Genital Lymphoedema

Genital Lymphoedema

By Dr Kristiana Gordon, Consultant in Dermatology and Lymphovascular Medicine, Lymphoedema Clinical Lead, St. George’s Hospital, London

This article is taken from the Spring 2018 issue of LymphLine, the LSN’s quarterly newsletter available to all LSN members. For details of how to become a member, click here.

GENITAL LYMPHOEDEMA remains rather a taboo subject, even in today’s society. The LSN and I sincerely hope this article will raise awareness of the condition and empower anyone who is affected, but previously too shy to mention it, to speak with their lymphoedema therapist or the LSN if they are in need of support or treatment.

Introduction

The majority of patients with lymphoedema suffer with swelling of one or more limbs. However, lymphoedema can affect any part of the body, including the genital tissues. Genital lymphoedema may affect men and women but is more common in men, probably because of the anatomy and dependent nature of male genitalia. Genital lymphoedema rarely occurs in isolation, and is more likely to occur with swelling of the lower limbs.

As with lymphoedema of a limb, genital lymphoedema may be due to ‘primary lymphoedema’ i.e. a genetically predetermined weakness of the lymphatic system. More commonly, genital lymphoedema will be a ‘secondary lymphoedema’ where the swelling occurs as a result of damage to a previously healthy lymphatic system and prevents it from draining the body’s constant production of lymphatic fluid.

Patients may find it difficult to tell their doctor or lymphoedema therapist if genital lymphoedema has developed. However, it is important for them to realise that they can, and should, let their medical team know about these problems in case further investigations are needed. Early diagnosis of genital lymphoedema allows treatment to be introduced, and will reduce the risk of developing complications.

Causes of Genital Lymphoedema

Primary Lymphoedema:

Primary lymphoedema affecting the genital area is a rare problem. It may develop in childhood or young adulthood as a result of genetically predetermined abnormality of the lymphatic system. Although primary lymphoedema is a genetic problem, there does not necessarily need to be a family history of the condition as the genetic mistake (mutation) can, rarely, occur during development within the womb rather than being passed on from an affected parent. To date there are four rare conditions where genetic DNA mutations are known to cause genital swelling. All of these conditions cause lymphoedema of the legs, and sometimes the arms too. These rare diseases are: Emberger syndrome, lymphoedema distichiasis syndrome, Hennekam syndrome and Noonan syndrome. Sometimes, someone with primary genital lymphoedema may develop chylous lymph blisters on the scrotum, penis or vulval tissues. These occur because of abnormal lymphatic pathways in the intestines, resulting in the milky white fat that is absorbed by intestinal lymphatics draining (often as a result of gravity) to the genital tissues and escaping though the skin. These white blisters leak milky fluid after the affected person has eaten a very fatty meal i.e. the fat from the diet cannot be absorbed properly by the gut lymphatics. Management of this rare condition will include advising to avoid specific fats in the diet, with extremely effective and rapid results.

There are several other types of primary lymphoedema where genital lymphoedema occurs with limb swelling, and the underlying genetic fault has not yet been discovered but are the subjects of active research efforts within the lymphoedema clinics. It is important for healthcare professionals to recognise there may be an underlying genetic syndrome causing their patient’s genital lymphoedema as there may be some associated health problems that need to be excluded or treated. Lymphoedema therapists are able to identify when a patient’s genital lymphoedema may be due to a primary lymphoedema and will ensure they are referred to a Lymphoedema Consultant for further investigation and treatment.

Secondary Lymphoedema:

Secondary genital lymphoedema is much more common than primary lymphoedema. It can occur when one or more factors have damaged a previously healthy lymphatic system.

The most common worldwide cause of genital lymphoedema is the parasitic worm infection called Lymphatic Filariasis (also known as ‘Elephantiasis’). This is a neglected tropical disease affecting 40 million people. Fortunately, the disease is not found in the developed world and is so rarely encountered in the UK that there is no need to discuss it in more detail in this article.

The most common cause of genital lymphoedema in the UK is cancer of the male or female urogenital tract (e.g. prostate, penis, testicles, cervix, vulva) and its treatment. The cause of lymphoedema in these situations is usually the (necessary) cancer treatment rather than the cancer itself. The removal of cancerous tissue, regional lymph node(s) and targeted radiotherapy will cause irreversible damage to the drainage routes of lymphatic fluid from the genital region.

Healthy genital tissues are fortunate to have the option of bilateral lymph node drainage pathways. For genital lymphoedema to occur, lymphatic drainage pathways of both groin regions must fail or become blocked. This phenomenon explains why lymphoedema does not affect all patients receiving genital cancer treatment, as the tissues are still able to be drained by preserved lymphatic vessels on one side.

Other causes of secondary genital lymphoedema include any injuries or surgical procedure that disrupts the lymphatic pathway e.g. diagnostic biopsy of an enlarged lymph node in the groin. Occasionally, compression of leg lymphoedema through bandages or pneumatic compression pumps, can push fluid up to the trunk and result in genital lymphoedema, especially if care is not taken to redirect the lymph through collateral drainage routes. This is the reason it is recommended that the use of compression pumps should be in consultation with your lymphoedema therapist.

