Reducing Unnecessary Oophorectomies for Benign Ovarian Neoplasms in Pediatric Patients.

Importance: Although most ovarian masses in children and adolescents are benign, many are managed with oophorectomy, which may be unnecessary and can have lifelong negative effects on health. Objective: To evaluate the ability of a consensus-based preoperative risk stratification algorithm to discriminate between benign and malignant ovarian pathology and decrease unnecessary oophorectomies. Design, Setting, and Participants: Pre/post interventional study of a risk stratification algorithm in patients aged 6 to 21 years undergoing surgery for an ovarian mass in an inpatient setting in 11 children's hospitals in the United States between August 2018 and January 2021, with 1-year follow-up. Intervention: Implementation of a consensus-based, preoperative risk stratification algorithm with 6 months of preintervention assessment, 6 months of intervention adoption, and 18 months of intervention. The intervention adoption cohort was excluded from statistical comparisons. Main Outcomes and Measures: Unnecessary oophorectomies, defined as oophorectomy for a benign ovarian neoplasm based on final pathology or mass resolution. Results: A total of 519 patients with a median age of 15.1 (IQR, 13.0-16.8) years were included in 3 phases: 96 in the preintervention phase (median age, 15.4 [IQR, 13.4-17.2] years; 11.5% non-Hispanic Black; 68.8% non-Hispanic White); 105 in the adoption phase; and 318 in the intervention phase (median age, 15.0 [IQR, 12.9-16.6)] years; 13.8% non-Hispanic Black; 53.5% non-Hispanic White). Benign disease was present in 93 (96.9%) in the preintervention cohort and 298 (93.7%) in the intervention cohort. The percentage of unnecessary oophorectomies decreased from 16.1% (15/93) preintervention to 8.4% (25/298) during the intervention (absolute reduction, 7.7% [95% CI, 0.4%-15.9%]; P = .03). Algorithm test performance for identifying benign lesions in the intervention cohort resulted in a sensitivity of 91.6% (95% CI, 88.5%-94.8%), a specificity of 90.0% (95% CI, 76.9%-100%), a positive predictive value of 99.3% (95% CI, 98.3%-100%), and a negative predictive value of 41.9% (95% CI, 27.1%-56.6%). The proportion of misclassification in the intervention phase (malignant disease treated with ovary-sparing surgery) was 0.7%. Algorithm adherence during the intervention phase was 95.0%, with fidelity of 81.8%. Conclusions and Relevance: Unnecessary oophorectomies decreased with use of a preoperative risk stratification algorithm to identify lesions with a high likelihood of benign pathology that are appropriate for ovary-sparing surgery. Adoption of this algorithm might prevent unnecessary oophorectomy during adolescence and its lifelong consequences. Further studies are needed to determine barriers to algorithm adherence.

Use and Accuracy of Intraoperative Frozen Section Analysis for Ovarian Masses in Children and Adolescents.

STUDY OBJECTIVE: Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian masses DESIGN: Prospective cohort study from 2018 to 2021 SETTING: Eleven children's hospitals PARTICIPANTS: Females age 6-21 years undergoing surgical management of an ovarian mass INTERVENTIONS: Obtaining intraoperative FS pathology MAIN OUTCOME MEASURE: Diagnostic accuracy of FS pathology RESULTS: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (44.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensitivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor (n = 1), mucinous borderline tumor (n = 2), mucinous carcinoma (n = 1), and immature teratoma (n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (44.4%) had malignant final pathology. CONCLUSIONS: FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses.

Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium.

