Faculty Bibliography

INTRODUCTION/BACKGROUND:We sought to understand the impact of locum tenens surgeons on pediatric surgical care delivery. METHODS:We conducted a cross-sectional survey of Children's Hospital Association pediatric surgical practices. Anonymous electronic surveys were used to investigate locum tenens utilization, primary reason for use, limitations on clinical activities, and variations in practice standards or quality. Bivariate analysis and multivariable logistic regression were performed to evaluate for associations between practice characteristics and locum tenens use. RESULTS:Of 172 practices, 71% (n = 122) completed the survey. Median hospital size was 203 beds (interquartile range = 130-350). Median number of surgeons per practice was 5 (interquartile range = 3-8). Thirty-seven practices (30%) employed locum tenens at primary (n = 27) or satellite (n = 12) sites. Locum tenens utilization was higher in suburban (odds ratio [OR] = 3.78, P = 0.006) and rural (OR = 4.96, P = 0.041) locations and lower at sites with a level 4 neonatal intensive care unit (OR = 0.35, P = 0.035). Most (51%) used locum tenens ≥ 1 time monthly but < 1 time weekly and for ongoing or interim coverage (87%). In total, 14% of practices reported clinical restrictions for locum tenens surgeons, including limitations on extracorporeal membrane oxygenation, neonatal index cases, and operative trauma. Most (76%) practices using locum tenens reported variations in practice standards or quality; all were perceived as negative (57%) or neutral (43%). CONCLUSIONS:Locum tenens providers are utilized most commonly in suburban and rural sites and hospitals without the highest level of neonatal intensive care. While locum tenens surgeons may help maintain access to pediatric surgical care where gaps exist, there may be a need to improve the quality and reliability of care rendered.

Extracorporeal membrane oxygenation (ECMO) to support neonates and children with cardiopulmonary failure was first described in the 1970s, since which time its use has expanded to an increasingly complex and heterogenous pediatric population. Despite preserved survival outcomes, complications of ECMO use, including iatrogenic vascular injury, are common. Here, we provide a brief overview of the epidemiology of pediatric ECMO and associated vascular complications; describe common peripheral cannulation equipment and techniques, trends in cannulation and decannulation strategies, and respective incidence of vascular complications; and review existing evidence for best practices in cannula site selection, cannulation technique, decannulation strategies, and management of vascular complications, with the goal of providing a comprehensive review for interventionalists involved in the care of pediatric ECMO patients. Areas of wide practice variation in vessel management-application of vessel-sparing cervical venoarterial cannulation, the use of distal perfusion catheters in femoral arterial cannulation, and best practices for percutaneous single-lumen venovenous cannulation, as examples-areas of focus for future research, and the potential role of vascular surgeons and other subspecialty proceduralists in the care of pediatric ECMO patients are highlighted. LEVEL OF EVIDENCE: V.

OBJECTIVE:To examine survival and outcomes in neonates who received therapeutic hypothermia (TH) for neonatal encephalopathy (NE) and extracorporeal membrane oxygenation (ECMO) versus ECMO alone. STUDY DESIGN/METHODS:This is a retrospective review of Extracorporeal Life Support Organization (ELSO) Registry data from 2007 to 2017 for neonates undergoing ECMO and TH for NE (TH/ECMO) or ECMO alone. Primary outcomes were ECMO survival and survival to discharge. Secondary outcomes were complications while on ECMO. Statistical analysis was performed using Fisher's Exact and Mann-Whitney U testing. Multivariate regression was performed to identify predictors of ECMO survival. RESULTS:Of 3 672 neonates, 215 (6%) received TH/ECMO, while 3 457 (94%) received ECMO alone. There was no significant difference in ECMO survival (92% vs. 92%, P=0.70) or survival to discharge (87% vs. 85%, P=0.43) between groups. TH/ECMO group had higher hemorrhagic (29% vs. 20%, P<0.01), neurologic (24% vs.12%, P<0.01) , and metabolic (28% vs. 15%, p<0.01) complications. Multivariate regression identified higher gestational age, absence of inotropes during ECMO, and lack of neurologic, pulmonary, or hemorrhagic complications as independent predictors of ECMO survival. CONCLUSION/CONCLUSIONS:Neonates undergoing ECMO and TH for NE had survival rates comparable to those receiving ECMO alone. These findings suggest that ECMO can be considered for neonates with NE undergoing TH who meet criteria for ECMO.

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly often accompanied by other structural anomalies and/or neurobehavioral manifestations. Rare de novo protein-coding variants and copy-number variations contribute to CDH in the population. However, most individuals with CDH remain genetically undiagnosed. Here, we perform integrated de novo and common-variant analyses using 1,469 CDH individuals, including 1,064 child-parent trios and 6,133 ancestry-matched, unaffected controls for the genome-wide association study. We identify candidate CDH variants in 15 genes, including eight novel genes, through deleterious de novo variants. We further identify two genomic loci contributing to CDH risk through common variants with similar effect sizes among Europeans and Latinx. Both loci are in putative transcriptional regulatory regions of developmental patterning genes. Estimated heritability in common variants is ∼19%. Strikingly, there is no significant difference in estimated polygenic risk scores between isolated and complex CDH or between individuals harboring deleterious de novo variants and individuals without these variants. The data support a polygenic model as part of the CDH genetic architecture.

