A & A case reports
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Case Reports
Contemporary perioperative management of adult familial dysautonomia (Riley-Day syndrome).
Familial dysautonomia (Riley-Day syndrome) is a rare multisystem disorder associated with an excess risk of perioperative morbidity and mortality. Because life expectancy is limited, few reports consider the perioperative management of familial dysautonomia in adults with advanced disease and end-organ dysfunction. Here, we report on the management of an adult patient with familial dysautonomia, highlighting recent developments in perioperative technology and pharmacology of special relevance to this challenging population.
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Case Reports
Airway Compromise and Perioperative Management of a Patient with Acquired Factor XIII Inhibitor.
Perioral hematomas can lead to acute airway compromise and can present significant challenges in both direct and indirect approaches to airway instrumentation. In patients with normal cell counts and routine coagulation tests, spontaneous hematomas are rare, but when encountered, they elicit a limited differential diagnosis that includes von Willebrand factor deficiency, platelet dysfunction, and the acquired factor XIII (FXIII) deficiency. Although spontaneous hematoma formation resulting from FXIII inhibition has been reported, we describe what may be the first reported case of FXIII inhibitor-related hematoma leading to acute airway compromise. Successful management of this patient required multidisciplinary cooperation among anesthesiologists, intensivists, otolaryngologists, and hematologists.
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Hyperekplexia is a hereditary disorder characterized by exaggerated startle reflex in response to unexpected acoustic, tactile, and other stimuli. Neonates with hyperekplexia may present with hypertonia, developmental delays, apnea, and sudden death. ⋯ In some cases, a mutation encoding the postsynaptic inhibitory glycine receptors (GLRA1, GLRB) or presynaptic glycine transporter (SLC6A5) resulting in abnormal glycinergic neurotransmission is present. We report the case of a 38-year-old gravida 6 para 1 (G6P1) parturient with hyperekplexia who underwent successful vaginal delivery managed by the anesthesiology and neonatology service teams from initial antenatal consultation to labor and delivery to hospital discharge.
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Venous gas embolism is a complication of fibrin sealant application and is a well-described event during various modes of prostate resection. We describe the case of a nitrogen venous gas embolism during Greenlight laser photovaporization of the prostate during the application of fibrin sealant to the operative site for hemostasis. Fibrin sealant application by a compressed gas applicator is a cause of venous air embolism, and this case highlights the need to keep venous gas embolism in mind when compressed gas applicators are used.