GIST with a twist: An extra-luminal pedunculated gastric GIST presenting with tumor torsion and hemoperitoneum

AbstractGastrointestinal stromal tumors (GIST) are rare mesenchymal tumors. Most present with nonspecific symptoms. We present a case of a 17-year-old male with sudden abdominal pain. Imaging revealed a hypodense mass inferior to the stomach. Serial blood work showed a decreasing hemoglobin level. In the operating room a pedunculated, torted, hemorrhagic mass on the anterior surface of the stomach was resected. Pathology confirmed a GIST, c-kit positive with involved margins. The patient was started on imatinib. Pediatric GISTS are different than adult GISTs; the majority are c-kit negative (wild-type) and typically indolent. Despite a high recurrence rate, aggressive resection of pediatric GIST is generally not indicated. This pediatric GISTs was c-kit and PDGFR exon 18 mutation positive and thus was treated according to the National Comprehensive Cancer Network (NCCN) guidelines (for adults)

Post-operative management of esophageal atresia-tracheoesophageal fistula and gastroesophageal reflux: A Canadian Association of Pediatric Surgeons annual meeting survey

Background: Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), is commonly associated with gastroesophageal reflux (GER) after surgical repair. One risk factor for anastomotic stricture is post-operative GER. This survey assessed practice patterns among attendees at the Canadian Association of Pediatric Surgeons (CAPS) annual meeting with respect to management of GER post EA-TEF repair. Methods: A pre-piloted survey was handed out and collected at the 2012 CAPS annual meeting. Data were entered and coded, and descriptive statistics were calculated. Results: We distributed 70 surveys, and 57 (81.4%) surveys were returned. On average, the incidence of EA-TEF is 8-10 cases per institution, per year. Anti-reflux medication is started immediately post-operatively in 74% of patients at institution of feeds (11%), or if symptoms of reflux develop (14%). Proton pump inhibitors and H2-receptor antagonists are used in approximately equal proportion. Patients are typically kept on antireflux medication for 3-6 months (37%) or 6-12 months (35%). Conclusions: Most CAPS attendees treat postoperative GER prophylactically. However, there is no consistency in management strategy regarding which anti-reflux agent to use or for how long. A multi-centered study is required to establish a standardized protocol for the post-operative management of EA-TEF to prevent reflux and its effect on anastomotic strictures

Intrapleural Dornase and Tissue Plasminogen Activator in pediatric empyema (DTPA): a study protocol for a randomized controlled trial

Background: A randomized controlled trial of adults with empyema recently demonstrated decreased length of stay in hospital in patients treated with intrapleurally administered dornase alfa and fibrinolytics compared to fibrinolytics alone. Whether this treatment strategy is safe and effective in children remains unknown. Methods/design: This study protocol is for a superiority, placebo-controlled, parallel-design, multicenter randomized controlled trial. The participants are previously well children admitted to a children’s hospital with a diagnosis of empyema requiring chest tube insertion and fibrinolytics administered intrapleurally. Children will be randomized after the treating physician has decided that pleural drainage is required but prior to chest tube insertion. After chest tube insertion, participants in the treatment group will receive intrapleurally administered tissue plasminogen activator (tPA) 4 mg followed by dornase alfa 5 mg. Participants in the placebo group will receive tPA 4 mg followed by normal saline. Study treatments will be administered once daily for 3 days. All participants, parents or caregivers, clinicians, and research personnel will remain blinded. The primary outcome is length of stay from chest tube insertion to discharge from hospital. Secondary outcomes include time to meeting discharge criteria, chest tube duration, fever duration, need for additional procedures, adverse events, hospital readmission, cost of hospitalization, and mortality. Discussion: This multicenter randomized controlled trial will assess the safety, effectiveness, and cost-effectiveness of combined treatment with dornase alfa and fibrinolytics compared to fibrinolytics alone for the treatment of empyema in children. Trial registration: ClinicalTrials.gov: NCT01717742 . Registered on 8 October 2012.Medicine, Faculty ofNon UBCMedicine, Department ofPediatrics, Department ofRespiratory Medicine, Division ofReviewedFacult