Automated Evaluation of Intracranial Vessel Morphology in Normal Versus Pediatric Moyamoya Disease

Abstract INTRODUCTION Evaluation of intracranial artery morphology plays an important role in diagnosing a variety of neurovascular diseases. In addition to clinical symptoms, diagnosis currently relies on qualitative rather than quantitative evaluation of vascular imaging sequences such as magnetic resonance angiography (MRA). However, previously described statistical cerebroarterial atlases have focused primarily on healthy adults and little information exists about what constitutes normal artery morphology in the pediatric population and across brain development. We aimed to quantitatively assess normal, age-related changes in artery morphology and compare normal morphology to that of children with Moyamoya disease (MMD). METHODS MRAs from 98 children (49 M/49F) aged .6 to 20 yr (median = 11.5 yr) with normal MRAs and and 18 children with radiographically confirmed MMD (10 M/8 F, median age = 7.1 yr) were retrospectively collected. All arteries were automatically segmented in both MRA datasets. Using an atlas-based approach, the radiuses of the main arteries of the anterior circulation (internal carotid artery (ICA), anterior cerebral artery (ACA), and middle cerebral artery (MCA)) and posterior circulation (PCA, BA) were measured at corresponding locations. Artery radii were compared between the 2 groups using MANCOVA with age and sex as covariates. RESULTS The artery radius was relatively consistent across age for all main arteries in normal patients. MANCOVA revealed that children with MMD exhibit significantly smaller ICA, MCA-M1, MCA-M2, and ACA radii (P < .001) compared to normal controls (mean vessel radii: ICA 1.27 vs 1.64 mm, MCA M1 0.92 vs 1.14 mm, MCA M2 0.66 vs 0.82 mm, ACA 0.72 vs 0.83 mm). There were no significant differences in the posterior circulation radii. CONCLUSION We present normal artery morphology data for children based on automatic segmentation of MRAs, and demonstrate that artery caliber is smaller in children with MMD. This resource will allow neurosurgeons to quantitatively assess MMD and the impact of bypass surgery on disease progression.

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Posterior cerebral artery stenosis and posterior circulation revascularization surgery in pediatric patients with moyamoya disease

Journal of Neurosurgery Pediatrics ◽

10.3171/2018.1.peds17367 ◽

2018 ◽

Vol 21 (6) ◽

pp. 632-638 ◽

Cited By ~ 3

Author(s):

Tomomi Kimiwada ◽

Toshiaki Hayashi ◽

Reizo Shirane ◽

Teiji Tominaga

Keyword(s):

Moyamoya Disease ◽

Cerebral Artery ◽

Pediatric Patients ◽

Posterior Cerebral Artery ◽

Posterior Circulation ◽

Initial Diagnosis ◽

Occipital Artery ◽

Anterior Circulation ◽

Before And After ◽

Revascularization Surgery

OBJECTIVESome pediatric patients with moyamoya disease (MMD) present with posterior cerebral artery (PCA) stenosis before and after anterior circulation revascularization surgery and require posterior circulation revascularization surgery. This study evaluated the factors associated with PCA stenosis and assessed the efficacy of posterior circulation revascularization surgery, including occipital artery (OA)–PCA bypass, in pediatric patients with MMD.METHODSThe presence of PCA stenosis before and after anterior circulation revascularization surgery and its clinical characteristics were investigated in 62 pediatric patients (< 16 years of age) with MMD.RESULTSTwenty-three pediatric patients (37%) with MMD presented with PCA stenosis at the time of the initial diagnosis. A strong correlation between the presence of infarction and PCA stenosis before anterior revascularization was observed (p < 0.001). In addition, progressive PCA stenosis was observed in 12 patients (19.4%) after anterior revascularization. The presence of infarction and a younger age at the time of initial diagnosis were risk factors for progressive PCA stenosis after anterior revascularization (p < 0.001 and p = 0.002, respectively). Posterior circulation revascularization surgery, including OA-PCA bypass, was performed in 9 of the 12 patients with progressive PCA stenosis, all of whom showed symptomatic and/or radiological improvement.CONCLUSIONSPCA stenosis is an important clinical factor related to poor prognosis in pediatric MMD. One should be aware of the possibility of progressive PCA stenosis during the postoperative follow-up period and consider performing posterior circulation revascularization surgery.

