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class="vector-body" aria-labelledby="firstHeading" data-mw-ve-target-container> <div class="vector-body-before-content"> <div class="mw-indicators"> </div> <div id="siteSub" class="noprint">From Wikipedia, the free encyclopedia</div> </div> <div id="contentSub"><div id="mw-content-subtitle"></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><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">Lymphangiomatosis</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data">LYMF</td></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:Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/0/0d/Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png/220px-Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png" decoding="async" width="220" height="165" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/0/0d/Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png/330px-Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/0/0d/Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png/440px-Lung_biopsy_showing_infiltration_of_lymphatic_tissue.png 2x" data-file-width="512" data-file-height="383" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">Lung biopsy showing infiltration of lymphatic tissue.</td></tr></tbody></table> <p><b>Lymphangiomatosis</b> is a condition where a <a href="/wiki/Lymphangioma" class="mw-redirect" title="Lymphangioma">lymphangioma</a> is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare type of <a href="/wiki/Tumor" class="mw-redirect" title="Tumor">tumor</a> which results from an abnormal development of the <a href="/wiki/Lymphatic_system" title="Lymphatic system">lymphatic system</a>.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p><p>It is thought to be the result of congenital errors of lymphatic development occurring prior to the 20th week of gestation.<sup id="cite_ref-faul_2-0" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Lymphangiomatosis is a condition marked by the presence of cysts that result from an increase both in the size and number of thin-walled lymphatic channels that are abnormally interconnected and dilated.<sup id="cite_ref-faul_2-1" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pernick_3-0" class="reference"><a href="#cite_note-pernick-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-tazelaar_4-0" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> 75% of cases involve multiple organs.<sup id="cite_ref-faul_2-2" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> It typically presents by age 20 and, although it is technically benign, these deranged lymphatics tend to invade surrounding tissues and cause problems due to invasion and/or compression of adjacent structures.<sup id="cite_ref-faul_2-3" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> The condition is most common in the bones and lungs<sup id="cite_ref-faul_2-4" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> and shares some characteristics with <a href="/wiki/Gorham%27s_disease" title="Gorham's disease">Gorham’s disease</a>. Up to 75% of patients with lymphangiomatosis have bone involvement, leading some to conclude that lymphangiomatosis and Gorham’s disease should be considered as a spectrum of disease rather than separate diseases.<sup id="cite_ref-faul_2-5" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-aviv_5-0" class="reference"><a href="#cite_note-aviv-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> When it occurs in the lungs, lymphangiomatosis has serious consequences and is most aggressive in the youngest children.<sup id="cite_ref-faul_2-6" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-tazelaar_4-1" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> When the condition extends into the chest it commonly results in the accumulation of chyle in the linings of the heart and/or lungs.<sup id="cite_ref-faul_2-7" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-tazelaar_4-2" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>Chyle is composed of lymph fluid and fats that are absorbed from the small intestine by specialized lymphatic vessels called lacteals during digestion. The accumulations are described based on location: <a href="/wiki/Chylothorax" title="Chylothorax">chylothorax</a> is chyle in the chest; chylopericardium is chyle trapped inside the sack surrounding the heart; chyloascites is chyle trapped in the linings of the abdomen and abdominal organs. The presence of chyle in these places accounts for many of the symptoms and complications associated with both lymphangiomatosis and Gorham’s disease.