LYMPHEDEMA GENITAL

Genital Lymphedema

This page has been updated, please see the following pages:

Male Genital Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=male_genital_lymphedema

Female Genital Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=female_genital_lymphedema

Charles Procedure

http://www.lymphedemapeople.com/wiki/doku.php?id=charles_procedure

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With anyone male or female that has lymphedema of the lower limbs, genital lymphedema is a very real possibility. This is especially true of those whose lymphedema expresses itself early in childhood.

While this subject may embarrass some, there needs to be discussion and enlightenment on it.

Related terms: genital lymphedema, lymphedema of the scrotum, lymphedema of the penis, scrotal edema, scrotal lymphedema, labia swelling, vulvar lymphangioma, genital oedema, lymph scrotum, male genital lymphedema, vulvar lymphedema, Chron's Disease, Buck's Fascia, lymphedema of the externa genitalia, genital lymphedema in children

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Tips for Female Genital Lymphedema

Very Special Thanks to Cheri Hoskins and Cyndi Ortiz for their response to questions posted on our discussion boards

Cheri L. Hoskins, CCT
President
Healthtronix Lymphedema Management, Inc.

Link no longer available

It is very difficult to answer questions of this nature without seeing someone or having a history (iteology, etc).

However, there are a few tricks that you can try biker shorts worn daily with compression in the crotch, Elvarex (a Jobst product) makes a wonderful product, just make sure when you are measured the therapist knows that you need compression in the crotch.

When sitting, sit in a reclined position so as to relax the
inguinal nodes and prevent a crimp in that very important drainage area.

In addition, do deep breathing exercises several times a day to create a vacuum in your abdomen.

An excellent resource on genital lymphedema is Gunther
Klose's video you can contact him at 866-621-7888 

Website: http://klosetraining.com/

or Steve Norton 866-445-9674 

Website: http://www.nortonschool.com/managementtraining.html

these two gentlemen are the most knowledgeable in the US on
genital lymphedema and who I go to for consultations. I hope this helps.

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Here is the url for the Jobst/Elvarex garment

http://www.jobst-usa.com/en/products/lymphedema/customelvarex/page.html

and JoviPak has some excellant compression garments as well

http://www.jovipak.com/

I saw Cheri Hoskins gave a few tips on treatment of genital edema. My tips would be pretty much the same. I have seen genital lymphedema respond really well to manual lymph drainage, and some good compression. For daytime compression, bike shorts work really well. For nighttime compression Solaris Tribute garments work well. Solaris has a website at http://www.solarismed.com/ 

If you need insurance approval for that, as they are custom and rather expensive, ask for coverage on code E1399 which is a miscellaneous code for compression. Your doctor will probably need to write a letter of medical necessity. Make sure he reviews the cost of complications that commonly occur with untreated lymphedema such as infections. I have had several patients apply this treatment and it has been effective. Make sure you locate a therapist for MLD who is certified and who has had at least 135 hour of training. There are some people who say they are lymphedema therapist who have maybe been to an afternoon class and are not helpful due to their lack of knowledge.
Hope things go well. Keep us posted.

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Treatment for Genital Lymphedema

Our Deepest appreciation to Denise from

St. Ann's Hospice Lymphoedema Clinic

and to Silkie for obtaining this for us!

Trunk massage and exercise routine (TMER) for patients with Genital Lymphoedema.

Choose a time each day to carry out this massage and exercise programme when you can lie on the bed and relax while you are doing it. You will also need to remove and or loosen any clothing which would get in the way of the massage.

Your skin should not look red or pink at the end of the massage- if it is you must be pressing too hard- go lighter.

1. Lie with your knees bent up, feet on the bed. Place both hands on your stomach just below your ribcage. Breathe in as deeply as you can through your nose so that the air pushes your stomach up under your hands. Then breathe out through your mouth, pulling your stomach muscles in at the same time to squeeze all the air out. Repeat 5 times

2. Place one arm above your head, place your other hand just below your arm pit and gently and slowly move the skin round in as big a circle as possible with your hand. After approximately one minute change and repeat the same routine under the other arm. For approx. 1 minute on each side.

