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REVIEW ARTICLES |
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| Year : 2005 | Volume
: 21
| Issue : 1 | Page : 39-43 |
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Management of genital manifestations of lymphatic filariasis
G Manokaran
Consultant Plastic Surgeon & Lymphologist, Apollo Hospitals, Chennai, India
Correspondence Address:
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-1591.19550
 Abstract | | |
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.
Keywords: Filariasis, Genitalia, Lymphedema
How to cite this article:
Manokaran G. Management of genital manifestations of lymphatic filariasis. Indian J Urol 2005;21:39-43 |
| Introduction | |  |
The problem of genital lymphedema is commonly secondary to filariasis in most regions of the world. Parasitic nematode worms which produce filariasis in humans place approximately one billion people at risk in more than 75 countries. Filariasis is a significant health problem and more than 100 million people are infected with these diseases. [1],[2],[3],[4],[5] Repeated filarial attacks lead to obstruction of lymph flow resulting in various types of genital manifestations in both males and females .
| Pathophysiology | | |
Primary lymphatics arise in the interstitial tissue as capillaries that consist of an endothelial layer one cell thick. These lymphatic capillaries are valveless and enlarge to form secondary lymphatics. The secondary lymphatics have some valves, but it is only in the tertiary lymphatics that the valves become numerous and occur at regular intervals. The walls of the tertiary lymphatics consist of three layers; an outer adventitia, an intermediate muscular layer, and an inner endothelium. The muscular layer is responsible for rhythmic contractions of the lymphatics. The intrinsic lymphatic muscle contractions, together with the muscle pump action, the presence of valves, and the negative intrathoracic pressure, account for the unidirectional flow of lymph. The intralymphatic pressure is subitmospheric and becomes more so at the thoracic duct level. This descending gradient increases during deep inspiration and accounts for the flow of lymph in an anesthetized patient when the muscle pump action is absent. The main superficial lymphatic trunks accompany the main superficial veins, and the deep lymphatics accompany the deep artery and vein. There are only a few communications among the superficial and the deep lymphatics. The direction of flow is from the superficial to the deep lymphatics. [2],[4]
Edema occurs only when the number of functioning lymphatics is reduced below a critical level or when there is an increased lymphatic load. Primary lymphedema may develop at birth (congenital lymphedema) or later (lymphedema praecox and lymphedema tarda). Secondary lymphedema can develop years after filariasis, surgical or radiation therapy. A decrease in the number of functioning lymphatics after firosis or sclerosis results in high protein edema, which increases the tissue osmotic pressure and causes an additional shift of fluid into the interstitial tissue. This process provokes an inflammatory reaction resulting in perilymphatic scarring and obliteration of lymphatic trunks, reducing lymphatic transport even more. The effect of lymphedema is seen mainly in the more expansile superficial tissue. There is increased thickness of the skin (primarily of the dermal elements) and scarring of the subcutaneous tissue. Extensive scarring in the subcutaneous tissue can result in venous obstruction, but this is a rare and a late phenomenon. Experimental work demonstrated that it is the deep lymphatics that first show evidence of obstruction. The communications between the deep and the superficial lymphatics become incompetent, leading to dilatation of the superficial lymphatics in the more expansile subcutaneous tissue. Dilatation of the superficial lymphatics precedes the appearance of edema by months or years.
