Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative. METHODS: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively. RESULTS: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]. CONCLUSION: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Association of Sociodemographic Factors with Surgical Management of Hepatoblastoma and Hepatocellular Carcinoma in Children.

OBJECTIVE: To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN: In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS: A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS: Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.

Critical elements of pediatric liver cancer surgery.

The appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon.

Unwrapping Nephrogenic Rests and Nephroblastomatosis for Pediatric Surgeons: A Systematic Review Utilizing the PICO Model by the APSA Cancer Committee.

BACKGROUND: Nephrogenic rests (NR) may represent precursor lesions for Wilms tumor (WT), but their clinical course is not fully understood and no guidelines for treatment exist. This study sought to evaluate the outcomes of pediatric patients with NRs related to traditional chemotherapy and surgery. METHODS: A PRISMA-P-compliant literature search was conducted in MEDLINE, Embase, CINAHL, Web of Science, COCHRANE, and SCOPUS from inception to June 2021. Clinical questions regarding the treatment of NRs, including chemotherapy and surgery, were developed in the population, intervention, comparison, and outcome format. RESULTS: Twenty-five studies including 1445 patients met inclusion criteria for evaluating chemotherapy compared to observation for NRs. Eighteen studies including 1392 patients met inclusion criteria for evaluating the role of surgery for NRs. Patients with isolated NRs who underwent observation progressed to WT 33% of the time; chemotherapy reduced the rate of WT to 3.9%. Observation of multiple NRs and diffuse hyperplastic perilobar nephroblastomatosis (DHPLN) led to progression to WT 50% and 100% of the time, respectively. Chemotherapy reduced the rate of WT to 34% for multiple NRs and 46% for DHPLN. Surgery for isolated NRs reduced the risk of WT development from 23.7% in patients with incomplete excision to 3.3% with complete excision; however, 96% of patients with incompletely excised NRs had bilateral disease. CONCLUSIONS: Observation with close surveillance for isolated NRs is safe. Treatment with chemotherapy is recommended for patients with multiple NRs and DHPLN. Surgical management of NRs should focus on renal function preservation. LEVEL OF EVIDENCE: Treatment study, Level III.

Machine learning to predict pediatric choledocholithiasis: A Western Pediatric Surgery Research Consortium retrospective study.

BACKGROUND: The purpose of this study was to accurately predict pediatric choledocholithiasis with clinical data using a computational machine learning algorithm. METHODS: A multicenter retrospective cohort study was performed on children <18 years of age who underwent cholecystectomy between 2016 to 2019 at 10 pediatric institutions. Demographic data, clinical findings, laboratory, and ultrasound results were evaluated by bivariate analyses. An Extra-Trees machine learning algorithm using k-fold cross-validation was used to determine predictive factors for choledocholithiasis. Model performance was assessed using the area under the receiver operating characteristic curve on a validation dataset. RESULTS: A cohort of 1,597 patients was included, with an average age of 13.9 ± 3.2 years. Choledocholithiasis was confirmed in 301 patients (18.8%). Obesity was the most common comorbidity in all patients. Choledocholithiasis was associated with the finding of a common bile duct stone on ultrasound, increased common bile duct diameter, and higher serum concentrations of aspartate aminotransferase, alanine transaminase, lipase, and direct and peak total bilirubin. Nine features (age, body mass index, common bile duct stone on ultrasound, common bile duct diameter, aspartate aminotransferase, alanine transaminase, lipase, direct bilirubin, and peak total bilirubin) were clinically important and included in the machine learning algorithm. Our 9-feature model deployed on new patients was found to be highly predictive for choledocholithiasis, with an area under the receiver operating characteristic score of 0.935. CONCLUSION: This multicenter study uses machine learning for pediatric choledocholithiasis. Nine clinical factors were highly predictive of choledocholithiasis, and a machine learning model trained using medical and laboratory data was able to identify children at the highest risk for choledocholithiasis.

Multi-institutional assessment of the prevalence of neuroendocrine tumors in children undergoing laparoscopic appendectomy for acute appendicitis in the United States.

As non-operative management of acute appendicitis in children has become more common, missed incidental appendiceal pathology can be an unintended consequence. We assessed the prevalence of neuroendocrine tumors in appendectomy specimens from eight US children's hospitals from 2012 to 2021. The prevalence of neuroendocrine tumors (NET) was found to be 1:271, with a median age of 14 years and 62% female. Most tumors were small (median 6 mm; interquartile range [IQR]: 3-10), and no recurrence was noted during the follow-up period (median 22.5 months; IQR: 3-53). The possibility of delayed diagnosis of these tumors should be part of the discussion for non-operative management of pediatric acute appendicitis.

