Surgical Outcomes for Patients With Trisomy 21 and Hirschsprung's Disease: An NSQIP-Pediatric Study.

INTRODUCTION: Previous studies have demonstrated worse outcomes for Hirschsprung's disease (HD) procedures in Trisomy 21 (T21) patients. Using a large national database, we sought to investigate surgical outcomes in HD patients with T21 compared to non-T21 patients. METHODS: We utilized the deidentified National Surgical Quality Improvement Program Pediatric database from 2012 to 2021. Using International Classification of Diseases, Ninth Revision codes, children <18 y old with HD were included and stratified by T21 diagnosis. Demographics, Current Procedural Terminology codes, case characteristics, length of hospital stay, and postoperative complications were analyzed. RESULTS: Of 3456 HD patients, 12.0% (n = 413) patients had a concurrent diagnosis of T21. Pull-through (PT) procedures accounted for 54.9% of surgeries (n = 1896), of which 10.0% (n = 189) had T21. T21 patients who underwent PT had a younger gestational age (P < 0.0001), cardiac risk factors (P < 0.0001), hematologic disorders (P < 0.0001), higher American Society of Anesthesiologists class (P < 0.0001), and were older at their index operation (P = 0.03). Though operative times were similar, T21 patients had a longer total length of stay (P = 0.0263), postoperative length of stay (P = 0.0033), and more unplanned reoperations (P = 0.0094). Though only significant in unadjusted analyses, T21 patients had more postoperative complications after PT (P = 0.0034), specifically deep surgical site infections (P = 0.009), organ/space surgical site infections (P = 0.004), wound disruption (P < 0.001), and sepsis (P = 0.025). CONCLUSIONS: We confirm significant differences exist between T21 and non-T21 patients undergoing HD procedures, particularly increased total length of stay, postoperative length of stay, and unplanned reoperations. Understanding these differences will lead to more optimal treatment plans for this unique patient population.

Integration of Certified Child Life Specialists to Decrease in Periprocedural Benzodiazepine Use: A Pilot Study.

INTRODUCTION: Periprocedural anxiety is common in pediatric patients and is characterized by tension, anxiety, irritability, and autonomic activation. Periprocedural anxiety increases during certain events including admission to the preoperative area, separation from caregivers, induction of anesthesia, and IV placement. A study of children aged 2-12 showed that perioperative anxiety in children may be influenced by high parental anxiety and low sociability of the child. While these are nonmodifiable variables in the perioperative setting, there are numerous ways to ameliorate both parental and patient anxiety including the use of certified child life specialists (CCLSs) to aid in child comfort. In this study, our objective was to evaluate the integration of CCLS in our perioperative setting on the rate of benzodiazepine use. METHODS: We used a prospectively maintained database to identify patients undergoing outpatient elective surgical and radiologic procedures from July 2022 to September 2023 and January 2023 to September 2023 respectively. CCLSs were used to work with appropriately aged children in order to decrease the use of benzodiazepines and reduce possible adverse events associated with their use. RESULTS: A total of 2175 pediatric patients were seen by CCLS in same day surgery from July 2022 to September 2023. During this period, midazolam use decreased by an average of 11.4% (range 6.2%-19.3%). An even greater effect was seen in the radiologic group with 73% reduction. No adverse events were reported during this period. CONCLUSIONS: CCLSs working with age-appropriate patients in the periprocedural setting is a useful adjunct in easing anxiety in pediatric patients, reducing the need for periprocedural benzodiazepine administration and the risk of exposure to unintended side effects.

Initiation of the Virtual Interview: A 5-Y Evaluation of General Surgery Residency Match Results.

INTRODUCTION: The virtual residency application season posed numerous challenges for the 2021 residency match process. Many residency programs are exploring relationships between this novel format and the match process. The purpose of this study was to compare one of the largest general surgery residency program's applications and match data from preCOVID years to the 2021 virtual cycle. MATERIALS AND METHODS: A retrospective review was performed of applicants to a single general surgery residency program from 2017 to 2021. The primary outcome was the number of locally matched applicants. The secondary outcomes were the total number of applications, change in applicant demographics, and variability of the geographic spread during this study period. Chi-square tests of independence, Fisher's exact tests, and negative binomial regression were performed. RESULTS: 6819 applicants were included in the study. In 2021, an increase in applications was observed. The distribution of 2021 applications was statistically different from previous years regarding gender and race (P < 0.0001). The 2021 application cycle had a greater proportion of applicants from the United States (P < 0.0001) and southern US medical schools (P = 0.008). While the 2021 cycle had significantly more interviews (P = 0.013), there were no significant differences in the demographic composition of interviewees. During the 2021 application year, all 11 matches were from southern medical schools and there was a trend to more matched female applicants compared to previous years. CONCLUSIONS: During the 2021 COVID-19 virtual match cycle, an increase in both the number of applications and number of interviews for general surgery residency was identified. The characteristics of applicants who interviewed and matched were not different when compared to previous years. As virtual interviews may become more commonplace, it is important to assess all factors that may be involved in the dynamic residency application process.

