Innovative Fetal Therapy for a Giant Congenital Pulmonary Airway Malformation with Hydrops.

INTRODUCTION: Congenital pulmonary airway malformations (CPAMs) complicated by hydrops portend significant morbidity and mortality, with fetal survival estimates less than 10%. CASE PRESENTATION: We report successful use of ultrasound-guided radiofrequency ablation at 21-week gestation in a hydropic fetus with CPAM, with subsequent resolution of hydrops. Thirty-two-week MRI noted persistent mediastinal shift, and US at 36 weeks and 5 days noted polyhydramnios. Maternal gestational hypertension prompted delivery at 37 weeks, with a cesarean section performed after a failed trial of labor. The infant required CPAP at 100% and weaned to 21%. Tachypnea persisted, and chest CT on day of life 2 demonstrated multiple large cysts in the right lower lobe with anterior pneumothorax. On day of life 3, she successfully underwent a thoracoscopic right lower lobectomy. Adhesions to the chest wall and rib abnormalities were noted. She was extubated to CPAP at the conclusion of the procedure. She was able to wean to 21% on POD2 and transitioned to oral feeds. Her chest tube was removed with resultant ex vacuo pneumothorax noted. She remained asymptomatic and was discharged home on room air POD11. Pathology confirmed a type 1 CPAM. CONCLUSION: In utero radiofrequency ablation may be an adjunct to the management of large CPAM.

An arrow that missed the mark: a pediatric case report of remarkable neurologic improvement following penetrating spinal cord injury.

Penetrating spinal cord injuries are rare in children but result in devastating impacts on long-term morbidity and mortality-with little known about the recovery capacity in this age group. We present the case of an eight-year-old child who sustained a penetrating injury through the right anterior thorax. Thoracic CT showed the arrow tip extending through the spinal canal at T6. Neurologic examination revealed no motor or sensory function below T6. The arrow was surgically removed without complications through an anterior-only approach. MRI on post-operative day (POD) 4 showed focal T2 hyperintensity at the T6 spinal cord. Patient was discharged on POD33 with an American Spinal Injury Association (ASIA)-D score and trace voluntary control over bowel and bladder function. Remarkably, four months later, he had near normal bowel and bladder function, with near-intact lower extremity strength and self-sustained ambulation. Follow-up imaging revealed hemicord formation at the level of injury. We review our case of penetrating spinal cord injury in a child and similar reports in the literature. Penetrating thoracic spinal cord trauma portends poor clinical outcomes, particularly when employing available adult prognostic spinal cord injury scoring metrics. Incomplete spinal cord injury, and often-associated spinal shock, can mimic a complete injury-as in our patient, which improved to near-complete motor and sensory restoration of function and resulted in the formation of a split hemicord. This case represents a unique penetrating spinal cord injury with remarkable neurologic recovery, which would advocate against definitive early prognostication in the pediatric population.

Imaging pediatric acute appendicitis during the coronavirus disease 2019 (COVID-19) pandemic: collateral damage is variable.

BACKGROUND: Since coronavirus disease 2019 (COVID-19) was declared a worldwide pandemic in March 2020, many authors have noted the collateral damage on non-COVID-19-related illnesses. These indirect effects of the pandemic have resulted in people presenting later and with more severe stages of disease, even if their diagnoses are not directly related to SARS-CoV-2, the virus that causes COVID-19. OBJECTIVE: We studied these indirect effects of COVID-19 on the imaging workup and outcomes for pediatric patients at our center who had acute appendicitis during the pandemic. MATERIALS AND METHODS: We performed a retrospective review of cases in children ≤18 years who were evaluated for acute appendicitis during the same period, March 1 to May 31, in both 2019 and 2020. We compared demographic and clinical data as well as surgical and pathological findings, and we graded imaging findings according to severity. Differences in patient outcomes were assessed using the Wilcoxon rank sum test and the Pearson chi-square test. RESULTS: The total number of pediatric patients evaluated with imaging for acute appendicitis dropped by 43% between 2019 and 2020 (298 vs. 169), but the total number of children treated remained similar (59 vs. 51). There was proportionate use of US and CT in each timeframe but a higher percentage of positive imaging findings in 2020 (50/169, 29.6% vs. 56/298, 18.7% in 2019, P=0.04). There were more imaging examinations with features of complicated appendicitis among positive cases (9/51, 18% vs. 5/59, 8% in 2019, P=0.08) and more pathologically proven perforated cases during the pandemic (14/51, 27% vs. 6/59, 10% in 2019, P=0.11), although these results did not reach statistical significance. There were no changes in surgical management, vital signs, laboratory values, length of stay or complication rates. CONCLUSION: There was a large drop in the number of pediatric patients imaged for acute appendicitis during the acute phase of the COVID-19 pandemic despite similar numbers of patients treated. The utilization trends of US vs. CT remained stable between time periods. The differences in imaging findings and perforation rates were less pronounced compared to other published studies.