The Utility of Intraoperative Neuromonitoring in Pediatric Surgical Oncology.

BACKGROUND: Intraoperative nerve monitoring (IONM) is a technique used to decrease the possibility of nerve-associated morbidity and damage to nearby neural structures during complex surgical procedures. The use and potential benefits of IONM in pediatric surgical oncology are not well-described. METHODS: An overview of the current literature was performed to elucidate the various techniques that may be useful to pediatric surgeons for resection of solid tumors in children. RESULTS: The physiology and common types of IONM relevant to the pediatric surgeon are described. Important anesthetic considerations are reviewed. Specific applications for IONM that may be useful in pediatric surgical oncology are then summarized, including its use for monitoring the recurrent laryngeal nerve, the facial nerve, the brachial plexus, spinal nerves, and lower extremity nerves. Troubleshooting techniques regarding common pitfalls are then proposed. CONCLUSION: IONM is a technique that may be beneficial in pediatric surgical oncology to minimize nerve injury during extensive tumor resections. This review aimed to elucidate the various techniques available. IONM should be considered as an adjunct for the safe resection of solid tumors in children in the proper setting with the appropriate level of expertise. A multidisciplinary approach is advised. Additional studies are necessary to further clarify the optimal use and outcomes in this patient population. LEVELS OF EVIDENCE: Level III.

Pediatric DUCT Score: A Highly Specific Predictive Model for Choledocholithiasis in Children.

BACKGROUND: Current adult guidelines for the management of choledocholithiasis (CDL) may not be appropriate for children. We hypothesized adult preoperative predictive factors are not reliable for predicting CDL in children. STUDY DESIGN: A multicenter retrospective cohort study was performed evaluating children (≤18 years of age) who underwent cholecystectomy for gallstone disease at 10 children's hospitals. Univariate and multivariable analyses were used to identify factors independently associated with CDL. Patients were stratified into risk groups demonstrating the presence of predictive factors for CDL. Statistical analyses were performed, and chi-square analyses were used with a significance of p < 0.05. RESULTS: A total of 979 cholecystectomy patients were analyzed. The diagnosis of CDL was confirmed in 222 patients (22.7%) by magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, or intraoperative cholangiography. Three predictive factors were identified: (1) Dilated common bile duct ≥6 mm; (2) Ultrasound with Choledocholithiasis; and (3) Total bilirubin ≥1.8 mg/dL (pediatric DUCT criteria). Risk groups were based on the number of predictive factors: very high (3), high (2), intermediate (1), and low (0). The pediatric DUCT criteria demonstrated accuracies of >76%, specificity of >78%, and negative predictive values of >79%. Adult factors (elevated aspartate aminotransferase/alanine aminotransferase, pancreatitis, BMI, and age) did not independently predict CDL. Based on risk stratification, the high- and very-high-risk groups demonstrated higher predictive capacity for CDL. CONCLUSIONS: Our study demonstrated that the pediatric DUCT criteria, incorporating common bile duct dilation, choledocholithiasis seen on ultrasound, and total bilirubin ≥1.8 mg/dL, highly predicts the presence of choledocholithiasis in children. Other adult preoperative factors are not predictive of common bile duct stone in children.

Extended Lymph Node Sampling During Surgery for Pediatric Renal Tumors Concerning for Malignancy Does Not Increase Postoperative Complication Rates.

PURPOSE: Although Children's Oncology Group renal tumor protocols mandate lymph node sampling during extirpative surgery for pediatric renal tumors, lymph node sampling is often omitted or low yield. Concerns over morbidity associated with extended lymph node sampling have led to hesitancy in adopting a formal lymph node sampling template. We hypothesized that complications in children undergoing lymph node sampling for renal tumors would be rare, and not associated with the number of lymph nodes sampled. MATERIALS AND METHODS: A single-institution, retrospective review of patients aged 0-18 years undergoing extirpative renal surgery with lymph node sampling for a suspected malignancy between 2005 and 2019 was performed. Patients with 0 or an unknown number of lymph nodes sampled or <150 days of follow-up were excluded. A "clinically significant" complication was defined as any Clavien complication ≥III, small-bowel obstruction, chylous ascites, organ injury, or wound infection. The number of lymph nodes sampled and its influence on the odds of experiencing a clinically significant complication was examined. RESULTS: A total of 144 patients met inclusion criteria. Median patient age was 38 months. Twenty-one patients (15%) had a clinically significant complication, the most common of which was ileus/small-bowel obstruction (n=16). In a multivariable analysis, increased lymph node yield was not found to influence the odds of experiencing a clinically significant complication (P = .6). CONCLUSIONS: In this cohort, there was no statistically significant difference in clinically significant complications in patients who underwent more extensive lymph node sampling during surgery for a suspected malignant pediatric renal tumor. Future studies on protocol adherence, staging accuracy, and survival trends using a lymph node sampling template in these patients should be performed.

