Primary Lymphoedema and Lymphatic Malformation: Are they the Two Sides of the Same Coin?
lymphedemapeople
of the Same Coin?
Eur J Vasc Endovasc Surg. 2010 Feb 2
Lee BB, Villavicencio JL.
Division of Vascular Surgery, Georgetown University School of
Medicine, Washington, DC 20007, USA; Georgetown University Hospital,
4th floor PHC, 3800 Reservoir Road NW, Washington, DC 20007, USA.
OBJECTIVES: To clear the confusion regarding the relationship between
the 'primary lymphoedema' and (truncular) lymphatic malformation (LM);
the latter is one of congenital vascular malformations.
MATERIALS & METHODS: A literature review was carried out on the
primary lymphoedema either existing as an independent LM lesion or as
a component of the Klippel-Trenaunay syndrome.
RESULTS: The review was able to provide a contemporary guide/
conclusion on the definition and classification, clinical evaluation
and clinical management regarding conservative (physical) therapy,
reconstructive surgical therapy and ablative/excisional surgical
therapy, for the primary lymphoedema as an LM.
CONCLUSIONS: Primary lymphoedema can be considered as 'congenital'
since its majority represents a clinical manifestation of the
truncular type of LM arising during the later stages of
lymphangiogenesis. Such embryological staging information of the LM is
critical for proper management of the primary lymphoedema when it
exists with other congenital vascular malformations (Klippel-Trenaunay
syndrome). 2. Basic non-invasive to minimally invasive tests will
provide an adequate diagnosis and lead to the correct
multidisciplinary, specifically targeted and sequenced treatment
strategy. 3. The mainstay of current management of the primary
lymphoedema/truncular LM is complex decongestive therapy; and the
reconstructive as well as ablative surgical therapy remain adjunctive
therapies at best. 2010 European Society for Vascular Surgery. ed.