Choice of topical substances in the conservative management of Exomphalos - A systematic review.

AIM: Exomphalos is a congenital anomaly found in 1/4500 newborns. Choice of non-operative management of exomphalos major unamenable to primary repair is controversial. This study aims at reviewing conservative management modalities and compare outcomes and complications. METHODS: A systematic review was performed according to PRISMA guidelines of all English publications in MEDLINE and EMBASE databases. Search words were exomphalos OR omphalocoele AND conservative OR non-operative AND management. Studies were scrutinised for patient demographics, co-morbidities, mode of treatment, time to full feeds, time to full epithelialisation, length of stay, complications and mortality. Studies not specifically describing mode of management and/or describing primary or staged surgical repairs were excluded. RESULTS: Initial search resulted in 1243 studies. Forty-two studies were deemed suitable offering 822 patients for analysis after excluding duplicates and non-eligible studies. Management methods varied including painting with Alcohol, Mercurochrome, silver products, Povidone Iodine, honey and other materials. Mortality was mostly due to associated anomalies. There was mixed reporting of alcohol, silver, Povidone Iodine and mercury toxicity as well as infection during the course of treatment. CONCLUSION: This report has recognised the variations in topical substances employed for conservative management with no clear consensus. Reports on safety of different methods remain unclear.

Laparoscopic hernia repair in children: does recreating the open operation improve outcomes? A systematic review.

PURPOSE: The use of laparoscopy for paediatric inguinal hernia repairs has increased significantly over the past 2 decades. However, there is significant variation in the reported recurrence rates in the literature, with many studies reporting higher rates than the open operation. This may be explained by the range of different techniques currently included under the term laparoscopic inguinal hernia repair. The purpose of this study is to determine whether dividing the hernia sac before ligation improves surgical outcomes following a paediatric laparoscopic inguinal hernia repair compared to ligation alone. METHODS: A systematic review of the literature was performed following PRISMA guidelines of all studies reporting the outcomes following paediatric laparoscopic inguinal hernia repair where the technique was recorded as laparoscopic suture ligation alone (LS) or laparoscopic sac division and suture ligation (LSDS). Studies were assessed for risk of bias and exclusion criteria included reported follow-up of less than 6 months. RESULTS: A total of 8518 LS repairs and 6272 LSDS repairs were included in the final analysis. LSDS repair was associated with a significantly lower recurrence rate (odds ratio 0.51, 95% CI 0.36-0.71, p = 0.001). There was no significant difference in the rates of testicular ascent or atrophy. CONCLUSION: Recreating the open operation by hernia sac division followed by suture ligation significantly reduces the risk of hernia recurrence.

Gastrostomies in children requiring long-term ventilation.

PURPOSE: Children requiring long-term ventilation (LTV) via tracheostomy often require enteral tube feeding. We sought to investigate what proportion of these children underwent gastrostomy insertion to inform decision making at time of tracheostomy formation. METHODS: A retrospective review of all children commenced on LTV via a tracheostomy at Royal Manchester Children's Hospital over a 9-year period (2012-2020). Data are presented as median [IQR]. RESULTS: Forty-one LTV patients had tracheostomy insertion with an average age of 167 days [101-604]. Reasons for tracheostomy insertion were upper airway obstruction (18), central neurological condition (7), neuromuscular condition (12) and lower respiratory tract disease (4). Twenty-two patients were born preterm and chronic lung disease of prematurity was a contributory factor in their requirement for LTV. Eight children had gastrostomies inserted prior to tracheostomy formation. A further 22 children had a gastrostomy inserted at an average of 139 days [99-227] following tracheostomy. Four children remained on nasogastric feed and the rest were fed orally. Seventy-three percentage of LTV children with tracheostomy were gastrostomy fed. Neither indication for LTV nor prematurity predicted whether a child was gastrostomy fed. CONCLUSION: The large majority of children requiring LTV are tube fed and gastrostomy insertion should be considered at time of formation of tracheostomy.

Surgical Management of Esophageal Achalasia in Pediatrics: A Systematic Review.

