613 [43%] p = 0.003). There were 23 recurrences in 20 patients, of whom 10 had been born prematurely. The only factor significantly associated with a lower recurrence was use of a second stitch (p = 0.011). CONCLUSION: This is the largest single-center reported series of LIHR. LIHR is safe at any age, the risk of recurrence is low, and can be corrected by re-laparoscopy. Use of a Z-stitch or second purse string is associated with a significantly lower rate of recurrence.

Ultrasonographic features associated with previous torsion and the impact of surgery in managing neonatal ovarian cysts: a 20-year single-centre retrospective study.

PURPOSE: To identify markers of previous ovarian torsion and outline the outcomes according to US appearance and operative management. METHODS: A retrospective single-centre review of neonatal ovarian cysts from January 2000 to January 2020. Data on postnatal cyst size and sonographic features and operative treatment were co-related with outcomes of ovarian loss and histology. RESULTS: 77 females were included with 22 simple and 56 complex cysts, one patient had bilateral cysts. 9/22 (41%) simple cysts regressed spontaneously in a median of 13 weeks (8-17). Complex cysts regressed spontaneously less frequently, 7/56(12%, P = 0.01), in 13 weeks (7-39). 38/56 (68%) complex and 12/22 (55%) simple cysts were treated operatively. 21/22 (95%) ovaries with initially simple cyst were salvaged compared to 20/56(36%) with initially complex cyst (P < 0.001). A fluid-debris level in 23/26 complex cysts was most associated with ovarian loss (P = 0.0006). Presence of viable ovarian stromal tissue was seen in 8/20 (40%) excised specimens during ovarian sparing procedures and in 5/30 (17%) oophorectomies for necrotic appearing ovaries. CONCLUSIONS: Fluid-debris level on US is significantly associated with ovarian loss likely due to previous torsion. Simple cysts are viable and often regress spontaneously. The finding of viable ovarian stromal tissue in resected specimens supports attempting ovarian preservation wherever possible.

Minimal access surgery for congenital diaphragmatic hernia: surgical tricks to facilitate anchoring the patches to the ribs.

OBJECTIVE: Minimal Access Surgery (MAS) for Congenital Diaphragmatic Hernia (CDH) repair is well described, yet only a minority of surgeons report this as their preferred operative approach. Some surgeons find it particularly difficult to repair the defect using MAS and convert to laparotomy when a patch is required. We present in this study our institutional experience in using an easy and relatively cheap methodology to anchor the patch around the ribs using Endo Close™. This device has an application in MAS for tissue approximation using percutaneous suturing. METHODS AND TECHNIQUE: We retrospectively reviewed our database for patients undergoing MAS repair of CDH between 2009 and 2021. Outcome measures included length of surgery and recurrence rates after patch repair. Endo Close™ was used in all patients who required patch repair. We declare no conflict of interest and to not having received any funding from Medtronic (UK). The technique is as follows: (1) The edges of the diaphragm are delineated by dissection. When primary suture repair of the diaphragmatic hernia was unfeasible without tension, a patch was used. (2) The patch is anchored in place by two corner stitches at the medial and lateral borders. (3) The posterior border of the patch is fixed to the diaphragmatic edge by running or interrupted stitches. (4) For securing the anterior border, a non-absorbable suture is passed through the anterior chest wall and the patch border is taken with intracorporeal instruments. (5) Without making another stab incision, the Endo Close™ is tunnelled subcutaneously through the anterior chest wall. (6) The suture end is pulled through the Endo Close™ and the knot is tied around the rib. This procedure can be performed as many times as required to secure the patch. RESULTS: 58 patients underwent MAS surgery for repair of CDH between 2009 and 2021. 48 (82%) presented with a left defect. 34 (58%) had a patch repair. The length of patch repair surgery for CDH ranged from 100-343 min (median 197). There was only one patient (3%) in the patch repair cohort that had a recurrent hernia, diagnosed 12 months after the initial surgery. CONCLUSIONS: In our experience, MAS repair of CDH is feasible. We adopted a low threshold in using a patch to achieve a tension-free repair. We believe that the Endo Close™ is a cheap and safe method to help securing the patch around the ribs.

