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Available in 16 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-16" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">16 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D9%88%D8%B1%D9%85_%D9%88%D8%B9%D8%A7%D8%A6%D9%8A_%D9%84%D9%85%D9%81%D9%8A" title="ورم وعائي لمفي – Arabic" lang="ar" hreflang="ar" data-title="ورم وعائي لمفي" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-ca mw-list-item"><a href="https://ca.wikipedia.org/wiki/Limfangioma" title="Limfangioma – Catalan" lang="ca" hreflang="ca" data-title="Limfangioma" data-language-autonym="Català" data-language-local-name="Catalan" class="interlanguage-link-target"><span>Català</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/Lymphangiom" title="Lymphangiom – German" lang="de" hreflang="de" data-title="Lymphangiom" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Linfangioma" title="Linfangioma – Spanish" lang="es" hreflang="es" data-title="Linfangioma" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fa mw-list-item"><a href="https://fa.wikipedia.org/wiki/%D9%84%D9%86%D9%81%D8%A7%D9%86%DA%98%DB%8C%D9%88%D9%85" title="لنفانژیوم – Persian" lang="fa" hreflang="fa" data-title="لنفانژیوم" data-language-autonym="فارسی" data-language-local-name="Persian" class="interlanguage-link-target"><span>فارسی</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Malformation_lymphatique" title="Malformation lymphatique – French" lang="fr" hreflang="fr" data-title="Malformation lymphatique" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Linfangioma" title="Linfangioma – Italian" lang="it" hreflang="it" data-title="Linfangioma" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-kk mw-list-item"><a href="https://kk.wikipedia.org/wiki/%D0%9B%D0%B8%D0%BC%D1%84%D0%B0%D0%BD%D0%B3%D0%B8%D0%BE%D0%BC%D0%B0" title="Лимфангиома – Kazakh" lang="kk" hreflang="kk" data-title="Лимфангиома" data-language-autonym="Қазақша" data-language-local-name="Kazakh" class="interlanguage-link-target"><span>Қазақша</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E3%83%AA%E3%83%B3%E3%83%91%E7%AE%A1%E8%85%AB" title="リンパ管腫 – Japanese" lang="ja" hreflang="ja" data-title="リンパ管腫" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/Linfangioma" title="Linfangioma – Portuguese" lang="pt" hreflang="pt" data-title="Linfangioma" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-ru mw-list-item"><a href="https://ru.wikipedia.org/wiki/%D0%9B%D0%B8%D0%BC%D1%84%D0%B0%D0%BD%D0%B3%D0%B8%D0%BE%D0%BC%D0%B0" title="Лимфангиома – Russian" lang="ru" hreflang="ru" data-title="Лимфангиома" data-language-autonym="Русский" data-language-local-name="Russian" class="interlanguage-link-target"><span>Русский</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/%D0%9B%D0%B8%D0%BC%D1%84%D0%B0%D0%BD%D0%B3%D0%B8%D0%BE%D0%BC" title="Лимфангиом – Serbian" lang="sr" hreflang="sr" data-title="Лимфангиом" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-sv mw-list-item"><a href="https://sv.wikipedia.org/wiki/Lymfangiom" title="Lymfangiom – Swedish" lang="sv" hreflang="sv" data-title="Lymfangiom" data-language-autonym="Svenska" data-language-local-name="Swedish" class="interlanguage-link-target"><span>Svenska</span></a></li><li class="interlanguage-link interwiki-tt mw-list-item"><a href="https://tt.wikipedia.org/wiki/%D0%9B%D0%B8%D0%BC%D1%84%D0%B0%D0%BD%D0%B3%D0%B8%D0%BE%D0%BC%D0%B0_(%D0%BA%D2%AF%D0%B7%D0%B4%D3%99)" title="Лимфангиома (күздә) – Tatar" lang="tt" hreflang="tt" data-title="Лимфангиома (күздә)" data-language-autonym="Татарча / tatarça" data-language-local-name="Tatar" class="interlanguage-link-target"><span>Татарча / tatarça</span></a></li><li class="interlanguage-link interwiki-tr mw-list-item"><a href="https://tr.wikipedia.org/wiki/Lenfanjiyoma" title="Lenfanjiyoma – Turkish" lang="tr" hreflang="tr" data-title="Lenfanjiyoma" data-language-autonym="Türkçe" data-language-local-name="Turkish" class="interlanguage-link-target"><span>Türkçe</span></a></li><li class="interlanguage-link interwiki-ur mw-list-item"><a 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Unsourced material may be challenged and removed.<br /><small><span class="plainlinks"><i>Find sources:</i> <a rel="nofollow" class="external text" href="https://www.google.com/search?as_eq=wikipedia&q=%22Lymphatic+malformations%22">"Lymphatic malformations"</a> – <a rel="nofollow" class="external text" href="https://www.google.com/search?tbm=nws&q=%22Lymphatic+malformations%22+-wikipedia&tbs=ar:1">news</a> <b>·</b> <a rel="nofollow" class="external text" href="https://www.google.com/search?&q=%22Lymphatic+malformations%22&tbs=bkt:s&tbm=bks">newspapers</a> <b>·</b> <a rel="nofollow" class="external text" href="https://www.google.com/search?tbs=bks:1&q=%22Lymphatic+malformations%22+-wikipedia">books</a> <b>·</b> <a rel="nofollow" class="external text" href="https://scholar.google.com/scholar?q=%22Lymphatic+malformations%22">scholar</a> <b>·</b> <a rel="nofollow" class="external text" href="https://www.jstor.org/action/doBasicSearch?Query=%22Lymphatic+malformations%22&acc=on&wc=on">JSTOR</a></span></small></span> <span class="date-container"><i>(<span class="date">September 2024</span>)</i></span><span class="hide-when-compact"><i> (<small><a href="/wiki/Help:Maintenance_template_removal" title="Help:Maintenance template removal">Learn how and when to remove this message</a></small>)</i></span></div></td></tr></tbody></table> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Lymphatic malformation or Lymphangioma</th></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Lymphangioma.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/2/2b/Lymphangioma.jpg/220px-Lymphangioma.jpg" decoding="async" width="220" height="148" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/2/2b/Lymphangioma.jpg/330px-Lymphangioma.