Genital Lymphedema Research Articles

Best practice in managing scrotal lymphoedema

Managing lymphoedema of the male genitals is challenging, distressing and potentially life-threatening if not managed appropriately. Consideration is made of the classification, clinical presentation, lymphatic anatomy and management of genital lymphoedema in clinical practice. The article addresses the issues raised when caring for patients suffering from this condition. A case history demonstrates the use of a novel innovation. The Whitaker Pouch, which can relieve and manage symptoms facilitating self-management and appropriate best practice.

Surgical Management of Scrotal Lymphedema Using Local Flaps

Lymphedema affects all parts of the body, including the scrotum and penis. Genital lymphedema can be a functionally and emotionally incapacitating problem for patients. Patients suffer pain, chronic irritation, repeated infections, drainage, and sexual dysfunction. No ideal surgical or medical therapy exists for the treatment of male genital lymphedema. Fasciocutaneous thigh flaps have been used for coverage of the testes after scrotal lymphedema resection, but these flaps alter testicular thermoregulation and may cause infertility. Skin grafts have also been used for coverage. Use of posteriorly based perineal flaps may preserve perirectal lymphatics that provide collateral lymphatic drainage. We present 2 cases of severe scrotal lymphedema treated by lymphangiectomy and reconstruction with local flaps. Both patients were satisfied with their results and had improved quality of life. We present our miniseries of scrotal lymphedema treated by excision and anterior and posterior flap reconstruction as a successful treatment of this difficult problem.

Linfedema escrotal gigante

To report the case of a patient with long-term giant genital lymphedema as a consequence of partial penectomy with bilateral inguinal lymphadenectomy 26 years before.58-year-old male patient consulting for genital volume increase (penis and scrotum). Diagnostic tests were performed and surgery was indicated.Complete bilateral scrotal excision including testicles and complete amputation of the penile remnant were carried out. Urethra was anastomosed to skin in the root of the scrotum. Post-operative outcome was satisfactory.Genital lymphedema is a disease associated with severe aesthetic and psychological disorders. The present case demonstrates that these patients may be rehabilitated.

A modified therapeutic option for a long lasting success in the treatment of genital lymphedema

In cases of chronic genital lymphedema with an excessive proliferation of scrotal or labial tissue, lymph cysts, and fistulae, complex decongestive therapy (CDT) alone is not enough. After decongestion has been achieved by CDT, a major reconstructive procedure followed by a renewed phase of CDT is required. We report on our good cosmetic and functional long-time results achieved in 24 patients suffering from lymphedema of the genitalia treated by this combination of conservative CDT and surgery. In 17 male patients (16 primary and one secondary lymphedema), reduction of the scrotum was performed. Bilateral reduction of the labia was carried out in seven female patients (five secondary and two primary lymphedema). The mean follow-up was 67.8 months (36–84 months). The etiology of the lymphedema had no effect on the operation procedure or the operative outcome.

Commentary on “A modified therapeutic option for long-lasting success in the treatment of genital lymphedema” by Lang, et al.

Commentary on “A modified therapeutic option for long-lasting success in the treatment of genital lymphedema” by Lang, et al.

Lymphedema of the external genitalia: Experience of the Conakry University Hospital

Objective: This study was carried out to evaluate the management of lymphedema of the external genitalia at the department of general surgery, National Hospital Ignace Deen, Conakry, Republic of Guinea. Patients and Methods: In a retrospective study covering a period of 10 years, the files of all patients treated for genital lymphedema at the Ignace Deen Hospital were reviewed. The patient group consisted of 4 male patients and one female patient with a mean age of 46 years (range: 30 – 71 years). The four male patients had penoscrotal lymphedema with concealment of the penis, while in the only female patient the right labium major and the buttock were affected. No microfilaria could be detected in any of the cases. All patients were subjected to resection of the lymphedematous tissue and reconstructive surgery. Results: In three patients convalescence took 21 days and the postoperative period was uneventful. The two remaining patients developed scrotal suppuration and recovered after 60 days. After two years of follow-up, good cosmetic and functional results could be reported in four patients. Conclusion: Surgery consisting of total excision of the lymphedematous tissue followed by reconstructive surgery remains the only effective method of treatment. It usually yields good cosmetic and functional results and minimizes the recurrence rate. African Journal of Urology Vol. 12 (4) 2006: pp.209-213

Lymphoscintigraphic Appearance of Scrotal Lymphatic Reflux in a Patient With Idiopathic Genital Lymphedema

A 25-year-old, single man presented with penile and scrotal swelling and leakage of fluid from his scrotal skin for the last 12 years. On physical examination, the penile and scrotal skin was diffusely thickened as a result of lymphedema. There were multiple lymphangiomas, which caused eruptions and erythema on the scrotum, and the scrotal skin was wet with leaking lymph fluid. He had no history of surgery, radiation therapy, malignancy, or sexually transmitted disease. Lymphoscintigraphy showed abnormal reflux to the scrotum from the inguinal lymphatics. The patient was diagnosed with idiopathic genital lymphedema and treated with excision of the involved tissue followed by skin graft reconstruction.