Caring for Children in the Juvenile Justice System: A Trauma and Surgical Subspecialty-Focused Approach.

INTRODUCTION: Youth in the juvenile justice system are a vulnerable, high-risk population. While the role of pediatricians and mental health professionals in providing care for these children is well studied, the surgical needs of this population are not well understood. We sought to characterize the physical trauma and surgical subspecialty needs of this population. METHODS: A retrospective chart review was performed of all children transported under custody to a stand-alone urban children's hospital. Demographic information and inpatient and outpatient encounter data were collected and analyzed. RESULTS: Between January 2020 and March 2021, 74 patients were transported for 199 subspecialty evaluations. Sixty-nine (93%) were male, 66 (89%) identified as Black, and the median age was 16 y (range, 13-20). Of all patients, 19% had at least one documented medical condition, 43% had behavioral health history, and 73% had previous arrest. Of the 199 encounters, 137 were for physical trauma (65%). Of these, 47 (34%) were for physical trauma incurred at the time of their arrest. Sixty-three patients (85%) experienced previous physical trauma (69% blunt, 12% penetrating, and 7% both), 54% had documented head trauma, 23% had a history of self-harm, and 60% of girls had experienced sexual trauma. Of the 54 children with a previous arrest, 91% had a history of physical trauma compared to 70% who were not previously incarcerated (P = 0.03). CONCLUSIONS: Most subspecialty and emergency encounters for incarcerated children are for physical trauma, revealing an opportunity for trauma-focused care in this vulnerable population. Pediatric surgeons and emergency physicians play a major role in the care of incarcerated children.

Inpatient Rehabilitation After Pediatric and Adolescent Trauma: Outcomes and Discharge Needs.

INTRODUCTION: Traumatic injury is the leading cause of pediatric mortality and morbidity in the United States. Pediatric trauma survivors requiring inpatient rehabilitation (IPR) require coordinated, multispecialty follow-up. Knowledge of the nature and level of disability is necessary for planning this continued care that is specific to the needs of pediatric trauma patients. This study aims to describe the outcomes of pediatric and adolescent trauma patients using measures of functional progression. MATERIALS AND METHODS: A retrospective review of trauma patients aged ≤18 y admitted to IPR between January 2018 and December 2020 at the only certified pediatric rehabilitation center in the region was performed. RESULTS: Ninety five children and adolescents were admitted to IPR after traumatic injury with diagnoses of multitrauma (MT, N = 18), traumatic brain injury (TBI, N = 59), and spinal cord injury (SCI, N = 18). School aged children returned to school at high rates for all injury types (MT: 86.7%, TBI: 97.4%, SCI: 93.8%, P = ns). All groups had similar hospital and rehabilitation length of stay, and most patients required a durable medical equipment at discharge (79%). Using pediatric functional independence measure scoring progression from admission to discharge from IPR, SCI patients made significant improvement in bladder function and the least improvement in stair function. Patients sustaining a TBI made significant improvement in memory and comprehension tasks. CONCLUSIONS: Pediatric and adolescent trauma patients admitted to IPR had a positive progression during their therapy but required variable ongoing care depending on the mechanism of injury. Excellent rates of returning to school were seen across the three injury types.

Prominin-1 Promotes Biliary Fibrosis Associated With Biliary Atresia.