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/2/2b/Lymphangioma.jpg/440px-Lymphangioma.jpg 2x" data-file-width="605" data-file-height="407" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">Lymphatic malformation of the neck</td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Oncology" title="Oncology">Oncology</a> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q1545750?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr></tbody></table> <p><b>Lymphatic malformations</b> are benign slow-flow type of <a href="/wiki/Vascular_malformation" title="Vascular malformation">vascular malformation</a> of the <a href="/wiki/Lymphatic_system" title="Lymphatic system">lymphatic system</a> characterized by lymphatic vessels which do not connect to the normal lymphatic circulation. The term <i>lymphangioma</i> is outdated and newer research reference the term <i>lymphatic malformation</i>. </p><p>Lymphatic malformations can be macrocystic, microcystic, or a combination of the two.<sup id="cite_ref-Elluru_1-0" class="reference"><a href="#cite_note-Elluru-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> Macrocystic have cysts greater than 2 cubic centimetres (0.12 cu in), and microcystic lymphatic malformation have cysts that are smaller than 2 cubic centimetres (0.12 cu in).<sup id="cite_ref-NIH_2-0" class="reference"><a href="#cite_note-NIH-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas are often associated with <a href="/wiki/Chromosomal_abnormalities" class="mw-redirect" title="Chromosomal abnormalities">chromosomal abnormalities</a> such as <a href="/wiki/Turner_syndrome" title="Turner syndrome">Turner syndrome</a>, although they can also exist in isolation. Lymphangiomas are commonly diagnosed before birth using <a href="/wiki/Fetal_ultrasonography" class="mw-redirect" title="Fetal ultrasonography">fetal ultrasonography</a>. Acquired lymphangiomas may result from trauma, inflammation, or lymphatic obstruction. </p><p>Most lymphangiomas are benign lesions that result only in a soft, slow-growing, "doughy" mass. Since they have no chance of becoming malignant, lymphangiomas are usually treated for cosmetic reasons only. Rarely, impingement upon critical organs may result in complications, such as <a href="/wiki/Respiratory_distress" class="mw-redirect" title="Respiratory distress">respiratory distress</a> when a lymphangioma compresses the airway. Treatment includes <a href="/wiki/Needle_aspiration_biopsy" class="mw-redirect" title="Needle aspiration biopsy">aspiration</a>, <a href="/wiki/Surgical_excision" class="mw-redirect" title="Surgical excision">surgical excision</a>, <a href="/wiki/Laser_ablation" title="Laser ablation">laser</a> and <a href="/wiki/Radiofrequency_ablation" title="Radiofrequency ablation">radiofrequency ablation</a>, and <a href="/wiki/Sclerotherapy" title="Sclerotherapy">sclerotherapy</a>. </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Terminology">Terminology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=1" title="Edit section: Terminology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The International Society for the Study of Vascular Anomalies (ISSVA) classification is a basic and systematic classification of vascular anomalies with international acceptance.<sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>As such, terms such as "lymphangioma" and "cystic hygroma", which were used widely in the past, are outdated. Newer research may only reference ISSVA terminology and, as a consequence, sources of information can be missed by doctors and patients unaware of the ISSVA convention. </p> <table class="wikitable"> <caption>Terminology </caption> <tbody><tr> <th>ISSVA name</th> <th>Outdated names </th></tr> <tr> <td>Lymphatic malformation</td> <td>Lymphangioma, Cystic hygroma, Lymphangioma circumscriptum, Cavernous lymphangioma, lymphangiomatosis </td></tr> <tr> <td>Venous malformation</td> <td>Cavernous hemangioma </td></tr></tbody></table> <div class="mw-heading mw-heading3"><h3 id="Notes">Notes</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=2" title="Edit section: Notes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li>When cysts were very large, particularly on the neck, doctors used to classify them as "cystic hygromas". Location aside, they are not different from other lymphatic malformations.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li></ul> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=3" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There are three distinct types of lymphangioma, each with their own symptoms. They are distinguished by the depth and the size of abnormal lymph vessels, but all involve a malformation of the lymphic system. Lymphangioma circumscriptum can be found on the skin's surface, and the other two types of lymphangiomas occur deeper under the skin.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p> <ul><li><b>Lymphangioma circumscriptum</b>, a microcystic lymphatic malformation, resembles clusters of small blisters ranging in color from pink to dark red.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> They are benign and do not require medical treatment, although some patients may choose to have them surgically removed for cosmetic reasons.</li> <li><b>Cavernous lymphangiomas</b> are generally present at birth, but may appear later in the child's life.<sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> These bulging masses occur deep under the skin, typically on the neck, tongue and lips,<sup id="cite_ref-Fernandez_7-0" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> and vary widely in size, ranging from as small as a centimeter in diameter to several centimeters wide. In some cases, they may affect an entire extremity such as a hand or foot. Although they are usually painless, the patient may feel mild pain when pressure is exerted on the area. They come in the colors white, pink, red, blue, purple, and black; and the pain lessens the lighter the color of the bump.