Significance of Cerebral Blood Flow Analysis in the Acute Stage after Revascularization Surgery for Moyamoya Disease

Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by steno-occlusive changes at the terminal portion of the internal carotid artery, either bilaterally or unilaterally, and an abnormal vascular network at the base of the brain. Surgical revascularization such as extracranial-intracranial (EC-IC) bypass is the preferred procedure for moyamoya disease. Despite the favorable long-term outcome, cerebral infarction and hyperperfusion syndrome are potential complications of this procedure, which can lead to neurological deterioration in the acute stage. In light of the similar clinical presentations between perioperative ischemia and hyperperfusion, it is essential to attempt a prompt cerebral blood flow (CBF) measurement in the acute stage after EC-IC bypass for moyamoya disease to differentiate these distinct pathologies, because the management of cerebral ischemia and hyperperfusion is contradictory to each other. Routine CBF analysis by single-photon emission computed tomography and/or magnetic resonance imaging not only facilitated a safer perioperative management but also provided important information about dynamic pathology of the hemodynamic conversion in the acute stage after revascularization surgery for moyamoya disease. We represent the current status of CBF analysis during the perioperative period of revascularization surgery for moyamoya disease, and sought to discuss its significance and efficacy to avoid surgical complications.     

Diagnostic value of perfusion-weighted MRI for evaluating postoperative alteration of cerebral hemodynamics following STA-MCA anastomosis in patients with moyamoya disease

OBJECTIVE: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). But little is known about the changes of CBF and its effect on neurological status during the acute stage after direct bypass for moyamoya disease. METHODS AND RESULTS: Perfusion-weighted magnetic resonance imaging (PWI) by the SE-EPI method was performed 4 and 10 days after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 11 sides of 9 patients (male:female = 2:7, 24-61 years old) with moyamoya disease. All patients underwent single-photon emission computed tomography (SPECT) 1 and 7 days after surgery. The time-to-peak (TTP) by PWI showed focal decrease at the site of the anastomosis 4 days after STA-MCA anastomosis compared to preoperative TTP, and was further decreased in larger territory 10 days after surgery in all patients. Postoperative cerebral blood volume (CBV) by PWI showed significant increase compared to preoperative CBV in four patients who suffered symptomatic cerebral hyperperfusion after surgery. Postoperative SPECT revealed apparent increase of CBF in all patients and the patency of the STA-MCA bypass was confirmed by MRA. All patients were discharged without neurological deterioration compared to preoperative neurological status, and none of them suffered ischemic attack after surgery. CONCLUSION: Postoperative decrease in TTP by PWI could be a sensitive parameter for the normalization of CBF after STA-MCA anastomosis in patients with moyamoya disease. Postoperative increase in CBV may suggest cerebral hyperperfusion and could be the optimal predictor for hyperperfusion syndrome after STA-MCA anastomosis in patients with moyamoya disease.     

Asymptomatic intracerebral hemorrhage under strict blood pressure control due to postoperative cerebral hyperperfusion in a patient with moyamoya disease

We report a case of moyamoya disease manifesting as asymptomatic intracerebral hemorrhage due to postoperative cerebral hyperperfusion, despite the prophylactic intensive blood pressure control. This 35-year-old man initially suffered from right upper quadrantanopsia and was found to have cerebral infarction in the left occipital lobe due to moyamoya disease. He also manifested preoperatively a small intracerebral hemorrhage at the left caudate nucleus. The left cerebral hemisphere showed apparent hemodynamic compromise, thus he underwent left superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis with indirect pial synangiosis. He was subjected to prophylactic strict blood pressure control postoperatively to avoid hyperperfusion syndrome. The N-isopropyl-p-¹²³I-Iodoamphetamine SPECT (¹²³I-IMP-SPECT) 1 day after surgery showed intense increase in cerebral blood flow (CBF) at the site of the anastomosis, and further blood pressure lowering was attempted. He did not suffer from neurologic deterioration during the postoperative period, while computed tomography (CT) 7 days after surgery revealed asymptomatic intracerebral hematoma (ICH) at the subcortex under the site of the anastomosis. Cerebral hyperperfusion is a potential complication of revascularization surgery for moyamoya disease. Accurate diagnosis and proper management of hyperperfusion are essential to avoid deleterious neurologic event due to hyperperfusion.     

Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease

BACKGROUND: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving CBF, but little is known about the change in CBF and its effect on neurologic status during the acute stage after revascularization. METHODS: 123I-IMP-SPECT was performed 1 and 7 days after STA-MCA anastomosis on 34 sides of 27 consecutive patients with adult-onset moyamoya disease (6 men, 21 women; 22-62 years old). The follow-up period ranged from 5 to 28 months (mean, 17.6 months). RESULTS: Thirteen patients (13 sides, 38.2%) suffered temporary neurologic deterioration due to hyperperfusion several days after surgery, which was sustained for several days (7.4 days in average). Postoperative magnetic resonance imaging/angiography showed the STA as a higher intensity signal than the preoperative finding without ischemic changes in all 13 patients. Postoperative SPECT revealed focal intense increase in CBF at the sites of anastomosis in all 13 patients. Eleven patients (32.4%) had transient focal neurologic deficit mimicking ischemic attack. Two patients (5.9%) had cerebral hyperperfusion syndrome associated with subarachnoid hemorrhage extending to the ipsilateral sylvian cistern. Symptoms were relieved by intensive blood pressure control, and no patients had permanent neurologic deficit or delayed neurologic deterioration during the follow-up period. CONCLUSIONS: Surgical revascularization including STA-MCA anastomosis is a safe and effective treatment for moyamoya disease, although temporary neurologic deterioration due to hyperperfusion could occur at a substantial rate. Routine CBF measurement is recommended for accurate diagnosis of postoperative hyperperfusion in moyamoya disease because its treatment is contradictory to that for ischemia.     

Temporal change of 3-T magnetic resonance imaging/angiography during symptomatic cerebral hyperperfusion following superficial temporal artery-middle cerebral artery anastomosis in a patient with adult-onset moyamoya disease

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is a safe and effective treatment for moyamoya disease, although recent evidence suggests its substantial risk for symptomatic cerebral hyperperfusion. The diagnostic value of single-photon emission computed tomography (SPECT) for postoperative hyperperfusion in moyamoya patients is well established, but that of magnetic resonance (MR) imaging/angiography is undetermined. A 22-year-old woman with hemorrhagic-onset moyamoya disease underwent STA-MCA anastomosis on the right hemisphere, but she suffered from transient left hemiparesis and facial paresis owing to cerebral hyperperfusion from 3 to 11 days after surgery as delineated by SPECT. The time-sequential 3-T MR angiography revealed intense high signal of donor STA and dilated branches of MCA around the site of the anastomosis. These findings were most prominent at 8 days after surgery, when her neurologic signs were most apparent. Intensive blood pressure control relieved her symptom and she was discharged without neurologic deficit. MR findings normalized 3 months later. The characteristic findings of 3-T MR angiography, which was not evident by 1.5-T MR angiography in the previous studies, may reflect intrinsic pathology of postoperative cerebral hyperperfusion. It could be a useful diagnostic tool after revascularization surgery for moyamoya disease.     

Temporal change of 3-T magnetic resonance imaging/angiography during symptomatic cerebral hyperperfusion following superficial temporal artery–middle cerebral artery anastomosis in a patient with adult-onset moyamoya disease

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is a safe and effective treatment for moyamoya disease, although recent evidence suggests its substantial risk for symptomatic cerebral hyperperfusion. The diagnostic value of single-photon emission computed tomography (SPECT) for postoperative hyperperfusion in moyamoya patients is well established, but that of magnetic resonance (MR) imaging/angiography is undetermined. A 22-year-old woman with hemorrhagic-onset moyamoya disease underwent STA-MCA anastomosis on the right hemisphere, but she suffered from transient left hemiparesis and facial paresis owing to cerebral hyperperfusion from 3 to 11 days after surgery as delineated by SPECT. The time-sequential 3-T MR angiography revealed intense high signal of donor STA and dilated branches of MCA around the site of the anastomosis. These findings were most prominent at 8 days after surgery, when her neurologic signs were most apparent. Intensive blood pressure control relieved her symptom and she was discharged without neurologic deficit. MR findings normalized 3 months later. The characteristic findings of 3-T MR angiography, which was not evident by 1.5-T MR angiography in the previous studies, may reflect intrinsic pathology of postoperative cerebral hyperperfusion. It could be a useful diagnostic tool after revascularization surgery for moyamoya disease.     