Thoracoscopic vs open repair of congenital diaphragmatic hernia after extracorporeal membrane oxygenation: a comparison of intra-operative data.

PURPOSE: ECMO is an escalation treatment for hypoxic respiratory failure in patients with CDH. Open repair has been advocated after ECMO indicating that physiological changes associated to thoracoscopic repair were not well tolerated. METHODS: We have performed a retrospective review of all patients who underwent ECMO prior CDH repair over a 7 year period (2015-2021). Outcome measures were intra-operative Ph, PCO2, PO2 and FiO2 at 30 min, 1 h 30 min, and 2 h 30 min of surgery, operative time and recurrence rate. Data are shown in median (range). RESULTS: Eleven patients required ECMO prior CDH repair. Six of eleven (55%) were done thoracoscopically (Group A) and five of eleven (45%) via laparotomy (Group B). Two of six (33%) patients (Group A) were converted to a laparotomy, one of six (16%) patient developed a recurrence, and there was no recurrence in Group B. Two of five (40%) patients died within the first 60 days of life, whilst there was no death in Group A. Intra-operative values are shown below. CONCLUSION: Whilst this is a preliminary report of a limited number of patients, there is no obvious difference of intra-operative blood gas parameters during surgical repair in patients after ECMO. Thoracoscopic CDH repair may be considered in patients after ECMO.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.

Maintaining a minimally invasive surgical service during a pandemic.

PURPOSE: The safety of minimally invasive surgery (MIS) was questioned in the COVID-19 pandemic due to concern regarding disease spread. We continued MIS during the pandemic with appropriate protective measures. This study aims to assess the safety of MIS compared to Open Surgery (OS) in this setting. METHODS: Operations performed during 2020 lockdown were compared with operations from the same time-period in 2019 and 2021. Outcomes reviewed included all complications, respiratory complications, length of stay (LOS) and operating surgeon COVID-19 infections (OSI). RESULTS: In 2020, MIS comprised 52% of procedures. 29% of MIS 2020 had complications (2019: 24%, 2021: 15%; p = 0.08) vs 47% in OS 2020 (p = 0.04 vs MIS). 8.5% of MIS 2020 had respiratory complications (2019: 7.7%, 2021: 6.9%; p = 0.9) vs 10.5% in OS 2020 (p = 0.8 vs MIS). Median LOS[IQR] for MIS 2020 was 2.5[6] days vs 5[23] days in OS 2020 (p = 0.06). In 2020, 2 patients (1.2%) were COVID-19 positive (MIS: 1, OS: 1) and there were no OSI. CONCLUSION: Despite extensive use of MIS during the pandemic, there was no associated increase in respiratory or other complications, and no OSI. Our study suggests that, with appropriate protective measures, MIS can be performed safely despite high levels of COVID-19 in the population.

Current treatment and outcomes of pediatric gastrointestinal stromal tumors (GIST): a systematic review of published studies.

Gastrointestinal stromal tumor (GIST) is a rare cancer of mesenchymal origin mostly seen in adult and elderly populations. Therefore, the prognostic and therapeutic features of pediatric GIST are not clearly defined. Clinical knowledge has been largely extrapolated from case series and adult studies. In this systematic review, we aimed to analyze the health outcome metrics of pediatric GIST. Medline and Embase databases were searched using relevant key terms. The original search retrieved 1,892 titles; 27 studies with 184 patients (68% female) were included for final review. The primary tumors were located in the stomach (165/184, 90%), small bowel (12/184, 7%), and elsewhere (7/184, 4%). Individual patient data were available in 125 cases with a median follow-up of 6.7 years. All patients underwent surgical resection, which varied from wide local excision to total gastrectomy. There were 12 deaths (10%), 65 (52%) patients were alive with no evidence of disease, and 31 cases (25%) were alive with disease. Tumor size > 5 cm, high mitotic index, and spindle morphology were predictive of mortality. Pediatric GIST has a more favorable prognosis and different characteristics versus adult tumors. There is a crucial need for international consensus and specific pediatric guidelines for the treatment of this rare tumor.

