Abordagem anestésica em paciente pediátrico com síndrome de Leigh

Abstract

Background and objectives: Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Surgical and anesthetic procedures stimulate the tracheal irritability, and could exacerbate risks of aspiration, wheezing, breathing diffi culties, gasping, hypoventilation, and apnea. Case Report: We present the anesthetic management for a six-year-old boy with severe form of LS, involving repair of a femur fracture. Propofol and remifentanil were infused for general anesthesia. The patient was closely monitored during anesthesia and in the intensive care unit in the early postoperative period. Conclusions: Close intraoperative monitoring of patients, including invasive arterial blood pressure monitoring and frequently measuring the levels of blood gases, glucose, and lactate, made this procedure run smoothly. Intensive care and breathing support for the patient with LS, under sedation with an analgesic combination during the early postoperative period, minimized the stress response due to pain after surgery.

Alterações Genéticas E Suas Implicações Na Resistência À Anestesia E Na Escolha Da Técnica Anestésica: Análise Dos Aspectos Clínicos

“…Segundo Kocamanoglu e Sarihasan (2013), pacientes com SL sofrem de retardo psicomotor, panencefalite necrosante, convulsões, espasmos musculares e articulares e episódios de apneia, aumento de secreções das vias aéreas e taquipneia, pode apresentar deformidades. O paciente com esse quadro tende a precisar de terapia com oxigênio e umidificação por causa do aumento de secreção das vias aéreas após a cirurgia (8). A extubação tardia é recomendada, haja vista o favorecimento da irritação das vias aéreas devido à condição genética.…”