Table 1

 Clinical findings

NumberM/F
F, female; M, male.
Total patients with VEGFR-3 mutations7135/36
Lymphoedema present64/71 (90%)31/33
Age of onset of oedema
    Congenital62/64 (97%)31/31
    Non-congenital2/64 (3%)1/1
Extent of oedema
    Below knee60/64 (94%)28/32
    Above knee4/64 (6%)3/1
Bilateral oedema55/64 (85%)26/29
Symmetry of oedema
    Symmetrical swelling35/64 (55%)14/21
    Right>left15/64 (23%)11/4
    Left>right5/64 (8%)1/4
Unilateral swelling
    Right4/64 (6%)2/2
    Left5/64 (8%)3/2
Hydrocoele13/35 (37%)
Prominent veins16/71 (23%)9/7
Cellulitis14/71 (20%)11/3
“Ski jump” toenails10/71 (14%)5/5
Papillomatosis7/71 (10%)5/2
Urethral abnormality3/71 (4%)3/0