Inelastic Compression Use in Primary Lymphedema

Primary lymphedema in children is a rare, chronic condition caused by congenital or inherited developmental abnormalities in the lymphatic system that lead to persistent fluid buildup and swelling. While there is no cure, early and regular intervention helps control swelling and allows children to lead active lives. While compression therapy (multilayer compression bandaging and compression garment use) is a standard of lymphedema treatment for adults clinical trials evaluating compression therapy specifically for children with primary lymphedema are severely limited. Management algorithms are heavily adopted from adults secondary lymphedema protocols. However the results obtained from clinical trials in adults cannot be directly extrapolated to children with primary lymphedema. Inelastic, short-stretch materials-is highly effective for treating children with primary lymphedema, but requires precise clinical caution to protect fragile pediatric skin. Dose-finding to establish safe and effective interface pressure threshold (measured in mmHg) appropriate for different age cohorts (e.g., infants under 2 years old, toddlers, and adolescents) is needed. Objective of the study is to assess efficacy, physical functioning, safety and adherence to compression therapy based on individually adapted (made to measure, MTM) compression garments (flat knit compression stockings) with medium pressure (23-32 mmHg, ccl2) in children and young adults with primary lymphedema during one and three months of maintenance therapy. The aim of this study is also verification if pressure and stiffness measured in vivo correlate with laboratory findings (compression garment in ccl2 delivered by manufacture) and whether there is a relationship between interface pressures under compression and limb volume measurements in children and young people. The compression garments will be selected individually on the basis of circumference measurements, taking during morning hours with detailed information how long to wear them (i.e. for about 10 hours each day) and will come from the same producer (Medi GmbH \& Co. Bayreuth, Germany). All physical activities are recommended while wearing compression garments.

Inclusion Criteria: * children with recognised primary lymphedema of limbs * age \< 1 year and ≥ 25 year * positive Stemmer's sign * vascular malformation, * no contraindications to compression use * written consent Exclusion Criteria: * primary lymphedema in adults, age ≤ 26 * secondary lymphedema * heart and kidney diseases