Clinical Neurology and Neurosurgery 99 Suppl. 2 (1997) S110 – S113
Anesthetic management of children with Moyamoya disease Masumi Kansha a,*, Kazuo Irita a, Shosuke Takahashi a, Toshio Matsushima b a
Department of Anesthesiology and Critical Care Medicine, Fukuoka 812 -82, Japan b Neurosurgery, Kyushu Uni6ersity School of Medicine, Fukuoka 812 -82, Japan
Abstract A review of the surgical and postoperative records of 127 revascularization procedures performed on 82 children with Moyamoya disease was done to evaluate changes we made in anesthetic management in response to perioperative complications. From 1982 to 1996, out of 82 children who underwent revascularization surgery at our hospital, five developed perioperative complications. One developed circulatory instability during surgery; the cause seemed to be a depth of anesthesia insufficient for preventing surgical stress. To rectify this problem, an increased dose of fentanyl was used to improve the maintenance of anesthesia. Four patients developed cerebral infarction during the early postoperative period due, in part, to inadequate management of postoperative pain. We began to administer supplemental doses of meperidine to patients after they emerged from anesthesia to provide better control of postoperative pain. Our review confirmed the effectiveness of these measures. The data suggest that during the perioperative management of children with Moyamoya disease, close attention should be paid to balancing the patients’ anesthetic state against surgical stress and providing adequate postoperative analgesia. © 1997 Elsevier Science B.V. Keywords: Anesthetic management; Moyamoya disease in childhood
1. Introduction Moyamoya disease is a progressive occlusive cerebrovascular disease which causes recurrent ischemic attacks in children. To treat the disease several revascularization procedures are done [1 – 6]. Because patients with the disease are susceptible to cerebral infarction, careful perioperative management is required to maintain cerebral oxygen supply-demand balance. In this study, we evaluated our anesthetic management of children with Moyamoya disease in relation to perioperative complications.
2. Method At Kyushu University Hospital from January 1982 to June 1996, we performed 127 revascularization proce* Corresponding author. Tel.: +81 92 6415714; fax: + 81 92 6425722. 0303-8467/97/$17.00 © 1997 Elsevier Science B.V. All rights reserved. PII S 0 3 0 3 - 8 4 6 7 ( 9 7 ) 0 0 0 6 8 - 1
dures on 82 children with Moyamoya disease. We reviewed the perioperative records of these patients to find cases in which complications developed within a week after the operations. The data were analyzed statistically using Student’s t-test; P B 0.05 was considered statistically significant.
3. Results
3.1. Procedures Fig. 1 shows the annual distribution of the various surgical procedures performed in the 127 operations. The choice of procedures has changed over time. At present, our preferred choice is the combination of encephalo-duro-arterio-synangiosis (EDAS), enchephalo-myo-synangiosis (EMS) and encephalo-myo-arterio-synangiosis (EMAS): the three procedures are performed during a single operation.
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3.2. Perioperati6e complications Among the 82 patients, five developed perioperative complications. Table 1 summarizes the significant facts about the five cases. One patient developed circulatory instability during surgery. He was scheduled to undergo the combined procedure of EDAS, EMS and EMAS on the left side of his brain. His preoperative sedation was sufficient, and anesthetic induction with sevoflurane, N2O and vecuronium went well. Anesthesia was maintained with isoflurane, N2O and supplemental fentanyl (9.7 mg/kg). An infusion of crystalloid (6.25 ml/kg per h) was begun to maintain intravascular volume. Although such a request is unusual in our hospital, the surgeon asked to use PGE1 to maintain blood circulation through the graft. Immediately after skin incision, the patient’s heart rate and blood pressure began to fluctuate. Three hours after surgery was started, the patient’s heart rate and blood pressure began to fluctuate severely. Due to the patient’s unstable circulation, the surgery had to be terminated before the EMAS procedure could be performed. The unstable circulatory condition was probably caused by failure to balance anesthetic depth against surgical stress, hypovolemia and the use of PGE1. Four months later, the patient underwent the combined procedure of EDAS, EMS and EMAS on the right side of his brain. During this operation, the patient received a larger dose of fentanyl (13.7 mg/kg) than he had in the previous operation; the skin was infiltrated with lidocaine before the incision was made, and a larger volume of crystalloid (9.38 ml/kg per h) was infused to maintain intravascular volume. These measures resulted in a far more stable circulatory state. Four patients developed cerebral infarctions postoperatively. In three of the cases, the infarction developed on the first postoperative day; in the fourth case, it developed on the fifth postoperative day (POD). In patients 3 and 4, ischemic attacks occurred just after the patients began crying, which suggests that hypocapnea probably caused the attack. Patient 4 also had anemia (Hb 7.3 g/dl); but other three did not. We could find no other disadvantageous factors regarding to cerebral oxygen supply-demand balance in these four cases. It seems likely that pain due to insufficient post-
Fig. 1. Annual changes in surgical procedures for children with Moyamoya disease. Symbols: Light-shaded, EDAS; open, EMAS or EMS; dark shaded, EDAS with either EMAS or EMS; filled, EDAS with EMAS and EMS; horizontal, STA – MCA; crossed, STA–MCA with either EMS or EMAS, or with both EDAS and EMS. The circled numbers indicate the five cases with perioperative complications. The following abbreviations are used: EDAS, encephalo-duroarterio-synangiosis; EMS, enchephalo-myo-synangiosis; EMAS, encephalo-myo-arterio-synangiosis; STA-MCA, superficial temporal artery-middle cerebral artery anastomosis.
operative analgesia contributed to the occurrence of the infarctions.
