Hepatoduodenal ligament teratoma with immature elements.

Pediatric germ cell tumors comprise 1-3% of all malignant pediatric tumors and are found in variable locations. We present the case of a term 3.7 kg neonate who was found to have a giant liver mass at birth, later determined to be an immature teratoma arising from the hepatoduodenal ligament. This case report and images add to the limited literature a very rare presentation of a teratoma.

Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation.

The vast majority of congenital diaphragmatic cases present with severe respiratory distress in the immediate neonatal period. Obstructive jaundice secondary to CDH is a rare presenting symptom, previously unreported with Morgagni type hernias which comprise 2% of all CDHs. Although the safety of minimally invasive repair for CDH has been shown previously, there has been little data on its safety for patients with complex congenital cardiac disease. In this case report, we present an 18-month old infant with Fontan circulation presenting with jaundice secondary to Morgagni hernia who ultimately underwent laparoscopic repair. A brief discussion of the safety of laparoscopic surgery in patients post-Fontan is included.

APSA Members experience bias and discrimination in training and practice.

INTRODUCTION: Bias and discrimination remain pervasive in the medical field and increase the risk of burnout, mental health disorders, and medical errors. The experiences of APSA members with bias and discrimination are unknown, therefore the APSA committee on Diversity, Equity and Inclusion conducted a survey to characterize the prevalence of bias and discrimination. METHODS: 1558 APSA members were sent an anonymous survey, of which 423 (27%) responded. Respondents were asked about their demographics, knowledge of implicit bias, and experience of bias and discrimination within their primary workplace, APSA, and APSA committees. Data were analyzed using Fisher's Exact test, Kruskal-Wallis test, and multivariable logistic regression as appropriate with significance defined as p<0.05. RESULTS: Discrimination was reported across all levels of practice, academic appointments, race, ethnicity, and gender identities. On multivariable analysis, surgical trainees (OR 3.6) as well as Asian American and Pacific Islander (OR 4.8), Black (OR 5.2), Hispanic (OR 8.2) and women (OR 8.7) surgeons were more likely to experience bias and discrimination in the workplace. Community practice surgeons were more likely to experience discrimination within APSA committees (OR 3.6). Members identifying as Asian (OR 0.4), or women (OR 0.6) were less likely to express comfort reporting instances of bias and discrimination. CONCLUSION: Workplace discrimination exists across all training levels, academic appointments, and racial and gender identities. Trainees and racial- and gender-minority surgeons report disproportionately high prevalence of bias and discrimination. Improving reporting mechanisms and implicit bias training are possible initiatives in addressing these findings.

Diversity and demographics of APSA members: Understanding our identity.

INTRODUCTION: There are existing healthcare disparities in pediatric surgery today. Identity and racial incongruity between patients and providers contribute to systemic healthcare inequities and negatively impacts health outcomes of minoritized populations. Understanding the current demographics of the American Pediatric Surgical Association and therefore the cognitive diversity represented will help inform how best to strategically build the organization to optimize disparity solutions and improve patient care. METHODS: 1558 APSA members were sent an anonymous electronic survey. Comparative data was collected from the US Census Bureau and the Association of American Medical Colleges. Results were analyzed using standard statistical tests. RESULTS: Of 423 respondents (response rate 27%), the race and ethnicity composition were 68% non Hispanic White, 12% Asian American and Pacific Islander, 6% Hispanic, 5% multiracial, and 4% Black/African American. Respondents were 35% women, 63% men, and 1% transgender, androgyne, or uncertain. Distribution of sexual identity was 97% heterosexual and 3% LGBTQIA. Religious identity was 50% Christian, 22% Agnostic/Atheist, 11% Jewish, 3% Hindu, and 2% Muslim. 32% of respondents were first-generation Americans. Twenty-four different primary languages were spoken, and 46% of respondents were conversational in a second language. These findings differ in meaningful ways from the overall American population and from the population of matriculants in American medical schools. CONCLUSION: There are substantial differences in the racial, gender, and sexual identity composition of APSA members compared with the overall population in the United States. To achieve excellence in patient care and innovate solutions to existing disparities, representation, particularly in leadership is essential. TYPE OF STUDY: Survey; original research. LEVEL OF EVIDENCE: Level IV.