Development of a core outcome set for congenital pulmonary airway malformations: study protocol of an international Delphi survey.

INTRODUCTION: A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. METHODS AND ANALYSIS: This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. ETHICS AND DISSEMINATION: Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM.

Neuroblastoma: the association of anatomical tumour site, molecular biology and patient outcomes.

BACKGROUND: Numerous factors have been identified as carrying prognostic value in neuroblastoma (NB) and therefore incorporated in risk stratification of disease. Here, we investigate the association of anatomical site of NB with molecular biology and clinical outcomes. METHODS: A total of 117 patients with NB were studied over a 30-year period. Tumour location was confirmed with computed tomography/magnetic resonance imaging. Data on molecular biology were obtained as testing became available. Chi-squared, Fisher's exact test and Kaplan-Meier log-rank tests were used for statistical analysis. RESULTS: Tumour originated in the thoracic region (thoracic NB, TNB) in 15 patients (13%), adrenal gland (adrenal NB, ANB) in 88 patients (75%) and abdominal/paravertebral chain (paravertebral NB, PVNB) in 14 patients (12%). Overall survival (OS) for ANB was significantly lower (38%; P = 0.015). ANB cases were more frequently diagnosed at stage IV (69%; P = 0.001). MYCN amplification was noted in 33% of ANB cases and associated with lower OS (17% versus 62% MYCN non-amplified ANB; P = 0.01). The vast majority of TNB and PVNB were non-MYCN amplified (100% and 86%, respectively) and carried better prognosis (OS 86% and 83%, respectively). Forty-two percent of ANB cases were diploid and had lower OS (20% versus 71% hyperdiploid ANB; P = 0.079). TNB and PVNB were found to be mostly hyperdiploid (86% and 100%, respectively) with better OS (83% and 33%, respectively). Segmental chromosomal alterations had prognostic significance in those with PVNB (P = 0.03). CONCLUSION: TNB tumours have better outcomes than adrenal tumours. This may be due to varied factors reported here including non-metastatic disease at presentation, non-amplification of the MYCN oncogene and overall favourable molecular biology characteristics.

Stage 4S Neuroblastoma: What Are the Outcomes? A Systematic Review of Published Studies.

INTRODUCTION: The prognosis of stage 4S/MS neuroblastoma has traditionally been reported as excellent, yet conflicting treatment protocols exist for this enigmatic disease. To critically address this question, we have undertaken a systematic review of published studies to accurately determine outcomes for infants with stage 4S/MS neuroblastoma. MATERIALS AND METHODS: Studies were identified using MEDLINE, Embase, and Cochrane databases using the relevant search terms. Literature reviews, case reports, and adult studies were excluded. Data were extracted independently following article selection by three authors and reviewed by the senior author. RESULTS: The original search retrieved 2,325 articles. Following application of exclusion criteria and removing duplicate data, 37 studies (1,105 patients) were included for final review. Overall patient survival was 84%. Twelve studies (544 patients) recorded MYCN status. Mortality in MYCN amplified tumors was 56%. Chromosome 1p/11q status was reported in four studies and 1p/11q deletion carried a 40% fatality rate. Management included observation only (201 patients, 8.5% mortality), surgical resection of primary tumor only (153 patients, 6.5% mortality), chemotherapy only (186 patients, 21% mortality), radiotherapy (5 deaths, 33% mortality), chemotherapy with surgery (160 patients, 10% mortality), surgery with radiotherapy (21 patients, 19% mortality), radiotherapy with chemotherapy (42 patients, 29% mortality), and surgery with chemotherapy and radiotherapy (27 patients, 33% mortality). CONCLUSION: There is a significant mortality observed in stage 4S/MS neuroblastoma infants with a dismal outcome observed in those patients with MYCN amplification and 1p/11q deletion. Those patients suitably amenable for conservative management or surgery to excise the primary tumor carry the best prognosis.

