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

SUMMARY Resende MAC, Silva EV, Nascimento OJM, Gemal AE, Quintanilha G, Vasconcelos EM – Total Intravenous Anesthesia (TIVA) in an In-fant with Werdnig-Hoffmann Disease. Case Report. 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 character-istic. The majority of the infants die before two years of age as a con-sequence 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 gas-trostomy, fundus gastroplication, and tracheostomy. After venoclysis, the patient was monitored with cardioscope, non-invasive blood pres-sure, 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 ad-ministered for anesthetic induction. After tracheal intubation, she was ventilated with manual controlled system without CO