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
PDF Português
PDF English
Downloads: 0
Views: 872

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

References

Kaufman P, Finkel R. Learning to walk: challenges for spinal muscular atrophy clinical trials. Neurology. 2007;68:11-12.

Menkes JH, Sarnat HB. Diseases of the Motor Unit. Child Neurology. 2000:469-471.

Hardart MKM, Truog RD. Spinal muscular atrophy-type I. Arch Dis Child. 2003;88:848-850.

Cobben JM, Lemmink HH, Snoeck I. Survival in SMA type I: a prospective analysis of 34 consecutive cases. Neuromuscul Disord. 2008;18:541-544.

Chung BHY, Wong VCN, Ip P. Spinal muscular atrophy: survival pattern and functional status. Pediatrics. 2004;114:e548-553.

Prior TW. Spinal muscular atrophy diagnostics. J Child Neurol. 2007;22:952-956.

Burlet P, Burglen L, Clermont O. Large scale deletions of the 5q13 region are specific to Werdnig-Hoffmann disease. J Med Genet. 1996;33:281-283.

Baranov D, Kelton T, McClung H. Neurologic Diseases. Anesthesia and Uncommon Diseases. 2006.

Carvalho M. Anestesia para crianças hipotônicas. CEDAR. 2004:4-7.

Bush A, Fraser J, Jardine E. Respiratory management of the infant with type 1 spinal muscular atrophy. Arch Dis Child. 2005;90:709-711.

Videira RLR, Cruz JRS. Remifentanil na prática clínica. Rev Bras Anestesiol. 2004;54:114-128.

Martins CEC, Albuquerque MAC, Simoni RF. Remifentanil. Curso de Educação à Distância em Anestesiologia. 2007:126-145.

Ehran E, Ugur G, Gunusen I. Propofol: not thiopental or etomidate - with remifentanil provides adequate intubating conditions in the absence of neuromuscular blockade. Can J Anaesth. 2003;50:108-15.

Batra YK, Al Qattan AR, Ali SS. Assessment of tracheal intubating conditions in children using remifentanil and propofol without muscle relaxant. Paediatr Anaesth. 2004;14:452-456.

Crawford MW, Hayes J, Tan JM. Dose-response of remifentanil for tracheal intubation in infants. Anesth Analg. 2005;100:1599-1604.

Morgan JM, Barker I, Peacock JE. A comparison of intubating conditions in children following induction of anaesthesia with propofol and suxamethonium or propofol and remifentanil. Anaesthesia. 2007;62:135-139.

Kretz FJ. The future of paediatric anaesthesia is total intravenous anaesthesia. Curr Opin Anaesthesiol. 2002;15:305-307.

Nora FS, Fortis EAF. Remifentanil: por que precisamos de outro opióide?. Rev Bras Anestesiol. 2001;51:146-159.

Nora FS, Klipel R, Ayala G. Remifentanil: o regime de infusão faz diferença na prevenção das respostas circulatórias à intubação traqueal?. Rev Bras Anestesiol. 2007;57:247-260.

Wysowski DK, Pollock ML. Reports of Death with use of propofol (Diprivan) for nonprocedural (long-term) sedation and literature review. Anesthesiology. 2006;105:1047-1051.

Wolf AR, Potter F. Propofol infusion in children: when does an anesthetic tool become an intensive care liability. Pediatr Anesth. 2004;14:435-438.

Burow BK, Johnson ME, Packer DL. Metabolic acidosis associated with propofol in the absence of other causative factors. Anesthesiology. 2004;101:239-241.

Muñoz HR, Cortinez LI, Ibacache ME. Remifentanil requirements during propofol administration to block the somatic response to skin incision in children and adults. Anesth Analg. 2007;104:77-78.

Lerman J, Johr M. Inhalational anesthesia vs total intravenous anesthesia (TIVA) for pediatric anesthesia. Pediatr Anesth. 2009;19:521-534.

Flick RP, Gleich SJ, Herr MMH. The risk of malignant hyperthermia in children undergoing muscle biopsy for suspected neuromuscular disorder. Pediatr Anesth. 2007;17:22-27.

5dd2e47c0e8825c429c63494 rba Articles
Links & Downloads
2352-2291 (Electronic) 0104-0014 (Printed)
Braz J Anesthesiol ©2024 All rights reserved.

Braz J Anesthesiol

Share this page
Page Sections