The role of fetal hemoglobin in the artificial placenta: A premature ovine model.

INTRODUCTION: A radical paradigm shift in the treatment of premature infants failing conventional treatment is to recreate fetal physiology using an extracorporeal Artificial Placenta (AP). The aim of this study is to evaluate the effects of changing fetal hemoglobin percent (HbF%) on physiology and circuit function during AP support in an ovine model. METHODS: Extremely premature lambs (n = 5) were delivered by cesarean section at 117-121 d estimated gestational age (EGA) (term = 145d), weighing 2.5 ± 0.35 kg. Lambs were cannulated using 10-14Fr cannulae for drainage via the right jugular vein and reinfusion via the umbilical vein. Lambs were intubated and lungs were filled with perfluorodecalin to a meniscus with a pressure of 5-8 cm H2O. The first option for transfusion was fetal whole blood from twins followed by maternal red blood cells. Arterial blood gases were used to titrate AP support to maintain fetal blood gas values. RESULTS: The mean survival time on circuit was 119.6 ± 39.5 h. Hemodynamic parameters and lactate were stable throughout. As more adult blood transfusions were given to maintain hemoglobin at 10 mg/dL, the HbF% declined, reaching 40% by post operative day 7. The HbF% was inversely proportional to flow rates as higher flows were required to maintain adequate oxygen saturation and perfusion. CONCLUSIONS: Transfusion of adult blood led to decreased fetal hemoglobin concentration during AP support. The HbF% was inversely proportional to flow rates. Future directions include strategies to decrease the priming volume and establishing a fetal blood bank to have blood rich in HbF.

Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years.

OBJECTIVE: To determine if risk-adjusted survival of patients with CDH has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA: The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS: We divided registry data into 5-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS: 5203 patients from 23 centers with >22years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all P > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all P < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) ( P = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; P = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS: Risk-adjusted and observed-to-expected CDH mortality have improved over time.

Contrast Challenge Algorithms for Adhesive Small Bowel Obstructions Are Safe in Children: A Multi-Institutional Study.

OBJECTIVE: The purpose of this study was to evaluate the safety of a water-soluble contrast challenge as part of a nonoperative management algorithm in children with an adhesive small bowel obstruction (ASBO). BACKGROUND: Predicting which children will successfully resolve their ASBO with non-operative management at the time of admission remains difficult. Additionally, the safety of a water-soluble contrast challenge for children with ASBO has not been established in the literature. METHODS: A retrospective review was performed of patients who underwent non-operative management for an ASBO and received a contrast challenge across 5 children's hospitals between 2012 and 2020. Safety was assessed by comparing the complication rate associated with a contrast challenge against a pre-specified maximum acceptable level of 5%. Sensitivity, specificity, negative (NPV) and positive (PPV) predictive values of a contrast challenge to identify successful nonoperative management were calculated. RESULTS: Of 82 children who received a contrast challenge, 65% were successfully managed nonoperatively. The most common surgical indications were failure of the contrast challenge or failure to progress after initially passing the contrast challenge. There were no complications related to contrast administration (0%; 95% confidence interval: 0-3.6%, P = 0.03). The contrast challenge was highly reliable in determining which patients would require surgery and which could be successfully managed non-operatively (sensitivity 100%, specificity 86%, NPV 100%, PPV 93%). CONCLUSION: A contrast challenge is safe in children with ASBO and has a high predictive value to assist in clinical decision-making.

Neonatal peri-procedural extracorporeal membrane oxygenation in the management of tracheo-esophageal fistula/esophageal atresia and left pulmonary agenesis.

Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk.

Reducing Unnecessary Oophorectomies for Benign Ovarian Neoplasms in Pediatric Patients.