BACKGROUND/PURPOSE: Controversy persists regarding the ideal surgical approach for repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We examined complications and outcomes of infants undergoing thoracoscopy and thoracotomy for repair of Type C EA/TEF using propensity score-based overlap weights to minimize the effects of selection bias. METHODS: Secondary analysis of two databases from multicenter retrospective and prospective studies examining outcomes of infants with proximal EA and distal TEF who underwent repair at 11 institutions was performed based on surgical approach. Regression analysis using propensity score-based overlap weights was utilized to evaluate outcomes of patients undergoing thoracotomy or thoracoscopy for Type C EA/TEF repair. RESULTS: Of 504 patients included, 448 (89%) underwent thoracotomy and 56 (11%) thoracoscopy. Patients undergoing thoracoscopy were more likely to be full term (37.9 vs. 36.3 weeks estimated gestational age, p < 0.001), have a higher weight at operative repair (2.9 vs. 2.6 kg, p < 0.001), and less likely to have congenital heart disease (16% vs. 39%, p < 0.001). Postoperative stricture rate did not differ by approach, 29 (52%) thoracoscopy and 198 (44%) thoracotomy (p = 0.42). Similarly, there was no significant difference in time from surgery to stricture formation (p > 0.26). Regression analysis using propensity score-based overlap weighting found no significant difference in the odds of vocal cord paresis or paralysis (OR 1.087 p = 0.885), odds of anastomotic leak (OR 1.683 p = 0.123), the hazard of time to anastomotic stricture (HR 1.204 p = 0.378), or the number of dilations (IRR 1.182 p = 0.519) between thoracoscopy and thoracotomy. CONCLUSION: Infants undergoing thoracoscopic repair of Type C EA/TEF are more commonly full term, with higher weight at repair, and without congenital heart disease as compared to infants repaired via thoracotomy. Utilizing propensity score-based overlap weighting to minimize the effects of selection bias, we found no significant difference in complications based on surgical approach. However, our study may be underpowered to detect such outcome differences owing to the small number of infants undergoing thoracoscopic repair. LEVEL OF EVIDENCE: Level III.

Natural history and consequence of patent processus vaginalis: An interim analysis from a multi-institutional prospective observational study.

BACKGROUND: The prevalence and natural history of patent processus vaginalis (PPV) are unknown. An interim analysis was performed of a multi-institutional, prospective, observational study in neonates undergoing laparoscopic pyloromyotomy during which bilateral inguinal canals were evaluated. METHODS: Infants under 4 months undergoing laparoscopic pyloromyotomy were enrolled at 8 children's hospitals. The presence of a PPV was evaluated and measurements recorded. Patients with a PPV are undergoing annual phone follow-up to 18 years of age. Interim analysis was performed. RESULTS: In a cohort of 610 patient, 80 did not have a PPV examined, 4 had consent issues and were excluded, leaving 526 patients. Of these, 433 (82%) were male, median age 1.2 months (IQR 0.9, 1.6), median weight 3.89 kg (IQR 3.4, 4.46), and EGA 39 weeks (IQR 37, 40). There were 283 PPVs, 132 bilateral (47%), 116 right (41%), and 35 left (12%). Patients with a PPV were significantly younger (1.1 months (IQR 0.9, 1.5) vs 1.3 months (IQR 0.9, 1.7), p=0.02), weighed less (3.76kg (IQR 3.35, 4.26) vs 3.9kg (IQR 3.4, 4.5) p=0.03) and had a significantly lower EGA at birth (38 weeks (IQR 37, 40) vs 39 weeks (IQR 38, 40) p=0.003). Of 246 eligible infants, 208 (85%) responded to at least one annual follow-up. Two patients had an inguinal hernia repair for a symptomatic hernia, 49- and 51-days post pyloromyotomy. One had an orchiopexy and incidental inguinal hernia repair 120 days post pyloromyotomy; for a total of 3 (1.2%) hernia repairs. No additional hernias were identified in 116 patients with the PPV patients who have been followed for > 1 year. CONCLUSIONS: The presence of a PPV at the time of pyloromyotomy is common but the need for hernia repair is rare within the first year of life. Continued long-term longitudinal follow-up of this cohort is needed. LEVEL OF EVIDENCE: II.

Disparities in the Management of Pediatric Breast Masses.

