An etiological study of lymphedema based upon the Kinmonth classification as modified by Cordeiro.
Lymphedema is a diffused edema of a specific region of the body and whose etiology is a dysfunction of the superficial lymphatic system and, in some rare cases, also affects the deep lymphatic system. As a result of lymphatic system overload, glossary:protein|proteins]] and liquids accumulate within subcutaneous cellular tissues causing them to increase in size. Weight is also increased and function and aesthetic appearance are impaired.
Complications resulting from lymphedema are fibrosis edema (a hardening of the skin and subcutaneous tissue due to high protein concentrations), repeat lymphangitis and/or erysipelas and lymphangiosarcoma (a malignant neoplasm).
Despite the large number of classification systems currently in existence and despite the fact that many vascular surgeons have adopted clinical criteria, our clinic has adopted the Kinmonth classification, as modified by Cordeiro, which groups patients and treatments according to lymphedema etiology, because of its practicality and scope.2-8
The Kinmonth classification defines lymphedema as either primary or secondary. Primary lymphedema may be congenital and is defined as praecox when the first manifestation occurs before 15 years and tarda when it appears after this age. Secondary lymphatic edema can be post-infectious, post-surgical, post-tuberculous, neoplastic, filarial, post-radiotherapeutic, post-traumatic, post-phlebitic or due to chylous reflux.9
The objective of this study was to investigate the most common forms of lymphedema at the Lymphedema and Angiodysplasia Clinic of the Santa Casa de Misericórdia in São Paulo.
This is a retrospective study of the period between January 1997 and June 2003. Patients were cataloged from medical records completed at the Vascular Surgery Clinic at the Santa Casa de Misericórdia in São Paulo.
Patients were classified according to criteria proposed by Kinmonth and Cordeiro.
The lymphedema protocol created for this study contained the following items: date of admission, patient name, date of birth, profession, race, weight, origin, onset of symptoms, infectious episodes, questions on different medical apparatus, personal and family background.
The physical examination identified the affected member (upper or lower, left, right or bilateral), checked for associated injury and recorded limb measurements.
A total of 229 lymphedema patients were studied, of whom 149 (65%) had secondary lymphedema and just 80 (35%) presented with primary lymphedema (Figure 1).
Figure 1 - Overall lymphedema distribution.
An analysis of primary lymphedema cases demonstrated the following distribution: 14 had congenital lymphatic edema (i.e. 17.5%), 27 had praecox (33.7%) and 39 had tarda (48.8%) (Figure 2).
Figure 2 - Primary lymphedema distribution.
Analysis of the secondary lymphedema patients revealed that 102 cases (68.5%) were post-infectious, 24 (16.1%) were post-surgical, in 12 cases (8%) the lymphedema was the result of chylous reflux (penile-scrotal), five patients (3.3%) exhibited post-neoplastic lymphedema, four cases (2.7%) were post-traumatic and there was one case (0.7%) each of post-phlebitic and post-filarial lymphedema (Figure 3).
Figure 3 - Secondary lymphedema distribution.
An overall analysis of lymphatic edema cases treated at the Vascular Surgery Clinic at the Santa Casa de Misericórdia in São Paulo revealed the following incidence: 102 post-infectious secondary lymphedema patients (44.5%), 39 (17,1%) with primary lymphedema tarda, 27 (11.8%) primary lymphedema praecox cases, 24 (10.4%) secondary post-surgical lymphedema patients, 14 (6.1%) cases of congenital primary lymphedema, 12 (5.2%) patients whose secondary lymphedema was due to chylous reflux (penile-scrotal) and a further 11 (4.9%) cases that were due to other causes (Figure 4).
Figure 4 - Specific lymphedema distribution.
The function of the lymphatic system is to reabsorb plasma proteins which escape from the capillary bed and to transport liquids. It also processes cellular excretions, removes organic particles, absorbs fats, eliminates mutated cells and produces lymphocytes.
Lymph vessels begin as blind-ended tubes, located in the interstitium. These drain into the pre-lymphatic system, formed by the pre-collectors, which, in turn drain to blood vessels or lymphatic capillaries. Lymphatic capillaries have the same structure as pre-collectors, although they have fewer open junctions and more intra-lymphatic valves. Lymphatic capillaries then drain into lymph nodes, where open junctions cease, the middle smooth muscle layer appears and intra-lymphatic valves become more frequent. The adventitial layer also appears. From the lymph nodes, the lymph proceeds via the lymph ducts and returns to the circulation.