Importance: Although most ovarian masses in children and adolescents are benign, many are managed with oophorectomy, which may be unnecessary and can have lifelong negative effects on health. Objective: To evaluate the ability of a consensus-based preoperative risk stratification algorithm to discriminate between benign and malignant ovarian pathology and decrease unnecessary oophorectomies. Design, Setting, and Participants: Pre/post interventional study of a risk stratification algorithm in patients aged 6 to 21 years undergoing surgery for an ovarian mass in an inpatient setting in 11 children's hospitals in the United States between August 2018 and January 2021, with 1-year follow-up. Intervention: Implementation of a consensus-based, preoperative risk stratification algorithm with 6 months of preintervention assessment, 6 months of intervention adoption, and 18 months of intervention. The intervention adoption cohort was excluded from statistical comparisons. Main Outcomes and Measures: Unnecessary oophorectomies, defined as oophorectomy for a benign ovarian neoplasm based on final pathology or mass resolution. Results: A total of 519 patients with a median age of 15.1 (IQR, 13.0-16.8) years were included in 3 phases: 96 in the preintervention phase (median age, 15.4 [IQR, 13.4-17.2] years; 11.5% non-Hispanic Black; 68.8% non-Hispanic White); 105 in the adoption phase; and 318 in the intervention phase (median age, 15.0 [IQR, 12.9-16.6)] years; 13.8% non-Hispanic Black; 53.5% non-Hispanic White). Benign disease was present in 93 (96.9%) in the preintervention cohort and 298 (93.7%) in the intervention cohort. The percentage of unnecessary oophorectomies decreased from 16.1% (15/93) preintervention to 8.4% (25/298) during the intervention (absolute reduction, 7.7% [95% CI, 0.4%-15.9%]; P = .03). Algorithm test performance for identifying benign lesions in the intervention cohort resulted in a sensitivity of 91.6% (95% CI, 88.5%-94.8%), a specificity of 90.0% (95% CI, 76.9%-100%), a positive predictive value of 99.3% (95% CI, 98.3%-100%), and a negative predictive value of 41.9% (95% CI, 27.1%-56.6%). The proportion of misclassification in the intervention phase (malignant disease treated with ovary-sparing surgery) was 0.7%. Algorithm adherence during the intervention phase was 95.0%, with fidelity of 81.8%. Conclusions and Relevance: Unnecessary oophorectomies decreased with use of a preoperative risk stratification algorithm to identify lesions with a high likelihood of benign pathology that are appropriate for ovary-sparing surgery. Adoption of this algorithm might prevent unnecessary oophorectomy during adolescence and its lifelong consequences. Further studies are needed to determine barriers to algorithm adherence.

Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative.

OBJECTIVE: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. SUMMARY BACKGROUND DATA: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. METHODS: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. RESULTS: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection ( P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. CONCLUSIONS: In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.

Switching to centrifugal pumps may decrease hemolysis rates among pediatric ECMO patients.

Recent advances in ECLS technology have led to the adoption of centrifugal pumps for the majority of patients worldwide. Despite several advantages of centrifugal pumps, they remain controversial because a number of studies have shown increased rates of hemolysis. The aim of this study was to assess the impact of transitioning from roller to centrifugal pumps on hemolysis rates at our center. A retrospective analysis of all pediatric ECMO patients at a single center between 2005 and 2017 was undertaken. Hemolysis was defined as a plasma free hemoglobin >50 mg/dL. Multivariable logistic regression was performed correcting for several factors to determine risk factors for hemolysis and analyze outcomes among patients with hemolysis. Significant findings were those with p < 0.05. A total of 590 patients were identified during the study period. Multivariable logistic regression for risk factors for hemolysis showed roller pumps (OR 1.92, CI 1.11-3.33) and ECMO duration (OR 1.002 per hour, CI 1.00-1.01) to be significant factors. Rates of hemolysis significantly improved following conversion from roller to centrifugal pumps, with significantly lower rates of hemolysis in 2012, 2015, 2016, and 2017 when compared to the historical average with roller pumps from 2005 to 2009 (34.7%). Additionally, hemolysis was associated with an increased risk of death (OR 3.59, CI 2.05-6.29) when correcting for other factors. These data suggest decreasing rates of hemolysis with centrifugal pumps compared to roller pumps. Since hemolysis was also associated with increased risk of death, these data support the switch from roller to centrifugal pumps at ECMO centers.

Pediatric and neonatal extracorporeal life support: current state and continuing evolution.

