Bilateral progressive steno-occlusive changes of supraclinoid ICA segments involving carotid fork...
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Transcript of Bilateral progressive steno-occlusive changes of supraclinoid ICA segments involving carotid fork...
CHUNG, J.I., Department of Radiology, Suncheon Pyunghwa Hospital, Suncheon City, Republic of Korea
WEON, Y.C., Department of Radiology, Samsung Medical Center, Seoul, Republic of Korea
Unilateral Moyamoya Disease vs. Asymmetric Manifestations of Definite Moyamoya disease: Angiographic Pattern Analysis with Its Pathognomonic Find-
ings
Bilateral progressive steno-occlusive changes of supraclinoid ICA segments involving carotid fork region; Unilateral involvements (overall 20%) are also seen
“Puff of Smoke”; (1) Classical basal collater-als recruiting leptomeningeal vessels and deep parenchymal vessels of striatum (2) Collateral vessels are formed during prena-tal period
Pathophysiology; Fibrocellular intimal thick-enings with wavy internal elastic lamina and thinning of media
Moyamoya disease (MMD)
INTRODUCTION
Etiology; UnknownSome Genetic Predispositions; 10% of familial
incidence, hereditary and multifactorial, au-tosomal dominant familial MMD with re-duced penetrance
Genetically Susceptible Loci; 3p, 6p, 17q, and band 8q23
Angiographic Classification of MMD; Suzuki & Takaku since 1969, but often fail to repre-sent the disease progression
Controversies about its etiologies as congeni-tal vs. acquired
Moyamoya disease (MMD)
INTRODUCTION
Moyamoya disease (MMD)
Presumptive prenatal occlusive changes of carotid arteries during certain embryonic pe-riod: Stage IV & Stage V, Padget DH. The development of the cranial arteries in the human embryo. Contrib Em-bryol 1948;32:207-261
INTRODUCTION
Visualization of primitive embry-onic arteries with remnant anas-tomosis and annexations : cru-cial to understand the patho-physiologic mechanisms
Pathophysiologic target arterial segment could be located in proximal MCA just distal to AChA bifurcation during 14-20mm embryo stage: Stage IV & V, Pad-get DH
Persisted and annexed embryonic vessels which depicted as col-laterals in MMD patients: reveal the time specific occlusive changes of carotid arteries and show adjusted hemodynamic balances during the early em-bryonic period.
Moyamoya disease (MMD)
INTRODUCTION
Moyamoya disease (MMD)
Ophthalmic Rete Mirabile (ORM) annexations with PVOA (primi-tive ventral ophthalmic artery) and POlfA (primitive olfactory
artery): Visualization of Embryonic Remnant Vascular Configurations
Interventional Neuroradiology 14: 293-296, 2008
INTRODUCTION
Moyamoya disease (MMD)
Ophthalmic Ethmoidal Collaterals
20mm Stage: dwindling SOD of Stapedial Artery anasto-mosis with Ophthalmic Artery “arterial ring” which passes around the dorsal side of the optic nerve.
Stem of Ophthalmic artery -”arterial ring” of 2ndary anasto-mosis between SOD of SA and stem of OA – Anterior ethmoidal artery along the course of Nasociliary nerve. INTRODUCTION
Progression of Unilateral MMD: A Clinical Series. Kelly ME et al. Cerebrovasc Dis 2006;22:109-15
7/18 (38.9 %) OF ANGIO-GRAPHIC PROGRESSION
6/7 (85.7 %) OF INITIAL CAROTID FORK ABNORMAL-ITIES
Presence of contralateral carotid fork abnormalities are impor-tant prognostic factors of pro-gression
“True definition of Unilateral MMD is Unclear”
UNILATERAL MMD VS. ASYMMET-RIC DEFINITE MMD
PURPOSE
Anatomic configuration of the cerebral vessels of Uni-lateral MMD :
Presence of Contralateral carotid fork abnormali-ties & Remnant embryonic annexations and anas-tomosis represented as COLLATERALS
“UNDOUBTEDLY PLAYS A ROLE IN OUTCOME”Immediate Surgery for Moyamoya Syndrome? Not Nec-
essarily Roach ES. Arch Neurol 2001;58:130-31 Try to discern the Pathognomonic Angiographic
Manifestations Verifications and Predictable Natural History about
MMD “ TRUE DEFINITION OF UNILATERAL MMD ?”
UNILATERAL MMD VS. ASYMMET-RIC DEFINITE MMD
PURPOSE
March 2002 to April 2004Angiographic Analysis: Independently Reviewed by Two
Experienced Neuroradiologists (C.J.I & W.Y.C.) Based upon the Significant Contributions by Dorcas
Hager Padget (1906-1973) “The development of the cranial arteries in the
human embryo. Contrib Embryol 32:205-261, 1948”
Analysis of the Pathognomonic Angiographic Manifesta-tions
Carotid Fork Appearances Identification and Classification of Remnant Em-
bryonic Anastomosis and Annexations
RETROSPECTIVE REVIEW 204 ANGIOGRAMS (102 MMD PA-
TIENTS)
METHODS
All 20 patient showed Asymmetric Contralateral Carotid Fork Abnormalities with Typical MMD Collaterals
Disproportional Vessel Caliber Changes (2/19, 10.5%) and
Mild degree Stenosis of Carotid Fork Region (17/19, 89.5%)
TYPICAL MMD COLLATERALS POlfA with or without ORM (ophthalmic rete
mirabile): 8/19, 42.1% Ophthalmic Ethmoidal Collaterals: 2/19, 10.5% Callosal Artery Collaterals: 3/19, 15.8%
20 (19.6%) OF UNILATERAL MMD VS. ASYMMETRIC DEFINITE MMD
RESULTS
Dispropor-tional Vessel
Caliber Changes
RESULTS
Mild Degree Stenosis of Carotid Fork Regions: A1
segment nar-rowings
POlfA Collat-erals
RESULTS
Typical MMD Collaterals of POlfA with or without ORM (Ophthalmic
Rete Mirabile)
POlfA Collater-als
Basal Collater-als
RESULTS
Ophthalmic Ethmoidal Col-
laterals
Basal Collat-erals
RESULTS
Callosal Artery Collaterals
POlfA Collaterals with ORM (Oph-
thalmic Rete Mirabile)
RESULTS
The definition of Unilateral MMD is IncorrectMMD is Bilateral Disease without Exception, but
Sometimes do Manifests as Asymmetric In-volvements
Must Understand the Characteristic Angiographic Findings of Typical MMD Collaterals: Remnant Embryonic Anastomosis and Annexations
Pathognomonic Angiographic Findings of Asymmet-ric MMD Carotid Fork Abnormalities
Identification and Understand the patterns of Remnant Embryonic Anastomosis and Annexa-tions
UNILATERAL MMD VS. ASYMMETRIC DEFINITE MMD
CONCLUSION