Lymphœdèmes pénoscrotaux : étude rétrospective de 33 cas

The aim of this retrospective study was to describe the main characteristics and treatment of male external genitalia lymphedema.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).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.Lymphedema of external genitalia is responsible for discomfort and local complications. Surgical treatment is the main procedure of this disorder.

Management of genital manifestations of lymphatic filariasis

Genital lymphedema secondary to filariasis is a common problem in most of the filarial endemic regions of the world. Repeated filarial attacks lead to obstruction of lymph flow resulting in various types of genital manifestations in both males and females. Currently there is no cure for lymphedema. As yet no operative procedure has restored normal lymphatic function, and significant swelling recurs after all of the currently available approaches. Progress has been made by micro-lymphatic operations combined with conservative measures to relieve many of these patients of much of their swelling without resort to lifetime use of conservative measures.

MANUAL LYMPHATIC DRAINAGE FOR THE TREATMENT OF ACUTE GENITAL LYMPHEDEMA

Lymphedema is the swelling of a body part due to the accumulation of excessive amounts of regional interstitial fluid and decrease or blockage of lymphatic transport.1 Secondary lymphedema is the most common form and usually results from surgical, traumatic or inflammatory disruption or obstruction of the lymphatic pathways. Acute genital lymphedema usually resolves spontaneously but occasionally becomes chronic and may require surgical treatment.2 We report a case of severe acute postoperative genital edema following radical retropubic prostatectomy and bilateral pelvic lymphadenectomy that was successfully managed with 2 weeks of manual lymphatic drainage (MLD) therapy. To our knowledge this is the first case reported in the urological literature of postoperative genital edema treated with decongestive lymphatic therapy. CASE REPORT A 71-year-old man with a prostate specific antigen of 4.5 ng/ml and clinical stage T2a Gleason grade 5 4 9 adenocarcinoma of the prostate underwent bilateral pelvic lymphadenectomy and radical retropubic prostatectomy. Lymphadenectomy included removal of all the tissue from the obturator fossa and along the hypogastric vessels bilaterally. Both neurovascular bundles were excised widely around the prostate because of high volume, poorly differentiated tumor. Final pathological evaluation revealed bilateral Gleason grade 5 4 9 adenocarcinoma of the prostate confined within the prostatic capsule, with all surgical margins, seminal vesicles and lymph nodes negative for tumor. Postoperative hospital stay was uneventful, and the patient was discharged home on postoperative day 3. At postoperative day 11 the patient complained of severe debilitating swelling and pain in the penis and scrotum that interfered with sitting and walking. Physical examination demonstrated the genitalia to be markedly edematous with the scrotum the size of a grapefruit. There was mild penile edema that was not troublesome, and there was no lower extremity edema. There was no evidence of cellulitis. Venous duplex ultrasonography showed no evidence of either deep venous thrombosis or venous obstruction in the lower extremities or pelvis, and pelvic computerized tomography revealed no evidence of a pelvic fluid collection. During the next week the genital edema persisted and was refractory to the usual treatments of genital support and diuretics. Three weeks postoperatively daily decongestive therapy was started, consisting of MLD, lower extremity stretching exercises and overlapping circumferential compressive wrappings to the scrotum that transmitted low pressure compression (20 to 30 mm Hg). MLD was initiated in the inguinal region, moving cephalad, with the goal of transferring lymph from the edematous tissues to nonedematous cutaneous sites. Nonelastic compressive wrappings were applied after each session of MLD and worn continuously between treatments. Each daily therapy session of MLD lasted 3 to 4 hours. Between therapy sessions the patient was also instructed to wear tight briefs or an athletic supporter continuously. After several days of therapy the genital lymphedema and pain improved considerably, and after 2 weeks of treatment it had resolved completely. The patient was able to return to normal activities of daily living without sequelae. He was instructed to continue to wear tight supportive undergarments at home for 2 weeks. At 3 months postoperatively he was doing well, with no residual genital edema, and with excellent urinary control and an undetectable serum prostate specific antigen. DISCUSSION

Lymphangiectasies intestinales primitives (maladie de Waldmann) révélées par un lymphœdème des membres

Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.