3.3. Changes in perioperati6e care We have made two changes in our perioperative management of children with Moyamoya disease. The first is in our choice of anesthetics and the dosages we use; the second is in our postoperative treatment of pain. Fig. 2 shows the annual changes in our choice of anesthetics to maintain anesthesia in the 127 revascularization procedures. We used inhalation anesthetics, and our choice of inhalation anesthetics has changed over time owing to continuing technological advances. At present, we use isoflurane supplemented with fentanyl.
Table 1 Cases with perioperative complications
1 2 3 4 5
Age
Sex
Operation
Complication
Onset
3 9 9 7 4
F F F F M
Lt.EMS Rt.EDAS, EMS Rt.EDAS, EMAS Rt.EDAS, EMS Lt. EDAS, EMS
Small cerebral infarction Cerebral infarction Small cerebral infarction Small cerebral infarction Circulatory instability
5 POD 1 POD 1 POD 1 POD Intraoperative
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cause not all children complained of pain following surgery just after awakening from general anesthesia, and because we were unable to find a possible explanation for the first two ischemic attacks. Afterwards, we began to control postoperative pain by administering meperidine intravenously (0.9790.59 mg/kg: mean9 S.D.; n= 48) to patients just after they emerged from general anesthesia.
4. Discussion
Fig. 2. Annual changes in choice of anesthetics for children with Moyamoya disease. Symbols: open, halothane; crossed, enflurane; light shaded, isoflurane; dark-shaded horizontal, isoflurane supplemented with fentanyl. The circled numbers indicate the five cases with perioperative complications.
After the operation on the patient who experienced circulatory instability, the mean dose of supplemental fentanyl was increased from 5.7 9 2.6 to 9.6 94.9 mg/ kg (mean9 S.D.; n= 10 and 15, respectively) (Fig. 3). Before the third and fourth cases of postoperative cerebral ischemia occurred, we only occasionally used sedatives and analgesics at the end of anesthesia be-
Fig. 3. Doses of supplemental fentanyl. The mean doses of supplemental fentanyl used in the operations for children with Moyamoya disease before and after experiencing Case 5 were 5.7 9 2.6 and 9.69 4.9 mg/kg (mean 9S.D.), respectively.
Recently, when treating children with Moyamoya disease, a combination of EDAS, EMS and EMAS has been performed in cases where direct anastomosis is difficult [7,8], and the complicated techniques needed to perform these procedures have lengthened the time required for a given operation. We generally use inhalation anesthetics because they increase the cerebral blood flow/cerebral metabolic rate ratio. When performing surgery on children with Moyamoya disease, we now use isoflurane and fentanyl. Fentanyl is a strong analgesic which makes it possible to avoid circulatory depression and possible cerebral steal phenomenon, both of which have been reported to be induced by high concentrations of isoflurane [9]. We have increased the mean dose of supplemental fentanyl to 9.694.9 mg/kg. This dose creates adequate anesthetic depth and contributes to circulatory stability without depressing respiratory function. The circumstances in which the four cases of cerebral infarction occurred suggest that management of postoperative pain is important. Because two of the attacks may have been triggered by hypocapnea associated with crying due to pain, we use meperidine, diazepam and indometacin or diclofenac sodium to keep all patients adequately sedated and pain free during the early postoperative period. It is probable that the ischemic attack in patient 4 was also due to anemia. However, for children with Moyamoya disease, it is difficult to determine the minimal acceptable level of hemoglobin because their cerebrovascular metabolism has been reported to vary depending on the patients, the regions of the brain in which vascular occlusion occurs, and the stage of the disease [10]. At present, we maintain their hemoglobin level at 10 g/dl and over. In the perioperative management of children with Moyamoya disease, in order to maintain cerebral blood flow and cerebral oxygenation, the following conditions should be avoided: hypoxemia; hypocapnea and hypercapnea during controlled ventilation; cerebral vasoconstriction; hypotension; hypovolemia; anemia and an increase in blood viscosity. Measures to decrease the cerebral metabolic rate for oxygen and to avoid its increase should be taken, and attention to prevent hyperglycemia, which has been reported to exacerbate
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ischemic damage [11 – 14], should be paid. Our study suggests in addition that the maintenance of an adequate depth of anesthesia and proper postoperative analgesia are also necessary.
[7]
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