What Is Genital Lymphoedema

Genital lymphedema is a condition characterized by swelling and disability in the genital area. You've come to the right place if you are seeking information regarding this health condition. Learn more to get your desired answers regarding its types, signs and symptoms, causes, diagnosis, and management strategies. 

Genital lymphedema is characterized by the swelling and accumulation of lymphatic fluid in the genital area. It occurs when there is a disruption or obstruction in the normal flow of lymph in the genital region, which is a clear fluid that helps remove waste products and toxins from the body. The condition can affect both males and females and can vary in severity. 

3- Explore the comprehensive overview of genital lymphedema, including its types, causes, symptoms, and management options. Discover the significance of early diagnosis and patient education in improving the quality of life for those affected. Further insights into this rare condition await you.

Overview

Genital lymphoedema is a rare condition that affects a small percentage (0.6%⁷) of all lymphoedema cases globally. It occurs when the lymphatic system, which normally drains fluids from tissues, does not function properly, leading to fluid buildup in the genital region. There are two types of genital lymphoedema: primary (present from birth) and secondary (resulting from trauma or medical procedures). Risk factors include obesity, removal of genital lymph nodes, and specific infections. Common symptoms include swelling, pain, discomfort, and changes in the skin. Early diagnosis and treatment are crucial to prevent complications. Treatment options include conservative therapies, surgery, exercise, and patient education. Recognizing symptoms early can help seek medical attention promptly.

Types of genital lymphoedema

Based on the aetiology, genital lymphoedema is classified into primary and secondary lymphoedema. 

Primary Genital Lymphoedema

Primary genital lymphoedema is a rare condition with a prevalence of approximately 1 in 100,000 individuals.¹  It is inherited or congenital, which means it is present from birth or recognized within the first two years of life. It occurs due to a malformation of the lymphatic system, often resulting from genetic mutations. 

Rarely, boys may experience isolated congenital lymphoedema of the external genitalia.²

In cases of primary lymphoedema, genital involvement is often observed alongside oedema in other parts of the body, particularly in one or both lower extremities. This condition can lead to swelling and fluid retention in multiple areas due to the development of congenital abnormalities in the lymphatic system. 

This condition is uncommon and can have significant physical and emotional effects on the affected children.

Secondary genital lymphoedema

Secondary lymphoedema occurs after any trauma or injury to the lymphatic system. The damage to the lymphatic system could be due to factors such as surgical procedures, radiation therapy and infections. ³

Study shows that patients who undergo pelvic lymph node dissection followed by pelvic radiotherapy have a higher prevalence of genital lymphedema,i.e., ranging from 2% to 22%.¹⁰ 

Causes of genital lymphedema

Any abnormality occurring in the normal function of the lymphatic system causes genital lymphoedema. Following are some specific causes of primary and secondary lymphoedema.

Causes of Primary genital lymphedema:

1. Congenital Lymphatic Dysplasia: It is a very rare condition where the lymphatic system doesn't develop properly from birth

Common causes of secondary genital lymphedema:

1. Injury to the genital region
2. Sexually transmitted diseases
3. Surgical treatment of malignant tumours in the genital area
4. Removal of genital lymph nodes(20-80% of males and females are affected)⁵
5. Radiation therapy for tumours in the genital region
6. Untreated infections of lymph nodes and vessels  caused by bacteria, viruses, fungi, or threadlike worms known as filarial

Rare causes of secondary genital lymphoedema:

1. Lymphadenitis: This term refers to the inflammation of lymph nodes.
2. Crohn's disease is characterized by irritation, swelling, and inflammation of the intestines.
3. Sarcoidosis: It is the development of lesions on the lymph nodes.
4. Vasculitis: It involves inflammation of the blood vessels in the genital region.
5. Rosacea: A chronic skin inflammation condition. ⁴

These external factors can disrupt the normal functioning of the lymphatic system in the genital area, leading to the development of lymphoedema.

Signs and symptoms of genital lymphoedema

Males and females are affected differently by genital lymphoedema. In females, it can affect the labias, vulvar vestibule, clitoris, perineal tissue, and the vagina. Sometimes, only one side, that is, only one labium, is affected. On the other hand, in males, it may involve parts of the penis, scrotum, or both. Sometimes, the penis can become buried within the prepubic skin due to significant scrotal swelling.

