Recurrent intussusception with ileal perforation as a complication of Blue Rubber Bleb Nevus Syndrome in a pediatric patient

Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital condition, characterized by multiple venous malformations that may involve any organ system, most commonly the skin or the gastrointestinal tract. These lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may cause severe complications such as intussusception, volvulus, and intestinal infarction. Intussusception as a complication of BRBNS, although a known complication of the disease, has rarely been reported, especially in the Philippines. In the Philippine Society for Orphan Disorders, only 2 cases of BRBNS are currently included in the organization, including the patient presented in the case report. The treatment of BRBNS that involves the gastrointestinal tract depends on the extent of intestinal involvement and severity of the disease. The treatment aims to preserve the GI tract as much as possible due to the high recurrence in the disease. In this case report, we present a 13 year-old male with BRBNS with previous history of intussusception, successfully managed conservatively;however, upon recurrence, underwent exploratory laparotomy wherein a subcentimeter perforation in the antimesenteric border of the proximal ileum was noted, together with a gangrenous intussuscipiens, and multiple mulberry-like formations on the antimesenteric border of the small bowels. Histopathological findings of the resected bowels showed multiple cavernous hemangiomas consistent with BRBNS. The postoperative course of the patient was unremarkable.Copyright © 2023 The Authors

Focused Ultrasound in Critical Care Outreach for acutely ill and deteriorating patients on the wards

Introduction: Focused Ultrasound in Intensive Care (FUSIC) refers to the use of ultrasound by a trained bedside clinician to guide patient management in real-time. Ultrasound is widely applied in practice and there is growing consensus that it is an essential tool for managing acutely ill patients in the intensive care unit (ICU). The Critical Care Outreach Team uses FUSIC as an additional assessment tool to guide management and decision-making plan for deteriorating patients on the wards. Objectives: To investigate whether how often information gained fromFUSICimaging had an impact on patient care and management decisions in a critical care outreach setting. Methods: A single-centre observational study at an academic tertiary referral institution. We included all patients reviewed by critical care outreach who were assessed by ultrasound during a 12-month period. Routine procedures for teaching purposes were not included. Results: Forty-six patients were assessed and supported by a combined focused lung and heart ultrasound performed at the patient bedside on the wards. In 46 patients FUSIC was instrumental in the differential diagnostic workup and in guiding the clinical management. In 32 (70%) patients FUSIC aided fluid therapy or diuresis (in case of pulmonary oedema) and helped targeting fluid balance. In three patients though to have consolidation on chest x-ray we were able to identify significant pleural effusions without needing an additional CT scan. In four patients with hypotension, an additional CT-PA was warranted due to dilated right ventricle (RV) with abnormal septal motion and decreased left ventricle (LV) size ratio (i.e. sign of right heart strain) as highly suspicious of pulmonary embolus. In two young patients with Coronavirus disease 2019 (COVID-19), using FUSIC we identified severe LV dysfunction which was subsequentially diagnosed as myocarditis and Angiotensin-converting enzyme (ACE) inhibitors therapy was commenced within 24 hours. Further diagnosis included cardiac tamponade (n = 2) requiring pericardiocentesis and pneumothorax (n =1). In all cases, the use of ultrasound helped in promptly referring patients to the specialist team (i.e. respiratory or cardiology) and to the ICU consultant. Conclusions: In our critical care outreach practice, FUSIC is considered an indispensable tool for safe and accurate management of acutely ill and deteriorating patients on the wards.

Nurse-led PoCUS in the Management of the Critically Ill COVID-19 Patient

Introduction: Point-of-Care Ultrasound (PoCUS) can rapidly diagnose presence and severity of COVID-19 disease and associated pathologies.1 PoCUS identifies life-threatening complications at the bedside, with the potential to reduce the need for out-of-department transfers for imaging, alongside associated radiation exposure and spread of infection.2 Use of PoCUS by doctors in the intensive care unit (ICU) is becoming increasingly widespread. However, uptake by ICU nurses is poor despite evidence to suggest comparable accuracy in acquiring and reporting PoCUS scans, and the potential benefit to patients as a result of an increased workforce of competent PoCUS clinicians.3-5 Objective: To report findings in critically ill COVID-19 patients identified through nurse-led cardiac and 6-point lung PoCUS. Method: This case series was part of the national service evaluation led by the Intensive Care Society, SAM, FUSIC, and FAMUS. Conduct was approved by the departmental lead for critical care ultrasound. An ICU nurse trained in Focused Intensive Care Echocardiography (FICE) and Focused Ultrasound in Intensive Care (FUSIC) performed cardiac and 6-point lung PoCUS scans on ICU patients with confirmed COVID-19 disease during the recovery phase. Severity of disease was scored between 0-3 (Table 1) in each lung region (upper anterior;mid-anterior;posterolateral) and a total score calculated (0-18). PoCUS scans were only performed on patients identified by the treating ICU consultant. Correlations between PoCUS findings and patient demographics, key clinical data, physiological parameters, and 30-day outcome were analysed using Pearson's coefficient. Descriptive statistics analysis (mean;standard deviation/ mode;interquartile range) were used to describe data. Results: A cardiac and 6-point lung PoCUS scan was performed on 15 patients. Fourteen (93%) scans were performed to answer lung-specific clinical questions including assessment of ventilation strategy (ventilation mode;PEEP level) in 5 (33%) patients, extravascular lung water assessment in 9 (60%), and lung assessment prior to tracheostomy decannulation in 1 (7%). Moderate to severe COVID-19 was apparent in all lung fields with severity scores from 6 to 14 (Figure 1). Left ventricular (LV) function was normal in 13 (87%) patients, 2 (13%) demonstrated signs of a dilated right ventricle (RV), and 1 (6%) had impaired LV and RV function (Figure 2). Ten scans identified pathologies that contributed to a change in clinical management immediately following the scan (Figure 3). Interventions included: (1) change in fluid management (increased fluid removal on renal filtration, new furosemide prescription) 4 (27%) patients) and a level 2 echo assessment due to identification of new cardiac pathologies (3 (20%) patients). Five patients had no change in care. We identified a moderate positive correlation between lung severity score and APACHE II (Pearson's coefficient: 0.69;p value <0.01). Weak correlation was found between lung severity score and white cell count, SOFA score, and PaO2/ FiO2. There was no difference in 30-day outcome in patients with a higher lung severity score or abnormal cardiac scan. Conclusion: Cardiac and lung PoCUS is a vital tool in the assessment of COVID-19 disease. The addition of ICU nurses to the growing workforce of PoCUS competent clinicians increases availability of real-time imaging.