INTRODUCTION: There are no evidence-based guidelines on the surgical management of esophageal achalasia (OA) in children. This can be a challenging condition with significant physical and psychological morbidity. Our aim was to identify the most common management modalities and their outcomes. MATERIALS AND METHODS: A systematic review was performed through a literature search of health care databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, aiming at identifying pediatric series discussing the diagnosis and management of OA. Duplicates, case series with < 9 patients, and follow-up of < 1 year were excluded. The included papers were analyzed for diagnostic methods, primary treatment method, complications, follow-up duration, outcome measures recorded, and outcome. RESULTS: Data from 33 papers for 742 children treated for OA was analyzed. Eleven mentioned multiple management modalities. In summary, 25 described Heller's esophagomyotomy (HM), 13 esophageal dilatation (EOD), and 6 peroral esophageal myotomy (POEM). Mean follow-up was 43.7 months (12-180). Outcome measures were heterogeneous. However, analysis of reported success showed a mean success of 78% for HM (p = 1.79 × 10-7), 44.9% for EOD (p = 0.24), and 99.3% for POEM (p = 0.001). Reported complications were 12.8% for HM, 5% for EOD, and 24.4% for POEM. Further interventions were required for 10.9% of HM, 62.3% of EOD, and 0.01% of POEM patient groups. CONCLUSION: Methods of diagnosis and measures of successful outcomes were heterogeneous, limiting the strength of evidence. HM showed superior short-term success rates to EOD. POEM is a promising modality but requires investment in equipment and training. Information about sustainability of response and long-term outcomes is lacking.

The Cambridge experience with buried bumpers.

AIM: Buried Bumper (BB) is a complication of percutaneous endoscopic gastrostomy (PEG) that leads to tube dysfunction and major morbidity. Although many techniques have been described to manage BB, none are universally adopted, and laparotomy remains the mainstay. We introduce a novel endoscopic technique in paediatric surgery that avoids laparotomy. METHODS: A retrospective review of medical records of patients who presented with BB to Cambridge University Hospital, UK, between January 2012 and June 2018 was done. Data collected included: demographics, tube size and type, interval between insertion and diagnosis of BB, hospital stay, technique used, and postoperative complications. The technique involved using an endoscopic snare passed from inside the stomach lumen through the PEG lumen to the outside, guided if required by a stiff nylon thread if no part of the PEG was visible, grasping the PEG tube externally after cutting it short, followed by a retrograde pull to remove the buried tube via the mouth. MAIN RESULTS: Fifteen BBs were found in ten patients. Median patient age was 5.25 years (1.2-16.6). Median time between gastrostomy insertion and diagnosis of BB was 9 months (1-32). Twelve BBs were removed endoscopically with no postoperative complications. Patients had a replacement inserted through the original track and were discharged within 24 h. Two underwent laparotomies performed by surgeons unfamiliar with endoscopic technique, and one was converted to laparotomy owing to inability to transverse an encrusted and closed PEG tube lumen. CONCLUSION: Endoscopic retrograde BB removal is a safe, easy, and quick technique with minimal complications. We strongly advocate widespread adoption of the technique before considering a laparotomy. LEVEL OF EVIDENCE: Treatment study: Level IV.

Effect of previous abdominal surgery and gallbladder appearance on biliary atresia outcomes.

BACKGROUND: Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis. METHODS: A retrospective case note review was performed of all BA patients at a single centre from 1999 to 2016. Demographics and clinical outcome data were collected. Additional data on laparotomy, parenteral nutrition, and referral were collected from patients who underwent PAS. Data are median (range). MAIN RESULTS: Two-hundred-and-fifty-seven children were reviewed. Of these, 16 (6.2%) underwent PAS on day 3 (0-23), during which 5 atretic gallbladders were noted. Gallbladder appearance was not referenced in the operation notes of 8 infants. Jaundice and acholic stools were noted at 4 (0-56) days and 21 (0-60) days, respectively. Age at KPE was comparable between PAS and the other patients (50 vs. 51days; P=0.78), but native liver survival was significantly lower after PAS (p<0.0001). Mortality rate was higher in PAS patients (25% vs. 4.5%; P=0.0007). Survival was unaffected by early referral of patients on finding an atretic gallbladder at surgery. CONCLUSION: About 6% of infants have already undergone abdominal surgery for biliary atresia associated intestinal anomalies. Routine gallbladder examination at time of laparotomy could have aided earlier diagnosis and treatment of biliary atresia in up to 80% of patients in this cohort. However, our data suggest that clinical outcome is poorer in biliary patients who undergo prior abdominal surgery and is not improved by earlier referral. LEVEL OF EVIDENCE: Prognostic study: Level III.