Congenital (primary) isolated penile lymphedema is extremely rare and can be a challenge to manage.
The underlying cause is a malformation or hypoplasia of the lymphatic vessels.
Clinical features:
(1) lymphedema limited to the skin and subcutaneum of the penis and/or scrotum
(2) present at or detected soon after birth
(3) variable presence of other congenital malformations
(4) variable leakage of accumulated lymph
(5) variable risk of infection
It may be limited to the prepuce.
The diagnosis requires exclusion of acquired lymphedema.
If persistent and severe then surgical resection of the lymphedematous tissue may be necessary.
