Manual lymphatic drainage with infantile klippel-trenaunay syndrome: Case report and literature review

• Main Author: Mag. Margit Eidenberger
• Format: Article
• Language: English
• Published: Taylor & Francis Group 2018-01-01
• Series: Cogent Medicine
• Subjects: infantile klippel-trenaunay syndrome; manual lymphatic drainage; compression therapy
• Online Access: http://dx.doi.org/10.1080/2331205X.2018.1524342

Aim: Klippel-Trenaunay Syndrome is a rare vascular malformation combining venous and lymphatic dysfunctions, oedema and limb overgrowth. Manual Lymphatic Drainage is an efficient therapy in lymphoedemas, but has scarcely been tried in infantile Klippel-Trenaunay Syndrome. Method: Case Report of a two-year-old girl suffering from congenital Klippel-Trenaunay-Syndrome with oedema of the right upper limb and trunk quadrant. The girl was treated with MLD in combination with Compression Therapy until a compression sleeve could be fitted. Results: Arm circumference measurements showed significant differences at wrist and elbow level, when taking the healthy arm as control, also skin temperature was significantly different. MLD could improve oedema during the therapy session, but not in the long term. There was no limb overgrowth during the observation period. MLD therapy protocol had to be interrupted because of thrombophlebitis episodes. Cautious CT including fingers up to axillary region is possible in breast-fed infants and toddlers. Close cooperation with the parents is crucial for therapy adherence and maintenance. Conclusion: MLD and CT can be applied as a combination in small children with specific adaptations, with CT remaining the first line of therapy.