Sequential Minimally Invasive Fetal Interventions for Two Life-Threatening Conditions: A Novel Approach.

INTRODUCTION: In utero interventions are performed in fetuses with "isolated" major congenital anomalies to improve neonatal outcomes and quality of life. Sequential in utero interventions to treat 2 anomalies in 1 fetus have not yet been described. CASE PRESENTATION: Here, we report a fetus with a large left-sided intralobar bronchopulmonary sequestration (BPS) causing mediastinal shift, a small extralobar BPS, and concomitant severe left-sided congenital diaphragmatic hernia (CDH). At 26-week gestation, the BPS was noted to be increasing in size with a significant reduction in right lung volume and progression to fetal hydrops. The fetus underwent ultrasound-guided ablation of the BPS feeding vessel leading to complete tumor regression. However, lung development remained poor (O/E-LHR: 0.22) due to the left-sided CDH, prompting fetal endoscopic tracheal occlusion therapy at 28-week gestation to allow increased lung growth. After vaginal delivery, the newborn underwent diaphragmatic repair with resection of the extralobar sequestration. He was discharged home with tracheostomy on room air at 9 months. DISCUSSION/CONCLUSION: Sequential in utero interventions to treat 2 severe major anomalies in the same fetus have not been previously described. This approach may be a useful alternative in select cases with otherwise high morbidity/mortality. Further studies are required to confirm our hypothesis.

Comparison of the Pediatric Resuscitation and Trauma Outcome (PRESTO) Model and Pediatric Trauma Scoring Systems in a Middle-Income Country.

BACKGROUND: The pediatric resuscitation and trauma outcome (PRESTO) model was developed to aid comparisons of risk-adjusted mortality after injury in low- and middle-income countries (LMICs). We sought to validate PRESTO using data from a middle-income country (MIC) trauma registry and compare its performance to the Pediatric Trauma Score (PTS), Revised Trauma Score, and pediatric age-adjusted shock index (SIPA). METHODS: We included children (age < 15 years) admitted to a single trauma center in South Africa from December 2012 to January 2019. We excluded patients missing variables necessary for the PRESTO model-age, systolic blood pressure, pulse, oxygen saturation, neurologic status, and airway support. Trauma scores were assigned retrospectively. PRESTO's previously high-income country (HIC)-validated optimal threshold was compared to MIC-validated threshold using area under the receiver operating characteristic curves (AUROC). Prediction of in-hospital death using trauma scoring systems was compared using ROC analysis. RESULTS: Of 1160 injured children, 988 (85%) had complete data for calculation of PRESTO. Median age was 7 (IQR: 4, 11), and 67% were male. Mortality was 2% (n = 23). Mean predicted mortality was 0.5% (range 0-25.7%, AUROC 0.93). Using the HIC-validated threshold, PRESTO had a sensitivity of 26.1% and a specificity of 99.7%. The MIC threshold showed a sensitivity of 82.6% and specificity of 89.4%. The MIC threshold yielded superior discrimination (AUROC 0.86 [CI 0.78, 0.94]) compared to the previously established HIC threshold (0.63 [CI 0.54, 0.72], p < 0.0001). PRESTO showed superior prediction of in-hospital death compared to PTS and SIPA (all p < 0.01). CONCLUSION: PRESTO can be applied in MIC settings and discriminates between children at risk for in-hospital death following trauma. Further research should clarify optimal decision thresholds for quality improvement and benchmarking in LMIC settings.

Surgical Considerations for Pediatric Snake Bites in Low- and Middle-Income Countries.

