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We sought to characterize the incidence, clinical features, risk factors and management of chronic lymphedema in patients with KHE.<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We conducted a multicenter retrospective analysis of patients who had a minimum of 3\u00a0years of follow-up after the onset of KHE and\/or Kasabach\u2013Merritt phenomenon (KMP). Clinical features were reviewed to determine the possible cause of chronic lymphedema. The degree of lymphedema, risk factors and management strategies were analyzed.<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Among the 118 patients, chronic lymphedema was confirmed by lymphoscintigraphy 1\u00a0year after the onset of KHE and\/or KMP in 13 patients. In 8 patients with lymphedema, extremity swelling was evident in the presence of KHE and\/or KMP. In all patients with lymphedema, a unilateral extremity was affected, along with ipsilateral KHE. Most (84.6%) patients reported moderate lymphedema. Lymphedema was more common in patients with larger (\u2265\u200910\u00a0cm) and mixed lesions involving the extremities (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01). A history of KMP and sirolimus treatment were not predictors of lymphedema (<jats:italic>P<\/jats:italic>\u2009&gt;\u20090.05). Overall, 76.9% of patients received sirolimus treatment after referral, including 53.8% who presented extremity swelling before referral. Seven (53.8%) patients received compression therapy. Five (38.5%) patients reported lymphedema-associated decreased range of motion at the last follow-up.<\/jats:p>\n              <\/jats:sec>\n              <jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Chronic lymphedema is a common sequela of KHE and can occur independently of KMP and sirolimus treatment. Patients with large and mixed KHE involving extremities should be closely monitored for this disabling complication.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-020-01595-2","type":"journal-article","created":{"date-parts":[[2020,11,7]],"date-time":"2020-11-07T12:02:44Z","timestamp":1604750564000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management"],"prefix":"10.1186","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9289-9660","authenticated-orcid":false,"given":"Yi","family":"Ji","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siyuan","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chuncao","family":"Xia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiangyuan","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xian","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuewen","family":"Xu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaiying","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xuepeng","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feiteng","family":"Kong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guoyan","family":"Lu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yongbo","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,11,7]]},"reference":[{"key":"1595_CR1","doi-asserted-by":"crossref","unstructured":"Mulliken JB, Burrows PE, Fishman SJ. 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Written informed consent was obtained from the patients\u2019 parents, according to the provisions of the Declaration of Helsinki.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent for publication this study was obtained from the patients\u2019 parents. Copies of the signed informed consent forms are available for review by the Series Editor.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to publish"}},{"value":"The authors declare that they have no competing interests, either financial or non-financial, that could be perceived as prejudicing the impartiality of the research reported.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"313"}}