The lymphatic system is responsible for the return of just 10% of fluids to the heart, while the remaining 90% is left to the venous system. The 10% that does pass through the lymphatic system is between 2 and 4 liters per day.10
Lymphedema is the result of a deficiency in the lymphatic transport and of extra lymphatic proteolysis insufficiency. It may be the result of high output when the lymphatic load is heavy and its transport capacity is passed. Lymphedema may also be low output when lymphatic load is normal, but there is reduced transport capacity (interstitial protein concentrations are also elevated), or be “mixed”, resulting from “safety valve insufficiency” when lymphatic load is high and transport capacity is reduced.
It is important to take into account the fact that while the lymphatic injury may be anchored in a certain point in time, the lymphedema itself may take some time to appear, years even. This is because the capillaries located in the interstitial space, which are fine and numerous, have the capacity to become lymphatic plexuses, increasing in size and thus avoiding lymphatic stasis. This is known as lymphatic reserve. With the passage of time, proteins accumulating in the subcutaneous cellular tissues obstruct the lymphatic plexuses, resulting in imbalances within the system and the onset of manifest lymphatic edema.
The mention of lymphedema in the presence of doctors from other specializations elicits stereotypical images of the disease such as the mastectomy patient who presents lymphedema of an upper limb or the patient with elephantiasis of a lower member who lives in a filariasis-endemic area.
The vascular surgeons that receive these patients, however, observe that the most common etiologies are other and that the means of treating and preventing them are different and can have significant effects on improving the quality of life of these patients.
In this study we found that the most common form of lymphedema was secondary post-infectious, which affects patients from lower social classes who have greater difficulty accessing medical services and who, having had erysipelas episodes, were treated unsatisfactorily and badly advised in terms of the care needed to prevent lymphedema developing.
We also observed changes in the frequency of lymphedema types in relation to an earlier study published by our group. In that study secondary, post-surgical lymphedema was the second-most common and primary lymphedema tarda was third. In the current research, primary lymphedema tarda was the second-most common, followed by primary lymphedema praecox while secondary post-surgical lymphedema was fourth.
We credit this change to two factors: the first is that our group has become better-known for lymphedema treatment, which has resulted in an increase in referrals to our clinic of patients with chronic edema with onset during childhood or adolescence without apparent cause and which have been resistant to a number of different treatments; the second is related to the tendency towards reducing the number of radical mastectomies performed for breast cancer treatment in the face of their not having been demonstrated to be superior at controlling cancer than more economical resections.11
Another lymphedema etiology that has become more frequent is penile-scrotal, reaching sixth-most common at 5.2% of cases. We believe that this too is the result of publishing the results of our treatment of this lymphedema manifestation.12
It is worth pointing out that the least common situation in which lymphedema presented was post-filariasis. This was observed both in our research and in a study performed in a filariasis-endemic area by the Recife group (PE).13
Lymphedema, once developed, is a chronic disease leading to patient discomfort due to the increased weight of the member involved and to aesthetic abnormalities, which may result in social isolation. Diagnosis is made based on clinical history and physical examination and may be complemented with imaging studies such as duplex scanning, which can rule out venous thrombosis which may provoke edema, and lymphoscintigraphy, which can define the degree to which lymphatic return is compromised and, sometimes even the point of obstruction.
Lymphatic edema can be controlled by means of combined physiotherapy (CPT), which makes the affected member return to a size approximating its original dimensions, although never identical. The remission period of this disease depends on the etiology of the lymphedema, on the degree of lymphatic involvement, on the initial size of the edema, on control of factors aggressive to the lymphatic system, on obesity, on associated conditions, and on the patient's level of physical activity and eating habits.14
There are also certain drugs which promote extra-lymphatic proteolysis and aid CPTY to “cure” lymphatic edema. It should be remembered that diuretics do not aid in the reduction of lymphedema.