<sup id="cite_ref-faul_2-8" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-duffy_6-0" class="reference"><a href="#cite_note-duffy-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> The incidence of lymphangiomatosis is unknown and it is often misdiagnosed. It is separate and distinct from <a href="/wiki/Lymphangiectasis" class="mw-redirect" title="Lymphangiectasis">lymphangiectasis</a>, <a href="/wiki/Lymphangioleiomyomatosis" title="Lymphangioleiomyomatosis">lymphangioleiomyomatosis</a> (LAM), <a href="/wiki/Pulmonary_capillary_hemangiomatosis" title="Pulmonary capillary hemangiomatosis">pulmonary capillary hemangiomatosis</a>, <a href="/wiki/Kaposi%E2%80%99s_sarcoma" class="mw-redirect" title="Kaposi’s sarcoma">Kaposi’s sarcoma</a>, and kaposiform hemangioendothelioma.<sup id="cite_ref-tazelaar_4-3" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Its unusual nature makes lymphangiomatosis (and Gorham’s disease) a diagnostic and therapeutic challenge.<sup id="cite_ref-tazelaar_4-4" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-yeager_7-0" class="reference"><a href="#cite_note-yeager-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> A multidisciplinary approach is generally necessary for optimal diagnosis and symptom management. The term literally means lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis). </p> <meta property="mw:PageProp/toc" /> <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=Lymphangiomatosis&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Lymphangiomatosis is a multi-system disorder. Symptoms depend on the organ system involved and, to varying degrees, the extent of the disease. Early in the course of the disease patients are usually asymptomatic, but over time the abnormally proliferating lymphatic channels that constitute lymphangiomatosis are capable of massive expansion and infiltration into surrounding tissues, bone, and organs.<sup id="cite_ref-faul_2-9" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Because of its slow course and often vague symptoms, the condition is frequently under-recognized or misdiagnosed.<sup id="cite_ref-venkatramani_8-0" class="reference"><a href="#cite_note-venkatramani-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p><p>Early signs of disease in the chest include wheezing, cough, and feeling short of breath, which is often misdiagnosed as asthma.<sup id="cite_ref-faul_2-10" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> The pain that accompanies bone involvement may be attributed to "growing pains" in younger children. With bone involvement the first indication for disease may be a pathological fracture. Symptoms may not raise concern, or even be noted, until the disease process has advanced to a point where it causes restrictive compression of vital structures. Further, the occurrence of chylous effusions seems to be unrelated to the pathologic "burden" of the disease, the extent of involvement in any particular tissue or organ, or the age of the patient.<sup id="cite_ref-zisis_9-0" class="reference"><a href="#cite_note-zisis-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> This offers one explanation as to why, unfortunately, the appearance of chylous effusions in the chest or abdomen may be the first evidence of the disease.<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. (March 2021)">citation needed</span></a></i>]</sup> </p><p>Following are some of the commonly reported symptoms of lymphangiomatosis, divided into the regions/systems in which the disease occurs: </p> <div class="mw-heading mw-heading3"><h3 id="Heart_and_chest">Heart and chest</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=2" title="Edit section: Heart and chest"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Symptoms that arise from disease of the cardiothoracic region include a chronic cough, wheezing, dyspnea (shortness of breath)—especially serious when occurring at rest or when lying down—fever, chest pain, rapid heartbeat, dizziness, anxiety, and coughing up blood or chyle.<sup id="cite_ref-faul_2-11" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> As the deranged lymphatic vessels invade the organs and tissues in the chest they put stress on the heart and lungs, interfering with their ability to function normally. Additionally, these lymphatic vessels may leak, allowing fluid to accumulate in the chest, which puts further pressure on the vital organs, thus increasing their inability to function properly.<sup id="cite_ref-faul_2-12" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Accumulations of fluid and chyle are named based on their contents and location: pulmonary edema (the presence of fluid and/or chyle in the lung), pleural effusions (fluid in the lung lining), pericardial effusions (fluid in the heart sack), chylothorax (chyle in the pleural cavity); and chylopericardium (chyle in the heart sack).<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. (March 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading3"><h3 id="Abdominal">Abdominal</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=3" title="Edit section: Abdominal"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Lymphangiomatosis has been reported in every region of the abdomen, though the most reported sites involve the intestines and peritoneum; spleen, kidneys, and liver. Often there are no symptoms until late in the progression of the disease. When they do occur, symptoms include abdominal pain and/or distension; nausea, vomiting, diarrhea; decreased appetite and malnourishment. When the disease affects the kidneys the symptoms include flank pain, abdominal distension, blood in the urine, and, possibly, elevated blood pressure, which may result in it being confused with other cystic renal disease.