3. Using both hands stroke gently and very slowly from your groins on both sides up towards your armpits. Then stroke from the centre- just above your genital area, up and out towards your arm pits You can do both sides at the same time, or just one side at a time which ever is easier. When massaging try to make sure that your hands are relaxed and the whole hand is in contact with the skin.

Try also to massage your back from the central crease between your buttocks up over your waist-line or ideally get somebody else to help you with this. Massage for at least 2-3 minutes on each side.

4. Place your hands in your groins and as you did in ‘2’, slowly move the skin round in as big a circle as you can. For approx. 1 minute

5. With your knees bent up, squeeze your buttocks together as firmly as possible hold this while you tighten the muscles of your pelvic floor between your legs and then pull in your stomach muscles as hard as you can- hold them tight all together- and then relax. Imagine you are trying to zip up a really tight pair of jeans and having to pull everything in to get the zip to close. As you tighten everything up, breathe out- as you relax, breathe in. Repeat 5 times.

6. Hip and knee exercise. Bend your knee up towards you. Clasp your hands round your thigh and gently pull your knee towards your chest hold it there for a count of 2 then release the pressure by straightening your elbows repeat this slowly a further 4 times. Change legs and go through the same routine on the other side.

7. Finish off with an ankle exercise. Pump each foot up and down at the ankle, slowly and deliberately, 20 times.

This combination of exercise and massage will generally improve the lymphatic drainage from your lower body. Movement and exercise always helps to stimulate lymph drainage. Try not to sit for long periods without movement, keep exercising the muscles of your pelvic floor it will help. You may need advice from a physiotherapist about this.

CARE OF THE SKIN of your lower body and genital area is just as important as of the legs.

Moisturise with a very bland cream such as Aqueous Cream, gently massaging any very firm areas of swelling to soften them. This is best done after the massage and exercise routine above, so that you have cleared the way ahead for lymph to drain. .

Use Aqueous Cream to wash the genital area instead of soap, it is less drying and will reduce irritation.

Always dry very carefully in skin creases and folds and don’t let cream accumulate in them. Too much moisture in the creases encourages fungal infections. If the skin in the creases looks red and irritated, consult your doctor, you might need an anti-fungal cream.

Any infection can make the lymphoedema worse and needs prompt attention- particularly cellulitis.

SUPPORT for the genital area to reduce swelling can be helpful. Some of the hosiery companies do make garments rather like cycling shorts to provide compression in this area. But sometimes buying lycra firm support panties with legs in and placing a pad inside to put additional pressure on the genital area gives further support. Obviously it is important that they don’t constrict the lymph drainage from the legs.

Manual Lymphatic Drainage Massage (MLD) can be helpful with genital oedema. Find out if this is available from your nearest Lymphoedema Clinic. It is available privately in some areas- lists of practitioners are available from MLD UK

(c) Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted. It must be available free and without charge and not used for financial renumeration or gain. Please include an acknowledgement to the author and a link to Lymphedema People. All links associated with this article must be listed as well.

. . . . . . . . . . .

Other sources of advice and support are:

Lymphoedema Support Network LSN - www.lymphoedema.org/lsn

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The Risk of Genital Edema After External Pump Compression for Lower Limb Lymphedema

http://www.lymphedema-therapy.com/98paper.html

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List of available articles from Pub Med

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Link&db=PubMed&dbFrom=PubMed&from_uid=12913680

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Lymphedema Treatments Are Poorly Utilized

Posted 10/29/2003

Ob/Gyn Practice Today

http://www.medscape.com/viewarticle/463235

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Lymphedema Bulletin Board

http://www.lymphedemamanagement.com/bulletin/_article/0000002d.htm

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When Compression Is Not Appropriate

Scrotal Edema

Dr. Reid's Corner

I have seen several patients over the last months that highlight the risk of inappropriate use of compression. One patient had scrotal edema. He had non-Hodgkin's lymphoma and developed edema of the lower extremities and as this became worse, he developed edema of the scrotum. The edema was initially treated with diuretics, which temporarily resulted in decreased lower extremity edema but had very little effect on the edema of the scrotum. Unfortunately, the patient applied a compressive wrap. The scrotal skin is very thin and delicate and the edema further stretched the skin. The compressive garment did not help and caused area of skin breakdown leading to a severe infection. The proper treatment for this patient was to treat the cancer causing the problem, not applying compression of the swollen scrotum. The infection complicated the management of this patient since the infection had to be treated before the chemotherapy could be started. Fortunately, non-Hodgkin's lymphoma is a very treatable cancer and once the patient received the proper treatment with chemotherapy, the cancer decreased significantly in size and the scrotal edema resolved. For additional information on scrotal edema see Dr. Reid's Corner here