BACKGROUND: Snake envenomation is associated with major morbidity especially in low- and middle-income countries and may require fasciotomy. We determined patient factors associated with the need for fasciotomy after venomous snake bites in children located in KwaZulu-Natal, South Africa. METHODS: Single institutional review of historical data (2012-2017) for children (<18 years) sustaining snake envenomation was performed. Clinical data, management, and outcomes were abstracted. Syndromes after snake bite were classified according to Blaylock nomenclature: progressive painful swelling (PPS), progressive weakness (PW), or bleeding (B), as it is difficult to reliably identify the species of snake after a bite. Comparative and multivariable analyses to determine factors associated with fasciotomy were performed. RESULTS: There were 72 children; mean age was 7 (±3) years, 59% male. Feet were most commonly affected (n = 27, 38%) followed by legs (n = 18, 25%). Syndromes (according to Blaylock) included PPS (n = 63, 88%), PW (n = 5, 7%), and B (n = 4, 5%). Eighteen patients underwent fasciotomy, and one required above knee amputation. Nine patients received anti-venom. Few patients (15%) received prophylactic beta-lactam antibiotics. Hemoglobin < 11 mg/dL, leukocytosis, INR >1.2, and age-adjusted shock index were associated with fasciotomy. On regression, age-adjusted shock index and hemoglobin concentration < 11 mg/dL, presentation >24 h after snake bite, and INR >1.2 were independently associated with fasciotomy. Model sensitivity was 0.89 and demonstrated good fit. CONCLUSIONS: Patient factors were associated with the fasciotomy. These factors, coupled with clinical examination, may identify those who need early operative intervention. Improving time to treatment and the appropriate administration of anti-venom will minimize the need for surgery. LEVEL OF EVIDENCE: III.

Trauma registry data as a tool for comparison of practice patterns and outcomes between low- and middle-income and high-income healthcare settings.

PURPOSE: There is a lack of data-driven, risk-adjusted mortality estimates for injured children outside of high-income countries (HIC). To inform injury prevention and quality improvement efforts, an upper middle-income country (UMIC) pediatric trauma registry was compared to that of a HIC. METHODS: Clinical data, injury details, and mortality of injured children (< 18 years) hospitalized in two centers (USA and South African (SA)) from 2013 to 2017 were abstracted. Univariate and multivariable analyses evaluated risk of mortality and were expressed as odds ratios (OR) with 95% confidence intervals (CI). RESULTS: Of 2089 patients, SA patients had prolonged transfer times (21.1 vs 3.4 h) and were more likely referred (78.2% vs 53.9%; both p < 0.001). Penetrating injuries were more frequent in SA (23.2% vs 7.4%, p < 0.001); injury severity (9 vs 4) and shock index (0.90 vs 0.80) were greater (both p < 0.001). SA utilized cross-sectional imaging more frequently (66.4% vs 37.3%, p < 0.001). In-hospital mortality was similar (1.9% SA, 1.3% USA, p = 0.31). Upon multivariable analysis, ISS > 25 [210.50 (66.0-671.0)] and penetrating injury [5.5 (1.3-23.3)] were associated with mortality, while institution [1.7 (0.7-4.2)] was not. CONCLUSIONS: Despite transfer time, the centers demonstrated comparable survival rates. Comparison of registry data can alert clinicians to problematic practice patterns, assisting initiatives to improve trauma systems.

Management and outcomes of peripancreatic fluid collections and pseudocysts following non-operative management of pancreatic injuries in children.

