Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction
Identifieur interne : 000E09 ( Istex/Corpus ); précédent : 000E08; suivant : 000E10Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction
Auteurs : Satish C. Muluk ; Leo C. Ginns ; Marc J. Semigran ; John A. Kaufman ; Jonathan P. GertlerSource :
- Journal of Vascular Surgery [ 0741-5214 ] ; 1995.
Abstract
A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J VASC SURG 1995;21:686-90.)
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DOI: 10.1016/S0741-5214(95)70199-0
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title><author><name sortKey="Muluk, Satish C" sort="Muluk, Satish C" uniqKey="Muluk S" first="Satish C." last="Muluk">Satish C. Muluk</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Ginns, Leo C" sort="Ginns, Leo C" uniqKey="Ginns L" first="Leo C." last="Ginns">Leo C. Ginns</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Semigran, Marc J" sort="Semigran, Marc J" uniqKey="Semigran M" first="Marc J." last="Semigran">Marc J. Semigran</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Kaufman, John A" sort="Kaufman, John A" uniqKey="Kaufman J" first="John A." last="Kaufman">John A. Kaufman</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Gertler, Jonathan P" sort="Gertler, Jonathan P" uniqKey="Gertler J" first="Jonathan P." last="Gertler">Jonathan P. Gertler</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:1E4F06ECB32B11D760BAD59AEC7B954969333335</idno><date when="1995" year="1995">1995</date><idno type="doi">10.1016/S0741-5214(95)70199-0</idno><idno type="url">https://api.istex.fr/document/1E4F06ECB32B11D760BAD59AEC7B954969333335/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">000E09</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">000E09</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title><author><name sortKey="Muluk, Satish C" sort="Muluk, Satish C" uniqKey="Muluk S" first="Satish C." last="Muluk">Satish C. Muluk</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Ginns, Leo C" sort="Ginns, Leo C" uniqKey="Ginns L" first="Leo C." last="Ginns">Leo C. Ginns</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Semigran, Marc J" sort="Semigran, Marc J" uniqKey="Semigran M" first="Marc J." last="Semigran">Marc J. Semigran</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Kaufman, John A" sort="Kaufman, John A" uniqKey="Kaufman J" first="John A." last="Kaufman">John A. Kaufman</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author><author><name sortKey="Gertler, Jonathan P" sort="Gertler, Jonathan P" uniqKey="Gertler J" first="Jonathan P." last="Gertler">Jonathan P. Gertler</name><affiliation><mods:affiliation>Boston, Mass.</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Journal of Vascular Surgery</title><title level="j" type="abbrev">YMVA</title><idno type="ISSN">0741-5214</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1995">1995</date><biblScope unit="volume">21</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="686">686</biblScope><biblScope unit="page" to="690">690</biblScope></imprint><idno type="ISSN">0741-5214</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0741-5214</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J VASC SURG 1995;21:686-90.)</div></front></TEI><istex><corpusName>elsevier</corpusName><keywords><teeft><json:string>venous</json:string><json:string>embolus</json:string><json:string>surg</json:string><json:string>arteriovenous</json:string><json:string>malformation</json:string><json:string>syndrome</json:string><json:string>lymphedema</json:string><json:string>obstruction</json:string><json:string>varicose</json:string><json:string>servelle</json:string><json:string>warfarin</json:string><json:string>embolism</json:string><json:string>varicosity</json:string><json:string>nevus</json:string><json:string>dyspnea</json:string><json:string>abnormality</json:string><json:string>radiology</json:string><json:string>syndactyly</json:string><json:string>klippel</json:string><json:string>vascular