Potential Benefits of Laparoscopic Repair of Duodenal Atresia: Insights from a Retrospective Comparative Study.

INTRODUCTION: Congenital duodenal obstruction (CDO) repair can be performed open or laparoscopically. We aimed to determine the potential benefit of laparoscopic repair regarding tolerance of enteral feeding, postoperative pain, hospital stay, and complication rate. MATERIALS AND METHODS: In a single-center retrospective cohort study, we compared neonates with isolated CDO operated open versus laparoscopically from 2010 to 2019. No transanastomotic tubes were used, and anastomoses were created in a side-to-side fashion in all cases. An early feeding policy is applied for all cases operated at our institution. Statistical comparison was performed using the Mann-Whitney's test or Fisher's exact test where appropriate. RESULTS: Forty-one patients analyzed were similar regarding body weight, gestational age, and proportion of patients with trisomy 21. Median follow-up was 21 months. Four (20%) out of 20 laparoscopic procedures started laparoscopically were converted to open. Comparing the 21 open with the 16 laparoscopically completed patients, median anesthetic duration was shorter by 18% in the open versus laparoscopic completed group (218 vs. 179 minutes, respectively; p = 0.025). Median postoperative time to full enteral feeds was shorter by 4 days in the first group (7 vs. 11 days, respectively; p = 0.028). In accordance, the median duration of parenteral nutrition (PN) was less than half in the laparoscopic completed compared with the open group (5 vs. 11.5 days, respectively; p = 0.031). Postoperative opioids were required for only half the duration in the laparoscopically completed group compared with open (2 vs. 4 days, respectively; p = 0.026). Outcomes such as length of stay, the occurrence of strictures or adhesions requiring reintervention, or line sepsis were similar in both groups. CONCLUSION: Patients undergoing laparoscopic CDO repair at our institution benefited from shorter time to full enteral feeds, and reduced the need for PN as well as postoperative pain medication.

Congenital intrathoracic stomach can be safely managed laparoscopically.

PURPOSE: Congenital intrathoracic stomach (CIS) is an uncommon pediatric surgical diagnosis where > 2/3rd of the stomach lies within the chest through a hiatus defect. We reviewed our recent experience with this condition. METHODS: A retrospective single-center review of children with a diagnosis of CIS (2007-2018) was performed. Patient demographics, presentation, imaging and management were assessed. Results are expressed as median (range). RESULTS: Eleven patients (6 girls) were identified with onset of symptoms at 2 (0-26) months of age. Presenting symptoms were vomiting (8/11), respiratory symptoms (4/11) and failure to thrive (2/11). Two patients had Marfan's syndrome. An upper gastrointestinal contrast study demonstrated gastric herniation in all. All were corrected laparoscopically with hiatus repair and fundoplication [age at surgery 10.5 (1.5-34.5) months]. A concurrent gastrostomy was done in children ≤ 6 months (n = 5). Enteral feeds were commenced on post-operative day one in 9 and second post-operative day in 2. At 7 (0-95) months follow-up, all were on full enteral feeds. One patient had a recurrence 6 months post-operatively, which was re-operated laparoscopically without any further recurrence. CONCLUSION: This is the largest reported series of children with CIS. All could be managed laparoscopically with no conversions and a low recurrence.

Role of Routine Dilatations after Anorectal Reconstruction-Comparison of Two Tertiary Centers.