<sup id="cite_ref-hakeem_10-0" class="reference"><a href="#cite_note-hakeem-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> When lymphangiomatosis occurs in the liver and/or spleen it may be confused with polycystic liver disease.<sup id="cite_ref-o'sullivan_11-0" class="reference"><a href="#cite_note-o'sullivan-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> Symptoms may include abdominal fullness and distension; anemia, disseminated intravascular coagulopathy (DIC), fluid accumulation in the abdomen(ascites), decreased appetite, weight loss, fatigue; late findings include liver failure.<sup id="cite_ref-faul_2-13" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-o'sullivan_11-1" class="reference"><a href="#cite_note-o'sullivan-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-miller_12-0" class="reference"><a href="#cite_note-miller-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Bones">Bones</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=4" title="Edit section: Bones"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Symptoms of lymphangiomatosis in the skeletal system are the same as those of <a href="/wiki/Gorham%27s_disease" title="Gorham's disease">Gorham’s disease</a>. Frequently asymptomatic, skeletal lymphangiomatosis may be discovered incidentally or when a pathological fracture occurs. Patients may experience pain of varying severity in areas around the effected bone. When the disease occurs in the bones of the spine, neurological symptoms such as numbness and tingling may occur due to spinal nerve compression.<sup id="cite_ref-watkins_13-0" class="reference"><a href="#cite_note-watkins-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> Progression of disease in the spine may lead to paralysis. Lymphangiomatosis in conjunction with Chiari I malformation also has been reported.<sup id="cite_ref-jea_14-0" class="reference"><a href="#cite_note-jea-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> </p> <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=Lymphangiomatosis&action=edit&section=5" title="Edit section: Causes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The cause of lymphangiomatosis is not yet known. As stated earlier, it is generally considered to be the result of congenital errors of lymphatic development occurring prior to the 20th week of gestation.<sup id="cite_ref-faul_2-14" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> However, the root causes of these conditions remains unknown and further research is necessary.<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. (September 2020)">citation needed</span></a></i>]</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=Lymphangiomatosis&action=edit&section=6" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Because it is rare and has a wide spectrum of clinical, histological, and imaging features, diagnosing lymphangiomatosis can be challenging.<sup id="cite_ref-shah_15-0" class="reference"><a href="#cite_note-shah-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> Plain x-rays reveal the presence of lytic lesions in bones, pathological fractures, interstitial infiltrates in the lungs, and chylous effusions that may be present even when there are no outward symptoms.<sup id="cite_ref-faul_2-15" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-aviv_5-1" class="reference"><a href="#cite_note-aviv-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-yeager_7-1" class="reference"><a href="#cite_note-yeager-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>The most common locations of lymphangiomatosis are the lungs and bones and one important diagnostic clue is the coexistence of lytic bone lesions and chylous effusion.<sup id="cite_ref-faul_2-16" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> An isolated presentation usually carries a better prognosis than does multi-organ involvement; the combination of pleural and peritoneal involvement with chylous effusions and lytic bone lesions carries the least favorable prognosis.<sup id="cite_ref-cs_16-0" class="reference"><a href="#cite_note-cs-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p><p>When lung involvement is suspected, high resolution computed tomography (HRCT) scans may reveal a diffuse liquid-like infiltration in the mediastinal and hilar soft tissue, resulting from diffuse proliferation of lymphatic channels and accumulation of lymphatic fluid; diffuse peribronchovascular and interlobular septal thickening; <a href="/wiki/Ground-glass_opacities" class="mw-redirect" title="Ground-glass opacities">ground-glass opacities</a>; and pleural effusion.