BACKGROUND:Radioactive iodine therapy (RAI) is a frequently chosen therapy for Graves' disease. The aim of this study was to determine whether RAI for Graves' disease increases the risk of thyroid malignancy. METHODS:A retrospective analysis was performed of all Graves' disease patients who underwent thyroidectomy at a single institution between 2013 and 2022. Comparative analyses were performed with cohorts based on RAI therapy as the primary grouping variable. RESULTS:413 patients were identified, of which 38 received RAI prior to surgery. RAI treated patients were more likely to undergo surgery for known malignancy or indeterminate nodules. RAI patients were also more likely to have malignancies larger than 1 ​cm. Among RAI treated patients, those who developed malignancy were older at the time of Graves' diagnosis and received early RAI therapy. CONCLUSIONS:Use of RAI for treatment of Graves' disease increases the progression of thyroid carcinoma, but not the prevalence. Older age and early RAI therapy may be risk factors for malignancy in RAI treated patients.

BACKGROUND:Radioactive iodine therapy (RAI) is a frequently chosen therapy for Graves' disease. The aim of this study was to determine whether RAI for Graves' disease increases the risk of thyroid malignancy. METHODS:A retrospective analysis was performed of all Graves' disease patients who underwent thyroidectomy at a single institution between 2013 and 2022. Comparative analyses were performed with cohorts based on RAI therapy as the primary grouping variable. RESULTS:413 patients were identified, of which 38 received RAI prior to surgery. RAI treated patients were more likely to undergo surgery for known malignancy or indeterminate nodules. RAI patients were also more likely to have malignancies larger than 1 ​cm. Among RAI treated patients, those who developed malignancy were older at the time of Graves' diagnosis and received early RAI therapy. CONCLUSIONS:Use of RAI for treatment of Graves' disease increases the progression of thyroid carcinoma, but not the prevalence. Older age and early RAI therapy may be risk factors for malignancy in RAI treated patients.

OBJECTIVE:Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS:The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS:A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS:Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

BACKGROUND:Significant variation in management strategies for lymphatic malformations (LMs) in children persists. The goal of this systematic review is to summarize outcomes for medical therapy, sclerotherapy, and surgery, and to provide evidence-based recommendations regarding the treatment. METHODS:Three questions regarding LM management were generated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Publicly available databases were queried to identify articles published from January 1, 1990, to December 31, 2021. A consensus statement of recommendations was generated in response to each question. RESULTS:The initial search identified 9326 abstracts, each reviewed by two authors. A total of 600 abstracts met selection criteria for full manuscript review with 202 subsequently utilized for extraction of data. Medical therapy, such as sirolimus, can be used as an adjunct with percutaneous treatments or surgery, or for extensive LM. Sclerotherapy can achieve partial or complete response in over 90% of patients and is most effective for macrocystic lesions. Depending on the size, extent, and location of the malformation, surgery can be considered. CONCLUSION/CONCLUSIONS:Evidence supporting best practices for the safety and effectiveness of management for LMs is currently of moderate quality. Many patients benefit from multi-modal treatment determined by the extent and type of LM. A multidisciplinary approach is recommended to determine the optimal individualized treatment for each patient.

Extracorporeal life support (ECLS) therapy is increasingly being used to support children with refractory cardiorespiratory failure, but its use is occasionally associated with complications.1 Neonatal aortic dissection in association with ECLS is rare and the clinical sequelae of aortic dissection in neonates are poorly understood. We report a case of extensive type B aortic dissection in a neonate receiving ECLS therapy for refractory cardiogenic shock secondary to tachycardia-induced cardiomyopathy and Wolf Parkinson White (WPW) syndrome. The patient was noted to have aortic dissection along with multiple abdominal organ ischemic injury a day after ECLS arterial cannula position adjustment. The patient was rapidly decannulated from ECLS and the aortic dissection was managed conservatively with good outcome. We discuss our approach and rationale behind conservative management of this rarely reported complication associated with ECLS therapy and discuss available literature.

INTRODUCTION/BACKGROUND:The diagnosis and management of biliary dyskinesia in children and adolescents remains variable and controversial. The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee (APSA OEBP) performed a systematic review of the literature to develop evidence-based recommendations. METHODS:Through an iterative process, the membership of the APSA OEBP developed five a priori questions focused on diagnostic criteria, indications for cholecystectomy, short and long-term outcomes, predictors of success/benefit, and outcomes of medical management. A systematic review was conducted, and articles were selected for review following Preferred Reporting Items for Systematic Review and Meta-analyses (PRISMA) guidelines. Risk of bias was assessed using Methodologic Index for Non-Randomized Studies (MINORS) criteria. The Oxford Levels of Evidence and Grades of Recommendation were utilized. RESULTS:The diagnostic criteria for biliary dyskinesia in children and adolescents are not clearly defined. Cholecystectomy may provide long-term partial or complete relief in some patients; however, there are no reliable predictors of symptom relief. Some patients may experience resolution of symptoms with non-operative management. CONCLUSIONS:Pediatric biliary dyskinesia remains an ill-defined clinical entity. Pediatric-specific guidelines are necessary to better characterize the condition, guide work-up, and provide management recommendations. Prospective studies are necessary to more reliably identify patients who may benefit from cholecystectomy. LEVEL OF EVIDENCE/METHODS:Level 3-4. TYPE OF STUDY/METHODS:Systematic Review of Level 3-4 Studies.