surgeon</json:string><json:string>case report</json:string><json:string>venous obstruction</json:string><json:string>varicose veins</json:string><json:string>progressive dyspnea</json:string><json:string>pulmonary arteriogram</json:string><json:string>congenital</json:string><json:string>anticoagulation</json:string><json:string>vascular</json:string><json:string>warfarin anticoagulation</json:string><json:string>deep venous thrombosis</json:string><json:string>heart catheterization</json:string><json:string>angiodysplastic syndrome</json:string><json:string>vital capacity</json:string><json:string>clin nucl</json:string><json:string>contrast venography</json:string><json:string>vascular nevus</json:string><json:string>pulmonary emboli</json:string><json:string>vascular surgery</json:string><json:string>venous drainage</json:string><json:string>deep venous malformations</json:string><json:string>venous stasis</json:string><json:string>venous system</json:string><json:string>congenital lymphedema</json:string><json:string>vascular surgery volume</json:string><json:string>surgical relief</json:string><json:string>hypertrophy</json:string></teeft></keywords><author><json:item><name>Satish C. Muluk MD</name><affiliations><json:string>Boston, Mass.</json:string></affiliations></json:item><json:item><name>Leo C. Ginns MD</name><affiliations><json:string>Boston, Mass.</json:string></affiliations></json:item><json:item><name>Marc J. Semigran MD</name><affiliations><json:string>Boston, Mass.</json:string></affiliations></json:item><json:item><name>John A. Kaufman</name><affiliations><json:string>Boston, Mass.</json:string></affiliations></json:item><json:item><name>Jonathan P. Gertler MD</name><affiliations><json:string>Boston, Mass.</json:string></affiliations></json:item></author><language><json:string>eng</json:string></language><originalGenre><json:string>Full-length article</json:string></originalGenre><abstract>A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J VASC SURG 1995;21:686-90.)</abstract><qualityIndicators><score>4.636</score><pdfVersion>1.3</pdfVersion><pdfPageSize>576 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><keywordCount>0</keywordCount><abstractCharCount>1490</abstractCharCount><pdfWordCount>2128</pdfWordCount><pdfCharCount>14076</pdfCharCount><pdfPageCount>5</pdfPageCount><abstractWordCount>209</abstractWordCount></qualityIndicators><title>Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title><pii><json:string>S0741-5214(95)70199-0</json:string></pii><genre><json:string>research-article</json:string></genre><host><title>Journal of Vascular Surgery</title><language><json:string>unknown</json:string></language><publicationDate>1995</publicationDate><issn><json:string>0741-5214</json:string></issn><pii><json:string>S0741-5214(05)X7163-4</json:string></pii><volume>21</volume><issue>4</issue><pages><first>686</first><last>690</last></pages><genre><json:string>journal</json:string></genre></host><categories><wos><json:string>science</json:string><json:string>surgery</json:string><json:string>peripheral vascular disease</json:string></wos><scienceMetrix><json:string>health sciences</json:string><json:string>clinical medicine</json:string><json:string>cardiovascular system & hematology</json:string></scienceMetrix><inist><json:string>sciences appliquees, technologies et medecines</json:string><json:string>sciences biologiques et medicales</json:string><json:string>sciences