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Revista Brasileira de Anestesiologia

Print version ISSN 0034-7094On-line version ISSN 1806-907X

Rev. Bras. Anestesiol. vol.52 no.3 Campinas May/June 2002 



Moyamoya disease and sevoflurane anesthesia outside the surgery center. Case report *


Enfermedad de Moyamoya y anestesia con sevoflurano fuera del centro quirúrgico. Relato de caso



Sheila Braga Machado, M.D.I; Florentino Fernandes Mendes, TSA, M.D.II; Adriana de Campos Angelini, M.D.III

IME2 do CET/SBA da Santa Casa de Misericórdia de Porto Alegre
IIChefe do Serviço de Anestesiologia da Santa Casa de Misericórdia de Porto Alegre
IIIAnestesiologista da Santa Casa de Misericórdia de Porto Alegre





BACKGROUND AND OBJECTIVES: Moyamoya disease is a progressive cerebrovascular disorder implying anesthetic challenges due to patients’ poor brain perfusion, in addition to being a major cause for stroke in young people. This report aimed at describing a case of Moyamoya’s disease in a patient submitted to general anesthesia with sevoflurane for a diagnostic procedure outside the surgery center.
CASE REPORT: Male child, 13 years old, physical status ASA IV, with Moyamoya disease and neurological sequelae after three previous strokes, chronic renal failure and systemic hypertension admitted for high digestive endoscopy. In the supine position and after monitoring, inhalational induction was attained through the tracheostomy canulla with sevoflurane (gradual inhaled concentration increase up to 6%) in a mixture of 50% oxygen/nitrous oxide. An intravenous catheter was inserted for 5% glucose solution infusion. Manual controlled ventilation was started and anesthesia was maintained with 4% sevoflurane in 50% oxygen/nitrous oxide. At the end of the procedure, all anesthetic agents were simultaneously withdrawn and 100% oxygen was administered. Anesthesia was satisfactory, with good hemodynamic stability, without complications and with early emergence.
CONCLUSIONS: Sevoflurane may open new perspectives for inhalational anesthesia in patients with neurological diseases to be submitted to outpatient procedures, since it provides hemodynamic stability and early emergence, while preserving brain physiology.

Key words: ANESTHETICS, Volatile: sevoflurane; DISEASES, Neurologic: Moyamoya disease


JUSTIFICATIVA Y OBJETIVOS: La enfermedad de Moyamoya es un desorden cerebrovascular progresivo que representa un desafío anestésico en virtud de la precaria circulación cerebral de estos pacientes, constituyéndose una importante causa de accidente vascular cerebral en individuos jóvenes. El objetivo de este relato es presentar el caso de un paciente con enfermedad de Moyamoya que fue sometido a anestesia general con sevoflurano para procedimiento diagnóstico fuera del centro quirúrgico.
RELATO DEL CASO: Niño con 13 años, estado físico ASA IV, portador de enfermedad de Moyamoya con secuela neurológica después de tres accidentes vasculares cerebrales, insuficiencia renal crónica e hipertensión arterial sistémica, sometido a endoscopia digestiva alta. En decúbito dorsal y después monitorización, se realizó inducción inalatoria por la cánula de traqueostomía con sevoflurano (aumento gradual de la concentración inspiratoria hasta 6%) y mezcla de oxígeno/óxido nitroso a 50%. Un catéter venoso fue introducido para infusión de solución glucosada a 5%. Fue realizada ventilación controlada manual, siendo la manutención de la anestesia hecha con sevoflurano a 4% y mezcla de oxígeno/óxido nitroso a 50%. Al final del procedimiento los agentes anestésicos fueron descontinuados simultáneamente y fue administrado oxígeno a 100%. La anestesia fue satisfactoria, con buena estabilidad hemodinámica, sin ocurrencia de complicaciones durante el procedimiento y con un precoz despertar.
CONCLUSIONES: El sevoflurano puede ofrecer nuevas perspectivas para la anestesia inhalatoria en pacientes con enfermedad neurológica que realizan procedimiento ambulatorial, ya que permite buena estabilidad hemodinamica y despertar precoz, preservando la fisiología cerebral.




Moyamoya disease is characterized by internal bilateral carotid arteries terminals narrowing or occlusion, creating an abnormal blood vessels network, called Moyamoya vessels 1-3. Moyamoya means “cigarette smoke cloud” and the word was used to describe the classic appearance of abnormal vessels seen in brain angiography 3.

It affects primarily children and young adults 1,2,4, and is more prevalent among females 4. Disease’s etiology is still unknown but a multifactorial heritage is considered due to the high incidence in Japanese and Korean individuals 1. Acquired forms have also been described and may result from a variety of diseases including meningitis, neurofibromatosis, connective tissue disease and chronic neck inflammation 2.

Most common presentations in children are recurrent transient ischemic attacks, often followed by hemiparesias or seizures, with gradual development of fixed neurological deficits 1,3. Adults and children may have consciousness disorders, aphasia, sensory and cognitive deficits, involuntary movements and vision problems.

Although rare, Moyamoya disease is an important brain ischemia factor in young people and may be underreported; only 18 cases were published in Brazil until 1997 2.