http://www.lymphedema.com/wcina.htm

Acknowledgment

Peninsula Medical, Dr. Reid's Corner

http://www.lymphedema.com/

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Scrotal Edema

Dr. Reid's Corner

I have received a number of questions about scrotal edema over the last few months. These questions have asked about using compression for treating edema of the scrotum. In short, I do not think this is a good general practice, let me explain why. Scrotal edema is generally divided into acute or chronic causes. Acute cases are generally a surgical issue and require evaluation by ultrasound. Torsion or twisting of the spermatic cord is the most common etiology of acute scrotum in children. Children with torsion usually present with acute scrotal pain, nausea and vomiting. Surgical treatment, within 6 hours of the onset of symptoms, may ensure the preservation of the testis. For that reason any acute scrotal pain with edema requires urgent and specialized evaluation.

Chronic edema of the scrotum can be caused by a number of conditions such as heart failure, liver failure, venous obstruction, lymphatic obstruction or prior surgery or trauma. For example, patients who have kidney failure and as a result have peritoneal dialysis catheters put in place can develop edema of the scrotum due to drainage from the peritoneal cavity through the inguinal canal and into the scrotum. The proper treatment in this case is surgical evaluation and treatment.

In the case of congestive heart failure or liver failure, the problem is that blood flow to the heart or through the liver is impaired. This results in back flow and accumulation of edema in the legs and often in the scrotum. The proper treatment in these cases is the management of the congestive heart failure or the liver failure. For example, in the case of congestive heart failure, scrotal edema will often improve when some of the stress on the heart is removed by medications. These medications reduce the work of the heart in pumping blood. Other medications cause excess fluid to be eliminated by urination. In liver failure, diuretics are used to remove excess fluid and help reduce the edema.

Edema of the scrotum can also occur due to compression of the veins in the pelvis or abdomen. For example, cancers such as prostate cancer or lymphoma can grow and put pressure on the veins or lymphatics and cause edema. In this case, the proper treatment is control of the cancer so that the pressure exerted on the veins and lymphatics is relieved. I have had many cases of severe edema of the scrotum that have resolved after effective treatment of the cancer that was putting pressure on the veins or lymphatics.

In some cases prostate cancer or non-cancerous enlargement of the prostate can make urination difficult, resulting in the retention of urine in the bladder. If the bladder gets large enough, it can cause compression of the pelvic veins resulting in bilateral lower extremity and scrotal edema. These patients improve dramatically when the excess urine in the bladder is removed and the enlarged prostate is treated by surgical reduction.

Since edema of the scrotum often occurs due to a blockage at the level of either the heart or the liver or the draining lymphatics or veins, application of compression of the scrotum will not fix the underlying problem and may result in worse edema. The scrotum has a limited blood supply and compression of the scrotum could further diminish that blood supply. This could potentially worsen the condition or even result in serious tissue breakdown. There are support devices to help support and cushion an enlarged scrotum. However, I do not know of any approved devices for compression of the scrotum to treat scrotal edema. I am including a figure of the anatomy of the blood flow to the testicles to make my point. The figure shows the arteries in red and the veins in blue. Please note the limited blood supply to the scrotum. Compression of an edematous scrotum may further diminish venous outflow potentially worsening the condition. In addition, the skin of the scrotum is very thin and compression could lead to skin breakdown. This could lead to further serious complications including infection and tissue necrosis.

Acknowledgment

Peninsula Medical, Dr. Reid's Corner

http://www.lymphedema.com/

http://www.lymphedema.com/scrotal.htm

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From The Yale School of Medicine

The treatment of scrotal lymphedema.

Answer:
Worldwide, most cases of scrotal lymphedema result from inflammation as a sequela of filarial infection, usually in tropical regions where the filariasis is endemic. 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 lymphedema, however, should be treated prior to attempting surgical therapy.

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.

Courtesy Yale Surgery, Yale.edu

link no longer available

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Congenital lymphedema of the penis: a method of reconstruction.

