Journal of Neurosurgical Anesthesiol Vol.10,No.I,pp aed] © 1998 Lippincott-Raven Publishers, Philadelphia Postoperative Neurological Deterioration Following the Revascularization Surgery in Children With Moyamoya Disease Takanori Sakamoto, Masahiko Kawaguchi, Koukichi Kurehara, Katsuyasu Kitaguchi, Hitoshi Furuya, and Jun Karasawa, M.D.* Department of Anesthesiology, Nara Medical University, Nara, Japan, and *Department of Neurosurgery, Osaka Neurological Institute, Osaka, Japan Summary: We describe four children with moyamoya disease who developed neuro- logic deterioration following revascularization surgery. In all cases, anesthesia was smoothly induced and the intraoperative course was uneventful. Emergence from anes- thesia was prompt and no new neurological deficit was observed. However, the children suffered strokes on 2, 4, 5, and 10 days, postoperatively, respectively. Dehydration and crying were thought to be closely associated with the stroke in each case. This report suggests that attention should be paid during entire the perioperative period to avoid stroke in patients with moyamoya disease. Key Words: Moyamoya disease—Compli- cation—Stroke. Moyamoya disease is a progressive cerebrovascular occlusive disease characterized by angiographic findings of intracranial carotid artery stenosis and occlusion, and a fine network of vessels at the base of the brain (1,2). In the juvenile type, the characteristic symptoms are transient ischemic attacks (TIAs), which cause motor weakness of the limbs that finally reach to the permanent deficits. Surgical revascularization for this disease began in the 1970s (1) and the effectiveness of this procedure is com- monly recognized (3). However, complications associated with the surgery have also been reported (4). Stroke is undoubtedly the most detrimental complication. Two cases of intraoperative stroke were previously reported by Sumikawa et al. (5). Those patients experienced delayed emergence from anesthesia and developed new neurologic Address correspondence and reprint requests to Dr. T. Sakamoto, Department of Anesthesiology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634, Japan, 37 deficit immediately following surgery, most likely due to intraoperative hypocapnia (5). Since then, prevention of stroke has been the major anesthetic challenge for patients with this disease, and there are several reports of success- ful anesthetic management(6—10). In those reports, all patients experienced prompt emergence from anesthesia and suffered no neurologic deterioration during the early post- operative period. However, stroke can occur at anytime dur- ing entire perioperative period in patients with this disease, in particular during the postoperative course. The total inci- dence of stroke during the postoperative period has been reported as four of 81 (4.9%) patients (4), 16 of 112( 14.3%) patients (11), and one of 22(4.5%) patients (7). As mentioned by Soriano et al. (7), it is difficult to deter- mine the real cause of perioperative stroke, since the effects of anesthesia, surgery, and perioperative stress on cerebral circulation are complex. In this report, we describe four patients with childhood moyamoya disease who developed postoperative stroke after reconstructive surgery and dis- cuss the probable causes—dehydration and bitterly cry- ing—of these strokes. 38 T. SAKAMOTO ET AL. CASE REPORT Case 1 The patient was a 7-year-old girl weighing 22 kg. She was diagnosed as having moyamoya disease with cerebral angiography. Omental transplantation bilaterally to the occipital regions was scheduled to repair cerebral blood circulation in the territory of the posterior cerebral artery. Anesthesia was induced with thiopental, and maintained with enflurane and supplemental pentazocine. The trachea was intubated and ventilation was maintained to adjust Paco, to normocapnia. Arterial blood pressure was main- tained at the preoperative level. Since urine output gradu- ally decreased at initiation of craniotomy after harvesting of omentum, 200 ml of mannitol and 4 mg of furosemide were administered. Volumes of intraoperative infusion, bleeding, and urine output were 2,800 ml, 425 g, and 950 ml, respectively. Anesthesia time was 10 hours 30 min- utes. The child awoke promptly and no neurologic deteri- oration was observed at emergence from anesthesia. Post- operatively, she complained of headache, abdominal pain, and discomfort, and frequently vomited. Although 1,000 ml of fluid volume was infused each day, she had no appetite and her oral intake was almost nil. In the early morning of postoperative day (POD) 2, her parents found that she did not respond to their voices, and