In patients with biliary atresia (BA), the extent of intrahepatic biliary fibrosis negatively correlates with successful surgical bypass of the congenital cholangiopathy as well as subsequent transplant-free survival. We recently linked the expansion of a population of prominin-1 (Prom1)-expressing hepatic progenitor cells to biliary fibrogenesis. Herein, we hypothesized that Prom1-expressing progenitor cells play a role in BA-associated fibrosis. Rhesus rotavirus (RRV)-mediated experimental BA was induced in newborn mice homozygous for the transgene Prom1cre-ert2-nlacz , which was knocked in to the Prom1 gene locus, thus creating functional Prom1 knockout (KO) mice, and their wildtype (WT) littermates. Clinical data and tissue samples from BA infants from the Childhood Liver Disease Research Consortium were analyzed. Extrahepatic biliary obliteration was present in both WT and KO mice; there was no difference in serum total bilirubin (TBili) levels. The intrahepatic periportal expansion of the PROM1pos cell population, typically observed in RRV-induced BA, was absent in KO mice. RRV-treated KO mice demonstrated significantly fewer cytokeratin-19 (CK19)-positive ductular reactions (P = 0.0004) and significantly less periportal collagen deposition (P = 0.0001) compared with WT. RRV-treated KO mice expressed significantly less integrin-ß6, which encodes a key biliary-specific subunit of a transforming growth factor (TGF) ß activator (P = 0.0004). Infants with successful biliary drainage (Tbili ≤1.5 mg/dL within 3 months postoperatively), which is highly predictive of increased transplant-free survival, expressed significantly less hepatic PROM1, CK19, and COLLAGEN-1α compared with those with TBili >1.5 (P < 0.05). Conclusion: Prom1 plays an important role in biliary fibrogenesis, in part through integrin-mediated TGF pathway activation.

Impact of Additional Anomalies on Postnatal Outcomes in Congenital Lung Malformations.

BACKGROUND: Approximately 20% of fetuses diagnosed with congenital lung malformations (CLMs) are found to have additional anomalies. We aim to determine if additional anomalies have an impact on postnatal outcomes for patients with CLMs. METHODS: After institutional review board approval, we performed a retrospective review of live-born patients with CLMs from 2008 to 2018. All patients were prenatally diagnosed with CLMs. Clinical information pertaining to additional congenital anomalies and outcomes was collected from the electronic health record and analyzed. RESULTS: Of the 88 patients who had a prenatal diagnosis of CLMs, 20.5% had additional anomalies. Ten of the 18 patients (56%) were considered to have a major anomaly in addition to CLMs. Outcomes for patients electing nonoperative management of CLMs were similar between those with and without an additional anomaly. Although patients with an additional anomaly were more likely to have perinatal respiratory complications (44% versus 17%, P = 0.03), the number of preoperative clinic and emergency department visits, age at surgery, minimally invasive approach to surgical resection of CLM, estimated blood loss, length of hospital stay, intubation, duration of intubation, 30-day postoperative complications, and long term sequelae were not statistically different. This held true when stratified for major versus minor anomalies. CONCLUSIONS: Twenty percent of fetuses diagnosed with CLM in our population have additional anomalies. Newborns with additional anomalies have a higher risk of pre-excision pulmonary complications. However, the overall outcomes of all patients with CLMs are similar.

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12-24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs. >24 months postoperatively, respectively; P < 0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. CONCLUSION: This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645-1654).

Liquid chromatography-mass spectroscopy in the diagnosis of biliary atresia in children with hyperbilirubinemia.

BACKGROUND: Biliary atresia (BA) is difficult to distinguish from other causes of cholestasis. We evaluated the use of liquid chromatography-mass spectroscopy (LC-MS) and bile acid profiles in the rapid, noninvasive diagnosis of BA. MATERIALS AND METHODS: Following Institutional Animal Care and Use Committee and Institutional Review Board approval, we used LC-MS to measure 26 bile acids in serum and stool samples from experimental models of BA and in urine, stool, and serum samples from non-cholestatic and cholestatic human infants. RESULTS: We first evaluated the utility of LC-MS to distinguish bile acid profiles between sham, bile duct ligation, and 3,5-diethoxycarbonyl-1,4-dihydrocollidine mouse models of BA. Serum bile acids were significantly higher and stool bile acids were significantly lower in experimental BA. Next, we evaluated samples from non-cholestatic, cholestatic non-BA, and BA infants. There was no significant difference between cholestatic non-BA and BA stool and urine samples. However, primary bile acids were significantly higher in BA versus cholestatic non-BA samples (128.1 ± 14.2 versus 61.2 ± 20.5 µM). In addition, the primary, conjugated bile acids glycochenodeoxycholic acid and taurochenodeoxycholic acid were significantly elevated in BA compared with cholestatic non-BA serum samples. Using a receiver operating characteristic curve, we found that a serum glycochenodeoxycholic acid concentration of 30 µM had a sensitivity of 100%, specificity of 83.3%, positive predictive value of 88.9%, and negative predictive value of 100% in the diagnosis of BA. CONCLUSIONS: Our data indicate that bile acid patterns can be used to distinguish experimental and human BA from non-cholestatic and, more importantly, cholestatic disease. This suggests that LC-MS may be useful in the accurate, rapid, and non-invasive diagnosis of BA.

