Conus medullaris-cauda arteriovenous malformation and Klippel-Trenaunay syndrome: what is the treatment goal?
A 29-year-old man with Klippel-Trenaunay syndrome (KTS) presented with a symptomatic conus medullaris-cauda arteriovenous malformation (AVM) manifesting as back and right limb pain, which abruptly worsened with the onset of right limb weakness and urinary retention. He was treated by multisession en...
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| Veröffentlicht in: | Neurologia medico-chirurgica 2013, Vol.53 (2), p.110-114 |
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| container_title | Neurologia medico-chirurgica |
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| creator | Sgubin, Donatella Kanai, Ryuichi Di Paola, Francesco Perin, Alessandro Longatti, Pierluigi |
| description | A 29-year-old man with Klippel-Trenaunay syndrome (KTS) presented with a symptomatic conus medullaris-cauda arteriovenous malformation (AVM) manifesting as back and right limb pain, which abruptly worsened with the onset of right limb weakness and urinary retention. He was treated by multisession endovascular embolization resulting in improved neurological status. KTS is a sporadic disease with unknown etiology, but genetic susceptibility may lead to the over-expression of angiogenic factors and increased angiogenesis. KTS may be exceptionally associated with slow-flow spinal AVM, but there is no consensus about the optimal treatment for these symptomatic lesions. Embolization treatment may represent a safe option to minimize complications and possibly improve the neurological status in patients with spinal AVM associated with KTS, if one or both legs are already impaired by hypertrophy or other vascular malformations. Genetic analysis may reveal an underlying angiogenesis change, so closer follow up might be indicated in selected patients. |
| doi_str_mv | 10.2176/nmc.53.110 |
| format | Article |
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He was treated by multisession endovascular embolization resulting in improved neurological status. KTS is a sporadic disease with unknown etiology, but genetic susceptibility may lead to the over-expression of angiogenic factors and increased angiogenesis. KTS may be exceptionally associated with slow-flow spinal AVM, but there is no consensus about the optimal treatment for these symptomatic lesions. Embolization treatment may represent a safe option to minimize complications and possibly improve the neurological status in patients with spinal AVM associated with KTS, if one or both legs are already impaired by hypertrophy or other vascular malformations. Genetic analysis may reveal an underlying angiogenesis change, so closer follow up might be indicated in selected patients.</description><identifier>ISSN: 0470-8105</identifier><identifier>EISSN: 1349-8029</identifier><identifier>DOI: 10.2176/nmc.53.110</identifier><identifier>PMID: 23438663</identifier><language>eng</language><publisher>Japan</publisher><subject>Adult ; Cauda Equina - blood supply ; Central Nervous System Vascular Malformations - diagnosis ; Central Nervous System Vascular Malformations - therapy ; Embolization, Therapeutic ; Humans ; Klippel-Trenaunay-Weber Syndrome - diagnosis ; Klippel-Trenaunay-Weber Syndrome - therapy ; Magnetic Resonance Angiography ; Male ; Neurologic Examination ; Retreatment ; Spinal Cord - blood supply</subject><ispartof>Neurologia medico-chirurgica, 2013, Vol.53 (2), p.110-114</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3020-9f3f08848140b3defc83af0768d02b88049e53cf144399a676fab49e1ae1f5ef3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,860,4010,27900,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23438663$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sgubin, Donatella</creatorcontrib><creatorcontrib>Kanai, Ryuichi</creatorcontrib><creatorcontrib>Di Paola, Francesco</creatorcontrib><creatorcontrib>Perin, Alessandro</creatorcontrib><creatorcontrib>Longatti, Pierluigi</creatorcontrib><title>Conus medullaris-cauda arteriovenous malformation and Klippel-Trenaunay syndrome: what is the treatment goal?</title><title>Neurologia medico-chirurgica</title><addtitle>Neurol Med Chir (Tokyo)</addtitle><description>A 29-year-old man with Klippel-Trenaunay syndrome (KTS) presented with a symptomatic conus medullaris-cauda arteriovenous malformation (AVM) manifesting as back and right limb pain, which abruptly worsened with the onset of right limb weakness and urinary retention. He was treated by multisession endovascular embolization resulting in improved neurological status. KTS is a sporadic disease with unknown etiology, but genetic susceptibility may lead to the over-expression of angiogenic factors and increased angiogenesis. KTS may be exceptionally associated with slow-flow spinal AVM, but there is no consensus about the optimal treatment for these symptomatic lesions. Embolization treatment may represent a safe option to minimize complications and possibly improve the neurological status in patients with spinal AVM associated with KTS, if one or both legs are already impaired by hypertrophy or other vascular malformations. Genetic analysis may reveal an underlying angiogenesis change, so closer follow up might be indicated in selected patients.