Scrotal elephantiasis, or massive scrotal lymphedema, is a disease that is caused by obstruction, aplasia, or hypoplasia of the lymphatic vessels draining the scrotum. The scrotal skin is thickened and may exhibit ulcerations in severe cases. It can be either congenital or acquired in nature, with the most common acquired etiology being infection.
With this condition, the entire scrotum becomes extremely large as lymph fluid and tissue accumulates. Eventually, the skin of the scrotum becomes very bumpy and irregular and the penis becomes buried within the tissue. This condition is also referred to as scrotal elephantiasis, but the preference it to use the term scrotal lymphedema, especially when describing a condition limited to the scrotum, generally in obese men. Patients referred with this condition may have massive scrotums so large that the sheer size interferes with walking.
The most common method of staging was defined by the Fifth WHO Expert Committee on Filariasis:
- Stage 0 (latent): The lymphatic vessels have sustained some damage which is not yet apparent. Transport capacity is still sufficient for the amount of lymph being removed. Lymphedema is not present.
- Stage 1 (spontaneously reversible): Tissue is still at the "nonpitting" stage: when pressed by the fingertips, the tissue bounces back without any indentation. Usually upon waking in the morning, the limb or affected area is normal or almost normal in size.
- Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is considered "pitting": when pressed by the fingertips, the affected area indents and holds the indentation. Fibrosis found in stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.
- Stage 3 (lymphostatic elephantiasis): At this stage, the swelling is irreversible and usually the limb(s) or affected area is very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery, called "debulking". This remains controversial, however, since the risks may outweigh the benefits, and the further damage done to the lymphatic system may in fact make the lymphedema worse.
Lymphedema of the genital region is relatively uncommon, but is extremely uncomfortable and distressing for the patients who suffer with this condition. It can affect both men and women alike, but is seen more frequently in males due to the anatomical differences between the genders and effects of gravity. Around ten percent of people who develop leg edema will have associated genital swelling, but some patients can have genital oedema alone.
In some circumstances, genital oedema can occur acutely due to trauma or cellulitis and may be able to resolve completely by itself. Far more usual however, is the chronic genital oedema, which is unfortunately irreversible, but can be controlled and reduced through appropriate lymphedema management. The main cause of genital oedema is either due to primary or secondary lymphoedema.
Primary lymphedema affecting only the genitals is rare. It can be noticed from birth or during the teens, and as the affected individual grows, the involved lymphatic system becomes ever more under pressure to drain the tissue fluid and the swelling becomes far more obvious. The main reasons for primary genital lymphoedema are that the lymph vessels are absent or reduced in number or simply don't work as well as they should i.e. functional failure. It has also been thought that primary lymphedema patients who are obese, have an increased risk of genital swelling due to greater pressure on the groin from the enlarged abdomen.
Secondary lymphedema more commonly affects the genital region than primary lymphoedema. In Africa, India and other tropical countries, genital swelling is frequently seen due to infectious diseases like filariasis. This can lead to gross elephantiasis of the penis and scrotum. In the Western world, the majority of genital oedemas are from trauma or surgery to remove gynaecological, urological, abdominal or prostatic cancers. It has been reported that up to 70% of patients treated for carcinoma to the vulva will have lower body swelling. Radiotherapy to the lymph nodes in the groin or abdominal region can also cause genital lymphoedema. The incidence also increases if there has been surgery and radiotherapy plus episodes of cellulitis.
1. Marked deformity or enlargement of the external genitalia.
2. Skin hard and thick.
3. Presence of knobs or bumps.
Various parts of the genital anatomy can become swollen. In males, both the penis and scrotum, or each, can swell independently. Very few patients just have penile oedema, but it does happen, as can be seen from the case study.
Sometimes, the scrotum becomes so swollen, that the patient has difficulty in walking. As the swelling increases, it can involve the area above the base of the penis (called the pubic area), thus causing the penis to retract into the scrotum. This clearly causes problems for micturition (urination)and sexual activity.
In females, the inner and outer lips of the vagina (labia) can become so swollen that they extend out of the vagina by up to 6 inches; again this creates problems for sexual activity and urination. In both genders, the pubic area on the lower abdomen alone can become oedematous, with associated skin changes and fibrosis.
Genital swelling can occur due to other causes. Palliative patients who have renal, cardiac or hypoproteinaemia (high output failure due to low protein) and patients who have had venous problems, could develop genital oedema. A clear diagnosis and medical investigations are needed, prior to lymphoedema management.
Skin changes are readily seen in genital oedema. Thickening and dry, flaking skin (hyperkeratosis) or warty blisters (papillamatosis) do occur as the swelling progresses.
Pain is a problem for some patients, who describe a dragging, heavy, bursting sensation or an ache around the genital region. This is usually eased when the area is decongested or lifted by a jock straplike support or cycling shorts.
Lymphorrhea occurs when the tissue pressure increases and causes leakage of fluid from the thin layer of skin. Lymphorrhoea can continue for a few days or weeks and carries a high risk of developing infections. It can be very distressing for patients, as some have to wear incontinence/sanitary pads to absorb the copious fluid. Lymphoedema treatment is necessary to stop this leakage.
Sexual Dysfunction happens as the oedema increases. In males, impotence or painful erections impede sexual intercourse. Females find that the presence of oedema dampens sexual activity, due to decreased libido and pain.
- sexually transmitted infections.
- leprosy tuberculosis or deep fungal infection.
- after surgery or lymph node dissection.
- scarring and fibrosis from other causes.
Worldwide, most cases of scrotal lymphedema result from inflammation as a sequela of filarial infection. The most common infections leading to scrotal elephantiasis are lymphogranuloma venereum or filarial infestation with Wuchereria bancrofti.