Retrospective Cohort Comparison of Fall Height in Children in the Greater Los Angeles Area: Targeting Populations for Injury Prevention.

The purpose of this study was to determine whether falls from significant height occur more frequently in young children. We conducted a 10-year (2004-2014), comparative study using a retrospective cohort of 4713 children (< 18 years) from the Los Angeles county trauma database who were evaluated for a fall. Exposure was fall height, dichotomized into < 10 ft/low-risk fall and > 10 ft/high-risk fall. Primary outcome was age of fall. Secondary outcomes were disposition from emergency department, injuries, resource utilization, and mortality. Of all falls, 4481 (95%) were low-risk and 232 (5%) high-risk. High-risk falls were more frequent in children 1-3 years old (58 vs. 30%, p < 0.01), associated with higher frequency of intracranial hemorrhage (19 vs. 10%, p < 0.01), intubation (11 vs. 1%, p < 0.01), and neurosurgical procedure (2 vs. 0.8%, p = 0.04). There was no difference in mortality (0.86 vs. 0.13%, p = 0.06). In Los Angeles County, children 1-3 years old are most likely to suffer high-risk falls, which are associated with serious injury. Integration of fall prevention education into routine anticipatory guidance should be strongly considered for children 1-3 years old.

Evaluation of Highest Level Pediatric Trauma Activation Criteria.

BACKGROUND: Despite the presence of a tiered in-hospital trauma triage system for the past decade, trauma centers still struggle with a definitive list of highest level activation criteria. In 2002, the American College of Surgeons (ACS) mandated 6 criteria for highest level activation. However, it is unknown if pediatric trauma centers follow these criteria. The purpose of this study is to identify and categorize the highest level pediatric trauma criteria used by pediatric trauma centers in the United States. METHODS: In collaboration with the ACS, we reviewed activation criteria for highest level trauma activation for all ACS-verified level I pediatric trauma centers in the United States. Criteria were sorted by 2 reviewers into categories of indicators used for activation: patient demographic, physiologic, anatomic, intervention/resource usage, mechanism, and other. RESULTS: A total of 51 unique criteria for highest level trauma activation were identified from 54 (96%) of 56 level I pediatric trauma centers. Each center used between 1 and 29 criteria. A total of 42.6% of pediatric trauma centers followed all 6 criteria recommended by ACS. The most commonly omitted criterion was emergency physician discretion. The most common criteria not included in the ACS recommendations, but included in the highest level activation criteria, were amputation proximal to wrist or ankle (63%), and spinal cord injury/paralysis (63%). CONCLUSIONS: There is wide variation in the criteria used for highest level trauma activation among pediatric trauma centers. Further research investigating individual or grouped criteria to determine the most sensitive and specific criteria are necessary for appropriate triage and resource usage.

Notch signaling promotes ductular reactions in biliary atresia.

BACKGROUND: Biliary atresia (BA) is a congenital, progressive, fibro-obliterative disease of the extrahepatic biliary tree and the most common cause of end-stage liver disease in children. BA is characterized by extensive intrahepatic proliferating ductular reactions that may contribute to biliary fibrosis. Lineage tracing during experimental cholestasis indicates that cells within ductular reactions derive from PROM1-expressing hepatic progenitor cells. Given the role of Notch signaling in normal biliary development, we hypothesize that activated Notch signaling promotes the formation of ductular reactions in BA. METHODS: Liver samples collected from BA infants at Kasai portoenterostomy and age-matched controls, as well as from wild-type and Prom1 knockout mice with 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC)-induced experimental cholestasis were analyzed histologically using immunofluorescence and by quantitative polymerase chain reaction. RESULTS: Increased expression of genes encoding Notch ligand JAG1 and its receptor NOTCH2 was observed in BA livers compared with control by quantitative polymerase chain reaction analyses. Livers of DDC-treated mice, which exhibit cytokeratin-19-positive ductular reactions typical of BA livers, demonstrated significant increases in the expression level of the gene encoding Notch2, as well as downstream Notch target gene Hes1 compared with control. Prom1 knockout mice exhibit diminished ductular reactions and decreased levels of Jag1 and Hes1 compared with littermate controls. CONCLUSIONS: Human BA and cholestasis induced by DDC are associated with Notch signaling activation. Null mutation of Prom1 is associated with decreased ductular reactions and decreased Notch signaling activation during DDC treatment. These data are consistent with Notch signaling promoting ductular reactions of Prom1 expressing progenitor cells in BA.

