The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study.

PURPOSE: The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia. METHODS: A multi-institutional, retrospective cohort study was performed at nine tertiary-level children's hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance. RESULTS: Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97-0.99). CONCLUSION: Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.

Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene.

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that is often accompanied by other anomalies. Although the role of genetics in the pathogenesis of CDH has been established, only a small number of disease-associated genes have been identified. To further investigate the genetics of CDH, we analyzed de novo coding variants in 827 proband-parent trios and confirmed an overall significant enrichment of damaging de novo variants, especially in constrained genes. We identified LONP1 (lon peptidase 1, mitochondrial) and ALYREF (Aly/REF export factor) as candidate CDH-associated genes on the basis of de novo variants at a false discovery rate below 0.05. We also performed ultra-rare variant association analyses in 748 affected individuals and 11,220 ancestry-matched population control individuals and identified LONP1 as a risk gene contributing to CDH through both de novo and ultra-rare inherited largely heterozygous variants clustered in the core of the domains and segregating with CDH in affected familial individuals. Approximately 3% of our CDH cohort who are heterozygous with ultra-rare predicted damaging variants in LONP1 have a range of clinical phenotypes, including other anomalies in some individuals and higher mortality and requirement for extracorporeal membrane oxygenation. Mice with lung epithelium-specific deletion of Lonp1 die immediately after birth, most likely because of the observed severe reduction of lung growth, a known contributor to the high mortality in humans. Our findings of both de novo and inherited rare variants in the same gene may have implications in the design and analysis for other genetic studies of congenital anomalies.

Attitudes Affecting Decision-Making for Use of Radiologic Enteral Contrast in the Management of Pediatric Adhesive Small Bowel Obstruction: A Survey Study of Pediatric Surgeons.

BACKGROUND: Pediatric surgeons are often asked to treat clinical problems for which little high-quality data exist. For adults with adhesive small bowel obstruction (ASBO), water soluble contrast-based protocols are used to guide management. Little is known about their utility in children. We aimed to better understand key factors in clinical decision-making processes and integration of adult based data in pediatric surgeon's approach to ASBO. METHODS: We administered a web-based survey to practicing pediatric surgeons at institutions comprising the Western Pediatric Surgery Research Consortium. RESULTS: The response rate was 69% (78/113). Over half of respondents reported using contrast protocols to guide ASBO management either routinely or occasionally (n = 47, 60%). Common themes regarding the incorporation of adult-based data into clinical practice included the need to adapt protocols for pediatric patients, the dearth of pediatric specific data, and the quality of the published adult evidence. CONCLUSIONS: Our findings demonstrate that pediatric surgeons use contrast-based protocols for the management of ASBO despite the paucity of pediatric specific data. Furthermore, our survey data help us understand how pediatric surgeons incorporate adult based evidence into their practice.

Courtesy Authorship Practices Among First and Senior Authors: Evaluation of Motivations, Gender Bias, and Inequities.

OBJECTIVES: A courtesy author is an individual who has not met authorship criteria but is listed as an author. This practice is common and often seen as victimless. Because publications are used for funding and promotion decisions, it is critical to understand biases in this practice. METHODS: An anonymous survey was conducted from March to October 2020 of first and senior authors of publications from 2014 to 2015 in 8 surgical journals. Authors were surveyed about demographic data, practice setting, and courtesy author practices. RESULTS: Three hundred forty-one authors responded (16% response rate). 75% were from academic practice settings. 14% reported adding courtesy authors 5 or more times in the past year. Courtesy authors were more often male (80%, P = 0.023), older (75%), and of higher academic rank (65%) than first/senior authors. All author groups were >75% white. When a reason was reported, 46% added a courtesy author due to avoid retaliation; 64% to avoid awkwardness. 26% expected reciprocal authorship offers. 92% of respondents acknowledge understanding International Committee of Medical Journal Editors authorship criteria. Women were less common among those added from goodwill than those added from fear (P = 0.039.) When courtesy authors were of a lower rank than first/senior authors, they were nearly twice as likely to be female (P = 0.0056) or non-white (P = 0.0184.). CONCLUSION: Courtesy authors were more often male, older, and higher rank than first/senior authors. Fear of career consequences was a major motivator for including courtesy authors. Understanding the motivations and pressures leading to courtesy authorship will help to correct this practice.

Minimizing Variance in Gastroschisis Management Leads to Earlier Full Feeds in Delayed Closure.

