Thursday, November 7, 2013

Giant scrotal elephantiasis due to urinary tract infection

Massive scrotal lymphedema, also termed elephantiasis, can be caused by obstruction or abnormalities of the lymphatic vessels. The most common cause of elephantiasis are parasitic filarial nematodes such as Wuchereria bancrofti. I've recently posted about a young man in Haiti in whom filarial infestation caused a huge scrotal mass weighing 55 kg (that's more than I weigh). Outside endemic filariasis regions in Africa and India, scrotal elephantiasis is extremely rare - however, it does occur, as this case from Germany shows.

In April 2005, a 40-year-old Caucasian male was admitted to the Department of Urology of the University of Regensburg with a massive scrotal swelling. He had been undergoing antibiotic treatment in another hospital for the previous three weeks. Paralyzed from the hips down, the patient had a history of spina bifida, neurogenic bladder dysfunction and insulin dependent diabetes mellitus type 2. He was also slightly mentally retarded. Up to the age of 30 he had used a wheelchair; however, the increasing size of his scrotum had made him bedridden for the past 10 years. He had also undergone a cystostomy because his bladder problem resulted in persistent urinary tract infections due to residual urine.

On examination, the patient had a massively enlarged scrotum extending below his knees. The huge solid scrotal mass, measuring 65 × 55 × 25 cm, made it impossible to differentiate the anatomic structures. The penis was invisible underneath swollen scrotal skin, while numerous superficial pressure ulcers were present on the rear surface of the mass. There was no accompanying swelling of the legs. 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. The results of laboratory tests for HIV and a variety of parasites were all in the normal range.

A surgical intervention was the only option here. Surgeons removed the entire swollen mass, preserving the penis, both testes and spermatic cords, and reconstructed the scrotum using a skin flap. Possibly due to the patient's diabetes, healing was impaired - repeated debridement, vacuum assisted closure therapy for two weeks and a mesh skin graft were necessary before the wound finally healed.

Massive scrotal elephantiasis before corrective surgery. The penis is hidden underneath the swollen skin. Image from: Denzinger et al. (2007)

The patient's scrotum 12 weeks after surgery. Image from: Denzinger et al. (2007)

Scrotal elephantiasis is both emotionally distressing and physically disabling. It is not only unsightly, but causes difficulties with hygiene, urinary incontinence, severe problems with mobility and loss of libido. In the case presented above, the patient was lucky - although the wound after surgery took 10 weeks to heal, the operation markedly improved his quality of life. Not only did it enable him to move by wheelchair again, he even regained the ability to have normal sexual intercourse.

The resected mass, containing numerous fluid-filled cysts, weighed nearly 12 kilograms (that's around 26 pounds). Meticulous histopathologic examination and electron microscopy of the elephantoid tissues showed nonspecific chronic inflammation. Thus, with no other evidence, this case of scrotal elephantiasis was attributed to chronic lower urinary tract infection. Residual urine related to the patient's neurogenic bladder led to chronic inflammation that was further enhanced by his type 2 diabetes mellitus - and resulted in the consequences described above. 

Source: Denzinger S, Watzlawek E, Burger M, Wieland WF, Otto W. (2007) Giant scrotal elephantiasis of inflammatory etiology: a case report. J Med Case Reports. 1: 23. doi: 10.1186/1752-1947-1-23

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