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Background/purpose: Cancer predisposition syndromes (CPS) are a heterogeneous group of inherited disorders that greatly increase the risk of developing malignancies. CPS are particularly relevant to pediatric surgeons since nearly 10% of cancer diagnoses are due to inherited genetic traits, and CPS often contribute to cancer development during childhood., Materials/methods: The English language literature was searched for manuscripts, practice guidelines, and society statements on "cancer predisposition syndromes in children". Following review of these manuscripts and cross-referencing of their bibliographies, tables were created to summarize findings of the most common CPS associated with surgically treated pediatric solid malignancies., Results: Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors, such as WT. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms' tumor, hepatoblastoma, and medullary thyroid cancer. Frequently associated non-tumor manifestations of these CPS are also included as a guide to increase surgeon awareness. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available., Conclusion: Pediatric surgeons play an important role as surgical oncologists and are often the first point of contact for children with solid tumors. In their role of delivering a diagnosis and developing a follow-up and treatment plan as part of a multidisciplinary team, familiarity with common CPS will ensure evidence-based practices are followed, including important principles such as organ preservation and intensified surveillance plans. This review defines and summarizes the CPS associated with common childhood solid tumors encountered by the pediatric surgeon, as well as common non-cancerous disease stigmata that may help guide diagnosis., Type of Study: Summary paper., Level of Evidence: 5., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background/purpose: Melanoma is the most common skin cancer in children and often presents in an atypical fashion when compared to adults. The purpose of this review is to present an update on the epidemiology, surgical and medical management and prevention strategies in pediatric melanoma., Methods: A comprehensive review of the current literature on the epidemiology, surgical and medical management and prevention of adult and pediatric melanoma was performed by the authors and the results of this review are summarized in the manuscript., Results: Most recently, the incidence of melanoma in children has been declining, possibly owing to increased awareness and sun exposure prevention. The mainstay of therapy is surgical resection, often with sentinel lymph node biopsy. A positive sentinel node has prognostic value; however, completion node dissection is no longer recommended in the absence of clinically or radiographically positive nodes. Those with advanced disease also receive adjuvant systemic therapy using increasingly targeted immunologic therapies., Conclusions: Sentinel lymph node positive patients no longer require completion lymph node dissection and instead may be followed by ultrasound. However, it is important to note that children have been excluded from most melanoma clinical trials to date, and therefore, recommendations for management are based on existing pediatric retrospective data and extrapolation from adult studies., Level of Evidence: IV., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Complex wounds associated with the Nuss procedure are a resource intensive complication that may lead to significant morbidity with potential removal of the implanted device and abandonment of the repair. We report our management technique of this complication utilizing microdeformational wound therapy (MDWT) that is safe, is efficacious and allows for salvage of the repair., Operative Technique: We defined a complex wound as a wound that became suppurative and drained in the postoperative period and failed to resolve with a trial of conventional wound management and antibiotics. Upon recognition of a complex wound, we recommend an initial operative wound debridement. This allows wound cultures, wound assessment and precise initiation of MDWT. It is not uncommon to have exposed hardware in the wound early in the course of therapy. Metal allergy must be excluded. The patient is transitioned to oral antibiotics following resolution of the acute process. MDWT is performed until the wounds are completely epithelialized with no clinical signs of drainage or infection. The average length of MDWT in our patients was 39 days. Following complete wound healing the patients are maintained on antibiotics until implant removal., Conclusions: The use of microdeformational wound therapy in complex wounds associated with the Nuss procedure is a safe and effective modality. The technique may reduce the likelihood of implant removal with potential recurrent pectus excavatum., Type of Study: Operative technique., Level of Evidence: Level IV, case series with no comparison group., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aims: Extracorporeal membrane oxygenation (ECMO) is a commonly used modality of life support for children with cardiopulmonary failure. Consensus on pediatric cannulation strategies and management does not currently