Multimodal treatment of neck lymphatic malformations in children

Today, choosing the optimal management of patients with neck lymphatic malformations (NLM) remains a big challenge. In our study, we analyzed data of 115 patients with NLM who had received surgery and/or sclerotherapy at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia from May 2012 to December 2022 inclusive. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of Russia. The median follow-up time from diagnosis verification was 21.4 months (Q1; Q3; 4.4; 58.3). Macrocystic lymphatic malformations were found to increase the probability of complete remission 7.4-fold (95% confidence interval (CI) 2.7–20.5; p 0.001), while surgery performed either as single-modality therapy or in combination with other treatment increased it 6.3-fold (95% CI 2.3–16.9; p 0.001). Disease stage ≥ III as classified by de Serres decreased the probability of complete remission fourfold (95% CI 1.5–10.9; p = 0.006), while CDS score ≤ 6 points – 3.2 fold (95% CI 1.3–8.4; p = 0.015). At the same time, disease stage ≥ III according to de Serres increased the risk of continued growth or a small ( 50%) reduction in NML volume 4.2-fold (95% CI 1.5–11.7; p = 0.007). Meanwhile, combined therapy and single-modality surgery increased the patients' survival time 7.4 fold (95% CI 2.0–27.8; p = 0.003) and 11.5 fold (95% CI 2.2–58.8; p = 0.004) respectively. Notably, only a relatively small number of minor (n = 22; 19.1%) and major (n = 9; 7.8%) postoperative complications were registered in our study. The results of our comprehensive analysis highlighted the importance of surgery as part of multimodal personalized treatment and laid the basis for a new algorithm for choosing the optimal management of patients with NLMs.