Total intravenous anesthesia (TIVA) in an infant with Werdnig-Hoffmann disease. Case report

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.

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 microg) and propofol (30 mg) were administered for anesthetic induction. After tracheal intubation, she was ventilated with manual controlled system without CO(2) absorber, Baraka (Mapleson D system), FGF of 4 L.min(-1), and FiO(2) 0.5 (O(2)/N(2)O). Anesthesia was maintained with continuous manual infusion of propofol, 250 microg x kg(-1) x min(-1), and remifentanil, 0.3 microg x kg(-1) x 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.

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.