Microneurosurgical treatment of a small perimesencephalic pure pial arterial malformation: an under-recognized etiology of angiographically occult subarachnoid hemorrhage. Illustrative case
Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is limited by the lesion's rarity, limited anatomopathological studies, and frequent misclassifications. A 23-year-old male experienced two spo...
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Published in | Journal of neurosurgery. Case lessons Vol. 6; no. 5 |
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Main Authors | , , , , |
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Language | English |
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American Association of Neurological Surgeons
31.07.2023
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Abstract | Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is limited by the lesion's rarity, limited anatomopathological studies, and frequent misclassifications.
A 23-year-old male experienced two spontaneous subarachnoid hemorrhages (SAHs) over 6 months with initially unremarkable diagnostic cerebral angiograms. Magnetic resonance imaging (MRI) and angiography after the second SAH revealed a small perimesencephalic ovoid lesion within the left crural cistern, between the left superior and posterior cerebral arteries, appearing to be an exophytic cavernoma, a thrombosed aneurysm, or a hemorrhagic tumor. Microsurgical resection was achieved with a pterional craniotomy and anterior clinoidectomy. The resected lesion was characteristic of a pure PAM arising from superior cerebellar arterial branches.
Small pure PAMs can be deceitfully dynamic lesions causing episodes of hemorrhage, complete thrombosis (angiographically occult), recanalization, and rehemorrhage. Small thrombosed vascular malformations or aneurysms should be included in differential diagnoses of angiographically occult SAH. MRI can be diagnostic, but the true angioarchitecture can only be elucidated with microneurosurgery. The only definitive cure is removal. The microneurosurgical strategy should account for worst-case scenarios, provide adequate skull base exposures, and include bypass revascularization options when thrombosed aneurysms are encountered. |
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AbstractList | Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is limited by the lesion's rarity, limited anatomopathological studies, and frequent misclassifications.
A 23-year-old male experienced two spontaneous subarachnoid hemorrhages (SAHs) over 6 months with initially unremarkable diagnostic cerebral angiograms. Magnetic resonance imaging (MRI) and angiography after the second SAH revealed a small perimesencephalic ovoid lesion within the left crural cistern, between the left superior and posterior cerebral arteries, appearing to be an exophytic cavernoma, a thrombosed aneurysm, or a hemorrhagic tumor. Microsurgical resection was achieved with a pterional craniotomy and anterior clinoidectomy. The resected lesion was characteristic of a pure PAM arising from superior cerebellar arterial branches.
Small pure PAMs can be deceitfully dynamic lesions causing episodes of hemorrhage, complete thrombosis (angiographically occult), recanalization, and rehemorrhage. Small thrombosed vascular malformations or aneurysms should be included in differential diagnoses of angiographically occult SAH. MRI can be diagnostic, but the true angioarchitecture can only be elucidated with microneurosurgery. The only definitive cure is removal. The microneurosurgical strategy should account for worst-case scenarios, provide adequate skull base exposures, and include bypass revascularization options when thrombosed aneurysms are encountered. BACKGROUNDPial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is limited by the lesion's rarity, limited anatomopathological studies, and frequent misclassifications. OBSERVATIONSA 23-year-old male experienced two spontaneous subarachnoid hemorrhages (SAHs) over 6 months with initially unremarkable diagnostic cerebral angiograms. Magnetic resonance imaging (MRI) and angiography after the second SAH revealed a small perimesencephalic ovoid lesion within the left crural cistern, between the left superior and posterior cerebral arteries, appearing to be an exophytic cavernoma, a thrombosed aneurysm, or a hemorrhagic tumor. Microsurgical resection was achieved with a pterional craniotomy and anterior clinoidectomy. The resected lesion was characteristic of a pure PAM arising from superior cerebellar arterial branches. LESSONSSmall pure PAMs can be deceitfully dynamic lesions causing episodes of hemorrhage, complete thrombosis (angiographically occult), recanalization, and rehemorrhage. Small thrombosed vascular malformations or aneurysms should be included in differential diagnoses of angiographically occult SAH. MRI can be