exist. The goal of this study was to investigate individual surgeon approaches towards ECMO cannulations in children., Methods: A 21-question online survey was developed and disseminated to the American Pediatric Surgical Association (APSA) membership. Participant responses were summarized as counts and percentages. Effect of ECMO volume and surgeon experience on responses was assessed., Results: There were 252 APSA members who participated in this study for a response rate of 21%, with 225 (89.3%) performing ECMO. Sixty respondents (28.3%) reported using neck vessels exclusively for cannulation regardless of age or weight of the patient. After neck decannulation, 13 (6.6%) repaired the carotid artery for all patients, and 21 (10.7%) repaired only for children older than 5years. Of those performing femoral cannulation, 56 (26.4%) would perform at 5years or older and 66 (31.1%) at 12years. The most common challenge for femoral cannulation was the need for distal perfusion (n=119; 59.8%). Assistance from vascular surgery was requested by 32 (16.4%) for distal perfusion catheter placement, and by 79 (40.5%) for decannulation. Regarding femoral cannulation, lack of training was more likely to be a challenge if performing <5 cannulations per year (25.2% vs 12.5%; p=0.03). Surgeons with <10years of experience were more likely to consult vascular surgery compared to those with >10years of experience (18.5% vs 8%; p=0.03)., Conclusion: Considerable variation exists in individual surgeon cannulation practices in pediatric ECMO, in particular in the management of school age and adolescent VA ECMO. Mixed approaches across several ECMO management case study questions indicate that further work is needed to evaluate specific risks with cannulations in children., Level of Evidence: IV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background/purpose: Premature neonates can develop intraabdominal conditions requiring emergent bowel resection and enterostomy. Parenteral nutrition (PN) is often required, but results in cholestasis. Mucous fistula refeeding allows for functional restoration of continuity. We sought to determine the effect of refeeding on nutrition intake, PN dependence, and PN associated hepatotoxicity while evaluating the safety of this practice., Methods: A retrospective review of neonates who underwent bowel resection and small bowel enterostomy with or without mucous fistula over 2years was undertaken. Patients who underwent mucous fistula refeeding (RF) were compared to those who did not (OST). Primary outcomes included days from surgery to discontinuation of PN and goal enteral feeds, and total days on PN. Secondary outcomes were related to PN hepatotoxicity., Results: Thirteen RF and eleven OST were identified. There were no significant differences among markers of critical illness (p>0.20). In the interoperative period, RF patients reached goal enteral feeds earlier than OST patients (median 28 versus 43days; p=0.03) and were able to have PN discontinued earlier (median 25 versus 41days; p=0.04). Following anastomosis, the magnitude of effect was more pronounced, with RF patients reaching goal enteral feeds earlier than OST patients (median 7.5 versus 20days; p≤0.001) and having PN discontinued sooner (30.5 versus 48days; p=0.001)., Conclusions: RF neonates reached goal feeds and were able to be weaned from PN sooner than OST patients. A prospective multicenter trial of refeeding is needed to define the benefits and potential side effects of refeeding in a larger patient population in varied care environments., (Published by Elsevier Inc.)

Introduction: The intracorporeal placement and tying of suture (IT) can be challenging leading to prolonged CO2 insufflation, anesthesia, and potential morbidity. The unidirectional barbed knotless suture (V-LOC) has emerged as an innovative technology that has been shown to reduce the time associated with IT. Therefore, we conducted a retrospective analysis comparing our initial experience utilizing V-LOC to perform a novel continuous stitch laparoscopic fundoplication (CF) to standard laparoscopic Nissen fundoplication (NF)., Methods: Institutional review board approval was obtained to analyze data on patients who underwent V-LOC CF and NF. Data retrieval included age, gender, weight, diagnosis, procedure, operative time, major complications (reoperation for wrap failure/migration or recurrent symptoms), and follow up., Results: Twenty patients underwent the V-LOC CF and gastrostomy placement (GT) from January to October 2013. Seventeen patients underwent NF and GT from March 2012 to February 2013. There were no significant differences in age, weight, or incidence of major complications. V-LOC CF led to a significant 30% reduction in operative time compared to NF (79.1±24.2 min vs. 113.8±25.9 min, respectively, P<0.05)., Conclusions: This is the first report documenting the continuous stitch fundoplication utilizing the unidirectional barbed knotless suture in children. Although follow-up is short, the V-LOC CF appears to be a safe and effective technique that may reduce operative time in children with gastroesophageal reflux disease. This technology may be beneficial in other minimally invasive applications in pediatric surgery., (Copyright © 2015. Published by Elsevier Inc.)