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></li> <li><b>Cystic hygroma</b> shares many commonalities with cavernous lymphangiomas, and some doctors consider them to be too similar to merit separate categories. However, cystic lymphangiomas usually have a softer consistency than cavernous lymphangiomas, and this term is typically the one that is applied to lymphangiomas that develop in fetuses. They usually appear on the neck (75%), arm pit or groin areas. They often look like swollen bulges underneath the skin.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></li></ul> <div class="mw-heading mw-heading2"><h2 id="Causes">Causes</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=4" title="Edit section: Causes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The direct cause of lymphatic malformation is a blockage of the lymphatic system as a <a href="/wiki/Fetus" title="Fetus">fetus</a> develops, although symptoms may not become visible until after the baby is born. The cause remains unknown. Why the embryonic lymph sacs remain disconnected from the rest of the lymphatic system is also not known.<sup id="cite_ref-Fernandez_7-1" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>Cystic lymphangioma that emerges during the <a href="/wiki/Pregnancy#Second_trimester" title="Pregnancy">first two trimesters</a> of pregnancy is associated with genetic disorders such as <a href="/wiki/Noonan_syndrome" title="Noonan syndrome">Noonan syndrome</a> and trisomies <a href="/wiki/Patau_syndrome" title="Patau syndrome">13</a>, <a href="/wiki/Edwards_syndrome" class="mw-redirect" title="Edwards syndrome">18</a>, and 21. <a href="/wiki/Aneuploidy" title="Aneuploidy">Chromosomal aneuploidy</a> such as <a href="/wiki/Turner_syndrome" title="Turner syndrome">Turner syndrome</a> or <a href="/wiki/Down_syndrome" title="Down syndrome">Down syndrome</a><sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> were found in 40% of patients with cystic hygroma.<sup id="cite_ref-pmid1603503_9-0" class="reference"><a href="#cite_note-pmid1603503-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Pathophysiology">Pathophysiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=5" title="Edit section: Pathophysiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:SkinTumors-PA100979.JPG" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/a/ab/SkinTumors-PA100979.JPG/220px-SkinTumors-PA100979.JPG" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/ab/SkinTumors-PA100979.JPG/330px-SkinTumors-PA100979.JPG 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/ab/SkinTumors-PA100979.JPG/440px-SkinTumors-PA100979.JPG 2x" data-file-width="1600" data-file-height="1200" /></a><figcaption>Proliferating lymphangioma, <a href="/wiki/H%26E_stain" title="H&E stain">H&E stain</a>. Sometimes <a href="/wiki/Endothelium" title="Endothelium">endothelial cells</a> begin to divide excessively.</figcaption></figure> <p>In 1976, Whimster studied the <a href="/wiki/Pathogenesis" title="Pathogenesis">pathogenesis</a> of lymphangioma circumscriptum, finding lymphatic cisterns in the deep subcutaneous plane are separated from the normal network of lymph vessels. They communicate with the superficial lymph vesicles through vertical, dilated lymph channels. Whimster theorized the cisterns might come from a primitive lymph sac that failed to connect with the rest of the lymphatic system during embryonic development.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p><p>A thick coat of muscle fibers that cause rhythmic contractions line the sequestered primitive sacs. Rhythmic contractions increase the intramural pressure, causing dilated channels to come from the walls of the cisterns toward the skin. He suggested that the vesicles seen in lymphangioma circumscriptum are outpouchings of these dilated projecting vessels. Lymphatic and radiographic studies support Whimsters observations. Such studies reveal that big cisterns extend deeply into the skin and beyond the clinical lesions. Lymphangiomas that are deep in the dermis show no evidence of communication with the regular lymphatics. The cause for the failure of lymph sacs to connect with the lymphatic system is not known.<sup id="cite_ref-Fernandez_7-2" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>Microscopically, the vesicles in lymphangioma circumscriptum are greatly dilated lymph channels that cause the <a href="/wiki/Papillary_dermis" class="mw-redirect" title="Papillary dermis">papillary dermis</a> to expand. They may be associated with <a href="/wiki/Acanthosis" class="mw-redirect" title="Acanthosis">acanthosis</a> and <a href="/wiki/Hyperkeratosis" title="Hyperkeratosis">hyperkeratosis</a>. There are many channels in the upper dermis which often extend to the <a href="/wiki/Subcutis" class="mw-redirect" title="Subcutis">subcutis</a> (the deeper layer of the dermis, containing mostly fat and connective tissue). The deeper vessels have large calibers with thick walls which contain <a href="/wiki/Smooth_muscle" title="Smooth muscle">smooth muscle</a>. The lumen is filled with lymphatic fluid, but often contains red blood cells, lymphocytes, <a href="/wiki/Macrophages" class="mw-redirect" title="Macrophages">macrophages</a>, and <a href="/wiki/Neutrophils" class="mw-redirect" title="Neutrophils">neutrophils</a>. The channels are lined with flat <a href="/wiki/Endothelial_cells" class="mw-redirect" title="Endothelial cells">endothelial cells</a>. The <a href="/wiki/Interstitium" title="Interstitium">interstitium</a> has many lymphoid cells and shows evidence of <a href="/wiki/Fibroplasia" class="mw-redirect" title="Fibroplasia">fibroplasia</a> (the formation of fibrous tissue). Nodules (A small mass of tissue or aggregation of cells) in cavernous lymphangioma are large, irregular channels in the reticular dermis and subcutaneous tissue that are lined by a single layer of <a href="/wiki/Endothelial_cells" class="mw-redirect" title="Endothelial cells">endothelial cells</a>. Also an incomplete layer of smooth muscle also lines the walls of these channels. The stroma consists of loose connective tissue with a lot of inflammatory cells. These tumors usually penetrate muscle. Cystic hygroma is indistinguishable from cavernous lymphangiomas on histology.