460 lines
35 KiB
Markdown
460 lines
35 KiB
Markdown
---
|
|
title: "Moyamoya"
|
|
docid: "e15385dc-824d-431a-8df0-2b28bf909a2d"
|
|
authors:
|
|
- key: "47381de4-c9fd-4999-8dd0-1808cd72db6b"
|
|
value: "Luke L. Linscott, MD"
|
|
breadcrumbs:
|
|
-
|
|
name: "Pediatrics"
|
|
slug: "pediatrics"
|
|
treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4"
|
|
-
|
|
name: "Diagnosis"
|
|
slug: "diagnosis"
|
|
treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026"
|
|
-
|
|
name: "Pediatric Neuroradiology"
|
|
slug: "pediatric-neuroradiology"
|
|
treeNodeId: "d0eb8f4a-e769-43dd-896c-8c9c27ce8759"
|
|
-
|
|
name: "Brain"
|
|
slug: "brain"
|
|
treeNodeId: "feaaadba-649b-4f0a-9aad-9188a8f9926a"
|
|
-
|
|
name: "Pathology-Based Diagnoses"
|
|
slug: "pathology-based-diagnoses"
|
|
treeNodeId: "2d26053f-23a7-4062-bf35-a93775ae1209"
|
|
-
|
|
name: "Stroke"
|
|
slug: "stroke"
|
|
treeNodeId: "83689efc-5f25-40a3-9ae7-06fb2a4a069f"
|
|
-
|
|
name: "Moyamoya"
|
|
slug: "moyamoya"
|
|
treeNodeId: null
|
|
category: "Pediatrics"
|
|
cmeTopicId: "8ba35550-ab01-4c3c-a79f-25aaa06da86c"
|
|
documentVersionId: "635703ac-cce9-46c4-a51b-3a94faa9236a"
|
|
imageCount: 25
|
|
lastUpdated: "02/07/24"
|
|
pageDescription: "Moyamoya"
|
|
pageKeywords: "Pediatrics, Diagnosis, Pediatric Neuroradiology, Brain, Pathology-Based Diagnoses, Stroke, Moyamoya"
|
|
pageTitle: "Moyamoya | STATdx"
|
|
enhancedTitle: "Moyamoya"
|
|
type: "DX"
|
|
references: true
|
|
breadcrumbs:
|
|
- "Pediatrics"
|
|
- "Diagnosis"
|
|
- "Pediatric Neuroradiology"
|
|
- "Brain"
|
|
- "Pathology-Based Diagnoses"
|
|
- "Stroke"
|
|
- "Moyamoya"
|
|
---
|
|
# KEY FACTS
|
|
|
|
- ## Terminology
|
|
|
|
|
|
- Progressive narrowing of distal internal carotid artery (ICA) & proximal circle of Willis (COW) vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography
|
|
- Moyamoya disease = primary (idiopathic) moyamoya
|
|
- More common in Japan, Korea
|
|
- Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) due to other disorders
|
|
- ## Imaging
|
|
|
|
|
|
- Absent or narrowed distal ICA & abnormal COW
|
|
- Excessive tiny collaterals in basal ganglia & cisterns
|
|
- "Puff of smoke" (moyamoya in Japanese) of lenticulostriate & thalamoperforator collaterals
|
|
- Prominent collaterals in sulci
|
|
- Ivy sign on FLAIR & T1 C+ MR
|
|
- Acute & chronic infarcts
|
|
- CT/CTA: Acute use for ischemia or hemorrhage
|
|
- MR C+/MRA: Vascular protocol with DWI & perfusion
|
|
- DWI: Helpful to identify "acute on chronic" injury
|
|
- ## Pathology
|
|
|
|
|
|
- Moyamoya disease: Inherited idiopathic disorder
|
|
- Moyamoya arteriopathy: Secondary process
|
|
- Sickle cell disease, trisomy 21, neurofibromatosis type 1, radiation therapy, Alagille syndrome, morning glory syndrome, TB meningitis, among others
|
|
- ## Clinical Issues
|
|
|
|
|
|
- Bimodal age peaks: 6 & 35 years
|
|
- Most frequent cause of stroke in Asian children
|
|
- Presentation (children): Transient ischemic attacks (TIAs), alternating hemiplegia (exacerbated by crying), headache
|
|
- Presentation (adults): TIAs, hemorrhage (~ 30%), & cerebral infarct
|
|
- Prognosis depends on etiology, ability to form collaterals, age/stage at diagnosis
|
|
- Treatment: Indirect (more common in children) or direct (more common in adults) vascular bypass
|
|
- ## Diagnostic Checklist
|
|
|
|
|
|
- Seek underlying causes of secondary moyamoya
|
|
|
|
# TERMINOLOGY
|
|
|
|
- ## Synonyms
|
|
|
|
|
|
- Progressive stenoocclusive arteriopathy; spontaneous occlusion of circle of Willis (COW)
|
|
- ## Definitions
|
|
|
|
|
|
