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Klippel–Trénaunay syndrome, formerly Klippel–Trénaunay–Weber syndrome [1] and sometimes angioosteohypertrophy syndrome and hemangiectatic hypertrophy, [2] is a rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly.
These include Klippel–Trenaunay syndrome, macrocephaly-capillary malformation syndrome, ... Surgery is the last resort for non-responding hypertrophied CM. However ...
Overgrowth and malformations of solid tissues can be treated with surgery. Sclerotherapy can be used to treat vascular malformations . [ 5 ] In CLOVES syndrome experimental medical therapy using PIK3CA inhibitor, BYL719 , has been reported to be effective to relieve pain and diminish the malformations.
Syndromes associated with capillary malformations are: Sturge–Weber syndrome and Klippel–Trénaunay syndrome. [14] Capillary malformations can be treated with IPL-(Intensed-pulsed-light)-therapy or surgical resection. [3]
Children with some overgrowth syndromes such as Klippel–Trénaunay syndrome can be readily detectable at birth. [3] In contrast, other overgrowth syndromes such as Proteus syndrome usually present in the postnatal period, characteristically between the second and third year of life. [ 2 ]
Primary lymphedema may be congenital or may arise sporadically. Multiple syndromes are associated with primary lymphedema, including Turner syndrome, Milroy's disease, and Klippel–Trénaunay syndrome. In these syndromes it may occur as a result of absent or malformed lymph nodes or lymphatic channels.
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Parkes Weber syndrome (PWS) is a congenital disorder of the vascular system. It is an extremely rare condition, and its exact prevalence is unknown. [1] [2] [3] It is named after British dermatologist Frederick Parkes Weber, who first described the syndrome in 1907. [4] In the body, the vascular system consists of arteries, veins and capillaries.