INTRODUCTION: Disparities in surgical management have been documented across a range of disease processes. The objective of this study was to investigate sociodemographic disparities in young females undergoing excision of a breast mass. METHODS: A retrospective study of females aged 10-21 y who underwent surgery for a breast lesion across eleven pediatric hospitals from 2011 to 2016 was performed. Differences in patient characteristics, workup, management, and pathology by race/ethnicity, insurance status, median neighborhood income, and urbanicity were evaluated with bivariate and multivariable regression analyses. RESULTS: A total of 454 females were included, with a median age of 16 y interquartile range (IQR: 3). 44% of patients were nonHispanic (NH) Black, 40% were NH White, and 7% were Hispanic. 50% of patients had private insurance, 39% had public insurance, and 9% had other/unknown insurance status. Median neighborhood income was $49,974, and 88% of patients resided in a metropolitan area. NH Whites have 4.5 times the odds of undergoing preoperative fine needle aspiration or core needle biopsy compared to NH Blacks (CI: 2.0, 10.0). No differences in time to surgery from the initial imaging study, size of the lesion, or pathology were observed on multivariable analysis. CONCLUSIONS: We found no significant differences by race/ethnicity, insurance status, household income, or urbanicity in the time to surgery after the initial imaging study. The only significant disparity noted on multivariable analysis was NH White patients were more likely to undergo preoperative biopsy than were NH Black patients; however, the utility of biopsy in pediatric breast masses is not well established.

Virtual curriculum delivery in the COVID-19 era: the pediatric surgery boot camp v2.0.

PURPOSE: We evaluated the impact of a virtual Pediatric Surgery Bootcamp curriculum on resource utilization, learner engagement, knowledge retention, and stakeholder satisfaction. METHODS: A virtual curriculum was developed around Pediatric Surgery Milestones. GlobalCastMD delivered pre-recorded and live content over a single 10-h day with a concluding social hour. Metrics of learner engagement, faculty interaction, knowledge retention, and satisfaction were collected and analyzed during and after the course. RESULTS: Of 56 PS residencies, 31 registered (55.4%; 8/8 Canadian and 23/48 US; p = 0.006), including 42 learners overall. The virtual BC budget was $15,500 (USD), 54% of the anticipated in-person course. Pre- and post-tests were administered, revealing significant knowledge improvement (48.6% [286/589] vs 66.9% [89/133] p < 0.0002). Learner surveys (n = 14) suggested the virtual BC facilitated fellowship transition (85%) and strengthened peer-group camaraderie (69%), but in-person events were still favored (77%). Program Directors (PD) were surveyed, and respondents (n = 22) also favored in-person events (61%). PDs not registering their learners (n = 7) perceived insufficient value-added and concern for excessive participants. CONCLUSIONS: The virtual bootcamp format reduced overall expenses, interfered less with schedules, achieved more inclusive reach, and facilitated content archiving. Despite these advantages, learners and program directors still favored in-person education. LEVEL OF EVIDENCE: III.

Guidance for Structuring a Pediatric Intermediate Care Unit.

The purpose of this policy statement is to update the 2004 American Academy of Pediatrics clinical report and provide enhanced guidance for institutions, administrators, and providers in the development and operation of a pediatric intermediate care unit (IMCU). Since 2004, there have been significant advances in pediatric medical, surgical, and critical care that have resulted in an evolution in the acuity and complexity of children potentially requiring IMCU admission. A group of 9 clinical experts in pediatric critical care, hospital medicine, intermediate care, and surgery developed a consensus on priority topics requiring updates, reviewed the relevant evidence, and, through a series of virtual meetings, developed the document. The intended audience of this policy statement is broad and includes pediatric critical care professionals, pediatric hospitalists, pediatric surgeons, other pediatric medical and surgical subspecialists, general pediatricians, nurses, social workers, care coordinators, hospital administrators, health care funders, and policymakers, primarily in resource-rich settings. Key priority topics were delineation of core principles for an IMCU, clarification of target populations, staffing recommendations, and payment.

