Chronic lymphedema in patients with kaposiform hemangioendothelioma: incidence, clinical features, risk factors and management

Orphanet Journal of Rare Diseases

Xuewen Xu¹  Xian Jiang²  Yongbo Zhang³  Feiteng Kong⁴  Chuncao Xia⁵  Jiangyuan Zhou⁶  Yi Ji⁶  Kaiying Yang⁶  Siyuan Chen⁷  Xuepeng Zhang⁷  Guoyan Lu⁸ 

[1] Department of Burn and Plastic Surgery, West China Hospital of Sichuan University, 610041, Chengdu, China;Department of Dermatology, West China Hospital of Sichuan University, 610041, Chengdu, China;Department of Pediatric Surgery, Chengdu Women and Children’s Central Hospital, 610031, Chengdu, China;Department of Pediatric Surgery, Sichuan Women and Children’s Hospital, 610045, Chengdu, China;Department of Radiology, West China Hospital of Sichuan University, 610041, Chengdu, China;Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, 610041, Chengdu, China;Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, #37# Guo-Xue-Xiang, 610041, Chengdu, China;Pediatric Intensive Care Unit, West China Second University Hospital, Sichuan University, 610041, Chengdu, China;

关键词: Kaposiform hemangioendothelioma;    Kasabach–Merritt phenomenon;    Clinical characteristics;    Complication;    Risk factor;   

DOI  :  10.1186/s13023-020-01595-2

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY