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   Table of Contents      
CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 1  |  Page : 73-75

Anesthetic Management in Pediatric Patient with Moyamoya Disease in Orthopaedic Surgery: “Managing the Precarious Circulation”


1 PGIMS Rohtak, New Delhi, India
2 Department Of Anesthesia, Maulana Azad Medical College, New Delhi, India

Date of Submission01-Jan-2021
Date of Decision14-Dec-2021
Date of Acceptance02-Jan-2022
Date of Web Publication17-Jan-2022

Correspondence Address:
Dr. Divya Gahlot
Department of Anesthesia, MAMC, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mamcjms.mamcjms_3_21

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  Abstract 


A 12-year-old female, diagnosed case of Down phenotype with Moyamoya disease (MMD) was posted for internal fixation of compound fracture shaft of femur. Preanesthetic evaluation revealed history of acute URI, abnormal body movements, left hemiparesis and mental retardation. Patient was managed under general anesthesia with proseal LMA and caudal epidural was administered for adequate analgesia in the perioperative period. The goal of anaesthetic management in a case of MMD is to maintain the balance between cerebral metabolic oxygen consumption rate (CMRO2) and cerebral blood flow (CBF) in order to prevent any neurologic morbidity. Though there is no ideal anaesthetic agent in MMD, the anaesthetic technique should ensure normotension, normocapnia and normothermia in the perioperative period. To our knowledge regional analgesia has not been used in the pediatric orthopedic surgeries in patients diagnosed with Moya moya. The technique of general anaesthesia supplemented with caudal epidural analgesia ensures that all the anesthetic goals are met adequately and the precarious cerebral circulation in moya moya patient is not compromised.

Keywords: Moyamoya, dexmedetomidine, cerebral blood flow, caudal analgesia, sacral epidural


How to cite this article:
Manohar M, Wadhwa B, Saxena K, Gahlot D. Anesthetic Management in Pediatric Patient with Moyamoya Disease in Orthopaedic Surgery: “Managing the Precarious Circulation”. MAMC J Med Sci 2022;8:73-5

How to cite this URL:
Manohar M, Wadhwa B, Saxena K, Gahlot D. Anesthetic Management in Pediatric Patient with Moyamoya Disease in Orthopaedic Surgery: “Managing the Precarious Circulation”. MAMC J Med Sci [serial online] 2022 [cited 2022 May 24];8:73-5. Available from: https://www.mamcjms.in/text.asp?2022/8/1/73/335901




  Introduction Top


Moyamoya disease (MMD) is an idiopathic condition that is characterised by chronic progressive narrowing of the bilateral supraclinoid internal carotid arteries, proximal anterior cerebral arteries and middle cerebral arteries.The use of regional analgesia in pediatric patient with moya moya has not been established yet. We discuss the successful anesthetic management of a pediatric patient, planned for orthopaedic intervention, under general anesthesia with caudal epidural analgesia


  Case report Top


A 12-year-old, 15 kg female child, diagnosed case of Downs phenotype with MMD was posted for internal fixation of left sided compound fracture shaft of femur. Preanesthetic evaluation revealed history of acute URI, abnormal body movements, left hemiparesis and mental retardation. Since the duration of these symptoms was more than 24 hours, transient ischemic attack was ruled out. The facial profile was abnormal with low set ears, large tongue, sandal gap and flattened nasal bridge. The child was on levetiracetam treatment since one month following a seizure episode. Airway examination revealed a high arched palate, MP class 0 with poor oro-dental hygiene. The routine biochemical investigations were normal but T wave inversion in V1 −V5 was reported on Electrocardiography. Echocardiography done subsequently reported global hypokinesia with ejection fraction of 45 %. We decided to take up the patient under general anaesthesia with caudal epidural for peri-operative analgesia. Patient was premedicated with syrup midazolam, leviteracetam was given before surgery. Inhalation induction was accomplished with sevoflurane (6-8%) in oxygen. After securing intravenous line, 30 mg hydrocortisone and 30 μg Fentanyl were administered. Following muscle relaxation with vecuronium bromide, airway was secured with Proseal LMA #2. Hydrocortisone was prophylactically given to prevent perioperative respiratory complications as a result of preexisting URI. Caudal epidural analgesia with 11 ml of 0.25% bupivacaine was administered with patient in left lateral position. Sevoflurane was gradually tapered off and anaesthesia maintained under Bi spectral index monitoring on oxygen and 0.3 μg/Kg/hr of dexmedetomidine, under BIS (Bi spectral index) monitoring for rest of the procedure. Standard intraoperative monitoring was done. Twenty minutes prior to extubation, 150 mg paracetamol IV was administered and Dexmedetomidine infusion was stopped. The intra-operative period was uneventful and extubation was smooth with rapid recovery. Post operatively patient was calm, pain free and comfortable


