Similarities and differences in the clinical features and management of primary lymphedema and kaposiform hemangioendothelioma associated with lymphedema in children

Yujia Zhang Tong Qiu Congxia Yang Jiangyuan Zhou Ming Yang Xue Gong Zixin Zhang Yuru Lan Xuepeng Zhang Siyuan Chen Yi Ji ^*

• West China Hospital, Sichuan University, Chengdu, China

Background: Primary lymphedema (PLE) and kaposiform hemangioendothelioma-related lymphedema (KLE) are rare vascular anomalies (VAs). The aim of this study was to examine the clinical features, management and prognosis of PLE and KLE.The clinical features, imaging, treatments and outcomes of 12 patients with PLE and 12 patients with KLE were retrospectively reviewed.The mean age at which signs/symptoms were diagnosed was 68.2 months for PLE patients and 25.0 months for KLE patients. In PLE, the involvement of multiple sites is common, whereas in KLE, it typically affects a single site. Morbid obesity, which is common in adult patients, is rare in pediatric PLE and KLE patients. Imaging agent accumulation was observed in KLE but not in PLE via lymphoscintigraphy. In contrast, complications of PLE primarily involve skin and soft tissue, whereas musculoskeletal system complications are more common in KLE. In terms of prognosis, most patients stabilize or even experience lesion regression after standard treatment.PLE and KLE share clinical symptoms. PLE often involves multiple sites, whereas KLE typically presents unilaterally with local lymphatic stasis. Standardized treatment enables the majority of children with lymphedema to control the disease without progression, with KLE showing potential reversibility. Given their rarity, a multidisciplinary approach is crucial for diagnosis and management.