Lymphaticovenous communications can occur normally. These communications also are seen in abnormal circumstances but are not adequate to prevent edema. Researchers have attempted artificial lymphovenous anastomoses in an effort to drain the excess lymph in obstructive lymphedema. Mainly for technical reasons the initial emphasis was placed on anastomosis of a transected lymph node into the side of a vein. Doubts exist whether these anastomoses continue to be patent, although good clinical results have been reported. With microsurgical techniques, increased patency has been obtained by direct lymphaticovenous anastomosis and by lymphaticolymphatic anastomosis.[2],[3],[4]
| Diagnostic tests | | |
Investigations are carried out to establish the diagnosis when it is not clear from a careful history. It is essential to establish a diagnosis because in primary lymphedema the lymphatics are either hypoplastic or absent and are unsuitable for drainage procedures. Ten percent of the patients with secondary or obstructive lymphedema do not have lymphatics of a suitable size for microlymphaticovenous anastomoses. It would be useful to know the condition of the lymphatics preoperatively thus avoiding an unnecessary exploration, but such a precise test is not available. In secondary lymphedema it is necessary also to exclude active malignant growth. Lymphoscintigraphy using Tc rhenium sulphide colloid or WmTc pertechnetate stannous sulphur enables identification of the lymphatics and the draining nodes but does not enable direct visualization of the anastomosis; however, the rate of clearance of the radiotraccr is good indirect evidence of lymphatic function. [Figure - 1]. [1],[2],[3],[4],[5]
| Treatment | | |
Conservative treatment
Mild lymphedema can be managed effectively by nonsurgical measures. These measures are also essential preoperatively and postoperatively as an adjunct to various procedures. The aims of conservative treatment are to reduce hydrostatic pressure, minimize valvular incompetence, decrease infection, and treat lymphangitis vigorously when it occurs. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15]
Drug treatment
Benzopyrones (coumarin and 7 hydroxy- coumarin) have been used in the treatment of lymphedema. These drugs increase the proteinase activity, and the large protein levels are broken down into smaller groups. The resultant peptides and amino acids have a favourable osmotic balance and can diffuse readily into the local veins. This reduces protein concentration in the interstitial tissues and the associated edema. Drug treatment may have a place in the treatment of mild edema, although the benefit appears to be inconsistent and the drugs take a long time to act.
Surgical treatment
Surgical intervention is considered only after the medical management has failed. Despite the fact that cosmetic indications have been minimized in the past, it must be remembered that this condition frequently affects adolescent girls, and the associated physiologic problems must be considered. Finally, in some patients, regardless of conscientious medical management, recurrent lymphangitis cannot be controlled. In general, these operations may be divided into two divergent categories: physiologic or drainage procedures and excisional procedures. The excisional procedures remove varying amounts of involved subcutaneous tissue and skin. The physiologic procedures attempt to reconstruct lymphatic drainage by introducing distant or local pedicles or by microvascular techniques and thus bypass the obstruction in the lymphatics and reestablish lymphatic flow. The dermal flap and omental transposition combine excisional and physiologic components. These drainage techniques have greatly improved the results over last decades. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15]
Physiologic Operations (Drainage procedures)
(i) Lymphatic bridging procedures
In lymphatic bridging, pedicled flaps and omental flaps are transposed across the lymphatic block. It was hoped that regeneration of lymphatics would establish communication between the blocked lymphatics and normal lymph trunks in the flap. Although regeneration of lymphatics can occur, it usually follows relatively minor incisions when the gap between the divided lymphatics is small. Delayed healing of the flap or infection reduces re-generation of the lymphatics. Intraabdomonal complications can sometimes occur with harvesting of omental flaps intraabdominal complications from harvesting can occur. [5] Because the incidence of complications is high and the results have been inconsistent, lymphatic bridging is not recommended. [1],[2],[3],[4],[5],[6],[7]
(ii) Drainage into deep lymphatics
Drainage from the superficial into the deep lymphatics has been attempted to establish an anastomosis between the superficial blocked lymphatics and the deep normal lymphatics. Because the primary abnormality may be in the deep lymphatics, in that case it is unlikely that the dermal wick operation will drain the lymph from the superficial system to a blocked deep system. Sawhney, evaluating the procedure using radioactive serum albumin uptake (RISA), found no increase in lymph drainage, and the initial reduction in size was proportional to the excision of edematous tissue. This reduction was not maintained at follow-up examinations.[8] These procedures are no longer recommended.
(iii) Lymph node- venous anastomosis
Anastomosis of the transected lymph node to the vein was first carried out by Olszewski and Neilubowicz (1986), who demonstrated patency of the anastomosis.[9] Calnan and associates obtained 100 percent patency at one month, but all anastomosis were obstructed at 3 months. [10] The technique is most useful in filarial lymphedema, and the results are better when, more than one anastomoses are done [Figure - 2].
(iv) Microlymphaticovenous anastomosis
Lymphaticovenous anastomosis (LVA) is preferred to lymph node-venous anastomosis (I.NVA) because the latter is not possible when the draining regional nodes have been excised. In addition, better patency is obtained by LVA (11). The prime determining factor in the success of the anastomosis is the achievement of correct apposition of the cut edges of the vessels, which depends directly on the number and spacing of the sutures and on the tension applied when they are tied. Huing et al (1986) observed that lymphatic pressure is higher than venous pressure normally, and it is even higher during muscular activity and in lymphedematous limbs. Because muscular activity increases lymphatic pressure without increasing venous pressure, lymph should drain through the anastomoses into the venous system. It was also observed a fluctuation in lymphatic pressure correlated with rhythmic contraction of the lymphatics. The authors concluded that lymphatic flow depends principally on spontaneous intrinsic contraction of the lymphatics.[12] Lymph flow is obvious under anesthesia. It has also been observed clinically that patients who have good results from surgical treatment do not have a relapse of lymphedema.