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Abstract P568: Incidence and Progression of Posterior Circulation Involvement in Moyamoya Disease

Stroke ◽

10.1161/str.52.suppl_1.p568 ◽

2021 ◽

Vol 52 (Suppl_1) ◽

Author(s):

Yiping Li ◽

Allan R Wang ◽

Gary K STEINBERG

Keyword(s):

Moyamoya Disease ◽

Functional Outcomes ◽

Posterior Circulation ◽

External Carotid ◽

Collateral Flow ◽

Anterior Circulation ◽

Bilateral Disease ◽

Operative Complications ◽

The Impact

Introduction: The incidence and natural history of posterior circulation moyamoya disease (PCMMD) is not well-characterized. Methods: Consecutive patients with moyamoya disease prospectively treated at our institution from 2015-2018 were reviewed. Results: 262 consecutive patients with moyamoya disease undergoing 370 revascularization procedures were enrolled. Concurrent PCMMD was identified in 68 (26%) patients – all of which were confined to the anterior circulation. There were no differences in age, sex, race, or rates of diabetes, hypertension, smoking, or symptoms upon presentation between patients with or without PCMMD. Patients with PCMMD were more likely to present with bilateral disease (p<0.001), higher Suzuki grade (p=0.001), more extensive collateral angiopathy (p<0.001), and pial-pial collaterals from the PCA territory (p=0.03). Patients with PCMMD were more likely to suffer from ischemic peri-operative complications in both the anterior (p=0.03) and posterior (p<0.001) circulation territories after anterior circulation revascularization. No differences were observed for hemorrhagic complications (p=1.0). Over a mean follow-up of 1.91±1.5 years, 19 (7.3%) developed new or progressive PCMMD. Patients with PCMMD progression had an Asian predilection and were less likely to present with sentinel symptoms such as headaches (p=0.05 and 0.01 respectively). Patients with bilateral disease (p=0.02), higher Suzuki (p<0.001) and collateral angiopathy grades (p=0.002), and more extensive external carotid artery supply (p=0.03) were also more likely to experience PCMMD progression. There was no association between PCMMD progression and delayed neurological events or functional outcomes, but patients with concurrent PCMMD upon presentation were associated with poor functional outcome (mRS >2) at last follow-up (p=0.02). Conclusions: Posterior circulation disease involvement often presents in the later stages of moyamoya and is associated with higher rates of ischemic peri-operative complications and poor functional outcomes likely due to reduced collateral flow. Progression of PCMMD is not uncommon. Future studies are needed to assess the impact of PCMMD progression on long-term outcomes.

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Strategy and effect of repeat bypass surgery for anterior/posterior circulation in refractory moyamoya disease

Journal of Neurosurgery ◽

10.3171/2019.3.jns181979 ◽

2020 ◽

Vol 132 (6) ◽

pp. 1889-1899

Author(s):

Haruto Uchino ◽

Daina Kashiwazaki ◽

Naoki Akioka ◽

Masaki Koh ◽

Naoya Kuwayama ◽

...

Keyword(s):