- Progressive narrowing of distal internal carotid artery (ICA) & proximal COW vessels → characteristic adjacent clusters of collateral flow appearing as "puff of smoke" on real-time angiography
|
|
- Moyamoya disease: Primary (idiopathic) moyamoya
|
|
- More common in Japan, Korea
|
|
- Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya) occurs in association with other disorders or after radiation treatment
|
|
|
|
# IMAGING
|
|
|
|
- ## General Features
|
|
|
|
|
|
- Best diagnostic clue: Multiple enhancing punctate dots (CECT) & flow voids (MR) in basal ganglia & cisterns
|
|
- Arterial occlusions: Distal ICA, COW, branches
|
|
- Anterior > posterior circulation
|
|
- Posterior circulation affected in ~ 25%
|
|
- Leads to prominent clusters of nearby collaterals
|
|
- "Cloud-like" lenticulostriate & thalamoperforator collaterals on angiography: "Puff of smoke" (moyamoya in Japanese)
|
|
- Also leads to prominent sulcal collaterals distally
|
|
- ## CT Findings
|
|
|
|
|
|
- ### NECT
|
|
|
|
|
|
- Children: Acute ischemia ± old infarcts
|
|
- Older children/adults: Usually ischemia but may present with intracranial hemorrhage
|
|
- CTA: Abnormal COW + basilar net-like collaterals
|
|
- Xe-133 CT: ↓ cerebral reserve with acetazolamide challenge
|
|
- ## MR Findings
|
|
|
|
|
|
- ### T2WI
|
|
|
|
|
|
- ↑ signal in gliotic areas from prior infarcts
|
|
- Collateral vessels: Net-like cisternal flow voids
|
|
- ### FLAIR
|
|
|
|
|
|
- Bright sulci = leptomeningeal ivy sign
|
|
- Slow-flowing engorged pial collateral vessels, thickened arachnoid membranes
|
|
- Correlates with ↓ cerebral vascular reserve
|
|
- ### T2* GRE
|
|
|
|
|
|
- Hemosiderin if prior hemorrhage
|
|
- ### DWI
|
|
|
|
|
|
- Very useful for "acute on chronic" infarcts
|
|
- ### PWI
|
|
|
|
|
|
- ↓ cerebral blood flow (CBF) (ASL) in affected territories
|
|
- ↑ MTT in affected territories
|
|
- Variable rCBV depending on degree of collateral formation
|
|
- May be used to measure response to revascularization
|
|
- ### T1WI C+
|
|
|
|
|
|
- Lenticulostriate collaterals → enhancing "dots" in basal ganglia & net-like thin vessels in cisterns
|
|
- Leptomeningeal enhancement (ivy sign)
|
|
- Vessel wall imaging
|
|
- Most consistent finding is negative remodeling (local shrinkage of vessel size) of affected vessels
|
|
- Variable enhancement of affected vessel segments
|
|
- ↑ wall thickening and ↑ stenosis correlates with ↑ wall enhancement
|
|
- May help distinguish from other vasculopathies
|
|
- ### MRA
|
|
|
|
|
|
- Narrowed/occluded distal ICA & COW vessels
|
|
- ### MRS
|
|
|
|
|
|
- Lactate in acutely infarcted tissue
|
|
- NAA:Cr & Cho:Cr ratios in frontal white matter improve ↑ after revascularization
|
|
- ## Ultrasonographic Findings
|
|
|
|
|
|
- Grayscale: Reduction of ICA lumen size
|
|
- Pulsed Doppler
|
|
- Spectral waveforms in ICA show no flow (occluded) or proximal high-resistance flow pattern
|
|
- ↑ end-diastolic flow velocity, ↓ vascular resistance in external carotid artery (ECA) collaterals
|
|
- Color Doppler: Aliasing suggests stenoses
|
|
- Power Doppler: Improves visualization of slow-flow stenotic vessels & collaterals
|
|
- Can be used for vessel mapping prior to revascularization surgery
|
|
- ## Angiographic Findings
|
|
|
|
|
|
- Predominantly (not exclusively) anterior circulation
|
|
- Narrow proximal COW & ICA (early phase)
|
|
- Lenticulostriate & thalamoperforator collaterals (intermediate phase)
|
|
- Transdural/transosseous ECA-ICA collaterals (late phase)
|
|
- Dilation & branch extension of anterior choroidal artery predict adult hemorrhagic events
|
|
- ## Nuclear Medicine Findings
|
|
|
|
|
|