  Discussion Top


First described in 1961 by Nomura and Takeuchi [1],[2] MMD is an idiopathic condition that is characterised by chronic progressive narrowing of the bilateral supraclinoid internal carotid arteries, proximal anterior cerebral arteries and middle cerebral arteries. Gradual reduction of cerebral blood flow stimulates collateral vessel formation, producing the characteristic blush seen on angiography. The term Moyamoya, a Japanese word meaning “puff of smoke” describes the angiographic appearance of abnormal collateral vasculature related to the circle of Willis and proximal intracranial vessels. Once believed to be specific to the Japanese population, Moyamoya occurs in worldwide distribution and has a bimodal presentation with the first peak in the first decade of life and second peak in the fifth decade.[3],[4],[5],[6]

Onset in childhood usually present as ischemic stroke, while adults typically present with intracranial haemorrhages. Moyamoya is the aetiology in approximately 6% of all childhood strokes. Its natural history includes recurrent ischemic episodes with neurological and cognitive deterioration. Our patient presented at 6 years of age with clinical features suggestive of an ischemic stroke as well as delayed milestones which could be attributed to hypoxic injury secondary to reduced cerebral blood flow. During preanesthetic assessment of a child with MMD, special attention should be paid to the neurological status, frequency of ischemic attacks, CT and/or MRI evidence of infarction, and angiographic signs of low perfusion or cerebrovascular reactivity. Frequent ischemic attacks indicate compromise to cerebral blood flow and signal high risk for ischemic complications. Hyperventilation has been identified as a factor that may induce ischemic symptoms in Moyamoya patients. Every effort should be made to avoid hyperventilation and hypocapnia intra-operatively as well as secondary to crying or inadequate analgesia. Matsushima et al. [7] found that 3.6% of the paediatric patients undergoing encephaloduroarteriosynangiosis develop cerebral infarctions as a result of hyperventilation associated with crying. Therefore, adequate premedication with smooth induction and perioperative analgesia is desirable. When securing venous access before induction of anaesthesia, pain can be minimized by applying topical anesthetic creams or by using inhalational induction and then securing iv line under deep plane of anaesthesia. Since, we anticipated a difficult intravenous iv cannulation, we decided to secure the iv line under a deep plane of sevoflurane anaesthesia with adequate premedication with midazolam. Hypoventilation leading to hypercapnia induced vasodilation diverts the blood flow to the normal cerebral vessels as the diseased vessels, which are already maximally dilated, show minimal response leading to further reduction in blood flow in the already ischemic regions. In a study on cerebral autoregulation in patients with MMD, Ogawa et al. [8] reported that the autoregulatory response to hypotension is substantially diminished in children. Hence, hypotension is to be avoided. Due to the significant mismatching of CBF and CMR02, these children are prone to developing neurologic deficits during hypotensive episodes. An adequate preoperative hydration, early use of vasopressors, lower concentration of inhalation agents to maintain the blood pressure close to preoperative values helps in maintaining the cerebral blood flows well and ensures delicate balance between the cerebral oxygen delivery and consumption. We ensured normotension by administering appropriate fluids, titrated drug use and close blood pressure monitoring. Continuous temperature monitoring and maintenance of normothermia is of utmost importance as hypothermia may induce cerebral vascular spasm while hyperthermia increases the cerebral metabolic rate which causes an increase in oxygen consumption and possible cerebral ischemia[9]. Volatile anaesthetics and nitrous oxide can cause cerebral vasodilation which may result in intraoperative cerebral steal in MMD patients. Dexmedetomidine, a selective alpha 2 agonist is believed to preserve the regional blood flow and produce an optimal microregional oxygen supply and consumption. Shown to have neuroprotective effect in various experimental studies done in animals, it can be used for intraoperative hemodynamic stability and smooth emergence in patients with Moyamoya. Agarwal et al. [10] concluded in a case series of paediatric patients that dexmedetomidine can be used as an effective adjuvant for stable hemodynamics and smooth emergence in patients with MMD undergoing EDAS procedure. Encephaloduroarteriosynangiosis (EDAS) involves the transposition of a segment of a scalp artery onto the surface of the brain, is a surgical treatment aimed at improving collateral blood flow. A metanalysis by Schnabel et al [11] revealed a lower risk for postoperative pain and the need for post-operative opioids following intraoperative dexmedetomidine in comparison with placebo or opioids in children undergoing surgery. Considering that inadequate analgesia will lead to inconsolable crying which in turn leads to hyperventilation and hypocapnia that may cause cerebral vasoconstriction and post-operative cerebral infarction, we planned caudal epidural analgesia with 0.25 % bupivacaine to ensure a pain free post-operative period and also to reduce the intraoperative consumption of anaesthetic agents. Though regional analgesia has not been extensively studied in Moyamoya patients, Kwang Suk Shim et al. [12] reported successful conduct of elective LSCS as well as postoperative pain control in a 27-year-old primipara diagnosed as Moyamoya under combined spinal epidural anaesthesia. Given their frail cerebrovascular reserve paediatric patients with Moyamoya have very low tolerance for any sort of physiologic stress. The goal of anaesthetic management in a case of MMD is to maintain the balance between cerebral metabolic oxygen consumption rate (CMRO2) and cerebral blood flow (CBF) in order to prevent any neurologic morbidity. Near infrared spectroscopy (NIRS) should be used if available for these patients, due to non availability of NIRS we didn’t use in our case. Care should be taken to evaluate cardiovascular system as association between MMD and coronary heart disease (CHD) has been constantly suggested in the literature, Though there is no ideal anaesthetic agent in MMD, the anaesthetic technique should ensure normotension, normocapnia and normothermia in the perioperative period. To our knowledge regional analgesia has not been used in the pediatric orthopedic surgeries in patients diagnosed with Moya moya.The technique of general anaesthesia supplemented with caudal epidural analgesia ensures that all the anaesthetic goals are met adequately and the precarious cerebral circulation in moya moya patient is not compromised