Intraoperative brain surface blood flow monitoring using IRIS V thermographic imaging system in patients with Moyamoya disease

Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving CBF. But little is known about the changes of intraoperative cerebral hemodynamics and its effect on postoperative neurological status including symptomatic cerebral hyperperfusion. To address this issue, we applied a novel infrared camera system (IRIS-V thermographic system) for real-time, visual monitoring of surface CBF during surgery in patients with moyamoya disease. Seven patients (8 sides, male:female= 3:4, 7-62 years old) with moyamoya disease were included in the study. After STA-MCA anastomosis, STA were occluded transiently and recanalized, and whole sequence was recorded by IRIS-V system. Correlation between clinical, radiological findings and infrared imaging were investigated. Patency of bypass was confirmed by this camera during surgery in all cases. The intraoperative imaging patterns were divided into two groups. Group A: Change of brain surface color (++) (3 cases). Group B: Change of brain surface color (-) (4 cases). Transient symptomatic hyperperfusion occurred in all patients in Group A, whereas all patients in Group B showed non-symptomatic transient focal hyperperfusion on SPECT. No patient suffered permanent neurological deterioration compared to preoperative status. Characteristic pattern of the intraoperative cerebral hemodynamics as delineated by IRIS-V could be the optimal predictor for postoperative transient symptomatic hyperperfusion after direct bypass in patients with moyamoya disease.     

Focal hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease. Case report

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is a standard surgical therapeutic option in patients with moyamoya disease. Most patients experience improvement in their clinical symptoms immediately after surgery. The authors report on the case of a 39-year-old man with moyamoya disease who suffered from temporary and frequent neurological deterioration after undergoing a left STA-MCA anastomosis. Hemodilution and hypervolemia therapies did not improve his course. Technetium-99m hexamethylpropyleneamine oxime single-photon emission tomography scans demonstrated focal intense accumulation of the tracer in the frontal operculum on the side of the surgery. Although diffusion-weighted magnetic resonance (MR) imaging demonstrated no abnormalities except for the postoperative change, perfusion-weighted MR images and MR digital subtraction angiography revealed focal hyperperfusion in the left frontal operculum where the cerebral perfusion reserve was severely disturbed preoperatively. This evidence strongly supports the notion that focal hyperperfusion observed after STA-MCA anastomosis could occur in the poor perfusion reserve area preoperatively and could cause temporary neurological deterioration.     

Postoperative temporary neurological deficits in adults with moyamoya disease

BACKGROUND: Several authors have reported temporary neurologic deterioration after revascularization surgery in some patients with moyamoya disease. The present study examined the incidence and mechanisms of PONDs in adult patients with moyamoya disease. METHODS: Postoperative neurological deficits were retrospectively evaluated 1 month or less postoperatively on 32 hemispheric sides of 17 symptomatic adult patients with moyamoya disease treated surgically with direct and/or indirect revascularization. RESULTS: Various PONDs were observed in 9 sides (28%) from 7 patients 1 month or less after surgery. Symptoms were recognized in 7 (39%) of 18 sides with ischemic onset, and 2 (14%) of 14 sides with hemorrhagic onset. Postoperative neurological deficits were usually observed 1 week or less after surgery, and resolved within 2 weeks. Postoperative neurological deficits were divided into 3 groups based on duration of symptoms: single transient neurologic deficits in 3 sides; repeated transient neurologic deficits in 3 sides; and continuous neurologic deficits in 3 sides. Radiologic examinations demonstrated no ischemic changes in any patients, and subsequent focal hyperemia after surgery on 3 sides. Postoperative neurological deficits occurred more frequently in younger patients or those with poor vascular response before surgery. CONCLUSIONS: Postoperative neurological deficits frequently occur in patients with moyamoya disease, but are temporary. These deficits appear to result from focal hyperperfusion after surgery, rather than from ischemic changes.     

Transient crossed cerebellar diaschisis due to cerebral hyperperfusion following surgical revascularization for moyamoya disease: case report