Moyamoya Disease ◽

Cerebral Artery ◽

Transient Ischemic Attack ◽

Bypass Surgery ◽

Posterior Circulation ◽

Therapeutic Effects ◽

Anterior Circulation ◽

Group A ◽

Ischemic Attack ◽

Group B

OBJECTIVEIn this study the authors aimed to describe clinical features, surgical techniques, and long-term outcomes of repeat bypass surgery required for a certain subset of patients with moyamoya disease.METHODSThe authors retrospectively reviewed a total of 22 repeat bypass surgeries for 20 patients (age range 1–69 years) performed during the last 20 years at their institutions. The patients were classified into 2 groups. Group A included 10 patients who underwent repeat bypass surgery for anterior circulation due to insufficient revascularization on the ipsilateral side. Group B included 10 patients who underwent repeat bypass surgery for posterior circulation due to the involvement of the posterior cerebral artery (PCA) after successful initial surgery for anterior circulation.RESULTSPreoperative symptoms included headache in 3 patients, transient ischemic attack in 10, cerebral infarction in 3, and intracranial hemorrhage in 4 patients. Intervals between the initial bypass surgery and repeat bypass surgery were 0.3–30 years (median 3 years). In group A, superficial temporal artery to middle cerebral artery (MCA) anastomosis and indirect bypass were performed on 7 hemispheres. Only indirect bypass was performed on 3 hemispheres because of the lack of suitable donor or recipient arteries. In group B, occipital artery (OA) to PCA anastomosis and indirect bypass were conducted on 4 hemispheres, and OA-MCA anastomosis and indirect bypass on 1 hemisphere. Only indirect bypass was conducted on 7 hemispheres because of the lack of suitable recipient arteries. All 22 repeat bypass surgeries were successfully conducted. During follow-up periods (median 4 years), none of the patients suffered repeat stroke except 1 patient who died of recurrent intracerebral hemorrhage 3 years after repeat bypass surgery for anterior circulation.CONCLUSIONSRepeat bypass surgery was feasible and effective to reduce further incidence of headache attack, transient ischemic attack, and ischemic/hemorrhagic stroke in moyamoya disease patients. Through precise radiological analysis, surgical procedures should be planned to yield maximal therapeutic effects.

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Treatment of aneurysms in patients with moyamoya disease: a 10-year single-center experience

Journal of Neurosurgery ◽

10.3171/2017.3.jns162290 ◽

2018 ◽

Vol 128 (6) ◽

pp. 1813-1822 ◽

Cited By ~ 6

Author(s):

Wei Ni ◽

Hanqiang Jiang ◽

Bin Xu ◽

Yu Lei ◽

Heng Yang ◽

...

Keyword(s):

Moyamoya Disease ◽

Posterior Circulation ◽

Cerebral Revascularization ◽

Endovascular Coiling ◽

Single Center ◽

Anterior Circulation ◽

Main Trunk ◽

Aneurysm Embolization ◽

Peripheral Aneurysms

OBJECTIVEMoyamoya disease (MMD) is occasionally accompanied by intracranial aneurysms. The purpose of this study was to delineate the efficacy of the authors’ current surgical strategy in the management of MMD-associated aneurysms of different types.METHODSBetween January 2007 and March 2016, a consecutive cohort of 34 patients with 36 MMD-associated aneurysms was enrolled in this prospective single-center cohort study. The lesions were classified as peripheral (17 aneurysms) or main trunk aneurysms (13 in the anterior circulation and 6 in the posterior circulation). For the peripheral aneurysms, revascularization with or without endovascular treatment was suggested. For the main trunk aneurysms, revascularization alone, revascularization with aneurysm clipping, or revascularization with aneurysm embolization were used, depending on the location of the aneurysms.RESULTSOf the peripheral aneurysms, 4 were treated endovascularly with staged revascularization, and 13 were treated solely with cerebral revascularization. Of the 13 main trunk aneurysms in the anterior circulation, 10 were clipped followed by revascularization, and 3 were coiled followed by staged cerebral revascularization. Of the 6 main trunk aneurysms in the posterior circulation, 4 underwent endovascular coiling and 2 were treated solely with revascularization. One patient died of contralateral intracerebral hemorrhage 6 months after the operation. No other patients suffered recurrent intracranial hemorrhage, cerebral ischemia, or aneurysm rupture. An angiographic follow-up study showed that all the bypass grafts were patent. Complete occlusion was achieved in all 21 aneurysms that were clipped or embolized. Of the remaining 15 aneurysms that were not directly treated, 12 of 13 peripheral aneurysms were obliterated during the follow-up, whereas 1 remained stable; 1 of 2 posterior main trunk aneurysms remained stable, and the other became smaller.CONCLUSIONSThe authors’ current treatment strategy may benefit patients with MMD-associated aneurysms.