Tapper D, Eraklis AJ, Colodny AH, Schwartz M.

Congenital lymphedema of the genitalia has profound physical and psychological consequences for the growing child. Extensive resection of this tissue and reconstruction by skin grafting offers a less than satisfactory cosmetic result. Over the past year we have employed a method of total excision of the lymphedematous tissue of the penile shaft with cosmetic reconstruction without skin grafting. A circumferential incision was made 5-10 mm from the coronal sulcus and deepened to the level of Buck's fascia. The skin and subcutaneous tissue were then completely dissected away from the penis. The skin was everted and all of the abnormal lymphedematous tissue excised up to the dermal skin margin. The skin was then tailored to the size of the penile shaft and reapproximated. This method has been employed in two patients with the advantages of (1) shorter hospitalization, (2) lack of morbidity associated with the skin donor site, and (3) satisfactory cosmetic results.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7411361&itool=iconabstr

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There is also an extended list of articles at this site from
PubMed (government site).

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Display&dopt=pubmed_pubmed&from_uid=3993854

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SCROTAL LYMPHEDEMA

Buck's Fascia Surgery

For scrotal lymphedema, the safest and most effective surgery is called Buck's Fascia. In this surgery, the subcutaneous tissues (layer of swelling/fluid collection) of the scrotum is removed, the skin is then resected with the excess being removed.

You may find additional information under our section on genital lymphedema. The two surgical procedures described here are the safest and most effective techniques used. However, both also may require skin grafts.

For his leg lymphedema, he should be referred to a certified therapist to have decongestive therapy. Once the leg edema is brought under control, there are wraps and garments available that
will hold that swelling in check.

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Labial swelling, clear discharge? Suspect genital lymphedema.(After Pelvic, Gyn. Surgery)
OB/GYN News, August 1, 2003, by Kate Johnson

MONTREAL -- Unusual gynecologic complaints of labial swelling or clear labial/vaginal discharge could be symptoms of genital lymphedema, especially if the patient has had previous pelvic or gynecologic surgery or radiation affecting lymph nodes or vessels.

Awareness of this phenomenon is slowly growing among gynecologic oncologists, but until recently "there was [little recognition] that gynecologic lymphedema could complicate their treatments," Dr. Andrea Cheville, director of the University of Pennsylvania Cancer Center's Lymphedema Program, said at a meeting sponsored by the World Federation for Ultrasound in Medicine and Biology.

"I have found limited receptivity regarding lymphedema on the part of gynecologic oncologists. This reflects the general emphasis in cancer care on disease and worrying about recurrence. Historically, there hasn't been emphasis on addressing the non-life-threatening sequelae," she said in an interview.

Lymphedema can occur after treatment of gynecologic malignancies such as ovarian, endometrial, or cervical cancer, because of the extensive use of pelvic lymph node dissection and radiation therapies. In addition, treatments for bladder, colon, and renal cancer also have potential to compromise the deep lymphatic structures, increasing the risk of gynecologic lymphedema, she said.

The incidence of genital lymphedema is not known, largely because it often goes undiagnosed, but it has been estimated to occur following 10%-20% of all gynecologic oncology surgery and radiation therapy. Like other forms of lymphedema, it most commonly occurs in the first 3-4 years after cancer treatment, but can occur up to 30 years later.

"For patients with this history, if they have any genital swelling; changes in the skin texture; changes in hair growth; thickening of the labia; the presence of papillomas or discreet warty growths; or lymphorrhea, which is leakage of serous fluid through compromised or intact skin, think lymphedema," she said.

Lymphorrhea may be difficult to recognize, especially if it is occurring intravaginally, but physicians can distinguish it from normal vaginal discharge or vaginal infections in a number of ways. "Many times vaginal discharge is whitish or curdish, thick, and opaque, but this is not. Lymphorrhea tends to be clear or a little bit yellow colored. If you culture it, it will be negative. But patients may sometimes complain that it is malodorous. Lymph has no odor, but it is very proteinaceous, which makes it a good culture medium for bacteria," she said.

Genital lymphedema is a devastating condition, but unlike breast cancer, it is not a topic of polite conversation, Dr. Cheville said. She urged physicians to ask patients about these symptoms.