The use of extracorporeal life support (ECLS) for the pediatric and neonatal population continues to grow. At the same time, there have been dramatic improvements in the technology and safety of ECLS that have broadened the scope of its application. This article will review the evolving landscape of ECLS, including its expanding indications and shrinking contraindications. It will also describe traditional and hybrid cannulation strategies as well as changes in circuit components such as servo regulation, non-thrombogenic surfaces, and paracorporeal lung-assist devices. Finally, it will outline the modern approach to managing a patient on ECLS, including anticoagulation, sedation, rehabilitation, nutrition, and staffing.

Efficacy of Early Pleurectomy for Severe Congenital Chylothorax.

BACKGROUND: Severe congenital chylothorax (SCC) may result in respiratory failure, malnutrition, immunodeficiency, and sepsis. Although typically managed with bowel rest, parenteral nutrition, and octreotide, persistent chylothoraces require surgical management. At our institution, a pleurectomy, unilateral or bilateral, in combination with mechanical pleurodesis and thoracic duct ligation is performed for SCC, and we describe our approach and outcomes. MATERIALS AND METHODS: We reviewed over 15-year period neonatal patients with SCC managed surgically with pleurectomy after medical therapy was unsuccessful. Patients were divided into two groups: those who underwent pleurectomy within 28 d of diagnosis (early group) and those who underwent pleurectomy after 28 d (late group). Resolution of chylothorax was defined by the absence of clinical symptoms as well as absent or minimal pleural effusion on chest X-ray. RESULTS: Of 40 patients diagnosed with SCC over the study period, 15 underwent pleurectomy, eight early [mean time to operation = 20 (IQR 17, 23) d] and 7 late [59 (42, 75) d, P = 0.001]. Overall survival was 67% (10 of 15). Seven of 8 (88%) neonates who underwent early pleurectomy survived versus 3 of 7 (43%) who underwent late pleurectomy (P = 0.07). Length of stay was lower in the early group than the late group [73 (57, 79) versus 102 (109, 213) d, P = 0.05]. All patients who survived to discharge had resolution of their chylothorax. CONCLUSIONS: Pleurectomy with mechanical pleurodesis and thoracic duct ligation is effective in the management of severe congenital chylothorax. When performed earlier, pleurectomy for severe congenital chylothorax may be associated with improved survival and shorter hospital length of stay.

Association of Nonoperative Management Using Antibiotic Therapy vs Laparoscopic Appendectomy With Treatment Success and Disability Days in Children With Uncomplicated Appendicitis.

Importance: Nonoperative management with antibiotics alone has the potential to treat uncomplicated pediatric appendicitis with fewer disability days than surgery. Objective: To determine the success rate of nonoperative management and compare differences in treatment-related disability, satisfaction, health-related quality of life, and complications between nonoperative management and surgery in children with uncomplicated appendicitis. Design, Setting, and Participants: Multi-institutional nonrandomized controlled intervention study of 1068 children aged 7 through 17 years with uncomplicated appendicitis treated at 10 tertiary children's hospitals across 7 US states between May 2015 and October 2018 with 1-year follow-up through October 2019. Of the 1209 eligible patients approached, 1068 enrolled in the study. Interventions: Patient and family selection of nonoperative management with antibiotics alone (nonoperative group, n = 370) or urgent (≤12 hours of admission) laparoscopic appendectomy (surgery group, n = 698). Main Outcomes and Measures: The 2 primary outcomes assessed at 1 year were disability days, defined as the total number of days the child was not able to participate in all of his/her normal activities secondary to appendicitis-related care (expected difference, 5 days), and success rate of nonoperative management, defined as the proportion of patients initially managed nonoperatively who did not undergo appendectomy by 1 year (lowest acceptable success rate, ≥70%). Inverse probability of treatment weighting (IPTW) was used to adjust for differences between treatment groups for all outcome assessments. Results: Among 1068 patients who were enrolled (median age, 12.4 years; 38% girls), 370 (35%) chose nonoperative management and 698 (65%) chose surgery. A total of 806 (75%) had complete follow-up: 284 (77%) in the nonoperative group; 522 (75%) in the surgery group. Patients in the nonoperative group were more often younger (median age, 12.3 years vs 12.5 years), Black (9.6% vs 4.9%) or other race (14.6% vs 8.7%), had caregivers with a bachelor's degree (29.8% vs 23.5%), and underwent diagnostic ultrasound (79.7% vs 74.5%). After IPTW, the success rate of nonoperative management at 1 year was 67.1% (96% CI, 61.5%-72.31%; P = .86). Nonoperative management was associated with significantly fewer patient disability days at 1 year than did surgery (adjusted mean, 6.6 vs 10.9 days; mean difference, -4.3 days (99% CI, -6.17 to -2.43; P < .001). Of 16 other prespecified secondary end points, 10 showed no significant difference. Conclusion and Relevance: Among children with uncomplicated appendicitis, an initial nonoperative management strategy with antibiotics alone had a success rate of 67.1% and, compared with urgent surgery, was associated with statistically significantly fewer disability days at 1 year. However, there was substantial loss to follow-up, the comparison with the prespecified threshold for an acceptable success rate of nonoperative management was not statistically significant, and the hypothesized difference in disability days was not met. Trial Registration: ClinicalTrials.gov Identifier: NCT02271932.