Excisional operation
Excisional procedures are indicated in primary lymphedema and in secondary lymphedema when the lymphatic trunks are sclerosed and unsuitable for micro-lymphatic anastomoses or when conservative measures have failed or are not applicable.
Total subcutaneous excision
Originally described by Charles in 1912 and commonly used since then, total subcutaneous excision is an extensive procedure that removes all of the skin, subcutaneous tissue (except in the foot and region overlying the calcaneal tendon), and deep fascia, covering the bare muscle with split or full-thickness skin grafts.[13] Although split-thickness grafts are technically easier and initially appear satisfactory, late scarring is marked and the grafts are likely to be injured easily, ulcerate frequently, and commonly develop a severe hyperkeratotic, weeping, chronically infected dermatitis.
On the other hand, full-thickness grafts taken from the excised tissue are considerably more durable although it is admittedly a formidable technical challenge to achieve a complete take of full-thickness grafts over such a large area.[14],[15],[16] Moreover, if a problem with graft vascularization arises, the situation is exacerbated and morbidity is prolonged. Even when the grafts are successful, substantial scarring and chronic breakdown of these areas are not uncommon. With chronic, long-standing lymphedema, however, where there is a substantial element of fibrosis, this procedure may be the only technically feasible option.
Management of filarial hydrocele
Surgery remains the only therapeutic choice for hydroceles. Children with Hydrocele can usually be followed till the age of one year and failing spontaneous resolution should undergo herniotomy. It is important in this age group to differentiate this condition from a hernia which needs early management to prevent complications. In adults small hydroceles can usually be managed by Lord's plication or fenestration procedures in which can be safely done under local anaesthesia. Very large hydroceles generally require some form of regional or general anaesthesia. Various procedures routinely done for such cases are eversion of sac, excision of sac or sometimes a combination of above two. Aspiration and use of sclerotherapy is to be condemmened as it has high chances of recurrence, inflammation, infection, infertility and even leading to orchiectomy. It can however be used in carefully selected patients because of age and other comorbidities making them unfit for anaesthesia and surgery. [17]
Management of vesicles (Lymphorea)
Lymphorea is relatively a complex condition and can be managed by constructing bilateral nodovenous shunts and cauterization or total excision of scrotum with some form of plastic surgical reconstruction or reimplantation of testes.
Epididymo-orchitis
Presenting with pain and inflamed scotum can usually be managed with antibiotics, analgesics, anti-inflammatory, antifilarial agents and scrotal support.
Ramhorn penis [Figure - 3]A
Presenting as severely malformed penis posing difficulty in micturation and sexual function can be managed by bilateral nodovenous shunts and circumcision. If not responding then by excision of skin and thickened underlying tissue followed by split skin grafting [Figure - 3]B.
Vulval oedema [Figure - 4]A
Similar to the condition of Ramhorn penis in males interferes with micturation and sexual function along with difficulty in maintaining a local hygiene. It initially requires creation of bilateral nodovenous shunts follwed by excision and primary suturing and in severe cases not resolving may require excision and split skin grafting or a local flap cover which provides a local cover and helps in improving lymph drainage [Figure - 4]B and C.
Complications
Like any surgical procedure, surgery for lymphoedema especially due to filariasis has its own complications due to poor wound healing and dehiscence. There is also a higher incidence of infection and lymphorea due to poor lymph drainage by the obstructed lymphatics.
| Conclusion | | |
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 microlymphatic operations combined with conservative measures to relieve many of these patients of much of their swelling without resort to lifetime use of conservative measures. Additional work is required to increase understanding of the mechanism of lymphatic transport before a perfect solution can be found.
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| 13. | Charles RH. A System of Treatment. Vol. 3. London: Churchill, 1912;504. |
| 14. | McKee DM, Edgerton MT. Surgical treatment of lymphedema of the lower extremities. Plast Reeonst Surg 1959;23:480. |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
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