Outcomes of congenital diaphragmatic hernia repair on extracorporeal life support.

PURPOSE: Extracorporeal life support (ECLS) is applied to refractory pulmonary hypertension in congenital diaphragmatic hernia (CDH). We evaluate the single-center outcomes of infants with CDH to determine the utility of late repair on ECLS versus repair post-decannulation. METHODS: Records of infants with CDH (2004-2014) were retrospectively reviewed. RESULTS: CDH was diagnosed in 177 infants. Sixty six (37%) underwent ECLS, of which, 11 died prior to repair, 33 were repaired post-decannulation, and 22 were repaired on ECLS. Repair was delayed in patients on ECLS (19 versus 10 days, p < 0.001). Patients repaired on ECLS had longer ECLS runs (22 versus 12 days, p < 0.001) and higher rates of bleeding and mortality than those repaired post-decannulation. Survival was 54% in infants undergoing ECLS, 65% in those who underwent repair, 36% in those repaired during ECLS, and 85% in those who were decannulated prior to repair. Eighteen percent (N = 4) of deaths after repair on ECLS were attributable to surgical bleeding. The remainder was due to pulmonary hypertension or sepsis. CONCLUSION: Infants who underwent CDH repair post-decannulation had excellent outcomes and no mortalities attributable to repair. Neonates who underwent repair on ECLS late on bypass had the lowest survival rate with only 18% of mortality in this cohort attributable to surgical bleeding.

Re-evaluation of liver transaminase cutoff for CT after pediatric blunt abdominal trauma.

PURPOSE: Current guidelines for computed tomography (CT) after blunt trauma were developed to capture all intra-abdominal injuries (IAI). We hypothesize that current AST/ALT guidelines are too low leading to unnecessary CT scans for children after blunt abdominal trauma (BAT). METHODS: Patients who received CT of the abdomen after blunt trauma at our Level I Pediatric Trauma Center were stratified into a high risk (HR) (liver/spleen/kidney grade ≥III, hollow viscous, or pancreatic injuries) and low risk (LR) (liver/kidney/spleen injuries grade ≤II, or no IAI) groups. RESULTS: 247 patients were included. Of the 18 patients in the HR group, two required surgery (splenectomy and sigmoidectomy). Transfusion was required in 30% of grade III and 50% of grade IV injuries. Eleven (5%) patients in LR group were transfused for indications other than IAI, and none were explored surgically. Both AST (r = 0.44, p < 0.001) and ALT (r = 0.43, p < 0.001) correlated with grade of liver injury. Using an increased threshold of AST/ALT, 400/200 had a negative predictive value of 96% in predicting the presence of HR liver injuries. CONCLUSION: The current cutoff of liver enzymes leads to over-identification of LR injuries. Consideration should be given to an approach that aims to utilize CT in pediatric BAT that identifies clinically HR injury.

Characterization of hepatic stellate cells, portal fibroblasts, and mesothelial cells in normal and fibrotic livers.