<sup id="cite_ref-Fernandez_7-3" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>The typical history of Lymphangioma circumscriptum shows a small number of vesicles on the skin at birth or shortly after. In subsequent years, they tend to increase in number, and the area of skin involved continues to expand. Vesicles or other skin abnormalities may not be noticed until several years after birth. Usually, lesions are asymptomatic or do not show any evidence of a disease, but, mostly, patients may have random break outs of some bleeding and major drainage of clear fluid from ruptured vesicles.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p><p><i>Cavernous lymphangioma</i> first appears during infancy, when a rubbery nodule with no skin changes becomes obvious in the face, trunk, or extremity. These lesions often grow at a rapid pace, similar to that of raised <a href="/wiki/Hemangioma" title="Hemangioma">hemangiomas</a>. No family history of prior lymphangiomas is described.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p><p><a href="/wiki/Cystic_hygroma" title="Cystic hygroma">Cystic hygroma</a> causes deep subcutaneous cystic swelling, usually in the axilla, base of the neck, or groin, and is typically noticed soon after birth. If the lesions are drained, they will rapidly fill back up with fluid. The lesions will grow and increase to a larger size if they are not completely removed in surgery.<sup id="cite_ref-Fernandez_7-4" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=6" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Cases of lymphangioma are diagnosed by <a href="/wiki/Histopathology" title="Histopathology">histopathologic</a> inspection.<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> In prenatal cases, lymphangioma is diagnosed during the late first trimester or early second trimester using an ultrasound. Other imaging methods such as CT and MRI scans are useful in treatment planning, delineate the size of the lesion, and determine its surrounding vital structures. T2-weight MRI is useful to differentiate lymphangioma from surrounding structures due to its high T2 signal.<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Classification">Classification</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=7" title="Edit section: Classification"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:SkinTumors-PB061069.JPG" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/1/10/SkinTumors-PB061069.JPG/220px-SkinTumors-PB061069.JPG" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/1/10/SkinTumors-PB061069.JPG/330px-SkinTumors-PB061069.JPG 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/1/10/SkinTumors-PB061069.JPG/440px-SkinTumors-PB061069.JPG 2x" data-file-width="1600" data-file-height="1200" /></a><figcaption>Cavernous lymphangioma, <a href="/wiki/H%26E_stain" title="H&E stain">H&E stain</a>. Irregular, dilated spaces are visible in <a href="/wiki/Dermis" title="Dermis">dermis</a>.</figcaption></figure> <p>Lymphangiomas have traditionally been classified into three subtypes: <i>capillary</i> and <i>cavernous lymphangiomas</i> and <a href="/wiki/Cystic_hygroma" title="Cystic hygroma">cystic hygroma</a>. This classification is based on their microscopic characteristics. A fourth subtype, the <i>hemangiolymphangioma</i> is also recognized.<sup id="cite_ref-Giguère_12-0" class="reference"><a href="#cite_note-Giguère-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <dl><dt>Capillary lymphangiomas</dt> <dd>Capillary lymphangiomas are composed of small, capillary-sized lymphatic vessels and are characteristically located in the <a href="/wiki/Epidermis_(zoology)" title="Epidermis (zoology)">epidermis</a>.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></dd> <dt>Cavernous lymphangiomas</dt> <dd>Composed of dilated lymphatic channels, cavernous lymphangiomas characteristically invade surrounding tissues.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></dd> <dt><a href="/wiki/Cystic_hygroma" title="Cystic hygroma">Cystic hygromas</a></dt> <dd><a href="/wiki/Cystic_hygroma" title="Cystic hygroma">Cystic hygromas</a> are large, macrocystic lymphangiomas filled with straw-colored, protein-rich fluid.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></dd> <dt>Hemangiolymphangioma</dt> <dd>As suggested by their name, hemangiolymphangiomas are lymphangiomas with a vascular component.</dd></dl> <p>Lymphangiomas may also be classified into <i>microcystic</i>, <i>macrocystic</i>, and <i>mixed</i> subtypes, according to the size of their cysts.<sup id="cite_ref-Giguère_12-1" class="reference"><a href="#cite_note-Giguère-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <dl><dt>Microcystic lymphangiomas</dt> <dd>Microcystic lymphangiomas are composed of cysts, each of which measures less than 2 cm<sup>3</sup> in volume.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup></dd> <dt>Macrocystic lymphangiomas</dt> <dd>Macrocystic lymphangiomas contain cysts measuring more than 2 cm<sup>3</sup> in volume.</dd> <dt>Mixed lymphangiomas</dt> <dd>Lymphangiomas of the mixed type contain both microcystic and macrocystic components.</dd></dl> <p>Finally, lymphangiomas may be described in stages, which vary by location and extent of disease. In particular, stage depends on whether lymphangiomas are present above or <i>superior</i> to the <a href="/wiki/Hyoid_bone" title="Hyoid bone">hyoid bone</a> (<i>suprahyoid</i>), below or <i>inferior</i> to the hyoid bone (<i>infrahyoid</i>), and whether the lymphangiomas are on one side of the body (<i>unilateral</i>) or both (<i>bilateral</i>).