Childhood Moyamoya Disease Research Articles

Risk Factors for Preoperative Cerebral Infarction in Infants with Moyamoya Disease.

There have been few reports on the risk factors for preoperative cerebral infarction in childhood moyamoya disease (MMD) in infants under 4 years. The aim of this retrospective study is to identify clinical and radiological risk factors for preoperative cerebral infarction in infants under 4 years old with MMD, and the optimal timing for EDAS was also considered. We retrospectively analyzed the risk factors for preoperative cerebral infarction, confirmed by magnetic resonance angiography (MRA), in pediatric patients aged ˂4 years who underwent encephaloduroarteriosynangiosis between April 2005 and July 2022. The clinical and radiological outcomes were determined by two independent reviewers. In addition, potential risk factors for preoperative cerebral infarction, including infarctions at diagnosis and while awaiting surgery, were analyzed using a univariate model and multivariate logistic regression to identify independent predictors of preoperative cerebral infarction. A total of 160 hemispheres from 83 patients aged <4 years with MMD were included in this study. The mean age of all surgical hemispheres at diagnosis was 2.17±0.831 years (range 0.380-3.81 years). In the multivariate logistic regression model, we included all variables with P<0.1 in the univariate analysis. The multivariate logistic regression analysis indicated that preoperative MRA grade (odds ratio [OR], 2.05 [95% confidence interval [CI], 1.3-3.25], P=0. 002), and age at diagnosis (OR, 0.61 [95% CI, 0.4-0.92], P=0. 018) were predictive factors of infarction at diagnosis. The analysis further indicated that the onset of infarction (OR, 0.01 [95% CI, 0-0.08], P<0.001), preoperative MRA grade (OR, 1.7 [95% CI, 1.03-2.8], P=0.037), and duration from diagnosis to surgery (Diag-Op) (OR, 1.25 [95% CI, 1.11-1.41], P<0.001) were predictive factors for infarction while awaiting surgery. Moreover, the regression analysis indicated that family history (OR, 8.88 [95% CI, 0.91-86.83], P=0.06), preoperative MRA grade (OR, 8.72 [95% CI, 3.44-22.07], P<0.001), age at diagnosis (OR, 0.36 [95% CI, 0.14-0.91], P=0.031), and Diag-Op (OR, 1.38 [95% CI, 1.14-1.67], P=0.001) were predictive factors for total infarction. Therefore, during the entire treatment process, careful observation, adequate risk factor management, and optimal operation time are required to prevent preoperative cerebral infarction, particularly in pediatric patients with a family history, higher preoperative MRA grade, duration from diagnosis to operation longer than 3.53 months, and aged ˂3 years at diagnosis.

Transient neurological events in childhood moyamoya disease.

Patients sometimes experience transient neurological events (TNEs) during the early postoperative period after revascularization surgery for childhood moyamoya disease. The clinical background and pathophysiology of TNEs remain unclear. This study aimed to evaluate the incidence rate of TNEs and discuss the factors associated with pediatric moyamoya disease. The authors retrospectively reviewed 110 cerebral hemispheres of 61 pediatric patients younger than 15 years who were treated from 2011 to 2020. All children underwent either simple double-direct or combined revascularization surgery. Of these 61 patients, 52 underwent bilateral surgery and 9 underwent unilateral surgery. The authors calculated the incidence of TNEs in accordance with a previously reported TNE definition. Cerebral blood flow (CBF) was evaluated in all eligible cases with xenon CT immediately after revascularization surgery. The incidence rate of TNEs in patients with childhood moyamoya disease was 26.4%. TNEs occurred after an average (range) of 6.26 (2-12) days postoperatively without triggers, and all identified TNEs spontaneously resolved within 2 weeks. The most common symptoms were dysarthria, facial palsy, and numbness around the mouth, followed by sensory disturbance of the upper extremities. The presence of focal hyperperfusion on xenon CT performed immediately after revascularization surgery was strongly correlated with the incidence of TNEs (p = 0.0001). Focal hyperperfusion was observed in 43 of 110 operative sides (39.1%). Notably, only 25.6% of patients with focal hyperperfusion showed numerical global hyperperfusion. In addition, a decrease in CBF compared with the thalamic region in the contralateral side was observed in TNE-affected cases (p = 0.0443). TNEs occurred more frequently in childhood moyamoya disease patients than expected. The clinical background, including symptoms, timing, and duration, was almost identical to TNEs in adults. Focal hyperperfusion, rather than numerical global hyperperfusion, was strongly correlated with the incidence of TNEs. Furthermore, the authors advocate the notion that a clinical course where symptoms occur without triggers may be a unique characteristic of TNEs, especially in childhood moyamoya disease.