Crossed cerebellar diaschisis (CCD) often occurs after ischemic or hemorrhagic stroke that damages the cortico-ponto-cerebellar pathway. However, CCD due to cerebral hyperperfusion following cerebrovascular reconstruction is rare. A 61-year-old woman presented with transient CCD due to cerebral hyperperfusion following bypass surgery for adult moyamoya disease. She developed transient weakness of the right extremities and was diagnosed with moyamoya disease. First, she underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis with indirect synangiosis on the left. Postoperative course was uneventful. Subsequently, she underwent STA-MCA anastomosis with indirect synangiosis on the right. She complained of mild headache on the right, and single photon emission computed tomography (SPECT) performed on the 7th postoperative day demonstrated hyperperfusion in the right frontal and temporal lobes associated with hypoperfusion in the left cerebellum. Magnetic resonance (MR) imaging demonstrated no new lesions and MR angiography showed patent STA-MCA bypass. Subsequent SPECT showed disappearance of both hyperperfusion and CCD. This case strongly suggests that cerebral hyperperfusion after bypass surgery for moyamoya disease may cause transient CCD. Although the clinical significance is still obscure, this phenomenon indicates the cortico-ponto-cerebellar pathway is interrupted due to hyperperfusion, suggesting the development of hyperperfusion syndrome. Careful observation of cerebral hemodynamics after bypass surgery is warranted to avoid hyperperfusion-related complications.     

Seizure following superficial temporal-middle cerebral artery anastomosis in patients with moyamoya disease: possible contribution of postoperative cerebral hyperperfusion

Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). Seizure is known as a rare complication after revascularization for moyamoya disease, although its underlying mechanism is undetermined. We investigated the relationship between seizure attack and postoperative alteration in CBF in patients with moyamoya disease. CBF was routinely measured by N-isopropyl-p-[123I] iodpamphetamine (123I-IMP-SPECT) 1 and 7 days after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 64 sides of the 44 consecutive patients (male:female = 13:31, 2-62 years old) with moyamoya disease. Three patients (male:female = 1:2, 40-55 years old) suffered from seizure attack at 1 to 10 days after surgery. Two of the three patients manifested as seizures at 8-10 days after surgery and presented transient neurologic deteriolation before seizure. Postoperative SPECT revealed significant increase in CBF at the sites of the anastomosis in all three patients. Postoperative magnetic resonance imaging showed no ischemic changes, and magnetic resonance angiography showed the apparently patent STA-MCA anastomosis as thick high signal intensity sign in all three patients. The anatomical location and the temporal profile of hyperperfusion were completely in accordance with the focus of seizure attack. Intensive blood pressure control and the use of antiepileptic agents were instituted. The neurologic deficits were resolved and no seizure attack recurred in three patients. Seizure following STA-MCA anastomosis can be caused by hyperperfusion in patients with moyamoya disease. When seizure attacks occur, routine CBF measurement is recommended to differentiate hyperperfusion and ischemia, since the treatments for these conditions are contradictory.     

Cerebral ischemia owing to compression of the brain by swollen temporal muscle used for encephalo-myo-synangiosis in moyamoya disease

Compression of the brain by swollen temporal muscle used for indirect pial synangiosis is a rare complication after the revascularization surgery for moyamoya disease, and its mechanism and clinical presentation are undetermined. A 26-year-old woman, who had been suffering transient ischemic attack (TIA), underwent superficial temporal artery-middle cerebral artery anastomosis with encephalo-myo-synangiosis (EMS) on the affected hemisphere. The ¹²³I-IMP-SPECT 1 day after surgery demonstrated an improvement of cerebral blood flow (CBF) on the operated hemisphere. Two days later, however, she suffered fluctuating aphasia when computed tomography scan revealed marked swelling of the temporal muscle used for EMS. The ¹²³I-IMP-SPECT 4 days after surgery showed significant decrease in CBF by the compression of the brain. Then, we performed revision of EMS. The base of the temporal muscle was markedly compressed by the edge of the free bone flap, which resulted in swelling of the entire temporal muscle used for EMS. We drilled out the edge of the free bone flap for decompression. Her aphasia disappeared postoperatively, and CBF normalized 7 days after the initial surgery. Her TIA disappeared, and there was no deterioration during the follow-up period. The STA-MCA bypass has been patent since the initial surgery. Surgical revascularization including EMS has a substantial risk for cerebral ischemia owing to compression of the brain by temporal muscle swelling. Relative wide bone window for temporal muscle insertion is necessary to avoid this rare complication. Once the flow compromise is confirmed, we recommend early decompression by the revision of EMS.     

