The Current Pediatric Surgery Job Market: A Perspective of Recent Fellowship Graduates.

PURPOSE: This study describes the job market from the perspective of recent pediatric surgery graduates. METHODS: An anonymous survey was circulated to the 137 pediatric surgeons who graduated from fellowships 2019-2021. RESULTS: The survey response rate was 49%. The majority of respondents were women (52%), Caucasian (72%), and had a median student debt burden of $225,000. Considering job opportunities, respondents strongly emphasized camaraderie (93%), mentorship (93%), case mix (85%), geography (67%), faculty reputation (62%), spousal employment (57%), compensation (51%), and call frequency (45%). 30% were satisfied with the employment opportunities available, and 21% felt strongly prepared to negotiate for their first job. All respondents were able to secure a job. Most jobs were university-based (70%) or hospital employed (18%) positions where surgeons covered median of two hospitals. 49% wanted protected research time, and 12% of respondents were able to secure substantial, protected research time. The median compensation for university-based jobs was $12,583 below the median AAMC benchmark for assistant professors for the corresponding year of graduation. CONCLUSION: These data highlight the ongoing need for assessment of the pediatric surgery workforce and for professional societies and training programs to further assist graduating fellows in preparing to negotiate their first job. TYPE OF STUDY: Survey LEVEL OF EVIDENCE: Level V.

A definition of gentle ventilation in congenital diaphragmatic hernia: a survey of neonatologists and pediatric surgeons.

Ventilation practices have changed significantly since the initial reports in the mid 1980 of successful use of permissive hypercapnia and spontaneous ventilation [often called gentle ventilation (GV)] in infants with congenital diaphragmatic hernia (CDH). However, there has been little standardization of these practices or of the physiologic limits that define GV. We sought to ascertain among Diaphragmatic Hernia Research and Exploration; Advancing Molecular Science (DHREAMS) centers' GV practices in the neonatal management of CDH. Pediatric surgeons and neonatologists from DHREAMS centers completed an online survey on GV practices in infants with CDH. The survey gathered data on how individuals defined GV including ventilator settings, blood gas parameters and other factors of respiratory management. A total of 87 respondents, from 12 DHREAMS centers completed the survey for an individual response rate of 53% and a 92% center response rate. Approximately 99% of the respondents defined GV as accepting higher carbon dioxide (PCO2) and 60% of the respondents also defined GV as accepting a lower pH. There was less consensus about the use of sedation and neuromuscular blocking agents in GV, both within and across the centers. Acceptable pH and PCO2 levels are broader than the goal ranges. Despite a lack of formal standardization, the results suggest that GV practice is consistently defined as the use of permissive hypercapnia with mild respiratory acidosis and less consistently with the use of sedation and neuromuscular blocking agents. GV is the reported practice of surveyed neonatologists and pediatric surgeons in the respiratory management of infants with CDH.

Increased burden of de novo predicted deleterious variants in complex congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a serious birth defect that accounts for 8% of all major birth anomalies. Approximately 40% of cases occur in association with other anomalies. As sporadic complex CDH likely has a significant impact on reproductive fitness, we hypothesized that de novo variants would account for the etiology in a significant fraction of cases. We performed exome sequencing in 39 CDH trios and compared the frequency of de novo variants with 787 unaffected controls from the Simons Simplex Collection. We found no significant difference in overall frequency of de novo variants between cases and controls. However, among genes that are highly expressed during diaphragm development, there was a significant burden of likely gene disrupting (LGD) and predicted deleterious missense variants in cases (fold enrichment = 3.2, P-value = 0.003), and these genes are more likely to be haploinsufficient (P-value = 0.01) than the ones with benign missense or synonymous de novo variants in cases. After accounting for the frequency of de novo variants in the control population, we estimate that 15% of sporadic complex CDH patients are attributable to de novo LGD or deleterious missense variants. We identified several genes with predicted deleterious de novo variants that fall into common categories of genes related to transcription factors and cell migration that we believe are related to the pathogenesis of CDH. These data provide supportive evidence for novel genes in the pathogenesis of CDH associated with other anomalies and suggest that de novo variants play a significant role in complex CDH cases.

