Scrotal elephantiasis is a chronic pathology caused by a violation of the drainage work of the lymphatic system, leading to massive edema of the scrotal skin, fiber, and interstitial tissue. The main symptoms are enlargement of the scrotum and skin changes: hyperpigmentation, keratinization, dryness. The patient complains of a feeling of bursting, sexual function suffers. Diagnostics includes pelvic MRI (CT), ultrasound, lymphoscintigraphy, laboratory tests. With a reversible process, conservative therapy is possible, in advanced cases, an operation is performed aimed at removing the affected skin with subsequent plastic surgery.
ICD 10
I89.0 B74.0
General information
Scrotal elephantiasis is a rare disease in clinical andrology. Limbs are more often subject to lymphostasis, but there is also elephantiasis of the external genitals. The parasitic form with filariasis is recorded in endemic areas of Africa, Southeast Asia, tropical, subtropical zones of South America and the Caribbean. Congenital elephantiasis of the genitals is extremely rare and is practically not detected in isolation. Secondary scrotal elephantiasis is associated with STIs, other diseases and injuries of the scrotum. The peculiarity of scrotal elephantiasis is a gradual progressive course.
Causes
The pathological process is caused by lymphostasis and inflammatory-dystrophic changes in the adjacent fatty tissue, skin, interstitials. Primary elephantiasis develops due to congenital malformations of the structure of the lymphatic pathways, for example, against the background of Meigs syndrome. Secondary lesions of the lymphoid apparatus occur under the influence of some alterating factor and lead to acquired elephantiasis. Conditions that can be complicated by scrotal elephantiasis:
- Sexually transmitted infections. Venereal granuloma (donovanosis), inguinal lymphogranulomatosis are considered as the main sexual infections in which the lymphatic apparatus is affected. Infection occurs more often during a stay in countries with a tropical climate. Before the scrotum increases in size, the appearance of granulomas (ulcers with transparent or serous-purulent exudate) in the genital area is characteristic.
- Other infectious diseases. The extrapulmonary form of tuberculosis, persistent erysipelas can lead to damage to the lymphatic pathways. Lymphadenitis, lymphangitis, accompanying inflammation of any genesis (streptococcal infection, pyoderma, skin cellulite), often occur with impaired lymphatic drainage function, edema.
- Traumatization. Obstruction of lymphatic vessels often develops after wounds, bites or burns of this area, undergoing radiation therapy. Elephantiasis can be a complication of operations on the organs of the scrotum or pelvis, especially those performed for the removal of malignant tumors with regional lymphodisection. Inadequate outflow of lymph against the background of scarring with fibrosis gradually deforms the vessels, as a result of which they rupture, and the fluid is deposited in the skin, subcutaneous fat.
- Compression of the lymphatic pathways. With the progression of the oncological process with vascular compression, the size of the scrotum increases, which is associated with the formation of edema, an increase in lymphogenic insufficiency. Neoplasms detected in areas of chronic lymphostasis are squamous cell carcinoma, Kaposi’s sarcoma, B-cell lymphoma, malignant fibrous histiocytoma. Similar changes occur with the growth and metastasis of tumors localized in the pelvis.
Pathogenesis
The purpose of the lymphatic system is to return proteins, lipids, and water from the interstitial tissue to the intravascular space. In case of violation of lymph outflow (obstruction, compression, inflammation, congenital malformations of the lymphatic system), extravascular tissue contains a large amount of protein, which increases osmotic pressure. This process contributes to the deposition of liquid. Against the background of changes, an inflammatory reaction and proliferation of fibroblasts that produce collagen are triggered. The activity of macrophages increases, which leads to the destruction of elastic fibers and the formation of fibrosclerotic tissue.
With the involvement of venous and arterial vessels, trophism is disrupted, hypoxia joins. Fibrous-sclerotic processes in the skin, adjacent fiber and stroma, lack of oxygen, nutrients, background immunosuppression lead to a modification of the skin: they thicken, pigmented, keratinized. The progression of venous and lymphatic insufficiency is complicated by the appearance of ulceration, erosion, secondary bacterial or fungal infection. Traumatization of lymphatic vessels is accompanied by lymphorrhea.
Classification
There is no unified systematization of the pathological process, according to the severity of the disease, a classification is used, in which five degrees of elephantiasis are distinguished:
- First. The scrotum is slightly enlarged, the skin is swollen, not captured in the fold. Skin pattern according to the type of cobblestone pavement.
- The second. The enlargement of the scrotum is significant, reaching the level of the middle third of the thigh. Lymphorrhea may be present. Lymphedema spreads to the penis.
- Third. Lymphedema or fibredema (fibrotic-sclerotic changes) of the scrotum is determined, the penis is located outside or immersed in its thickness. The scrotum is below the level of the lower third of the thigh.
- Fourth. The penis is hidden in the scrotum, while the dimensions of the cavernous bodies correspond to age. There is a further progression of edema and fibroplastic process. The enlarged scrotum with modified skin is lowered to the knee.
- The fifth. Fibrous-sclerotic altered skin is visualized. (fibredema), the penis is completely hidden. Cases of sagging of the scrotum to the ground are described. The weight of the formation can reach 20 kg or more.
Symptoms
Symptoms depend on the degree of lymphostasis and pathogenetic factor. The onset of the disease is determined by the underlying cause. At the initial stage, the scrotum is slightly enlarged in size due to chronic edema, a fossa remains when pressed. With STIs, ulcers, papules, subcutaneous nodules on the genitals, discharge from the urethra are initially present. In the future, the lymph nodes increase (local adenopathy), abscessing along the course of lymphatic vessels or lymph nodes is possible.