Treatment for the condition, as with general lymphedema, involves combined decongestive therapy consisting of compressive bandaging and manual lymph drainage, but this treatment approach can prove very problematic in gynecologic lymphedema.

"Bandaging is very difficult, because it's tricky to adequately compress the vulvar region," she noted, adding that she uses a specially designed bandage with Velcro straps and odor control pads.

She recommended that unless physicians have training in lymphedema management, they should refer the patient, but she acknowledged the difficulty in finding well-trained therapists.

"There are very few therapists who have comfort and experience treating genital lymphedema. Predominantly these would be physical therapists, but some nurses and some occupational therapists do it as well."

http://www.findarticles.com/cf_dls/m0CYD/15_38/107275640/p2/article.jhtml?term=

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The Medical Alogorithms Project - Chapter 16

16.28 Male Genital Complications of Chronic Lymphatic Obstruction (Hydrocele Lymphedema Elephantiasis Lymph Scrotum)

Overview:

Obstruction of lymphatic drainage from the male genitals can result in retention of interstitial fluid or chyle in the scrotum and/or penis. The duration and extent of the obstruction as well as development of complications determine the eventual outcome for the patient.

Causes of chronic lymphatic obstruction affecting the penis and/or scrotum:

(1) filariasis

(2) sexually transmitted infections

(3) leprosy tuberculosis or deep fungal infection

(4) malignancy

(5) idiopathic

(6) after surgery or lymph node dissection

(7) scarring and fibrosis from other causes

Manifestations:

(1) hydrocele – fluid accumulation in the scrotal sac without skin changes

(2) lymphedema - (elephantiasis when extreme) affects the scrotum and/or penis with changes in size and the skin quality

(3) lymph scrotum – vesicles filled with chylous fluid that easily break and leak

Distinction between hydrocele and lymphedema:

(1) A hydrocele may be unilateral while scrotal lymphedema is bilateral.

(2) A hydrocele does not affect the penis.

(3) In lymphedema the skin is abnormal while in hydrocele it is normal and soft.

(4) It may be hard to exclude hydrocele if lympedema is present.

Features of elephantiasis:

(1) marked deformity or enlargement of the external genitalia

(2) skin hard and thick

(3) presence of knobs or bumps

Complications:

(1) cellulitis

(2) recurrent trauma

(3) infertility

(4) psychological distress or embarassment

Preventive measures:

(1) drainage of hydrocele

(2) frequent cleansing with soap and water

(3) monitoring for breaks in the skin with prompt therapy of cellulitis

(4) proper wrapping to minimize trauma and to collect any exudate or lymph drainage

References:

Dreyer G Addiss D et al. Basic Lymphoedema Management. Treatment and Prevention of Problems Associated with Lymphatic Filariasis. Hollis Publishing Company. 2002. pages 53-62.

link no longer available

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Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature

Abstract

Mu XC, Tran TA, Dupree M, Carlson JA.

Department of Pathology and Laboratory Medicine, Albany Medical Center Hospital, New York, USA

http://www.ncbi.nlm.nih.gov/pubmed?term=Acquired%20vulvar%20lymphangioma%20mimicking%20genital%20warts.%20A%20case%20report%20and%2

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Vulvar lymphedema: unusual manifestation of metastatic Crohn's disease

Servicio de Dermatologia. Hospital Universitari Vall dHebron. Barcelona.

Cutaneous-mucosal lesions constitute one of the most frequent extraintestinal manifestations of Crohn's disease and in some cases may be the first symptom of intestinal disease. We describe the case of a 45-year-old female patient who sought medical help for genital tumefaction of 20 years' evolution. For the previous 15 years, she had been experiencing digestive symptomatology attributed to irritable bowel syndrome. Two months before the consultation, and coinciding with aggravation of the condition, the patient had been diagnosed with colonic Crohn's disease. Skin biopsy of the labia minora revealed sarcoid granulomas. The results of microbiological studies (staining for microorganisms and cultures) were negative. A diagnosis of metastatic vulvar Crohn's disease was made and, treatment with metronidazole was started, which improved the genital edema after 2 months. Genital lymphedema is an exceptionally rare manifestation of metastatic Crohn's disease that may appear several years before intestinal symptomatology develops. Treatment with metronidazole seems to be a good therapeutic option.