Developmental outcomes of children with congenital diaphragmatic hernia: a multicenter prospective study.

PURPOSE: To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age. METHODS: This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age. RESULTS: BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors. CONCLUSION: CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.

Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Trends in operative experience of pediatric surgical residents in the United States and Canada.

PURPOSE: Expansion of the number of training programs in pediatric surgery occurred from 2003 through 2010. We sought to determine the effect of program expansion on case volume and distribution of operative experience. METHODS: Public domain data on pediatric surgery resident summary statistics available from the Accreditation Council for Graduate Medical Education (ACGME) from July 2003 through June 2010 were analyzed. Total case volume as primary surgeon or teaching assistant, mean case volume per resident, standard deviation, mode, minimum, and maximum number of cases per resident were evaluated. Mean total cases per resident, minimally invasive laparoscopic and thoracoscopic cases, and requisite cases as defined by the ACGME categories of: tumor, important pediatric surgical, and neonatal cases were analyzed by a Cuzick Wilcoxon-type nonparametric trend statistic using a significance level of 0.05. Skew was assessed by Pearson coefficient with levels of -0.5 to 0.5 defining a parametric distribution. RESULTS: The number of pediatric surgical training residents increased by 42% during the years reported, from 24 to 34. No statistically significant difference was found in the mean number of total cases or requisite cases per resident. The mean volume of minimally invasive procedures increased significantly. Case volume per resident was non-parametrically distributed with increasing positive skew over time. CONCLUSIONS: The increase in number of pediatric surgical resident training positions has not adversely affected overall operative experience or exposure to highly specialized requisite cases, on average. The increasing positive skew of total and index cases, however, suggests that variability between programs in case exposure is increasing over time.

First employment characteristics for the 2011 pediatric surgery fellowship graduates.

PURPOSE: Information regarding initial employment of graduating pediatric surgery fellows is limited. More complete data could yield benchmarks of initial career environment. METHODS: An anonymous survey was distributed in 2011 to 41 pediatric surgery graduates from all ACGME training programs interrogating details of initial positions and demographics. RESULTS: Thirty-seven of 41 (90%) fellows responded. Male to female ratio was equal. Graduates carried a median debt of $220,000 (range: $0-$850,000). The majority of fellows were married with children. 70% were university/hospital employees, and 68% were unaware of a business plan. Median starting compensation was $354,500 (range: $140,000-$506,000). Starting salary was greatest for >90% clinical obligation appointments (median $427,500 vs. $310,000; p=0.002), independent of geographic location. Compensation had no relationship to private practice vs. hospital/university/military position, coastal vs. inland location, and practice sites number. Median clinical time was 75% and research time 10%. 49% identified a formal mentor. Graduates covered 1-5 different offices (median 1) and 1-5 surgery sites (median 2). 60% were satisfied with their compensation. CONCLUSION: Recent pediatric surgery graduates are engaged mainly in clinical care. Research is not incentivized. Compensation is driven by clinical obligations. Graduates have limited knowledge of the business plan supporting their compensation, nature of malpractice coverage, and commitments to resources including research. Graduates have important fiscal and parenting obligations.

Lung to head ratio in infants with congenital diaphragmatic hernia does not predict long term pulmonary hypertension.