Bypass surgery for moyamoya disease: concept and essence of sugical techniques

This review describes the basic concepts of surgical revascularization for moyamoya disease, including direct and indirect bypass surgery. Direct bypass surgery can improve cerebral hemodynamics and reduce further ischemic events immediately after surgery, but may be technically challenging in some pediatric patients. Indirect bypass surgery is simple and has widely been used. However, its beneficial effects can be achieved 3 to 4 months after surgery, and surgical design is quite important to determine the extent of surgical collateral pathways. Combined bypass procedure, especially superficial temporal artery (STA) to middle cerebral artery anastomosis and indirect bypass, encephalo-duro-myo-arterio-pericranial synangiosis, is a safe and effective option to improve the short- and long-term outcome in patients with moyamoya disease. Alternative techniques are also described for specific cases with profound cerebral ischemia in the anterior cerebral artery or posterior cerebral artery territory. Special techniques to safely complete bypass surgery and avoid perioperative complications are presented, including methods to prevent delayed wound healing, to avoid facial nerve palsy after surgery, and to preserve the STA and middle meningeal artery during skin incision and craniotomy. Finally, the importance of careful management of patients is emphasized to reduce the incidence of perioperative complications, including ischemic stroke and hyperperfusion syndrome.     

Clinical outcome of standard extracranial-intracranial bypass surgery in patients with symptomatic atherosclerotic occlusion of the internal carotid artery

Summary Objective. In this retrospective study we wanted to determine the role of cerebral revascularization in patients with symptomatic occlusive cerebrovascular disease. Special emphasis was put on subsequent cerebrovascular events, benefit in neurological functioning and bypass patency, as evaluated during the follow-up period.Methods. A total of 73 superficial temporal artery to the middle cerebral artery bypasses were performed on 67 patients from 1986–2000. All patients exhibited a symptomatic occlusion of the internal carotid artery verified by angiography. All patients in our group were refractory to medical treatment. 65 patients (69 bypasses) with a mean age of 61 years (range: 38–79 years) were followed up over an average time of 44 months (range: 1.5–150 months).Results. The peri-operative morbidity rate was 3% with no mortality. 55 patients (85%) had no further cerebrovascular events after surgery, and only 7 (11%) patients experienced another cerebrovascular event. 57 (88%) patients showed an improvement of symptoms after surgery and only 1 patient fared worse during the follow-up peroid due to a stroke he suffered two years after bypass surgery. 90% of all bypasses remained patent during the follow-up.Conclusions. Although bypass surgery for occlusive cerebrovascular disease is still controversial, our retrospective study suggests both an improvement of symptoms and signs and a risk-reduction for future cerebrovascular events after surgery.     

Efficacy of revascularization surgery for moyamoya syndrome associated with Graves' disease

Appropriate management of moyamoya syndrome associated with Graves' disease is undetermined because of the rarity of this combination. Patients tend to present with cerebrovascular events such as transient ischemic attack (TIA) in a thyrotoxic state, which is relieved by proper antithyroid therapy. Four patients with moyamoya syndrome associated with Graves' disease were successfully treated with revascularization surgery on 5 hemispheres among 58 consecutive patients (2-62 years old, mean 34.4 years) with moyamoya disease in 80 hemispheres treated from March 2004 to May 2007. Three patients presented with TIA, and one patient presented with intracerebral hemorrhage. Three patients were thyrotoxic at the onset of the cerebrovascular events. All patients underwent revascularization surgery after normalization of thyroid function. Euthyroid state was strictly maintained perioperatively. One patient developed symptomatic cerebral hyperperfusion, which was resolved by blood pressure control. Postoperative courses of the other patients were uneventful, and all 4 patients have remained neurologically stable after discharge. Cerebrovascular reconstruction surgery is a successful treatment option for moyamoya syndrome associated with Graves' disease. Timing of surgery during the euthyroid state and perioperative management considering the thyroid function and the cerebral hemodynamic change are the keys to successful surgical treatment.     

Cerebral revascularization for moyamoya disease in children

Ninety-four patients with moyamoya disease (56 in the pediatric age group and 38 adults) were treated by revascularization surgery in the past 21 years (1979--2000). Combined surgery of the superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis and indirect revascularization of encaphalo-duro-arterio-myo-synangiosis (EDAMS) was performed. Ischemic attacks disappeared in most patients within 1 year (mean) after surgery in pediatric cases. No morbidity or mortality was experienced in the pediatric group. Some children with cerebral infarction before the surgery, however, had mild mental retardation even after the surgery. Early diagnosis and proper prophylactic surgical treatment is indispensable for pediatric patients with moyamoya. MR angiography is an important diagnostic modality for the screening and longitudinal follow-up of moyamoya disease.     

