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Revista Brasileira de Anestesiologia

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

Rev. Bras. Anestesiol. vol.55 no.3 Campinas May/June 2005 



Anesthesia in patient with moyamoya disease. Case report*


Anestesia en paciente portadora de enfermedad de moyamoya. Relato de caso



Adriano Bechara de Souza Hobaika, M.D.I; Vera Coelho Teixeira, TSA, M.D.II; Marcos Guilherme Cunha Cruvinel, TSA, M.D.III; Alexandre Cordeiro Ulhoa, M.D.IV

IEx-ME2 em Anestesiologia do CET/SBA do Hospital Felício Rocho
IICoordenadora do CET/SBA do Hospital Felício Rocho
IIIEspecialista em Clínica Médica; Anestesiologista do Hospital Life Center
IVNeuroradiologista do Hospital Felício Rocho e do Hospital das Clínicas da UFMG





BACKGROUND AND OBJECTIVES: Moyamoya disease is an uncommon progressive cerebral vasculopathy, more frequently diagnosed among Asian individuals, but which has also been described in Brazil. Moyamoya patients may be submitted to different surgical procedures throughout their lives. Anesthesiologists must understand the pathophysiology of the disease and institute adequate perioperative measures to improve patients' prognosis.
CASE REPORT: Female patient, 22 yr-old, chronic renal failure, with moyamoya disease, scheduled for surgical arterial-venous fistula installation. Anesthesia was induced with fentanyl, propofol and atracurium and maintained with sevoflurane. Patient was maintained in normocapnia and normothermia throughout the procedure. Patient was extubated and transferred to the post-anesthetic care unit without complications.
CONCLUSIONS: This article describes the anaesthetic care of a moyamoya disease patient.

Key words: ANESTHESIA, General: inhalational; DISEASES: moyamoya


JUSTIFICATIVA Y OBJETIVOS: La enfermedad de moyamoya es una vasculopatia cerebral progresiva rara, más frecuentemente diagnosticada en individuos asiáticos, y que también viene siendo identificada en Brasil. Durante su vida, pacientes portadores de esta enfermedad pueden ser sometidos a los más variados tipos de procedimientos quirúrgicos. El anestesiologista debe entender la fisiopatología de la enfermedad e instituir las medidas peri-operatorias más adecuadas, en el designio de mejorar el pronóstico de estos enfermos.
RELATO DEL CASO: Paciente del sexo femenino, 22 años, insuficiencia renal crónica, portadora de la enfermedad de moyamoya, sometida a intervención quirúrgica para la instalación de fístula arteriovenosa. La anestesia fue inducida con fentanil, propofol y atracúrio y mantenida con sevoflurano. Durante el procedimiento, la paciente fue mantenida en normocapnia y normotermia. La extubación fue realizada y la paciente transferida a la sala de recuperación pos-anestésica sin complicaciones.
CONCLUSIONES: Este artículo presenta los cuidados anestésicos dados a una paciente portadora de la enfermedad de moyamoya.




Moyamoya disease is a non-inflammatory, non-atherosclerotic and non-amyloidal cerebral vasculopathy more often affecting Japanese and descendents, although being worldwide described. It is an uncommon condition characterized by bilateral stenosis or occlusion of the terminal portion of the internal carotid and/or proximal portions of anterior cerebral or median cerebral arteries. Diagnosis is confirmed by brain angiography, where pathological vessels are presented similar to "cigarette smoke cloud", hence the origin of the Japanese world moyamoya 1,2. Congenital vascular malformation or acquired arteritis may be involved in the etiology of the disease. Autopsies have revealed decreased vessel size with fibrosis by increased intima thickness and internal elastic lamina degeneration.

There are two age peaks for the appearance of symptoms: the first and the third decades. In children, moyamoya disease is primarily manifested by ischemic neurological phenomena, such as paresis, sensory changes, headache, seizure and involuntary movements; in adults it is more commonly manifested as intracranial hemorrhage 3. With the evolution of the disease, IQ may be decreased in up to 60% in a period of 5 to 9 years 4. In general, the disease is associated to other morbidities, such as asthma, Down syndrome and hypothyroidism.

Some surgical revascularization techniques have been used aiming at increasing intracranial collateral circulation flow through extracranial - intracranial bypass, and have shown good results in preventing ischemic episodes in children 5,6. Due to hemorrhagic events, these patients may also have to be submitted to ventricular drainage, hematoma drainage, aneurysm clipping and ventriculoperitoneal shunt 7.



