Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and mana
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RESEARCH
Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management Yi Ji1*† , Siyuan Chen2*†, Chuncao Xia3†, Jiangyuan Zhou1, Xian Jiang4, Xuewen Xu5, Kaiying Yang1, Xuepeng Zhang2, Feiteng Kong6, Guoyan Lu7 and Yongbo Zhang8
Abstract Objectives: There 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. Methods: We 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. Results: Among 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 0.05). 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. Conclusions: 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. Keywords: Kaposiform hemangioendothelioma, Kasabach–Merritt phenomenon, Clinical characteristics, Complication, Risk factor
*Correspondence: [email protected]; [email protected] † Yi Ji, Siyuan Chen and Chuncao Xia have contributed equally to this work 1 Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China 2 Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, #37# Guo‑Xue‑Xiang, Chengdu 610041, China Full list of author information is available at the end of the article
Introduction Lymphedema is a localized form of tissue swelling due to anomalies and/or a functional overload of lymphatics and/or lymph nodes, which leads to the inadequate clearance of lymph. Lymphedema is clinically and histopathologically distinct from common lymphatic malformation and classically involves an entire limb or anatomic region.
© The Author(s) 2020. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation
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