J Emerg Med
-
RIPPLY2-associated spondylocostal dysostosis is a rare disorder that leads to segmentation defects of the vertebrae. These vertebral defects can result in severe instability of the cervical spine, leading to cardiac arrest after only minor whiplash injury. ⋯ We present the case of a healthy 7-year-old child who experienced an out-of-hospital cardiac arrest. He was reported to have profound respiratory distress and collapsed after going down a slide, without trauma. He was resuscitated in the field, and presented to the emergency department, where return of spontaneous circulation was achieved. Imaging of his cervical spine revealed multiple abnormalities. It was determined that a whiplash injury led to hypoxia and bradycardia due to the anatomic abnormalities of his cervical spine, resulting in cardiovascular collapse. He recovered fully and was later diagnosed with SCDO6, an autosomal recessive inherited disorder caused by a mutation in the RIPPLY2 gene. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Unfamiliarity of providers with this mechanism of cardiac arrest, and the rarity of the syndrome itself, make early recognition very difficult. Late diagnosis and lack of preventative measures, including immediate cervical spine stabilization, can lead to catastrophic outcomes. In patients with cardiac arrest of unclear etiology, early consideration of cervical spine immobilization and evaluation can be lifesaving.
-
Evaluation and treatment of the acutely ill patient is typically complicated by multiple comorbidities and incomplete medical histories. This is exemplified by patients with sepsis, whose care is complicated by variable presentations, shifting definitions, and a variety of potential sources. Many practitioners fail to consider and recognize less-common sources of infection in a timely manner. Additionally, multiple noninfectious conditions can present with the fever and tachycardia typical of the septic patient. The errors of anchoring and premature closure may lead to delay in, or failure of, diagnosis of these conditions. ⋯ In the seemingly septic patient who does not respond to antimicrobials and fluids, the differential should be broadened to include acutely life-threatening conditions that can mimic sepsis. A review of the patient's medical history, medications, and recent exposures can assist in identifying the source of the patient's elevated body temperature and tachycardia. Consideration of potential sources and other mimics of sepsis is needed in the emergency department.
-
Central vein catheter (CVC) placement using the modified Seldinger technique is a common procedure in the emergency department, but can be time consuming due to the multiple pieces of equipment included in central line kits and the number of steps in the procedure. Preassembled devices combine a needle, guidewire, dilator, and sheath into one unit and potentially simplify the process and reduce time required for CVC placement using the accelerated Seldinger technique. ⋯ In the simulated setting, the accelerated Seldinger technique using combination CVC devices is a faster and easier method for CVC placement compared to the modified Seldinger technique.
-
Case Reports
Acute Presentation and Management of the Encephalopathic Child With an Undiagnosed Inborn Error of Metabolism.
Inborn errors of metabolism (IEM) commonly present in infancy and, less commonly, later in life. ⋯ This case describes an IEM, specifically, ornithine transcarbamylase deficiency, in a previously healthy 7-year-old boy who presented to an emergency department with vomiting for approximately 24 h prior to admission. The child became progressively encephalopathic while in the emergency department, but an ammonia level was not obtained until several hours after admission. Irreversible brain damage with cerebral edema was already present at time of diagnosis, leading to death. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case emphasizes that acute hyperammonemia can rapidly cause irreversible neurological damage and, in the case of a newly encephalopathic pediatric patient, ammonia levels should be evaluated early to facilitate proper diagnostic tests and treatment.