In April 2005, a 40-year-old Caucasian male was admitted to the Department of Urology of the University of Regensburg, Germany, with massive scrotal elephantiasis. He had been undergoing antibiotic treatment in another hospital for the previous three weeks.

The patient had a history of spina bifida with paralysis of the lower extremities, neurogenic bladder dysfunction, insulin dependent diabetes mellitus type 2 and slight mental retardation. Up to the age of 30 the patient was mobile by the use of a wheelchair, however the increasing size of his scrotum had made him bed-ridden for the past 10 years. Suprapubic cystostomy had been performed outside our institution ten years ago due to residual urine and recurrent urinary tract infections. There was no history of sexual contact, surgery, irradiation or travel.

On examination, the patient had a massively enlarged scrotum extending below his knees. The huge solid verrucous scrotal mass of 65 × 55 × 25 cm made it impossible to differentiate the anatomic structures (figure 1) and the urethral orifice emerged as a deep pit on the anterior surface of the mass. The scrotal skin was thickened and edematous hiding the penis. No inguinal adenopathy was found. Many superficial decubitus ulcers were found on the dorsal aspect of the scrotum. There was no accompanying swelling of the lower extremities. The testes and cords were not palpable, but no abnormalities were shown by ultrasonography. A CT scan of the abdomen indicated no cause of the condition. Laboratory testing, including human immunodeficiency virus, markers for testicular cancer, antibodies to schistosomes, Chlamydia trachomatis and filariae, were all in the normal range.

We decided to perform a subtotal scrotectomy with preservation of the penis and both testes and subsequent reconstruction of the scrotum by rotation flap. Primary closure of the wound was achieved.

The excised scrotal tissue weighed 11.6 kg. Grossly, the specimen contained multiple fluid-filled cysts. Histopathologic examination showed nonspecific chronic inflammation with areas of epidermal thickening and dermal fibrosis. Possibly due to the patient's diabetes mellitus, wound healing was impaired necessitating repeated wound debridement, vacuum assisted closure therapy for two weeks and the use of a mesh skin graft. Finally, wound healing was achieved with acceptable cosmetic results and only moderate distortion of the penis (figure 2).

Scrotal elephantiasis is a rare condition outside regions endemic for Chlamydia trachomatis or Wuchereria bancrofti. McDougal presented an overview of etiologic factors of lymphedema of the external genitalia in 2003. Besides infections, neoplasms or chronic inflammation, scrotal lymphedema is rarely caused by congenital conditions, e.g. the rare Meige syndrome leading to malformation of lymphatic vessels of the external genitalia 3.

In this case extensive diagnostics indicated no other cause than chronic lower urinary tract infection and thus the case presented here was attributed to inflammation. Residual urine related to the patient's neurogenic bladder led to chronic inflammation that was further enhanced by his type 2 diabetes mellitus. Postoperatively, even after meticulous histopathologic and electron microscopic preparation of the tissue, no other etiologic evidence was found apart from nonspecific inflammatory reactions.

Some cases of scrotal lymphedema warrant elimination of the cause of the disease e.g. by antibiotic therapy. Usually there is no permanent damage to the skin, lymphatic or subcutaneous tissue 167. However, in a case of persistent scrotal lymphedema, irreversible damage of the involved tissue can occur with the danger of necrotising fasciitis.

In this case we finally opted for surgical intervention. In extensive disease, complete excision of all elephantoid tissue, preferably saving the penis, spermatic cord and testes, is appropriate 289. In accordance with the desires of the patient we preserved the spermatic cords and both testes despite the extent of the disease. If available, scrotal flaps are most suitable for reconstruction of the scrotum. Medial thigh flaps can be used in the absence of adjacent scrotal tissue. Mesh skin graft is widely accepted for use in penile skin defects 10.

Despite protracted wound healing taking 10 weeks until final closure, the patient gained considerably in quality of life. The patient now is able to move himself by wheelchair again. After a follow-up period of 18 months the patient's erectile function was reestablished enabling sexual intercourse. This case shows that surgical therapy can provide good functional and cosmetic results even in massive scrotal elephantiasis.