A & A case reports
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Case Reports
Avoiding Cardiovascular Collapse: Pediatric Cutaneous Mastocytosis and Anesthetic Challenges.
Mastocytosis includes a spectrum of diseases characterized by abnormal mast cell infiltration in various organs, which can lead to mast cell mediator release and immediate hypersensitivity. We review anesthetic challenges presented by a 6-year-old girl with a history of mast cell mediator release because of the urticaria pigmentosa variant of cutaneous mastocytosis, factor VII deficiency, increasing episodes of urinary tract infections, and pyelonephritis. She underwent spine magnetic resonance imaging, subsequent lumbar laminectomy for fatty filum release, and a cystourethroscopy. Perioperative management included factor VII desensitization, avoidance of triggers, minimizing histamine-releasing medications, mast cell stabilization, and preparation for potential immediate hypersensitivity.
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PHACE syndrome is a disorder that features posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. PHACE syndrome includes abnormalities in several organ systems that may influence anesthetic management. We discuss the anesthetic management of a 26-year-old woman with PHACE syndrome presenting for cesarean delivery. Management included careful airway examination, slowly dosed epidural anesthesia, close hemodynamic monitoring aided by a radial arterial line, and continuous intraoperative neurologic assessment.
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Neonatal progeroid syndrome is a unique condition wherein features of aging are apparent in a newborn at birth. It is a very rare genetic disorder. The first case in India was reported in December 2011. ⋯ Independently described by Rautenstrauch and Wiedemann, neonatal progeroid syndrome poses significant challenges to the anesthesiologist for a variety of anatomic and physiologic reasons. Coronary and cerebrovascular atherosclerosis pose significant concerns in such children. Here, we present the successful anesthetic management of a 6-month-old male infant with neonatal progeroid syndrome operated on for bilateral inguinoscrotal swellings.
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We present a case of an ex-28-week, extremely low-birth-weight infant who was transferred to our institution for bronchoscopically assisted removal of an aspirated foreign body. This case presented several challenges because of the patient's extreme prematurity as well as the need for repeated tracheal extubations and reintubations during the procedure to accommodate surgical instruments in the patient's airway. We discuss the respiratory physiology, common comorbidities, and management of aspirated foreign bodies in the premature infant and emphasize the importance of clear communication in the operating room between the multidisciplinary team of physicians involved in this patient's care.
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Anti-N-methyl-D-aspartate receptor encephalitis is caused by autoantibodies to N-methyl-D-aspartate receptors. This disorder is poorly understood and occurs very rarely in children. We describe a total IV anesthetic for a 4-year-old boy with anti-N-methyl-D-aspartate receptor encephalitis and review the potential drug interactions with this autoimmune disease.