examination disclosed left hemiplegia. Computed tomography (CT) per- formed on the same day demonstrated a new low density area (LDA) in the right parietal region (Fig.1). Motor func- tion gradually improved, but mild hemiparesis persisted. Journal of Neurosurgical Anesthesiology, Vol. 10, No. 1, 1998 Case 2 A 6-year-girl suffered TIAs 1 month before admission to our hospital, where she was diagnosed with moyamoya disease. A left superficial temporary artery (STA)-middle cerebral artery (MCA) anastomosis with (EMS) was sched- uled. Anesthesia was induced with thiopental and main- tained with fentanyl and flunitrazepam. The trachea was intubated and ventilation was maintained to adjust the Paco, at =45 mm Hg. Arterial blood pressure was main- tained within her preoperative level. The intraoperative course was uneventful and she awoke smoothly from anes- thesia. No neurologic deterioration was observed at emer gence from anesthesia. Postoperatively, she often cried vigorously for fear of clinical procedures. She suffered postoperative pain and sore throat, then had poor appetite and decreased urine output. On POD 3, left facial palsy began to occur occasionally. On POD 5, she developed left hemiparesis, motor aphasia, and facial palsy. CT scan on POD 6 demonstrated a new LDA in the right temporal region (Fig.2). By the time of discharge from our hospi- tal, the motor aphasia and facial palsy had improved, but mild monoparesis of the left upper extremity persisted. Case 3 The patient, a 5-year-old girl had her first onset of TIA at 4 years of age. At that time, she was diagnosed with moyamoya disease and elected to undergo a right STA-MCA anastomosis with EMS. This perioperative course was uneventful. At 5 years of age, a left STA-MCA anastomosis with EMS was scheduled. Anesthesia was FIG. 1. CT scans in Case no. [. A: Imme- diately following omental transplantation to the bilateral occipital regions. B: On POD 5, a new low density area (LDA) was observed in the right parietal region. POSTOPERATIVE STROKE IN CHILDREN WITH MOYAMOYA DISEASE 39 FIG. 2. CT scans in Case no. 2. A: Immediately following left STA-MCA anastomosis with EMS. B: On POD 6, a new LDA appeared in the right temporal region. C: At 4 months following surgery, the LDA was definitive and mild monoparesis of left upper extremity persisted. induced with thiopental and maintained with 1% enflu- rane. The trachea was intubated and ventilation was main- tained to adjust PaCo, at 36-43 mm Hg. Arterial blood pressure was maintained within her preoperative level. She awoke promptly from anesthesia. No postoperative neu- tologic deterioration was observed at that time. The post- operative course was favorable. However, she frequently complained of pharyngalgia. On POD 8, edema and ery- thema in the left pharyngia was observed and she was diag- nosed with pharyngitis. The pain was so severe that she could not obtain sufficient nutrition and had an increase in body temperature to 39°C. At midnight of POD 10, she arose suddenly from her bed and began to cry vigorously. At that time, she was observed to move all her extremities fully as if climbing up a tree. She subsequently lost con- sciousness for 10 minutes, with conjugate deviation of the eyes to the left. After regaining consciousness, involuntary movement, resembling chorea, of the left extremities devel- oped. This movement promptly ceased and no motor weak- ness of the extremities was observed. CT on the following day demonstrated a new LDA in the right watershed region between the anterior and middle cerebral arteries (Fig.3). Similar involuntary movements occurred on POD 11, but disappeared after administration of an anticonvulsant drug. Case 4 This moyamoya patient was an 8-year-girl who had undergone an uneventful right STA-MCA anastomosis with EMS 3 months before admission. Subsequently, a left STA-MCA anastomosis with EMS was scheduled. Anes- thesia was induced and maintained with fentanyl and sevoflurane/oxygen/nitrous oxide. The trachea was intu- bated and ventilation was maintained to adjust normocap- nia. The intraoperative course was uneventful. Postoper- atively, she was ill-humored and exhibited loss of appetite as result of an increase in body temperature. Despite fre- quent use of antiinflammatory drugs, body temperature remained >38°C for several days. On POD 4, she devel- oped facial nerve palsy and dysarthria, and a new LDA in the left thalamus was confirmed by CT scan. DISCUSSION We described four childhood moyamoya patients who developed stroke during the postoperative period. These cases illustrate the close association of dehydration and/or crying with occurrence of stroke. Dehydration