Evaluating the risk of peri-umbilical hernia after sutured or sutureless gastroschisis closure.

INTRODUCTION: We evaluate the incidence, outcomes, and management of peri­umbilical hernias after sutured or sutureless gastroschisis closure. METHODS: A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri­umbilical hernia, time to spontaneous closure or repair, and associated complications. RESULTS: Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p< 0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p< 0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure. CONCLUSION: Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach. LEVELS OF EVIDENCE: Level II.

Acid suppression duration does not alter anastomotic stricture rates after esophageal atresia with distal tracheoesophageal fistula repair: A prospective multi-institutional cohort study.

INTRODUCTION: Anastomotic stricture is the most common complication after esophageal atresia (EA) repair. We sought to determine if postoperative acid suppression is associated with reduced stricture formation. METHODS: A prospective, multi-institutional cohort study of infants undergoing primary EA repair from 2016 to 2020 was performed. Landmark analysis and multivariate Cox regression were used to explore if initial duration of acid suppression was associated with stricture formation at hospital discharge (DC), 3-, 6-, and 9-months postoperatively. RESULTS: Of 156 patients, 79 (51%) developed strictures and 60 (76%) strictures occurred within three months following repair. Acid suppression was used in 141 patients (90%). Landmark analysis showed acid suppression was not associated with reduction in initial stricture formation at DC, 3-, 6- and 9-months, respectively (p = 0.19-0.95). Multivariate regression demonstrated use of a transanastomotic tube was significantly associated with stricture formation at DC (Hazard Ratio (HR) = 2.21 (95% CI 1.24-3.95, p<0.01) and 3-months (HR 5.31, 95% CI 1.65-17.16, p<0.01). There was no association between acid suppression duration and stricture formation. CONCLUSION: No association between the duration of postoperative acid suppression and anastomotic stricture was observed. Transanastomotic tube use increased the risk of anastomotic strictures at hospital discharge and 3 months after repair.

Inclusion and representation in the pediatric surgery workforce: Strategies to mitigate bias in the fellowship application process.

Diverse perspectives are critical components of effective teams in every industry. Underrepresentation of minorities in medicine leads to worse outcomes for minority patients, and efforts to increase diversity in the health care workforce are critical. Presently, about 70% of the pediatric surgery workforce is white, and pediatric surgeons at large do not reflect the racial or ethnic diversity of the populations they serve. Pediatric surgery fellowship training programs are the gateway to the field, and fellow selection processes should be optimized to support diversity and inclusion. The Association of Pediatric Surgery Training Program Directors (APSTPD) Diversity Equity and Inclusion subcommittee compiled best practices for bias mitigation during fellow selection, drawing from published literature and personal experiences in our own programs. A list of concrete recommendations was compiled, which can be implemented in every phase from applicant screening to rank list creation. We present these as a position statement that has been endorsed by the executive committee of the APSTPD. Pediatric surgery fellowship programs can utilize this focused review of best practices to mitigate bias and support diverse applicants.

Success in pediatric surgery: An updated survey of Program Directors 2020.

BACKGROUND: One of the most competitive surgical sub-specialty fellowships remains Pediatric Surgery (PS), which requires candidates to develop a strong and research-oriented curriculum vitae. Although some objective factors of matriculation are known, factors for the interview selection and ranking per the program directors (PDs) have not been reviewed in over a decade. METHODS: A web-based survey of US and Canadian PS program directors (PDs) (n = 58) was used to evaluate a comprehensive list of factors in the selection criteria for PS fellowships. A mix of dichotomous, ranking, five-point Likert scale, and open-ended questions evaluated applicant characteristics, ABSITE scores, research productivity, interview day, and rank order criteria. RESULTS: Fifty-five programs responded to the survey for a 95% participation rate. PDs desired an average of two years in dedicated research and weighted first authorship and total number of publications heavily. Only 38% of programs used an ABSITE score cutoff for offering interviews; however, the majority agreed that an overall upward trend was important. Quality letters of recommendation, especially from known colleagues, carried weight when deciding to offer interviews. Interview performance, being a team player, observed interpersonal interactions, perceived operative skills and patient care, and leadership were some of the notable factors when finalizing rank lists. CONCLUSIONS: A multitude of factors define a successful matriculant, including quality of letters of recommendation, quality and quantity of publications, supportive phone calls, observed interactions, interview performance, perceptions of being team player with leadership skills as well as perceptions of good operative skills and patient care. LEVEL OF EVIDENCE: Type II. TYPE OF STUDY: Prognostic (retrospective).