medicales</json:string></inist></categories><publicationDate>1995</publicationDate><copyrightDate>1995</copyrightDate><doi><json:string>10.1016/S0741-5214(95)70199-0</json:string></doi><id>1E4F06ECB32B11D760BAD59AEC7B954969333335</id><score>1</score><fulltext><json:item><extension>pdf</extension><original>true</original><mimetype>application/pdf</mimetype><uri>https://api.istex.fr/document/1E4F06ECB32B11D760BAD59AEC7B954969333335/fulltext/pdf</uri></json:item><json:item><extension>zip</extension><original>false</original><mimetype>application/zip</mimetype><uri>https://api.istex.fr/document/1E4F06ECB32B11D760BAD59AEC7B954969333335/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/1E4F06ECB32B11D760BAD59AEC7B954969333335/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>ELSEVIER</publisher><availability><p>©1995 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter</p></availability><date>1995</date></publicationStmt><notesStmt><note>From the Division of Vascular Surgery, Department of Surgery, the Pulmonary and Critical Care Unit (Dr. Ginns), and Division of Cardiology (Dr. Semigran), General Medical Services, and the Department of Radiology (Mr. Kaufman), Massachusetts General Hospital, Harvard Medical School, Boston.</note><note>Reprint request: Jonathan P. Gertler, MD, Massachusetts General Hospital, Ambulatory Care Center, Suite 464, 15 Parkman St., Boston, MA 02114.</note><note>0741-5214/95/$3.00 + 0 24/4/61920</note><note type="content">Table I: Major findings that led to diagnosis of KTS</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title><author xml:id="author-0000"><persName><forename type="first">Satish C.</forename><surname>Muluk</surname></persName><roleName type="degree">MD</roleName><affiliation>Boston, Mass.</affiliation></author><author xml:id="author-0001"><persName><forename type="first">Leo C.</forename><surname>Ginns</surname></persName><roleName type="degree">MD</roleName><affiliation>Boston, Mass.</affiliation></author><author xml:id="author-0002"><persName><forename type="first">Marc J.</forename><surname>Semigran</surname></persName><roleName type="degree">MD</roleName><affiliation>Boston, Mass.</affiliation></author><author xml:id="author-0003"><persName><forename type="first">John A.</forename><surname>Kaufman</surname></persName><affiliation>Boston, Mass.</affiliation></author><author xml:id="author-0004"><persName><forename type="first">Jonathan P.</forename><surname>Gertler</surname></persName><roleName type="degree">MD</roleName><affiliation>Boston, Mass.</affiliation></author><idno type="istex">1E4F06ECB32B11D760BAD59AEC7B954969333335</idno><idno type="DOI">10.1016/S0741-5214(95)70199-0</idno><idno type="PII">S0741-5214(95)70199-0</idno></analytic><monogr><title level="j">Journal of Vascular Surgery</title><title level="j" type="abbrev">YMVA</title><idno type="pISSN">0741-5214</idno><idno type="PII">S0741-5214(05)X7163-4</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="1995"></date><biblScope unit="volume">21</biblScope><biblScope unit="issue">4</biblScope><biblScope unit="page" from="686">686</biblScope><biblScope unit="page" to="690">690</biblScope></imprint></monogr></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1995</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J VASC SURG 1995;21:686-90.)</p></abstract></profileDesc><revisionDesc><change when="1995">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/document/1E4F06ECB32B11D760BAD59AEC7B954969333335/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Elsevier doc found" wicri:toSee="Elsevier, no converted or simple article"><istex:xmlDeclaration>version="1.0" encoding="utf-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//ES//DTD journal article DTD version 5.0.1//EN//XML" URI="art501.dtd" name="istex:docType"><istex:entity SYSTEM="gr1a" NDATA="IMAGE" name="gr1a"></istex:entity><istex:entity SYSTEM="gr1b" NDATA="IMAGE" name="gr1b"></istex:entity><istex:entity SYSTEM="gr2a" NDATA="IMAGE" name="gr2a"></istex:entity><istex:entity SYSTEM="gr2b" NDATA="IMAGE" name="gr2b"></istex:entity></istex:docType><istex:document><article