Aggressive Surgical Management of Congenital Diaphragmatic Hernia: Worth the Effort?: A Multicenter, Prospective, Cohort Study.

OBJECTIVE: The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. BACKGROUND: Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. METHODS: Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. RESULTS: A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P < 0.05) and increased incidence of anomalies (P < 0.0001). Low-volume centers ("Lo", n=44 total, < 10 CDH pts/yr) and high-volume centers ("Hi", n = 21) had median nonrepair rates of 19.8% (range 0%-66.7%) and 16.7% (5.1%-38.5%), respectively. High-volume centers were further dichotomized by rate of nonrepair (HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P < 0.0001). For every 100 CDH patients, HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. CONCLUSIONS: There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

Comparing outcomes with thoracic epidural and intercostal nerve cryoablation after Nuss procedure.

BACKGROUND: This study aimed to evaluate postoperative outcomes after minimally invasive repair of pectus excavatum (Nuss procedure) using video-assisted intercostal nerve cryoablation (INC) compared to thoracic epidural (TE). MATERIALS AND METHODS: We performed a single center retrospective review of pediatric patients who underwent Nuss procedure with INC (n = 19) or TE (n = 13) from April 2015 to August 2017. Preoperative, intraoperative, and postoperative characteristics were collected. The primary outcome was length of stay (LOS) and secondary outcomes were intravenous and oral opioid use, pain scores, and complications. Opioids were converted to oral morphine milligram equivalents per kilogram (oral morphine equivalent [OME]/kg). Mann-Whitney U test was used for continuous and chi-squared analysis for categorical variables. RESULTS: There were no significant differences in patient characteristics, except Haller Index (INC: median [interquartile range] 4.3 [3.6-4.9]; TE: 3.2 [2.8-4.0]; P = 0.03). LOS was shorter with INC (INC: 3 [3-4] days; TE: 6 [5-7] days; P < 0.001). Opioid use was higher intraoperatively (INC: 1.08 [0.87-1.37] OME/kg; TE: 0.46 [0.37-0.67] OME/kg; P = 0.002) and unchanged postoperatively (INC: 1.78 [1.26-3.77] OME/kg; TE: 1.82 [1.05-3.37] OME/kg; P = 0.80), and prescription doses were lower at discharge in INC (INC: 30 [30-40] doses; TE: 42 [40-60] doses; P = 0.005). There was no significant difference in postoperative complications (INC: 42.1%; TE: 53.9%; P = 0.51). CONCLUSIONS: INC during Nuss procedure reduced LOS, shifting postoperative opioid use earlier during admission. This may reflect the need for improved early pain control until INC takes effect. Prospective evaluation after INC is needed to characterize long-term pain medication requirements.

Clinic-day surgery for children: a patient and staff perspective.