Introduction: Improved surgical outcomes in children have been associated with pediatric surgical specialization, previously defined by surgeon operative volume or fellowship training. The present study evaluates pediatric surgical outcomes through classifying surgeons by degrees of pediatric versus adult operative experience., Methods: A cross-sectional study was performed using nationally representative hospital discharge data from 1998 to 2007. Patients under 18 years of age undergoing inpatient operations in neurosurgery, otolaryngology, cardiothoracic, general surgery, orthopedic surgery, and urology were included. An index was created, calculating the proportion of children treated by each surgeon. In-hospital mortality and length of stay were compared by index quartiles. Multivariate analysis was adjusted for patient and hospital characteristics., Results: A total of 119,164 patients were operated on by 13,141 surgeons. Within cardiothoracic surgery, there were 1.78 (p=0.02) and 2.61 (p<0.01) increased odds of mortality comparing surgeons in the lowest two quartiles for pediatric specialization respectively with the highest quartile. For general surgery, a 2.15 (p=0.04) increase in odds for mortality was found when comparing surgeons between the lowest and the highest quartiles. Comparing the least to the most specialized surgeons, length of stay increased 1.14 days (p=0.02) for cardiothoracic surgery, 0.58 days (p=0.04) for neurosurgery, 0.23 days (p=0.02) for otolaryngology, and decreased by 1.06 days (p<0.01) for orthopedic surgery., Conclusion: The present study demonstrates that surgeons caring preferentially for children-as a proportion of their overall practice-generally have improved mortality outcomes in general and cardiothoracic surgery. These data suggest a benefit associated with increased referral of children to pediatric practitioners, but further study is required., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background/purpose: The pediatric quality indicators (PDIs) were developed by the Agency for Healthcare Research and Quality to compare patient safety and quality of pediatric care. These are being considered for mandatory reporting as well as pay-for-performance efforts. The present study evaluates the PDIs' predictive value for surgical outcomes in children., Methods: A cross-sectional study was performed using nationwide inpatient data from 1988 to 2007. Patients younger than 18 years with an inpatient surgical procedure were included and evaluated for 10 PDIs. Odds ratios for mortality, increase in length of stay, and total charges were calculated using multivariate regression adjusting for age, sex, race, region, hospital type, and comorbidities., Results: A total of 1,964,456 pediatric discharges were included. Mortality rates were 5.4% for patients with at least 1 PDI and 0.6% for those with none. Multivariate analysis showed that occurrence of any PDI was associated with a 20% increased risk of mortality. The PDIs were associated with an increased length of stay and total hospital charges., Conclusion: The present study shows that PDIs are associated with increased mortality risk as well as increased hospital stay and total hospital charges. This provides positive evidence for the utility of these indicators as metrics for quality and patient safety., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Increasing national focus on patient safety has promoted development of the pediatric quality indicators (PDIs), which screen for preventable events during provision of health care for children. Our objective is to apply these safety metrics to compare 2 surgical procedures in children, specifically laparoscopic and open esophagogastric fundoplication for gastroesophageal reflux., Methods: A retrospective analysis using 20 years of data from national representative state inpatient databases through the Healthcare Cost and Utilization Project was conducted. Patients younger than 18 years with International Classification of Diseases, Ninth Revision, Clinical Modification, codes for open or laparoscopic esophagogastric fundoplication were included. Pediatric quality indicators were linked to each patient's profile. Demographics, comorbidities, outcomes, and 8 selected PDIs between open and laparoscopic fundoplications were compared using Pearson χ(2) tests and t tests., Results: Of 33,533 patients identified, 28,141 underwent open and 5392 underwent laparoscopic fundoplication. Comorbidities occurred more frequently in open surgery. In-hospital mortality, length of stay, and hospital charges were less in laparoscopic surgery. Of the 8 PDIs evaluated, decubitus ulcer (P = .04) and postoperative sepsis (P = .003) had decreased rates with laparoscopic surgery compared with open., Conclusion: Laparoscopic fundoplication for gastroesophageal reflux in children can be performed safely compared with the open approach with equivalent or improved rates of PDIs., (Copyright © 2011 Elsevier Inc. All rights reserved.)