BACKGROUND: Limited guidance exists regarding appropriate timing for feed initiation and advancement in gastroschisis. We hypothesized that implementation of a gastroschisis management protocol would allow for standardization of antibiotic and nutritional treatment for these patients. METHODS: We conducted a retrospective comparison of patients with simple gastroschisis at two pediatric hospitals before and after initiation of our gastroschisis care protocol. Complicated gastroschisis and early mortality were excluded. The control group extended from January 2012 to January 2014 and the protocol group from July 2014 to July 2016. Variables of interest included time to feed initiation, time to goal feeds, length of stay, and National Surgical Quality Improvement Program-defined complications. We performed a subgroup analysis for primary versus delayed gastroschisis closure. Statistical analyses, including F-tests for variance, were conducted in Prism. RESULTS: Forty-seven patients with simple gastroschisis were included (control = 22, protocol = 25). Protocol compliance was 76% with no increase in complication rates. There was no difference in length of stay or time from initiation to full feeds overall between the control and protocol groups. However, neonates who underwent delayed closure reached full feeds significantly earlier, averaging 9 d versus 15 d previously (P = 0.04). CONCLUSIONS: For infants undergoing delayed closure, the time to full feeds in this group now appears to match that of patients undergoing primary closure, indicating that delayed closure should not be a reason for slower advancement. Additional studies are needed to assess the impact of earlier full enteral nutrition on rare complications and rates of necrotizing enterocolitis.

Guidelines for Opioid Prescribing in Children and Adolescents After Surgery: An Expert Panel Opinion.

Importance: Opioids are frequently prescribed to children and adolescents after surgery. Prescription opioid misuse is associated with high-risk behavior in youth. Evidence-based guidelines for opioid prescribing practices in children are lacking. Objective: To assemble a multidisciplinary team of health care experts and leaders in opioid stewardship, review current literature regarding opioid use and risks unique to pediatric populations, and develop a broad framework for evidence-based opioid prescribing guidelines for children who require surgery. Evidence Review: Reviews of relevant literature were performed including all English-language articles published from January 1, 1988, to February 28, 2019, found via searches of the PubMed (MEDLINE), CINAHL, Embase, and Cochrane databases. Pediatric was defined as children younger than 18 years. Animal and experimental studies, case reports, review articles, and editorials were excluded. Selected articles were graded using tools from the Oxford Centre for Evidence-based Medicine 2011 levels of evidence. The Appraisal of Guidelines for Research & Evaluation (AGREE) II instrument was applied throughout guideline creation. Consensus was determined using a modified Delphi technique. Findings: Overall, 14 574 articles were screened for inclusion, with 217 unique articles included for qualitative synthesis. Twenty guideline statements were generated from a 2-day in-person meeting and subsequently reviewed, edited, and endorsed externally by pediatric surgical specialists, the American Pediatric Surgery Association Board of Governors, the American Academy of Pediatrics Section on Surgery Executive Committee, and the American College of Surgeons Board of Regents. Review of the literature and guideline statements underscored 3 primary themes: (1) health care professionals caring for children who require surgery must recognize the risks of opioid misuse associated with prescription opioids, (2) nonopioid analgesic use should be optimized in the perioperative period, and (3) patient and family education regarding perioperative pain management and safe opioid use practices must occur both before and after surgery. Conclusions and Relevance: These are the first opioid-prescribing guidelines to address the unique needs of children who require surgery. Health care professionals caring for children and adolescents in the perioperative period should optimize pain management and minimize risks associated with opioid use by engaging patients and families in opioid stewardship efforts.

Likely damaging de novo variants in congenital diaphragmatic hernia patients are associated with worse clinical outcomes.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant mortality and long-term morbidity in some but not all individuals. We hypothesize monogenic factors that cause CDH are likely to have pleiotropic effects and be associated with worse clinical outcomes. METHODS: We enrolled and prospectively followed 647 newborns with CDH and performed genomic sequencing on 462 trios to identify de novo variants. We grouped cases into those with and without likely damaging (LD) variants and systematically assessed CDH clinical outcomes between the genetic groups. RESULTS: Complex cases with additional congenital anomalies had higher mortality than isolated cases (P = 8 × 10-6). Isolated cases with LD variants had similar mortality to complex cases and much higher mortality than isolated cases without LD (P = 3 × 10-3). The trend was similar with pulmonary hypertension at 1 month. Cases with LD variants had an estimated 12-17 points lower scores on neurodevelopmental assessments at 2 years compared with cases without LD variants, and this difference is similar in isolated and complex cases. CONCLUSION: We found that the LD genetic variants are associated with higher mortality, worse pulmonary hypertension, and worse neurodevelopment outcomes compared with non-LD variants. Our results have important implications for prognosis, potential intervention and long-term follow up for children with CDH.