Management of complicated biliary disease in the pediatric population.

BACKGROUND: Cholesterol stones and biliary dyskinesia have replaced hemolytic disease as the primary indication for pediatric cholecystectomy. This study looks at the cohort of pediatric patients with complicated biliary disease, defined as choledocholithiasis and/or gallstone pancreatitis, to determine the incidence and best treatment options. METHODS: A retrospective review of all cholecystectomies performed over 15 years admitted to the surgical service at a single free-standing children's hospital was performed. Patient factors, indications for cholecystectomy, and final treatment were recorded. Complicated gallbladder disease was defined as having image-confirmed choledocholithiasis or gallstone pancreatitis. High-risk patients were those with imaging that demonstrated definitive choledocholithiasis or cholelithiasis with common bile duct enlargement. Low risk patients were those with cholelithiasis or gallbladder sludge on imaging combined with an elevated bilirubin and/or lipase. RESULTS: A total of 695 cholecystectomies were performed over the 15-year time period. Average patient age was 13.4 years. Of the 695 cholecystectomies, 457 were performed for stone disease (66%) (64 hemolytic) and 236 (34.0%) were performed for biliary dyskinesia. Hundred and three (14.8% of all cholecystectomies, 22.5% of those with stone disease) presented with choledocholithiasis and/or gallstone pancreatitis (complicated disease). In high-risk patients, 28/47 (59.6%) underwent endoscopic retrograde cholangiopancreatography/sphincterotomy. In low-risk patients (no choledocholithiasis or common duct enlargement), 13/56 (23.2%) required endoscopic retrograde cholangiopancreatography/sphincterotomy (P < .05). The indication for endoscopic retrograde cholangiopancreatography after cholecystectomy was choledocholithiasis and none of these patients had bile leak complications. CONCLUSION: The incidence of pediatric complicated biliary disease due to cholesterol stones is equal to that of adults. These data suggest that a patient with imaging evidence of choledocholithiasis or common bile duct enlargement may require endoscopic retrograde cholangiopancreatography, dependent on clinical course, and this should be strongly considered before cholecystectomy. Those without such radiographic findings can undergo laparoscopic cholecystectomy and have postoperative endoscopic retrograde cholangiopancreatography if needed.

Does Use of a Feeding Protocol Change Outcomes in Gastroschisis? A Report from the Midwest Pediatric Surgery Consortium.

INTRODUCTION: Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol. MATERIALS AND METHODS: A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed.Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted. RESULTS: Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p = 0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p = 0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences. CONCLUSION: Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes.

Is the changing landscape of fellowship recruitment during COVID-19 here to stay?

