Bauer, Philippe R.

Abstract Background Intracranial hypertension from cerebral edema is a feared complication in patients with acute liver failure. Diagnosing cerebral edema in critically ill patients can be challenging as it may be insidious in onset. We present a case of fulminant hyperammonemia-induced cerebral edema in a patient with newly diagnosed alpha-1-antitrypsin deficiency (A1AD) in the setting of intestinal ileus and ineffective lactulose therapy. Case A 48-year-old male with newly diagnosed A1AD-related cirrhosis, alcohol use, and obesity (BMI 48.9) presented at an outside facility with altered mental status. Physical exam showed waxing and waning mentation, and serum ammonia was 245 mcmol/L. Upon transfer to our facility, hemoglobin was 10.3 g/dL, creatinine 1.77 mg/dL, total bilirubin 1.5 mg/dL, INR 1.2, ALT 60 U/L, AST 107 U/L, ALP 173 U/L, and serum ammonia 138 mcmol/L. Serum alpha-1-antitrypsin returned at 41 mg/dL with SZ phenotype. Abdominal ultrasound showed cirrhosis with moderate ascites. Paracentesis demonstrated no acute peritonitis. After initiation of lactulose and rifaximin, his mental status improved initially. However, he experienced 2 episodes of coffee-ground emesis with hemoglobin dropping to 7.0 g/dL requiring blood transfusions. Urgent esophagogastroduodenoscopy, limited by large residual contents, found no active bleed. A nasogastric tube was placed. CT abdomen pelvis showed diffuse colonic and small bowel dilation consistent with adynamic ileus impairing lactulose administration. His mental status worsened, necessitating ICU transfer and intubation for airway protection. Serum ammonia peaked at 397 mcmol/L. Neostigmine did not restore any effective bowel movement, and continuous renal replacement therapy was initiated to control the hyperammonemia. A convulsive episode prompted imaging. Head CT showed diffuse cerebral edema, later confirmed on MRI (Figure 1). Continuous electroencephalogram showed diffuse slowing without epileptiform discharges. His protracted hospital course was marked by minimal neurological recovery requiring tracheostomy and ongoing supportive measures per family wishes. Discussion Cerebral edema is one of the leading causes of morbidity and mortality in patients with severe hyperammonemia, which can result in irreversible cortical and deep grey matter injury that typically spares perirolandic and medial occipital regions. Although the exact mechanism continues to be researched elevated serum ammonia plays a critical role in disrupting the innate cerebral autoregulation. Prompt diagnosis and early intracranial pressure control (head of the bed elevation, hyperventilation, mannitol, hypertonic saline) are paramount. Typical hyperammonemia treatment includes lactulose, rifaximin, or polyethylene glycol, and correction of any precipitating factors. Dialysis should be considered early to prevent cerebral toxicity in hyperammonemia refractory to conventional treatments.

Background Patients undergoing medical procedures benefit from preprocedural education. Objective To evaluate a multisensory virtual reality preoperative educational program for patients undergoing elective cardiovascular surgical procedures with postoperative recovery in the intensive care unit (ICU) and assess its impact on patients’ outcomes and experience. Methods Patients scheduled for elective cardiovascular surgical procedures with expected recovery in the ICU were enrolled. A multidisciplinary team designed the virtual reality simulation. Educational objectives focused on patient safety, family presence, ICU machinery and activities, reorientation, and communication with the care team. Historical control patients (n = 94) underwent medical record review and were contacted to complete surveys. Virtual reality simulation patients (n = 44) underwent medical record review, viewed the simulation at a preoperative appointment, and completed surveys. The study included patients admitted from June 4, 2019, to May 12, 2022. Results Durations of postoperative sedation and mechanical ventilation were lower in patients receiving virtual reality simulation. Most patients in the virtual reality simulation group (92%) said the simulation alleviated their anxiety and helped them understand what to expect in the ICU. The simulation improved their feeling of safety and decreased their fear of the unknown. Delirium incidence was not different in the 2 groups. Conclusions Preprocedural education via virtual reality simulation can improve the experience and outcomes of patients undergoing elective cardiothoracic surgery with recovery in the ICU.

OBJECTIVES: Acute hypoxemic respiratory failure in immunocompromised patients remains the leading cause of admission to the ICU, with high case fatality. The response to the initial oxygenation strategy may be predictive of outcome. This study aims to assess the response to the evolutionary profiles of oxygenation strategy and the association with survival. DESIGN: Post hoc analysis of EFRAIM study with a nonparametric longitudinal clustering technique (longitudinal K-mean). SETTING AND PATIENTS: Multinational, observational prospective cohort study performed in critically ill immunocompromised patients admitted for an acute respiratory failure. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 1547 patients who did not require invasive mechanical ventilation (iMV) at ICU admission were included. Change in ventilatory support was assessed and three clusters of change in oxygenation modality over time were identified. Cluster A: 12.3% iMV requirement and high survival rate, n = 717 patients (46.3%); cluster B: 32.9% need for iMV, 97% ICU mortality, n = 499 patients (32.3%); and cluster C: 37.5% need for iMV, 0.3% ICU mortality, n = 331 patients (21.4%). These clusters demonstrated a high discrimination. After adjustment for confounders, clusters B and C were independently associated with need for iMV (odds ratio [OR], 9.87; 95% CI, 7.26–13.50 and OR, 19.8; 95% CI, 13.7–29.1). CONCLUSIONS: This study identified three distinct highly performing clusters of response to initial oxygenation strategy, which reliably predicted the need for iMV requirement and hospital mortality.

Abstract Background: Diaphragmatic paralysis can present with orthopnea. We report a unique presentation of bilateral diaphragmatic paralysis, an uncommon diagnosis secondary to an unusual cause, brachial plexitis. This report thoroughly describes the patient’s presentation, workup, management, and outcome. It also reviews the literature on diaphragmatic paralysis and Parsonage-Turner syndrome. Case presentation: A 50-year-old male patient developed insidious orthopnea associated with left shoulder and neck pain over three months with no associated symptoms. On examination, marked dyspnea was observed when the patient was asked to lie down; breath sounds were present and symmetrical, and the neurological examination was normal. The chest radiograph showed an elevated right hemidiaphragm. Echocardiogram was normal. There was a 63% positional reduction in Forced Vital Capacity and maximal inspiratory and expiratory pressures on pulmonary function testing. The electromyogram was consistent with neuromuscular weakness involving both brachial plexus and diaphragmatic muscle (Parsonage and Turner syndrome). Conclusions: Compared to unilateral, bilateral diaphragmatic paralysis may be more challenging to diagnose. On PFT, reduced maximal respiratory pressures, especially the maximal inspiratory pressure, are suggestive. Parsonage-Turner syndrome is rare, usually with unilateral diaphragmatic paralysis, but bilateral cases have been reported.