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.

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.

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.

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.

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:Fluorodeoxyglucose uptake on positron emission tomography imaging has been shown to be an independent risk factor for malignancy in thyroid nodules. More recently, a new positron emission tomography radiotracer-Gallium-68 DOTATATE-has gained popularity as a sensitive method to detect neuroendocrine tumors. With greater availability of this imaging, incidental Gallium-68 DOTATATE uptake in the thyroid gland has increased. It is unclear whether current guideline-directed management of thyroid nodules remains appropriate in those that are Gallium-68 DOTATATE avid. METHODS:We retrospectively reviewed Gallium-68 DOTATATE positron emission tomography scans performed at our institution from 2012 to 2022. Patients with incidental focal Gallium-68 DOTATATE uptake in the thyroid gland were included. Fine needle aspiration biopsies were characterized via the Bethesda System for Reporting Thyroid Cytopathology. Bethesda III/IV nodules underwent molecular testing (ThyroSeq v3), and malignancy risk ≥50% was considered positive. RESULTS:In total, 1,176 Gallium-68 DOTATATE PET scans were reviewed across 837 unique patients. Fifty-three (6.3%) patients demonstrated focal Gallium-68 DOTATATE thyroid uptake. Nine patients were imaged for known medullary thyroid cancer. Forty-four patients had incidental radiotracer uptake in the thyroid and were included in our study. Patients included in the study were predominantly female sex (75%), with an average age of 62.9 ± 13.9 years and a maximum standardized uptake value in the thyroid of 7.3 ± 5.3. Frequent indications for imaging included neuroendocrine tumors of the small bowel (n = 17), lung (n = 8), and pancreas (n = 7). Thirty-three patients underwent subsequent thyroid ultrasound. Sonographic findings warranted biopsy in 24 patients, of which 3 were lost to follow-up. Cytopathology and molecular testing results are as follows: 12 Bethesda II (57.1%), 6 Bethesda III/ThyroSeq-negative (28.6%), 1 Bethesda III/ThyroSeq-positive (4.8%), 2 Bethesda V/VI (9.5%). Four nodules were resected, revealing 2 papillary thyroid cancers, 1 neoplasm with papillary-like nuclear features, and 1 follicular adenoma. There was no difference in maximum standardized uptake value between benign and malignant nodules (7.0 ± 4.6 vs 13.1 ± 5.7, P = .106). Overall, the malignancy rate among patients with sonography and appropriate follow-up was 6.7% (2/30). Among patients with cyto- or histopathology, the malignancy rate was 9.5% (2/21). There were no incidental cases of medullary thyroid cancer. CONCLUSION/CONCLUSIONS:The malignancy rate among thyroid nodules with incidental Gallium-68 DOTATATE uptake is comparable to rates reported among thyroid nodules in the general population. Guideline-directed management of thyroid nodules remains appropriate in those with incidental Gallium-68 DOTATATE uptake.

PURPOSE/OBJECTIVE:The premature infant brain may be particularly vulnerable to anesthesia effects, but there is conflicting evidence on the association between anesthesia exposure and developmental outcomes. Twin studies can control for confounding factors. A twin cohort of premature twins provides internal control of difficulty to measure confounders and delivers added power to a study examining the effects of anesthesia on neurodevelopmental outcomes. METHODS:We conducted a retrospective cohort study of sets of premature twins and multiples born at an academic medical center, in which 1 member of the set was exposed to general anesthesia. The primary outcome was the composite scores using Bayley Scale of Infant and Toddler Development III performed at age 6 months to 18 months. Unpaired and paired analyses were performed with linear regression models, Wilcoxon signed rank test, and Mann-Whitney U test. RESULTS:We identified 81 children born at less than 32 weeks gestation within 39 sets of twins and 1 set of triplets for a total of 18 paired observations. All of the exposed infants had a single exposure to general anesthesia. There was no significant association between anesthesia exposure and a diagnosis of developmental delay (OR = 0.8; 95% confidence interval, 0.2-3.2; p = 0.99). Regression models demonstrated no association between anesthesia exposure and cognitive (96.67 vs 97.50; p = 0.74), language (98.33 vs 98.61; p = 0.94), or motor (96.25 vs 96.44; p = 0.91) composite Bayley scores. There was no association between duration of anesthesia and the 3 composite Bayley scores ( p = 0.33; p = 0.40; p = 0.74). CONCLUSION/CONCLUSIONS:Using a premature twin cohort with discordant exposure to anesthesia, our data did not demonstrate any association between anesthesia exposure and developmental delay in this vulnerable population of premature infants.

OBJECTIVE:Hungry bone syndrome (HBS) is a known complication of parathyroidectomy. Patients with renal hyperparathyroidism are particularly vulnerable to HBS because of their prolonged exposure to electrolyte abnormalities and elevated parathyroid hormone (PTH). However, in-depth characterization of predictive factors for HBS in these patients is lacking. METHODS:A retrospective analysis was performed of patients with renal hyperparathyroidism who underwent parathyroidectomy at a single institution from 2011-2021. Patient demographics, clinical characteristics, and biochemical data were collected and analyzed. Boruta and binary logistic regression analyses were used to develop a scoring system. RESULTS:Thirty-three patients were identified; 16 (48%) developed HBS. Patients with HBS had significantly higher preoperative levels of serum PTH (mean difference [MS] = 2167.2 pg/mL, P <.001), phosphorus (MD = 3.5 mg/dl, P <.001), and alkaline phosphatase (ALP) (MD = 344.2 U/L, P =.002) and significantly lower levels of preoperative serum calcium (MD = -0.96 mg/dL, P =.004). Stepwise regression analysis identified elevated ALP (>150 U/L) and markedly elevated PTH (>1000 pg/mL) as positive predictors of HBS. A two-point scoring system with these 2 variables had overall diagnostic accuracy of 96.8% (sensitivity 100% and specificity 94.1%) with 1 point conferring 93.8% positive predictive value and 2 points conferring 100% positive predictive value. CONCLUSION/CONCLUSIONS:Preoperative serum PTH and ALP are significantly associated with HBS in patients with renal hyperparathyroidism undergoing parathyroidectomy for renal hyperparathyroidism. A scoring system with these 2 variables may be of clinical utility in predicting patients at high risk of HBS.

Unique challenges face pediatric surgeons at community-based nonteaching hospitals. Communication and collaboration among and between healthcare providers, hospital administrators, and quaternary referral programs is crucial for the success of these smaller hospitals as they care for children.