INTRODUCTION: For the past 3 years, our institution has implemented a same clinic-day surgery (CDS) program, where common surgical procedures are performed the same day as the initial clinic evaluation. We sought to evaluate the patient and faculty/staff satisfaction following the implementation of this program. METHODS: After IRB approval, patients presenting for the CDS between 2014 and 2017 were retrospectively reviewed. Of these, patient families who received CDS were contacted to perform a telephone survey focusing on their overall satisfaction and to obtain feedback. In addition, feedback from faculty/staff members directly involved in the program was obtained to determine barriers and satisfaction with the program. RESULTS: Twenty-nine patients received CDS, with the most commonly performed procedures being inguinal hernia repair (34%) and umbilical hernia repair (24%). Twenty (69%) patients agreed to perform the telephone survey. Parents were overall satisfied with the CDS program, agreeing that the instructions were easy to understand. Overall, 79% of parents indicated that it decreased overall stress/anxiety, with 75% saying it allowed for less time away from work, and 95% agreeing to pursue CDS again if offered. The most common negative feedback was an unspecified operative start time (15%). While faculty/staff members agreed the program was patient-centered, there were concerns over low enrollment and surgeon continuity, because there were different evaluating and operating surgeons. CONCLUSION: This study successfully evaluated the satisfaction of patients and faculty/staff members after implementing a clinic-day surgery program. Our results demonstrated improved patient family satisfaction, with families reporting decreased anxiety and less time away from work. Despite this, faculty and staff members reported challenges with enrollment and surgeon continuity.

Effect of Perflubron-induced lung growth on pulmonary vascular remodeling in congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) involves lung hypoplasia and pulmonary hypertension (PH). Post-natal Perflubron ventilation induces lung growth. This phenomenon is called Perflubon-induced lung growth (PILG). However, it does not appear to ameliorate PH in CDH. We aim to determine the effect of PILG on pulmonary vascular remodeling in neonates with CDH and PH requiring extracorporeal membrane oxygenation (ECMO). METHODS: Lung tissue from four patients was obtained, three treated with PILG + ECMO, and one maintained on conventional ventilation + ECMO (control). The distribution of collagen was assessed with Masson's trichrome stain. Immunohistochemistry was done to assess cell proliferation and immunofluorescence to assess vascular morphology. RESULTS: Comparing PILG vs. control, there was an increase in vessel wall diameter (6.85 µm, 10.28 µm, and 10.35 µm vs. 4.34 µm), increase in collagen thickness in two PILG patients (35.66 µm, 14.23 µm, and 38.46 µm vs. 22.16 µm), and decrease in lumen diameter despite similar total area (48.99 µm, 41.74 µm, and 36.32 µm vs. 51.56 µm) for each PILG patient vs. the control patient, respectively. CONCLUSION: PILG does not appear to improve pulmonary vascular remodeling that occurs with PH. The findings are descriptive and will require larger samples to validate the significance of the findings. Overall, further studies will be required to identify the mechanistic causes of PH in CDH to create effective treatments.

Sledding injuries a practice-based study is it time to raise awareness?

BACKGROUND: The purpose of this review is to describe our practice-based incidence of sledding injuries in children. METHODS: An 8-year (2003-2011) retrospective review of all hospitalized children (<18 years) from a level one pediatric trauma center due to sledding injuries was performed. Demographic, injury severity score (ISS), hospital stay, ICU stay, and discharge status were analyzed and compared to all other trauma hospitalizations. RESULTS: Fifty-two children were hospitalized from sledding injuries. There were 34 males and 18 females with an average age of 10.1 ± 3.7 years. Impact with a tree was the most common mechanism of injury in 33/52 (63.5 %). Strikingly 20 (37 %) patients suffered a head injury with average ISS scores of 13.21 ± 2.30 and 70 % of them were admitted to the ICU. Three children had permanent disability including cognitive impairment and two others required long-term hospitalization rehabilitation. Other injuries included fractures (17), solid organs (10), chest trauma (1), and vertebral fractures (3). CONCLUSIONS: Sledding was a significant component of hospitalized children during winter months. 30 % suffered significant head inquires and nearly 10 % had permanent disabilities. Injury control strategies ensuring safe environments away from trees, and head protection should be publicized.

Anti-TNF-α treatment for post-anastomotic ulcers and inflammatory bowel disease with Crohn's-like pathologic changes following intestinal surgery in pediatric patients.