BACKGROUND: Peripancreatic fluid collection and pseudocyst development is a common sequela following non-operative management (NOM) of pancreatic injuries in children. Our purpose was to review management strategies and assess outcomes. METHODS: A multicenter, retrospective review was conducted of children treated with NOM following blunt pancreatic injury at 22 pediatric trauma centers between the years 2010 and 2015. Organized fluid collections were called "acute peripancreatic fluid collection" (APFC) if identified < 4 weeks and "pseudocyst" if > 4 weeks following injury. Data analysis included descriptive statistics Wilcoxon rank-sum, Kruskal-Wallis and t tests. RESULTS: One hundred patients with blunt pancreatic injury were identified. Median age was 8.5 years (range 1-16). Forty-two percent of patients (42/100) developed organized fluid collections: APFC 64% (27/42) and pseudocysts 36% (15/42). Median time to identification was 12 days (range 7-42). Most collections (64%, 27/42) were observed and 36% (15/42) underwent drainage: 67% (10/15) percutaneous drain, 7% (1/15) needle aspiration, and 27% (4/15) endoscopic transpapillary stent. A definitive procedure (cystogastrostomy/pancreatectomy) was required in 26% (11/42). Patients with larger collections (≥ 7.1 cm) had longer time to resolution. Comparison of outcomes in patients with observation vs drainage revealed no significant differences in TPN use (79% vs 75%, p = 1.00), hospital length of stay (15 vs 25 median days, p = 0.11), time to tolerate regular diet (12 vs 11 median days, p = 0.47), or need for definitive procedure (failure rate 30% vs 20%, p = 0.75). CONCLUSIONS: Following NOM of blunt pancreatic injuries in children, organized fluid collections commonly develop. If discovered early, most can be observed successfully, and drainage does not appear to improve clinical outcomes. Larger size predicts prolonged recovery. LEVEL OF EVIDENCE: III STUDY TYPE: Case series.

Building a Case for Pediatric Fall Prevention.

Falls are the leading cause of traumatic injury and injury-related emergency department visits in the state of Minnesota for children aged 0-14 years. We hypothesize that few of the Minnesota trauma centers and public health departments responsible for injury prevention (IP) efforts in the community are focusing on fall prevention interventions for children. The purpose of this study was to examine the current state of childhood IP interventions in Minnesota, identify potential partners to collectively address pediatric fall prevention, and utilize survey results to lead future IP efforts. An electronic survey was administered to state/American College of Surgeons verified trauma center and county health department staff in Minnesota. We compared opinions related to leading causes of traumatic injury, current IP efforts, data sources used to prioritize IP efforts, barriers to implementing childhood fall prevention interventions, and partnerships among trauma centers and health departments. Completed surveys were analyzed, with 37 responses from trauma centers and 25 from county health departments. On the basis of opinion, 47% of trauma center staff listed falls as the number one cause of traumatic injury to children compared with 25% of health department staff. Eighteen percent of survey respondents report that they have, or are, providing fall prevention programing. Limited resources were listed as the leading barrier. Significant variation exists regarding opinions related to the leading cause of pediatric traumatic injury. The feedback generated from this survey will be shared with Minnesota stakeholders in an effort to encourage collective action toward fall prevention interventions for Minnesota children.

Minimal cosmetic revision required after minimally invasive pectus repair.

BACKGROUND: Despite surgical correction procedures for pectus deformities, remaining cosmetic asymmetry may have significant psychological effects. We sought to evaluate factors associated with plastic surgery (PS) consultation and procedures for these deformities at an academic institution. METHODS: We reviewed patients aged 0-21 diagnosed with a pectus excavatum or carinatum deformity at our institution between January 2001 and October 2016. Pectus diagnoses were identified by ICD-9/ICD-10 codes and surgical repair by CPT codes; patients receiving PS consultation were identified by clinical note service codes. Student's t tests, Fisher's exact tests, and Chi-squared tests were utilized. RESULTS: 2158 patients were diagnosed with a pectus deformity; 442 (20.4%) underwent surgical correction. 19/442 (4.3%) sought PS consultation, either for pectus excavatum [14/19 (73.7%)], carinatum [4/19 (21.0%)], and both [1/19 (5.3%)], (p = 0.02). Patients seeking PS consultation were more likely to be female (p < 0.01), have scoliosis (p = 0.02), or undergo an open repair (p < 0.01). The need for PS consultation did not correlate with Haller index, p = 0.78. CONCLUSION: PS consultation associated with pectus deformity repair was rare, occurring in < 5% of patients undergoing repair. Patients who consulted PS more commonly included females, patients with scoliosis, and those undergoing open repair. These patients would likely benefit most from multidisciplinary pre-operative discussions regarding repair of the global deformity.

Comparison of diagnostic imaging modalities for the evaluation of pancreatic duct injury in children: a multi-institutional analysis from the Pancreatic Trauma Study Group.

