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Concurrent stenoocclusive disease of intracranial and extracranial arteries in a patient with polycythemia vera.

Hua le H, Dodd RL, Schwartz NE - Case Rep Med (2012)

Bottom Line: We describe the novel case of polycythemia vera associated with severe steno-occlusive disease of both intracranial and extracranial large arteries.A 47-year-old woman with polycythemia vera had multiple transient ischemic attacks, and noninvasive vessel imaging revealed steno-occlusive disease of bilateral supraclinoid internal carotid arteries with moyamoya-type collaterals, proximal left subclavian artery, right vertebral artery origin, bilateral renal arteries, superior mesenteric artery, and right common iliac artery.The pathophysiology may be due to a prothrombotic state leading to repeated endothelial injury, resultant intimal hyperplasia, and progressive steno-occlusion.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Neurological Sciences, Stanford University School of Medicine, 300 Pasteur Drive, Room A343, Stanford, CA 94305, USA.

ABSTRACT
Moyamoya disease is a stenoocclusive disease involving the intracranial carotid and proximal middle cerebral arteries. There are rarely any additional extracranial stenoses occurring concurrently with moyamoya. The pathophysiology of moyamoya remains obscure, but hematologic disorders, notably sickle-cell anemia, have been associated in some cases. We describe the novel case of polycythemia vera associated with severe steno-occlusive disease of both intracranial and extracranial large arteries. A 47-year-old woman with polycythemia vera had multiple transient ischemic attacks, and noninvasive vessel imaging revealed steno-occlusive disease of bilateral supraclinoid internal carotid arteries with moyamoya-type collaterals, proximal left subclavian artery, right vertebral artery origin, bilateral renal arteries, superior mesenteric artery, and right common iliac artery. Laboratory workup for systemic vasculitis was negative. She required bilateral direct external carotid to internal carotid bypass procedures and percutaneous balloon angioplasty of her right VA origin stenosis. This case suggests that hematologic disorders can lead to vessel stenoses and occlusion. The pathophysiology may be due to a prothrombotic state leading to repeated endothelial injury, resultant intimal hyperplasia, and progressive steno-occlusion.

No MeSH data available.


Related in: MedlinePlus

Multimodality imaging demonstrating stenoocclusive disease of intracranial and extracranial arteries. (a) CT angiogram chest demonstrating complete occlusion of left subclavian artery (arrow) with distal filling from left VA (arrowhead). Remainder of VA is out of plane of view, but patent distally. (b) MR Angiogram of abdomen demonstrating bilateral renal artery stenoses (arrows). (c) Time-of-flight MR angiogram of head showing absence of bilateral MCA vessels and decreased flow of both ICAs. The posterior circulation is relatively plethoric. (d) Conventional angiogram of the right ICA (AP projection) showing typical moyamoya appearance of intracranial occlusion of supraclinoid ICA (arrow) with reconstitution of the MCA via multiple small moyamoya collaterals (arrowhead). (e) Right subclavian digital subtraction angiogram demonstrating high-grade (>70%) right VA stenosis (arrow).
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fig1: Multimodality imaging demonstrating stenoocclusive disease of intracranial and extracranial arteries. (a) CT angiogram chest demonstrating complete occlusion of left subclavian artery (arrow) with distal filling from left VA (arrowhead). Remainder of VA is out of plane of view, but patent distally. (b) MR Angiogram of abdomen demonstrating bilateral renal artery stenoses (arrows). (c) Time-of-flight MR angiogram of head showing absence of bilateral MCA vessels and decreased flow of both ICAs. The posterior circulation is relatively plethoric. (d) Conventional angiogram of the right ICA (AP projection) showing typical moyamoya appearance of intracranial occlusion of supraclinoid ICA (arrow) with reconstitution of the MCA via multiple small moyamoya collaterals (arrowhead). (e) Right subclavian digital subtraction angiogram demonstrating high-grade (>70%) right VA stenosis (arrow).

Mentions: Her symptoms and differential blood pressure were concerning for subclavian steal phenomenon. This was confirmed on CT angiogram which demonstrated complete occlusion of the left subclavian artery proximal to the vertebral artery (VA) origin with a patent left vertebral and distal subclavian artery (Figure 1(a)). Interestingly, her CT angiogram also revealed severe stenosis at the origin of the right VA and stenoocclusive disease of the bilateral supraclinoid internal carotid arteries (ICA) with multiple small collaterals reconstituting the proximal middle cerebral (MCA) and anterior cerebral arteries. MR angiography confirmed the above arterial stenoses (Figure 1(c)), while MRI demonstrated multiple chronic watershed cerebral infarcts. Conventional angiogram also demonstrated bilateral supraclinoid ICA occlusions with moyamoya-type collaterals (Figure 1(d)), high-grade (70%) right VA stenosis (Figure 1(e)), and left subclavian artery occlusion. MR angiogram of her chest and abdomen demonstrated mild wall thickening of the aortic arch and abdominal aorta, 50% bilateral renal artery stenoses (Figure 1(b)), superior mesenteric artery stenosis, and right common iliac artery stenosis (not shown).