Pediatric Small Renal Masses: Can Tumor Size Predict Histology and the Potential for Nephron-sparing Surgery?

PURPOSE: The majority of children with unilateral renal masses suspicious for malignancy undergo radical nephrectomy, while nephron-sparing surgery is reserved for select cases. We investigated the impact of tumor size on the probability of histology. We hypothesized that pediatric small renal masses are more likely benign or non-Wilms tumor, thus potentially appropriate for nephron-sparing surgery. MATERIALS AND METHODS: The SEER (Surveillance, Epidemiology, and End Results) database was analyzed for patients aged 0-18 years diagnosed with a unilateral renal mass from 2000-2016. Statistical analysis was performed to help determine a tumor size cut point to predict Wilms tumor and assess the predictive value of tumor size on Wilms tumor histology. Additionally, a retrospective review was performed of patients 0-18 years old who underwent surgery for a unilateral renal mass at a single institution from 2005-2019. Statistical analysis was performed to assess the predictive value of tumor size on final histology. RESULTS: From the SEER analysis, 2,016 patients were included. A total of 1,672 tumors (82.9%) were Wilms tumor. Analysis revealed 4 cm to be a suitable cut point to distinguish non-Wilms tumor. Tumors ≥4 cm were more likely Wilms tumor (OR 2.67, P ≤ .001), but this was driven by the statistical significance in children 5-9 years old. From the institutional analysis, 134 patients were included. Ninety-seven tumors (72.3%) were Wilms tumor. Tumors ≥4 cm had higher odds of being Wilms tumor (OR 30.85, P = .001), malignant (OR 6.75, P = .005), and having radical nephrectomy-appropriate histology (OR 46.79, P < .001). CONCLUSIONS: The probability that a pediatric unilateral renal mass is Wilms tumor increases with tumor size. Four centimeters is a logical cut point to start the conversation around defining pediatric small renal masses and may help predict nephron-sparing surgery-appropriate histology.

Interhospital variability in localization techniques for small pulmonary nodules in children: A pediatric surgical oncology research collaborative study.

BACKGROUND: Pulmonary nodules that are deep within lung parenchyma and/or small in size can be challenging to localize for biopsy. This study describes current trends in performance of image-guided localization techniques for pulmonary nodules in pediatric patients. METHODS: A retrospective review was performed on patients < 21 years of age undergoing localization of pulmonary nodules at 15 institutions. Localization and resection success, time in interventional radiology (IR), operating room (OR) and total anesthesia time, complications, and technical problems were compared between techniques. RESULTS: 225 patients were included with an average of 1.3 lesions (range 1-5). Median nodule size and depth were 4 mm (range 0-30) and 5.4 mm (0-61), respectively. The most common localization techniques were: wire + methylene blue dye (MBD) (28%), MBD only (25%), wire only (14%), technetium-99 only (11%), coil + MBD (7%) and coil only (5%). Localization technique was associated with institution (p < 0.01); technique and institution were significantly associated with mean IR, OR, and anesthesia time (all p < 0.05). Comparing techniques, there was no difference in successful IR localization (range 92-100%, p = 0.75), successful resection (94-100%, p = 0.98), IR technical problems (p = 0.22), or operative complications (p = 0.16). CONCLUSIONS: Many IR localization techniques for small pulmonary nodules in children can be successful, but there is wide variability in application by institution and in procedure time. LEVEL OF EVIDENCE: Retrospective review, Level 3.

The pediatric surgery match from the applicant perspective.

