ArticleOrphanet journal of rare diseases2020
Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 19 citations in OpenAlex.
- Sirolimus for vascular anomalies in the first year of life: a systematic review.Journal of perinatology : official journal of the California Perinatal Association · 2024Pooled it
- Potential utilization of lymphoscintigraphy in patients with kaposiform hemangioendothelioma.Quantitative imaging in medicine and surgery · 2026Article
- Prenatal Diagnosis and Management of Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon: Imaging Features and First Experience With Maternal Sirolimus Therapy.Prenatal diagnosis · 2026Article
- Kaposiform hemangioendothelioma: Diagnosis and treatment.Pediatric investigation · 2026Review
- Case Report: Life-threatening Kasabach-Merritt phenomenon in a 2-month-old child.Frontiers in pediatrics · 2025Article
- Similarities and differences in the clinical features and management of primary lymphedema and kaposiform hemangioendothelioma associated with lymphedema in children.Frontiers in pediatrics · 2025Article
- Editorial: Vascular malformations: advancements, debates, and consensus.Frontiers in medicine · 2025Article
- Case Report: 3 cases of abdominal lesions with lower limb lymphedema-rare mutations in complex lymphatic anomalies.Frontiers in oncology · 2025Article
- Impact of age and tumor size on the development of the Kasabach-Merritt phenomenon in patients with kaposiform hemangioendothelioma: a retrospective cohort study.Precision clinical medicine · 2023Article
- Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.British journal of clinical pharmacology · 2022Article
- Antiproliferative therapy with sirolimus and propranolol for congenital vascular anomalies in newborns (Case reports).Experimental and therapeutic medicine · 2021Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesThere are no cohort studies of chronic lymphedema in patients with kaposiform hemangioendothelioma (KHE). We sought to characterize the incidence, clinical features, risk factors and management of chronic lymphedema in patients with KHE.
methodsWe conducted a multicenter retrospective analysis of patients who had a minimum of 3 years of follow-up after the onset of KHE and/or Kasabach-Merritt 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.
resultsAmong the 118 patients, chronic lymphedema was confirmed by lymphoscintigraphy 1 year 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 (≥ 10 cm) and mixed lesions involving the extremities (P < 0.01). A history of KMP and sirolimus treatment were not predictors of lymphedema (P > 0.05). 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.
conclusionsChronic 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.
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