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On-line version ISSN 1806-907X
Rev. Bras. Anestesiol. vol.55 no.6 Campinas Nov./Dec. 2005
Anesthesia in Guillain-Barré pediatric patient after measles vaccination. Case report*
Anestesia en un niño con síndrome de Guillain-Barré después de la vacuna de sarampión. Relato de caso
Deoclécio Tonelli, TSA, M.D.I; Paula de Camargo Neves Sacco, TSA, M.D.II; Desire Calegari, M.D.III; Gustavo Cimerman, M.D.IV; Carolina Mourão dos Santos, M.D.V; Rodrigo Gonzales Farath, M.D.V
pelo CET Integrado de Anestesiologia da Faculdade de Medicina ABC
IIAnestesiologista, Instrutora do CET Integrado de Anestesia da Faculdade de Medicina ABC
IIIAnestesiologista, Responsável pelo Serviço de Anestesiologia do Hospital Municipal Universitário de São Bernardo do Campo, Assistente da Disciplina de Anestesiologia da FMABC
IVME3 do CET Integrado de Anestesia da Faculdade de Medicina ABC
VME2 do CET Integrado de Anestesia da Faculdade de Medicina ABC
BACKGROUND AND OBJECTIVES:
Guillain-Barré syndrome following measles vaccination is uncommon.
Diagnosis is often delayed, leading to increased morbidity. This report describes
an advanced Guillain-Barré case and the special approaches required during
CASE REPORT: Male patient, four years old, with Guillain-Barré syndrome diagnosed at 1 year of age, submitted to gastrostomy under uneventful general anesthesia with sevoflurane, without neuromuscular blockers.
CONCLUSIONS: The case highlights the low frequency with which this syndrome so important for anesthetic practice is diagnosed, post-vaccination adverse events, the best choice for the anesthetic team and complications of pediatric Guillain-Barré syndrome.
Key words: ANESTHESIA, General: inhalational; DISEASE: Guillain-Barré syndrome
JUSTIFICATIVA Y OBJETIVOS:
El síndrome de Guillain-Barré después de la vacuna de sarampión
es rara. El diagnóstico en la mayoría de las veces es tardío,
lo que lleva a un aumento de la morbidez. Este actual relato presenta un caso
avanzado y todas las atenciones especiales exigidas durante la anestesia.
RELATO DEL CASO: Paciente del sexo masculino, con cuatro años de edad con síndrome de Guillain-Barré desde hace un año de edad, fue sometido a gastrostomía bajo anestesia general sin intercurrencias, con sevoflurano y sin bloqueadores neuromusculares.
CONCLUSIONES: El caso ilustra la rareza etiológica de una síndrome importante en la práctica anestésica, así como los eventos adversos pos-vacunación, la mejor elección para el equipo anestésico y las complicaciones de la síndrome de Guillain-Barré en la infancia.
The incidence of this disease is estimated in 0.5 to 1.5 out of 100 thousand patients below 18 years of age 1. It is an acute motor axonal neuropathy (AMAN) and is the most common cause of generalized paralysis 2. There is peripheral and non-peripheral nerves inflammation pathologically characterized by lymphocytes and macrophages infiltration and medullary destruction 3,4. Typical symptoms are distal paralysis and lower limbs weakness. Weakness progresses to upper limbs and there is often pain in long lower limb and dorsal region muscles. Syndrome evolves to reach respiratory muscles and eye movements, also causing dysphagia and autonomic dysfunction. It presents as paresis, areflexia, symmetric flaccidity, ataxia and ophthalmoplegia 5.
The syndrome is caused by an immunomodulating reaction triggered by previous bacterial or viral infection. Most commonly associated agents are Epstein-Barr, Varicella Zoster, HIV, Cytomegalovirus and Campilobacter 6. Infectious organisms act on peripheral nerves and trigger autoimmune responses leading to axonal degeneration 2.
Gangliosides GM1, GM1b, GD1 and GaINAc-GD1, present on motor axolemma, are the affected molecules 2.
The disease evolves to acute respiratory failure in 15% of cases with the need for mechanical ventilation. There is permanent neurological deficit in 5 to 10% of cases. There is also autonomic nervous system dysfunction and stimulation may trigger tachycardia, arrhythmia and cardiac arrest 1-6. There is 20% morbidity and 10% mortality caused by sepsis, pulmonary thromboembolism, ARDS and heart diseases 2.
