Long-term outcomes of lymphatic malformations in children: An 11-year experience from a tertiary referral center.

Background: Lymphatic malformations (LMs) are benign, congenital lesions that display considerable heterogeneity in terms of size, location and characteristics. This study aims to describe the long-term outcomes of current management strategies for patients with simple (cystic) LMs.

Methods: The case records of all patients (age ≤16 years) with simple (cystic) LMs at our tertiary institution between 2008 and 2019 were assessed for clinical features, imaging and details of management, including complications.

Results: Of a total of 164 patients (60% male), 66% were diagnosed aged <2 years. The median follow-up was 5 (0.3-16) years from diagnosis. LMs were located in the head and neck (40%), extremities (27%), trunk (23%), mediastinum (4%), or intra-abdominally (6%). Types were macrocystic in 47%, microcystic in 21% and mixed in 32%. Sclerotherapy was the most common intervention (38%). Primary surgery had been performed in 12%. Symptomatic improvement, reduction in size, or complete regression were observed in 82/102 (80%) of LMs after interventions; complications from treatment were uncommon (Clavien-Dindo grade I-II: 6%; grade III-IIId: 1%). Sixty-two patients (38%; median age 0.5 (range, 0-12) years) had not required interventions to date; spontaneous regression of the LM occurred in 16 (26%) of these expectantly followed-up cases.

Conclusions: Most studies to date have focused on LMs in selected anatomical locations. Herein the outcomes of an entire population from a single tertiary unit of patients are presented, demonstrating the wide heterogeneity of simple (cystic) LMs and highlighting the importance of individualized, multidisciplinary approaches to care in achieving optimal outcomes.