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Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 112-114

Anesthetic management of children with moyamoya disease undergoing encephalomyoarteriosynangiosis

Department of Anaesthesiology and Intensive Care, PGIMER and Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Submission23-Aug-2020
Date of Decision16-Nov-2020
Date of Acceptance22-Nov-2020
Date of Web Publication16-Apr-2021

Correspondence Address:
Dr. Uma Hariharan
Department of Anesthesiology and Intensive Care, [MBBS, DNB, MNAMS, PGDHM, CCEPC, FICA, DESA], 3rd Floor, PGIMER Building, ABVIMS and Dr. Ram Manohar Lohia Hospital, New Delhi - 110 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/BJOA.BJOA_196_20

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Moyamoya disease (MMD) is chronic cerebrovascular disease with progressive stenosis in various vessels of cerebral circulation leading to the development of collateral vessels. Patients may exhibit ischemic or hemorrhagic complications. Surgical management is usually complicated by impairment in cerebral blood flow and metabolism. The goal of anesthetic management of such patients is to maintain adequate oxygen demand supply balance. We present a case of MMD in a 6-year-old male child and discuss anesthetic management of such patients undergoing indirect vascularization procedures.

Keywords: Cerebral ischemia, indirect revascularization procedures, moyamoya disease, neuroanesthesia

How to cite this article:
Itishri I, Gupta A, Hariharan U. Anesthetic management of children with moyamoya disease undergoing encephalomyoarteriosynangiosis. Bali J Anaesthesiol 2021;5:112-4

How to cite this URL:
Itishri I, Gupta A, Hariharan U. Anesthetic management of children with moyamoya disease undergoing encephalomyoarteriosynangiosis. Bali J Anaesthesiol [serial online] 2021 [cited 2021 Jun 23];5:112-4. Available from: https://www.bjoaonline.com/text.asp?2021/5/2/112/313879

  Introduction Top

Moyamoya disease (MMD) is an inherited vaso-occlusive disease of the cerebrovascular system where progressive stenosis in the internal carotid artery (ICA), anterior cerebral artery, middle cerebral artery (MCA), and anterior communicating artery occurs. As a result, collateral vasculature develops at the base of the brain giving it characteristic “puff of smoke” (Moyamoya in Japanese) appearance on angiography.[1],[2]

The anesthesiologist should be well appraised of the pathophysiology and perioperative management of the disease.[3] The goal in the perioperative period is to maintain adequate cerebral oxygen demand and supply balance.[4]

  Case Report Top

A 6-year-old male, 15 kg, presented with left-sided hemiparesis 8 months back and was diagnosed to be MMD with acute ischemic stroke [Figure 1]. He was managed medically and discharged on aspirin and phenytoin. Thereafter, he was referred to our hospital for surgical management for MMD. Indirect vascularization procedure (encephalomyoarteriosynangiosis) was chosen by neurosurgeons in view of technical difficulty in dissection of the superficial temporal artery in the pediatric population and side effects related to direct vascularization procedure.[5] The patient was evaluated preoperatively and found to have normal birth and developmental history. Other congenital anomalies were ruled out.
Figure 1: Computed tomography angiography showing characteristic puff of smoke appearance

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On the morning of surgery, the patient was premedicated with oral midazolam 0.5 mg/kg. Topical anesthesia was obtained with EMLA cream for venous access and the patient was taken to the operation theater. Standard ASA monitoring was applied. The intravenous line was secured and the patient was preoxygenated with 100% oxygen for 3 min. Intravenous induction was done using titrated doses of propofol 2 mg/kg and fentanyl 2 mcg/kg. After confirmation of successful bag and mask ventilation, vecuronium 0.1 mg/kg given and the airway secured with a cuffed polyvinyl chloride endotracheal tube of 5 mm internal diameter while doing smooth and gentle laryngoscopy.

Lignocaine 1.5 mg/kg was administered intravenously to blunt sympathetic response. The patient was, then, put on controlled mechanical ventilation. Anesthesia was maintained using oxygen, air, and sevoflurane. Apart from above mentioned parameters, end-tidal carbon dioxide, peak airway pressures, end-tidal inhalational agent concentration, temperature, and urine output were also monitored. Forced air warming system was used to keep the patient warm.

Encephalomyoarteriosynangiosis lasted 120 min. Blood loss was 100 ml (maximum allowable blood loss was 240 ml). Adequate crystalloid was given intraoperatively and good urine output of 1.0 ml/kg/h was obtained. Vital signs of the patient were stable throughout the procedure. Fentanyl 1.0 kg and paracetamol 15 mg/kg was administered for analgesia perioperatively. At the end of the procedure, we administered neostigmine 0.05 mg/kg and injection glycopyrrolate 10 μ/kg for reversal, and he was successfully extubated. Postoperative analgesia was managed with intravenous paracetamol, tramadol, and fentanyl. The patient was shifted to the neurosurgical intensive care unit (ICU) for monitoring. The postoperative course was uneventful and there were no neurological deficits.

  Discussion Top

Basic pathology in MMD is impaired cerebral circulation and cerebral metabolism. MMD has a bimodal age distribution and signs and symptoms vary with the age of patients. Children usually present with ischemia/infarction in regions supplied by ICA and MCA resulting in hemiparesis, dysarthria, aphasia, and cognitive impairment.