PURPOSE: Determining the integrity of the pancreatic duct is important in high-grade pancreatic trauma to guide decision making for operative vs non-operative management. Computed tomography (CT) is generally an inadequate study for this purpose, and magnetic resonance cholangiopancreatography (MRCP) is sometimes obtained to gain additional information regarding the duct. The purpose of this multi-institutional study was to directly compare the results from CT and MRCP for evaluating pancreatic duct disruption in children with these rare injuries. METHODS: Retrospective study of data obtained from eleven pediatric trauma centers from 2010 to 2015. Children up to age 18 with suspected blunt pancreatic duct injury who had both CT and MRCP within 1 week of injury were included. Imaging findings of both studies were directly compared and analyzed using descriptive statistics, Chi square, Wilcoxon rank-sum, and McNemar's tests. RESULTS: Data were collected for 21 patients (mean age 7.8 years). The duct was visualized more often on MRCP than CT (48 vs 5%, p < 0.05). Duct disruption was confirmed more often on MRCP than CT (24 vs 0%), suspected based on secondary findings equally (38 vs 38%), and more often indeterminate on CT (62 vs 38%). Overall, MRCP was not superior to CT for determining duct integrity (62 vs 38%, p = 0.28). CONCLUSIONS: In children with blunt pancreatic injury, MRCP is more useful than CT for identifying the pancreatic duct but may not be superior for confirmation of duct integrity. Endoscopic retrograde cholangiogram (ERCP) may be necessary to confirm duct disruption when considering pancreatic resection. LEVEL OF EVIDENCE: III.

One-year Results of Minimally Invasive Sutured Fixation of the Slipped Ribs in the Pediatric Population.

PURPOSE: Costal cartilage resection with or without rib resection is the gold standard surgery for slipping rib syndrome. Minimally invasive restoration of normal anatomy via nonabsorbable sutures has been described in the adult population with encouraging results. We sought to assess the efficacy of minimally invasive sutured fixation of the hypermobile rib in the pediatric population. METHODS: A retrospective review was performed at Mayo Clinic involving 31 pediatric patients diagnosed with slipped rib syndrome. Minimally invasive sutured open reduction internal fixation was performed between 2020 and 2022. The standardized Örebro Musculoskeletal Pain Screening Questionnaire was given at dedicated time points pre- and post-operatively to assess efficacy. Significance was determined via the Wilcoxon rank sum test. RESULTS: SRS was diagnosed clinically in 31 patients (24 females, 7 males, 1220 years-old). Symptoms were present for an average of 18.9 months and patients had seen an average of 4.7 specialists. Traumatic causes were identified in three patients and eight patients had hypermobility. At one month follow up, there were no surgical complications and patients reported significantly less pain (p < 0.001). Preoperative analgesics reduced by 80%. Ultimately, seven patients underwent a second operation; three patients underwent a third operation; one patient underwent five total operations. Recurrent pain was reported in six patients. Only nine patients followed-up at 1-year post-operation. CONCLUSION: Pediatric patients with SRS demonstrated an early positive response to suture fixation without costal cartilage excision. Reoperation and recurrent pain, however, remain significant in this population. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Case series with no comparison group.

Prevalence and Outcomes of High versus Low Ratio Plasma to Red Blood Cell Resuscitation in a Multi-Institutional Cohort of Severely Injured Children.

BACKGROUND: The benefit of targeting high ratio fresh frozen plasma (FFP):red blood cell (RBC) transfusion in pediatric trauma resuscitation is unclear as existing studies are limited to patients who retrospectively met criteria for massive transfusion. The purpose of this study is to evaluate the use of high ratio FFP:RBC transfusion and the association with outcomes in children presenting in shock. METHODS: A post-hoc analysis of a 24-institution prospective observational study (4/2018-9/2019) of injured children <18 years with elevated age-adjusted shock index was performed. Patients transfused within 24 hours were stratified into cohorts of low (<1:2) or high (>1:2) ratio FFP:RBC. Nonparametric Kruskal-Wallis and chi-square were used to compare characteristics and mortality. Competing risks analysis was used to compare extended (≥75th percentile) ventilator, intensive care, and hospital days while accounting for early deaths. RESULTS: Of 135 children with median (IQR) age 10 (5,14) years and weight 40 (20,64) kg, 85 (63%) received low ratio transfusion and 50 (37%) high ratio despite similar activation of institutional massive transfusion protocols (MTP; low-38%, high-46%, p = .34). Most patients sustained blunt injuries (70%). Median injury severity score was greater in high ratio patients (low-25, high-33, p = .01); however, hospital mortality was similar (low-24%, high-20%, p = .65) as was the risk of extended ventilator, ICU, and hospital days (all p > .05). CONCLUSION: Despite increased injury severity, patients who received a high ratio of FFP:RBC had comparable rates of mortality. These data suggest high ratio FFP:RBC resuscitation is not associated with worst outcomes in children who present in shock. MTP activation was not associated with receipt of high ratio transfusion, suggesting variability in MTP between centers. LEVEL OF EVIDENCE: Prospective cohort study, Level II.