Lymphedema management

Lymphedema, defined as the abnormal accumulation of protein rich fluid dysfunction of the lymphatic system, is a common sequela of cancer therapy. The incidence is highest among patients who have undergone resection and irradiation of a lymph node bed. Recently, increased attention has been focused on the modification of anticancer therapies in an effort to minimize lymphatic compromise. Sentinel lymph node biopsy is an example of a surgical procedure developed to preserve lymphatic function. Concurrent with the development of less invasive treatments, the field of lymphedema management has evolved rapidly over the past decade. Combined manual therapy, often referred to as complex decongestive physiotherapy (CDP), has emerged as the standard of care. CDP combines compression bandaging, manual lymphatic drainage (a specialized massage technique), exercise, and skin care with extensive patient education. Case series collectively describing a mean 65% volume reduction in over 10,000 patients attest its efficacy. Pneumatic compression pumps were historically widely used to control lymphedema. Their use as an isolated treatment modality is now rare. Reliance on pumps diminished with the recognition that they may exacerbate truncal and genital lymphedema, as well as injure peripheral lymphatics when applied at high pressures. Many noncompressive approaches, particularly the use of benzopyrone medications and liposuction, continue to be used abroad.

Prise en charge des lymphœdèmesl'expérience de l'hôpital Cognacq-Jay

Treatment of lymphedema includes practical advices, manual lymph drainage, bandaging, self-bandaging, elastic compression garment, skin care and physical exercises. Weight loss may be useful if obesity. Surgery is indicated in genital lymphedema more than in limb lymphedema. In 2001, in Lymphology Unit, 248 women were treated for secondary upper limb lymphedema after breast cancer. Excess of volume of lymphedema was reduced by 31.3% after a mean time of 2.1 weeks. For lower limb lymphedema (primary or secondary), 136 patients were treated with a mean reduction of excess of lymphedema by 29.8% after a mean time of 2.2 weeks. After this treatment, long term follow-up is necessary to maintain results and the motivation of the patient.

Lymphologie 2002.Du diagnostic au traitement des lymphœdèmes

Lymphedema are primary (lower limb) or secondary for the upper limb after treatment of breast cancer. Genetic research allows to explain better pathophysiology of lymphedema. Lymphoscintigraphy is the main imaging technique for lymphedema. Venous or compressive etiologies may be first excluded. Treatment of lymphedema includes decongestive physiotherapy: manual lymph drainages, bandages, elastic stockings, exercises, skin care to avoid infections. Other treatments may be associated: weight loss, drugs (procyanidolic oligomers), surgery for genital lymphedema, pneumatic compression pumps for lymphovenous insufficiency.

High prevalence of Brugia timori infection in the highland of Alor Island, Indonesia.

To identify areas endemic for Brugia timori infection, a field survey was carried out in 2001 on Alor, East Nusa Tenggara Timor, Indonesia. Elephantiasis was reported on this island by villagers as a major health problem. Bancroftian filariasis was detected in four villages in the coastal area, whereas B. timori was identified in four rice-farming villages. No mixed infections with both species were found. In the highland village Mainang (elevation = 880 m), 586 individuals were examined for B. timori infection and 157 (27%) microfilaria carriers were detected. The prevalence of microfilaremic individuals standardized by sex and age was 25%. The geometric mean microfilarial density of microfilaremic individuals was 138 microfilariae/ml. Among teenagers and adults, males tended to have a higher microfilarial prevalence than females. Microfilaria prevalence increased with age and a maximum was observed in the fifth decade of life. In infected individuals, the microfilarial density increased rapidly and high levels were observed in those individuals 11-20 years old. The highest microfilaria density was found in a 27-year-old woman (6,028 microfilariae/ml). Brugia timori on Alor was nocturnally periodic, but in patients with high parasite loads, a small number of microfilariae was also detected in the day blood. The disease rate was high and many persons reported a history of acute filarial attacks. Seventy-seven (13%) individuals showed lymphedema of the leg that occasionally presented severe elephantiasis. No hydrocele or genital lymphedema were observed. This study showed that B. timori infection is not restricted to the lowland and indicated that it might have a wider distribution in the lesser Sunda archipelago than previously assumed.

PRIMARY LYMPHEDEMA OF THE GENITALIA IN CHILDREN AND ADOLESCENTS

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.

Skin graft reconstruction of chronic genital lymphedema

We present a simple, reliable method of scrotal and penile reconstruction yielding satisfactory cosmetic and functional results for patients with disabling chronic genital lymphedema. 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. All patients have had excellent cosmetic results without recurrence of genital lymphedema or compromise of sexual function postoperatively. Single-stage reconstruction for idiopathic genital lymphedema by radical skin excision and split-thickness skin grafting provides gratifying functional and cosmetic results.