A Retrospective Study on Clinical Features of Childhood Moyamoya Disease

BackgroundChildhood moyamoya disease (MMD) can lead to progressive and irreversible neurological impairment. Early age at onset is likely associated with a worst prognosis of the disease. The study aims to summarize the clinical characteristics of childhood MMD for supporting the diagnosis and treatment of early MMD. MethodsA retrospective study was conducted on children aged zero to 16 years who were diagnosed with MMD in the Department of Neurology and neurosurgery of our hospital from October 2016 to April 2020. The clinical characteristics of children with MMD were summarized for analysis, and the distribution of sex and initial attack type among different age groups was determined by data comparison. ResultsThe study surveyed 114 children (male to female sex ratio of 1:1.07) with MMD, and 6.1% of them had family history. The mean age of onset was 7.15 ± 3.30 years, and the peak age of onset was five to eight years. The most common initial attack type was transient ischemic attack (TIA) (62 cases, 54.4%) with limb weakness. The incidence of the initial attack type in the three age groups was varied (P < 0.05). The result of overall prognosis was good in 86 cases (89.6%). ConclusionsIn this study, MMD cases were mainly ischemic type and TIA was the most common initial attack type. Infant group was more prone to have cerebral infarction, whereas preschool and school-age groups tended to have TIA. The treatments and prognosis of the studied MMD cases were achieved with good outcomes.

Postoperative change of neuropsychological function after indirect revascularization in childhood moyamoya disease: a correlation with cerebral perfusion study.

The relationships between postoperative functional improvement in various cognitive domains and regional hemodynamic change have not been sufficiently studied in childhood moyamoya disease (MMD). The present study aimed to examine the cognitive benefit of indirect revascularization, the underlying biological mechanism, and factors affecting surgical outcome in childhood MMD. Twenty-three patients with MMD aged under 20years received neuropsychological examinations before and after indirect revascularization surgery, evaluating intellectual function, verbal and visual memory, and executive function. Among them, 13 patients had magnetic resonance perfusion (MRP) studies, in which regional cerebral perfusion was rated. Postoperative improvement was observed in verbal memory performances (p = 0.02-0.03) and in cerebral perfusion at all 26 cerebral hemispheres (p = 0.003-0.005), especially in the middle cerebral artery (MCA) territories (p = 0.001-0.003). Hemodynamic improvement in the left MCA territories was significantly correlated with improvement of both verbal new learning (p = 0.01) and intellectual function (p = 0.004). Postoperative cognitive improvement of immediate recall and verbal intellectual function was associated with female sex (r = - 0.42) and symptom duration (p = - 0.03), respectively. Hemodynamic improvement in the MCA territories was related to longer follow-up intervals (p = 0.02). The findings revealed that the selective postoperative cognitive improvement was associated with increased regional perfusion in the MCA territories, and indicate the importance of early intervention and the potential of indirect revascularization regarding long-term outcome.