docsubtype="fla" xml:lang="en"><item-info><jid>YMVA</jid><aid>61920</aid><ce:pii>S0741-5214(95)70199-0</ce:pii><ce:doi>10.1016/S0741-5214(95)70199-0</ce:doi><ce:copyright type="other" year="1995">Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter</ce:copyright></item-info><ce:floats><ce:table id="tab1" colsep="0" rowsep="0" frame="topbot"><ce:label>Table I</ce:label><ce:caption><ce:simple-para>Major findings that led to diagnosis of KTS</ce:simple-para></ce:caption><tgroup cols="1"><colspec colname="col1" colsep="0"></colspec><tbody><row><entry align="center">Long-standing varicose veins</entry></row><row><entry align="center">Soft tissue and bony hypertrophy of left leg and arm</entry></row><row><entry align="center">Vascular nevus</entry></row><row><entry align="center">Syndactyly</entry></row><row><entry align="center">Long-standing lymphedema</entry></row></tbody></tgroup></ce:table></ce:floats><head><ce:article-footnote><ce:label>☆</ce:label><ce:note-para>From the Division of Vascular Surgery, Department of Surgery, the Pulmonary and Critical Care Unit (Dr. Ginns), and Division of Cardiology (Dr. Semigran), General Medical Services, and the Department of Radiology (Mr. Kaufman), Massachusetts General Hospital, Harvard Medical School, Boston.</ce:note-para></ce:article-footnote><ce:article-footnote><ce:label>☆☆</ce:label><ce:note-para>Reprint request: Jonathan P. Gertler, MD, Massachusetts General Hospital, Ambulatory Care Center, Suite 464, 15 Parkman St., Boston, MA 02114.</ce:note-para></ce:article-footnote><ce:article-footnote><ce:label>★</ce:label><ce:note-para>0741-5214/95/$3.00 + 0 <ce:bold>24/4/61920</ce:bold></ce:note-para></ce:article-footnote><ce:title>Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</ce:title><ce:author-group><ce:author><ce:given-name>Satish C.</ce:given-name><ce:surname>Muluk</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:author><ce:given-name>Leo C.</ce:given-name><ce:surname>Ginns</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:author><ce:given-name>Marc J.</ce:given-name><ce:surname>Semigran</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:author><ce:given-name>John A.</ce:given-name><ce:surname>Kaufman</ce:surname></ce:author><ce:author><ce:given-name>Jonathan P.</ce:given-name><ce:surname>Gertler</ce:surname><ce:degrees>MD</ce:degrees></ce:author><ce:affiliation><ce:textfn>Boston, Mass.</ce:textfn></ce:affiliation></ce:author-group><ce:date-received day="13" month="7" year="1994"></ce:date-received><ce:date-accepted day="5" month="11" year="1994"></ce:date-accepted><ce:abstract><ce:section-title>Abstract</ce:section-title><ce:abstract-sec><ce:simple-para>A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J V<ce:small-caps>ASC</ce:small-caps> S<ce:small-caps>URG</ce:small-caps> 1995;21:686-90.)</ce:simple-para></ce:abstract-sec></ce:abstract></head><body><ce:sections><ce:para>The Klippel-Trénaunay syndrome (KTS) is a rare congenital angiodysplastic syndrome classically consisting of the triad of a vascular nevus, varicose veins, and bony and soft-tissue hypertrophy. Myriad other associated abnormalities, including varying degrees of lymphedema, may also be present. Recognition of the syndrome is important because identification of the underlying angiodysplastic syndrome carries major implications for therapy and prognosis of the problems that the vascular surgeon may be asked to see.</ce:para><ce:para>Manifestations of the syndrome are numerous because of the wide range of anomalies that may be present. We detail herein the fifth English-language report of a patient with multiple pulmonary emboli as a consequence of KTS.<ce:cross-refs refid="bib1 bib2 bib3 bib4"><ce:sup>1-4</ce:sup></ce:cross-refs></ce:para><ce:para>KTS has been reported extensively in the French, Italian, and German literature.