Uncommon causes of genital lymphoedema include recurrent infections of the genital region, such as cellulitis (a bacterial infection of the skin), which may damage the lymphatic drainage routes. Other rare causes of genital lymphoedema include severe chronic inflammatory skin conditions such as Anogenital Granulomatosis (a rare form of Crohn’s disease/inflammatory bowel disease inflammation affecting the genital region). Healthcare professionals understand the need to consider these rare causes of genital lymphoedema as they may require additional treatments, such as daily antibiotics, to prevent further cellulitis, or anti-inflammatory medicines to control the Crohn’s disease inflammation within the skin.

 

‘Gential lymphoedema remains rather a taboo subject, even in today’s society.’

Complications

Genital lymphoedema can be complicated by infection (cellulitis) of the swollen area. In this situation the skin will become red and hot, and the swelling usually worsens. Prompt treatment with oral antibiotics (taken by mouth), as per the LSN/BLS Cellulitis Guidelines, will hopefully eradicate the infection after 2 weeks of treatment. However, a small number of patients may find the infection returns and will need to consider using daily prophylactic antibiotics to prevent recurrent cellulitis infections. It is important to prevent further infections because each infection causes further lymphatic damage and will exacerbate lymphoedema. Other complications of genital lymphoedema include the leakage of lymphatic fluid from lymph blisters that may form on the penis, scrotum or vulval skin. Chronic leakage from these lymph blisters (lymphangiectasia) can upset the surrounding skin and cause eczema of the area.

Men with penile lymphoedema may find the swelling interferes with passing of urine and perhaps their sexual function.

Genital lymphoedema may also impact on a person’s self-esteem and quality of life. The burden of this condition upon psychological health should not be underestimated and it is important that affected individuals realise that psychological support is available, usually via their GP services.

Management

The prognosis of genital lymphoedema is somewhat dependent on the underlying cause, but established swelling is incurable. However, prompt investigation/diagnosis and treatment will reduce the swelling and the risks of complications.

If someone develops lymphoedema following genital cancer treatment, or after other diseases/procedures that have damaged genital lymphatic drainage pathways, then further investigation is not usually required. In cases of unexplained genital lymphoedema, or of suspected primary lymphoedema, a patient is often referred to a Lymphoedema Consultant for further investigation and advice on management.

All patients with genital lymphoedema will benefit from compression hosiery. This may be in the form of self-bandaging of penile lymphoedema; jock-strap style support garments for scrotal swelling; cycling shorts-style compression garments for men and women (with foam or silicone gusset inserts for women), and there are many different garments that can be offered by lymphoedema therapists. Manual Lymphatic Drainage (MLD) massage may also stimulate and transiently improve genital lymphoedema, as well as keeping the tissues soft and pliable. It may be helpful for patients to become confident in performing self-lymphatic drainage and for this to be used in conjunction with the daily use of compression hosiery.

Affected individuals will benefit from weight management, as obesity can make genital lymphoedema worse (an obese abdomen will further obstruct genital drainage by squashing the lymphatic vessels in the groin region, especially when in the seated position). Maintaining an active lifestyle will ensure the lymphatic system is stimulated. This is important as genital lymphoedema typically occurs in the presence of lower limb lymphoedema (in both primary lymphoedema and cancer-related lymphoedema), and the lymphatic drainage of the lower limb is dependent upon healthy drainage routes via the groins. It is important that lymphoedema of all sites is managed in combination, i.e. with exercise, weight maintenance, compression, and good skin care, in order to prevent complications and to improve quality of life.

Surgery may alleviate some symptoms of genital lymphoedema but is unable to cure the condition. Cautery of lymph blisters (when the small blisters are gently burnt off under a quick general or local anaesthetic) can be undertaken to reduce the amount of lymphatic fluid leakage. This procedure may have the added benefit of reducing the rate of recurrent infections/cellulitis by preventing bacteria from entering the skin via the small blisters if they burst.

Male circumcision can be offered to remove a lymphoedematous foreskin that may be causing problems with passing urine (e.g. spraying of urine, or trapping of urine under the foreskin so that it leaks later on). This operation is technically straightforward and could be offered local to the patient, or the local Urologist may wish to seek advice from an experienced Lymphoedema Urologist before undertaking the procedure.

Debulking (the surgical removal of excess skin) can be offered by experienced surgeons for cases of scrotal lymphoedema. It is possible to consider similar operations for severe penile lymphoedema and female genital lymphoedema, but the surgery may be more complex and require careful planning.

The surgical treatments listed above are not intended to be curative, but may alleviate symptoms and significantly improve quality of life. Surgical results are unlikely to last forever, but can achieve positive results for many years with the daily use of post-operative compression therapy.

Summary

Genital lymphoedema is an uncommon problem but can have a significant impact on someone’s physical health and quality of life. There are several possible causes of the condition and these will be considered by lymphoedema therapists and/or other healthcare professionals involved in a patient’s care. However, in order to help someone with genital lymphoedema access the care that they need, the first step is for the individual to let their therapist know they are having problems. We hope that after reading this article, people will realise that there is effective treatment for genital lymphoedema and that there is no need to be shy in talking about this condition.

Editor’s note: A Genital Lymphoedema information sheet is also available from the LSN office.