Surgical treatment of peno-scrotal lymphedema in a patient
with Hennekam syndrome
Received: 4 February 2008 / Accepted: 24 March 2008 / Published online: 9 May 2008
Abstract The management of genital lymphedema due to
congenital agenesis of the lymphatic ducts is reported. The
surgical procedure consisted of the total excision of the
affected skin and subcutaneous tissue followed by scrotoplasty
and split-thickness skin graft to resurface the penile shaft. The
cosmetic and functional results were excellent.
Keywords Genital lymphedema
Hennekam syndrome is a rare autosomal-recessive disorder
characterized by lymphedema of limbs and genitalia,
intestinal lymphangiectasia, and growth retardation .
The degree of lymphedema, which is congenital and
gradually progressive, is extremely severe and represents
a severe limitation to the patient’s quality of life.
Twenty-nine cases of Hennekam syndrome have been
previously reported; however, to our knowledge, this is the
first detailed description of surgical treatment of genital
lymphedema in a man with this condition.
A 31-year-old man, the first born of unrelated parents, was
admitted for evaluation of peripheral edema. Since birth, he
presented protein-losing enteropathy and marked lymph-
edema of limbs and genitalia. An intestinal biopsy, performed
when he was 5 years old, revealed intestinal lymphangiectasia.
At 9 years of age, as the severity of lymphedema was
interfering with ambulation and daily activities, he underwent
derivative lymphatic microsurgery with temporary remission
On physical exam, the scrotum and the lower limbs were
elephantiasic (Fig. 1). The penis shaft was buried in the
penile tissue; the scrotum appeared such a thick, hard mass
with areas of cellulitis. Ambulation was difficult as well as
micturition in standing position and sexual intercourse.
Because of the severity of lymphedema, he needed support
to attend his common daily activities.
Accurate preoperative cleansing of the abdomen, genitalia,
and perineum with an antiseptic solution was performed. A
broad-spectrum antibiotic prophylaxis was given preopera-
tively and continued for several days postoperatively. No
prophylaxis of deep vein thrombosis was administered. The
following procedures were performed.
1. Excision of all the penile lymphedematous skin above
Buck’s fascia up to the balanopreputial sulcus; the penile
shaft was freed and a Foley catheter was inserted (Fig. 2).
2. Midline inverted Y-shaped incision of the scrotal skin
with elevation of a posterior perineal flap. Isolation of
both testicles and spermatic cords; eversion of the
tunica vaginalis (an hydrocele or a lymphocele, as in
our case, is always associated; Fig. 2).
3. Resection of a great fragment of scrotum. Scrotal
reconstruction was achieved using two butterfly-wing-
Eur J Plast Surg (2008) 31:197–200
P. Pepe (*)