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Pt presented with thalamic hemorrhage and ct angio dhowed

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Moyamoya disease CT angiogram cerebral vessels puff of smoke collaterals

This diagnostic image is a cerebral angiogram (CT angiography) at the level of the circle of Willis, illustrating a classic presentation of Moyamoya disease. The visual highlights a proximal obliteration or severe stenosis of the right middle cerebral artery (MCA), marked by black arrows. Distal to the site of occlusion, there is a dense, hazy network of fine collateral vessels. This cluster of small, anastomotic vessels creates the pathognomonic 'puff of smoke' (moyamoya) appearance, which develops as a compensatory mechanism for the chronic, progressive narrowing of the major intracranial arteries. The image demonstrates significant vascular remodeling and diminished vessel caliber in the primary arterial territory. This finding is clinically significant for the evaluation of pediatric stroke and ischemic risk, representing a primary vasculopathy that affects the internal carotid arteries and their branches.

This diagnostic image is a cerebral angiogram (CT angiography) at the level of the circle of Willis, illustrating a classic presentation of Moyamoya disease. The visual highlights a proximal obliteration or severe stenosis of the right middle cerebral artery (MCA), marked by black arrows. Distal to the site of occlusion, there is a dense, hazy network of fine collateral vessels. This cluster of small, anastomotic vessels creates the pathognomonic 'puff of smoke' (moyamoya) appearance, which develops as a compensatory mechanism for the chronic, progressive narrowing of the major intracranial arteries. The image demonstrates significant vascular remodeling and diminished vessel caliber in the primary arterial territory. This finding is clinically significant for the evaluation of pediatric stroke and ischemic risk, representing a primary vasculopathy that affects the internal carotid arteries and their branches.

This diagnostic image shows a 3D reconstructed CT angiogram (CTA) of the cerebral vasculature in two views (a and b), highlighting a complex vascular network characteristic of Moyamoya disease (MMD). The reconstruction demonstrates significant stenosis of the distal internal carotid artery (ICA) and proximal segments of the anterior and middle cerebral arteries (ACA/MCA). A prominent, dense network of tortuous, 'puff-of-smoke' collateral vessels is visible in the basal regions, compensating for the occlusive disease. A distinctive white arrow in both views points to a large, saccular aneurysm (approximately 7mm) originating from a fragile collateral vessel near the terminal branches of the ACA. The aneurysm exhibits a wide neck and irregular morphology, appearing as a significant outpouching compared to the slender parent vessel. The images illustrate the increased hemodynamic stress placed on collateral pathways in MMD, leading to the formation and rapid growth of secondary aneurysms, which pose a high risk for intracerebral hemorrhage.

This diagnostic image shows a 3D reconstructed CT angiogram (CTA) of the cerebral vasculature in two views (a and b), highlighting a complex vascular network characteristic of Moyamoya disease (MMD). The reconstruction demonstrates significant stenosis of the distal internal carotid artery (ICA) and proximal segments of the anterior and middle cerebral arteries (ACA/MCA). A prominent, dense network of tortuous, 'puff-of-smoke' collateral vessels is visible in the basal regions, compensating for the occlusive disease. A distinctive white arrow in both views points to a large, saccular aneurysm (approximately 7mm) originating from a fragile collateral vessel near the terminal branches of the ACA. The aneurysm exhibits a wide neck and irregular morphology, appearing as a significant outpouching compared to the slender parent vessel. The images illustrate the increased hemodynamic stress placed on collateral pathways in MMD, leading to the formation and rapid growth of secondary aneurysms, which pose a high risk for intracerebral hemorrhage.

This diagnostic image is a cerebral angiogram (digital subtraction angiography) illustrating significant neurovascular pathology in an 18-year-old patient. The image shows the right internal carotid artery and middle cerebral artery (MCA) territory. There is a near-total to complete occlusion of the right M1 segment of the MCA, occurring just distal to its origin. Characteristic of Moyamoya disease or syndrome, a dense, hazy network of fine, thread-like collateral vessels—known as 'puff of smoke' or Moyamoya collaterals—is visible, attempting to reform the distal M1 segment through small perforating arteries. Comparison reveals a reduced caliber of the right internal carotid artery relative to the contralateral side, suggesting proximal stenosis. The peripheral vasculature appears as a complex, delicate web of compensatory vessels. This finding is clinically significant for explaining ischemic events and stroke in young patients, particularly in the context of hyperhomocysteinemia or idiopathic Moyamoya. The image serves as a classic educational example of intracranial arterial stenosis with resultant secondary collateralization.