Significant practice variability exists in the prevention of venous thromboembolism in injured children: results from a joint survey of the Pediatric Trauma Society and the Trauma Center Association of America.

BACKGROUND/PURPOSE: The purpose of this study was to characterize current practices to prevent venous thromboembolism (VTE) in children and measure adherence to recent joint consensus guidelines from the Pediatric Trauma Society and Eastern Association for the Surgery of Trauma (PTS/EAST). METHODS: An 18-question survey was sent to the membership of PTS and the Trauma Center Association of American. Responses were compared with Chi-square test. RESULTS: One hundred twenty-nine members completed the survey. Most respondents were from academic (84.5%), Level 1 pediatric (62.0%) trauma centers. Criteria for VTE prophylaxis varied between hospitals with freestanding pediatric trauma centers significantly more likely to stratify children by risk factors than adult trauma centers (p = 0.020). While awareness of PTS/EAST guidelines (58.7% overall) was not statistically different between hospital types (44% freestanding adult, 52% freestanding pediatric, 71% combined adult pediatric, p = 0.131), self-reported adherence to these guidelines was uniformly low at 37.2% for all respondents. Lastly, in three clinical scenarios, respondents chose VTE screening and prophylaxis plans in accordance with a prospective application of PTS/EAST guidelines 55.0% correctly. CONCLUSION: Currently no consensus regarding the prevention of VTE in pediatric trauma exists. Prospective application of PTS/EAST guidelines has been limited, likely due to poor quality of evidence and a reliance on post-injury metrics. Results of this survey suggest that further investigation is needed to more clearly define the risk of VTE in children, evaluate, and prospectively validate alternative scoring systems for VTE prevention in injured children. LEVEL OF EVIDENCE: N/A-Survey.

Establishing best practices for structured NSQIP review.

INTRODUCTION: We describe an institutional program (INR- Interval NSQIP Review), to augment NSQIP utility through structured, multidisciplinary review of surgical outcomes in order to create near 'real-time' adverse event (AE) monitoring and improve surgeon awareness. METHODS: INR is a monthly meeting of quality analysts, surgeons and nursing leadership initiated to validate AE with NSQIP criteria, review data in real-time, and perform in-depth case analyses. Occurrence classification concerns were referred for national NSQIP review. Monthly reports were distributed to surgeons with AE rates and case-specific details. RESULTS: Since implementation, 377/3,026 AE underwent in-depth review. Of those, 7 occurrences were referred for clarification by central NSQIP review. Overall 37 (1.2%) were not consistent with NSQIP-defined AE after INR. Time from occurrence to surgeon review decreased by 223 days (296 vs. 73 days, p = 0.006). DISCUSSION: Structured monthly institutional review of AE prior to submission can create greater transparency and confidence of NSQIP data, reduce time from occurrence to surgeon recognition, and improve stakeholder understanding of AE definitions. This approach can be tailored to institutional needs and should be evaluated for downstream improvement in patient outcomes.

Validation of Shock Index Pediatric-Adjusted for children injured in warzones.