The generalized signs and symptoms of genital lymphedema in both males and females include:

• Pain, swelling, heaviness, and discomfort in the genital region
• Incontinence (bowel and bladder)
• Sexual dysfunctions
• Dysuria: This term is defined as pain with urination
• Urinary retention
• Frequent infections
• Constipation
• Skin thickening, or breakdown and wounds in the genital area
• Lymphorrhea (excessive lymphatic fluid leakage)
• Odor
• Limitations in mobility/ambulation
• Difficulty wearing certain types of clothing
• Limitations in activities of daily living
• Limitations with work-related activities
• Reduced quality of life
• Disfigurement
• Frequent bouts of cellulitis
• Psychological distress, including feelings of embarrassment, fear, and reduced libido due to the condition
• Noticeable alteration in gait due to the size of the genitals^{4, 5, 6}

Healthcare professionals must recognize and address these symptoms to provide appropriate care and support to individuals with genital lymphoedema.

Management and treatment for genital lymphoedema

To minimize the risk of infections and skin-related issues like fibrosis or papillomas, early conservative treatment is crucial to caring for genital lymphoedema. Additionally, it can help limit the extent of oedema involvement, potentially reducing the necessity for surgical interventions or enhancing the outcomes of surgeries.  

Now, let’s move on to several treatment and management options for genital lymphoedema.

1. Complete decongestive therapy 

It is the usual approach to managing genital lymphoedema conservatively.⁷ It includes the following components:

• Manual lymphatic drainage (MLD): This involves a specialized massage technique performed by trained healthcare providers to stimulate the lymphatic system and promote fluid drainage.
• Compression Therapy: Applying compression through bandages and garments to the affected genital area helps reduce oedema and improve elasticity. However, applying appropriate compression to the genitals can be challenging due to anatomical aspects and patient comfort concerns. ⁶
• Exercise: The exercises for genital lymphoedema should target pelvic floor muscles, abdominals, gluteals, and other hip/pelvic muscles to aid in fluid reduction through muscle pumps. Specific exercises to manage genital lymphoedema are discussed later in the exercise therapy section.

2. Surgical treatment

Several surgical treatments are available for managing genital lymphoedema. These surgical interventions address the affected tissue and lymphatic system in the external genitalia. The surgical procedures include:

• Excision and debulking surgery: This surgery involves the removal of the involved tissue from the external genitalia, including lymph vessels, adipose tissue, fibrosclerotic/fibrotic tissue, and layers of the skin. While this procedure can improve cosmesis and activities of daily living (ADL), it does not address the underlying lymphatic dysfunction. As a result, there is a high likelihood of requiring additional surgical interventions in the future.
• Microsurgery lymphovenous anastomosis (LVA): It is a procedure where lymph vessels are connected to venules to help remove or redirect lymphatic fluid from the genital area. LVA is usually recommended for the early stages of lymphedema when there are only a few tissue changes. ⁵

These surgeries can benefit but don't fully address the underlying lymphatic issues. Patients must continue or start complete decongestive therapy, especially compression therapy, after surgery to lower the risk of lymphoedema returning. Patients should talk to their healthcare providers about each surgical option's advantages, risks, and possible outcomes to make informed decisions about their treatment.

3. Exercise therapy

Physical activity and movement of the genital region help the fluid move and stimulate drainage. 

Following are some exercises for genital lymphoedema, but all of these should be performed with a soft touch during these exercises for manual lymph drainage (MLD). ⁸

Patient is asked to do the following:

Exercise 1: 

1. Lie down with knees bent and feet on the bed
2. Place both hands on the stomach, just below the ribcage
3. Take a deep breath through the nose, pushing the stomach under his hands
4. Exhale through the mouth while pulling stomach muscles (5 repetitions)

Exercise 2: 

1. Raise one arm above the head and place the other hand below the armpit
2. Slowly move the skin around in a big circle with his hand for about 1 minute
3. Repeat on the other arm for 1 minute

Exercise 3:

1. Use both hands and gently stroke away from the groin towards his armpits on both sides
2. Then, stroke from the centre, just above the genital area, up and out towards his armpits

Exercise 4:

Squeeze the buttocks and hold for 3-5 seconds. (5 repetitions)

Exercise 5:

Slowly move each foot up and down at the ankle joint. It is called ankle pumps. (20 Repetitions for each foot)

NOTE:  Skin should not turn red or pink after performing them.