PURPOSE: Lung-to-head ratio (LHR) has been used for antenatal evaluation of infants with congenital diaphragmatic hernia (CDH). We hypothesized that LHR was predictive of acute and chronic pulmonary hypertension in infants with CDH. METHODS: Echocardiograms on all inborn infants with CDH (December 2001-March 2011) were reviewed. Echocardiograms at 1 and 3 months post-repair and most recent follow-up were assessed for presence of pulmonary hypertension (PAH). LHR, gestational age, birth weight, extracorporeal membrane oxygenation (ECMO), and death rate were obtained. Bivariate and multivariate analyses were performed. RESULTS: 106 infants with CDH had LHR obtained at median 28 weeks gestation (median LHR=1.25 [range 0.4-5.3]). Median follow-up was 26.6 months (range 4.6-97.5). The long-term incidence of pulmonary hypertension was 16%. LHR was significantly associated with pulmonary hypertension at one month (p=0.0001) but not at 3 months (p=0.22) or long-term (p=0.54). LHR was predictive of ECMO use (p=0.01) and death (p=0.001). CONCLUSIONS: The overall incidence of PAH in infants with CDH decreases over time. Prenatal LHR predicts PAH at one month but not long-term in infants with CDH. The ability for LHR to predict PAH at one month but not long term may suggest remodeling of the pulmonary vasculature over time.

Congenital diaphragmatic hernia and protective ventilation strategies in pediatric surgery.

Infants affected with congenital diaphragmatic hernias (CDH) suffer from some degree of respiratory insufficiency arising from a combination of pulmonary hypoplasia and pulmonary hypertension. Respiratory care strategies to optimize blood gasses lead to significant barotrauma, increased morbidity, and overuse of extracorporeal membrane oxygenation (ECMO). Newer permissive hypercapnia/spontaneous ventilation protocols geared to accept moderate hypercapnia at lower peak airway pressures have led to improved outcomes. High-frequency oscillatory ventilation can be used in infants who continue to have persistent respiratory distress despite conventional ventilation. ECMO can be used successfully as a resuscitative strategy to minimize further barotrauma in carefully selected patients.

Prenatal lung-head ratio: threshold to predict outcome for congenital diaphragmatic hernia.

OBJECTIVE: The literature suggests that lung-head ratio (LHR) and liver position may inconsistently predict outcome for congenital diaphragmatic hernia (CDH). We reviewed our inborn neonates with isolated left-sided CDH to determine whether these variables predicted survival and to estimate the optimal LHR threshold. METHODS: Prenatal LHR and liver position were obtained from 2002 to 2009. The primary endpoint was survival. RESULTS: LHR was greater in survivors after adjusting for gestational age (median 1.40 versus 0.81; p < 0.001). LHR demonstrated excellent diagnostic discrimination, with area under receiver operating characteristic (ROC) curve 0.93 (95% CI 0.86-0.99). LHR threshold of 1.0 was 83% sensitive and 91% specific in predicting survival. An optimal LHR threshold of 0.85 predicted survival with 95% sensitivity and 64% specificity, reducing false negatives (survivors with low LHR). LHR > 0.85 predicted survival after adjustment for gestational age (OR = 33.6, 95% CI = 5.4-209.5). Liver position did not predict survival. CONCLUSIONS: Prenatal LHR >0.85 predicts survival for infants with isolated left-sided CDH without compromising discrimination of survivors from non-survivors. The diagnostic utility of LHR may be confounded by gestational age at measurement. Stringent LHR threshold may minimize false-negative attribution and improve utility of this measurement as predictor of survival.

The use of recombinant tissue-type plasminogen activator in a newborn with an intracardiac thrombus developed during extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation (ECMO) support is often used to support infants and children with hemodynamic or respiratory failure. One of the major obstacles of safely treating a child with ECMO is balancing the risk of hemorrhage with the potential for thrombus development. Managing thrombosis in the setting of ECMO is challenging and has no defined algorithm. The use of recombinant tissue-type plasminogen activator (tPA) for thrombolysis has been previously described in cases where thrombi have developed despite adequate anticoagulation. In such situations, the risk of hemorrhage must be carefully balanced with the benefit of dissolving the clot and reestablishing flow. We present a case of an infant who required ECMO because of severe primary pulmonary hypertension and subsequently developed a right atrial thrombus adjacent to the ECMO cannula. The patient was treated with tPA with immediate improvement but had fatal intracranial hemorrhage almost 3 days after the tPA was administered. In this report, we review the current literature on tPA use during ECMO support and suggest a rational approach.