- PET: ↓ hemodynamic reserve capacity
|
|
- SPECT I-123-iomazenil: Neuronal density preserved if asymptomatic, ↓ if symptomatic
|
|
- ## Imaging Recommendations
|
|
|
|
|
|
- Best imaging tool: MR C+/MRA
|
|
- Contrast improves detection: Collaterals, synangiosis
|
|
- Catheter angiography defines anatomy prior to bypass
|
|
- Protocol advice
|
|
- Acetazolamide challenge with ASL has been performed to measure cerebrovascular reserve, but Xe-133 CT is gold standard
|
|
|
|
# DIFFERENTIAL DIAGNOSIS
|
|
|
|
- ## Ivy Sign
|
|
|
|
|
|
- Leptomeningeal metastases, subarachnoid hemorrhage, meningitis, ↑ inspired oxygen, collateral veins of Sturge-Weber or other chronic venous occlusion
|
|
- [Large Vessel Inflammatory Vasculitis](/document/miscellaneous-vasculitis/5a4d4cbd-67e3-4722-8a44-8d411cbb98f0)
|
|
- Postvaricella vasculitis, lupus, & other CNS vasculitides
|
|
- May be reversible with treatment
|
|
- [Cerebral Arterial Atherosclerosis](/document/intracranial-atherosclerosis/8d21d962-9c43-47bd-b995-c73f20e46b47)
|
|
- Very rare in children & young adults
|
|
- ## Severely Attenuated Circle of Willis
|
|
|
|
|
|
- Subarachnoid hemorrhage (spasm), meningitis, tumor encasement
|
|
|
|
# PATHOLOGY
|
|
|
|
- ## General Features
|
|
|
|
|
|
- ### Etiology
|
|
|
|
|
|
- Moyamoya disease
|
|
- Inherited polygenic or autosomal dominant
|
|
- Low penetrance
|
|
- Gene loci: 3p26-p24.2, 17q25, 8q23
|
|
- ↑ in growth factors, cytokines, adhesion molecules in CSF implicates inflammation
|
|
- Moyamoya arteriopathy (a.k.a. moyamoya syndrome or secondary moyamoya)
|
|
- Sickle cell disease, neurofibromatosis type 1 (NF1), radiation therapy, trisomy 21, Alagille syndrome, morning glory syndrome, tuberculous meningitis, many others
|
|
- NF1 + suprasellar tumor + radiation can be disastrous
|
|
- Epidemiology: Moyamoya disease
|
|
- Incidence in Japan: 1:100,000
|
|
- Incidence in North America, Europe: 0.1:100,000
|
|
- 10-15% familial
|
|
- ## Staging, Grading, & Classification
|
|
|
|
|
|
- Staging criteria (Suzuki)
|
|
- Stage 1: Narrowing of ICA bifurcation
|
|
- Stage 2: Anterior, middle, and posterior cerebral arteries (ACA, MCA, PCA) dilated
|
|
- Stage 3: Maximal basal collaterals; small ACA/MCA
|
|
- Stage 4: Fewer collaterals (vessels); small PCA
|
|
- Stage 5: Further ↓ in collaterals; absent ACA/MCA/PCA
|
|
- Stage 6: Extensive ECA-pial collaterals
|
|
- ## Gross Pathologic & Surgical Features
|
|
|
|
|
|
- ↑ perforating (early) & ECA-ICA (late) collaterals in atrophic brain
|
|
- Hemorrhage (subarachnoid, intraventricular > parenchymal) in adults
|
|
- ↑ saccular aneurysms in adults (especially basilar)
|
|
- ## Microscopic Features
|
|
|
|
|
|
- Intimal thickening & hyperplasia
|
|
- Excessive infolding & thickening of internal elastic lamina
|
|
- Periventricular pseudoaneurysms (cause of hemorrhage)
|
|
|
|
# CLINICAL ISSUES
|
|
|
|
- ## Presentation
|
|
|
|
|
|
- ### Most common signs/symptoms
|
|
|
|
|
|
- Children: Transient ischemic attacks (TIAs), alternating hemiplegia (exacerbated by crying), headache
|
|
- Adults: TIAs, hemorrhage (~ 30%), cerebral infarct
|
|
- Hemorrhagic presentation more common in Asian adults
|
|
- ### Other signs/symptoms
|
|
|
|
|
|
- Children: Developmental delay, poor feeding, chorea
|
|
- ## Demographics
|
|
|
|
|
|
- ### Age
|
|
|
|
|
|
- Bimodal age peaks
|
|
- Japan, Korea: 6 years > 35 years
|
|
- North America, Europe: 35 years > 6 years
|
|
- ### Sex
|
|
|
|
|
|
- M:F = 1:1.8; in familial cases, M:F = 1:5
|
|
- ### Epidemiology
|
|
|
|
|
|
- Most frequent cause of stroke in Asian children
|
|
- ## Natural History & Prognosis
|
|
|
|
