Treatment of lymphatic malformations in children: 10 years of experience

Purpose is to identify the factors that determine the clinical features of the LMs, their complications and the choice of treatment options. Materials and methods. 225 children with LMs who underwent treatment since 2011 to 2020 were enrolled. They were grouped according to ISSVA 2018 classification. Clinical presentation, treatment options, complications and outcomes were analyzed. One-variant analysis using Pearson’s χ2 test was used to evaluate qualitative data. Results. 214 (95.1%) patients had cystic LMs, 11 (4.9%) had combined LMs. 129 (57.3%) LMs were cervicofacial, 17 (11.9%) patients developed airways compression and 6 (4.2%) required tracheostomy. 8 (3.6%) patients developed 10 lymphatic leaks episodes, 3 were lethal. 112 (86.8%) patients with cervicofacial, 5 (11.6%) abdominal, 10 (84.6%) axillar, and 12 (92.2%) extremities LMs underwent sclerotherapy. Surgical resections prevailed in abdominal LMs, 32 (74.5%), versus 4 (3.1%) cervicofacial LMs. 10 (4.4%) patients with combined and complicated cystic LMs underwent systemic sirolimus therapy. Results of treatment were excellent in 116 (51.6%) patients, good in 82 (36.4%), satisfactory in 23 (10.2%), and unsatisfactory in 4 (1.8%), with 4 mortalities caused by sepsis (n=1) and by massive lymph leakage (n=3). Conclusions. The most life-threatening LMs complications are airways compression and uncontrollable lymphatic leakages. Several approaches were used, including surgery, sclerotherapy, and target therapy. Surgery is optional for abdominal LMs but potentially harmful in cervicofacial. Treatment of combined LM is continuous and requires a combination of different methods. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: lymphatic malformations, children, sclerotherapy, target therapy.