Case report: Acute necrotizing encephalopathy: a report of a favorable outcome and systematic meta-analysis of outcomes with different immunosuppressive therapies.

Acute Necrotizing Encephalopathy (ANE) is a condition characterized by symmetric, bilateral lesions affecting the thalamus and potentially other areas of the brain following an acute febrile illness. It manifests clinically as abrupt development of encephalopathy, or alteration in mental status that often includes development of seizures and progression to coma. Treatment strategies combine immunosuppressive therapies and supportive care with varying levels of recovery, however there are no universally accepted, data-driven, treatment algorithms for ANE. We first report a case of a previously healthy 10-year-old female with acute onset diplopia, visual hallucinations, lethargy, and seizures in the setting of subacute non-specific viral symptoms and found to have bilateral thalamic and brainstem lesions on MRI consistent with ANE. She was treated with a combination of immunomodulatory therapies and ultimately had a good outcome. Next, we present a meta-analysis of 10 articles with a total of 158 patients meeting clinical and radiographic criteria for ANE. Each article reported immunosuppressive treatments received, and associated morbidity or mortality outcome for each individual patient. Through our analysis, we confirm the effectiveness of high-dose, intravenous, methylprednisolone (HD-IV-MP) therapy implemented early in the disease course (initiation within 24 h of neurologic symptom onset). There was no significant difference between patients treated with and without intravenous immunoglobulin (IVIG). There was no benefit of combining IVIG with early HD-IV-MP. There is weak evidence suggesting a benefit of IL-6 inhibitor tocilizumab, especially when used in combination with early HD-IV-MP, though this analysis was limited by sample size. Finally, plasma exchange (PLEX) improved survival. We hope this meta-analysis will be useful for clinicians making treatment decisions for patients with this potentially devastating condition.

Prevalence and Characteristics of Diagnostic Error in Pediatric Critical Care: A Multicenter Study.

OBJECTIVES: Effective interventions to prevent diagnostic error among critically ill children should be informed by diagnostic error prevalence and etiologies. We aimed to determine the prevalence and characteristics of diagnostic errors and identify factors associated with error in patients admitted to the PICU. DESIGN: Multicenter retrospective cohort study using structured medical record review by trained clinicians using the Revised Safer Dx instrument to identify diagnostic error (defined as missed opportunities in diagnosis). Cases with potential errors were further reviewed by four pediatric intensivists who made final consensus determinations of diagnostic error occurrence. Demographic, clinical, clinician, and encounter data were also collected. SETTING: Four academic tertiary-referral PICUs. PATIENTS: Eight hundred eighty-two randomly selected patients 0-18 years old who were nonelectively admitted to participating PICUs. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 882 patient admissions, 13 (1.5%) had a diagnostic error up to 7 days after PICU admission. Infections (46%) and respiratory conditions (23%) were the most common missed diagnoses. One diagnostic error caused harm with a prolonged hospital stay. Common missed diagnostic opportunities included failure to consider the diagnosis despite a suggestive history (69%) and failure to broaden diagnostic testing (69%). Unadjusted analysis identified more diagnostic errors in patients with atypical presentations (23.1% vs 3.6%, p = 0.011), neurologic chief complaints (46.2% vs 18.8%, p = 0.024), admitting intensivists greater than or equal to 45 years old (92.3% vs 65.1%, p = 0.042), admitting intensivists with more service weeks/year (mean 12.8 vs 10.9 wk, p = 0.031), and diagnostic uncertainty on admission (77% vs 25.1%, p < 0.001). Generalized linear mixed models determined that atypical presentation (odds ratio [OR] 4.58; 95% CI, 0.94-17.1) and diagnostic uncertainty on admission (OR 9.67; 95% CI, 2.86-44.0) were significantly associated with diagnostic error. CONCLUSIONS: Among critically ill children, 1.5% had a diagnostic error up to 7 days after PICU admission. Diagnostic errors were associated with atypical presentations and diagnostic uncertainty on admission, suggesting possible targets for intervention.

Antipsychotic Treatment of Delirium in Critically Ill Children: A Retrospective Matched Cohort Study.