This diagnostic image is a cerebral angiogram (digital subtraction angiography) illustrating significant neurovascular pathology in an 18-year-old patient. The image shows the right internal carotid artery and middle cerebral artery (MCA) territory. There is a near-total to complete occlusion of the right M1 segment of the MCA, occurring just distal to its origin. Characteristic of Moyamoya disease or syndrome, a dense, hazy network of fine, thread-like collateral vessels—known as 'puff of smoke' or Moyamoya collaterals—is visible, attempting to reform the distal M1 segment through small perforating arteries. Comparison reveals a reduced caliber of the right internal carotid artery relative to the contralateral side, suggesting proximal stenosis. The peripheral vasculature appears as a complex, delicate web of compensatory vessels. This finding is clinically significant for explaining ischemic events and stroke in young patients, particularly in the context of hyperhomocysteinemia or idiopathic Moyamoya. The image serves as a classic educational example of intracranial arterial stenosis with resultant secondary collateralization.

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I now have comprehensive data to give a full clinical analysis. Here is a detailed breakdown:

CT Cerebral Angiogram Report - Analysis

Patient: A Dhana Lakshmi | 38F | 27-07-2026 Center: Sai Balaji Medimage, Guntur

What the Report Shows

Anterior circulation (the critical finding):
  • Significantly reduced calibre of bilateral ICA throughout all intracranial segments
  • Non-visualization of cavernous and supracavernous segments of bilateral ICA, bilateral MCA, and bilateral ACA
  • Multiple pial-pial collateral circulation - this is the hallmark "puff of smoke" appearance
Posterior circulation: Normal - vertebrals, basilar, and major branches intact; no aneurysm or AVM
Brain findings:
  • Acute intraparenchymal hematoma in the left thalamus, 25 x 22 mm
  • Mass effect: sulcal space effacement; no midline shift
  • Intraventricular extension into bilateral lateral ventricles, 3rd and 4th ventricles
  • Mild obstructive hydrocephalus with periventricular seepage (transependymal CSF flow)
Impression: Moyamoya disease - catheter angiogram suggested for confirmation

Moyamoya Disease - Clinical Context

Definition: Moyamoya (Japanese: "puff of smoke") is a chronic, progressive, non-atherosclerotic, non-inflammatory intracranial vasculopathy of unknown cause. It involves fibrocellular intimal thickening and smooth muscle cell proliferation causing stenosis of the supraclinoid ICA bilaterally, extending to proximal MCA and ACA. As the main vessels occlude, an extensive compensatory network of perforating and anastomotic collateral vessels develops at the base of the brain - these look like a "puff of smoke" on angiography. - Bradley and Daroff's Neurology in Clinical Practice
Classic "Puff of Smoke" DSA in Moyamoya - left ICA occlusion with dense collateral network
Why this patient fits Moyamoya:
FeatureThis PatientMoyamoya Characteristic
Age38FBimodal peaks: 1st and 4th decades; adult onset = hemorrhagic type
Bilateral ICA stenosis/occlusionYes - bilateral, supracavernousPathognomonic
MCA + ACA non-visualizationYes, bilateralTypical extension
Pial-pial collateralsYes"Puff of smoke" vessels
Posterior circulation normalYesClassic pattern
Thalamic hemorrhageYesAdult Moyamoya bleeds in basal ganglia/thalamus
No aneurysm/AVMConfirmedHemorrhage from fragile collateral rupture

Why Thalamic Hemorrhage in Moyamoya?

In adult Moyamoya, hemorrhage is the dominant presentation (unlike children where ischemia predominates). The fragile, thin-walled collateral vessels (lenticulostriate perforators, choroidal arteries) bear high hemodynamic stress from compensating for occluded main trunks. These microaneurysm-prone vessels rupture, causing parenchymal hemorrhage preferentially in the basal ganglia and thalamus. - Adams and Victor's Principles of Neurology, 12th Ed.
The 25x22 mm left thalamic hematoma with IVH into all four ventricles and resulting mild hydrocephalus represents a serious, high-risk hemorrhagic presentation.

Suzuki's Angiographic Staging (for reference)

This patient likely corresponds to a late stage (Stage 4-5) given bilateral near-total occlusion of anterior circulation with established collateral network:
  1. Carotid fork stenosis
  2. Appearance of moyamoya vessels at base of brain
  3. Intensification of moyamoya vessels
  4. Minimization of moyamoya vessels
  5. Reduction of moyamoya vessels
  6. Disappearance of moyamoya vessels (only ECA collaterals remain)

