Faculty Bibliography

OBJECTIVE:Consensus on functional outcomes for anorectal malformations (ARM) is hindered by the heterogeneity of the available literature. Optimal patient counseling includes discussion of short- and long-term outcomes for bowel and urinary continence, sexual and psychosocial function, transitional care, and quality of life. This systematic review examines and summarizes the current literature available related to functional outcomes for children with ARM. METHODS:The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted consensus-based questions regarding anorectal malformations. Pertinent articles from 1985 to 2021 were reviewed. RESULTS:More than 10,843 publications were reviewed with 109 being included in the final recommendations. Recommendations are primarily based on C-D levels of evidence. Continence and constipation rates were higher in patients with perineal fistula and rectovestibular fistula, although symptoms tended to improve as patients got older. Urological anomalies are common and longer term urologic surveillance protocols for patients with ARM need to be further outlined. Sexual and psychosocial issues are common, but ARM patients can have a good quality of life when gastrointestinal symptoms are minimized. Many of the problems associated with ARM can persist into adulthood, supporting structured care plans as patients transition to adult care. CONCLUSIONS:Evidence to support best practices and achieve optimal outcomes for patients with ARM is lacking for many aspects of care. Multi-institutional registries have begun to address management and prognosis for these patients. Prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

INTRODUCTION/BACKGROUND:Ergonomic injuries pose significant risks to surgeons, affecting health, productivity, care access, and retirement age. Despite unique challenges in pediatric surgery, including varied patient sizes and operations, little is known about pediatric surgeons' ergonomics. This study aimed to assess ergonomic practices and associated injuries among pediatric surgeons. METHODS:A cross-sectional survey was distributed to the American Pediatric Surgical Association regular members and fellows. Data collected included demographics, physical health, surgical practices, operating habits, discomfort, injuries, interventions, and outcomes. Associations with injury were analyzed using Fisher's exact test, Pearson's Chi-squared test, and Wilcoxon rank-sum tests. RESULTS:One hundred seventeen (11%) surgeons responded, 53% were male with a median of 15 y in practice (interquartile range: 6-25). Regarding operating habits, 76% did not take regular breaks, 48% double-gloved, and 51% used loupes regularly. Notably, 90% experienced discomfort or pain, and 30% sustained injuries from operating, primarily affecting the neck and cervical spine (53%). White-identifying pediatric surgeons (80%) reported significantly more ergonomic injuries than other races (P < 0.01). Only 18% of respondents received ergonomic training. Ergonomics training and operating with a resident or co-surgeon were associated with less injury (P < 0.05). Among those experiencing discomfort or injury, 13% underwent a procedure, 63% experienced sleep disturbance, 74% reported contribution to burnout, and 88% used pain medications. CONCLUSIONS:Ergonomic-related discomfort and injuries occurred in nearly 90% of pediatric surgeons who responded. Few had ergonomic training and most reported an impact on well-being. Modifiable ergonomic factors for pediatric surgeons, along with targeted interventions to reduce injuries, can improve surgeon well-being.

BACKGROUND:Four-dimensional computed tomography is routinely used to localize parathyroid disease, with consistently excellent parathyroid gland localization rates reported. This study evaluated whether pairing 4-dimensional computed tomography results with preoperative clinical variables can accurately predict single-gland disease in primary hyperparathyroidism. METHODS:Patients with primary hyperparathyroidism who underwent both 4-dimensional computed tomography imaging and parathyroidectomy between January 2019 and September 2021 at a large academic health system were included. Patient demographics, preoperative characteristics, and peri- and postoperative data were collected. The accuracy of 4-dimensional computed tomography in correctly identifying patients with single-gland disease with and without preoperative calcium and parathyroid hormone levels was calculated. Single-gland disease was defined by intraoperative parathyroid hormone decrease >50% and a hypercellular gland on pathology. RESULTS:One hundred seventy-five patients had 4-dimensional computed tomography results suggestive of single gland disease. One hundred fifty-two patients (87%) were predicted correctly to have single-gland disease. The predictive accuracy increased when stratifying by preoperative calcium (≥10.5 mg/dL, ≥11 mg/dL, and ≥12 mg/dL) and parathyroid hormone levels (≥65 pg/mL, ≥100 pg/mL, and ≥200 pg/dL). The accuracy further increased when stratifying by age (≤50 years). Accuracy for single gland disease was 100% when combined with any of the following: (1) calcium ≥12 mg/dL, (2) parathyroid hormone ≥200 pg/dL, or (3) calcium ≥11 mg/dL in patients ≤50 years. CONCLUSION/CONCLUSIONS:Four-dimensional computed tomography alone accurately predicted single gland disease in 87% of patients with primary hyperparathyroidism. When combined with preoperative calcium, parathyroid hormone and age thresholds, predictive accuracy for single-gland disease approached 100%. Given the high likelihood of single-gland disease in these scenarios, clinicians may consider offering focused unilateral parathyroidectomy without intraoperative parathyroid hormone monitoring in selected patients.

BACKGROUND AND OBJECTIVES/OBJECTIVE:Diagnosis of adnexal torsion is challenging due to variable clinical presentations and often inconclusive imaging results. We hypothesized that diagnostic delays are common, leading to prolonged ischemia and subsequent tissue loss. We aimed to identify factors associated with diagnostic delays in pediatric patients with adnexal torsion. METHODS:We performed a multi-institutional retrospective review of females aged 5 to 18 years with confirmed adnexal torsion between 2013 to 2022. Delay to care was defined as prior emergency department discharge within 7 days of operation and/or hospital admission without initial plan for operation. RESULTS:862 patients were identified from 10 children's hospitals, with delayed diagnosis in 30%. Patients with delay were less likely to present with emesis or fever, have initial pediatric surgery consultation, or have typical ultrasound findings of torsion compared to those without delay (P < .05). For every unit increase in area deprivation index, the odds of delay increased by 1.3% (odds ratio 1.013, 95% CI, 1.007-1.018). The odds of delay were 81% greater for patients living > 30 miles from the hospital compared with 1-10 miles (odds ratio 1.812, 95% CI, 1.236-2.657). Oophorectomy and salpingectomy rates were 10% and 13%; those with delay had higher risk of oophorectomy (14% vs 7%, P = .002). CONCLUSION/CONCLUSIONS:Delayed diagnosis of adnexal torsion is common and associated with higher area deprivation index and farther distance from hospital. Risk of oophorectomy was higher in patients with delay. Improved diagnostics and increased awareness of social disparities are critical to decrease time to definitive treatment and improve rates of adnexal salvage.

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.

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.