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Beware of Nihilism: Favorable Outcome despite Poor Admission Status in Posterior Circulation Aneurysms after Aneurysmal Subarachnoid Hemorrhage

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0040-1719142 ◽

2021 ◽

Author(s):

Nina Brawanski ◽

Sepide Kashefiolasl ◽

Sae-Yeon Won ◽

Joachim Berkefeld ◽

Elke Hattingen ◽

...

Keyword(s):

Subarachnoid Hemorrhage ◽

Mortality Rate ◽

Cerebral Artery ◽

Coil Embolization ◽

Posterior Inferior Cerebellar Artery ◽

Posterior Circulation ◽

Anterior Circulation ◽

Ruptured Aneurysms ◽

Admission Status ◽

Posterior Circulation Aneurysms

Abstract Objective As shown in a previous study, aneurysm location seems to influence prognosis in patients with subarachnoid hemorrhage (SAH). We compared patients with ruptured aneurysms of anterior and posterior circulation, undergoing coil embolization, concerning differences in outcome and prognostic factors. Methods Patients with SAH were entered into a prospectively collected database. We retrospectively identified 307 patients with aneurysms of the anterior circulation (anterior cerebral artery, carotid bifurcation, and middle cerebral artery) and 244 patients with aneurysms of the posterior circulation (aneurysms of the basilar artery, posterior inferior cerebellar artery, posterior communicating artery and posterior cerebral artery). All patients underwent coil embolization. The outcome was assessed using the modified Rankin Scale (mRS; favorable [mRS 0–2] vs. unfavorable [mRS 3–6]) 6 months after SAH. Results In interventionally treated aneurysms of the anterior and posterior circulation, statistically significant risk factors for poor outcome were worse admission status and severe cerebral vasospasm. If compared with patients with ruptured aneurysms of the anterior circulation, patients with aneurysms of the posterior circulation had a significantly poorer admission status, and suffered significantly more often from an early hydrocephalus. Nonetheless, there were no differences in outcome or mortality rate between the two patient groups. Conclusion Patients with a ruptured aneurysm of the posterior circulation suffer more often from an early hydrocephalus and have a significantly worse admission status, possibly related to the untreated hydrocephalus. Nonetheless, the outcome and the mortality rate were comparable between ruptured anterior and posterior circulation aneurysms, treated by coil embolisation. Therefore, despite the poorer admission status of patients with ruptured posterior circulation aneurysms, treatment of these patients should be considered.

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Abstract TP361: Recreational Drug Use and Arterial Ischemic Stroke in a Pediatric Cohort

Stroke ◽

10.1161/str.51.suppl_1.tp361 ◽

2020 ◽

Vol 51 (Suppl_1) ◽

Author(s):

Ishvinder Bhathal ◽

Tazeen Qureshi ◽

Mahendranath Moharir ◽

Daune MacGregor ◽

Elizabeth Pulcine ◽

...

Keyword(s):

