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Abstract

A B S T R A C T
Klippel-Trenaunay syndrome is a rare congenital vascular disease. The pathogenesis
of this syndrome is unclear, but it is thought that most cases are the result of somatic
mutations that affect genes that play a role in vasculogenesis and angiogenesis. Some
patients come with a triad of capillary malformation (hemangioma or port-wine stain),
venous varicosities and bony or soft tissue hypertrophy. Clinical presentation of this
syndrome can lead to significant morbidities and mortalities due to severe bleeding
and emboli. Although the number of cases is low, a doctor must be able to distinguish
Klippel-Trenaunay Syndrome from other rare vascular disorders. Parkes Weber
syndrome is usually similar to Klippel-Trenaunay syndrome, except in the arterial
malformations associated with capillary malformations and soft tissue to skeletal or
bone hypertrophy. The diagnosis of Klippel-Trenaunay Syndrome is carried out
clinically and is quite difficult to do even with experienced doctors because there is no
precise pathognomonic test. There are several options in relation to the management
of Klippel-Trenaunay Syndrome and non-invasive procedure is considered to be the
most important of therapy modalities. Early diagnoses, progression monitoring, and
proper intervention should be carried out for better prognosis and preventing
complication.

Keywords

Klippel-Trenaunay Syndrome Capillary malformation Venous varicosities Bone Hypertrophy Congenital Vascular Disease

Article Details

How to Cite
Permatananda, P. A. N. K., & I Gusti Agung Made Adnyana Putra. (2021). Klippel Trenaunay Syndrome: A Brief Overview. Bioscientia Medicina : Journal of Biomedicine and Translational Research, 5(2), 387-394. https://doi.org/10.32539/bsm.v5i2.231