<sup id="cite_ref-Giguère_12-2" class="reference"><a href="#cite_note-Giguère-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <dl><dt>Stage I</dt> <dd>Unilateral infrahyoid.</dd> <dt>Stage II</dt> <dd>Unilateral suprahyoid.</dd> <dt>Stage III</dt> <dd>Unilateral suprahyoid and infrahyoid.</dd> <dt>Stage IV</dt> <dd>Bilateral suprahyoid.</dd> <dt>Stage V</dt> <dd>Bilateral suprahyoid and infrahyoid.</dd></dl> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=8" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Treatment for cystic hygroma involves the removal of the abnormal tissue; however, complete removal may be impossible without removing other normal areas. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows. Most patients need at least two procedures done for the removal process to be achieved. Recurrence is possible but unlikely for those lesions able to be removed completely via excisional surgery.<sup id="cite_ref-OReilly_13-0" class="reference"><a href="#cite_note-OReilly-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Radiotherapy" class="mw-redirect" title="Radiotherapy">Radiotherapy</a> and chemical cauteries are not as effective with the lymphangioma than they are with the <a href="/wiki/Hemangioma" title="Hemangioma">hemangioma</a>.<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> Draining lymphangiomas of fluid provides only temporary relief, so they are removed surgically. Cystic Hygroma can be treated with OK432 (Picibanil). </p><p>Lymphangioma circumscription can be healed when treated with a flashlamp pulsed <a href="/wiki/Dye_laser" title="Dye laser">dye laser</a>, although this can cause port-wine stains and other vascular lesions.<sup id="cite_ref-pmid2357907_15-0" class="reference"><a href="#cite_note-pmid2357907-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </p><p>Orbital lymphangiomas, which carry significant risks from surgical removal, can also be treated with sclerosing agents, systemic medication, or through observation.<sup id="cite_ref-Patel_16-0" class="reference"><a href="#cite_note-Patel-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Sclerotherapy"><a href="/wiki/Sclerotherapy" title="Sclerotherapy">Sclerotherapy</a></h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=9" title="Edit section: Sclerotherapy"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The least invasive and most effective form of treatment is now performed by interventional radiologists. A sclerosing agent, such as 1% or 3% sodium tetradecyl sulfate, bleomycin, doxycycline, or ethanol, may be directly injected into a lymphocele. "All sclerosing agents are thought to work by ablating the endothelial cells of the disrupted lymphatics feeding into the lymphocele."<sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Sirolimus">Sirolimus</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=10" title="Edit section: Sirolimus"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1236090951">.mw-parser-output .hatnote{font-style:italic}.mw-parser-output div.hatnote{padding-left:1.6em;margin-bottom:0.5em}.mw-parser-output .hatnote i{font-style:normal}.mw-parser-output .hatnote+link+.hatnote{margin-top:-0.5em}@media print{body.ns-0 .mw-parser-output .hatnote{display:none!important}}</style><div role="note" class="hatnote navigation-not-searchable">Main article: <a href="/wiki/Sirolimus" title="Sirolimus">Sirolimus</a></div><p> Sirolimus is used to treat lymphangioma. Treatment with sirolimus can decrease pain and the fullness of venous malformations, improve coagulation levels, and slow the growth of abnormal lymphatic vessels.<sup id="cite_ref-18" class="reference"><a href="#cite_note-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> Sirolimus is a relatively new medical therapy for the treatment of vascular malformations,<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> in recent years, sirolimus has emerged as a new medical treatment option for both vascular tumors and vascular malformations, as a mammalian target of rapamycin (mTOR), capable of integrating signals from the PI3K/AKT pathway to coordinate proper cell growth and proliferation.<sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> Sirolimus (rapamycin, trade name Rapamune) is a macrolide compound. It has <a href="/wiki/Immunosuppressant" class="mw-redirect" title="Immunosuppressant">immunosuppressant</a> and antiproliferative functions in humans. It inhibits activation of <a href="/wiki/T_cells" class="mw-redirect" title="T cells">T cells</a> and <a href="/wiki/B_cells" class="mw-redirect" title="B cells">B cells</a> by reducing their sensitivity to <a href="/wiki/Interleukin-2" class="mw-redirect" title="Interleukin-2">interleukin-2</a> (IL-2) through <a href="/wiki/MTOR" title="MTOR">mTOR</a> inhibition.<sup id="cite_ref-Mukherjee_2009_21-0" class="reference"><a href="#cite_note-Mukherjee_2009-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> </p><div class="mw-heading mw-heading2"><h2 id="Prognosis">Prognosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=11" title="Edit section: Prognosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The prognosis for lymphangioma circumscriptum and cavernous lymphangioma is generally excellent. This condition is associated with minor bleeding, recurrent <a href="/wiki/Cellulitis" title="Cellulitis">cellulitis</a>, and lymph fluid leakage. Two cases of <a href="/wiki/Lymphangiosarcoma" title="Lymphangiosarcoma">lymphangiosarcoma</a> arising from <a href="/wiki/Superficial_lymphatic_malformation" class="mw-redirect" title="Superficial lymphatic malformation">lymphangioma circumscriptum</a> have been reported; however, in both of the patients, the preexisting lesion was exposed to extensive <a href="/wiki/Radiation_therapy" title="Radiation therapy">radiation therapy</a>.