Odontogenic ameloblast associated protein as a novel biomarker for human breast cancer.

Odontogenic Ameloblast Associated Protein (ODAM) is a protein isolated in ameloblasts during odontogenesis. ODAM expression was identified in breast cancer, but its significance remains unknown. The purpose of this study is to determine if ODAM expression can serve as a prognostic marker and provide information regarding treatment in human breast cancer. Breast cancer patients were identified from our tumor registry from 1993 to 2003. Archived breast cancer tissue from 243 patients (stage 0 = 53, stage I = 51, stage II = 53, stage III = 47, stage IV = 39) was stained using monoclonal antibody for ODAM. Presence or absence of immunostaining was correlated with stage, histologic grade, response to chemotherapy, and survival using chi2 and logistic regression analyses. Tumor nuclear staining for ODAM increased with increasing group stage (P < 0.001). Staining for ODAM did not correlate with histologic grade or chemotherapy (P = 0.558, P = 0.093). Improved outcomes within each stage were noted with ODAM staining, statistically significant for stages 0, I, and II (P < 0.001, P = 0.003, P = 0.003) and underpowered for stages III and IV (P = 0.724, P = 0.059). Survival benefit associated with tumor nuclear staining increased with advancing stage (P < 0.001). These results show that ODAM predicts survival in breast cancer. Research is ongoing to determine ODAM's clinical utility and role in carcinogenesis.

Renal dysfunction in the pediatric surgical patient: When to intervene.

Renal dysfunction is very common in the pediatric surgical critical care patient, with an estimated incidence of up to 35% in the PICU population. It impacts multiple other organ systems, particularly ventilation, and adds to the morbidity and mortality in children with multisystem organ dysfunction. In this article, we review the definitions and stages of renal failure in the pediatric population, identify which of these are more likely to require renal replacement therapy, and identify the indications for the different types of intervention. In addition, the complications of each form of therapy, along with management options, will be discussed. Finally, we will discuss the immediate and long-term outcomes for pediatric patients from neonates to adolescents.

Avoiding misdiagnosis in postnatal presentation of congenital diaphragmatic hernia: A report of two cases and review of radiologic features.

Diagnosis of a congenital diaphragmatic hernia after the first day of life can be challenging. The clinical symptoms are often nonspecific, and initial radiographic findings in these patients may mimic many other acute chest conditions, including pneumonia, pleural effusion, and pneumothorax. In turn, diagnostic uncertainty may expose the patient to unnecessary and even potentially harmful interventions such as thoracostomy tube placement. As such, it is imperative that radiologists remain aware of this uncommon entity and recognize imaging findings which may provide clues to its diagnosis.

A tiered approach to optimize pediatric laparoscopic appendectomy outcomes.

BACKGROUND/PURPOSE: Surgical management of appendicitis accounts for ~30% of total expenditure in the practice of pediatric surgery and is associated with high cost variation. We hypothesize that incorporating single-incision laparoscopy (SILS) and the resultant by-product dual-incision laparoscopy (DILS) into a historically three-incision laparoscopic (TILS) appendectomy practice affords equal outcomes at lower cost. METHODS: Appendectomies performed at a large-volume tertiary care children's hospital from 1/2015-12/2017 were retrospectively reviewed. Appendectomy technique and appendicitis severity were stratified against operative and admission direct variable (DV) costs. Secondary outcomes included perioperative time course and 30-day postoperative outcomes. RESULTS: A total of 970 appendectomies were analyzed during the study period (61% acute, 39% complex appendicitis). SILS and DILS had significantly lower mean DV costs and OR times compared to TILS for both acute and complex appendicitis while maintaining equivalent outcomes. CONCLUSIONS: SILS and DILS appendectomy techniques can be incorporated into pediatric surgical practice at lower cost than TILS appendectomy while maintaining equivalent outcomes. Further, the introduction of a tiered approach to laparoscopic appendectomy, in which all cases are started as SILS with additional incisions added based on operative difficulty, is estimated to save $74,580 annually in operative DV costs at a pediatric surgical center averaging 314 laparoscopic appendectomies per year. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

Improving central line infection rates in the neonatal intensive care unit: Effect of hospital location, site of insertion, and implementation of catheter-associated bloodstream infection protocols.