Ischemic Stroke ◽

Drug Use ◽

Tertiary Care ◽

Case Series ◽

Posterior Circulation ◽

Recreational Drug ◽

Arterial Ischemic Stroke ◽

Anterior Circulation ◽

Recreational Drug Use ◽

The Impact

Background: Existing literature reports an association between recreational drug use and arterial ischemic stroke (AIS) in adults. Due to recent trends in legalization and concerns regarding the impact of drugs on the developing brain, there is an urgent need for increased awareness of recreational drug use as a risk factor for AIS in childhood. Purpose: To increase awareness of an association between AIS and recreational drug use in a pediatric cohort. Methods: We conducted a retrospective chart review of a consecutive cohort of patients at a tertiary care pediatric center diagnosed with AIS in the context of recreational drug use between 2008-2017. Drug use was confirmed using toxicology testing and clinical history. Demographic, clinical and radiological data were collected. Pediatric Stroke Outcome Measure scores (PSOM) were obtained from an institutional Stroke Registry. Results: Three males and one female were included in the study. Mean age at stroke presentation was 16.3 years (range 16-17 years). Three children presented with focal neurologic deficit and one with new onset seizure. Drug use for each patient was described as follows: Patient A - Marijuana; Patient B - Oxybutynin, Fluoxetine and unidentified compound; Patient C - Marijuana and Amphetamine; Patient D - Marijuana and alcohol. MRI demonstrated diffusion restriction in the anterior circulation in two children, anterior and posterior circulation in one child, and bilateral posterior circulation in one child also found to have a remote AIS. Vascular findings included: Patient A - normal; Patient B and C - right anterior circulation arteriopathy; Patient D - posterior circulation arteriopathy and bilateral vessel wall enhancement. ECHO and pro-thrombotic results were non-contributory. However, one patient required PFO closure. PSOM scores indicated mild-moderate disability initially and moderate disability at follow-up for three of four patients. Conclusions: This case series describes an association between recreational drug use and AIS in adolescents. We are unable to comment on the incidence of AIS related to drug use from our cohort. However, our data highlights a need for public health strategies that acknowledge AIS as a potential consequence of recreational drug use in adolescents.

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Developmental Pharmacokinetics in Pediatric Populations

The Journal of Pediatric Pharmacology and Therapeutics ◽

10.5863/1551-6776-19.4.262 ◽

2014 ◽

Vol 19 (4) ◽

pp. 262-276 ◽

Cited By ~ 10

Author(s):

Hong Lu ◽

Sara Rosenbaum

Keyword(s):

Drug Absorption ◽

Pediatric Population ◽

Drug Distribution ◽

Drug Dose ◽

Tubular Secretion ◽

Infants And Children ◽

Water Composition ◽

Age Related ◽

The Impact ◽

In Infants And Children

Information on drug absorption and disposition in infants and children has increased considerably over the past 2 decades. However, the impact of specific age-related effects on pharmacokinetics, pharmacodynamics, and dose requirements remains poorly understood. Absorption can be affected by the differences in gastric pH and stomach emptying time that have been observed in the pediatric population. Low plasma protein concentrations and a higher body water composition can change drug distribution. Metabolic processes are often immature at birth, which can lead to a reduced clearance and a prolonged half-life for those drugs for which metabolism is a significant mechanism for elimination. Renal excretion is also reduced in neonates due to immature glomerular filtration, tubular secretion, and reabsorption. Limited data are available on the pharmacodynamic behavior of drugs in the pediatric population. Understanding these age effects provide a mechanistic way to identify initial doses for the pediatric population. The various factors that impact pharmacokinetics and pharmacodynamics mature towards adult values at different rates, thus requiring continual modification of drug dose regimens in neonates, infants, and children. In this paper, the age-related changes in drug absorption, distribution, metabolism, and elimination in infants and children are reviewed, and the age-related dosing regimens for this population are discussed.

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Selective and Superselective Angiography of Pediatric Moyamoya Disease Angioarchitecture in the Posterior Circulation

Interventional Neuroradiology ◽

10.15274/inr-2014-10041 ◽

2014 ◽

Vol 20 (4) ◽

pp. 403-412 ◽

Cited By ~ 7

Author(s):

Gerasimos Baltsavias ◽

Nadia Khan ◽

Venko Filipce ◽

Anton Valavanis

Keyword(s):

Moyamoya Disease ◽

Posterior Cerebral Artery ◽

Posterior Circulation ◽

Anterior Circulation ◽

Feeding Vessels ◽

Occlusive Lesion ◽

Superselective Angiography ◽

Anterior Choroidal ◽

Premamillary Artery ◽

Choroidal Artery

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.