|
|
- Progressive narrowing, collateralization, & ischemia
|
|
- Prognosis depends on etiology, ability to form collaterals, age/stage at diagnosis
|
|
- Pediatric cases usually advance to stage 5 in < 10 years
|
|
- Infantile moyamoya progresses faster
|
|
- Hemorrhagic moyamoya more common in older patients
|
|
- May be due to large collateral vessels
|
|
- Has poorer outcome
|
|
- ## Treatment
|
|
|
|
|
|
- Aspirin therapy
|
|
- Direct bypass: Superficial temporal artery (STA)-MCA more common in adults
|
|
- Indirect bypass
|
|
- Pial synangiosis & encephaloduroarteriosynangiosis with STA more common in children
|
|
- 5-year risk of ipsilateral stroke post encephaloduroarteriosynangiosis = 15%
|
|
- Dural inversion with middle meningeal artery
|
|
- Correct/control prothrombotic states & inflammatory etiologies
|
|
- Transfusion therapy for sickle cell-related moyamoya
|
|
|
|
# DIAGNOSTIC CHECKLIST
|
|
|
|
- ## Consider
|
|
|
|
|
|
- Seek underlying causes of secondary moyamoya
|
|
- ## Image Interpretation Pearls
|
|
|
|
|
|
- Enhanced asymmetric atrophy found on childhood CT; look for abnormal vascular pattern
|
|
- Adult moyamoya can present with intracranial hemorrhage
|
|
- ## Reporting Tips
|
|
|
|
|
|
- Successful revascularization = ↓ basal collaterals, ↑ flow in MCA branches, ↑ caliber of STA (direct bypass)
|
|
|
|
4447a660-ce0e-4510-a9b1-a3c441465e85
|
|
|
|
## References
|
|
|
|
# Selected References
|
|
|
|
1. [Larson AS et al: Vessel wall imaging features of moyamoya disease in a North American population: patterns of negative remodelling, contrast enhancement, wall thickening, and stenosis. BMC Med Imaging. 22(1):198, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36397005%5Bpmid%5D)
|
|
1. [Larson AS et al: Implementation and rationale for a unified clinical and imaging protocol for evaluation and treatment of moyamoya angiopathy: a single institutional experience. Front Neurol. 12:662393, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34079514%5Bpmid%5D)
|
|
1. [Wang LX et al: Ivy sign in moyamoya disease: a comparative study of the FLAIR vascular hyperintensity sign against contrast-enhanced MRI. AJNR Am J Neuroradiol. 42(4):694-700, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33664105%5Bpmid%5D)
|
|
1. [Fan AP et al: Identifying hypoperfusion in moyamoya disease with arterial spin labeling and an [15O]-water positron emission tomography/magnetic resonance imaging normative database. Stroke. 50(2):373-80, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30636572%5Bpmid%5D)
|
|
1. [Lehman VT et al: Contemporary and emerging magnetic resonance imaging methods for evaluation of moyamoya disease. Neurosurg Focus. 47(6):E6, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31786551%5Bpmid%5D)
|
|
1. [Lee S et al: Monitoring cerebral perfusion changes after revascularization in patients with moyamoya disease by using arterial spin-labeling MR imaging. Radiology. 288(2):565-72, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29714677%5Bpmid%5D)
|
|
1. [Kim DY et al: Infarct pattern and collateral status in adult moyamoya disease: a multimodal magnetic resonance imaging study. Stroke. 48(1):111-6, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27909201%5Bpmid%5D)
|
|
1. [Qiao PG et al: Clinical assessment of cerebral hemodynamics in moyamoya disease via multiple inversion time arterial spin labeling and dynamic susceptibility contrast-magnetic resonance imaging: a comparative study. J Neuroradiol. 44(4):273-80, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28168990%5Bpmid%5D)
|
|