diagnostic, but the true angioarchitecture can only be elucidated with microneurosurgery. The only definitive cure is removal. The microneurosurgical strategy should account for worst-case scenarios, provide adequate skull base exposures, and include bypass revascularization options when thrombosed aneurysms are encountered. BACKGROUND Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is limited by the lesion’s rarity, limited anatomopathological studies, and frequent misclassifications. OBSERVATIONS A 23-year-old male experienced two spontaneous subarachnoid hemorrhages (SAHs) over 6 months with initially unremarkable diagnostic cerebral angiograms. Magnetic resonance imaging (MRI) and angiography after the second SAH revealed a small perimesencephalic ovoid lesion within the left crural cistern, between the left superior and posterior cerebral arteries, appearing to be an exophytic cavernoma, a thrombosed aneurysm, or a hemorrhagic tumor. Microsurgical resection was achieved with a pterional craniotomy and anterior clinoidectomy. The resected lesion was characteristic of a pure PAM arising from superior cerebellar arterial branches. LESSONS Small pure PAMs can be deceitfully dynamic lesions causing episodes of hemorrhage, complete thrombosis (angiographically occult), recanalization, and rehemorrhage. Small thrombosed vascular malformations or aneurysms should be included in differential diagnoses of angiographically occult SAH. MRI can be diagnostic, but the true angioarchitecture can only be elucidated with microneurosurgery. The only definitive cure is removal. The microneurosurgical strategy should account for worst-case scenarios, provide adequate skull base exposures, and include bypass revascularization options when thrombosed aneurysms are encountered. |
Author | Erginoglu, Ufuk Martínez Santos, Jaime L Baskaya, Mustafa K Sterner, Robert C Greeneway, Garret P |
Author_xml | – sequence: 1 givenname: Robert C surname: Sterner fullname: Sterner, Robert C organization: 1Department of Neurological Surgery, University of Wisconsin-Madison, Madison, Wisconsin; and – sequence: 2 givenname: Garret P surname: Greeneway fullname: Greeneway, Garret P organization: 1Department of Neurological Surgery, University of Wisconsin-Madison, Madison, Wisconsin; and – sequence: 3 givenname: Ufuk surname: Erginoglu fullname: Erginoglu, Ufuk organization: 1Department of Neurological Surgery, University of Wisconsin-Madison, Madison, Wisconsin; and – sequence: 4 givenname: Jaime L surname: Martínez Santos fullname: Martínez Santos, Jaime L organization: 2Department of Neurological Surgery, Medical University of South Carolina, Charleston, South Carolina – sequence: 5 givenname: Mustafa K surname: Baskaya fullname: Baskaya, Mustafa K organization: 1Department of Neurological Surgery, University of Wisconsin-Madison, Madison, Wisconsin; and |
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Keywords | pure PAM PAM superior cerebellar artery pure pial arterial malformation pure arterial malformation |
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Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 INCLUDE WHEN CITING Published July 31, 2023; DOI: 10.3171/CASE23246. Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. |
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References_xml | – volume: 135 start-page: 214 year: 2020 ident: b5 article-title: Spontaneous subarachnoid hemorrhage from a pure pial arterial malformation in the lateral cerebellomedullary junction: clinical images with a surgical video contributor: fullname: Li Y – volume: 16 start-page: 1548 issue: 7 year: 1995 ident: b20 article-title: Dolichoectasia of the anterior cerebral arteries in an adolescent contributor: fullname: Doran SE – volume: 131 start-page: e52 year: 2019 ident: b16 article-title: Progressive pure arterial malformations of the anterior cerebral artery contributor: fullname: Yue H – volume: 125 start-page: 95 issue: 2 year: 1969 ident: b10 article-title: Dolicho-ectatic intracranial arteries: symptomatology and pathogenesis of arterial elongation and distention contributor: fullname: Sacks JG – volume: 121 start-page: 1007 issue: 4 year: 2014 ident: b12 article-title: Letter to the editor. 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Snippet | Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM understanding is... BACKGROUND Pial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM... BACKGROUNDPial arterial malformations (PAMs) are rare vascular lesions consisting of dilated tortuous arteries without venous drainage. Current PAM... |
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Title | Microneurosurgical treatment of a small perimesencephalic pure pial arterial malformation: an under-recognized etiology of angiographically occult subarachnoid hemorrhage. Illustrative case |
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