This report aimed at describing a case of Guillain-Barré Syndrome developed after measles vaccination and the required differentiated anesthetic approaches.
Male patient, 4 years old, with Guillain-Barré syndrome diagnosed at 1 year of age after measles vaccination, admitted for 9 months due to repetition bronchopneumonia, bronchospasms and atelectasis.
The idea was to perform a gastrostomy with anti-reflux valve placement under general anesthesia to decrease bronchoaspiration episodes.
At preanesthetic evaluation, patient was admitted to the Intensive Care Unit of the Hospital Municipal, Santo André, with tracheostomy and under mechanical ventilation in SIMV with PEEP of 5 cmH2O, support pressure of 15 cmH2O and 12F nasogastric tube.
At physical evaluation patient was hypertensive, with mean blood pressure of 85 to 90 mmHg, tachycardic with heart rate around 140 bpm, and echocardiography revealing atrial septal defect. There were also lack of sphincter control and seizures.
Patient was under aldactone, furosemide, niphedipine, captopril, vancomicin and meropenem.
Patient was premedicated with intravenous midazolam (3 mg).
Patient was referred to the operating room with transport ventilator and was monitored with cardioscopy, pulse oximetry and noninvasive blood pressure, being transferred to the anesthesia machine under mechanical ventilation with tidal volume of 200 mL, PEEP 5 cmH2O, O2 40%, compressed air and sevoflurane maintaining expired concentration between 3% and 4%.
Procedure lasted 2 hours, without intercurrences being patient referred to the pediatric ICU. Patient died on the 60th postoperative day due to sepsis and disseminated intravascular coagulation.
We decided for premedication with midazolam due to anxiety, since patient presented a long disease with multiple previous admissions. Since there was already diaphragm involvement (tracheostomy), there has been no concern with respiratory depression.
Neuromuscular blockers should be avoided because they may worsen symptoms 7.
Succinylcholine should not be used due to hyperkalemia 6,7. Guillain-Barré patients have increased number of extra-junctional acetylcholine receptors, which allow further action and potassium release, which is not prevented with pre-curarization 7,8. There is also autonomic nervous system dysfunction and exacerbation 8; there may be hypertensive crisis, tachycardia and other arrhythmias, making succinylcholine a bad choice 6-8.
Since this is a demielinizing polyradiculoneuritis, these patients are sensitive to nondepolarizing muscle relaxant, which should be avoided for having their action time increased 3.
Sevoflurane was the inhalational anesthetic of choice for its muscle relaxing action, fast recovery, low metabolism rate and adequate autonomic nervous system protection.
There have been recent discussions on the potential association of vaccination and its adverse effects, among them autoimmune diseases 9-14.
Vaccines most commonly associated to adverse effects are DPT (diphtheria, pertussis, tetanus), Influenza, Polio, Hepatitis, Varicella and Measles 9,15.
Among major post-vaccination adverse reactions, most common are fever (25.8%), hypersensitivity at injection site (15.8%), skin erythema (10.8%), seizures (4.4%), abdominal pain (1.8%), neuropathy (0.9%), Guillain-Barré (0.6%), thrombocytopenia (0.5%), and meningitis (0.5%), among others, leading to 1.5% deaths 9.
Less frequent however equally important are shock, comma, encephalopathy, diabetes mellitus, lupus, stroke and intracranial hypertension 9.
Nevertheless, most studies challenge some adverse effects and emphasize the benefits of vaccination as compared to its risks 11-15.
Guillain-Barré patients should be carefully followed up and, when diagnosis is confirmed early, effective treatment may be achieved with plasmapheresis and immunoglobulin. When the disease is advanced, there must be multidisciplinary care to prevent complications worsening diagnosis, very often reserved 2,4.
When patient is to be submitted to surgical procedure under anesthesia, the case should be evaluated to individualize the choice according to existing complications.
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Submitted for publication
February 3, 2005
Accepted for publication September 20, 2005
* Received from CET Integrado de Anestesia da Faculdade de Medicina ABC, Santo André, SP