1. [Blauwblomme T et al: Cerebral blood flow improvement after indirect revascularization for pediatric moyamoya disease: a statistical analysis of arterial spin-labeling MRI. AJNR Am J Neuroradiol. 37(4):706-12, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26585258%5Bpmid%5D)
|
|
1. [Kim JS: Moyamoya disease: epidemiology, clinical features, and diagnosis. J Stroke. 18(1):2-11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26846755%5Bpmid%5D)
|
|
1. [Mossa-Basha M et al: Added value of vessel wall magnetic resonance imaging in the differentiation of moyamoya vasculopathies in a non-Asian cohort. Stroke. 47(7):1782-8, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27272486%5Bpmid%5D)
|
|
1. [Takagi Y et al: Histopathological characteristics of distal middle cerebral artery in adult and pediatric patients with moyamoya disease. Neurol Med Chir (Tokyo). 56(6):345-9, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27087193%5Bpmid%5D)
|
|
1. [Titsworth WL et al: National analysis of 2454 pediatric moyamoya admissions and the effect of hospital volume on outcomes. Stroke. 47(5):1303-11, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=27048697%5Bpmid%5D)
|
|
1. [Griessenauer CJ et al: Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease. J Neurosurg Pediatr. 16(1):64-73, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25837886%5Bpmid%5D)
|
|
1. [Derdeyn CP: Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke. 45(5):1245-6, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24668205%5Bpmid%5D)
|
|
1. [Ryoo S et al: High-resolution magnetic resonance wall imaging findings of moyamoya disease. Stroke. 45(8):2457-60, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24947295%5Bpmid%5D)
|
|
1. [Hishikawa T et al: Assessment of the difference in posterior circulation involvement between pediatric and adult patients with moyamoya disease. J Neurosurg. 119(4):961-5, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23909250%5Bpmid%5D)
|
|
1. [Noguchi T et al: Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging findings. Eur J Radiol. 82(12):e840-7, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24055185%5Bpmid%5D)
|
|
1. [Currie S et al: Childhood moyamoya disease and moyamoya syndrome: a pictorial review. Pediatr Neurol. 44(6):401-13, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21555050%5Bpmid%5D)
|
|
1. [Mugikura S et al: Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis. AJNR Am J Neuroradiol. 32(1):192-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20801761%5Bpmid%5D)
|
|
1. [Ibrahimi DM et al: Moyamoya disease in children. Childs Nerv Syst. 26(10):1297-308, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20607248%5Bpmid%5D)
|
|
1. [Kim SK et al: Pediatric moyamoya disease: an analysis of 410 consecutive cases. Ann Neurol. 68(1):92-101, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20582955%5Bpmid%5D)
|
|
1. [Mori N et al: The leptomeningeal "ivy sign" on fluid-attenuated inversion recovery MR imaging in moyamoya disease: a sign of decreased cerebral vascular reserve? AJNR Am J Neuroradiol. 30(5):930-5, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19246527%5Bpmid%5D)
|
|
1. [Park TS: Moyamoya disease in children. Neurosurg Focus. 24(2):E16a; discussion E16a, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18275293%5Bpmid%5D)
|
|
|
|
|
|
## Images
|
|
|
|
|
|
### Selected Images
|
|
|
|
|
|
*Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.*
|
|
|
|
|
|
*Axial DWI MR in a 13-month-old with increasing seizures shows left MCA distribution ischemia as well as a remote infarct in the right MCA territory. This is a typical acute on chronic ischemic pattern of moyamoya.*