<ce:cross-refs refid="bib5 bib6"><ce:sup>5,6</ce:sup></ce:cross-refs> This may be a result of lack of recognition of the syndrome as a distinct entity. For example, the patient presented in this report was not diagnosed as having KTS until this admission to our hospital at age 32, despite having had symptoms since childhood. We review this unusual syndrome, with special emphasis on aspects important to the vascular surgeon.</ce:para><ce:section><ce:section-title>CASE REPORT</ce:section-title><ce:para>A 32-year-old white man with a diagnosis of primary pulmonary hypertension was admitted to our institution for lung transplant evaluation. Vascular surgical consultation was requested to assist in management of his congenital lymphedema and recurrent cellulitis treated with intravenous antibiotics, because poor control of potential septic foci would increase the morbidity of immunosuppression.</ce:para><ce:para>His pulmonary problems dated back only 7 months, at which time he noted the gradual onset of dyspnea. Subsequently, he progressed to the point at which he was unable to walk more than 20 yards. He gave a history of "pleurisy" 5 years before his current admission but otherwise had no pulmonary history. Medical history was also notable for two previous left leg–shortening procedures at age 13 and 14, as well as surgically corrected left carpal tunnel syndrome.</ce:para><ce:para>Evaluation at another institution had included an echocardiogram, which reportedly showed right ventricular enlargement and moderate tricuspid regurgitation. A ventilation/perfusion scan was interpreted as being low probability for pulmonary embolus. Bilateral duplex lower extremity study was reported to show no evidence of deep venous thrombosis. Right-sided heart catheterization reportedly demonstrated pulmonary arterial pressure of 90/32 (mean 50) and no evidence of left-to-right shunt. A pulmonary arteriogram had been read as showing no evidence of emboli. Pulmonary function testing had revealed a first second vital capacity of 2.38 liters (60% predicted), with a vital capacity of 2.96 liters (63% predicted). Chest radiography demonstrated pleural thickening and a small effusion.</ce:para><ce:para>Physical examination of the extremities revealed soft tissue hypertrophy of both the left leg and left arm, as well as marked nonpitting edema in the legs (left-to-right), and syndactyly in both feet (<ce:cross-ref refid="fig1">Fig. 1</ce:cross-ref>).
<ce:display><ce:figure id="fig1"><ce:label>Fig. 1</ce:label><ce:caption><ce:simple-para>Photograph of both legs <ce:bold>(A)</ce:bold> and closeup of larger left leg <ce:bold>(B).</ce:bold></ce:simple-para></ce:caption> <ce:link locator="gr1a"></ce:link><ce:link locator="gr1b"></ce:link></ce:figure></ce:display>No other arm or hand abnormalities were noted. Prominent superficial varicosities in medial, lateral, and posterior aspects of his legs were observed on standing. The left leg was several inches longer and markedly warmer than the right, but no bruit or thrill was found. He had a capillary vascular malformation on the posterior left calf and in both legs demonstrated stasis dermatitis with hemosiderin deposition, vesicles in the skin, but no ulceration or cellulitis. Normal arterial pulses were palpated.</ce:para><ce:para>A diagnosis of KTS was made on the basis of the history and examination result (<ce:cross-ref refid="tab1">Table I</ce:cross-ref>).
<ce:float-anchor refid="tab1"></ce:float-anchor>Because of the known deep venous malformations seen in KTS, and the possibility of resultant pulmonary emboli, the diagnosis of primary pulmonary hypertension was critically reassessed. Review of the pulmonary arteriogram obtained at the referring institution showed it to be a suboptimal study. The study, along with right-sided heart catheterization, was therefore repeated. The new study showed unequivocal evidence of multiple subacute and chronic pulmonary embolism, as well as pleural effusions (<ce:cross-ref refid="fig2">Fig. 2</ce:cross-ref>).