INTRODUCTION: Catheter associated blood stream infections (CABSIs) are morbid and expensive for all ages, including neonates. Thus far, the impact of CABSI prevention protocols, such as insertion and maintenance bundles, in the neonatal intensive care unit (NICU) is largely unknown. We hypothesized that lines placed in the operating room (OR) would have a lower infection rate due to established insertion protocols and a more sterile environment. METHODS: A retrospective chart review of NICU patients who received a percutaneous or tunneled central venous catheter between 2005 and 2012 was performed. ECMO cannulas, PICC and umbilical catheters were excluded. Variables of interest included demographics, anatomical site, hospital location, line days, and line infection. Line infection was defined as a positive blood culture drawn through the catheter. RESULTS: A total of 368 catheters were placed in 285 NICU patients. Majority of catheters (65.5%) were placed in OR. Saphenous and femoral veins were most common anatomical sites (50.8%). Twenty-eight catheters were infected (7.6%). After adjusting for preoperative antibiotics, anatomical site, and SNAPPE-II scores, lines placed in OR were three times less likely to become infected (Odds Ratio=0.32, p=0.038). Although implementation of CABSI prevention protocols resulted in statistically significant reductions in infection (Odds Ratio=0.4, p=0.043), lines placed in the OR remained less likely to become infected. CONCLUSIONS: NICU line infection rates decreased with implementation of CABSI prevention protocols. Despite this implementation, catheters placed in the NICU continued to have higher infection rates. As a result, when patient status allows it, we recommend that central lines in newborns be placed in the operating room.

Preliminary findings of long-term neurodevelopmental outcomes of infants treated with intravenous fat emulsion reduction for the management of parenteral nutrition-associated cholestasis.

INTRODUCTION: Parenteral nutrition-associated cholestasis (PNAC) is linked with the administration of soybean-based intravenous fat emulsion (IVFE). IVFE reduction (IFER) may be an effective management strategy for PNAC; however, long-term associated neurodevelopmental outcomes (NDOs) for infants undergoing IFER have not been measured previously. This single-institution, prospective study examined the risk for negative NDOs and key predictors of NDOs associated with IFER. METHODS: Patients (2-5 years) treated with soybean-based IFER as neonates underwent NDO measurements, including Ages and Stages Questionnaires-3 (ASQ-3), Parents' Evaluations of Developmental Status (PEDS), and Behavior Assessment System for Children, Second Edition Preschool, Parent (BASC-2 PRS-P). The relationship between NDOs and predictive variables was evaluated. RESULTS: A total of 25 children had a complete PEDS survey, and 17 were found to be "not at risk." The BASC-2 PRS-P evaluation (n = 18 patients) showed that all 4 composite domains fell within the normative developmental range, and 67%-89% of patients were observed to be "typically developing." For the primary outcome measure, ASQ-3, 82.4%-94.4% of patients were "not at risk." Logistical regression analyses were performed to examine risk factors contributing to negative NDOs. Of children completing all NDO studies, IFER-related variables (eg, development of essential fatty acid deficiency, duration of IFER, and mean IVFE dose) were not found to be predictors of adverse NDOs. CONCLUSIONS: This study represents the first report of NDOs in pediatric patients treated with IFER. IFER-treated patients score within the normative range most of the time. IFER-related variables were not found to be associated with negative NDOs. The results set the stage for a larger, multicenter, prospective study.

Probiotic prophylaxis after pullthrough for Hirschsprung disease to reduce incidence of enterocolitis: a prospective, randomized, double-blind, placebo-controlled, multicenter trial.