</description><subject>Adult</subject><subject>Cauda Equina - blood supply</subject><subject>Central Nervous System Vascular Malformations - diagnosis</subject><subject>Central Nervous System Vascular Malformations - therapy</subject><subject>Embolization, Therapeutic</subject><subject>Humans</subject><subject>Klippel-Trenaunay-Weber Syndrome - diagnosis</subject><subject>Klippel-Trenaunay-Weber Syndrome - therapy</subject><subject>Magnetic Resonance Angiography</subject><subject>Male</subject><subject>Neurologic Examination</subject><subject>Retreatment</subject><subject>Spinal Cord - blood supply</subject><issn>0470-8105</issn><issn>1349-8029</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1UE1PwzAUixCITWMXfgDKkUtH0pe2KReEJr7EJC7jXL22LyxSk46kBe3fU8Q42bItSzZjl1KsUlnkN941qwxWUooTNpegykSLtDxlc6EKkWgpshlbxmhrIVKlFejinM1SmEiew5y5de_HyB21Y9dhsDFpcGyRYxgo2P6LfP9rY2f64HCwvefoW_7a2f2eumQbyOPo8cDjwbehd3TLv3c4cBv5sCM-BMLBkR_4R4_d3QU7M9hFWh5xwd4fH7br52Tz9vSyvt8kDYhUJKUBI7RWWipRQ0um0YBGFLluRVprLVRJGTRGKgVliXmRG6wnTSJJk5GBBbv-692H_nOkOFTOxoamhZ6mPZXMYCqHDGCKXh2jYz29UO2DdRgO1f9F8APVyWos</recordid><startdate>2013</startdate><enddate>2013</enddate><creator>Sgubin, Donatella</creator><creator>Kanai, Ryuichi</creator><creator>Di Paola, Francesco</creator><creator>Perin, Alessandro</creator><creator>Longatti, Pierluigi</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7TK</scope></search><sort><creationdate>2013</creationdate><title>Conus medullaris-cauda arteriovenous malformation and Klippel-Trenaunay syndrome: what is the treatment goal?</title><author>Sgubin, Donatella ; Kanai, Ryuichi ; Di Paola, Francesco ; Perin, Alessandro ; Longatti, Pierluigi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3020-9f3f08848140b3defc83af0768d02b88049e53cf144399a676fab49e1ae1f5ef3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adult</topic><topic>Cauda Equina - blood supply</topic><topic>Central Nervous System Vascular Malformations - diagnosis</topic><topic>Central Nervous System Vascular Malformations - therapy</topic><topic>Embolization, Therapeutic</topic><topic>Humans</topic><topic>Klippel-Trenaunay-Weber Syndrome - diagnosis</topic><topic>Klippel-Trenaunay-Weber Syndrome - therapy</topic><topic>Magnetic Resonance Angiography</topic><topic>Male</topic><topic>Neurologic Examination</topic><topic>Retreatment</topic><topic>Spinal Cord - blood supply</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sgubin, Donatella</creatorcontrib><creatorcontrib>Kanai, Ryuichi</creatorcontrib><creatorcontrib>Di Paola, Francesco</creatorcontrib><creatorcontrib>Perin, Alessandro</creatorcontrib><creatorcontrib>Longatti, Pierluigi</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>Neurosciences Abstracts</collection><jtitle>Neurologia medico-chirurgica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sgubin, Donatella</au><au>Kanai, Ryuichi</au><au>Di Paola, Francesco</au><au>Perin, Alessandro</au><au>Longatti, Pierluigi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Conus medullaris-cauda arteriovenous malformation and Klippel-Trenaunay syndrome: what is the treatment goal?</atitle><jtitle>Neurologia medico-chirurgica</jtitle><addtitle>Neurol Med Chir (Tokyo)</addtitle><date>2013</date><risdate>2013</risdate><volume>53</volume><issue>2</issue><spage>110</spage><epage>114</epage><pages>110-114</pages><issn>0470-8105</issn><eissn>1349-8029</eissn><abstract>A 29-year-old man with Klippel-Trenaunay syndrome (KTS) presented with a symptomatic conus medullaris-cauda arteriovenous malformation (AVM) manifesting as back and right limb pain, which abruptly worsened with the onset of right limb weakness and urinary retention. He was treated by multisession endovascular embolization resulting in improved neurological status. KTS is a sporadic disease with unknown etiology, but genetic susceptibility may lead to the over-expression of angiogenic factors and increased angiogenesis. KTS may be exceptionally associated with slow-flow spinal AVM, but there is no consensus about the optimal treatment for these symptomatic lesions. Embolization treatment may represent a safe option to minimize complications and possibly improve the neurological status in patients with spinal AVM associated with KTS, if one or both legs are already impaired by hypertrophy or other vascular malformations. Genetic analysis may reveal an underlying angiogenesis change, so closer follow up might be indicated in selected patients.</abstract><cop>Japan</cop><pmid>23438663</pmid><doi>10.2176/nmc.53.110</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; DOAJ Directory of Open Access Journals; J-STAGE (Japan Science & Technology Information Aggregator, Electronic) Freely Available Titles - Japanese; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; PubMed Central Open Access |
| subjects | Adult Cauda Equina - blood supply Central Nervous System Vascular Malformations - diagnosis Central Nervous System Vascular Malformations - therapy Embolization, Therapeutic Humans Klippel-Trenaunay-Weber Syndrome - diagnosis Klippel-Trenaunay-Weber Syndrome - therapy Magnetic Resonance Angiography Male Neurologic Examination Retreatment Spinal Cord - blood supply |
| title | Conus medullaris-cauda arteriovenous malformation and Klippel-Trenaunay syndrome: what is the treatment goal? |
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