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p><p>In cystic hygroma, large cysts can cause <a href="/wiki/Dysphagia" title="Dysphagia">dysphagia</a>, respiratory problems, and serious infection if they involve the neck. Patients with cystic hygroma should receive <a href="/wiki/Cytogenetics" title="Cytogenetics">cytogenetic analysis</a> to determine if they have chromosomal abnormalities, and parents should receive <a href="/wiki/Genetic_counseling" title="Genetic counseling">genetic counseling</a> because this condition can recur in subsequent pregnancies.<sup id="cite_ref-Fernandez_7-5" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>Complications after surgical removal of cystic hygroma include damage to the structures in the neck, infection, and return of the cystic hygroma.<sup id="cite_ref-OReilly_13-1" class="reference"><a href="#cite_note-OReilly-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=12" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Lymphatic malformations are rare, accounting for 4% of all vascular tumors in children.<sup id="cite_ref-Fernandez_7-6" class="reference"><a href="#cite_note-Fernandez-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> Although lymphangioma can become evident at any age, 50% are seen at birth,<sup id="cite_ref-Giguère_12-3" class="reference"><a href="#cite_note-Giguère-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> and 90% of lymphangiomas are evident by 2 years of age.<sup id="cite_ref-Giguère_12-4" class="reference"><a href="#cite_note-Giguère-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=13" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In 1828, Redenbacher first described a lymphangioma lesion. In 1843, Wernher gave the first case report of a cystic hygroma, from the <a href="/wiki/Ancient_Greek" title="Ancient Greek">Greek</a> "hygro-" meaning fluid and "oma" meaning tumor. In 1965, Bill and Summer proposed that <a href="/wiki/Cystic_hygroma" title="Cystic hygroma">Cystic hygromas</a> and lymphangiomas are variations of a single entity and that its location determines its classification.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (February 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphatic_malformations&action=edit&section=14" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-Elluru-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-Elluru_1-0">^</a></b></span> <span class="error mw-ext-cite-error" lang="en" dir="ltr">Cite error: The named reference <code>Elluru</code> was invoked but never defined (see the <a href="/wiki/Help:Cite_errors/Cite_error_references_no_text" title="Help:Cite errors/Cite error references no text">help page</a>).</span></li> <li id="cite_note-NIH-2"><span class="mw-cite-backlink"><b><a href="#cite_ref-NIH_2-0">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://rarediseases.info.nih.gov/diseases/13020/microcystic-lymphatic-malformation">"Microcystic lymphatic malformation | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"</a>. <i>rarediseases.info.nih.gov</i>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=rarediseases.info.nih.gov&rft.atitle=Microcystic+lymphatic+malformation+%7C+Genetic+and+Rare+Diseases+Information+Center+%28GARD%29+%E2%80%93+an+NCATS+Program&rft_id=https%3A%2F%2Frarediseases.info.nih.gov%2Fdiseases%2F13020%2Fmicrocystic-lymphatic-malformation&rfr_id=info%3Asid%2Fen.wikipedia.org%3ALymphatic+malformations" 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style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q1545750" class="extiw" title="d:Q1545750">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/D18">D18</a> (<a href="/wiki/ILDS" class="mw-redirect" title="ILDS">ILDS</a> D18.100)</li><li><b><a href="/wiki/International_Classification_of_Diseases_for_Oncology" title="International Classification of Diseases for Oncology">ICD-O</a></b>: 9170/0</li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D008202">D008202</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb7665.htm">7665</a></li><li><b><a href="/wiki/SNOMED_CT" title="SNOMED CT">SNOMED CT</a></b>: <a rel="nofollow" class="external text" href="http://snomed.info/id/400178008">400178008</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/derm/866-overview">derm/866</a></li></ul></div></div></td></tr></tbody></table></div> <ul><li><i><a rel="nofollow" class="external text" href="http://www.emedicine.com/ped/topic536.htm#">Cystic hygroma</a></i> at <a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></li></ul> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Tumours_of_blood_vessels101" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Vascular_tumors" title="Template:Vascular tumors"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Vascular_tumors" title="Template talk:Vascular tumors"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Vascular_tumors" title="Special:EditPage/Template:Vascular tumors"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Tumours_of_blood_vessels101" style="font-size:114%;margin:0 4em"><a href="/wiki/Vascular_tumor" title="Vascular tumor">Tumours of blood vessels</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Blood_vessel" title="Blood vessel">Blood vessel</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Hemangiosarcoma" title="Hemangiosarcoma">Hemangiosarcoma</a></li> <li><a href="/wiki/Blue_rubber_bleb_nevus_syndrome" title="Blue rubber bleb nevus syndrome">Blue rubber bleb nevus syndrome</a></li> <li><a href="/wiki/Hemangioendothelioma" title="Hemangioendothelioma">Hemangioendothelioma</a> <ul><li><a href="/wiki/Composite_hemangioendothelioma" class="mw-redirect" title="Composite hemangioendothelioma">Composite</a></li> <li><a href="/wiki/Endovascular_papillary_hemangioendothelioma" class="mw-redirect" title="Endovascular papillary hemangioendothelioma">Endovascular papillary</a></li> <li><a href="/wiki/Epithelioid_hemangioendothelioma" title="Epithelioid hemangioendothelioma">Epithelioid</a></li> <li><a