Page 77 - Virtual Vascular Vol 15

The tissue swelling is due to accumulation of
subcutaneous fluid due to deficiency in

lymphatic drainage.

Lymphoedema can be congenital or acquired.
Congenital inherited lymphedema can be due to:

Type 1: mutation in the FLT4 gene, which
encodes the vascular endothelial growth factor
receptor 3 (VEGFR-3), characterized by onset

soon after birth (Milroy’s disease).
Type 2: mutation in the FOXC2 gene, responsible
for a forkhead family transcription gene factor. It

is characterised by lymphedema below the waist
or in lower limbs. Lymphoedema precox
develops between puberty and age of 35, and

lymphedema tarda which develops in later adult
life.

Acquired lymphedema can be due to filariasis,

malignancy, post surgery or post irradiation.

Lymphoscintography can confirm the level of

obstruction.

Treatment is to investigate and treat the cause,

elevation, stockings, and lymphopress.

There are 2 surgical strategies: first is to provide

alternative lymphatic drainage with for instance
a tongue of omentum tunneled to inguinal
lymph nodes or lymphatic grafting. These have
poor to moderate results. Second is removing all

the oedematous subcutaneous tissues to deep
fascia, and to cover with split skin grafts (Charles

procedure), or skin flaps are elevated along one
border of the limb, and after the deeper swollen
tissue has been excised along with the fascia, the
skin flaps are replaced (Homans-Miller

procedure).

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