A&A practice
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Methyl CpG binding protein 2 (MECP2) duplication syndrome is a rare X-linked genetic disease. Core phenotypes include infantile hypotonia, developmental delay, and minimal speech with mild dysmorphic features. Many have refractory epilepsy and recurrent infections, which are the leading causes of mortality. This article presents a case of a patient with MECP2 duplication syndrome who required general anesthesia for respiratory workup and reviews the anesthetic management for these patients, which includes induction technique, choice of drugs, and other major anesthetic concerns.
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A 35-year-old parturient with antiphospholipid syndrome and a working diagnosis of hemolysis, elevated liver enzyme, and low platelets (HELLP) underwent a cesarean delivery 9 hours after receiving heparin. Her preoperative activated partial thromboplastin time and rotational thromboelastometry (ROTEM) intrinsic pathway (INTEM) clotting time were 120 and 1870 seconds, respectively. Fresh frozen plasma was administered for heparin neutralization. The ROTEM INTEM/heparinase assay (HEPTEM) ratio can help confirm heparin neutralization and guide intraoperative transfusion management.
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A 70-year-old Jehovah's Witness was treated with iron carboxymaltose intravenously, recombinant human erythropoietin alpha subcutaneously, and vitamin B12 and folate orally for 9 weeks to raise hemoglobin (Hb) from 10.8 to 17.0 g/dL before explantation of an infected hip joint prosthesis. The target Hb was calculated from the following formula: Hbtarget = Hbfinal/(1 - ABL/EBV), where Hbtarget= Hb to achieve before surgery, Hbfinal = lowest Hb patient could tolerate taking into consideration his comorbidities (7 g/dL), ABL = volume of blood the surgeon estimated the patient would lose intra- and postoperatively (3000 mL), and EBV = estimated blood volume (75 mL/kg for an adult man). Spinal anesthesia was provided with a single shot hyperbaric bupivacaine and fentanyl. ⋯ Surgical blood loss was estimated to be 2500 mL. Hb at the end of surgery was 13.3 g/dL; on postoperative day 5, 11.7 g/L. No blood products were utilized.
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Continuous regional analgesia techniques for ambulatory management of postoperative thoracic and abdominal wall pain are limited. We report the placement of an erector spinae plane (ESP) catheter in a pediatric patient who underwent rib resection for slipping rib syndrome and was discharged on postoperative day 1 with an elastomeric pump for continued regional analgesia in the ambulatory setting. The patient required minimal opioids while the catheter was in place and experienced a functional level that surpassed her preoperative state. Ambulatory ESP peripheral nerve catheters are a feasible and potentially effective option for the treatment of acute postsurgical pain in children.
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Near-infrared spectroscopy (NIRS) is a noninvasive monitor of regional brain tissue oxygenation, and jugular venous oximetry (SjvO2) is a monitor of global cerebral oxygenation. We report the role of intraoperative multimodal monitoring of cerebral oxygenation in the anesthetic management of a patient with grade III intracranial arteriovenous malformation (AVM) presenting for surgical excision. Real-time monitoring of cerebral oxygenation is of much relevance in high-grade AVMs where anesthetic management is focused on neuroprotection and prevention of cerebral hypoxia. Besides, it also helps in prediction, early detection, and judicious management of perioperative complications, which are commonly encountered in high-grade AVMs.