Researchers reviewed data on 137 pediatric primary lymphedema.

Median age of apparition of primary lymphedema was 7 years, and was significantly lower in boys than in girls.

Primary congenital lymphedema was more frequent in boys (51%) than in girls (27%),

Lower limbs were the most impacted (88.3%). 38 participants (63.3%) had a pertinent genetic mutation. The most common mutated gene was the FLT4 gene (in 9 patients). Seven patients (5.1%) had associated extensive/central lymphatic malformation.

They concluded that pediatric lymphedema is more frequent in girls, usually involves lower limb, and is often associated with a genetic mutation.