Loma Linda University Children's Hospital

To describe medical management surrounding withdrawal of life-sustaining therapy (WLST) in nine U.S. PICUs. Retrospective, secondary analysis of the "Death One Hour After Terminal Extubation" (DONATE) cohort (2009-2021) assessing usage patterns of: 1) analgesics and sedatives; 2) vasoactive infusions; 3) neuromuscular blockade; and 4) post-extubation respiratory support. Nine U.S. PICUs. Children and adolescents 0-21 years old, who had died after WLST (discontinuation of invasive mechanical ventilation). None. Of 905 patients, 680 (75.1%) died within 1 hour of WLST. Opioids were administered in 721 of 905 patients (79.7%); across sites the range was 68-89% ( p < 0.001). We did not observe a temporal trend. Benzodiazepines were used in 507 of 905 patients (56.0%; site range, 41-66%; p < 0.001), with lower odds of usage per year (odds ratio [OR], 0.95 per year; 95% CI, 0.90-0.99 per year; p = 0.04). Dexmedetomidine was used in 140 of 905 patients (15.5%; sites range, 4-21%; p = 0.002), with greater odds of usage per year (OR, 1.16 per year; 95% CI, 1.05-1.27 per year; p = 0.004). Vasoactive infusions were discontinued in 458 of 520 patients (88.1%) receiving this medication (site range, 59-100%; p < 0.001), with greater odds of discontinuation per year (OR, 1.15 per year; 95% CI, 1.04-1.26 per year; p = 0.007). Neuromuscular blockade was used in 46 of 905 patients (5.1%; sites range, 0-13%; p < 0.001), with greater odds of usage per year (OR, 1.23 per year; 95% CI, 1.08-1.40 per year; p = 0.002). Use of any post-extubation respiratory support occurred in 50 of 905 patients (5.5%), and we did not identify an association with site or year-on-year trend. The 2009-2021 DONATE dataset shows substantial institutional and temporal variability in WLST practices across our nine collaborating PICUs in the United States. Future studies should focus on understanding the drivers of variability to improve the consistency and quality of end-of-life management.

Background: Routine chest X-rays (CXR) are not recommended by the American Academy of Pediatrics in bronchiolitis, yet remain a mainstay in diagnostics. We aimed to understand the impact of obtaining CXRs in patients with critical bronchiolitis, assessing intensive care unit length of stay (ICU-LOS) and intensive care unit level of respiratory support (ICU-LRS). Methods/Design: This single-center retrospective cohort study assessed children less than three years of age admitted to the PICU, pediatric step-down ICU, and pediatric cardiac ICU. Two groups were used for analysis: patients with CXR and no-CXR. The primary outcome was the difference in ICU-LOS and ICU-LRS between the groups. The critical bronchiolitis score (CBS) was used to calculate a predicted ICU-LOS and ICU-LRS. The secondary outcome was the difference between actual and predicted ICU-LOS and ICU-LRS, comparing the groups. Results: Of the 107 patients included, 65 patients (61%) received a CXR. Patients who received a CXR had significantly longer ICU-LOS (p = 0.01) and ICU-LRS (p = 0.02), despite no difference in predicted illness severity (ICU-LOS, p = 0.4; ICU-LRS, p = 0.3). The difference between actual and predicted ICU-LOS was greater in the no-CXR group (–1.4 days) compared to the CXR group (–0.8 days; p = 0.04). A similar trend was observed in ICU-LRS (–0.1 vs. –0.6 days; p = 0.1), though not statistically significant. Conclusions: Routine CXRs are common in critically ill bronchiolitis patients and may be associated with longer ICU-LOS and ICU-LRS, despite similar illness severity.