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Conflicts of interest

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  References Top

1.
Nomura T. Atlas of cerebral angiography. Tokyo: Sgakushoin, 1961;192-5.  Back to cited text no. 1
    
2.
Takeachi S, Kobayashi K, Tsuchida T et al. Computed tomography in Moyamoya disease. J Comput Assist Tofftogr 1982;6:24-32  Back to cited text no. 2
    
3.
Wakai K, Tamakoshi A, Ikezaki K et al. Epidemiological features of Moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg. 1997;99:S1-5.  Back to cited text no. 3
    
4.
Baba T, Houkin K, Kuroda S. Novel epidemiological features of Moyamoya disease. J Neurol Neurosurg Psychiatry. 2008;79:900-904  Back to cited text no. 4
    
5.
Han DH, Nam DH, Oh CW. Moyamoya disease in adults: characteristics of clinical presentation and outcome after encephalo-duro-arterio-synangiosis. Clin Neurol Neurosurg. 1997;99:151-5.  Back to cited text no. 5
    
6.
Han DH, Kwon OK, Byun BJ et al. Korean society for cerebrovascular disease. A co-operative study: clinical characteristics of 334 Korean patients with Moyamoya disease treated at neurosurgical institutes(1976-1994). The Korean Society for Cerebrovascular Disease. Acta Neurochir .2000;142:1263-1274  Back to cited text no. 6
    
7.
Matsushima Y, Aoyagi M, Suzuki R et al. Perioperative complications of encephalo-duro-arterio-synangiosis: prevention and treatment. Surg Neurol 1991;36:343-353.  Back to cited text no. 7
    
8.
Ogawa A, Yoshimoto T, Suzuki J, Sakurai Y. Cerebral blood fow in Moyamoya disease. Part 1: correlation with age and regional distribution.Acta Neurochir (wein) 1990;105(1-2):30-34.  Back to cited text no. 8
    
9.
Kurokawa T, Chen YJ, Tomita S, Kishikawa T, Kitamura K. cerebrovascular occlusive disease with and without the moya moya vascular network in children. Neuropediatrics 1985;16:29-32  Back to cited text no. 9
    
10.
Agrawal P, Ganeriwal V. Dexmedetomidine as anesthetic adjuvant in moyamoya patients for EDAS procedure: Our institutional experience Journal of Neuro anaesthesiology and Critical Care 2017;04(04):S88  Back to cited text no. 10
    
11.
Schnabel A, Reichl SU, Poepping DM, Kranke P, Pogatzki-Zahn EM, Zahn PK. Paediatr Anaesth. Efficacy and safety of intraoperative dexmedetomidine for acute postoperative pain in children: a meta-analysis of randomized controlled trials. Paediatr Anaesth 2013 Feb;23(2):170-9  Back to cited text no. 11
    
12.
Kwang Suk Shim, Eun Ju Kim, Ji Hyang Lee, Sang Gon Lee, Jong Seouk Ban, Byung Woo Min. Combined spinal-epidural anesthesia for cesarean section in a patient with Moyamoya disease −A case report: Korean J Anesthesiol 2010 Dec;59:S150-S153.  Back to cited text no. 12
    




 

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