Therapy approaches for lymphedema.

Lymphedema, regardless of etiology, is essentially incurable but different therapy approaches exist which serve to contain swelling. The objectives of treatment are to reduce swelling, restore shape, and prevent inflammatory episodes, eg, recurrent cellulitis. There are essentially three main approaches to lymphedema treatment: physical therapy, drug therapy, and surgery. Any edema arises from an imbalance between capillary filtration and lymph drainage. The principle of physical therapy is to a) reduce excessive capillary filtration and b) improve drainage of interstitial fluid and macromolecules from congested regions to normally draining lymph node sites. This is achieved through a combination of compression, exercise, and if possible, massage. Control of recurrent inflammatory episodes can only be achieved through diabetic type skin care, a reduction in swelling, and if necessary, prophylactic antibiotics. Drug therapy comprises diuretics or the coumarin/flavonoid group of drugs. The use of diuretics for pure lymphedema is physiologically unsound but may be of use in edema of mixed origin and in palliative (cancer) circumstances. Coumarin/flavonoids have been shown to reduce swelling in all types of lymphedema. Surgery is indicated at an early stage for facial swelling, where vision is compromised, or genital lymphedema, where genitourinary function is affected. Otherwise, reducing operations should only be performed when alternative therapy has failed. Microsurgery for certain forms of lymphedema continues to be evaluated.

A Surgical Approach to Penoscrotal Lymphedema

In North America idiopathic genital lymphedema is a debilitating but rare disorder that causes pain, disfigurement, and difficulties with urination and potency. We present a simple surgical option for correcting genital lymphedema with good cosmetic and functional results. CASE REPORT A 41-year-old man without significant medical, travel or exposure history presented to the emergency room with a 12-year history of progressive, chronic, massive penile and scrotal edema. The condition had been treated elsewhere with oral antimicrobial agents and observation. Computerized tomography failed to reveal any underlying pathological condition other than chronic infection with resultant scarring and lymphedema. The patient was unable to perform sexually, was required to sit to urinate and had chronic discomfort from the size and weight of the phallus (fig. 1). After 2 weeks of antimicrobial therapy that slightly decreased the lymphedema, surgery was performed via an initial incision on the caudal scrotum at the interface between grossly normal and abnormal tissue. The incision was extended laterally and superiorly until the affected region of skin was circumscribed. While dissecting on the scrotum, both testes and spermatic cords were identified and spared. At the infrapubic region dissection was carried onto the penis to the level of Buck’s fascia (fig. 21, and distally along this plane until a circumscribing incision made just proximal to the glans allowed excision of the lymphedematous tissue in its entirety. Normal caudal scrotal skin was reapproximated to complete 1-stage scrotoplasty. The glans penis was sewn to a Foley catheter to ensure complete elongation. A split thickness skin graft was harvested from the thigh. The graft was tubularized with the suture line positioned on the ventrum of the penis. The distal ends were sewn in place circumferentially using running and interrupted 6-zero polyglactin. Xeroform gauze was placed around the penis and a Styrofoam cup was used for protection. Prostaglandin El (10 pg.) was given intracavernously in the early postoperative period to maintain graft suppleness and prevent contracture. There were no complications other than slight distal contracture. Convalescence was uneventful. The patient is now able to urinate standing and he has no pain. He has erections, engages in intercourse without pharmacological aids and is satisfied with the cosmetic and functional results. DISCUSSION

Reconstruction of Penile and Scrotal Lymphedema

Acquired genital chronic lymphedema and elephantiasis are not common problems in the United States. Most noncongenital cases are due to lymph node dissection, injury, or irradiation. Genital lymphedema is a functionally disabling and emotionally incapacitating entity, especially in the elderly population. Methods of reconstruction reported in the literature involve either lymphangioplasty or direct excision of the involved tissue with local tissue reconstruction. We report a case of acquired genital elephantiasis in an elderly man following radical cystectomy and pelvic irradiation for transitional cell bladder cancer. Our technique of reconstruction consisted of (1) excision of all involved lymphedematous skin of the penis and scrotum, and (2) use of posterior scrotal flaps, superiorly based flap of the pubic area for testicular coverage, and split-skin graft to the penis. Genital lymphedema presents a formidable challenge for reconstructive surgeons. The low morbidity and successful outcome observed in this case, however, indicate that the procedure described herein can and should be offered to elderly patients to improve function and quality of life.