BACKGROUND: The 2020 Pediatric Surgery (PS) fellowship selection process was heavily impacted by the COVID-19 pandemic. A review of lessons learned can help determine best practices for the future. The purpose of the study was to analyze the virtual interview experience and assess opportunities to improve the post-pandemic fellowship recruitment process. STUDY DESIGN: Using a 28-question survey of Program Directors (PDs) of PS fellowships as well as a 44-question survey of applicants to PS fellowships in the US and Canada, we gathered information on the recruitment process during the COVID-19 pandemic (2020). Dichotomous, multiple choice and open-ended questions about the changes in process, platforms used, format, comparison to on-site interviews and overall satisfaction were used for objective and subjective feedback. RESULTS: A 95% participation rate was recorded for the PD survey. 24 out of 55 programs (44%) changed their on-site interviews to virtual format due to the pandemic. Most PDs described their overall impression of virtual interviews as satisfactory (66%, 16/24) and did not have an impact on the applicant's success in the match (35/54; 65%). About 50% of PDs preferred to have on-site interviews with virtual screening in the future. While the participation rate from applicants was much less (26 of 70), responses confirmed our survey results. Majority preferred on-site interviews (17/26), 6 of which preferred virtual screening followed by on-site interviews. CONCLUSION: Components of virtual screening and interviews were found to have benefits financially and from both time and stress perspectives, and thus might survive past the pandemic. LEVELS OF EVIDENCE LEVEL IV: .

Outcomes in gastroschisis: expectations in the postnatal period for simple vs complex gastroschisis.

OBJECTIVE: To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling. STUDY DESIGN: A retrospective study of 394 neonates with gastroschisis at 11 children's hospitals from January 2013 to March 2017 was performed. Analysis by Fisher's exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported. RESULT: There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%). CONCLUSION: This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes.

Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

Management of Pediatric Breast Masses: A Multi-institutional Retrospective Cohort Study.

BACKGROUND: The objective of our study was to describe the workup, management, and outcomes of pediatric patients with breast masses undergoing operative intervention. MATERIALS AND METHODS: A retrospective cohort study was conducted of girls 10-21 y of age who underwent surgery for a breast mass across 11 children's hospitals from 2011 to 2016. Demographic and clinical characteristics were summarized. RESULTS: Four hundred and fifty-three female patients with a median age of 16 y (IQR: 3) underwent surgery for a breast mass during the study period. The most common preoperative imaging was breast ultrasound (95%); 28% reported the Breast Imaging Reporting and Data System (BI-RADS) classification. Preoperative core biopsy was performed in 12%. All patients underwent lumpectomy, most commonly due to mass size (45%) or growth (29%). The median maximum dimension of a mass on preoperative ultrasound was 2.8 cm (IQR: 1.9). Most operations were performed by pediatric surgeons (65%) and breast surgeons (25%). The most frequent pathology was fibroadenoma (75%); 3% were phyllodes. BI-RADS scoring ≥4 on breast ultrasound had a sensitivity of 0% and a negative predictive value of 93% for identifying phyllodes tumors. CONCLUSIONS: Most pediatric breast masses are self-identified and benign. BI-RADS classification based on ultrasound was not consistently assigned and had little clinical utility for identifying phyllodes.

The effects of early anesthesia on neurodevelopment: A systematic review.

BACKGROUND: There is growing concern regarding the impact of general anesthesia on neurodevelopment in children. Pre-clinical animal studies have linked anesthetic exposure to abnormal central nervous system development, but it is unclear whether these results translate into humans. The purpose of this systematic review from the American Pediatric Surgical Association (APSA) Outcomes and Evidence-Based Practice (OEBP) Committee was to review, summarize, and evaluate the evidence regarding the neurodevelopmental impact of general anesthesia on children and identify factors that may affect the risk of neurotoxicity. METHODS: Medline, Cochrane, Embase, Web of Science, and Scopus databases were queried for articles published up to and including December 2017 using the search terms "general anesthesia and neurodevelopment" as well as specific anesthetic agents. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to screen manuscripts for inclusion in the review. A consensus statement of recommendations in response to each study question was synthesized based upon the best available evidence. RESULTS: In total, 493 titles were initially identified, with 56 articles selected for full analysis and 44 included for review. Based on currently available developmental assessment tools, a single exposure to general anesthesia does not appear to have a significant effect on general neurodevelopment, although prolonged or multiple anesthetic exposures may have some adverse effects. Exposure to general anesthesia may affect different domains of development at different ages. Regional anesthetic techniques with the addition of dexmedetomidine and/or some intravenous agents may mitigate the risks of neurotoxicity. This approach may be performed safely in some patients and can be considered as an option in selected short procedures. CONCLUSION: There is no conclusive evidence that a single short anesthetic in infancy has a detectable neurodevelopmental effect. Data do not support waiting until later in childhood to perform general anesthesia for single short procedures. With the complexities and nuances of different anesthetic methods, patients and procedures, the planning and execution of anesthesia for the pediatric patient is generally best accomplished by an anesthesiologist, ideally a pediatric anesthesiologist. TYPE OF STUDY: Systematic review of level 1-4 studies. LEVEL OF EVIDENCE: Level 1-4 (mainly level 3-4).