AIM: Regular anal dilatations are commonly recommended in the postoperative management following posterior sagittal anorectoplasty (PSARP) in anorectal malformations (ARM). We hypothesized that routine postoperative dilatations may not affect surgical outcomes following PSARP. We compare surgical outcomes of routine postoperative dilatations versus no routine postoperative dilatations from two United Kingdom tertiary pediatric surgical centers. MATERIALS AND METHODS: This is retrospective records review of patients undergoing definitive surgery for ARM in two tertiary surgical centers in the UK over 5 years. Center A used a protocol of routine postoperative dilatations, and center B used a protocol, which used dilatations only for clinical indications of stricture. Data collected included ARM type, operative procedures, and postoperative interventions. All post-operative interventions under general anesthesia (GA) were compared between groups. RESULTS: From 2011 to 2015, 49 procedures (46 PSARPs) were performed in center A and 54 (52 PSARPs) in center B. Median follow up period was 31 months (interquartile range [IQR] 18-48). The first postoperative anal calibration under GA was documented for 43 (86%) patients in center A and for 42 (78%) patients in center B. Following this, center A followed routine postoperative dilatation (RPD) at home, and center B reserved further dilatations for specific indications. RPD was performed for 100% of patients in center A versus 8% in center B. Further anal dilatations under GA were performed in 19 (38%) children in center A and in 17 (34%) children in center B (p = 0.68). In center A, 10 patients (22%) needed further surgery versus 14 (28%) in center B (p = 0.48). CONCLUSION: The use of routine postoperative dilatations does not significantly improve surgical outcomes following PSARP in ARM.

Anterior mediastinal masses - A multidisciplinary pathway for safe diagnostic procedures.

OBJECTIVE: The objective of this study was to report our multidisciplinary diagnostic approach for patients with anterior mediastinal masses (AMM). METHODS: A retrospective review of patients with AMM at a tertiary pediatric surgical oncology centre (January 2011-December 2016) was performed. We analyzed data on clinical presentation, mode of tissue diagnosis, anesthetic techniques, and complications. RESULTS: Of the 44 patients admitted with AMM (median age 11 years, 27 males and 17 females), 22 had respiratory symptoms. Imaging revealed tracheobronchial compression in 26 children. Twenty patients had a lymph node biopsy. Ten patients had image-guided core biopsy of the mediastinal mass, and 2 had mediastinoscopic biopsy of a paratracheal lymph node. One patient with likely recurrence of a relapsed metastatic ethmoid carcinoma did not have a biopsy. The diagnosis was made from alternative tissues, such as pleural fluid in 4 and peripheral blood in 7 patients. Twenty-five anesthetics were assessed, as 14 patients required no or only local anesthesia, and 5 had unavailable anesthetic notes. Eighteen of 25 patients were anesthetized maintaining spontaneous breathing, mostly by means of ketamine sedation. There were no major anesthetic complications. CONCLUSION: Safe tissue diagnosis of anterior mediastinal masses can be obtained by a personalized multidisciplinary approach. Use of alternative tissues, local anesthesia, and ketamine sedation help minimize the need for general anesthesia, muscle paralysis, and controlled ventilation. LEVEL OF EVIDENCE: IV (Case Series with no Comparison Group).

Loop colostomies are safe in anorectal malformations.

AIM OF THE STUDY: Divided colostomy (DC) has been recommended in anorectal malformations (ARMs) with previously reported advantages of decreasing overflow into the distal limb and urinary tract infections (UTIs). Skin bridge loop colostomy (LC) is a technically easier alternative without an increase in these complications. We report our institutional experience of LC in ARM. METHODS: Retrospective study (Institution-approved Clinical Audit) reviewing the clinical records of all patients with ARM undergoing stoma formation in a single UK tertiary pediatric surgical center (2000-2015). Data collected included type of ARM, associated anomalies, type and level of colostomy, time to stoma closure, complications and UTIs. RESULTS: One hundred and eighty-two (95 female) patients underwent colostomy formation for ARM. The vast majority (171/ 94%) underwent LC; 9 (5%) had a divided colostomy (DC) and 2 (1%) had no available data. The spectrum of defects in girls included rectovestibular (62/65%), rectovaginal (4/4%) and cloaca (29/31%). In boys, 71 (82%) had a fistula to the urinary tract and 16 (18%) presented with a perineal fistula. Urological abnormalities coexisted in 87 (47.8%) patients. Thirty five (21%) patients developed UTIs. Among the 19 girls who developed UTI, 8 had rectovestibular fistula and 11 had cloaca. Of the 16 boys who developed UTI, 14 had a fistula to the urinary tract and 11 had an independent urological abnormality. The mean time from stoma formation to stoma closure was 10 (3-52) months. Complications were reported in 22 (12%) LCs. Fifteen patients (9%) developed a stoma prolapse following LC with 10 (6%) requiring surgical revision. CONCLUSIONS: This is the largest reported series of outcomes following LC for ARM. LC is easier to perform and to close, requiring minimal surgical access, with comparable complications and outcomes to those published for DC. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: III.