<ce:display><ce:figure id="fig2"><ce:label>Fig. 2</ce:label><ce:caption><ce:simple-para>Pulmonary arteriogram demonstrates both <ce:bold>(A)</ce:bold> recent embolus <ce:italic>(arrow)</ce:italic> and <ce:bold>(B)</ce:bold> chronic embolus with recanalization <ce:italic>(arrow).</ce:italic></ce:simple-para></ce:caption> <ce:link locator="gr2a"></ce:link><ce:link locator="gr2b"></ce:link></ce:figure></ce:display>Additionally, the repeated pulmonary artery pressure was only 36/12 (mean 22). In view of the new diagnosis, consideration of lung transplantation was deferred. 40 to 50 mm Hg–weight compressive stockings (thigh high) were fitted, and anticoagulation with warfarin was begun.</ce:para><ce:para>Contrast venography of the pelvis and abdomen demonstrated normal iliac veins and inferior vena cava. Magnetic resonance venography of the thighs showed multiple deep venous occlusions in both legs, as well as large superficial varicosities. Incompetent deep and superficial valves were evidenced on the magnetic resonance venogram by signal loss as a result of episodic retrograde flow to the level of the popliteal vein. There was no evidence of arteriovenous shunting.</ce:para><ce:para>The patient was discharged after therapeutic warfarin anticoagulation was achieved. He has done well in short-term follow-up without progression of dyspnea.</ce:para></ce:section><ce:section><ce:section-title>DISCUSSION</ce:section-title><ce:para>This case illustrates the importance of recognizing KTS. Identification of this syndrome led to a search for pulmonary emboli.</ce:para><ce:para>KTS, or angioosteohypertrophy, was first described by Klippel and Trénaunay in 1900.<ce:cross-ref refid="bib7"><ce:sup>7</ce:sup></ce:cross-ref> When the classical triad of (1) vascular nevus, (2) varicose veins, and (3) bony and soft-tissue hypertrophy is accompanied by hemodynamically significant arteriovenous fistulas (AVF), many authors follow the recommendation of Lindenauer<ce:cross-ref refid="bib6"><ce:sup>6</ce:sup></ce:cross-ref> and use the designation Parkes-Weber syndrome, recognizing the contribution of Parkes-Weber in 1918.<ce:cross-ref refid="bib8"><ce:sup>8</ce:sup></ce:cross-ref> The distinction is important, because the presence of clinically apparent AVF is associated with a significantly worse prognosis.<ce:cross-refs refid="bib5 bib6"><ce:sup>5,6</ce:sup></ce:cross-refs> The patient discussed in this report had no evidence of hemodynamically significant AVFs and would therefore be considered to have KTS alone.</ce:para><ce:para>The cause of KTS is unknown, but one plausible theory is that of an intrauterine insult at the time of vascular differentiation, with resultant malformation of the venous drainage of the extremity.<ce:cross-refs refid="bib3 bib9"><ce:sup>3,9</ce:sup></ce:cross-refs> Servelle,<ce:cross-ref refid="bib10"><ce:sup>10</ce:sup></ce:cross-ref> by experimental induction of venous stasis, suggested that the lack of normal venous drainage may be an etiologic factor in hypertrophied and elongated extremities. Others have suggested that intrauterine injury to the sympathetic ganglia or intermediate lateral tract may lead to loss of sympathetic tone, with arteriovenous shunt dilation and subsequent venous engorgement.<ce:cross-ref refid="bib11"><ce:sup>11</ce:sup></ce:cross-ref> A related theory<ce:cross-ref refid="bib12"><ce:sup>12</ce:sup></ce:cross-ref> holds that an underlying mesodermal defect during fetal development leads to the abnormal maintenance of microscopic AVF. Evidence of an inherited factor is rare, but a few familial cases have been reported.<ce:cross-refs refid="bib4 bib13 bib14"><ce:sup>4,13,14</ce:sup></ce:cross-refs></ce:para><ce:para>The feature that will most often be seen by the vascular surgeon is that of superficial venous varicosities. Onset in childhood is frequently noted, and the location of the varicosities is often atypical (often found to be on the lateral aspect of the limb, sweeping medially at the groin). Chronic venous insufficiency is common as patients advance into the second and third decades, and associated venous stasis ulcers can be found in unusual locations. The association of such venous disease with a congenital vascular nevus and limb hypertrophy should lead to a diagnosis of KTS.