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study.

INTRODUCTION: Experience in thoracoscopic congenital diaphragmatic hernia (CDH) repair has expanded, yet efficacy equal to that of open repair has not been demonstrated. In spite of reports suggesting higher recurrent hernia rates after thoracoscopic repair, this approach has widely been adopted into practice. We report a large, single institutional experience with thoracoscopic CDH repair with special attention to recurrent hernia rates. METHODS: We reviewed the records of neonates with unilateral CDH repaired between January 2006 and February 2010 at Morgan Stanley Children's Hospital. Completely thoracoscopic repairs were compared to open repairs of the same period. In addition, successful thoracoscopic repairs were compared with thoracoscopic repairs that developed recurrence. Data were analyzed by Mann-Whitney U and Fisher exact tests. RESULTS: Thirty-five neonates underwent attempted thoracoscopic repair, with 26 completed. Concurrently, 19 initially open CDH repairs were performed. Preoperatively, patients in the open repair group required more ventilatory support than the thoracoscopic group. Recurrence was higher after thoracoscopic repair (23% vs 0%; P = .032). In comparing successful thoracoscopic repairs to those with recurrence, none of the factors analyzed were predictive of recurrence. CONCLUSIONS: Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous.

Thoracoscopic lobectomy in infants less than 10 kg with prenatally diagnosed cystic lung disease.

PURPOSE: Thoracoscopic lobectomy for congenital cystic lung lesions is an accepted technique in pediatric surgery. Since an increasing number of these lesions are detected prenatally, the safety and efficacy of infant resections have been questioned. We reviewed our experience over a 10-year period to evaluate early resection of these lesions. METHODS: From January 2001 to August 2009, 75 patients under 1 year of age and weighing <10 kg underwent thoracoscopic lobectomy at two institutions. Patients carried the following diagnoses: 52 had congenital cystic adenomatoid malformation, 20 had bronchopulmonary sequestration, and 3 had congenital lobar emphysema. All lesions were confirmed after birth by computed tomography scan. Patient age at operation ranged from 4 days to 11 months and patient weight from 3.1 to 10 kg. RESULTS: Seventy-four of 75 lobectomies were thoracoscopically completed. There were 16 upper lobectomies, 1 middle lobectomy, and 55 lower lobectomies. Operative time ranged from 45 to 225 minutes. Hospital length of stay ranged from 1 to 5 days. A subset of 26 patients had surgery younger than 3 months of age and <5 kg, despite being asymptomatic. Their operative time averaged 90 minutes, and mean length of hospital stay was 1.5 days. CONCLUSION: Thoracoscopic lobectomy is safe for infants <10 kg and avoids the morbidity associated with thoracotomy. Operating early on younger patients may avoid the inflammatory changes associated with both clinically apparent and subclinical infections, even in patients weighing <5 kg. This may make the procedures less technically challenging and may result in lower complication and conversion rates.

Fulminant pH1N1-09 influenza-associated myocarditis in pediatric patients.