OBJECTIVE: Hirschsprung-associated enterocolitis (HAEC) is one of the most troublesome problems encountered after a pullthrough. We hypothesized that prophylactic administration of probiotics after a pullthrough procedure would decrease the incidence of HAEC. STUDY DESIGN: A prospective, double-blind, placebo-controlled, randomized trial was conducted at 2 children's hospitals. Infants undergoing pullthrough were randomized to probiotic or placebo for a period of 3 months post-pullthrough. Primary outcome was incidence of post-operative HAEC. Other outcomes included severity of HAEC by clinical grade, number of HAEC episodes and extent of aganglionosis. Pearson Chi Square analysis, as well as logistic regression, was used for statistical analysis. RESULTS: Sixty-two patients were recruited (Sites: A=40; B=22). One was lost to follow up and one immediate post-op death was not included in final analysis. Probiotics were administered to 32 patients. Distribution of placebo/probiotics was equal between sites (P=0.858). Mean age at pullthrough was 6.5 ± 8.1(± SD) months. The incidence of HAEC was 28.3%. The incidence of HAEC was not statistically different between probiotic and placebo study groups. CONCLUSIONS: Incidence of HAEC was not reduced with prophylactic probiotics. Future studies are needed to better determine the etiology and possible ways of preventing this complex condition.

Pyloromyotomy: randomized control trial of laparoscopic vs open technique.

PURPOSE: Open pyloromyotomy remains as the criterion standard treatment for hypertrophic pyloric stenosis with the laparoscopic approach rapidly gaining adoption. We present a prospective, randomized trial between the 2 approaches. METHODS: After institutional review board approval, 98 patients with hypertrophic pyloric stenosis were consecutively randomized to either open or laparoscopic pyloromyotomy. Postoperative and hospital course were evaluated by review of the hospital records and long-term follow-up with scripted telephone survey using Likert scales. The length of operating room time, surgical procedure, postoperative stay, time to refeeding, and complications were evaluated. Secondary outcomes of cosmetic results and parental satisfaction were determined. RESULTS: Ninety-eight patients were enrolled during a 4-year period. There were no significant differences between 2 groups on all primary outcomes. There were 3 complications in the open group-a wound dehiscence, a surgical site infection, and a gastric serosal tear-and 2 complications in the laparoscopic group-mucosal perforation and a suture granuloma. In long-term follow-up on 72 patients (56 months), parents described significant cosmetic results with laparoscopic approach. CONCLUSIONS: There was no difference in operating time, hospital stay, or refeeding patterns between open and laparoscopic pyloromyotomy. The complication rates were similar between the 2 methods. However, long-term cosmetic results were significantly superior in the laparoscopic group.

Efficacy of laparoscopic cholecystectomy in the pediatric population.

PURPOSE: Gallbladder disease is increasingly affecting the pediatric population. The advent of new technology in the 1980s, specifically, hepatobiliary scintigraphy and laparoscopic cholecystectomy, gave a dramatic rise in both the diagnosis and treatment of biliary disease in the pediatric population. The purpose of this study was to determine (a) whether laparoscopic cholecystectomy for biliary dyskinesia is efficacious in the treatment of children with biliary colic and (b) the ability of cholescintigraphy to predict which patients may benefit from an operative intervention. METHODS: We performed a retrospective review of the records of all patients (N = 184) who underwent laparoscopic cholecystectomy, correlating postoperative results with degree of dyskinesia (percentage of ejection fraction), histopathology, associated gastrointestinal diagnoses, age, and sex. Biliary dyskinesia was defined by ultrasonography without evidence of cholelithiasis with clinical diagnosis of biliary colic. RESULTS: Of the 184 patients who underwent laparoscopic cholecystectomy, 117 had a diagnosis of biliary dyskinesia and 108 were available for follow-up. Mean follow-up was 8.3 months. One hundred patients (92.6%) reported resolution or improvement of preoperative symptoms (64.8% reported complete resolution and 27.8% reported improvement in symptoms). The mean age of the patients was 14.1 years. No correlation was seen for degree of dyskinesia, histopathology, age, and sex. Patients with a preoperative diagnosis of gastroesophageal reflux were more likely to report resolution of symptoms, although this finding was not statistically significant. There was no major complication; 1 patient suffered a prolonged ileus, 1 patient suffered a wound infection, and 1 patient required incisional hernia repair. CONCLUSION: Laparoscopic cholecystectomy is safe, efficacious, and durable in children suffering from biliary dyskinesia.