</ce:para><ce:para>Even when clinically significant AVF are absent, the affected limb is often noticeably warmer. Generally, only one lower limb is affected. However, as in the patient presented in this report, combinations of upper and lower limb involvement can occur. Venous abnormalities can extend into the pelvis and abdomen, with resultant rectal bleeding (from colonic arteriovenous malformation) and hematuria (from bladder arteriovenous malformation).</ce:para><ce:para>Lymphedema (as noted in the patient reported herein) and cutaneous lymphatic vesicles are occasionally seen. There is also a high incidence of congenital bony abnormalities, including syndactyly (seen in our patient), pelvic nonfusion, talipes equinovarus, spina bifida, and scoliosis. A variety of orofacial anomalies have also been reported.</ce:para><ce:para>Although deep venous thrombosis is not uncommon in KTS, reports of pulmonary embolism are rare.<ce:cross-ref refid="bib15"><ce:sup>15</ce:sup></ce:cross-ref> There have been four previous English-language case reports of pulmonary emboli in patients with KTS.<ce:cross-refs refid="bib1 bib2 bib3 bib4"><ce:sup>1-4</ce:sup></ce:cross-refs> One of the two patients<ce:cross-ref refid="bib2"><ce:sup>2</ce:sup></ce:cross-ref> had progressive dyspnea over a long period, similar to the patient we described.</ce:para><ce:para>Although the diagnosis of KTS is clinical, imaging of the extremity's venous system is an important adjunctive study. Common findings include hypoplastic or absent deep venous system, venous obstruction by fibrous bands or adjacent arteries, and incompetent valves. Atypical venous drainage pathways are common. Although contrast venography has been the usual imaging modality, magnetic resonance imaging is an alternative technique, as demonstrated in the case presented here. Servelle et al.<ce:cross-refs refid="bib16 bib17 bib18"><ce:sup>16-18</ce:sup></ce:cross-refs> found complete or partial obstruction of the deep system in a large proportion of his patients with KTS. He noted a marked improvement in many patients with surgical relief of venous obstruction. However, other authors have found only a small incidence of surgically correctable venous obstruction.<ce:cross-refs refid="bib3 bib4 bib10 bib13 bib17"><ce:sup>3,4,10,13,17</ce:sup></ce:cross-refs> In selected cases, pelvic venous anatomy may have to be defined to assess for colonic and bladder arteriovenous malformation. Another useful diagnostic study is arteriography, to determine whether clinically important AVF exist.</ce:para><ce:para>Management of venous insufficiency in patients with KTS should certainly include lifelong compression stocking therapy. This is adequate therapy for patients with mild symptoms. When symptoms are more severe, surgical relief of venous obstruction is warranted when such obstruction can be demonstrated<ce:cross-ref refid="bib14"><ce:sup>14</ce:sup></ce:cross-ref> and when a correctable situation exists. The role of superficial vein ligation and stripping is controversial. Although some authors report improvement of symptoms after ligation and stripping,<ce:cross-refs refid="bib17 bib18"><ce:sup>17,18</ce:sup></ce:cross-refs> others have noted marked worsening of symptoms,<ce:cross-ref refid="bib4"><ce:sup>4</ce:sup></ce:cross-ref> possibly as a result of reduction of already compromised venous drainage. Even when early symptomatic relief has been noted, a high varicose vein recurrence rate is to be expected.<ce:cross-ref refid="bib17"><ce:sup>17</ce:sup></ce:cross-ref> Excisional surgery for lymphedema in KTS should be undertaken with great caution because of the risk of poor wound healing.<ce:cross-ref refid="bib17"><ce:sup>17</ce:sup></ce:cross-ref></ce:para><ce:para>Lack of any large experience with thromboembolic phenomena in patients with KTS makes impossible any firm treatment recommendations. However, warfarin anticoagulation, with caval filter placement for warfarin failures, seems to be a logical approach. Clear venographic documentation of intraabdominal collateral pathways is imperative. In a patient with such marked pulmonary compromise that recurrent embolism appears highly dangerous, primary filter placement is prudent.<ce:cross-ref refid="bib2"><ce:sup>2</ce:sup></ce:cross-ref> When filters are used, aberrant sources and pathways of emboli must be considered, with suprarenal caval filter placement a possibility based on venous anatomic findings.