|
|
|
|
|
|
*Anterior 3D TOF MRA in the same patient at 8 years of age shows occlusions of the terminal ICAs <img src='img/arrows/CS.png'/>, absence of the MCAs, & numerous lenticulostriate collaterals <img src='img/arrows/WS.png'/> forming a "puff of smoke." The PCAs are also occluded. Note the enlarged ECA collaterals <img src='img/arrows/CO.png'/> status post synangiosis & dural inversion.*
|
|
|
|
|
|
*Axial 3D TOF MRA in a 5-year-old with idiopathic moyamoya arteriopathy shows multiple small lenticulostriate <img src='img/arrows/CS.png'/> and thalamostriate <img src='img/arrows/CC.png'/> vessels within the deep nuclear structures.*
|
|
|
|
|
|
*Axial FLAIR MR in the same patient shows areas of gliosis <img src='img/arrows/CO.png'/> from vascular insufficiency, linear hyperintensities <img src='img/arrows/WS.png'/> representing deep medullary collateral vessels, and hyperintensities <img src='img/arrows/CC.png'/> conforming to the surface of the sulci representing pial collaterals (the leptomeningeal ivy sign).*
|
|
|
|
|
|
*Axial TOF MRA in a 14-year-old with neurofibromatosis type 1 shows absence of the right internal carotid terminus & MCA. In the expected location of the carotid terminus & MCA, there are multiple small leptomeningeal collaterals <img src='img/arrows/CS.png'/>.*
|
|
|
|
|
|
*Axial FLAIR MR in a 21-year-old with sickle cell disease shows high signal <img src='img/arrows/CS.png'/> within the right MCA distribution sulci, the so-called ivy sign. This abnormal signal corresponds to engorged pial collateral vessels & is often seen in moyamoya.*
|
|
|
|
|
|
*Frontal 3D TOF MRA in a 10-year-old with history of nasal rhabdomyosarcoma at age 3 treated with XRT shows absence of flow in the left distal ICA <img src='img/arrows/CO.png'/>, severe narrowing of the right MCA <img src='img/arrows/CS.png'/>, & absent right ACA. Terminal ICA & proximal MCA are the most common locations for moyamoya arteriopathy.*
|
|
|
|
|
|
*Axial T1 C+ MR in the same patient shows extensive leptomeningeal enhancement <img src='img/arrows/CO.png'/> in the bilateral ICA territories with prominent vessel enhancement <img src='img/arrows/CS.png'/> in the basal ganglia.*
|
|
|
|
|
|
*3D TOF MRA in a 6-year-old with trisomy 21 and severe moyamoya arteriopathy shows marked narrowing of the left ICA terminus <img src='img/arrows/CS.png'/> & complete occlusion of the right MCA origin <img src='img/arrows/CO.png'/>. Deep moyamoya collaterals <img src='img/arrows/CC.png'/> have formed in the left basal ganglia.*
|
|
|
|
|
|
*Axial ASL MR perfusion in the same patient shows near absence of signal <img src='img/arrows/CS.png'/> in the right MCA territory, consistent with dramatically reduced blood flow, corroborating the findings on MRA.*
|
|
|
|
|
|
### Additional Images
|
|
|
|
|
|
*Coronal graphic shows severe tapering of both distal ICAs <img src='img/arrows/WS.png'/> & strikingly enlarged lenticulostriate arteries <img src='img/arrows/WO.png'/> coursing through the basal ganglia. This is the characteristic puff of smoke (moyamoya) pattern.*
|
|
|
|
|
|
*Axial FLAIR MR in an 8-year-old boy with Alagille syndrome shows confluent gliosis <img src='img/arrows/CS.png'/> in the right frontal region & more patchy gliosis <img src='img/arrows/WS.png'/> in the left deep white matter. Also note the serpentine collateral vessels <img src='img/arrows/CO.png'/> near the midline. Moyamoya arteriopathy can be seen with Alagille syndrome.*
|
|
|
|
|
|
*Axial TOF MRA in an 8-year-old boy with Alagille syndrome shows absent signal in the region of the carotid termini & major ICA branch vessels. There are, however, small leptomeningeal collateral vessels in the midline inferior frontal <img src='img/arrows/CO.png'/> & right anterior temporal regions <img src='img/arrows/CS.png'/> & basilar cisterns <img src='img/arrows/CC.png'/>. The findings are compatible with moyamoya arteriopathy.*
|
|
|
|
|
|
*Anterior projection from a left ICA DSA injection shows no opacification of the expected ACA branches with multiple leptomeningeal collaterals seen in the midline subfrontal region <img src='img/arrows/CS.png'/>. Also note the enlarged left middle meningeal artery <img src='img/arrows/CO.png'/>, which supplies portions of the right ACA territory. This is an example of an ICA to ECA collateral pathway.*
|
|
|
|
|
|
*Axial T2 MR in a 21-year-old man with sickle cell disease shows absence of the carotid termini & major branches. Note the numerous small leptomeningeal collateral vessels <img src='img/arrows/CS.png'/> within the basilar cisterns, typical of moyamoya arteriopathy.*
|
|
|
|
|
|
*Axial MRA in a 21-year-old man with sickle cell disease shows multiple punctate foci of flow-related signal <img src='img/arrows/CS.png'/> within the basal ganglia & thalami, consistent with typical lenticulostriate & thalamoperforator collateral vessels of moyamoya arteriopathy.*
|
|
|
|
|
|
*Axial T1 C+ MR in a 21-year-old man with sickle cell disease shows abnormal leptomeningeal enhancement <img src='img/arrows/CS.png'/> in the right MCA distribution. This is the equivalent of the ivy sign on FLAIR & represents slow-flowing leptomeningeal collaterals that develop in areas of decreased cerebrovascular reserve (secondary to moyamoya arteriopathy).*
|
|
|
|
|
|
*3D MRA in a 16-year-old boy with moyamoya arteriopathy demonstrates severe stenosis/occlusion of the right carotid terminus <img src='img/arrows/WO.png'/>. The patient is status post revascularization using a direct bypass. Note the prominent right superficial temporal artery <img src='img/arrows/CS.png'/>, which is anastomosed to a distal right MCA branch <img src='img/arrows/CO.png'/>.*
|
|
|
|
|
|
*Lateral view of a selective internal carotid DSA shows severe stenosis of the supraclinoid ICA <img src='img/arrows/BS.png'/> with a "puff of smoke" <img src='img/arrows/BO.png'/> from the collateral lenticulostriate vessels.*
|
|
|
|
|
|
*Axial TOF MRA shows occlusion of both distal ICAs <img src='img/arrows/BS.png'/>, nonvisualization of the MCAs & ACAs, & stenosis of the PCAs <img src='img/arrows/BO.png'/> in an 8-year-old with hemiparetic migraines. This patient had an idiopathic arteriopathy of childhood.*
|
|
|
|
|
|
*Axial TOF MRA in another patient with idiopathic progressive arteriopathy of childhood shows occluded supraclinoid ICAs <img src='img/arrows/WS.png'/>. Note the bilateral synangiosis <img src='img/arrows/BO.png'/>.*
|
|
|
|
|
|
*Axial T1 MR shows right frontal & left temporooccipital atrophy <img src='img/arrows/WO.png'/> from remote ischemia. There are multiple small basal ganglia flow voids <img src='img/arrows/WS.png'/> from lenticulostriate collaterals.*
|
|
|
|
|
|
*Axial T1 C+ MR in the same patient shows multifocal white "dots" <img src='img/arrows/WS.png'/> due to slow flow in multiple enlarged lenticulostriate collaterals.*
|
|
|
|
|
|
*Axial TOF MRA in a 2-year-old boy with moyamoya and multiple infarcts shows proliferation of small vessels in the thalamus.*
|
|
|
|
|
|
*Axial FLAIR MR in the same patient shows multiple wedge-shaped areas of cortical signal abnormality <img src='img/arrows/CS.png'/>, consistent with multifocal infarcts.*
|
|
|