Single-Stage Direct Revascularization for Bilateral Anterior Cerebral Artery Regions in Pediatric Moyamoya Disease: A Technical Note

The authors present cases of childhood moyamoya disease successfully treated with bilateral superficial temporal artery to anterior cerebral artery (ACA) direct anastomoses and describe the surgical techniques used in this vascular reconstruction method. The authors conducted bilateral superficial temporal artery to ACA anastomoses, combined with indirect bypass in 3 pediatric patients with moyamoya disease from 2009 to 2015. All 3 patients presented with transient ischemic attacks related to ischemia of the ACAs and had cerebral blood flow insufficiency, predominantly in the ACA regions bilaterally. In all 3 cases, ischemic symptoms corresponding to ACA ischemia had disappeared after bypass surgery. No patients experienced any postoperative neurologic or wound complications associated with the procedure. Improvement of cerebral blood flow in bilateral ACA territories and the patency of the direct bypasses were confirmed by follow-up imaging in all 3 patients. The described technique can be considered an effective and uncomplicated revascularization procedure for the ACA regions, which are crucial for normal intellectual growth in the pediatric population.

Clinicoradiological Profile of Childhood Moyamoya Disease: Indian Study of 30 Children with Literature Review

Clinicoradiological Profile of Childhood Moyamoya Disease: Indian Study of 30 Children with Literature Review

Differential stroke pattern among childhood and adult onset moyamoya disease (MMD)

Differential stroke pattern among childhood and adult onset moyamoya disease (MMD)

Abstract TP397: Clinical And Functional Outcome Of Adult Patients Diagnosed With Moyamoya Disease In Childhood

Background: Moyamoya disease is a progressive occlusive arteriopathy of the terminal ICA and its branches, leading to the formation of unstable collateral vessels. The disease is found worldwide, and is associated with a number of predisposing conditions, termed moyamoya syndrome. Currently there is a paucity of data on the natural history of moyamoya disease in North America, especially the long-term outcome of pediatric moyamoya. Objective: Our objectives were to determine the rate of recurrent TIA, ischemic stroke and intracerebral hemorrhage in patients presenting with pediatric Moyamoya disease and to assess long-term functional outcome. Methods: This study was a retrospective chart review of 52 patients presenting with pediatric moyamoya disease. We included patients aged 18 or older with a diagnosis of moyamoya disease or syndrome. All patients had confirmatory baseline vascular imaging (MRA or CTA +/- cerebral angiography). Baseline demographic variables and annual vascular event recurrence risk were obtained from the records. Modified Rankin Score (MRS) at presentation and last follow-up were determined from clinical records. Results: Of the original cohort, 34 patients were included for analysis. The mean age of the patients was 23.9 years. The mean age at presentation was 9 years (SD=4) with an average follow-up of 11.3 years (SD=5.1) for a total of 383 patient years. There was slight female predominance (1.4:1). Fifty percent of patients (17) presented with ischemic stroke. A total of 26 patients (76%) underwent surgery, with 7 (21%) requiring repeat surgery. The annual vascular recurrent event rate was 5.0% (19/383), which was not significantly different between surgical and non-surgical groups or between different moyamoya syndrome subtypes. Most of the recurrent events were TIA (annual recurrence 3.7%). There was no significant difference between initial MRS, MRS at last follow-up and mean change in MRS between surgical and non-surgical groups. Conclusion: Our study is the largest North American natural history study of pediatric moyamoya. Our observations indicate that pediatric moyamoya in North America have low recurrent vascular event rates and long-term functional outcomes are good, even in conservatively managed patients.