Immediate Management Priorities

Acute phase (this admission):
  1. Hemorrhage management: Reverse any coagulopathy; target SBP <160 mmHg; INR correction if needed
  2. Hydrocephalus monitoring: Given IVH into all 4 ventricles + mild hydrocephalus + periventricular seepage - neurosurgical consult for possible EVD (external ventricular drain) if ICP rises or clinical deterioration occurs
  3. NO anticoagulation - contraindicated in hemorrhagic Moyamoya; also avoid aggressive antiplatelet agents acutely
  4. Seizure prophylaxis: Consider given thalamic location and mass effect
  5. ICU-level monitoring: GCS, pupillary responses, ICP signs
Diagnostic next steps:
  • Catheter (DSA) angiogram - rightly recommended by radiologist; gold standard for definitive Moyamoya diagnosis, Suzuki staging, and surgical planning
  • MRI brain with DWI, FLAIR, T2* (GRE/SWI) to characterize hemorrhage extent and identify any ischemic territories
Definitive/long-term:
  • Surgical revascularization is the mainstay for Moyamoya - STA-MCA (superficial temporal artery to MCA) direct bypass, indirect procedures (EDAS - encephaloduroarteriosynangiosis), or combined
  • This reduces rebleeding risk by relieving hemodynamic stress on fragile collateral vessels
  • According to a 2023 AHA/ASA Scientific Statement (Gonzalez et al., Stroke) and a 2025 review in Neurology (Sun et al.), revascularization is recommended for symptomatic Moyamoya; evidence for hemorrhagic type is less definitive but revascularization is generally favored

Key Clinical Red Flags in This Patient

  • Bilateral disease (both hemispheres at risk)
  • Young adult female - higher rebleeding risk if untreated
  • IVH with hydrocephalus - risk of acute decompensation from CSF obstruction
  • Posterior circulation is spared now but the basilar perforators that supply the thalamus arise from posterior circulation - the thalamic bleed is from Moyamoya collateral rupture, not basilar disease
  • No AVM/aneurysm found - confirms Moyamoya as source
The radiologist's recommendation for catheter angiogram is appropriate and should be prioritized once the patient is hemodynamically stable.
Moyamoya CT angiogram showing puff of smoke collaterals

Search for latest AHA/ASA guidelines on Moyamoya hemorrhage and revascularization

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AHA ASA 2023 Moyamoya disease guidelines hemorrhage revascularization recommendations

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PMID: 37609846

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PMID: 41195473

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PMID: 40036714

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https://www.ahajournals.org/doi/10.1161/STR.0000000000000443

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moyamoya hemorrhagic stroke revascularization bypass outcome rebleeding

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Gonzalez 2023 AHA Moyamoya statement surgical revascularization hemorrhagic type recommendations summary

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PMID: 37543198

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PMID: 41573187

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I now have comprehensive data. Here is the full evidence synthesis:

AHA/ASA Guidelines on Moyamoya: Hemorrhage & Revascularization

The Key Guideline Document

Gonzalez NR, Amin-Hanjani S, Bang OY, et al. "Adult Moyamoya Disease and Syndrome: Current Perspectives and Future Directions: A Scientific Statement From the American Heart Association/American Stroke Association." Stroke. 2023 Oct; 54(10):e465-e479. PMID: 37609846 | DOI: 10.1161/STR.0000000000000443
This is the most authoritative and current English-language guideline on adult Moyamoya. It supersedes the earlier 2021 version and was cited 197 times within 2 years. Below are its structured recommendations:

I. Medical Management Recommendations

RecommendationEvidence Basis
Antiplatelet therapy (aspirin) - reasonable for prevention of ischemic events in both surgical and non-surgical patients with ischemic Moyamoya vasculopathyLow level of evidence; may improve bypass patency perioperatively
Cilostazol (vasodilator antiplatelet) - may improve survival, cerebral blood flow, and cognition vs. other antiplateletsRequires further validation
Avoid vasoconstricting migraine drugs (triptans, ergotamines) - migraine-like headaches are common in MoyamoyaConsensus
No anticoagulation - not useful and contraindicated given hemorrhagic riskStandard
Control hypertension - hypertension is an independent risk factor for rebleeding in non-surgical patientsSee Hirano et al. 2023 below

II. Surgical Revascularization Recommendations (AHA/ASA 2023)

RecommendationStrength
Symptomatic MMD patients should be referred for surgical revascularizationRecommended
Endovascular treatment (stent/angioplasty) is NOT recommended for ischemic MoyamoyaExplicitly discouraged
Hemorrhagic aneurysms of Moyamoya vessels should be treated - either endovascularly OR with revascularization surgeryRecommended
For hemorrhagic MMD - revascularization is supported to reduce rebleeding by relieving hemodynamic stress on fragile collateral vesselsSupported; evidence evolving
Important caveat from the statement: Despite progress, evidence for preventing rebleeding in hemorrhagic MMD through surgery remains less robust than for ischemic MMD - this is an acknowledged evidence gap requiring further RCT-level data.