BACKGROUND: Shock Index Pediatric-Adjusted (SIPA) has been used to predict injury severity and outcomes after civilian pediatric trauma. We hypothesize that SIPA can predict the need for blood transfusion and emergent surgery among pediatric patients injured in warzones, where resources are limited and accurate triage is essential. METHODS: Retrospective review of the DoD Trauma Registry for all patients 17 years or younger, from 2008 to 2015. Shock Index Pediatric-Adjusted was determined using vital signs recorded upon arrival to the initial level of care. Patients were classified into two groups (normal vs. elevated SIPA) using age-specific threshold values. The need for blood product transfusion (BPT) within 24 hours and emergent surgical procedures (ESP) was compared between groups. Intensive care unit admission, injury severity, and mortality were also compared. Regression analysis was performed to evaluate the relationship between SIPA and primary outcomes. RESULTS: There were 2,121 patients included with a mean Injury Severity Score of 12 ± 10. The mechanism of injury was penetrating (63%), blunt (25%), and burns (12%). Patients with an elevated SIPA (43%) had a significantly greater need for BPT (49.2% vs. 25.0%) and ESP (22.9% vs. 16.0%), as well as mortality (10.3% vs. 4.8%) and intensive care unit admission (49.9% vs. 36.1%), all p less than 0.001. Regression analysis confirmed an elevated SIPA as independently associated with both BPT (odds ratio, 2.36; 95% confidence interval, 1.19-2.94; p < 0.001) and ESP (odds ratio, 1.29; 95% confidence interval, 1.01-1.64; p = 0.044). CONCLUSION: This is the first study of SIPA in pediatric warzone trauma. Elevated SIPA is associated with significantly increased need for BPT and emergent surgery and may therefore serve as a valuable tool for planning and triage in austere settings. LEVEL OF EVIDENCE: Prognostic/epidemiological, Level III.

Minimizing variance in pediatric surgical care through implementation of a perioperative colon bundle: A multi-institution retrospective cohort study.

BACKGROUND: Employing an institutional initiative to minimize variance in pediatric surgical care, we implemented a set of perioperative bundled interventions for all colorectal procedures to reduce surgical site infections (SSIs). METHODS: Implementation of a standard colon bundle at two children's hospitals began in December 2014. Subjects who underwent a colorectal procedure during the study period were analyzed. Demographics, outcomes, and complications were compared with Wilcoxon Rank-Sum, Chi-square and Fisher exact tests, as appropriate. Multivariable logistic regression was performed to assess the influence of time period (independent of protocol implementation) on the rate of subsequent infection. RESULTS: One hundred and forty-five patients were identified (preprotocol=68, postprotocol= 77). Gender, diagnosis, procedure performed and wound classification were similar between groups. Superficial SSIs (21% vs. 8%, p=0.031) and readmission (16% vs. 4%, p=0.021) were significantly decreased following implementation of a colon bundle. Median hospital days, cost, reoperation, intraabdominal abscess, and anastomotic leak were unchanged before and after protocol implementation (all p > 0.05). Multivariable logistic regression found time period to be independent of SSIs (OR: 0.810, 95% CI: 0.576-1.140). CONCLUSION: Implementation of a standard pediatric perioperative colon bundle can reduce superficial SSIs. Larger prospective studies are needed to evaluate the impact of colon bundles in reducing complications, hospital stay and cost. LEVEL OF EVIDENCE: III - Retrospective cohort study.

Comparative outcomes of right versus left congenital diaphragmatic hernia: A multicenter analysis.

BACKGROUND: Congenital diaphragmatic hernia (CDH) occurs in 1 out of 2500-3000 live births. Right-sided CDHs (R-CDHs) comprise 25% of all CDH cases, and data are conflicting on outcomes of these patients. The aim of our study was to compare outcomes in patients with right versus left CDH (L-CDH). METHODS: We analyzed a multicenter prospectively enrolled database to compare baseline characteristics and outcomes of neonates enrolled from January 2005 to January 2019 with R-CDH vs. L-CDH. RESULTS: A total of 588, 495 L-CDH, and 93 R-CDH patients with CDH were analyzed. L-CDHs were more frequently diagnosed prenatally (p=0.011). Lung-to-head ratio was similar in both cohorts. R-CDHs had a lower frequency of primary repair (p=0.022) and a higher frequency of need for oxygen at discharge (p=0.013). However, in a multivariate analysis, need for oxygen at discharge was no longer significantly different. There were no differences in long-term neurodevelopmental outcomes assessed at two year follow up. There was no difference in mortality, need for ECMO, pulmonary hypertension, or hernia recurrence. CONCLUSION: In this large series comparing R to L-CDH patients, we found no significant difference in mortality, use of ECMO, or pulmonary complications. Our study supports prior studies that R-CDHs are relatively larger and more often require a patch or muscle flap for repair. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level II.

Rotational thromboelastometry predicts transfusion and disability in pediatric trauma.