<ce:cross-refs refid="bib1 bib2"><ce:sup>1,2</ce:sup></ce:cross-refs></ce:para></ce:section></ce:sections></body><tail><ce:bibliography><ce:section-title>References</ce:section-title><ce:bibliography-sec><ce:bib-reference id="bib1"><ce:label>1</ce:label><sb:reference><sb:contribution><sb:authors><sb:author><ce:given-name>AN</ce:given-name><ce:surname>Awad</ce:surname></sb:author><sb:author><ce:given-name>DC</ce:given-name><ce:surname>Yang</ce:surname></sb:author><sb:author><ce:given-name>M</ce:given-name><ce:surname>Girgis</ce:surname></sb:author><sb:et-al></sb:et-al></sb:authors><sb:title><sb:maintitle>Evaluation of 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lang="en"><title>Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title></titleInfo><titleInfo type="alternative" lang="en" contentType="CDATA"><title>Klippel-Trénaunay syndrome with multiple pulmonary emboli – An unusual cause of progressive pulmonary dysfunction</title></titleInfo><name type="personal"><namePart type="given">Satish C.</namePart><namePart type="family">Muluk</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Boston, Mass.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Leo C.</namePart><namePart type="family">Ginns</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Boston, Mass.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Marc J.</namePart><namePart type="family">Semigran</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Boston, Mass.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">John A.</namePart><namePart type="family">Kaufman</namePart><affiliation>Boston, Mass.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jonathan P.</namePart><namePart type="family">Gertler</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Boston, Mass.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="Full-length article"></genre><originInfo><publisher>ELSEVIER</publisher><dateIssued encoding="w3cdtf">1995</dateIssued><copyrightDate encoding="w3cdtf">1995</copyrightDate></originInfo><language><languageTerm type="code" authority="iso639-2b">eng</languageTerm><languageTerm type="code" authority="rfc3066">en</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType></physicalDescription><abstract lang="en">A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented. (J VASC SURG 1995;21:686-90.)</abstract><note>From the Division of Vascular Surgery, Department of Surgery, the Pulmonary and Critical Care Unit (Dr. Ginns), and Division of Cardiology (Dr. Semigran), General Medical Services, and the Department of Radiology (Mr. Kaufman), Massachusetts General Hospital, Harvard Medical School, Boston.</note><note>Reprint request: Jonathan P. Gertler, MD, Massachusetts General Hospital, Ambulatory Care Center, Suite 464, 15 Parkman St., Boston, MA 02114.</note><note>0741-5214/95/$3.00 + 0 24/4/61920</note><note type="content">Table I: Major findings that led to diagnosis of KTS</note><relatedItem type="host"><titleInfo><title>Journal of Vascular Surgery</title></titleInfo><titleInfo type="abbreviated"><title>YMVA</title></titleInfo><genre type="journal">journal</genre><originInfo><dateIssued encoding="w3cdtf">199504</dateIssued></originInfo><identifier type="ISSN">0741-5214</identifier><identifier type="PII">S0741-5214(05)X7163-4</identifier><part><date>199504</date><detail type="volume"><number>21</number><caption>vol.</caption></detail><detail type="issue"><number>4</number><caption>no.</caption></detail><extent unit="issue pages"><start>549</start><end>711</end></extent><extent unit="pages"><start>686</start><end>690</end></extent></part></relatedItem><identifier type="istex">1E4F06ECB32B11D760BAD59AEC7B954969333335</identifier><identifier type="DOI">10.1016/S0741-5214(95)70199-0</identifier><identifier type="PII">S0741-5214(95)70199-0</identifier><accessCondition type="use and reproduction" contentType="copyright">©1995 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter</accessCondition><recordInfo><recordContentSource>ELSEVIER</recordContentSource><recordOrigin>Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter, ©1995</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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