III. The Japan Adult Moyamoya (JAM) Trial - The Pivotal Evidence

The JAM trial (Miyamoto et al.) is the only prospective study specifically addressing hemorrhagic MMD. Its findings underpin the 2023 AHA/ASA recommendation for surgery in hemorrhagic cases:
  • Direct bypass surgery significantly reduced rebleeding compared to conservative management in adult hemorrhagic MMD
  • Posterior circulation hemorrhages had the highest risk of rebleeding and gained the most benefit from surgery
  • Anterior hemorrhages also benefited (confirmed by Hirano et al. 2023 below)

IV. Latest Supporting Evidence (2023-2025)

[Review . 2023] Bypass Surgery for Adult-Onset Hemorrhagic MMD: Site of Initial Bleeding Hirano Y et al. World Neurosurg. 2023. PMID: 37543198
  • 41 adult patients, 45 hemispheres, 22-year follow-up
  • Surgical group: rebleeding rate 1.3%/year vs non-surgical group: 5.3%/year (p=0.002)
  • OR for rebleeding: 0.09 (surgical vs non-surgical) - dramatic benefit
  • Anterior hemorrhage: surgery also protective (1.3% vs 5.1%/year, p=0.019)
  • Hypertension significantly shortened time to rebleed in non-surgical patients (p=0.004)
  • Takeaway for your patient: Even with anterior/thalamic hemorrhage, bypass is beneficial. Hypertension control is critical.
[Cohort . 2025] Persistent Periventricular Anastomosis and Rebleeding After Bypass Hamano E et al. Stroke Vasc Interv Neurol. 2025. PMID: 41573187
  • 116 patients, 232 hemispheres, median follow-up 6.3 years
  • Post-bypass rebleeding: 2.0%/year in periventricular anastomosis (PA)-positive vs 0.46%/year in PA-negative hemispheres
  • Adjusted HR 4.11 for rebleeding if PA persists (especially lenticulostriate anastomosis)
  • Takeaway: Post-bypass imaging surveillance is essential. If periventricular anastomosis persists on follow-up angiogram, rebleeding risk remains elevated.
[Review . 2025] Moyamoya Across the Lifespan - Neurology Sun LR et al. Neurology. 2025. PMID: 40036714
  • Confirms surgical revascularization as definitive treatment; perioperative ischemic events occur in 4-18% of cases
  • Perioperative goals: normocarbia, normotension, normovolemia, normothermia, adequate oxygenation
  • Long-term neuroimaging surveillance, neuropsychological evaluation, epilepsy/headache management emphasized
[Review . 2025] Moyamoya Disease: Pathophysiology, Diagnosis, Treatment - Dtsch Arztebl Int Mertens R et al. 2025. PMID: 41195473
  • Combined bypass surgery: low complication rate (6.3%) at a high-volume center (>500 patients)
  • RNF213 gene mutation (especially R4810K variant) - key genetic factor; more severe/earlier in Asian populations
  • Modern diagnostic evaluation: DSA + MRI + PET perfusion + cerebrovascular reactivity assessment

V. Types of Surgical Revascularization

TypeDescriptionNotes
DirectSTA-MCA (superficial temporal artery to middle cerebral artery) anastomosisImmediate perfusion augmentation; technically demanding
IndirectEDAS (encephaloduroarteriosynangiosis), EMS, EMAS, burr hole surgeryGradual neovascularization; preferred in children
CombinedDirect + indirect in same or staged surgeryMost widely practiced in adults; best long-term outcomes per Mertens 2025

VI. Application to Your Patient (38F, Thalamic Hemorrhage + Bilateral MMD)

Based on the 2023 AHA/ASA Statement and supporting literature:
  1. Acute phase now: No tPA, no anticoagulation; standard hemorrhagic stroke care; manage ICP from IVH/hydrocephalus
  2. Hypertension control is non-negotiable - independent predictor of rebleeding in non-surgical patients (Hirano 2023)
  3. Catheter DSA - essential next step for Suzuki staging and surgical planning
  4. Surgical referral to a Moyamoya-experienced center as soon as she is medically stable (typically 4-8 weeks after hemorrhage stabilization)
  5. Combined bypass is the preferred surgical strategy for adult hemorrhagic MMD
  6. Post-operative surveillance: DSA/MRI at 3-6 months to assess persistence of periventricular anastomosis - if present, close monitoring required (Hamano 2025)
  7. Long-term: Antiplatelet therapy post-revascularization; neuropsychological follow-up; epilepsy screening
Evidence gap to note: No large RCTs exist on optimal timing of surgery after hemorrhagic Moyamoya. The JAM trial and institutional series support surgery, but the AHA/ASA 2023 statement acknowledges this as an area needing stronger evidence.
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