Background: Enhanced recovery after surgery (ERAS) protocols are standardized procedural guidelines proven to decrease hospital stay and postoperative narcotic use and improve overall patient experience. There is no existing ERAS protocol for open cranial vault remodeling (CVR) in the pediatric population. We believe that CVR is one such surgery meriting an ERAS protocol given its multidisciplinary and longitudinal treatment course. Such protocol is of particular interest in San Bernardino County, where the vast geographic area and wide socioeconomic spectrum result in unique barriers to healthcare accessibility that favor open remodeling over endoscopic approaches.Methods: The primary purpose of an ERAS protocol is to minimize both psychological and physiologic stress associated with a given procedure. This can be targeted at various phases of the patient encounter, including the preoperative, intraoperative, and postoperative stages. Here, we introduce our pilot protocol for ERAS implemented at Loma Linda University Children’s Hospital (LLUCH) starting since 2018.Results: Our CVR process begins at the craniofacial clinic, where interdisciplinary chart and imaging review ensure appropriate patient enrollment and clinical service engagement. Day of surgery checklists ensure standardized preoperative cleansing, fasting, and laboratory criteria. Intraoperative guidelines facilitate interdisciplinary transparency regarding antibiotics, blood transfusions, fluid status, pain control, and tranexamic acid and mannitol administration. Standardized postoperative criteria dictate progression through the intensive care unit through to discharge.Conclusion: Retrospective chart review will compare LLUCH outcomes to those reflected in the National Surgical Quality Improvement Program. We hope to demonstrate that the CVR process at LLUCH is comparable to the national average and that implementation of a standardized ERAS protocol can significantly improve clinical and social outcomes.

ABSTRACTHyaline fibromatosis syndrome (or HFS, subcategorized as infantile systemic hyalinosis and juvenile hyaline fibromatosis) has presenting clinical features that may overlap with findings typically identified in cases concerning for nonaccidental trauma. For example, hyperpigmentation over bony prominences (an early presenting finding seen in the most severe cases of HFS) can raise concern for bruising in young infants.In the following case report, we describe a 13‐week‐old female who initially presented as a hospital consult to our forensic child abuse team with concerns for bruising and re‐presented to our team as an emergency department consult at 10 months of age with anal lesions, raising concerns for sexual abuse.Both findings are known clinical features of HFS, which was subsequently diagnosed in this patient via genetic testing. While HFS is a rare syndrome, paediatricians should be aware of the symptoms and clinical features to consider when presented with a case that may be mistaken for abuse.

We describe a case of novel use of trametinib in treating arrythmia without concomitant cardiomyopathy. Our patient is a two-year-old female born with Costello syndrome due to heterozygous mutations in the HRAS gene c34 G > T p (G12C). Shortly after birth, she was diagnosed with multifocal atrial tachyarrhythmia. Her imaging studies have shown no hypertrophy or CHD. There was poor arrhythmia control despite triple antiarrhythmic therapy. Trametinib, a MEK1 and MEK2 inhibitor, was used in treating her isolated atrial arrythmia, allowing her to wean off other antiarrhythmics. Other case reports have shown trametinib to benefit certain RASopathy patients with lymphatic abnormalities, hypertrophic cardiomyopathy, and concurrent arrhythmias. This case demonstrates effective treatment of isolated arrhythmia without cardiomyopathy, broadening the potential indications for use of trametinib in certain RASopathy patients.

Aims & Objectives: Advances in supportive care including noninvasive oxygen and sedation with dexmedetomidine have made intubation less frequent in bronchiolitis, but the effects of dexmedetomidine on length of stay (LOS) are not understood. We sought to understand the association of dexmedetomidine administration on ICU and Hospital LOS in non-intubated patients with bronchiolitis. Methods: We performed a single-center retrospective study for patients younger-than 2 years-of-age admitted to the ICU with bronchiolitis requiring high flow nasal oxygen (HFNO) or non-invasive mechanical ventilation (NIMV) from 2020-2022. Patients were sorted into two groups: those receiving and those not receiving dexmedetomidine. Primary outcomes were ICU and hospital LOS. Linear regression was used to control for severity of illness. Results: We included 168 patients, 32 (19%) received dexmedetomidine. Those who received and did not receive dexmedetomidine had respective ICU LOS of 4.33 (SD=6.99) and 1.50 days (SD=3.47) (p<0.001) and Hospital LOS of 7.3 (SD =8.6) and 3.5 days (SD=3.9) (p<0.001). The linear regression model had an R-squared of 0.091 with a positive association with PICU LOS (Coefficient=2.61, p=0.005), a negative association for weight (coefficient=-0.40, p=0.037), and no association with age, viral load, HFNO support, or nCPAP support. Duration of NIMV was longer for patients receiving dexmedetomidine. Conclusions: Dexmedetomidine use in critical bronchiolitis is associated with an increased ICU and hospital LOS as well as NIMV duration when controlling for age, weight, and severity of illness. A prospective trial is warranted, but our data suggests routine dexmedetomidine use in bronchiolitis may increase LOS regardless of severity. Keywords: pediatrics, non-invasive respiratory support, Dexmedetomidine, bronchiolitis