The rare occurrence of these infections in Western nations makes scrotal elephantiasis an uncommon disease outside of Africa and Asia where the filariasis is endemic. Other causes of this disease include chronic inflammation, neoplasm, irradiation, and lymph node dissection. In the U.S., the cause is usually surgery, irradiation, and/or cancer.
The mainstay of therapy is surgical with medical therapy such as diuretics and scrotal elevation of little value except for very mild cases. Any underlying medical or infectious cause for the lymphoedema, however, should be treated prior to attempting surgical therapy.
Treatment of this condition is guided by the etiology. Response often depends on whether the lymphatic derangement can be reversed. In cases where the lymphedema is caused by fluid overload or congestive heart failure, diuretics can be of benefit. Mild and acute cases due to sarcoidosis may benefit from steroids.
Antibiotics may be all that is necessary in cases of acute infection. When the lymphoedema is chronic, with resultant skin and subcutaneous fibrosis, more aggressive therapy is warranted. There are several surgical options. In most cases requiring surgery, the skin is involved and needs to be removed. The testicular subcutaneous tissue is indurated and full of lymphatic fluid and similarly needs to be removed. The testicles and spermatic cord are generally preserved and unaffected by the lymphedema.
Surgical therapy can be categorized as either bypassing (lymphangioplasty) or excisional (lymphangiectomy). While numerous lymphangioplasty procedures have been conceived using autogenous material (skin bridges, omental transposition), prosthetic conduits (nonabsorbable suture threads), and microsurgical techniques (lymphaticovenous shunts), none have found to be consistently satisfactory in long-term results. It is generally agreed that excisional therapy, which was first described by Delpech in 1820, still provides the most expeditious and reproducible results.
Numerous variations of lymphangiectomy exist but they all have in common the excision of superficial lymphatics, subcutaneous tissue, and skin at the level of Buck’s fascia on the penis with dissection of the spermatic cord and testicles from the edematous scrotal mass. Scrotal reconstruction and coverage varies. If there is not enough scrotal skin left then split-thickness skin grafts and/or fasciocutaneous thigh flaps may be necessary.
Skin Care and meticulous hygiene of the genitals is imperative. Daily bathing with an antibacterial soap and drying the area afterwards is very important to reduce the likelihood of infections. Regular moisturising with an aqueous cream will deter any areas of dry, flaky skin and keep the area soft. As this area is prone to fungal infections and cellulitis, regular inspection will enable the patient to detect any early signs of inflammation.
If an infection occurs, prompt anti-fungal or antibiotic treatment is required. If a patient suffers from recurring cellulitis episodes, then long-term prophylactic antibiotics may be required. Compression Garments or Multi-Layered Bandaging techniques are needed to give the genital area support and compression. The penis, scrotum and labia areas will continue to swell until a firm outer casing prevents them from doing so. This outer casing works by providing the muscles with a base to press against, thereby, reducing the swelling.
The best form of compression garment comes in the form of custom-made tights or shorts. Spandex or padded cycling shorts and sports jock straps are also very useful to provide more comfort to the oedematous areas. Under garments must be firm and supportive, not loose. In some instances, two pairs, or an under garment plus swimming trunks, have been found to be effective.
Foam inserts also can increase the amount of compression to the penis, scrotum or female genital area. Ladies may find that the addition of a sanitary towel underneath garments is also helpful. For male patients with significant penile and scrotum swelling, a regime of multi-layered bandaging may be appropriate. This will consist of washable or disposable bandages and padding/foam being applied to the scrotum and penis separately.
A lymphedema specialist needs to have additional training in managing lymphedema of the genitals, as bandaging the genital area can be very awkward, particularly in getting the bandages to stay in place once the oedema has reduced. Occasionally, bandaging can cause an irritation at the base of the penis and the edge of the scrotal bandaging, thus care must be taken to ensure adequate padding is in place.
Simple solutions that have helped, include creating a harness for the swollen scrotum, using a soft pliable material like splint foam or 'Velfoam' prior to padding and bandaging. The harness creates more uplift for the scrotum and patients find it more comfortable as the bandages don't tend to slip. The harness and the penile bandaging can be kept in place using Velcro strips, as it is much easier to apply and reapply and does, therefore, tend to stay in place better.
The use of compression shorts, post bandaging, also draws the genitals close to the body and also keeps the bandages in place. All bandages can be easily removed for micturition or if soiled, and the patient taught how to apply/reapply them. The use of bandages can significantly reduce the oedema, which would be maintained by compression garments such as shorts or tights.
Exercise in any form is important, as it keeps all the joints and muscles working adequately. If there are no areas of broken skin, then an excellent form of exercise is swimming or walking in the water. The genital area will have some support from the swimming attire and the pressure from the water assists too. Other forms of aerobic exercise that are also useful are cycling and walking, but it is important that compression garments and padding are worn when cycling.
A specific form of exercise for female genital oedema is the pelvic floor exercise. Together with abdominal exercises and diaphragmatic breathing, pelvic floor exercises can help in reducing the oedema. Ask your lymphoedema specialist or physiotherapist for further advice.
Lymph Drainage is an important part of lymphoedema management. Manual Lymphatic Drainage (MLD) and Simple Lymphatic Drainage (SLD) are massage techniques designed to move fluid away from the swollen genital region, to parts that are not affected, to drain freely. The massage itself is very light and is not painful. It is also very useful in softening hard, fibrosed tissue. MLD is a technique that is carried out by trained therapists. SLD is a simplified form of MLD and can be taught to the patient or carer to do themselves.