<sup id="cite_ref-faul_2-17" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-du_17-0" class="reference"><a href="#cite_note-du-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Pulmonary function testing reveals either restrictive pattern or a mixed obstructive/restrictive pattern.<sup id="cite_ref-faul_2-18" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-tazelaar_4-5" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> While x-rays, HRCT scan, MRI, ultrasound, lymphangiography, bone scan, and bronchoscopy all can have a role in identifying lymphangiomatosis, biopsy remains the definitive diagnostic tool.<sup id="cite_ref-faul_2-19" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-aviv_5-2" class="reference"><a href="#cite_note-aviv-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-yeager_7-2" class="reference"><a href="#cite_note-yeager-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-du_17-1" class="reference"><a href="#cite_note-du-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-swensen_18-0" class="reference"><a href="#cite_note-swensen-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-wunderbaldinger_19-0" class="reference"><a href="#cite_note-wunderbaldinger-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> </p><p>Microscopic examination of biopsy specimens reveals an increase in both the size and number of thin walled lymphatic channels along with lymphatic spaces that are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis, and possibly underlying fascia and skeletal muscle.<sup id="cite_ref-pernick_3-1" class="reference"><a href="#cite_note-pernick-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> Additionally, Tazelaar, et al., described a pattern of histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL).<sup id="cite_ref-tazelaar_4-6" class="reference"><a href="#cite_note-tazelaar-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>Recognition of the disease requires a high index of suspicion and an extensive workup. Because of its serious morbidity, lymphangiomatosis must always be considered in the differential diagnosis of lytic bone lesions accompanied by chylous effusions, in cases of primary chylopericardium, and as part of the differential diagnosis in pediatric patients presenting with signs of interstitial lung disease.<sup id="cite_ref-faul_2-20" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-swensen_18-1" class="reference"><a href="#cite_note-swensen-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-martinez_20-0" class="reference"><a href="#cite_note-martinez-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-lynch_21-0" class="reference"><a href="#cite_note-lynch-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> </p> <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=Lymphangiomatosis&action=edit&section=7" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There is no standard approach to the treatment of lymphangiomatosis and treatment often is aimed at reducing symptoms.<sup id="cite_ref-faul_2-21" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-du_17-2" class="reference"><a href="#cite_note-du-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Surgical intervention may be indicated when complications arise and a number of reports of response to surgical interventions, medications, and dietary approaches can be found in the medical literature.<sup id="cite_ref-faul_2-22" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-cs_16-1" class="reference"><a href="#cite_note-cs-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-du_17-3" class="reference"><a href="#cite_note-du-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p><p>Unfortunately, there is no standardized treatment for lymphangiomatosis and no cure. Treatment modalities that have been reported in the medical literature, by system, include: </p> <div class="mw-heading mw-heading3"><h3 id="Heart_and_chest_2">Heart and chest</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=8" title="Edit section: Heart and chest"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Thoracocentesis, pericardiocentesis, pleurodesis, ligation of thoracic duct, pleuroperitoneal shunt, radiation therapy, pleurectomy, pericardial window, pericardiectomy, thalidomide, interferon alpha 2b, Total Parenteral Nutrition (TPN), medium chain triglyceride (MCT) and high protein diet, chemotherapy, sclerotherapy, transplant;<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. (September 2020)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading3"><h3 id="Abdominal_2">Abdominal</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=9" title="Edit section: Abdominal"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>interferon alpha 2b, sclerotherapy, resection, percutaneous drainage, Denver shunt, Total Parenteral Nutrition (TPN), medium chain triglyceride (MCT) and high protein diet, transplant, splenectomy;<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. (September 2020)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading3"><h3 id="Bone">Bone</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Lymphangiomatosis&action=edit&section=10" title="Edit section: Bone"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Interferon alpha 2b, bisphosphonates (i.e. pamidronate), surgical resection, radiation therapy, sclerotherapy, percutaneous bone cement, bone grafts, prosthesis, surgical stabilization.