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Cranial burr hole for revascularization in moyamoya disease

Journal of Neurosurgery ◽

10.3171/jns.1989.71.2.0180 ◽

1989 ◽

Vol 71 (2) ◽

pp. 180-185 ◽

Cited By ~ 66

Author(s):

Masataka Endo ◽

Nobuyuki Kawano ◽

Yoshio Miyasaka ◽

Kenzoh Yada

Keyword(s):

Moyamoya Disease ◽

Cerebral Artery ◽

Clinical Symptoms ◽

Superficial Temporal Artery ◽

Temporal Artery ◽

Burr Hole ◽

Frontal Region ◽

External Carotid ◽

Content Type ◽

Made In

✓ Currently, superficial temporal artery-middle cerebral artery (MCA) anastomosis, encephalomyosynangiosis (EMS), and encephalo-duro-arterio-synangiosis are used to treat moyamoya disease and are reported to effectively improve ischemic symptoms. All are methods of reversing the flow of blood from the external carotid artery system into the cortical branches of the MCA. As moyamoya disease advances, these operations alone will predictably not correct the deterioration in blood flow in the territory of the anterior cerebral artery. It was noted in a case of moyamoya disease with intraventricular hemorrhage that a burr hole, made in the frontal region for drainage purposes, induced marked neovascularization. Since then, similar frontal burr holes have been made in five juvenile cases of moyamoya disease; this procedure involved making a burr hole in both frontal bones and incising both the dura and the arachnoid membrane. In two cases a frontal burr hole was placed simultaneously with EMS, and in the others the frontal burr hole was made following EMS. The clinical symptoms improved after the frontal burr hole was made, and dynamic computerized tomography revealed improved circulation in the frontal regions. Together with conventional surgical therapy for juvenile cases of moyamoya disease, this operation is considered beneficial both to the circulation in the frontal region and for the protection of frontal brain function.

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Characterization of Clinical and Radiological Features of Quasi-Moyamoya Disease among European Caucasians Including Surgical Treatment and Outcome

Cerebrovascular Diseases ◽

10.1159/000448812 ◽

2016 ◽

Vol 42 (5-6) ◽

pp. 464-475 ◽

Cited By ~ 7

Author(s):

Güliz Acker ◽

Susanne Goerdes ◽

Peter Schmiedek ◽

Marcus Czabanka ◽

Peter Vajkoczy

Keyword(s):

Rare Disease ◽

Clinical Features ◽

Moyamoya Disease ◽

Pediatric Patients ◽

Clinical Symptoms ◽

Pediatric Population ◽

Demographic Data ◽

Associated Diseases ◽

Wide Range

Background: Moyamoya disease (MMD) associated with a potentially underlying disease, such as genetic disorders or other coexisting hematological pathologies, is called quasi-MMD. This very rare disease has been characterized mainly in Asian countries, so far. As MMD reveals several significant ethnic differences, the question is raised whether characteristics of quasi-MMD would also vary among different ethnic backgrounds. Here, we report a series of 61 patients with quasi-MMD and highlight the specific clinical features of this rare disease among European Caucasians. Methods: We retrospectively identified 61 European Caucasians with quasi-MMD who were treated in our institution between 1997 and 2014. We analyzed demographic data, clinical symptoms, associated diseases, angiographic characteristics and functional hemodynamic studies. Results: Thirty-three percent of our patients were juvenile. We observed an overall female predominance of 2.8:1. Seventy-nine percent presented with a typical quasi-MMD with more pronounced unilateral and atypical quasi-MMD in pediatric population (unilateral/atypical: pediatric patients 20/15%, adults 7/7%). We identified a wide range of associated diseases. Overall, 84 and 8% of our cohort presented initially with ischemic and hemorrhagic manifestation, respectively. The hemorrhagic manifestation of quasi-MMD occurred however only in adults. Angiographic analysis revealed steno-occlusive involvement of the posterior circulation (in addition to the anterior circulation) in 31% with a higher involvement in pediatric patients (40%) compared to adults (27%). Conclusions: The characterization of our European Caucasian cohort reveals several differences when compared to reported Asian quasi-MMD cohorts and also compared to European Caucasian MMD cohort. We conclude that quasi-MMD represents a distinct disease with different ethnic clinical features.

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