PURPOSE: To report a previously poorly recognized process of secondary formation of inflammatory bowel disease (IBD)-like process, specifically Crohn's-like changes in pediatric surgery patients who underwent major small bowel and colorectal surgery. We describe potential etiologies, presenting symptoms and treatment approaches. METHODS: Retrospective chart review of patients with history of either chronic, partial gastrointestinal (GI) obstruction or Hirschsprung disease (HD) and subsequent histopathologic findings similar to IBD. Pathology and case histories were reviewed and treatments were compared. RESULTS: Over the last 20 years, a total of nine patients were identified that had the diagnoses of either HD (n = 3) or chronic GI partial obstruction (n = 6) with subsequent development of histopathologic changes similar to those seen in IBD. Overall meantime to diagnosis of IBD-like changes after intestinal resection was 7.70 ± 5.6 years. Half of the patients were also being managed for short bowel syndrome (SBS), and associated GI symptoms may have prolonged the time to identifying these IBD-like changes. When SBS patients were excluded, mean time to IBD changes after pull through for HD was 2.4 ± 0.24 years and after chronic GI partial obstruction was 6.3 ± 2.1 years. Two of the nine patients who underwent a resection of this IBD-like lesion developed a recurrence of this lesion. Anti-TNF-α treatment was used in three of the GI partial obstruction cases: two with complete relief and one with partial response that was supplemented with steroids. Two HD patients were treated with anti-TNF-α and both had marked improvement of symptoms. CONCLUSION: We describe IBD-like intestinal changes following intestinal resection in the pediatric age group. We also present the novel finding that these lesions are responsive to anti-IBD treatment, including anti-TNF-α, and recommend it as part of the medical treatment regiment offered for such patients.

The diagnosis of fetal esophageal atresia and its implications on perinatal outcome.

The current diagnostic accuracy and perinatal outcome of fetuses with esophageal atresia (EA) continues to be debated. In this review, we report on our experience at a tertiary care fetal center with the prenatal ultrasound diagnosis of EA. Enrollment criteria included a small/absent stomach bubble with a normal or elevated amniotic fluid index between 2005 and 2013. Perinatal outcomes were analyzed and compared to postnatally diagnosed EA cases. Of the 22 fetuses evaluated, polyhydramnios occurred in 73%. Three (14%) died in utero or shortly after birth, but none had EA. In the presence of an absent/small stomach and polyhydramnios, the positive predictive value for EA was 67%. In fetal EA cases confirmed postnatally (group 1, n = 11), there were no differences in gestational age, birthweight, or mortality when compared to postnatally diagnosed infants (group 2, n = 59). Group 1 was associated with long-gap EA, need for esophageal replacement, and increased hospital length of stay. When taken in context with the current literature, we conclude that ultrasound findings suggestive of EA continue to be associated with a relatively high rate of false positives. However, among postnatally confirmed cases, there is an increased risk for long-gap EA and prolonged hospitalization.

How Many Operative Performance Ratings Does a Pediatric Surgery Fellow Need to Be Deemed Practice Ready?

PURPOSE: Identifying the number of cases required for a fellow to achieve competence has been challenging. Workplace-based assessment (WBA) systems make collecting performance data practical and create the opportunity to translate WBA ratings into probabilistic statements about a fellow's likelihood of performing to a given standard on a subsequent assessment opportunity. METHODS: We compared data from two pediatric surgery training programs that used the performance rating scale from the Society for Improving Medical Professional Learning (SIMPL). We used a Bayesian generalized linear mixed effects model to examine the relationship past and future performance for three procedures: Laparoscopic Inguinal Hernia Repair, Laparoscopic Gastrostomy Tube Placement, and Pyloromyotomy. RESULTS: For site one, 26 faculty assessed 9 fellows on 16 procedures yielding 1094 ratings, of which 778 (71%) earned practice-ready ratings. For site two, 25 faculty rated 3 fellows on 4 unique procedures yielding 234 ratings of which 151 (65%) were deemed practice-ready. We identified similar model-based future performance expectations, with prior practice-ready ratings having a similar average effect across both sites (Site one, B = 0.25; Site two, B = 0.25). Similar prior practice-ready ratings were needed for Laparoscopic G-Tube Placement (Site one = 13; Site two = 14), while greater differences were observed for Laparoscopic Inguinal Hernia Repair (Site one = 10; Site two = 15) and Pyloromyotomy (Site one = 10; Site two = 15). CONCLUSION: Our approach to modeling operative performance data is effective at determining future practice readiness of pediatric surgery fellows across multiple faculty and fellow groups. This method could be used to establish minimum case number requirements. TYPE OF STUDY: Original manuscript, Study of Diagnostic Test. LEVEL OF EVIDENCE: II.