BACKGROUND: Previous studies have examined characteristics critical to make a successful applicant in the pediatric surgery match. No previous study has focused on what applicants consider important as they interview pediatric surgery training programs. STUDY DESIGN: Successfully matched applicants to our training program from 2018 to 2020 were identified and a 49-question survey sent electronically. In addition to a descriptive analysis of responses, comparisons between those who matched into one of their top three programs and those who matched outside of their top three programs were made. A Likert scale was used and data are reported as mean (95% confidence intervals). RESULTS: One hundred and thirty individuals were surveyed. Sixty-three (48%) responded. The most important characteristics identified were total index case volume 1.94 (1.87, 2.00), interactions with faculty on the interview day 1.84 (1.74, 1.94), neonatal case volume 1.75 (1.61, 1.88) and degree of autonomy 1.63 (1.50, 1.77). Respondents also placed a high importance on the in-person interview experience 1.63 (1.49, 1.77). The least important training program characteristics were quantity of vacation time -0.24 (-0.52, 0.05), opportunity to participate in medical mission trips -0.25 (-0.54, 0.06), paid accommodation at the interview -0.29 (-0.57, 0.00), and access to a simulation laboratory -0.51 (-0.78, -0.24). CONCLUSION: Clinical volume, neonatal volume, faulty interactions and the experience on the interview day are the most important characteristics to applicants. There were few, if any, characteristics applicants felt were truly unimportant as they weighed their rank lists. Applicants also placed a high level of importance on an in-person interview LEVEL OF EVIDENCE: Level IV.

Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study.

INTRODUCTION: Kasai hepatoportoenterostomy is the standard of care for children with biliary atresia, but a majority of patients progress to end-stage liver disease and require a salvage liver transplant. Given the high failure rates of the hepatoportoenterostomy operation, some have advocated for primary liver transplantation as a superior treatment approach. The aim of this study was to compare outcomes of pediatric candidates with biliary atresia listed for primary vs. salvage liver transplantation. METHODS: The SRTR/OPTN database was retrospectively reviewed for all children with biliary atresia listed for liver transplant between March 2002 and February 2021. Candidates were categorized as primary liver transplant if they had not undergone previous abdominal surgery prior to listing and salvage liver transplant if they had. Salvage transplants were further categorized as early failure if listed within the first year of life or late failure if listed at an older age. RESULTS: 3438 children with biliary atresia were listed for transplant during the study period, with 15% of them listed for a primary transplant, 17% for salvage transplant after early failure, and 67% after late failure. Recipients of salvage liver transplant with late failure had lower bilirubin levels and were less critically ill as demonstrated by MELD/PELD scores and hospitalization status. Correspondingly, these recipients had higher waiting list and graft survival, though this did not remain statistically significant after adjustment in multivariable models. There were no differences in waiting list, recipient, or graft survival with primary vs. salvage liver transplant after early failure. CONCLUSION: Kasai hepatoportoenterostomy should remain the standard of care in biliary atresia as it may delay need for transplant beyond the first year of life in a subset of recipients and does not jeopardize subsequent transplant outcomes, even with early failure. LEVELS OF EVIDENCE: Retrospective cohort study (Level III).

Predictors of survival following liver transplantation for pediatric hepatoblastoma and hepatocellular carcinoma: Experience from the Society of Pediatric Liver Transplantation (SPLIT).

Management of unresectable pediatric hepatoblastoma (HB) and hepatocellular carcinoma (HCC) remains challenging. The Society of Pediatric Liver Transplantation (SPLIT) database was used to study survival predictors in pediatric liver transplantation (LT) for HB and HCC. Event-free survival (EFS), associated risk factors, and postoperative complications were studied in children requiring LT for HB/HCC at 16 SPLIT centers. Three-year EFS was 81% for HB (n = 157) and 62% for HCC (n = 18) transplants. Of HB transplants, 6.9% were PRETEXT II and 15.3% were POST-TEXT I/II. Tumor extent did not impact survival (p = NS). Salvage (n = 13) and primary HB transplants had similar 3-year EFS (62% versus 78%, p = NS). Among HCC transplants, 3-year EFS was poorer in older patients (38% in ≥8-year-olds vs 86% <8-year-olds) and those with larger tumors (48% for those beyond versus 83% within Milan criteria, p = NS). Risk of infection (HR 1.5, 95% CI 1.1-2.2, p = .02) and renal injury (HR 2.4, 95% CI 1.7-3.3, p < .001) were higher in malignant versus nonmalignant LT. Survival is favorable for pediatric HB and HCC LT, including outcomes after salvage transplant. Unexpected numbers of LTs occurred in PRE/POST-TEXT I/II tumors. Judicious patient selection is critical to distinguish tumors that are potentially resectable; simultaneously, we must advocate for patients with unresectable malignancies to receive organs.

Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children With Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY OF BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status ( P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor(HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs resected (HR 1.78; P = 0.02). Overall, patient-reported quality of life is not impaired after chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.

Inflammation Drives MicroRNAs to Limit Hepatocyte Bile Acid Transport in Murine Biliary Atresia.

BACKGROUND: Biliary atresia (BA) is an inflammatory pediatric cholangiopathy with only surgical means for treatment. Many contributors to bile acid synthesis and transport have previously been reported to be downregulated in patients with BA; yet, the driving factors of the abnormal bile acid synthesis and transport in regard to BA have not been previously studied. MATERIALS AND METHODS: Wild type or Ig-α-/- mice were injected with salt solution (control) or rotavirus on day of life 0, and analyses were performed on day of life 14. The mRNA levels of bile acid transporters/nuclear receptors and liver microRNAs (miRNAs) were compared between groups. A mouse hepatocyte cell line was used to examine the effects of innate cytokines on miRNA levels and bile acid transporter/nuclear receptor expression and miRNAs on bile acid transporter/nuclear receptor expression. RESULTS: BA mice had significantly increased mRNA expression of innate cytokines and miRNAs known to bind bile acid transporters/nuclear receptors (miRNAs -22-5p, -34a-5p, and -222-3p) and decreased mRNA expression of bile acid transporters and nuclear receptors. In vitro, TNF-α and IL-1ß decreased BSEP and CYP7A1 while increasing miRNA-34a-5p and miRNA 222-3p. LXR, SHP, CYP7A1, NTCP, and MRP2 were decreased by miRNA-34a-5p, whereas miRNA-222-3p decreased NTCP and MRP4. TNF-α and IL-1ß increased expression of miRNAs 34a-5p and 222-3p and these miRNAs then decrease expression of multiple bile acid transporters and nuclear receptors. CONCLUSIONS: Loss of bile acid transporters increases hepatotoxicity via bile acid retention. Therapeutic agents that increase bile acid transport or nuclear receptor functioning should be investigated in BA.

Filling the gap: Objective data to guide pediatric surgery applicants who do not match on the first attempt.

PURPOSE: Acquiring a pediatric surgery fellowship is an arduous process, with less than half of applicants matching each year. For those who fail to match initially but remain steadfast in their goal to become a pediatric surgeon, choosing a valuable postresidency path can be challenging. Furthermore, objective data to evaluate the utility of pediatric surgical subspecialty fellowships (PSSF) are lacking. METHODS: PSSF training programs were identified on the APSA website. Names and contact information of PSSF graduates between 2014 and 2018 were obtained from individual training programs. Each graduated fellow was then contacted to participate in a voluntary 14-question survey. RESULTS: Names of 47 graduated fellows were obtained from programs listed on the APSA website. Among them, 30 (64%) ultimately matriculated into a pediatric surgery fellowship, 11 (37%) of whom matched at the same institution they completed their PSSF. The type of PSSF was not predictive of matriculation into a pediatric surgical fellowship (p = 0.43). Thirty-two (68%) of the 47 study participants completed a satisfaction survey. While all but two graduates found their fellowships to be at least satisfactory, those who matched were more likely to recommend their PSSF compared to those who did not (100% vs 67%, respectively, p = 0.02). Within the cohort of fellows who did not ultimately match into pediatric surgery (n = 17) and completed a survey (n = 9), all who completed a critical care fellowship (4/4) currently use/or plan to use the skills obtained during their PSSF while only two of five (40%) completing other PSSFs use them. CONCLUSION: The matriculation rate from a PSSF into a pediatric surgery fellowship was 64% with no significant difference among subspecialties. Of fellows who did not match, those completing a critical care PSSF were more likely to use their fellowship training than those completing a different PSSF. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: III.

Error traps and culture of safety in biliary atresia.

Biliary atresia is a progressive inflammatory sclerosing disease of the bile ducts in the neonatal liver. Without surgical intervention these patients are destined to succumb to the disease. The development of the hepatoportoenterostomy in 1959 and liver transplantation in 1963 ushered a new era of success treating these patients. While many surgical modifications and adjuncts to treatment have been attempted over the last 50 years, the mainstay of treatment to give the child the best chance at prolonged survival with the native liver is a properly performed operation, in a timely fashion, with minimal post-operative complications. This review presents the authors current practice guideline to achieve these goals.