Management of intussusception in children: A systematic review.

OBJECTIVE: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to develop recommendations for the management of ileocolic intussusception in children. METHODS: The ClinicalTrials.gov, Embase, PubMed, and Scopus databases were queried for literature from January 1988 through December 2018. Search terms were designed to address the following topics in intussusception: prophylactic antibiotic use, repeated enema reductions, outpatient management, and use of minimally invasive techniques for children with intussusception. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived based on the best available evidence. RESULTS: A total of 83 articles were analyzed and included for review. Prophylactic antibiotic use does not decrease complications after radiologic reduction. Repeated enema reductions may be attempted when clinically appropriate. Patients can be safely observed in the emergency department following enema reduction of ileocolic intussusception, avoiding hospital admission. Laparoscopic reduction is often successful. CONCLUSIONS: Regarding intussusception in hemodynamically stable children without critical illness, pre-reduction antibiotics are unnecessary, non-operative outpatient management should be maximized, and minimally invasive techniques may be used to avoid laparotomy. LEVEL OF EVIDENCE: Level 3-5 (mainly level 3-4) TYPE OF STUDY: Systematic Review of level 1-4 studies.

Guidelines for Opioid Prescribing in Children and Adolescents After Surgery: An Expert Panel Opinion.

Importance: Opioids are frequently prescribed to children and adolescents after surgery. Prescription opioid misuse is associated with high-risk behavior in youth. Evidence-based guidelines for opioid prescribing practices in children are lacking. Objective: To assemble a multidisciplinary team of health care experts and leaders in opioid stewardship, review current literature regarding opioid use and risks unique to pediatric populations, and develop a broad framework for evidence-based opioid prescribing guidelines for children who require surgery. Evidence Review: Reviews of relevant literature were performed including all English-language articles published from January 1, 1988, to February 28, 2019, found via searches of the PubMed (MEDLINE), CINAHL, Embase, and Cochrane databases. Pediatric was defined as children younger than 18 years. Animal and experimental studies, case reports, review articles, and editorials were excluded. Selected articles were graded using tools from the Oxford Centre for Evidence-based Medicine 2011 levels of evidence. The Appraisal of Guidelines for Research & Evaluation (AGREE) II instrument was applied throughout guideline creation. Consensus was determined using a modified Delphi technique. Findings: Overall, 14 574 articles were screened for inclusion, with 217 unique articles included for qualitative synthesis. Twenty guideline statements were generated from a 2-day in-person meeting and subsequently reviewed, edited, and endorsed externally by pediatric surgical specialists, the American Pediatric Surgery Association Board of Governors, the American Academy of Pediatrics Section on Surgery Executive Committee, and the American College of Surgeons Board of Regents. Review of the literature and guideline statements underscored 3 primary themes: (1) health care professionals caring for children who require surgery must recognize the risks of opioid misuse associated with prescription opioids, (2) nonopioid analgesic use should be optimized in the perioperative period, and (3) patient and family education regarding perioperative pain management and safe opioid use practices must occur both before and after surgery. Conclusions and Relevance: These are the first opioid-prescribing guidelines to address the unique needs of children who require surgery. Health care professionals caring for children and adolescents in the perioperative period should optimize pain management and minimize risks associated with opioid use by engaging patients and families in opioid stewardship efforts.