Pub Med

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11459566&dopt=Abstract

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Male Genital Lymphedema - Filarial infection

Tropical Medicine Central Resource

http://tmcr.usuhs.mil/tmcr/chapter26/clinical1.htm

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Lymphedema of the external genitalia.

McDougal WS.

Department of Urology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.

PURPOSE: This article presents a simple classification of lymphedema of the external genitalia, which is useful for selecting the appropriate therapy, and evaluates our experience with the various therapeutic options used to treat this disorder. MATERIALS AND METHODS: The literature was reviewed and the records of patients treated for the disorder were analyzed. RESULTS: A convenient classification of the disorder divides cases into congenital and acquired. Therapy is primarily dependent on whether the disease is self-limited and whether there has been any pathological change in the skin, lymphatics and subcutaneous tissue. For self-limited diseases in which no permanent pathological sequelae occur conservative therapy is appropriate. For most chronic conditions a surgical procedure is required. Excisional techniques are most effective for severe forms of the disease. In select cases subcutaneous tissue excision with preservation of the overlying skin is appropriate. However, for most patients excision of the skin and subcutaneous tissue with split-thickness grafting is most effective. CONCLUSIONS: When patients with lymphedema of the external genitalia require surgery and are properly selected for the appropriate procedure, the functional and cosmetic results are excellent and patient rehabilitation is likely.

Pub Med

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12913680&itool=iconabstr

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Primary lymphedema of the genitalia in children and adolescents.

Ross JH, Kay R, Yetman RJ, Angermeier K.

Department of Plastic Surgery, Cleveland Clinic Foundation, Ohio, USA.

PURPOSE: Congenital lymphedema is a rare disorder that may result in disfiguring edema of the male genitalia. We reviewed our experience with 5 cases to advance our understanding of this challenging problem. MATERIALS AND METHODS: Four boys with significant lymphedema underwent excision of the involved subcutaneous genital tissue and coverage with local skin flaps. Two boys in whom this approach failed later underwent complete excision of the involved subcutaneous tissue and skin, and coverage with split thickness skin grafts. The boy with minimal edema was observed. RESULTS: Two of the 4 boys who underwent subcutaneous genital tissue resection and coverage with local skin flaps are markedly improved, although 1 requires further revision. In the other 2 boys treatment failed, necessitating repeat genital tissue excision and grafting. While there have been no recurrences in the grafted areas, each patient has required additional operations to manage recurrent edema in adjacent tissues of the perineum and inguinal region, and in 1 significant contraction of the grafted skin developed. Mild genital lymphedema in the remaining patient has remained stable during 10 years of followup. CONCLUSIONS: Congenital lymphedema of the genitalia is a challenging problem. Recurrences requiring multiple operations are common. We recommend expectant management of mild cases. In more severe cases excision without grafting should be attempted. While skin grafting may be the most definitive solution, it does not prevent recurrence in adjacent regions, and it carries the risk of skin contraction. Skin grafts should only be used when other techniques have failed.

MedLine

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9751400&itool=iconabstr

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Surgical management of congenital lymphedema in infants and children.

Fonkalsrud EW.

Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.

Pub Med

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=485822&itool=iconabstr

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A new surgical approach in genital lymphedema.

Yormuk E, Sevin K, Emiroglu M, Turker M.

Department of Plastic and Reconstructive Surgery, University of Ankara, Turkey.

A new surgical approach has been used in a case of genital lymphedema. After resection of the lymphedematous mass, U-shaped flaps were made from the suprapubic region anteriorly and the posterior scrotal skin posteriorly. The denuded penis was transposed to its original place by passing it through a buttonhole incision made on the anterior flap. The testicles were placed and fixed in pouches prepared between the anterior and posterior flaps. The patient had an acceptable postoperative outcome both in testicular function and habitual sexual activities.

PubMed

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=2243862&itool=iconabstr

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• Case Reports

PMID: 3990547 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=3990547&itool=iconabstr

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Scrotal reconstruction using thigh pedicle flaps: long-term follow-up of 12 cases.

Kochakarn W, Hotrapawanond P.

Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

INTRODUCTION: Genital skin loss in men may be caused by avulsion injuries of the penis and scrotum or by gangrene of the male genitalia. Reconstruction of the scrotum after complete loss of the overlying skin is a challenging problem. We report our experience on the management of this problem. MATERIAL AND METHOD: Medical records of all male patients with massive scrotal skin loss and exposed testes treated at Ramathibodi Hospital and Noparat Rajthanee Hospital from 1990 to 1999 were reviewed. The etiologies of scrotal skin loss, technique of treatment, post-operative consequence as well as complications were noted. RESULTS: Twelve patients were described in this study. Nine patients had avulsion injuries of the penile and scrotal skin secondary to agricultural machinery accidents. Three patients were after extensive debridement of Fournierris gangrene. The exposed testes had been placed in thigh pouches and scrotal reconstruction using thigh pedicle flaps was done 4-6 weeks later. No immediate and delayed complications were detected in all of the patients. They recovered without any sequelae and had a satisfactory cosmetic result. CONCLUSION: Extensive scrotal skin loss should be immediately treated surgically. Implantation of the exposed testes in the upper thigh pouch and delayed reconstruction of the scrotum using thigh pedicle flaps can provide excellent results

PMID: 11999821 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11999821&itool=iconabstr

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Lymphoedema 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 oedema 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 lymphoedema management. The main cause of genital oedema is either due to primary or secondary lymphoedema.

Primary lymphoedema 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 lymphoedema patients who are obese, have an increased risk of genital swelling due to greater pressure on the groin from the enlarged abdomen.

Secondary lymphoedema 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.

Clinical Features

Swelling - 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.

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.

Skin changes are readily seen in genital oedema. Thickening and dry, flaking skin (hyperkeratosis) or warty blisters (papillamatosis) do occur as the swelling progresses.

Acute Inflammatory Episodes (cellulitis) are commonly seen in oedematous skin, which is the ideal medium for bacteria as it is generally warm, moist and has numerous crevices. The bacteria multiply in the protein rich oedema fluid, and infections can spread throughout the genital region, causing it to be red, hot, tender and swell even further. More often than not, an infection is seen as the precipitating factor in causing the swelling.

Fungal Infections do occur, due to the area being moist, warm and having so many crevices. Sweating also can trigger fungal infections.

Lymphorrhoea 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.

Lymphoedema Treatment and Management

The four cornerstones of lymphoedema care can be modified to treat genital oedema.

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 longterm 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. Your lymphoedema specialist will need to have had additional training in managing lymphoedema 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.

Surgical Management
In some cases where conservative treatment does not control the swelling, surgical intervention may be required. Surgery could involve reducing the scrotum, penis or labia with the aid of plastic surgery skin grafting.

Case Study
Mr A is a 68-year-old gentleman who has suffered with genital oedema since November 2001.Whilst on holiday in 2001, Mr A developed a painful spot on the right buttock possibly from an insect bite. Unfortunately, this blemish continued to increase in size and eventually became an abscess. He was operated on 3 times in a generalist hospital and due to infections and gangrenous tissue, some of his inguinal lymph nodes were removed. Mr A's genital swelling started soon after the surgery and was sited in the penis area alone. He unluckily had numerous cellulitis episodes, which in turn increased the penile swelling. The scrotum area was severely distorted due to the previous operations and in December 2002, Mr A underwent plastic surgery to graft and lower the testicle area, which although improved the cosmetic appearance of the testicles increased the penile swelling.

Mr A was referred to the lymphoedema service and assessed in June 2003. On examination, the genital area was red, inflamed and had a discharge from the shaft of the penis, which was grossly oedematous. The lymphorrhoea had been present for the last 6 months and Mr A had to pad the area to stop it staining his under garments. Severe skin changes were apparent with brown discolouration patches, hyperkeratosis and fibrosis all over the penis. The pubic area was also swollen and fibrosed.

Functionally, Mr A felt all forms of activity were limited, as well as travelling and socialising. He suffered an extreme amount of discomfort and pain, which hindered his mobility, and psychologically he felt that the oedema had greatly affected his quality of life and the way in which he viewed himself as a man.

Treatment commenced immediately, with Mr A starting a 2-week course of antibiotics to manage the infection. Information regarding hygiene and daily moisturising with an aqueous cream was initiated to help the skin changes, and antibacterial talc was recommended to reduce friction in the groin region. A simple technique of bandaging was also taught to the patient to reduce penis size and stop the leaking fluid. MLD was started and SLD was taught, to improve the fibrosis and create collateral drainage.

