The Ins and Outs of Genital Lymphedema

Lymphedema, the result of a dysfunction in the body’s lymphatic system, is a chronic condition impacting nearly 250 million worldwide.¹ The lymphatic system is mainly responsible for the removal of interstitial fluid, including waste products, from the tissues and alerting the immune system to foreign particles. When there is a dysfunction in this system, fluid and waste products can accumulate in the tissues and a person’s immune response is weakened.
 
Lymphedema is typically categorized as primary or secondary. Primary lymphedema is when one is born with the dysfunction, although the signs/symptoms may not develop until later in life.¹ Secondary lymphedema is when an individual’s healthy lymphatic system is damaged by an event, like a trauma, parasitic infection (filariasis), or cancer treatment.^1,2 Lymphedema occurs most frequently in the extremities; yet it can also develop in the genitals, trunk, and head and neck regions.¹
 
The true prevalence of genital lymphedema for males and females is unknown. Depending on the source the incidence can include up to about 20–80% of males and females post–cancer treatments in the pelvic region, especially if patients undergo a lymphadenectomy.^3–10 This large prevalence range is due to a lack of consistent measurement guides/techniques for genital lymphedema and from individuals (patients and health care workers) not feeling comfortable discussing genital lymphedema.^11–13 From the time people are toddlers, they learn from family, teachers, and society that the genitals are private and should not be discussed with others. This ingrained behavior continues into adulthood and can impact the timely and proper diagnosis of genital lymphedema.^12,14
 
This article on genital lymphedema, the first of two parts, aims to address general concepts, diagnosing, obesity’s impact, and quality of life concerns. A second part will cover treatment options and infections/wounds.

A Closer Look at the Genital Anatomy

According to the update to Starling’s principle, all accumulated fluid in the tissues is due to lymphatic dysfunction. So, all edema in the genitals is due to lymphatic dysfunction and if the fluid remains longer than 3 months, it is considered lymphedema.^15–17 Genital lymphedema can impact portions or the entire aspect of one’s genitalia. For males this can involve the penis, the external portion of the scrotum, and/or the internal fascia/sac, or hydrocele. For females the edema can include the vaginal vault, labia majora and/or the labia minora. For both, the edema can also be in the pelvic cavity around the organs and structures in this region.^1,5,14,18 Due to the anatomical differences, females may show fluid accumulation more internally, vaginally, prior to external involvement.¹⁴
 
The anatomy of the pelvic/genital region for both males and females consists of multiple lymphatic drainage pathways for all portions of the genitals and pelvic organs. This is designed to limit the impact of edema on the components needed for survival (pelvic organs) and for procreation (genitals).^1,5,18 This system works with mild traumas, such as edema from infections, pregnancy, and mild surgeries without involvement of nodes. When individuals undergo lymphadenectomy of the pelvic region, especially involving the external inguinal, common inguinal and/or lumbar (preaortic/lateral aortic) nodes, the risk of lymphedema significantly increases. The higher the lymph node involvement along the nodal drainage pathways, the more likely multiple drainage routes will be involved.^1,5,18Figure 1 shows the multiple drainage pathways with the direct in blue and indirect in green.
 
The skin and tissues of the genital region are also unique. This region does not contain any subcutaneous adipose or direct attachment to bone or cartilage, resulting in constant “strain and tension.”¹⁹ The highly elastic skin has minimal to no keratinizing epithelium and reduced hair follicles. Typical skin tissue contains up to 8% elastin and the skin in the genital region consists of about 30%. This type of tissue is needed to allow for expansion during intimacy, childbirth, and mild traumas; yet it can be detrimental with significant trauma and/or lymphedema, allowing patients to develop severe edema.^19–21 This type of tissue and the presence of hormones, particularly estrogen, enhances the skin of the genitals’ ability to heal quickly with minimal to no scar tissue. The linings, or mucous surfaces, of the area and the multiple orifices create a moist environment perfect for microbes. This can become problematic if the microbes are out of balance.²¹ When an individual is immunocompromised, like with diabetes and/or lymphedema, the imbalance can lead to more serious complications at a faster rate than an individual with a healthy immune system.^19,20

Risk Factors for Genital Lymphedema

There are several risk factors for developing genital lymphedema. These include direct insult to the lymphatic system in the pelvic region, like lymphadenectomy, pelvic radiation, malignant growths invading the region, surgery (including scarring limiting drainage), injury/trauma, chronic inflammation, and infections in the pelvic area.^{3,5,9,22–25} Radiation can cause immediate and long-term damage with radiation-induced fibrosis or scarring, which can interfere with drainage and increase the risk of infections.^5,9
 
Other risk factors include bilateral lower extremity lymphedema, infections (filariasis, venereal) chronic granulomatous disease (including Crohn’s, lymphomas, hepatitis), immobility, and obesity.^3,22,25 Chronic inflammation from infections or other origins in the genital region can increase the creation of fibrotic and/or adipose tissue.
 