href="/wiki/Kaposiform_hemangioendothelioma" class="mw-redirect" title="Kaposiform hemangioendothelioma">Kaposiform</a></li> <li><a href="/wiki/Infantile_hemangioendothelioma" class="mw-redirect" title="Infantile hemangioendothelioma">Infantile</a></li> <li><a href="/wiki/Retiform_hemangioendothelioma" class="mw-redirect" title="Retiform hemangioendothelioma">Retiform</a>)</li> <li><a href="/wiki/Spindle_cell_hemangioendothelioma" class="mw-redirect" title="Spindle cell hemangioendothelioma">Spindle cell</a></li></ul></li> <li><a href="/wiki/Proliferating_angioendotheliomatosis" title="Proliferating angioendotheliomatosis">Proliferating angioendotheliomatosis</a></li> <li><a href="/wiki/Hemangiopericytoma" title="Hemangiopericytoma">Hemangiopericytoma</a> <ul><li><a href="/wiki/Infantile_hemangiopericytoma" title="Infantile hemangiopericytoma">Infantile hemangiopericytoma</a></li></ul></li> <li><a href="/wiki/Venous_lake" title="Venous lake">Venous lake</a></li> <li><a href="/wiki/Kaposi%27s_sarcoma" title="Kaposi's sarcoma">Kaposi's sarcoma</a> <ul><li><a href="/wiki/African_cutaneous_Kaposi_sarcoma" class="mw-redirect" title="African cutaneous Kaposi sarcoma">African cutaneous</a></li> <li><a href="/wiki/African_lymphadenopathic_Kaposi_sarcoma" class="mw-redirect" title="African lymphadenopathic Kaposi sarcoma">African lymphadenopathic</a></li> <li><a href="/wiki/AIDS-associated_Kaposi_sarcoma" class="mw-redirect" title="AIDS-associated Kaposi sarcoma">AIDS-associated</a></li> <li><a href="/wiki/Classic_Kaposi_sarcoma" class="mw-redirect" title="Classic Kaposi sarcoma">Classic</a></li> <li><a href="/wiki/Immunosuppression-associated_Kaposi_sarcoma" class="mw-redirect" title="Immunosuppression-associated Kaposi sarcoma">Immunosuppression-associated</a></li></ul></li> <li><a href="/wiki/Hemangioblastoma" title="Hemangioblastoma">Hemangioblastoma</a></li> <li><a href="/wiki/Hemangioma" title="Hemangioma">Hemangioma</a> <ul><li><a href="/wiki/Capillary_hemangioma" class="mw-redirect" title="Capillary hemangioma">Capillary</a></li> <li><a href="/wiki/Cavernous_hemangioma" title="Cavernous hemangioma">Cavernous</a></li> <li><a href="/wiki/Glomeruloid_hemangioma" title="Glomeruloid hemangioma">Glomeruloid</a></li> <li><a href="/wiki/Microvenular_hemangioma" title="Microvenular hemangioma">Microvenular</a></li> <li><a href="/wiki/Targetoid_hemosiderotic_hemangioma" title="Targetoid hemosiderotic hemangioma">Targeted hemosiderotic</a></li></ul></li> <li><a href="/wiki/Angioma" title="Angioma">Angioma</a> <ul><li><a href="/wiki/Cherry_angioma" title="Cherry angioma">Cherry</a></li> <li><a href="/wiki/Angioma_serpiginosum" title="Angioma serpiginosum">Seriginosum</a></li> <li><a href="/wiki/Spider_angioma" title="Spider angioma">Spider</a></li> <li><a href="/wiki/Tufted_angioma" title="Tufted angioma">Tufted</a></li></ul></li> <li>Universal angiomatosis</li> <li><a href="/wiki/Angiokeratoma" title="Angiokeratoma">Angiokeratoma</a> <ul><li><a href="/wiki/Angiokeratoma_of_Mibelli" class="mw-redirect" title="Angiokeratoma of Mibelli">of Mibelli</a></li> <li><a href="/wiki/Angiolipoma" title="Angiolipoma">Angiolipoma</a></li></ul></li> <li><a href="/wiki/Pyogenic_granuloma" title="Pyogenic granuloma">Pyogenic granuloma</a></li> <li><a href="/wiki/Intravascular_papillary_endothelial_hyperplasia" title="Intravascular papillary endothelial hyperplasia">Intravascular papillary endothelial hyperplasia</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Lymphatic_vessel_tumor" class="mw-redirect" title="Lymphatic vessel tumor">Lymphatic</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Lymphangioma" class="mw-redirect" title="Lymphangioma">Lymphangioma</a>/<a href="/wiki/Lymphangiosarcoma" title="Lymphangiosarcoma">lymphangiosarcoma</a> <ul><li><a href="/wiki/Lymphangioma_circumscriptum" title="Lymphangioma circumscriptum">Lymphangioma circumscriptum</a></li> <li><a href="/wiki/Acquired_progressive_lymphangioma" title="Acquired progressive lymphangioma">Acquired progressive lymphangioma</a></li></ul></li> <li><i><a href="/wiki/PEComa" class="mw-redirect" title="PEComa">PEComa</a></i> <ul><li><a href="/wiki/Lymphangioleiomyomatosis" title="Lymphangioleiomyomatosis">Lymphangioleiomyomatosis</a></li></ul></li> <li><a href="/wiki/Cystic_hygroma" title="Cystic hygroma">Cystic hygroma</a></li> <li><a href="/wiki/Multifocal_lymphangioendotheliomatosis" title="Multifocal lymphangioendotheliomatosis">Multifocal lymphangioendotheliomatosis</a></li> <li><a href="/wiki/Lymphangiomatosis" title="Lymphangiomatosis">Lymphangiomatosis</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Either</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Angioma" title="Angioma">Angioma</a>/<a href="/wiki/Angiosarcoma" title="Angiosarcoma">angiosarcoma</a></li> <li><a href="/wiki/Angiofibroma" title="Angiofibroma">Angiofibroma</a></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"><style data-mw-deduplicate="TemplateStyles:r1038841319">.mw-parser-output .tooltip-dotted{border-bottom:1px dotted;cursor:help}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1038841319"></div><div role="navigation" class="navbox authority-control" aria-label="Navbox791" style="padding:3px"><table class="nowraplinks hlist navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Help:Authority_control" title="Help:Authority control">Authority control databases</a>: National <span class="mw-valign-text-top noprint" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q1545750#identifiers" title="Edit this at Wikidata"><img alt="Edit this at Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><ul><li><span class="uid"><span class="rt-commentedText tooltip tooltip-dotted" title="Lymphangiomes"><a rel="nofollow" class="external text" href="https://catalogue.bnf.fr/ark:/12148/cb12153031n">France</a></span></span></li><li><span class="uid"><span class="rt-commentedText tooltip tooltip-dotted" title="Lymphangiomes"><a