Mr A was reassessed four weeks later and was delighted with the results. His penile swelling had reduced significantly, making it look far more normal. The skin condition was greatly improved with all areas of hyperkeratosis and leaking diminished. His mobility was normal due to the pain being relieved and he informed me that he had booked a holiday. He is continuing with his lymphoedema regime, consisting of SLD, multi-layered bandaging and daily use of his compression padded cycling shorts, which will keep him in control of his genital oedema.

For further information regarding genital oedema, ask your lymphoedema specialist or medical practitioner.

http://www.lymphoedema.org/lsn/lsn140.htm#TOP

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• Case Reports

PMID: 6343640 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6343640&dopt=Abstract

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Lymphedema of male external genitalia: a retrospective study of 33 cases

[Article in French] 2005

Vignes S, Trevidic P.

Unite de Lymphologie, Hopital Cognacq-Jay, Site Broussais, Paris. 

INTRODUCTION: The aim of this retrospective study was to describe the main characteristics and treatment of male external genitalia lymphedema. 

PATIENTS AND METHODS: From 1987 to 2003, all patients seen in a single hospital for lymphedema of male external genitalia were included. For each patient, the following characteristics were recorded: primary or secondary lymphedema, cause of secondary form, date of onset of lymphedema, associated lower limb lymphedema, clinical signs, and complications. In the primary forms, lower limb lymphoscintigraphy was performed. Specific surgery was proposed in all cases of symptomatic lymphedema (circumcision, scrotum and/or penile cutaneous excision).

RESULTS: Thirty-three patients with lymphedema of external genitalia (17 primary, 16 secondary) were recruited. Two primary lymphedema were congenital, one isolated. Mean age +/- SD of the onset of the 15 other primary genital lymphedema was 23.4 +/- 17.5 years, always after the appearance of lower limb lymphedema. Sixteen men had secondary lymphedema (bladder, prostate, or rectum cancer, Hodgkin or non-Hodgkin lymphoma, aorto-bifemoral bypass grafting, biopsy or curretage of inguinal nodes). Secondary genitalia lymphedema was not associated with lower limb lymphedema in two cases and, in the others it occurred 66 +/- 122 months after (n=11), at the same time (n=2) or before lower limb lymphedema (n=1). Clinically, we noted genitalia heaviness (n=31), lower limb lymphedema (n=30), vaginal hydrocele (n=13), impaired miction due to prepucial swelling (n=10), leakage of lymphatic fluid (n=10). Lower limb lymphedema was complicated by at least one erysipelas (n=20), spreading to the external genitalia (n=4). In primary forms, lymphoscintigraphy showed ipsilateral hypoplasia of inguinal nodes in lower limb lymphedema (n=14) and/or external genitalia backflow (n=7). Surgical treatment was performed in 17 cases (11 primary, 6 secondary) with good results after 21 months' median follow up (1 month-10 years). Two patients died of cancer. One secondary lymphedema improved spontaneously and one disappeared after withdrawal of lower limb pneumatic compression.

DISCUSSION: Lymphedema of external genitalia is responsible for discomfort and local complications. Surgical treatment is the main procedure of this disorder.

PMID: 15746602 [PubMed - indexed for MEDLINE]

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Skin graft reconstruction of chronic genital lymphedema.

Morey AF, Meng MV, McAninch JW.

Department of Urology, University of California, School of Medicine, San Francisco 94143-0738, USA.

OBJECTIVES: We present a simple, reliable method of scrotal and penile reconstruction yielding satisfactory cosmetic and functional results for patients with disabling chronic genital lymphedema. METHODS: Nine patients were treated with wide excision of the affected genital skin and subsequent coverage of exposed areas with split-thickness skin grafts in a single-stage procedure. RESULTS: All patients have had excellent cosmetic results without recurrence of genital lymphedema or compromise of sexual function postoperatively. CONCLUSIONS: Single-stage reconstruction for idiopathic genital lymphedema by radical skin excision and split-thickness skin grafting provides gratifying functional and cosmetic results.

PMID: 9301709 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9301709&dopt=Abstract

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The effects of reduction operation with genital lymphedema on the frequency of erysipelas and the quality of life.

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