Resumo
JUSTIFICATIVA E OBJETIVOS: A síndrome de Leigh (SL) é uma doença rara causada por anomalias na produção de energia mitocondrial. O sistema nervoso central é afetado com mais frequência, com retardo psicomotor, convulsões, nistagmo, oftalmoparesia, atrofia óptica, ataxia, distonia ou insuficiência respiratória. Os procedimentos cirúrgicos e anestésicos provocam irritabilidade traqueal e podem exacerbar os riscos de aspiração, sibilação, dificuldade respiratória, respiração ofegante, hipoventilação e apneia. RELATO DE CASO: Apresentamos uma abordagem anestésica usada em um menino de seis anos com a forma grave de SL que envolve reparação de uma fratura de fêmur. Propofol e remifentanil foram infundidos para anestesia geral. O paciente foi atentamente monitorado durante a anestesia e sua permanência na unidade de terapia intensiva no período pós-operatório inicial. CONCLUSÕES: Uma atenta monitoração intraoperatória dos pacientes, que inclui pressão arterial invasiva e mensurações frequentes da gasometria, glicose e lactato, faz esse procedimento transcorrer sem problemas. A terapia intensiva e a assistência respiratória ao paciente com SL sob sedação, com uma combinação de analgésicos durante o período pós-operatório inicial, minimizaram a resposta ao estresse causado pela dor pós-cirúrgica.
Palavras-chave
Doença de Leigh, ANESTESIA, Geral, MONITORAÇÃO, TERAPIA INTENSIVA
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 difficulties, 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.
Keywords
Leigh Disease, Anesthesia, General, Monitoring, Physiologic, Intensive Care
Referencias
Leigh D. Subacute necrotizing encephalomyopathy in an infant.. J Neurol Neurosurg Psychiatry.. 1951;14:216-221.
Chol M, Lebon S, Bénit P. The mitochondrial DNA G13513A MELAS mutation in the NADH dehydrogenase 5 gene is a frequent cause of Leigh-like syndrome with isolated complex I deficiency.. J Med Genet. 2003;40:188-191.
Finsterer J. Leigh and Leigh-like syndrome in children and adults.. Pediatr Neurol. 2008;39:223-35.
Shenkman Z, Krichevski I, Elpeleg ON, Joseph A, Kadari A. Anaesthetic management of a patient with Leigh's syndrome. Can J Anaesth. 1997;44:1091-1095.
Thorburn DR, Rahman S. Mitochondrial DNA-associated Leigh syndrome and NARP. Source Gene Reviews. 1993.
Shear T, Tobias JD. Anesthetic implications of Leigh's syndrome. Paediatr Anaesth. 2004;14:792-7..
Grattan-Smith PJ, Shield LK, Hopkins IJ, Collins KJ. Acute respiratory failure precipitated by general anesthesia in Leigh's syndrome. J Child Neurol. 1990;5:137-141.
Gozal D, Goldin E, Shafran-Tikva S, Tal D, Wengrower D. Leigh syndrome: anesthetic management in complicated endoscopic procedures. Paediatr Anaesth. 2006;16:38-42.
Sasaki R, Nanjo K, Hirota K. Perioperative anesthetic managements for the laryngo-tracheal separation and open fundoplication in a 17-year-old patient with Leigh syndrome. Paediatr Anaesth. 2008;18:1133-1134.
Facebook
Google+
Twitter
LinkedIn
Mendeley
StumbleUpon
CiteULike
Reddit
Email