BACKGROUND: Trauma-induced coagulopathy seen on rotational thromboelastometry (ROTEM) is associated with poor outcomes in adults; however, this relationship is poorly understood in the pediatric population. We sought to define thresholds for product-specific transfusion and evaluate the prognostic efficacy of ROTEM in injured children. METHODS: Demographics, ROTEM, and clinical outcomes from severely injured children (age, < 18 years) admitted to a Level I trauma center between 2014 and 2018 were retrospectively analyzed. Receiver operating characteristic curves were plotted and Youden indexes were calculated against the endpoint of packed red blood cell transfusion to identify thresholds for intervention. The ROTEM parameters were compared against the clinical outcomes of mortality or disability at discharge. RESULTS: Ninety subjects were reviewed. Increased tissue factor-triggered extrinsic pathway (EXTEM) clotting time (CT) >84.5 sec (p = 0.049), decreased EXTEM amplitude at 10 minutes (A10) <43.5 mm (p = 0.025), and decreased EXTEM maximal clot firmness (MCF) <64.5 mm (p = 0.026) were associated with need for blood product transfusion. Additionally, EXTEM CT longer than 68.5 seconds was associated with mortality or disability at discharge. CONCLUSION: Coagulation dysregulation on thromboelastometry is associated with disability and mortality in children. Based on our findings, we propose ROTEM thresholds: plasma transfusion for EXTEM CT longer than 84.5 seconds, fibrinogen replacement for EXTEM A10 less than 43.5 mm, and platelet transfusion for EXTEM MCF less than 64.5 mm. LEVEL OF EVIDENCE: Prognostic, Level III; Therapeutic, Level IV.

Conservative management of urachal anomalies.

PURPOSE: The purpose of this study was to evaluate trends in management of urachal anomalies at our institution and the safety of nonoperative care. METHODS: Based on our experience managing urachal remnants from 2000 to 2010 (reported in 2012), we adopted a more conservative approach, including preoperative antibiotic use, refraining from using voiding cystourethrograms (VCUG), postponing surgery until at least six months of age, and considering nonoperative management. A retrospective analysis of urachal anomaly cases was conducted (2011-2016) to assess trends in practice. Charts indicating anomalies of the urachus were pulled and trends in management (nonoperative versus surgical treatment), VCUG and antibiotic use, and outcomes were reviewed. RESULTS: Data from 2000-2010 and 2013-2016 were compared. Our findings indicate care has shifted towards nonoperative management. A smaller proportion of patients from 2013-2016 was treated surgically compared to 2000-2010. Patients receiving nonoperative treatment exhibited lower rates of complication relative to surgically managed cases. VCUGs were eliminated as a diagnostic tool for evaluating urachal anomalies. Prophylactic preoperative antibiotic use was standardized. No patients with a known urachal remnant presented later with an abscess or sepsis. CONCLUSIONS: We find that a shift towards nonoperative treatment of urachal anomalies did not adversely affect overall outcomes. We recommend observing minimally symptomatic patients, especially those under six months old. STUDY TYPE: Performance improvement. LEVEL OF EVIDENCE: Level IV.

De novo variants in congenital diaphragmatic hernia identify MYRF as a new syndrome and reveal genetic overlaps with other developmental disorders.

Congenital diaphragmatic hernia (CDH) is a severe birth defect that is often accompanied by other congenital anomalies. Previous exome sequencing studies for CDH have supported a role of de novo damaging variants but did not identify any recurrently mutated genes. To investigate further the genetics of CDH, we analyzed de novo coding variants in 362 proband-parent trios including 271 new trios reported in this study. We identified four unrelated individuals with damaging de novo variants in MYRF (P = 5.3x10(-8)), including one likely gene-disrupting (LGD) and three deleterious missense (D-mis) variants. Eight additional individuals with de novo LGD or missense variants were identified from our other genetic studies or from the literature. Common phenotypes of MYRF de novo variant carriers include CDH, congenital heart disease and genitourinary abnormalities, suggesting that it represents a novel syndrome. MYRF is a membrane associated transcriptional factor highly expressed in developing diaphragm and is depleted of LGD variants in the general population. All de novo missense variants aggregated in two functional protein domains. Analyzing the transcriptome of patient-derived diaphragm fibroblast cells suggest that disease associated variants abolish the transcription factor activity. Furthermore, we showed that the remaining genes with damaging variants in CDH significantly overlap with genes implicated in other developmental disorders. Gene expression patterns and patient phenotypes support pleiotropic effects of damaging variants in these genes on CDH and other developmental disorders. Finally, functional enrichment analysis implicates the disruption of regulation of gene expression, kinase activities, intra-cellular signaling, and cytoskeleton organization as pathogenic mechanisms in CDH.

