Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942010000200009
Brazilian Journal of Anesthesiology
Clinical Information

Anestesia venosa total (AVT) em lactente com doença de Werdnig-Hoffmann: relato de caso

Total intravenous anesthesia (TIVA) in an infant with Werdnig-Hoffmann disease: case report

Marco Antonio Cardoso de Resende; Elizabeth Vaz da Silva; Osvaldo José Moreira Nascimento; Alberto Esteves Gemal; Giseli Quintanilha; Eliana Maria Vasconcelos

http://dx.doi.org/10.1590/S0034-70942010000200009 Braz J Anesthesiol, vol.60, n2, p.170-172, 2010
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Resumo

JUSTIFICATIVA E OBJETIVOS: A doença de Werdnig-Hoffmann é a causa mais comum de hipotonia no lactente e quando presente logo após o nascimento tem pior prognóstico. Fraqueza muscular simétrica, arreflexia e fasciculações da língua são característicos. A maioria dos lactentes morre antes dos dois anos por insuficiência respiratória. O presente relato apresenta um caso com técnica venosa total durante anestesia. RELATO DO CASO: Paciente feminina, branca, um ano, 10 kg, estado físico ASA III, com doença de Werdnig-Hoffmann diagnosticada desde os dois meses de idade. Candidata à gastrostomia e fundogastroplicatura na técnica aberta e traqueostomia. Monitorização com cardioscópio, pressão arterial não invasiva, oxímetro de pulso, estetoscópio precordial e temperatura retal após venóclise. Foi préoxigenada e após bolus de atropina (0,3 mg) foi realizada indução anestésica com remifentanil bolus (20 µg) e propofol (30 mg). Após intubação traqueal foi ventilada de forma controlada manual, em sistema sem absorvedor de CO2, Baraka (sistema Mapleson D), FGF de 4 L.min-1, FiO2 0,5 (0(2)/N(2)0). Mantida sob anestesia com propofol 250 µg.kg-1.min-1 e remifentanil 0,3 µg.kg-1.min-1 em infusão contínua manual. O tempo cirúrgico foi de 150 minutos. O despertar ocorreu 8 minutos após o término da infusão, com ventilação espontânea. Duas horas depois foi transferida para unidade pediátrica e recebeu alta hospitalar no 4º dia de pós-operatório. CONCLUSÕES: A escolha da técnica anestésica prioriza a segurança que advém da familiaridade do manuseio dos fármacos existentes. Em crianças com doenças neuromusculares, a anestesia venosa total com remifentanil e propofol em sistemas de infusão, pela duração de ação extremamente curta, pode influenciar a evolução da doença favoravelmente.

Palavras-chave

DOENÇAS, Neurológica, ANESTESIA, Geral

Abstract

BACKGROUND AND OBJECTIVES: Werdnig-Hoffmann disease is the most common cause of hypotonia in infants and its prognosis is worse if it is present shortly after delivery. Symmetrical muscular weakness, areflexia, and fasciculations of the tongue are characteristic. The majority of the infants die before two years of age as a consequence of respiratory failure. The present report presents a case in which total intravenous anesthesia was used. CASE REPORT: This is a 1 year old white female weighing 10 kg, physical status ASA III, with Werdnig-Hoffmann disease diagnosed at two months of age. The patient was a candidate for open gastrostomy, fundus gastroplication, and tracheostomy. After venoclysis, the patient was monitored with cardioscope, non-invasive blood pressure, pulse oximeter, precordial stethoscope, and rectal temperature. She was oxygenated and, after bolus administration of atropine (0.3 mg), boluses of remifentanil (20 µg) and propofol (30 mg) were administered for anesthetic induction. After tracheal intubation, she was ventilated with manual controlled system without CO2 absorber, Baraka (Mapleson D system), FGF of 4 L.min-1, and FiO2 0.5 (O2/N2O). Anesthesia was maintained with continuous manual infusion of propofol, 250 µg.kg-1.min-1, and remifentanil, 0.3 µg.kg-1.min-1. The surgery lasted 150 minutes. The patient regained consciousness 8 minutes after the end of the infusion, ventilating spontaneously. Two hours later, she was transferred to the pediatric unit, being discharged from the hospital on the fourth postoperative day. CONCLUSIONS: The choice of anesthetic technique gives priority to the safety associated with the familiarity of handling available drugs. In children with neuromuscular diseases, due to the extremely short duration, total intravenous anesthesia with remifentanil and propofol in infusion systems can have a favorable influence on disease evolution.

Keywords

ANESTHESIA, General, DISEASES, Neurologic

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