is generally thought to be detrimental to maintenance of cerebral blood flow (CBF) in patients with occlusive cerebrovascular disease (12), since dehydration can induce an increase in hematocrit and the subsequent rise in blood viscosity may cause a decrease in regional CBE. Theoretically, a hematocrit of 30-34% yields the optimal combination of viscosity and oxygen-carrying capacity (13). In addition to the rise in viscosity, a decrease in systemic volume associated with dehydration can reduce cerebral perfusion pressure as result of a decrease in car- diac output and arterial blood pressure. Although there Journal of Neurosurgical Anesthesiology, Vol. 10, No. 1, 1998 40 T. SAKAMOTO ET AL. have been few reports that demonstrate the clinical evi- dence that dehydration can provoke the stroke, dehydra- tion undoubtedly played a major role in the development of stroke in our cases. In Case no. 1, the patient vomited frequently for several days and could not obtain sufficient nutrition because of abdominal discomfort. Therefore, syS- temic volume might be decreased to various degrees. Addi- tionally, surgical technique might have influenced water balance in that case. Since omental transplantation requires both craniotomy and laparotomy, loss of blood volume and infusion requirements are necessarily large, leading to difficulty in maintaining proper systemic blood volume and hemodynamics. In Case nos. 3 and 4, an elevation in body temperature was associated with insufficient oral intake. Increase in fever was recognized as one of precipitating factors for TIA in a patient with moyamoya disease (14). Fever not only increases cerebral oxygen consumption (15) but also decreases intravascular volume under certain conditions. Intravascular volume can be markedly altered by various factors during the perioperative period. Therefore, a decrease in systemic volume should be carefully avoided in order to maintain proper cerebral perfusion pressure. Crying is known to be the major precipitating factor for TIAs in children with moyamoya disease. In a study of the etiology of ischemic symptoms in this disease, it was found that ischemic attacks were induced by crying in 38 of 58 (65.6%) cases studied (16). This is due to the fact that cry- ing is inevitably associated with hyperventilation, which results in hypocapnia, and that cerebral vasoconstrictive reactivity to hypocapnia is well maintained in patients with Journal of Neurosurgical Anesthesiology, Vol. 10, No. 1, 1998 FIG. 3. CT scan in Case no. 3. A; On admission, this patient underwent left STA-MCA anastomosis with EMS. B: On postoperative day 11, a new LDA was observed in the right watershed region between the anterior and middle cerebral arteries. moyamoya disease. Indeed, Tagawa et al. (17) observed that children with moyamoya disease cried and developed transient hemiparesis at the end of regional CBF mea- surements. In their study, regional CBF decreased signif- icantly as the mean value of Paco, decreased from 39 to 29 mm Hg. Therefore, rigorous crying can be a direct trig- ger for permanent neurologic deficit in association with an unstable hemodynamic and/or dehydrated state during the postoperative period. Nevertheless, the risk associated with crying has not been mentioned in previous reports on anesthetic management of this disease. We believe that controlling crying is an important anes- thetic challenge to prevent perioperative stroke and should be employed in the preoperative anesthetic management of children with moyamoya disease. Children are apt to cry or become excited as result of being separated from their parents, feelings of anxiety, or fear of pain. There- ore, the anesthesiologist should pay sufficient attention to the psychological state of the patient. Appropriate effort should be made to reduce patients’ anxiety and eliminate painful stimuli. If necessary, proper sedative drugs should be administered throughout the perioperative period. Besides the possible causes stroke discussed earlier in this report, other etiologies, such as physiological changes affected by surgery, should not be ignored . For exam- ple, three of four patients (Case nos. 1-3) developed cere- bral infarction in the contralateral or other side. This sug- gests that CBF was redistributed following surgical revascularization, thus leading to deterioration of the oxy- gen balance previously established among various cere- bral regions. POSTOPERATIVE STROKE IN CHILDREN WITH MOYAMOYA DISEASE 41 In summary, four children with moyamoya disease developed postoperative stroke most probably due to dehy- dration and/or crying. 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