Quality of systematic reviews and meta-analyses published in pediatric surgery.

AIMS: Systematic reviews (SRs) and meta-analyses (MAs) studies may influence and direct surgical practice. Against this background we have analyzed the quality of systematic reviews and meta-analyses published in the specialty field of pediatric surgery using the Assessment of Multiple Systematic Reviews 11-item tool (AMSTAR). METHODS: MEDLINE and EMBASE databases and the three major journals in pediatric surgery were searched for SRs and MAs in pediatric surgery. Studies involving predominantly adult populations were excluded. Two reviewers independently scored included studies against AMSTAR criteria and disagreements were resolved by consensus. A total rating of 4 or less was considered 'poor' methodological quality, 5-8 as 'fair to good' and 9 or greater as 'good'. RESULTS: Original searching retrieved 1,281 articles. 126 articles were included for final analysis. Examining recent trends, 4 studies were published in 1995-2000 compared to 78 in 2011-2014. Using AMSTAR scoring criteria, 35 reviews (28%) were regarded as 'poor' in terms of methodological quality, 59 (47%) 'fair', and 32 (25%) 'good' quality. We observed no improvement in AMSTAR score before and after the development of the tool (mean score pre-2008 6.8, post-2008 5.9, p = 0.136). CONCLUSIONS: Despite an increase in the number of SRs / MAs published in pediatric surgery, a quarter of all studies were considered poor in terms of their quality and scientific validity. Journals must define and apply minimum criteria to ensure pediatric surgeons seeking to publish high quality SRs / MAs achieve these requirements.

Pediatric/"Wildtype" gastrointestinal stromal tumors.

Pediatric/"Wildtype" gastrointestinal stromal tumor (P/WT-GIST) is a rare cancer, distinct and markedly different from the phenotype found predominantly in older patients (adult, non-wildtype GIST). Having a different molecular signature, it is not responsive to standard adjuvant therapies utilized in adult GIST, and surgery remains the only effective cure. However, even with presumed complete resections in patients with localized disease at presentation, recurrence rates are high. Furthermore, it is an indolent cancer that can persist for decades, and treatment strategies must balance the possible morbid risks of intervention with the reality of preserving quality of life in the interim. Effective adjuvant therapies remain elusive, and research is critically needed to identify both targets and drugs for treatment consideration.

Incisional hernia in pediatric surgery - experience at a single UK tertiary centre.

AIMS: Incisional hernia (IH) is a recognized complication of open and laparoscopic visceral surgery, with reported rates of 10-50% in adult surgical literature. There is a paucity of data relating to incisional hernias in children. The aim of our study was to analyze the incidence and treatment of IH in children. METHODS: Retrospective review of all patients admitted for incisional hernia repair at a tertiary pediatric surgical centre in the UK more than a 7-year period was performed. Data collected included age at initial surgery, time to IH repair, and type of IH repair and postoperative complications. RESULTS: Twenty one patients (14 male) underwent IH repair during the study period. The incidence of IH among children who had primary abdominal surgery in our institution less than the age of 6months was 2.3%. Median age at repair was 7.9months (range: 18days-5years). Median time from primary surgery to diagnosis of IH was 2months (range 0day-3years), with 81% (17/21) diagnosed within 1year of the preceding abdominal procedure. The most common pathology necessitating the primary operative procedure was necrotising enterocolitis (n=9) in babies of gestational age less than 30weeks. The highest rates of IH were noted in infants following closure of stoma (7.5%) and pyloromyotomy (2.52%). Primary closure was undertaken in all cases. Two children had recurrence of IH, one of which underwent surgical repair. CONCLUSIONS: Incidence of IH in children is low but significant. IH was most commonly diagnosed following closure of stoma for NEC in this study.