MMD is readily diagnosed with radiological investigations. Anesthetic management is required for either direct or indirect revascularization procedures. Our patient presented to us for encephalomyoarteriosynangiosis, an indirect revascularization procedure, where temporalis muscle is placed in direct contact with the brain leading to ingrowth of new blood vessels to the cerebral cortex.

During preanesthetic checkup, special attention should be paid to preexisting neurological deficit, incidence of transient ischemic attacks, seizures, and any evidence of infarction. These patients may have coexisting conditions like Down's syndrome, sickle cell disease, neurofibromatosis, congenital heart disease.[3] Proper evaluation to rule out any other associated condition was done in our patient. Also, patients may be on various antiplatelet drugs, anticoagulants, and anticonvulsant medications. These may cause various drug interactions. Our patient was on oral aspirin (half tablet once a day) and phenytoin sodium (50 mg, eight-hourly).

The goal of anesthetic management is to prevent a mismatch between cerebral oxygen demand and supply. This is important because preexisting vessels are already stenosis and newer collaterals are fragile, thus impairing the cerebral autoregulation. Any decrease in cerebral oxygen supply is likely to precipitate ischemic manifestations while sudden surge in cerebral blood flow may cause hemorrhage due to the fragile nature of new collateral vessels.[3] Similarly, any increase in cerebral oxygen demand will cause cerebral vasodilation. However, stenosis vessels are already maximally dilated and any vasodilation which occurs happens in normal vasculature, thereby, inducing cerebral steal phenomenon. These patients are at significant risk for postoperative ischemic events as it takes few months for ingrowth of new vessels. Thus, again it is important to maintain normocarbia, normotension, normovolemia, and normothermia in the postoperative period.[3]

Our patient was induced through intravenous induction using propofol, and endotracheal intubation was done under smooth and gentle laryngoscopy. During the perioperative period, it is important to maintain normocarbia, normotension, normovolemia, and normothermia to prevent precipitation of ischemic event.[3]

Here, it is important to avoid hypotension and hypertension both. Hypotension may occur as a result of systemic vasodilation due to propofol. If it occurs during the induction of anesthesia or maintenance, it should be promptly treated with vasoconstrictors.[4] Also, smooth and gentle laryngoscopy is important to prevent hemodynamic response. It can also be attenuated by use of lignocaine, fentanyl, or additional doses of propofol.[4] We had used injection lignocaine intravenously to attenuate the same. Vecuronium was chosen as neuromuscular blocking drug as it causes minimal cardiovascular changes or histamine release. An alternative technique of inhalational induction using sevoflurane can also be done in patients without intravenous cannula.

Crying and hyperventilation by an anxious child causes hypocapnia. It leads to cerebral vasoconstriction and decreased cerebral blood flow.[5] This was prevented in our patient by adequately premedicating the patient with oral midazolam and use of topical anesthetic cream before securing the venous line. It should be noted that excessive sedation is avoided to prevent hypoventilation which can lead to hypercapnia.

Standard ASA monitoring is employed in all cases, in addition to neurological monitoring parameters like, electroencephalography and somatosensory evoked potential monitoring can be employed in complicated cases. Anesthesia can be maintained either with volatile agents and opioids or by total intravenous anesthesia. In our patient, we had maintained balanced anesthesia using sevoflurane (MAC 1) and air with fentanyl.

Generous intravenous fluids should be given to maintain adequate urine output. Blood loss should be replaced using blood and blood products. Temperature should be monitored and normothermia maintained. Hyperthermia may increase cerebral metabolism causing increased oxygen demand and hence cerebral ischemia.[6] On the other hand, hypothermia may precipitate cerebral vascular spasm.[7]

Early extubation is recommended after the conclusion of surgery. This enables early neurological assessment of the patient. Our patient was also extubated at the end of surgery and shifted to the ICU. Adequate pain management is important to decrease likelihood of postoperative stroke. Intravenous fentanyl, tramadol, and paracetamol were given to our patient to achieve the same.

  Conclusion Top

During the management of MMD for revascularization procedures, anesthetic goals should focus on adequate cerebral oxygenation by maintaining adequate cerebral blood flow and perfusion by careful titration of the anesthetic technique, avoidance of factors that precipitate any untoward event, and continued monitoring and vigilance in the postoperative period.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke 1998;29:1347-51.  Back to cited text no. 1
Suzuki J, Takaka A. Cerebrovascular “moyamoya” disease: Diseases showing abnormal netlike vessels in base of brain. Arch Neurol 1969;20:288-99.  Back to cited text no. 2
Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009;360:1226-37.  Back to cited text no. 3
Sharma VB, Prabhakar H, Rath GP, Bithal PK. Anaesthetic management of patients undergoing surgery for Moyamoya disease - our institutional experience. J Neuroanaesthesiol Crit Care 2014;1:131-6.  Back to cited text no. 4
  [Full text]  
Matsushima Y, Aoyagi M, Suzuki R, Tabata H, Ohno K. Perioperative complications of encephaloduroarteriosynangiosis: Prevention and treatment. Surg Neurol 1991;36:343-53.  Back to cited text no. 5
Kurokawa T, Chen YJ, Tomita S, Kishikawa T, Kitamura K. Cerebrovascular occlusive disease with and without the moyamoya vascular network in children. Neuropediatrics 1985;16:29-32.  Back to cited text no. 6
Malley RA, Frost EA. Moyamoya disease: Pathophysiology and anaesthetic management. J Neurosurg Anesthesiol 1989;2:110-4.  Back to cited text no. 7


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