Obesity, or excess adipose tissue, can hinder lymphatic drainage, increase lymphatic and circulatory system leakage, reduce the immune response, and cause additional inflammation in the region, creating a feed-forward process.^26,27 Obesity can also increase lymphedema with the enlarged abdomen, or pannus, compressing the inguinal drainage in seated positions.^25,28,29 Obesity is also linked to wound healing barriers.^24,28

What You Should Know About Symptoms and Patient Evaluation

The most common symptoms associated with genital lymphedema for males and females includes pain, heaviness, discomfort, incontinence (bowel and bladder), and sexual dysfunctions. Less common symptoms consist of dysuria (pain with urination), urinary retention, constipation, limitations in mobility/ambulation, difficulty wearing certain types of clothing, limitations in activities of daily living, limitations with work-related activities, and reduced quality of life (QOL).^{3–5,8,11–14,30} The size of the genitals can impact body image, mental health (depression, anxiety), and self-esteem.^3–5 The symptoms can impact one’s relationship with intimate partners, family members, friends, and/or colleagues.
 
Clinicians can use QOL tools (Figure 2), like the Lower Limb and Genital Lymphedema Questionnaire for Men (LLGLQm) or for Females (LLGLQw), to help capture a patient’s subjective symptoms and open the discussion between the healthcare provider (HCP) and the patient on these sensitive issues.^11–13
 
During the evaluation process a health care provider (HCP) may see wounds or sore on the external genitalia, leakage of clear and/or milky liquid (lymphorrhea), wart-like bumps (papillomas, lymphatic cysts, or acne inversa), distorted shapes and/or edema, a buried penis in males, hyperkeratosis, lymphangiectasia (dilated lymphatic vessels), signs of infection, and/or signs of poor hygiene.^{5,23,26,30,31}
 
When palpating the area, the HCP may notice pitting, fibrosis, reduced tissue mobility (need to check penis skin mobility especially), a (+) Stemmers Sign (can be done on scrotum and labia), and/or increased heat.^1,5,14,31 Pitting with females can be more challenging than with males, for often the edema begins more internally. Only HCPs trained in internal evaluations should assess vaginal internal pitting. All HCPs can assess pitting externally (on females and males) just distal the transverse perineal muscles, for this area will increase in edema if there is pelvic or internal genitalia involvement. Please refer to Figure 3 for testing pitting in the region.
 
This is also a way to ease into or reduce anxiety for the patient and/or HCP prior to testing the scrotum, penis, labias, and/or perineum.¹⁴ Infections, erysipelas or cellulitis, are very common with genital lymphedema due to the moist environment, increased microbe involvement, altered immune response, and the reduced lymphatic drainage.^{5,23,25,26,30} A study by Zvonik and colleagues involving 93 patients with genital lymphedema seeking surgical intervention found 85% had at least one erysipelas infection a year, with 23% experiencing at least 6 infections a year.³⁰ The study also found lymphatic cysts and lymphorrhea to be the most important risk factors for infections. A HCP must objectively capture the volume and/or edema involvement in the external genitals. This is most successfully accomplished with point-to-point girth measurements. The most common measurements include circumference of scrotum, anterior-posterior of scrotum, length of penis, circumference of penis, length of labias, and width of labias.¹⁴
 
Other objective findings can include odor, gait deviations, range of motion limitations of the pelvis and/or lower extremities (LE), altered balance, and/or strength deficits of the abdominals, pelvic floor, and/or hip/LE muscles.
 
Click here to read part two of this article on treatment for genital lymphedema.
 
Dr. DiCecco founded LymphEd in 2017. Her research and dissertation was on treatment techniques for females with genital lymphedema with or without lower extremity involvement. Dr. DiCecco completed her Lymphology Association of North America (LANA) Certification in 2013 and her training as a Casley-Smith International Certified Instructor in 2016. Dr. DiCecco is also certified by the Lymphoedema Training Academy in the Fill and Flush Technique for lymphedema treatment. Dr. DiCecco developed the lymphedema and pelvic floor service lines for the Sports Rehabilitation Center in Atlanta, where she is currently Director of the two programs. Dr. DiCecco recently joined the faculty as an Assistant Professor at the Georgia campus for the Philadelphia College of Osteopathic Medicine in the Physical Therapy Department. Dr. DiCecco joined the board for a non-profit organization, the Lighthouse Lymphedema Network (LLN) in 2002 and she is the lead grant writer for the organization. Dr. DiCecco contributed a chapter in the LLN’s book on lymphedema, The Puzzle. She is a member of the American Physical Therapy Association and its Oncology, Women’s Health, and Education Sections. Dr. DiCecco is a member of the Lymphology Association of North America, the Lymphoedema Association of Australia, the Casley-Smith International Group, and the lymphedema round table for therapists in Atlanta.

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