Pattern of Presentation of Moyamoya Disease (MMD) Patients in Bangladesh: Experience from Tertiary Care Hospitals

Background: Moyamoya disease is rare but not uncommon throughout the world. Clinical profile of childhood moyamoya (MMD) disease is not well delineated in Bangladesh. Methods: We conducted this cross sectional study in pediatrics and neurology department of Dhaka Medical College Hospital that involved 20 patients of MMD over a period of one year. Result: Among the cases about 2/3rd(65%) of the patients were within 8 years age at onset with mean age of the patients being 7.24(±3.34) years at onset with a male: female ratio of 1.2:1. Almost half of the patients had past history of intermittent episodes TIA which precipitated by hyperventilation and crying (p &lt;0.05). Important history related to prothombotic conditions (Family History of stroke, MI, Hyperlipidemia, Obesity, Coagulation disorders) were also statistically significant (p&lt;0.05). Sixteen patients in our series exhibited hemiparesis and out of them 4(25%) were alternating (p&lt;0.05), followed by dysarthria at onest 13(65%). Convulsions and visual impairment were seen in 5 (25%) different patients, 3(15%) different patients had altered consciousness, involuntary movements, ataxia, headache and cognitive impairment at onset. We observed intellectual impairment in and psycho–motor retardation in two different patients. MRA abnormalities were found in 19 cases out of 20. Among 19 cases bilateral ICA stenosis with collaterals seen in 18 cases (90%), MCA stenosis along with bilateral ICA stenosis were seen in 16(80%) cases, ACA stenosis along with bilateral ICA stenosis were seen in 07(35%) cases, PCA stenosis along with bilateral ICA stenosis were observed in 05(25%) cases. No collaterals and without typical “puff of smoke” appearance was seen in 01(5%) and unilateral ICA stenosis with collaterals was seen in 01(5%) cases (probable MMD). Diagnostic Cerebral DSA was done in 07 (35%) patients and typical angiographic findings of Moyamoya disease were present in all of them. Conclusion: C-MMD may have various presentations. Stroke and TIA are most common presentation. MRA may well delineate the characteristics angrographic abnormality. Bangladesh Journal of Neuroscience 2017; Vol. 33 (1): 24-29

小児もやもや病に対する浅側頭動脈－前大脳動脈・中大脳動脈同時吻合術に間接吻合を加えた拡大血行再建術

小児もやもや病に対する浅側頭動脈－前大脳動脈・中大脳動脈同時吻合術に間接吻合を加えた拡大血行再建術

Clinico-radiological profile of childhood moyamoya disease - a study of 30 children from India

Clinico-radiological profile of childhood moyamoya disease - a study of 30 children from India

Characteristics of brain magnetic resonance images at symptom onset in children with moyamoya disease.

To clarify the characteristics of brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) at symptom onset in patients with childhood moyamoya disease (MD) according to developmental stage. In 26 childhood MD patients who underwent brain MRI and MRA within 3 months from symptom onset, MRA scores and grades and MRI findings from symptomatic hemispheres were summarized according to developmental stage: infancy (0-1 years, three patients), toddlerhood/preschool age (2-5 years, nine patients), school age (6-10 years, seven patients), and adolescence (11-18 years, seven patients). Mean MRA score was 5.6, which was higher in adolescents (6.9). The most common MRA grade was grade 3, particularly in those under 10 years. Acute ischemic infarction presented in 17 patients (65%), was more common in patients below 5 years (83%) and was mainly in a gyral pattern (47%). Most hemorrhagic infarctions occurred in adolescents. Moyamoya vessels in the Sylvian valleys were apparent in 22 patients (85%), which were the most sensitive non-ischemic findings on T2 weighted images. The signal void in the distal internal carotid artery was diminished in 73% and apparent signal voids in basal ganglia were seen in three patients. The ivy sign was positive in 81% on fluid attenuated inversion recovery images and 69% on contrast-enhanced T1-weighted images in 21 and 16 patients, respectively. Brain MRIs and MRAs at symptom onset in childhood MD have characteristic findings, which differ in different developmental stage.

Selective and superselective angiography of pediatric moyamoya disease angioarchitecture: the anterior circulation.