Anesthetic management of such patients must take into consideration the importance of preserving an adequate balance between brain blood flow and oxygen consumption. It has been said that certain anesthetic agents may provide some degree of brain protection due to their ability to depress or abolish cortical electric activity and decrease brain oxygen consumption 5.

Sevoflurane is an inhalational anesthetics with desirable properties, such as low blood/gas partition coefficient (low blood solubility) 6-9. It is potentially ideal for children or neonates due to its pleasant odor and non-irritating properties 7,10, which allow for rapidly achieving high alveolar concentrations 9.

This report aimed at describing a patient with Moyamoya disease submitted to general anesthesia with sevoflurane for a diagnostic procedure outside the surgery center.



Male child, 13 years old, with weight/height unbalance (weight below percentile 10), with tracheostomy for 8 months, physical status ASA IV, diagnostic of Moyamoya disease, submitted to high gastric endoscopy. Patient presented with chronic renal failure, systemic hypertension and fixed neurological deficit (without reacting to life since the last stroke at 12 years of age). At 26 months of age, patient was submitted to brain arterial-venous fistula clipping and at 3 years of age he suffered nefrectomy, both procedures without intercurrences.

Patient had no fever, with respiratory rate of 18 rpm and heart rate (HR) of 85 bpm. Initial parameters in the evaluation room were: SpO2 = 96%, HR = 86 bpm and BP = 130 x 80 mmHg. After monitoring with cardioscopy in DII, pulse oximetry and non-invasive manual blood pressure, inhalational anesthesia was induced with sevoflurane and 50% oxygen/nitrous oxide by the tracheostomy canulla and spontaneous ventilation was maintained until loss of ciliary reflex. Inspired sevoflurane concentration was gradually increased at every 4 ventilations in the following order: 2%, 4% and 6%. Sevoflurane was administered by a gaged vaporizer. Anesthesia was induced with a Mapleson D circuit, via direct connection of gas mixture exit from the anesthesia machine to the tracheostomy tube. Fresh gas flow during induction was 4 L.min-1. Immediately after anesthetic induction, a venous catheter was inserted for 5% glucose solution infusion. Patient was maintained under manual controlled ventilation with 4% sevoflurane and 50% oxygen/nitrous oxide. At the end of the procedure, all anesthetic agents were simultaneously withdrawn and 100% oxygen was administered. According to the parameters observed (systolic blood pressure between 100-110 mmHg and HR between 75-85 bpm) there has been good hemodynamic stability. Anesthesia lasted for 40 minutes and the procedure approximately 30 minutes. Vital signs remained stable in the recovery room and emergence occurred 5 minutes after the end of anesthesia.



There are few data in the literature on the management of Moyamoya disease patients under general anesthesia. These patients have an already affected brain circulation so, the basic anesthetic handling principle should be the preservation of an adequate brain blood flow and oxygen consumption balance 4. The objective of the anesthetic induction was to maintain stable both brain and systemic hemodynamics. Partial carbon dioxide pressure was not measured in our case, but it is essential due to the major influence of CO2 in brain circulation. Both hypercapnia and hypocapnia affect brain circulation and normocapnia, then, should be maintained. Isoflurane is currently the agent of choice for anesthetic maintenance for its potentially beneficial brain effects. It is a brain vasodilator and a potent brain metabolic rate depressor, being able to protect against ischemia. Nitrous oxide may also cause brain vasodilation. Alternative techniques may be acceptable, provided the basic principle of maintaining brain and systemic hemodynamic stability is followed 4. Anesthetic agents allowing a fast and smooth induction, assuring easy and safe anesthetic maintenance with minimum hemodynamic changes, preserving brain physiology and assuring a fast recovery are considered adequate.

Sevoflurane seems to have a two-fold effect on brain vessels. Initially in low doses it induces indirect vasoconstriction by decreasing metabolic needs and, in higher doses, causes vasodilation due to agent’s intrinsic effects. When sevoflurane concentration is increased to 1.3 MAC, brain blood flow is clearly increased but there is no intracranial pressure increase, preserving brain vascular self-regulation 9.

Sevoflurane has pharmacokinetic and pharmacodynamic profiles similar to isoflurane, being a direct dose-dependent brain vasodilator. Sevoflurane at 1.5 MAC maintains brain self-regulation, while the same isoflurane concentration does not. Sevoflurane preserves brain physiology better than isoflurane, even in clinically high concentrations 9.

In our case, anesthesia was induced without intercurrences, with maintenance of stable vital signs in a patient with potential risk for brain ischemia and with an early emergence outside the surgery center. It is then suggested that sevoflurane might be an alternative for procedures in children with neurological diseases. It seems to be safe for systemic and brain hemodynamic stability and has a low incidence of adverse effects.



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Correspondence to
Dra. Sheila Braga Machado
Address: Rua Cel. Orlando Pacheco, 96
ZIP: 91440-050 City: Porto Alegre, RS

Submitted for publication August 15, 2001
Accepted for publication October 30, 2001



* Received from  Serviço de Anestesiologia da Santa Casa de Misericórdia de Porto Alegre, RS

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