4. Patient Education:

Patient education is vital in managing genital lymphoedema. HCPs should cover all important aspects to improve patient outcomes and quality of life. Following are some key points that the patients of genital lymphedema are educated about

1. Skin care is essential to prevent skin breakdown, wounds, and frequent infections
2. Maintaining proper hygiene of the pelvic area
3. Using cold compresses can also reduce inflammation
4. Take care of their positioning: Avoid sitting or standing for too long
5. Sex education: Healthcare professionals can provide information on altered positions for intercourse, achieving orgasm without penetration, incorporating self/partner manual lymph drainage (MLD) before and after intimacy, and other ways to enhance intimacy.
6. Maintaining a healthy diet, with less salt⁶

These treatment options are crucial to managing genital lymphoedema and making the patient's life easier. 

Diagnosis

Diagnosing genital lymphoedema involves a series of steps to identify and understand the condition. It is diagnosed based on:

1. Physical evaluation: Lymphoedema indicators include dryness, thickening, blisters with lymph fluid, warmth, darkened skin, and nodules¹
2. Medical history of patients
3. MRI: This scan can help visualise any potential abnormalities or obstructions in the lymphatic vessels
4. Lymphoscintigraphy: Genital lymphedema is confirmed by lymphoscintigraphy.⁹ In this process, an injection of a small amount of radioactive substance can be tracked through the lymph system with a scanner to see if it follows a normal path

Complications

Genital lymphoedema can lead to various complications, including

• Recurring infection
• Repeated bouts of cellulitis and inflammation of the soft tissue beneath the skin
• Superficial bacterial and fungal infections: infections on the skin's surface caused by bacteria or fungi
• Lymphangio-adenitis: it is the inflammation of lymph nodes and vessels.
• Deep vein thrombosis (DVT): a blood clot that forms in a deep vein, usually in the legs
• Severe functional impairment: significant difficulty in performing daily activities
• Psychosocial dysfunction: emotional and social challenges related to the condition
• Cosmetic embarrassment: feeling self-conscious about the appearance of the affected area
• In advanced cases, elephantiasis may develop, where the skin becomes extremely enlarged, thickened, and hardened
• Erysipelas, a bacterial infection that causes fever and inflammation of the skin and underlying tissue, can also occur.^{1, 4}

FAQs

How can I prevent genital lymphoedema

You can prevent genital lymphedema by

• Maintaining proper hygiene
• Taking care of skin
• Being physically active and exercising
• Maintaining proper body weight
• Getting treatment for any infections in the genital region. Alerting the doctor if the infection is not resolving
• Preventing heat exposure to your genital area

How common is genital lymphedema

Genital lymphedema is not a common condition. Worldwide, only 0.6% of cases of lymphedema involve the genital region.

Who is at risk of genital lymphoedema?

People with the following consequences are at higher risk of developing genital lymphedema:

• Obese (BMI higher than 40)
• Bilateral lower extremity lymphedema
• Infections, including filariasis and venereal infections
• Radiation-induced fibrosis or scarring from radiation therapy for cancer treatment.
• Direct blow/trauma to the pelvic region 

When should I see a doctor?

If you notice any of the following symptoms or signs, you need to make an appointment with a healthcare provider:

• Discomfort, swelling, pain, or a feeling of heaviness in the genital region.
• Skin changes, such as redness, thickening, or hardening.
• Recurrent infections or inflammation in the genital area.
• Difficulty with urination or sexual activity.
• Any other unusual or concerning changes in the genital region.

Seeing a healthcare provider promptly can help in early detection and proper management of any potential genital lymphedema or related conditions.

Summary

In conclusion, genital lymphoedema is a rare and distressing health condition, accounting for only 0.6% of all lymphedema cases worldwide. It can be classified into primary and secondary types, with primary lymphoedema being a congenital abnormality and secondary lymphoedema resulting from external trauma or medical intervention. Early diagnosis and proper management are crucial for improving the quality of life and minimizing potential complications. Healthcare providers should be vigilant in recognizing symptoms and providing appropriate care. Treatment options include conservative therapies and surgical interventions. Patient education is also essential for effective management. Continued research and support are vital for advancing our understanding and treating genital lymphoedema.