A&A practice
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We describe a case of refractory pulseless polymorphic ventricular tachycardia successfully treated with a bolus of propranolol intravenously, followed by an esmolol infusion and extracorporeal life support for 4 days in a 12-year-old boy later diagnosed with catecholaminergic polymorphic ventricular tachycardia. He had an excellent neurological outcome. Genetic testing for mutations associated with cardiac arrhythmias yielded a mutation of the syntrophin α-1 gene. ⋯ A mutation of this gene at a different locus is implicated in rare cases of long-QT syndrome. The patient subsequently underwent left cardiac sympathetic denervation followed by implantable cardiac defibrillator insertion. He remains symptom and arrhythmia free on atenolol.
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Case Reports
Perioperative Management of a Child With Glucose Transporter Type 1 Deficiency Syndrome: A Case Report.
Glucose transporter type 1 deficiency syndrome (GLUT1DS) causes central nervous system dysfunction including intractable epilepsy caused by impaired glucose transport to the brain. To prevent convulsions and maintain an energy source for the brain in patients with GLUT1DS, the maintenance of adequate ketone body concentrations, compensation of metabolic acidosis, and reduction of surgical stress are essential. We here report the perioperative management of a child with GLUT1DS.
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The perioperative implementation of continuous peripheral nerve blocks is poorly described within the literature for replantation surgeries beyond digital replantation. The management of replantation patients presents a challenging balance between pain control and limb perfusion. We report the successful use of a continuous interscalene catheter in a therapeutically anticoagulated patient after midshaft humerus arm replantation. The benefits of the continuous peripheral nerve block for the patient included improved pain control and potentially improved limb perfusion making it a valuable component of this patient's treatment.
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A 32-year-old woman at 36 weeks gestation with a medical history of corrected Type 1 Arnold Chiari malformation presented with an intractable headache. When methylprednisolone and morphine treatment provided no relief, we performed 2 topical transnasal sphenopalatine ganglion blocks by applying 4% lidocaine drops into each nostril via a cotton-tipped applicator. The patient's symptoms significantly improved, and she was discharged home the same day. She has been without relapse of headaches during the 6 months of follow-up by our pain service.