A Pumpless Pediatric Artificial Lung Maintains Function for 72 h Without Systemic Anticoagulation Using the Nitric Oxide Surface Anticoagulation System.

BACKGROUND: Children with end-stage lung disease are commonly managed with extracorporeal life support (ECLS) as a bridge to lung transplantation. A pumpless artificial lung (MLung) is a portable alternative to ECLS and it allows for ambulation. Both ECLS and pumpless artificial lungs require systemic anticoagulation which is associated with hemorrhagic complications. We tested the MLung with a novel Nitric Oxide (NO) Surface Anticoagulation (NOSA) system, to provide local anticoagulation for 72 h of support in a pediatric-size ovine model. METHODS: Four mini sheep underwent thoracotomy and cannulation of the pulmonary artery (inflow) and left atrium (outflow), recovered and were monitored for 72hr. The circuit tubing and connectors were coated with the combination of an NO donor (diazeniumdiolated dibutylhexanediamine; DBHD-N2O2) and argatroban. The animals were connected to the MLung and 100 ppm of NO was added to the sweep gas. Systemic hemodynamics, blood chemistry, blood gases, and methemoglobin were collected. RESULTS: Mean device flow was 836 ± 121 mL/min. Device outlet saturation was 97 ± 4%. Pressure drop across the lung was 3.5 ± 1.5 mmHg and resistance was 4.3 ± 1.7 mmHg/L/min. Activated clotting time averaged 170 ± 45s. Methemoglobin was 2.9 ± 0.8%. Platelets declined from 590 ± 101 at baseline to 160 ± 90 at 72 h. NO flux (x10-10 mol/min/cm2) of the NOSA circuit averaged 2.8 ± 0.6 (before study) and 1.9 ± 0.1 (72 h) and across the MLung 18 ± 3 NO flux was delivered. CONCLUSION: The MLung is a more portable form of ECLS that demonstrates effective gas exchange for 72 h without hemodynamic changes. Additionally, the NOSA system successfully maintained local anticoagulation without evidence of systemic effects.

Defining Practice Readiness in Pediatric Surgery Fellowship: A Qualitative Analysis.

INTRODUCTION: While competency-based training is at the forefront of educational innovation in General Surgery, Pediatric Surgery training programs should not wait for downstream changes. There is currently no consensus on what it means for a pediatric surgery fellow to be "practice-ready". In this study, we aimed to provide a framework for better defining competency and practice readiness in a way that can support the Milestones system and allow for improved assessment of pediatric surgery fellows. METHODS: For this exploratory qualitative study, we developed an interview guide with nine questions focused on how faculty recognize competency and advance autonomy among pediatric surgery fellows. Demographic information was collected using an anonymous online survey platform. We iteratively reviewed data from each interview to ensure adequate information power was achieved to answer the research question. We used inductive reasoning and thematic analysis to determine appropriate codes. Additionally, the Dreyfus model was used as a framework to guide interpretation and contextualize the responses. Through this method, we generated common themes. RESULTS: A total of 19 pediatric surgeons were interviewed. We identified four major themes from 127 codes that practicing pediatric surgeons associate with practice-readiness of a fellow: skill-based competency, the recognition and benefits of struggle, developing expertise and facilitating autonomy, and difficulties in variability of evaluation. While variability in evaluation is not typically included in the concept of practice readiness, assessment and evaluation were described by study participants as essential aspects of how practicing pediatric surgeons perceive practice readiness and competency in pediatric surgery fellows. Competency was further divided into interpersonal versus technical skills. Sub-themes within struggle included personal and professional struggle, benefits of struggle and how to identify and assist those who are struggling. Autonomy was commonly stated as variable based on the attending. CONCLUSION: Our analysis yielded several themes associated with practice readiness of pediatric surgery fellows. We aim to further refine our list of themes using the Dreyfus Model as our interpretive framework and establish consensus amongst the community of pediatric surgeons in order to define competency and key elements that make a fellow practice-ready. Further work will then focus on establishing assessment metrics and educational interventions directed at achieving such key elements.