OBJECTIVE: To describe the use of pharmacologic treatment in critically ill children treated according to a delirium protocol and compare those treated with antipsychotics to those treated non-pharmacologically. METHODS>: The study included a retrospective matched cohort describing patients who were pharmacologically treated for delirium compared to those with delirium but not treated in a PICU from December 2013 to September 2015, using a delirium management protocol. Patients were matched by age, sex, diagnosis, mechanical ventilation (MV), and presence of delirium. RESULTS: Of 1875 patients screened, 188 (10.03%) were positive for delirium. Of those, 15 patients (8%) were treated with an antipsychotic for delirium. Patients with delirium treated with antipsychotics were younger, had more delirium days (6 vs. 3, p=0.022), longer MV days (14 vs. 7, p=0.017), and longer PICU length of stay (34 vs. 16 days, p=0.029) than in the untreated group. Haloperidol, risperidone, and quetiapine were used in 9, 6, and 2 patients, respectively. Two patients were treated with multiple antipsychotics. Antipsychotic treatment was initiated on day 2 of delirium for 8 of 15 patients (53.3%). Ten patients in the treatment group had improved delirium scores by day 2 of treatment. No significant differences in sedation exposure between groups. No significant adverse effects were reported. CONCLUSIONS: No significant adverse events seen in this small cohort of critically ill pediatric patients with delirium treated with antipsychotic therapy. Patients with early-onset delirium refractory to non-pharmacologic treatment may have a more effective response to antipsychotic therapy than patients with late-onset refractory delirium.

Berlin heart as a bridge to recovery for a failing Fontan.

We report the temporary use of a Berlin Heart ventricular assist device (Berlin Heart AG, Berlin, Germany) for cardiac support of an 18-month-old girl with rapidly progressive ventricular failure after completion of a fenestrated Fontan. After 6 months of cardiac assistance with a single pneumatic pump, catheterization data showed improvement of the ventricular function and the ventricular assist device was successfully removed. A follow-up echocardiogram 6 months after hospital discharge demonstrated marked improvement of ventricular function.

Development of an observational tool on computer use during rounds.

We present an observational tool to capture computer usage patterns during rounds to inform designs of information and communication technology to support clinical discourse during rounds. The tool captures choreography and logistics of information exchanges supported by clinical information systems during rounds. We developed the tool as part of an ongoing video-recording study of communication to under-stand how, when, and why computers are used during multidisciplinary clinical rounds.

Levels of vasopressin in children undergoing cardiopulmonary bypass.

OBJECTIVES: It is accepted treatment to give vasopressin to adults in postcardiotomy shock, but such use in children is controversial. Cardiopulmonary bypass is presumed to attenuate the normal endogenous vasopressin response to shock. We hypothesized that levels of vasopressin in children are altered by bypass, and that children having low endogenous levels perioperatively are more likely to develop hypotension, or require vasopressors. METHODS: Serial levels of vasopressin were assessed prospectively in children undergoing bypass at a single center. RESULTS: Of 61 eligible patients, we enrolled 39 (63%). Their median age was 5 months. The mean level of vasopressin prior to bypass was 18.6 picograms per millilitre, with an interquartile range from 2.6 to 11.4. Levels of vasopressin peaked during bypass at 87.1, this being highly significant compared to baseline (p < 0.00005), remained high for 12 hours at a mean of 73.5, again significantly different from baseline (p = 0.002), were falling at 24 hours, with a mean of 28.1 (p = 0.04), and had returned to baseline by 48 hours, when the mean was 7.4 (p = 0.3). Age, gender, and the category for surgical risk had no influence on the levels of vasopressin. There was no statistically significant relationship between the measured levels and hypotension or the requirement for vasopressors, although a few persistently hypotensive patients had high levels subsequent to bypass. Higher levels correlated with higher levels of sodium in the serum (r(s) = 0.37, p < 0.00005), higher osmolality (r(s) = 0.37, p < 0.00005), and positive fluid balance (r(s) = 0.23, p < 0.008). Preoperative use of inhibitors of angiotensin converting enzyme, preoperative congestive cardiac failure, and longer periods of bypass predicted higher levels during the first eight postoperative hours. CONCLUSIONS: Children do not have deficient endogenous levels of vasopressin following bypass, and lower levels are not associated with hypotension. Any therapeutic efficacy of infusion of vasopressin for post-cardiotomy shock in children is likely due to reasons other than physiologic replacement.