With the filarial etiology of elephantiasis of the scrotum, fever with chills, pulmonary manifestations (nasal cough), conjunctivitis, rash, erythema precede. After 3-12 months, against the background of damage to the lymphatic apparatus, pain in the testicles and appendages, hyperemia, dense swelling of the skin, hydrocele join. With reinvention, clinical manifestations increase.
As fibrotic-sclerotic processes progress, the skin of the scrotum becomes rough (hard), looks like an orange peel or cobblestone pavement, cracks, erosion, keratinization, papillomatous plaques appear on its surface. Yellow or straw-colored lymph oozes from microfractures. Patients complain of a deterioration in the quality of life, difficulties with hygiene; a huge education does not allow them to do their usual things, leads to disability. In the later stages, there are symptoms of dysuria, urinary incontinence.
An acute process with an increased temperature reaction, pronounced weakness, sweating are suspicious of the tumor genesis of the lymphatic system lesion (metastasis), especially if prostate, bladder or testicular cancer was diagnosed earlier. But these same symptoms may indicate the addition of secondary microflora. In this case, wetness, itching, yellow crusts and an unpleasant smell appear.
Complications
Scrotal elephantiasis in men does not allow sexual activity, causes depressive disorders. Patients with chronic elephantiasis for 10 years have a risk of developing lymphangiosarcoma, the most serious complication of this disease. The consequences of elephantiasis also include relapses of cellulite, lymphangitis, bacterial or fungal lesions, lymphangioadenitis. Due to difficulties in care, recurrent urinary tract infections, balanitis, balanoposthitis are typical. For persistent lymphedema of the scrotum, the danger is irreversible damage to the affected tissue with the development of necrotic fasciitis.
Diagnostics
Scrotal elephantiasis at the first stage rarely serves as a reason to seek medical help, since there are no pronounced pain sensations and skin changes. After a preliminary conversation, where the family and epidemiological history (travel or living in dangerous areas), the onset and course of the disease are clarified, the urologist establishes a survey plan. You may need to consult an infectious disease specialist, oncologist, or surgeon. Diagnostics include:
- Visualization studies. Ultrasound of the scrotum and pelvis allows you to assess the condition of potentially involved organs and structures. Magnetic resonance imaging or computed tomography is prescribed if the tumor nature of the elephantiasis of the scrotum is suspected. Lymphoscintigraphy provides general visualization, with its help it is possible to determine the degree of obstruction.
- Laboratory tests. Blood tests for HIV, cancer markers of testicular and prostate cancer, serology or PCR diagnostics for Chlamydia trachomatis and filariae can be useful for determining the cause of elephantiasis. Changes in the general blood count (neutrophil shift, anemia) are typical for a parasitic infection. Evaluation of functional renal tests can confirm renal insufficiency.
- Morphological examination. The histological picture of the modified skin in elephantiasis includes hyperkeratosis with zones of parakeratosis, acanthosis and diffuse edema with dilated lymphatic vessels. With advanced forms, there is pronounced fibrosis, foci of inflammatory infiltration.
Differential diagnosis is carried out with hydrocele, manifestations of chronic heart and kidney failure, testicular cancer, etc. In doubtful cases, a biopsy of the lymph node and the site of chronic ulceration is possible. The scrotum can also reach gigantic sizes with bilateral inguinal hernias, which can be suspected palpationally and confirmed by ultrasound scanning.
Treatment
Management tactics correlate with the cause. Proper skin care (sanitation, moisturizing, softening) is important, the patient is explained the inadmissibility of additional injury to the skin. Secondary elephantiasis of the scrotum implies proper treatment of the underlying pathology in order to reduce lymphatic obstruction. To normalize the state , you can use:
- Conservative therapy. If the elephantiasis of the genitals has not reached irreversible manifestations (stage 1-2), conservative treatment is possible. Antibiotics are prescribed taking into account sensitivity, antiparasitic agents, benzopyrons, retinoids (normalize keratinization and reduce inflammatory and fibrous changes). Diuretics can help with cardiovascular and renal decompensation with scrotal edema. Drug treatment at advanced stages of elephantiasis with fibredema is ineffective and is mainly used to suppress infection before the planned operation and after it to prevent complications.
- Surgical intervention. The volume of the operation depends on the severity of the process, stage 3-5 implies a surgical approach. Incomplete amputation (resection) of the scrotum can be performed – incision of the affected skin, preservation of the penis, testicles and appendages, followed by plastic reconstruction of the defect by an autograft. In advanced cases, total amputation is indicated with the isolation of structures and their placement in the subcutaneous fat of the perineum or subcutaneous pocket of the inguinal region. In the future, plastic surgery is performed. In elderly people with immunosuppression, bilateral orchifuniculectomy is possible.
Prognosis and prevention
The prognosis depends on the stage of elephantiasis and its cause. In malignant disease complicated by lymphedema, the outcome is determined by the stage of the neoplasm and the effectiveness of the main therapy. After surgery, it is possible to restore sexual function and fertility. Genital elephantiasis without treatment progresses rapidly and can lead to disability.
Prevention implies the rejection of casual sexual relations (the use of a condom). For patients with lymphadenectomy and operations on the pelvic organs and scrotum, it is important to observe physical activity that helps preserve the drainage function of the lymphatic system. The risk of parasitic infection can be reduced if, after returning from the endemic zone, filariasis is examined, and protective mosquito nets and insecticides are used during the stay.