The angioarchitecture of the so-called moyamoya vessels in children has not been explicitly analyzed. We aimed to investigate the precise anatomy of the vascular anastomotic networks in patients with childhood moyamoya disease. Six children diagnosed with moyamoya disease for the first time underwent an angiographic investigation with selective and superselective injections. We recorded the arterial branches feeding the moyamoya anastomotic networks, their connections and the recipient vessels. Depending on the level of the steno-occlusive lesion, the feeding vessels included the medial striate arteries, the perforators of the choroidal segment of the carotid, the uncal artery, the medial and lateral branches of the intraventricular segment of the anterior choroidal artery, perforators of the communicating segment, the superior hypophyseal arteries, the prechiasmal branches of the ophthalmic artery, the ethmoidal arteries and the dural branches of the cavernous carotid. Through connections, which are described, the recipient vessels were the lateral striate arteries and the middle cerebral, the medial striate arteries and the anterior cerebral, medullary arteries around the ventricular system, anterior temporal branches of the middle cerebral, orbitofrontal and frontopolar branches of the anterior cerebral, as well as other cortical branches of the anterior and middle cerebral territories. The use of high quality selective and superselective angiography enabled us to clearly demonstrate for the first time aspects of the microangiographic anatomy of the moyamoya anastomotic network previously only vaguely or incompletely described.

前大脳動脈領域に骨膜と浅側頭動脈を用いた小児もやもや病に対する複合的血行再建術の有用性と問題点

前大脳動脈領域に骨膜と浅側頭動脈を用いた小児もやもや病に対する複合的血行再建術の有用性と問題点

Selective and superselective angiography of pediatric moyamoya disease angioarchitecture in the posterior circulation.

The anastomotic network of the posterior circulation in children with moyamoya disease has not been analyzed. We aimed to investigate the angiographic anatomy of this unique vascular network in patients with childhood moyamoya disease. Selective and superselective injections of the posterior circulation were performed in six children with newly diagnosed moyamoya disease. The arterial branches feeding the moyamoya anastomotic network, their connections and the recipient vessels were demonstrated. Depending on the level of the steno-occlusive lesion, the feeding vessels were the thalamoperforators, the posterior choroidals, the splenic artery, parietoccipital artery, other cortical posterior cerebral artery (PCA) branches, the dural branch of the PCA, the premamillary artery and other posterior communicating artery perforators. Through connections, which are described, the recipient vessels were the striate and medullary arteries, other thalamic arteries with or without medullary extensions, the pericallosal artery, medial parietoccipital cortical branches of the PCA and the anterior choroidal artery. High quality selective and superselective angiography helped in demonstrating the angiographic anatomy of the moyamoya posterior anastomotic network previously either vaguely or incompletely described, as well as connections within the posterior circulation but also its relevance as a collateral to the anterior circulation.

脳血管造影上中枢側STAが描出されず手術に工夫を要した小児もやもや病の1例

脳血管造影上中枢側STAが描出されず手術に工夫を要した小児もやもや病の1例

Early clinical characteristics according to developmental stage in children with definite moyamoya disease

The objective is to clarify the early clinical characteristics in childhood moyamoya disease (MD). Epidemiologic characteristics, symptoms and diagnostic rates were assessed in 64 children (0–18years) with definite MD according to developmental stage: infancy (5; 0–1years); toddlerhood/preschool age (22; 2–5years); school age (29; 6–10years); and adolescence (8; 11–18years). The median ages at onset was 6.25years and the female to male ratio was 1.9 (⩾2.5 in toddlerhood/preschool age and in adolescence, P=0.71). Previous headache was observed in 23% (14/64): frequently in school age (38%, P=0.02) and within 6months before main symptoms (6/11). As an initial symptom, weakness was observed in 78% (50/64) mainly as transient ischemic attack (TIA, 61%) in limbs (90%) and unilaterally (82%). TIA was less frequent in infancy (40%, P=0.04). Seizure was observed in 27% (17/64): frequently in infancy (100%, P<0.01), as the focal type (71%), and in the right extremity (3:1). Isolated seizures without other symptoms was frequent in children ⩽5years (P<0.01). Severe headache associated with MD was observed in 14% (9/64). Provoking events were positive in 42% (27/64): in school age, frequently during eating (28%); and in toddlerhood/preschool age, during crying (27%). The diagnostic rates at 3 and 12months from symptom-onset were 39% (80% during infancy vs. 28% in school age, P=0.14) and 67%, respectively. Symptomatic progression at diagnosis was observed in 38% (24/64). Initial clinical characteristics in childhood definite MD differed according to developmental stage and from at diagnosis.