Concurrent stenoocclusive disease of intracranial and extracranial arteries in a patient with polycythemia vera.

Hua le H, Dodd RL, Schwartz NE - Case Rep Med (2012)

Multimodality imaging demonstrating stenoocclusive disease of intracranial and extracranial arteries. (a) CT angiogram chest demonstrating complete occlusion of left subclavian artery (arrow) with distal filling from left VA (arrowhead). Remainder of VA is out of plane of view, but patent distally. (b) MR Angiogram of abdomen demonstrating bilateral renal artery stenoses (arrows). (c) Time-of-flight MR angiogram of head showing absence of bilateral MCA vessels and decreased flow of both ICAs. The posterior circulation is relatively plethoric. (d) Conventional angiogram of the right ICA (AP projection) showing typical moyamoya appearance of intracranial occlusion of supraclinoid ICA (arrow) with reconstitution of the MCA via multiple small moyamoya collaterals (arrowhead). (e) Right subclavian digital subtraction angiogram demonstrating high-grade (>70%) right VA stenosis (arrow).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3368357&req=5

fig1: Multimodality imaging demonstrating stenoocclusive disease of intracranial and extracranial arteries. (a) CT angiogram chest demonstrating complete occlusion of left subclavian artery (arrow) with distal filling from left VA (arrowhead). Remainder of VA is out of plane of view, but patent distally. (b) MR Angiogram of abdomen demonstrating bilateral renal artery stenoses (arrows). (c) Time-of-flight MR angiogram of head showing absence of bilateral MCA vessels and decreased flow of both ICAs. The posterior circulation is relatively plethoric. (d) Conventional angiogram of the right ICA (AP projection) showing typical moyamoya appearance of intracranial occlusion of supraclinoid ICA (arrow) with reconstitution of the MCA via multiple small moyamoya collaterals (arrowhead). (e) Right subclavian digital subtraction angiogram demonstrating high-grade (>70%) right VA stenosis (arrow).
Mentions: Her symptoms and differential blood pressure were concerning for subclavian steal phenomenon. This was confirmed on CT angiogram which demonstrated complete occlusion of the left subclavian artery proximal to the vertebral artery (VA) origin with a patent left vertebral and distal subclavian artery (Figure 1(a)). Interestingly, her CT angiogram also revealed severe stenosis at the origin of the right VA and stenoocclusive disease of the bilateral supraclinoid internal carotid arteries (ICA) with multiple small collaterals reconstituting the proximal middle cerebral (MCA) and anterior cerebral arteries. MR angiography confirmed the above arterial stenoses (Figure 1(c)), while MRI demonstrated multiple chronic watershed cerebral infarcts. Conventional angiogram also demonstrated bilateral supraclinoid ICA occlusions with moyamoya-type collaterals (Figure 1(d)), high-grade (70%) right VA stenosis (Figure 1(e)), and left subclavian artery occlusion. MR angiogram of her chest and abdomen demonstrated mild wall thickening of the aortic arch and abdominal aorta, 50% bilateral renal artery stenoses (Figure 1(b)), superior mesenteric artery stenosis, and right common iliac artery stenosis (not shown).

Bottom Line: We describe the novel case of polycythemia vera associated with severe steno-occlusive disease of both intracranial and extracranial large arteries.A 47-year-old woman with polycythemia vera had multiple transient ischemic attacks, and noninvasive vessel imaging revealed steno-occlusive disease of bilateral supraclinoid internal carotid arteries with moyamoya-type collaterals, proximal left subclavian artery, right vertebral artery origin, bilateral renal arteries, superior mesenteric artery, and right common iliac artery.The pathophysiology may be due to a prothrombotic state leading to repeated endothelial injury, resultant intimal hyperplasia, and progressive steno-occlusion.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Neurological Sciences, Stanford University School of Medicine, 300 Pasteur Drive, Room A343, Stanford, CA 94305, USA.

ABSTRACT
Moyamoya disease is a stenoocclusive disease involving the intracranial carotid and proximal middle cerebral arteries. There are rarely any additional extracranial stenoses occurring concurrently with moyamoya. The pathophysiology of moyamoya remains obscure, but hematologic disorders, notably sickle-cell anemia, have been associated in some cases. We describe the novel case of polycythemia vera associated with severe steno-occlusive disease of both intracranial and extracranial large arteries. A 47-year-old woman with polycythemia vera had multiple transient ischemic attacks, and noninvasive vessel imaging revealed steno-occlusive disease of bilateral supraclinoid internal carotid arteries with moyamoya-type collaterals, proximal left subclavian artery, right vertebral artery origin, bilateral renal arteries, superior mesenteric artery, and right common iliac artery. Laboratory workup for systemic vasculitis was negative. She required bilateral direct external carotid to internal carotid bypass procedures and percutaneous balloon angioplasty of her right VA origin stenosis. This case suggests that hematologic disorders can lead to vessel stenoses and occlusion. The pathophysiology may be due to a prothrombotic state leading to repeated endothelial injury, resultant intimal hyperplasia, and progressive steno-occlusion.

No MeSH data available.


Related in: MedlinePlus