Paediatric anaesthesia
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Awake craniotomies with functional cortical mapping are performed to minimize post-operative deficits from the resection of lesions adjacent to eloquent cortex. The procedure is well-established in the adult patient population and is increasingly applied to well-selected pediatric patients. A review of recent literature demonstrated that the most commonly reported anesthetic techniques were "asleep-awake-asleep" protocols that relied on propofol, remifentanil, or fentanyl. ⋯ Awake craniotomies can safely be performed in the pediatric population with appropriate patient sel7ection, planning, and a multi-disciplinary approach.
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Paediatric anaesthesia · Feb 2025
ReviewOptimizing the anesthetic care of patients with aromatic l-amino acid decarboxylase deficiency.
Aromatic l-amino acid decarboxylase (AADC) deficiency is a rare autosomal recessive disorder that results in a lack of the monoamine neurotransmitters dopamine, serotonin, norepinephrine, and epinephrine. Patients present with a wide spectrum of symptoms, including motor and autonomic dysfunction, hypotonia, and developmental delay, often before the age of one. Until recently, treatment options were limited to symptom control, but the recent approval of the first gene therapy for AADC deficiency in Europe and the UK has provided an alternative to treating symptoms for this disease. ⋯ Intraoperatively, utmost care must be taken to protect the skin, maintain body temperature, and to prepare for inotropic and/or glycemic support as needed. Postoperative intensive care management is necessary for consideration of postoperative extubation and provision of supportive care. With careful planning, preparation, and vigilance, patients with AADC deficiency can safely undergo anesthesia.