OBJECTIVE: To report an atypical presentation of pH1N1-09 influenza infection in children as fulminant myocarditis and tamponade and the successful treatment with extracorporeal membrane oxygenation. DESIGN: Case report. SETTING: Pediatric cardiac intensive care unit in a quarternary care children's hospital. PATIENTS: Two girls, 5 and 7 yrs of age, infected with pH1N1-09 influenza virus who presented in cardiogenic shock with a pericardial effusion and echocardiographic evidence of tamponade from fulminant myocarditis. INTERVENTIONS: Both patients received a pericardiocentesis. One was managed with multiple, high-dose inotropic agents, whereas the other required institution of extracorporeal membrane oxygenation. MEASUREMENTS AND MAIN RESULTS: Acute respiratory distress syndrome is the major reported clinical manifestation of pH1N1-09 influenza virus infection in hospitalized pediatric patients. In this report we describe two children with confirmed pH1N1-09 influenza infection that required intensive care for fulminant myocarditis. Neither patient had the typical symptoms of influenza-like illness, respiratory compromise, or evidence of pulmonary involvement. One child required extracorporeal membrane oxygenation. Both children survived to hospital discharge. CONCLUSIONS: pH1N1-09 influenza infection can cause fulminant myocarditis in the healthy pediatric population. The clinical presentation may be nonspecific, and the lack of pulmonary symptoms may make diagnosis difficult. Extracorporeal membrane oxygenation support may offer an effective bridge to the recovery of heart function.

Limb ischemia after common femoral artery cannulation for venoarterial extracorporeal membrane oxygenation: an unresolved problem.

PURPOSE: Extracorporeal Life Support Organization Registry data confirm that the number of pediatric patients being supported by extracorporeal membrane oxygenation (ECMO) is increasing. To minimize the potential neurologic effects of carotid artery ligation, the common femoral artery (CFA) is frequently being used for arterial cannulation. The cannula has the potential for obstructing flow to the lower limb, thus increasing ischemia and possible limb loss. We present a single institution's experience with CFA cannulation for venoarterial (VA) ECMO and ask whether any precannulation variables correlate with the development of significant limb ischemia. METHODS: We reviewed all pediatric patients who were supported by VA ECMO via CFA cannulation from January 2000 to February 2010. Limb ischemia was the primary variable. The ischemia group was defined as the patients requiring an intervention because of the development of lower extremity ischemia. The patients in the no-ischemia group did not develop significant ischemia. Continuous variables were reported as medians with interquartile ranges and compared using Mann-Whitney U tests. Differences in categorical variables were assessed using χ² testing (Fisher's Exact). Statistical significance was assumed at P < .05. RESULTS: Twenty-one patients (age, 2-22 years) were cannulated via the CFA for VA ECMO. Significant ischemia requiring intervention (ischemia group) occurred in 11 (52%) of 21. In comparing the 2 groups (ischemia vs no ischemia), no clinical variables predicted the development of ischemia (Table 1). In the ischemia group, 9 (81%) of 11 had a distal perfusion catheter (DPC) placed. Complications of DPC placement included one case of compartment syndrome requiring a fasciotomy and one patient requiring interval toe amputation. Of the 2 patients in the ischemia group who did not have a DPC placed, 1 required a vascular reconstruction of an injured superficial femoral artery and 1 underwent a below-the-knee amputation. Mortality was lower in the ischemia group (27% vs 60%). CONCLUSIONS: Limb ischemia remains a significant problem, as more than half of our patients developed it. The true incidence may not be known as a 60% mortality in the no-ischemia group could mask subsequent ischemia. Although children are at risk for developing limb ischemia/loss, no variable was predictive of the development of significant limb ischemia in our series. Because of the inability to predict who will develop limb ischemia, early routine placement of a DPC at the time of cannulation may be warranted. However, DPCs do not completely resolve issues around tissue loss and morbidity. Prevention of limb ischemia/loss because of CFA cannulation for VA ECMO continues to be a problem that could benefit from new strategies.

The role of fiberoptic endoscopy in the evaluation and management of long gap isolated esophageal atresia.

Accurate measurement of gap length is useful for operative planning in cases of esophageal atresia (EA) without distal fistula. This paper demonstrates how fiberoptic endoscopy of the distal esophagus enables measurement of the gap in the case of isolated EA, and compares other commonly practiced techniques.

University pediatric surgery: benchmarking performance.

The many ways monies enter and leave a university pediatric section are as poorly understood as the value and relationship to the parent hospital or university. This blinded confidential financial performance survey of similar university pediatric surgery sections begins to benchmark performance and define those relationships.