Friday, May 30, 2014

Giant scrotal elephantiasis: a case from India

Lymphedema forms when the lyphatic flow in a body part becomes obstructed. Possible causes include damage to the lymphatic vessels, pathological obesity, parasitic infestation (especially filariasis) and cancer. In very advanced cases of lymphedema, the permanently swollen tissues thicken and become hardened, fibrotic, and the condition is known as elephantiasis. It causes significant disability and distress; the swellings can be truly enormous, interfering with movement and self-care, to say nothing of the patient's appearance.

Giant scrotal lymphedema, also known as scrotal elephantiasis, is fairly rare but singularly unpleasant for the patient, and often emotionally devastating. Thankfully, it can be treated surgically. A while ago, I posted about two cases: a boy from Haiti suffering from advanced filariasis (the most common cause of this condition) and a disabled German man with chronic urinary infections that progressed to elephantiasis over time. Both were successfully operated on. Today, I will describe a grotesque case of giant scrotal lymphedema from India where the resected scrotal tissue weighed a whopping 32 kg - one of the largest scrotal masses described in medical literature.

A 54-year-old male patient weighing 110 kg was admitted to a hospital in Mangalore, India with a massively enlarged scrotum. The scrotal lymphedema had started 8 years earlier and had relentlessly progressed until the swelling reached gigantic proportions. There was no history of any radiation. A previous unsuccessful attempt at partial resection was done early in the course of the disease; after this procedure, the patient refused to be reviewed by physicians because he felt embarrassed. At present, he could not even walk easily and had to sleep on the floor because he was unable to climb onto a bed.

Clinical examination revealed giant elephantiasis totally engulfing the penis. Urine was voided from a deep pit on the scrotal skin. However, the scrotal skin was reasonably healthy. The testes were not palpable. All the routine blood investigations were found normal. Neither clinical examination nor laboratory testing revealed any other abnormality in the abdomen, inguinal regions or in the lower limbs. Though the cause of the lymphedema was thought to be filariasis owing to its endemicity in India, the presence of parasites could not be confirmed by laboratory tests.

A giant edematic scrotal mass dangling between the 54-year-old patient's legs. 
 Image from: Thejeswi et al. (2012)

In more advanced cases of scrotal lymphedema, excision of all the affected skin and soft tissue offers the only possibility of a cure. The procedure is sometimes called reduction scrotoplasty. The posterior skin of the scrotum can be used to reconstruct the scrotum as it is generally less diseased. Possible operative complications include hemorrhage, damage to the urethra, hematoma, wound infection and of course recurrence of the lymphedema.

a - The resected scrotal mass weighed 32 kg! b, c - The patient's genitalia after the operation. The penis was salvaged from deep within the swelling. An acceptable functional and cosmetic result was achieved. Image from: Thejeswi et al. (2012)

In this particular case, surgery was of course the only option. The scrotum was explored and penis was recovered deep within the pit of lymphoedema. Careful dissection was performed with cautery to delineate the penis circumferentially from the root of scrotal lymphoedema. Foleys catheterisation was done. After the separation of the penis, scrotal skin flaps were raised on either side by extending the incision horizontally. Debulking of lymphoedema was done and the remaining scrotal skin was closed in an Y-shape with the root of penis in centre. Meticulous surgical technique made it possible to excise all the diseased tissue with minimal blood loss. The excised specimen weighed 32 kg. The entire procedure took 3 h 20 min. The postoperative period was uneventful and sutures were removed after 2 weeks. A minor wound infection was controlled with antibiotics and regular dressing changes. After healing was complete, the patient was discharged with advice to wear tight undergarments.  

Thejeswi P, Prabhu S, Augustine AH, Ram S. (2012) Giant scrotal lymphoedema – A case report. Int J Surg Case Rep. 2012; 3(7): 269–271. 

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