Optimizing resource utilization: palliative care consultations in critically ill pediatric trauma patients.

Objectives: The American College of Surgeons Trauma Quality Improvement Program (TQIP) and Committee on Trauma released a best practice guideline for palliative care in trauma patients in 2017. Utilization of pediatric palliative care services for pediatric trauma patients has not been studied. We sought to identify patients who received the consultation and develop criteria for patients who would benefit from these resources at our institution. Methods: The institutional pediatric trauma registry was queried to identify all admissions age 0-17 years old to the pediatric intensive care unit (PICU) or trauma ICU (TICU) from 2014 to 2021. Demographic and clinical features were obtained from the registry. Electronic medical records were reviewed to identify and review consultations to the ComPASS team. A clinical practice guideline (CPG) for palliative care consultations was developed based on the TQIP guideline and applied retrospectively to patients admitted 2014-2021. The CPG was then prospectively applied to patients admitted from March through November 2022. Results: A total of 399 patients were admitted to the PICU/TICU. There were 30 (7.5%) deaths, 20 (66.7%) within 24 hours of admission. Palliative care consultations were obtained in 21 (5.3%). Of these, 10 (47.6%) patients were infants/toddlers

The Problematic PET: Using CT Albumin Tracer to Resect Suspicious Lymph Nodes Found on PET Scan in Pediatric Oncology Patients.

BACKGROUND: Management of small lymph nodes or lesions in dense nodal basins found on Positron Emission Tomography (PET) scans can be challenging to identify, access and locate intraoperatively. Herein we describe the first reported case series utilizing pre-operative CT-guided radionuclide-tagged macro-aggregated albumin (TC 99m MAA) for localization and resection of extra-pulmonary PET-avid lymph nodes in pediatric cancer patients. METHODS: Pediatric cancer patients (≤21 years) who underwent pre-operative TC 99m MAA localization of suspicious lymph nodes were identified and retrospectively reviewed. RESULTS: Ten procedures were performed on 10 children at our institution from 2017 to 2021. Median age was 14 [13, 18]; 70% were male. Primary tumor type was variable. Lymph nodes were in various nodal basins including the axilla, groin, neck, popliteal fossa, retroperitoneum, and mediastinum. Three patients underwent resection of both pulmonary and extra-pulmonary lesions during the same procedure. Median node size was 15 mm (range: 10 mm- 23 cm). In 60.0% of patients the localized lymph nodes of concern were non-palpable at the time of operation. In 90% of the patient, biopsy findings changed the course of disease management. CONCLUSION: Pre-operative labeling with TC 99m MAA is a safe and effective technique to facilitate the localization, biopsy, and resection of suspicious lymph nodes found on PET scans in pediatric cancer patients that are located in dense nodal basins. This technique enables accurate resection of small, concerning lymph nodes that might otherwise be difficult to operatively identify and excise; the resultant information can affect the staging and further treatment of these patients. LEVEL OF EVIDENCE: IV.

Plasma thrombin generation kinetics vary by injury pattern and resuscitation characteristics in pediatric and young adult trauma patients.