Venous Reddening as a Possible Sign of Hyperperfusion after Superficial Temporal Artery-Middle Cerebral Artery Anastomosis for Moyamoya Disease: Case Report

Here we report a case of moyamoya disease in which cortical veins reddened after superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis, following postoperative hyperperfusion. A 37-year-old man with moyamoya disease suffered cerebral infarction in his right hemisphere. Single photon emission computed tomography (SPECT) showed impaired cerebral blood flow (CBF) in both cerebral hemispheres. The patient underwent STA-MCA anastomosis in the right cerebral hemisphere. During operation, soon after declamping the STA, cortical veins near the anastomosis site changed its color from blue to red. This change was repeatable by clamping and declamping of the STA. Postoperative SPECT and computed tomography (CT) demonstrated increased CBF and subarachnoid hemorrhage at the anastomosis site, suggesting the occurrence of postoperative hyperperfusion. By strictly controlling the patient''s blood pressure, the syndrome resolved 1 week after the operation. We propose that the venous reddening after STA-MCA anastomosis may be a sign of postoperative hyperperfusion.     

Long-term follow-up of pediatric moyamoya disease treated by combined direct–indirect revascularization surgery: single institute experience with surgical and perioperative management

Moyamoya disease (MMD) is a rare occlusive cerebrovascular disease that mainly presents in children as cerebral ischemia. Prompt treatment with either a direct or indirect revascularization procedure is necessary for children with MMD in order to prevent repeated ischemic events. We herein present our experience with combined direct and indirect bypass surgery for the treatment of pediatric MMD as well as our uniquely designed perioperative protocol. Twenty-three patients with MMD, aged between 2 and 16 years old (mean 9.36), underwent 38 combined bypass procedures between 2008 and 2015. All patients underwent single superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis combined with encephalo-duro-myo-synangiosis (EDMS). The perioperative management protocol was stratified into two unique eras: the first era with normotensive care and the second era with strict blood pressure control (systolic 100–130 mmHg) and routine aspirin administration. Patients were followed after surgery for a period ranging between 3 and 131 months (mean 77 months) in yearly clinical and radiological follow-ups. Three postoperative complications were observed: two cases of cerebral hyperperfusion (2/38, 5.3 %) and one case of perioperative minor stroke (1/38, 2.6 %), two of which were in the first era. No strokes, either ischemic or hemorrhagic, were observed in the follow-up period, and the activity of daily living as shown by the modified Rankin Scale improved in 20 patients, with no deterioration being observed in any of our patients. STA-MCA bypass with EDMS is safe and effective for the management of pediatric MMD and provides long-term favorable outcomes. Perioperative care with blood pressure control combined with the administration of aspirin may reduce the potential risk of surgical complications.     

Combined encephaloduroarteriosynangiosis and bifrontal encephalogaleo(periosteal)synangiosis in pediatric moyamoya disease

OBJECTIVE: We compared the surgical results of simple encephaloduroarteriosynangiosis (EDAS) and EDAS with bifrontal encephalogaleo(periosteal)synangiosis for the treatment of pediatric moyamoya disease. METHODS: Data for 159 children (up to 15 yr of age, 76 boys and 83 girls) who underwent indirect revascularization procedures for the treatment of moyamoya disease between 1987 and 1998 were retrospectively reviewed. Group A patients underwent simple EDAS (n = 67). Group B patients underwent EDAS with bifrontal encephalogaleo(periosteal)synangiosis (n = 92). The surgical results for each group were compared, in terms of clinical outcomes, neuroimaging changes, extent of revascularization evident on angiograms, and hemodynamic changes evident on single-photon emission computed tomographic scans. The average follow-up periods were 45 months for Group A and 22 months for Group B. RESULTS: The overall clinical outcomes and neuroimaging changes tended to be better for Group B. In terms of single-photon emission computed tomographic changes of the whole brain after surgery, Group B patients exhibited more favorable outcomes than did Group A patients (62 versus 36%, P = 0.003). The surgical results for the anterior cerebral artery territory were significantly better for Group B than for Group A, with respect to outcomes of anterior cerebral artery symptoms (81 versus 40%, P = 0.015), revascularization on angiograms (79 versus 16%, P < 0.001), and hemodynamic changes on single-photon emission computed tomographic scans (70 versus 52%, P = 0.002). The incidences of postoperative infarctions were not significantly different between the two groups. CONCLUSION: EDAS with bifrontal encephalogaleo(periosteal)synangiosis is a more effective surgical modality for the treatment of pediatric moyamoya disease, compared with simple EDAS, because it covers both the middle cerebral artery and anterior cerebral artery territories of the brain.