小児もやもや病でのstable xenon enhanced CTを用いた脳血流量測定における実効線量軽減

Measurement of cerebral blood flow (CBF) is necessary for the management of childhood moyamoya disease. Computed tomography (stable Xe CT) enhanced by three minutes of inhalation of stable xenon is an established method for measurement of CBF. In this retrospective study, we evaluated a decrease in serial scan times in stable Xe CT in childhood moyamoya disease in order to lessen the effective dose. CBF values obtained by usually performed stable Xe CT were evaluated in 12 patients with childhood moyamoya disease. Clinically, nine serial scans were performed as usual. In this retrospective study, CBF was calculated based on nine serial scans (S9), based on seven serial scans out of the obtained nine serial scans (S7), and based on five scans out of the obtained nine serial scans (S5). Both S7 and S5 had a significantly simple linear correlation with S9 as follows: S9=1.642+1.031×S7, S9=2.726+0.983×S5. CBF maps obtained by S9, S7, and S5 were almost identical. In childhood moyamoya disease, measurement of CBF using stable Xe CT with performance of five serial scans in each slice can be used to lessen the effective dose.

Focal time-to-peak changes on perfusion MRI in children with Moyamoya disease: correlation with conventional angiography

Moyamoya disease is a chronic progressive steno-occlusion of the distal internal carotid arteries with unknown etiology. As the classical presentation of childhood Moyamoya disease is ischemic stroke, cerebral hemodynamic evaluation is important for patient selection for surgery to prevent recurrent ischemic attacks. Perfusion MR imaging has been applied to evaluate cerebral hemodynamics. To correlate the 'basal time-to-peak preservation sign', 'auto-synangiosis sign', and 'posterior involvement sign' on time-to-peak map of perfusion MRI with catheter angiography. Thirty-four children (6.91 ± 3.08 years) with Moyamoya disease who underwent both perfusion-weighted MRI and catheter angiography were enrolled in this study. Given catheter angiography as a reference standard, basal time-to-peak preservation sign, auto-synangiosis sign, and posterior involvement sign were evaluated on time-to-peak maps. The basal time-to-peak preservation sign was accurate for the diagnosis of childhood Moyamoya disease; both sensitivity and specificity were 100%. The auto-synangiosis sign showed lower sensitivity (65%), however, with an acceptable specificity (98%). The posterior involvement sign showed lower sensitivity (61%) but had an acceptable specificity (96%). The basal time-to-peak preservation sign may be a universal finding in childhood Moyamoya disease. The auto-synangiosis and posterior involvement sign may be useful in determining transdural collateral status and posterior circulation involvement in childhood Moyamoya disease.

Childhood Moyamoya Disease and Moyamoya Syndrome: A Pictorial Review

Moyamoya disease is an uncommon chronic cerebrovasculopathy, characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches, in association with the development of compensatory collateral vessels at the base of the brain. The etiology is unknown, and was originally considered exclusive to East Asia, with particular prevalence in Japan. Moyamoya disease is increasingly diagnosed throughout the world, and represents an important cause of childhood stroke in Western countries. In some cases, similar angiographic features are evident in children with other medical conditions, such as sickle cell disease and Down syndrome. In these instances, the term "moyamoya syndrome" is used. Diagnosing the vasculopathy, excluding possible associated conditions, and planning treatment and follow-up imaging comprise important aspects of clinical management. We review the key imaging features of childhood moyamoya disease and syndrome, present examples of its associations, and discuss new neuroradiologic methods that may be useful in management.