BACKGROUND: Thrombin generation kinetics are not well studied in children. This study aimed to assess how thrombin generation kinetics vary in pediatric and young adult (YA) trauma patients by clinical characteristics and injury pattern. METHODS: Prospective cohort study where plasma samples were obtained from pediatric (ages 0-17 years) and YA (ages 18-21 years) trauma patients upon emergency department arrival. Thrombin generation (calibrated automated thrombogram [CAT]) was quantified as lag time (LT, minutes), peak height (PH, nM), time to peak (ttPeak, minutes), and endogenous thrombin potential (ETP, nM × minute). Results are expressed as median and quartiles [Q1, Q3] and compared using Wilcoxon rank sum testing with p < 0.05 considered significant. RESULTS: We enrolled 47 pediatric (median age, 15 [14, 17] years, 78% male, 87% blunt, median Injury Severity Score, 12) and 49 YA (median age 20 [18, 21] years, 67% male, 84% blunt, median Injury Severity Score, 12) patients. Pediatric and YA patients had similar rates of operative intervention (51% vs. 57%), transfusion (25% vs. 20%), and traumatic brain injury (TBI) (53% vs. 49%). Pediatric patients who required an operation had accelerated initiation of thrombin generation, with shorter LT than those who did not (2.58 [2.33, 2.67]; 2.92 [2.54, 3.00], p = 0.034). Shorter LT (2.41 [2.22, 2.67]; 2.67 [2.53, 3.00]) and ttPeak (4.50 [4.23, 4.73]; 5.22 [4.69, 5.75], both p < 0.01) were noted in pediatric patients who required transfusion as compared with those who did not. The YA patients requiring transfusion had shorter LT (2.33 [2.19, 2.74]; 2.83 [2.67, 3.27]) and ttPeak (4.48 [4.33, 5.65]; 5.33 [4.85, 6.28] both p < 0.04) than those who were not transfused. Young adults with TBI had greater ETP than those without (1509 [1356, 1671]; 1284 [1154, 1471], p = 0.032). CONCLUSION: Thrombin generation kinetics in pediatric trauma patients prior to intervention vary with need for operation and transfusion, while thrombin generation kinetics in young adult patients are influenced by TBI and need for operation or transfusion. This is a promising tool for assessing coagulopathy in young trauma patients. LEVEL OF EVIDENCE: Prognostic and Epidemiological; Level III.

Risk reduction for small cell cancer of the ovary, hypercalcemic type in prepubertal patient: A clinical and bioethical perspective.

Loss of heterozygosity in the SMARCA4 gene is a hallmark feature of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), an aggressive ovarian cancer occurring in young adults and adolescents with an average age of 23 years and a median survival of less than fifteen months following diagnosis. Patients with germline pathogenic variants of SMARCA4 have a genetic predisposition to developing this aggressive ovarian cancer, a condition called rhabdoid tumor predisposition syndrome type 2 (RTPS2). Given the limited efficacy of surveillance imaging for ovarian neoplasm and the absence of an identified biomarker for the progression of this disease, asymptomatic patients who are found to possess pathogenic variants of the SMARCA4 gene following genetic testing are advised to consider risk-reducing bilateral salpingo-oophorectomy to eliminate the risk of SCCOHT. Given the reproductive impacts of this procedure, bioethical consultation must be considered when counseling patients with RTPS2, particularly for those who have not completed their desired course of family planning. In this report, we describe the bioethical considerations and outcomes for the case of a 6-year-old female with a pathogenic variant of SMARCA4 who underwent risk-reducing bilateral salpingo-oophorectomy (RRBSO). To our knowledge, this is the first time that this procedure has been reported in a prepubertal individual for cancer prevention in a patient with RTPS2.

Impact of institutional prophylaxis guidelines on rates of pediatric venous thromboembolism following trauma-A multicenter study from the pediatric trauma society research committee.