<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. (March 2021)">citation needed</span></a></i>]</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=Lymphangiomatosis&action=edit&section=11" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Lymphangiomatosis can occur at any age, but the incidence is highest in children and teenagers. Signs and symptoms are typically present before the age of 20 and the condition is often under-recognized in adults.<sup id="cite_ref-faul_2-23" class="reference"><a href="#cite_note-faul-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>It affects males and females of all races and exhibits no inheritance pattern. The medical literature contains case reports from every continent. Because it is so rare, and commonly misdiagnosed, it is not known exactly how many people are affected by this disease.<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. (March 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=Lymphangiomatosis&action=edit&section=12" 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-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-1">^</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 id="CITEREFMaromMoranMunden2004" class="citation journal cs1">Marom, EM; Moran, CA; Munden, RF (April 2004). 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T., Garg N, Olsen B.R. (2014). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.bone.2014.02.011">"Viewpoints on vessels and vanishing bones in Gorham-Stout disease"</a>. <i>Bone</i>. <b>63C</b>: 47–52. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.bone.2014.02.011">10.1016/j.bone.2014.02.011</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/24583233">24583233</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Bone&rft.atitle=Viewpoints+on+vessels+and+vanishing+bones+in+Gorham-Stout+disease&rft.volume=63C&rft.pages=47-52&rft.date=2014&rft_id=info%3Adoi%2F10.1016%2Fj.bone.2014.02.011&rft_id=info%3Apmid%2F24583233&rft.au=Dellinger+M.+T.%2C+Garg+N%2C+Olsen+B.R.&rft_id=https%3A%2F%2Fdoi.org%2F10.1016%252Fj.bone.2014.02.011&rfr_id=info%3Asid%2Fen.wikipedia.org%3ALymphangiomatosis" class="Z3988"></span><span class="cs1-maint citation-comment"><code class="cs1-code">{{<a href="/wiki/Template:Cite_journal" title="Template:Cite journal">cite journal</a>}}</code>: CS1 maint: multiple names: authors list (<a href="/wiki/Category:CS1_maint:_multiple_names:_authors_list" title="Category:CS1 maint: multiple names: authors list">link</a>)</span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFTrenor_C,_Chaudry_G2014" class="citation journal cs1">Trenor C, Chaudry G (Aug 2014). 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title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Skeletal+Radiol&rft.atitle=Gorham-Stout+disease+and+generalized+lymphatic+anomaly%E2%80%94clinical%2C+radiologic%2C+and+histologic+differentiation&rft.volume=42&rft.issue=7&rft.pages=917-24&rft.date=2013-07&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A22640055%23id-name%3DS2CID&rft_id=info%3Apmid%2F23371338&rft_id=info%3Adoi%2F10.1007%2Fs00256-012-1565-4&rft.au=Lala+S%2C+Mulliken+JB%2C+Alomari+AI%2C+Fishman+SJ%2C+Kozakewich+HP%2C+Chaudry+G&rfr_id=info%3Asid%2Fen.wikipedia.org%3ALymphangiomatosis" class="Z3988"></span><span class="cs1-maint citation-comment"><code class="cs1-code">{{<a href="/wiki/Template:Cite_journal" title="Template:Cite journal">cite journal</a>}}</code>: CS1 maint: multiple names: authors list (<a href="/wiki/Category:CS1_maint:_multiple_names:_authors_list" title="Category:CS1 maint: multiple names: authors list">link</a>)</span></li></ul> <div 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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_vessels" 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 class="mw-selflink selflink">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><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" href="https://en.wikipedia.org/w/index.php?title=Lymphangiomatosis&oldid=1170991675">https://en.wikipedia.org/w/index.php?title=Lymphangiomatosis&oldid=1170991675</a>"</div></div> <div id="catlinks" class="catlinks" data-mw="interface"><div id="mw-normal-catlinks" class="mw-normal-catlinks"><a href="/wiki/Help:Category" title="Help:Category">Categories</a>: <ul><li><a href="/wiki/Category:Dermal_and_subcutaneous_growths" title="Category:Dermal and subcutaneous growths">Dermal and subcutaneous 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