BACKGROUND & AIMS: Contribution of hepatic stellate cells (HSCs), portal fibroblasts (PFs), and mesothelial cells (MCs) to myofibroblasts is not fully understood due to insufficient availability of markers and isolation methods. The present study aimed to isolate these cells, characterize their phenotypes, and examine their contribution to myofibroblasts in liver fibrosis. METHODS: Liver fibrosis was induced in Collagen1a1-green fluorescent protein (Col1a1(GFP)) mice by bile duct ligation (BDL), 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) diet, or CCl4 injections. Combining vitamin A (VitA) lipid autofluorescence and expression of GFP and glycoprotein M6a (GPM6A), we separated HSCs, PFs, and MCs from normal and fibrotic livers by fluorescence-activated cell sorting (FACS). RESULTS: Normal Col1a1(GFP) livers broadly expressed GFP in HSCs, PFs, and MCs. Isolated VitA+ HSCs expressed reelin, whereas VitA-GFP+GPM6A- PFs expressed ectonucleoside triphosphate diphosphohydrolase-2 and elastin. VitA-GFP+GPM6A+ MCs expressed keratin 19, mesothelin, and uroplakin 1b. Transforming growth factor (TGF)-ß1 treatment induced the transformation of HSCs, PFs, and MCs into myofibroblasts in culture. TGF-ß1 suppressed cyclin D1 mRNA expression in PFs but not in HSCs and MCs. In biliary fibrosis, PFs adjacent to the bile duct expressed α-smooth muscle actin. FACS analysis revealed that HSCs are the major source of GFP+ myofibroblasts in the injured Col1a1(GFP) mice after DDC or CCl4 treatment. Although PFs partly contributed to GFP+ myofibroblasts in the BDL model, HSCs were still dominant source of myofibroblasts. CONCLUSION: HSCs, PFs, and MCs have distinct phenotypes, and PFs partly contribute to myofibroblasts in the portal triad in biliary fibrosis.

Recent advances in the pathogenesis and management of biliary atresia.

PURPOSE OF REVIEW: The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia. RECENT FINDINGS: Immunologic studies have further characterized roles of helper T-cells, B-cells, and natural killer cells in the immune dysregulation following viral replication within and damage of biliary epithelium. Prominin-1-expressing portal fibroblasts may play an integral role in the biliary fibrosis associated with biliary atresia. A number of genetic polymorphisms have been characterized as leading to susceptibility for biliary atresia. Postoperative corticosteroid therapy is not associated with greater transplant-free survival. Newborn screening may improve outcomes of infants with biliary atresia and may also provide a long-term cost benefit. SUMMARY: Although recent advances have enhanced our understanding of pathogenesis and clinical management, biliary atresia remains a significant challenge requiring further investigation.

Assessing the Impact of Neighborhood and Built Environment on Pediatric Perioperative Care: A Systematic Review of the Literature.

CONTEXT: Neighborhood and built environment encompass one key area of the Social Determinants of Health (SDOH) and is frequently assessed using area-level indices. OBJECTIVE: We sought to systematically review the pediatric surgery literature for use of commonly applied area-level indices and to compare their utility for prediction of outcomes. DATA SOURCES: A literature search was conducted using PubMed, Ovid MEDLINE, Ovid MEDLINE Epub Ahead of Print, PsycInfo, and an artificial intelligence search tool (1/2013-2/2023). STUDY SELECTION: Inclusion required pediatric surgical patients in the US, surgical intervention performed, and use of an area-level metric. DATA EXTRACTION: Extraction domains included study, patient, and procedure characteristics. RESULTS: Area Deprivation Index is the most consistent and commonly accepted index. It is also the most granular, as it uses Census Block Groups. Child Opportunity Index is less granular (Census Tract), but incorporates pediatric-specific predictors of risk. Results with Social Vulnerability Index, Neighborhood Deprivation Index, and Neighborhood Socioeconomic Status were less consistent. LIMITATIONS: All studies were retrospective and quality varied from good to fair. CONCLUSIONS: While each index has strengths and limitations, standardization on ideal metric(s) for the pediatric surgical population will help build the inferential power needed to move from understanding the role of SDOH to building meaningful interventions towards equity in care. TYPE OF STUDY: Systematic Review. LEVEL OF EVIDENCE: Level III.

The Sinister Surgeon: Advanced Pediatric Minimally Invasive Surgery for Left-Handed Surgeons.

Purpose: Left-handed (LH) surgeons face unique challenges in their training and practice. Contrary to the historical and unjust perception of technical inferiority, LH surgeons can thrive under appropriate mentorship and support. Here, we describe modifications to pediatric minimally invasive surgeries to benefit the LH surgeon. Methods: The surgical approaches to common and complex pediatric surgical operations were modified to facilitate training of an LH pediatric surgical fellow. Results: Preoperative preparation, including communication with the operating room team, patient positioning, and provision of appropriate equipment, allowed the procedures to be accomplished in a safe and efficient manner. Conclusions: Modifying complex minimally invasive surgery to allow for use of the dominant hand is feasible and safe.