BACKGROUND: A paucity of data exists with regard to the incidence, management, and outcomes of venous thromboembolism (VTE) in injured children. We sought to determine the impact of institutional chemoprophylaxis guidelines on VTE rates in a pediatric trauma population. METHODS: A retrospective review of injured children (≤15 years) admitted between 2009 and 2018 at 10 pediatric trauma centers was performed. Data were gathered from institutional trauma registries and dedicated chart review. The institutions were surveyed as to whether they had chemoprophylaxis guidelines in place for high-risk pediatric trauma patients, and outcomes were compared based on the presence of guidelines using χ 2 analysis ( p < 0.05). RESULTS: There were 45,202 patients evaluated during the study period. Three institutions (28,359 patients, 63%) had established chemoprophylaxis policies during the study period ("Guidelines"); the other seven centers (16,843 patients, 37%) had no such guidelines ("Standard"). There were significantly lower rates of VTE in the Guidelines group, but these patients also had significantly fewer risk factors. Among critically injured children with similar clinical presentations, there was no difference in VTE rate. Specifically within the Guidelines group, 30 children developed VTE. The majority (17/30) were actually not indicated for chemoprophylaxis based on institutional guidelines. Still, despite protocols only one VTE patient in the guidelines group who was indicated for intervention ended up receiving chemoprophylaxis prior to diagnosis. No consistent ultrasound screening protocol was in place at any institution during the study. CONCLUSION: The presence of an institutional policy to guide chemoprophylaxis for injured children is associated with a decreased overall frequency of VTE, but this disappears when controlling for patient factors. However, the overall efficacy is impacted by a combination of deficits in guideline compliance and structure. Further prospective data are needed to help determine the ideal role for chemoprophylaxis and protocols in pediatric trauma. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level IV.

Crystalloid volume is associated with short-term morbidity in children with severe traumatic brain injury: An Eastern Association for the Surgery of Trauma multicenter trial post hoc analysis.

OBJECTIVE: This study examined differences in clinical and resuscitation characteristics between injured children with and without severe traumatic brain injury (sTBI) and aimed to identify resuscitation characteristics associated with improved outcomes following sTBI. METHODS: This is a post hoc analysis of a prospective observational study of injured children younger than 18 years (2018-2019) transported from the scene, with elevated shock index pediatric-adjusted on arrival and head Abbreviated Injury Scale score of ≥3. Timing and volume of resuscitation products were assessed using χ 2t test, Fisher's exact t test, Kruskal-Wallis, and multivariable logistic regression analyses. RESULTS: There were 142 patients with sTBI and 547 with non-sTBI injuries. Severe traumatic brain injury patients had lower initial hemoglobin (11.3 vs. 12.4, p < 0.001), greater initial international normalized ratio (1.4 vs. 1.1, p < 0.001), greater Injury Severity Score (25 vs. 5, p < 0.001), greater rates of ventilator (59% vs. 11%, p < 0.001) and intensive care unit (ICU) requirement (79% vs. 27%, p < 0.001), and more inpatient complications (18% vs. 3.3%, p < 0.001). Severe traumatic brain injury patients received more prehospital crystalloid (25% vs. 15%, p = 0.008), ≥1 crystalloid boluses (52% vs. 24%, p < 0.001), and blood transfusion (44% vs. 12%, p < 0.001) than non-sTBI patients. Among sTBI patients, receipt of ≥1 crystalloid bolus (n = 75) was associated with greater ICU need (92% vs. 64%, p < 0.001), longer median ICU (6 vs. 4 days, p = 0.027) and hospital stay (9 vs. 4 days, p < 0.001), and more in-hospital complications (31% vs. 7.5%, p = 0.003) than those who received <1 bolus (n = 67). These findings persisted after adjustment for Injury Severity Score (odds ratio, 3.4-4.4; all p < 0.010). CONCLUSION: Pediatric trauma patients with sTBI received more crystalloid than those without sTBI despite having a greater international normalized ratio at presentation and more frequently requiring blood products. Excessive crystalloid may be associated with worsened outcomes, including in-hospital mortality, seen among pediatric sTBI patients who received ≥1 crystalloid bolus. Further attention to a crystalloid sparing, early transfusion approach to resuscitation of children with sTBI is needed. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level IV.

Congenital paraesophageal hernia with gastric outlet obstruction in a neonate with Cornelia de Lange Syndrome.

We describe a case of a newborn being treated for encephalopathy and seizures, whose radiographs since the first day of life demonstrate a persistent ovoid lucency over the central lower chest. A CT performed confirmed a type IV hiatal hernia, which is defined as a paraesophageal type hernia containing a portion of the abdominal viscera. This infant's hernia included the distal stomach, pylorus, and proximal duodenum. There was no volvulus or ischemic change at surgery. The patient underwent successful reduction, fundoplication, and gastrostomy placement with hospital discharge after further stabilization of additional medical problems. Genetic testing later confirmed Cornelia de Lange Syndrome Type V, which has been associated with gastrointestinal manifestations and congenital diaphragmatic hernias.