rel="nofollow" class="external text" href="https://data.bnf.fr/ark:/12148/cb12153031n">BnF data</a></span></span></li></ul></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Vascular_anomalies127" style="padding:3px"><table class="nowraplinks mw-collapsible mw-collapsed navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1239400231"><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Vascular_anomalies" title="Template:Vascular anomalies"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/w/index.php?title=Template_talk:Vascular_anomalies&action=edit&redlink=1" class="new" title="Template talk:Vascular anomalies (page does not exist)"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Vascular_anomalies" title="Special:EditPage/Template:Vascular anomalies"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Vascular_anomalies127" style="font-size:114%;margin:0 4em"><a href="/wiki/Vascular_anomaly" title="Vascular anomaly"> Vascular anomalies</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Vascular_tumor" title="Vascular tumor"> Vascular tumors</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">Benign</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Infantile_hemangioma" title="Infantile hemangioma"> Infantile hemangioma / Hemangioma of infancy</a></li> <li>Congenital hemangioma</li> <li><a href="/wiki/Tufted_angioma" title="Tufted angioma">Tufted angioma</a></li> <li>Spindle-cell hemangioma</li> <li><a href="/wiki/Angiolymphoid_hyperplasia_with_eosinophilia" title="Angiolymphoid hyperplasia with eosinophilia"> Epithelioid hemangioma</a></li> <li><a href="/wiki/Pyogenic_granuloma" title="Pyogenic granuloma">Pyogenic granuloma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Locally aggressive or borderline</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Vascular_tumor" title="Vascular tumor"> Kaposiform hemangioendothelioma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Malignant</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Angiosarcoma" title="Angiosarcoma">Angiosarcoma</a></li> <li><a href="/wiki/Epithelioid_hemangioendothelioma" title="Epithelioid hemangioendothelioma">Epithelioid hemangioendothelioma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Vascular_malformation" title="Vascular malformation"> Vascular malformations</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">Simple</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">Capillary malformations</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Nevus simplex</li> <li><a href="/wiki/Port-wine_stain" title="Port-wine stain"> Cutaneous and/or mucosal CM ("port-wine" stain)</a></li> <li>Reticulate CM</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Lymphatic_malformation" class="mw-redirect" title="Lymphatic malformation"> Lymphatic malformations (LM)</a> <hr /> <span style="font-size:85%;"> Incorrect name: Lymphangioma</span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Common (cystic) LM <ul><li>Macrocystic LM</li> <li>Microcystic LM</li> <li>Mixed cystic LM</li></ul></li> <li>Generalized lymphatic anomaly <ul><li>Kaposiform lymphangiomatosis</li></ul></li> <li>LM in <a href="/wiki/Gorham-Stout_disease" class="mw-redirect" title="Gorham-Stout disease">Gorham-Stout disease</a></li> <li>Channel type LM</li> <li>“Acquired” progressive lymphatic anomaly</li> <li><a href="/wiki/Primary_lymphedema" title="Primary lymphedema">Primary lymphedema</a> <ul><li><a href="/wiki/Milroy%27s_disease" title="Milroy's disease"> Nonne-Milroy syndrome</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Venous malformations</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Common VM</li> <li>Familial VM cutaneo-mucosal</li> <li><a href="/wiki/Blue_rubber_bleb_nevus_syndrome" title="Blue rubber bleb nevus syndrome">Blue rubber bleb nevus syndrome</a></li> <li>Glomuvenous malformation</li> <li>Cerebral cavernous malformation</li> <li>Familial intraosseous vascular malformation</li> <li>Verrucous venous malformation</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Arteriovenous_malformations" class="mw-redirect" title="Arteriovenous malformations">Arteriovenous malformations</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Sporadic</li> <li>In HHT</li> <li>In CM-AVM</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Arteriovenous_fistula" title="Arteriovenous fistula">Arteriovenous fistula</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Sporadic</li> <li>In HHT</li> <li>In CM-AVM</li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Combined</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Lymphatic-venous malformation</li> <li>Capillary-arteriovenous malformation</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Associated with other anomalies</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Klippel-Trenaunay_syndrome" class="mw-redirect" title="Klippel-Trenaunay syndrome">Klippel-Trenaunay syndrome</a></li> <li>Parkes Weber Syndrome</li> <li>Servelle-Martorell syndrome</li> <li><a href="/wiki/Sturge-Weber_syndrome" class="mw-redirect" title="Sturge-Weber syndrome">Sturge-Weber syndrome</a></li> <li>Limb CM + congenital non-progressive limb overgrowth</li> <li><a href="/wiki/Maffucci_syndrome" title="Maffucci syndrome">Maffucci syndrome</a></li> <li>Macrocephaly - CM (M-CM / MCAP)</li> <li>Microcephaly - CM (MICCAP)</li> <li><a href="/wiki/CLOVES_syndrome" title="CLOVES syndrome">CLOVES syndrome</a></li> <li>Proteus syndrome</li> <li><a href="/wiki/Bannayan-Riley-Ruvalcaba_syndrome" class="mw-redirect" title="Bannayan-Riley-Ruvalcaba syndrome">Bannayan-Riley-Ruvalcaba syndrome</a></li> <li>CLAPO syndrome</li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table></div>    </div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?useformat=desktop&type=1x1&usesul3=0" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" 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