Female patient, 22 years old, 41 kg, not Asian, with moyamoya disease diagnosed 16 years ago, submitted to surgical procedure for arteriovenous fistula installation in right upper limb. Patient presented frequent seizures, mental retardation and lower limbs atrophy, in addition to hypertension and chronic renal failure. Patient was under regular phentoine (anticonvulsant) and codergocrine (peripheral vasodilator).

The day before surgery, 600 mL of packed red cells  were administered via the double- lumen catheter during dialysis. Preoperative tests included hemoglobin 11.0 mg/dL, serum potassium 4.9 mmol/L and prothrombin activity 71%.

Monitoring consisted of pulse oximetry, ECG, noninvasive automatic blood pressure, capnography and nasopharyngeal thermometer. Anesthesia was induced with fentanyl (0.25 mg), propofol (150 mg) and atracurium (25 mg), and was maintained with inhalational sevoflurane (2% to 3%).

Mechanical ventilation was adjusted to keep PETCO2 around 36 mmHg. The patient was warmed through forced warm air circulation device and heated intravenous fluids. Procedure lasted 72 minutes and extubation was achieved without complications. Patient was transferred to the PACU.



Approximately 3800 cases of moyamoya disease were reported in 1997 5 in Japan, where genetic factors seem to be involved 8,9. In a recent cooperative study performed in Korea, more than 330 moyamoya disease patients were identified 3. In Brazil there are approximately 21 cases published until 1999, which is probably an underestimated figure 10.

Anesthetic management of such patients should prioritize brain blood flow maintenance because unfavorable perioperative states may trigger negative neurological events. Normocapnia is critical because there are evidences that both hypocapnia and hypercapnia decrease brain blood flow in these patients 11. Controlled ventilation is advantageous to establish normocapnia since it allows for measurable changes in ventilatory parameters.

Factors such as hypovolemia and hypotension have been also identified in the genesis of perioperative ischemia and should be prevented 12. Ideally, these patients should be kept hypervolemic with urinary output close to 2 Hematocrit values should not be above normal because they may trigger cerebral ischemia, and anemia should be corrected in the preoperative period to maximize oxygen transportation. It has been suggested that hematocrit values should remain between 30% and 42% with mild hemodilution, which may be beneficial for decreasing blood viscosity 13.

Normothermia is also recommended because it has been observed that moyamoya disease patients may present vasospasm in the presence of hypothermia, while hyperthermia may also trigger ischemic events 14. In children, ischemia caused by perioperative hyperventilation due to crying is a common and major complication primarily caused by anxiety and postoperative pain. Effective preoperative sedation and strict pain control should be routinely implemented and it is recommended that venous access be left in place for at least one week 15. Regarding volatile anesthetics, isoflurane and sevoflurane seem to be safe options for providing significant cerebral protection during transient ischemia 16-18.

Infrared spectroscopy is a noninvasive method for continuous transcranial monitoring of cerebral oxygenation. It should be used in case of risk of cerebral ischemia. In these situations, bilateral monitoring is recommended 19. Moyamoya disease patients are more susceptible to cerebral ischemia, so transcranial oximetry monitoring may be very useful.

Patient had also chronic renal failure what prevented the establishment of hypervolemia and hemodilution, in addition to making impossible to measure urinary output. Normocapnia was maintained with mechanical ventilation and adequate PETCO2 adjustment to capnography. Hypothermia was controlled with warmed 0.9% saline infusion and forced warm air circulation over patient's body.

Regional or local anesthesia associated to sedation could have been used in the patient, however due to the level of mental retardation and psychomotor agitation, we decided for general intravenous anesthesia which provided smooth anesthesia in addition to allowing adequate PETCO2 control.

Although there are some established anesthetic approaches for moyamoya disease, all co-morbidities should be thoroughly evaluated by the anesthesiologist and specific perioperative approaches should be determined on a case-by-case basis.



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Correspondence to
Dr. Adriano Bechara de Souza Hobaika
Address: Rua Elza Brandão Rodarte, 137/1602 Belvedere
ZIP: 30320-630 City: Belo Horizonte, Brazil

Submitted for publication July 27, 2004
Accepted for publication January 27, 2005



* Received from Hospital Felício Rocho, Belo Horizonte, MG

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