Analysis of Gender-based Differences in Surgery Faculty Compensation, Promotion, and Retention: Establishing Equity.

OBJECTIVES: The objectives of this study were to evaluate gender-based differences in faculty salaries before and after implementation of a university-wide objective compensation plan, Faculty First (FF), in alignment with Association of American Medical Colleges regional median salary (AAMC-WRMS). Gender-based differences in promotion and retention were also assessed. SUMMARY BACKGROUND DATA: Previous studies demonstrate that female faculty within surgery are compensated less than male counterparts are and have decreased representation in higher academic ranks and leadership positions. METHODS: At a single institution, surgery faculty salaries and work relative value units (wRVUs) were reviewed from 2009 to 2017, and time to promotion and retention were reviewed from 1998 to 2007. In 2015, FF supplanted specialty-specific compensation plans. Salaries and wRVUs relative to AAMC-WRMS, time to promotion, and retention were compared between genders. RESULTS: Female faculty (N = 24) were compensated significantly less than males were (N = 62) before FF (P = 0.004). Female faculty compensation significantly increased after FF (P < 0.001). After FF, female and male faculty compensation was similar (P = 0.32). Average time to promotion for female (N = 29) and male faculty (N = 82) was similar for promotion to associate professor (P = 0.49) and to full professor (P = 0.37). Promotion was associated with significantly higher retention for both genders (P < 0.001). The median time of departure was similar between female and male faculty (P = 0.73). CONCLUSIONS: A university-wide objective compensation plan increased faculty salaries to the AAMC western region median, allowing correction of gender-based salary inequity. Time to promotion and retention was similar between female and male faculty.

Minimizing variance in Care of Pediatric Blunt Solid Organ Injury through Utilization of a hemodynamic-driven protocol: a multi-institution study.

BACKGROUND: An expedited recovery protocol for management of pediatric blunt solid organ injury (spleen, liver, and kidney) was instituted across two Level 1 Trauma Centers, managed by nine pediatric surgeons within three hospital systems. METHODS: Data were collected for 18months on consecutive patients after protocol implementation. Patient demographics (including grade of injury), surgeon compliance, National Surgical Quality Improvement Program (NSQIP) complications, direct hospital cost, length of stay, time in the ICU, phlebotomy, and re-admission were compared to an 18-month control period immediately preceding study initiation. RESULTS: A total of 106 patients were treated (control=55, protocol=51). Demographics were similar among groups, and compliance was 78%. Hospital stay (4.6 vs. 3.5days, p=0.04), ICU stay (1.9 vs. 1.0days, p=0.02), and total phlebotomy (7.7 vs. 5.3 draws, p=0.007) were significantly less in the protocol group. A decrease in direct hospital costs was also observed ($11,965 vs. $8795, p=0.09). Complication rates (1.8% vs. 3.9%, p=0.86, no deaths) were similar. CONCLUSIONS: An expedited, hemodynamic-driven, pediatric solid organ injury protocol is achievable across hospital systems and surgeons. Through implementation we maintained quality while impacting length of stay, ICU utilization, phlebotomy, and cost. Future protocols should work to further limit resource utilization. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: Level II.

Mutations in BMPR2 are not present in patients with pulmonary hypertension associated with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a prevalent major congenital anomaly with significant morbidity and mortality. Thirty to 40% mortality in CDH is largely attributed to pulmonary hypoplasia and pulmonary hypertension (PH). We hypothesized that the underlying genetic risk factors for hereditary PH are shared with CDH associated PH. METHODS: Participants were recruited as part of the Diaphragmatic Hernia Research & Exploration; Advancing Molecular Science (DHREAMS) study, a prospective cohort of neonates with a diaphragmatic defect enrolled from 2005 to 2012. PH affected patients with available DNA for sequencing had one of the following: moderate or severe PH on echocardiography at 3months of age; moderate of severe PH at 1month of age with death occurring prior to the 3month echocardiogram; or on PH medications at 1month of age. We sequenced the coding regions of the hereditary PH genes bone morphogenetic protein receptor type II (BMPR2), caveolin 1 (CAV1) and potassium channel subfamily K, member 3 (KCNK3) to screen for mutations. RESULTS: There were 29 CDH patients with PH including 16 males and 13 females. Sequencing of BMPR2, CAV1, and KCNK3 coding regions did not identify any pathogenic variants in these genes. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level IV.