The treatment of choice for penile-scrotal lymphedema is a cosmetic resection of skin and subcutaneous cellular tissue from the region, giving good results. There are also surgical indications for other types of lymphedema when there is excessive skin and when subcutaneous cellular tissues have not exhibited lymphangitis crises for at least 1 year.15
It is important to explain to the patient that, while there is no true cure for this disease in terms of edema reduction, TFC should be performed in order to avoid secondary lymphedema complications.
Lymphedema is a chronic and irreversible disease with significant negative consequences for the patient since the aesthetic deformities which result may make it impossible to continue their career or lead to social isolation. Furthermore, treatment is highly time-consuming for both patient and doctor who are in search of uncertain, and sometimes temporary, results.
This being the case we believe that the best way to treat lymphatic edema is by averting its development. In order to do this it is necessary to precisely delimit the disease's trigger-factors.
The observed distribution of lymphedema at the Vascular Surgery Clinic of the Santa Casa de Misericórdia in São Paulo, as described in this paper, are exactly in agreement with data found in global literature with secondary lymphedema more common than primary.
Secondary post-infectious lymphatic edema was the most common among our patient sample (44.5%), followed by primary tarda (17.1%), primary praecox (11.8%) and secondary post-surgical (10.4%).
Prophylaxis therefore, along with better erysipelas treatment, particularly of the first episode and for patients who present frequently could contribute to a reduction in secondary lymphedema in our country.
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8. Cordeiro AK, Bacarat FF. Etiologia e Patogenia. In: Linfologia. São Paulo: Byxk-Procienx; 1983. p. 81-5.
9. Guedes Neto HJ. Linfedemas - classificação, etiologia, quadro clínico e tratamento não cirúrgico. In: Brito CJ. Cirurgia Vascular. Rio de Janeiro: Revinter; 2002. p. 1228-35.
10. Schirger A, Peterson LFA. Lymphedema. In: Allen E, editor. Allen, Barker, Hines Peripheral Vascular diseases. Philadelphia: W. B. Saunders Co.; 1972. p. 635-55.
11. Guedes Neto HJ. Post mastectomy lymphedema: an epidemiological study. In: Proceedings of the 15th International Congress of Lymphology. Recife - São Paulo, Brazil, 1995. p. 285-8.
12. Guedes Neto HJ. Surgical treatment of penoscrotal lymphedema. In: Proceedings of the 14th International Congress of Lymphology. Washington, DC, USA, 3: 821-4.
13. Carvalho A, Gomes S, Neves F. Filariose Linfática - Aspecto médico-social e estatístico da amostragem da angioclínica - Recife. I Congresso do Capítulo Latino Americano da União-Internacional de Angiologia; 9 a 12 de abril de 2003; Belo Horizonte, MG.
14. Guyton AC, Hall JE. A microcirculação e o sistema linfático: trocas de líquidos no capilar, líquido intersticial e fluxo de linfa. In: Guyton AC, editor. Tratado de Fisiologia Médica. Rio de Janeiro: Guanabara; 1996. p. 167-78.
15. Guedes Neto HJ. Surgical Treatment of penoscrotal lymphedema. In: Proceedings of the 15th International Congress of Lymphology. Recife - São Paulo, Brazil, 1995. p. 354-8.
Henrique Jorge Guedes Neto1, Fernando Tavares Saliture Neto2, Roberto Feres Júnior2, Valter Castelli Júnior3, Roberto Augusto Caffaro4
1. PhD. Assistant professor, Department of Surgery, School of Medicine, Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil. 2. Resident physician, Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil. 3. PhD. Associate professor, Department of Surgery, School of Medicine, Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil. 4. PhD. Chief of the Course of Vascular Surgery, Department of Surgery, School of Medicine, Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil.
Correspondence: Henrique Jorge Guedes Neto Avenida Angélica, 688/304 CEP 01228-000 - São Paulo - SP Brazil Tel.: +55 (11) 9943.2502 E-mail: email@example.com
J Vasc Br 2004;3(1):60-4
Lymphedema results from the inability of the lymphatic system to transport lymph fluid. Approximately 80% of lymphatic drainage must be nonfunctional before lymphedema becomes clinically evident.  Abnormal lymph flow may result from lymphatic hypoplasia, obstruction, or fibrosis. Cancer treatment (especially breast cancer treatment), malignancy, nematode infection (filariasis), and trauma are responsible for >90% of cases worldwide. 
Lymph node dissection, radiation therapy, or neoplastic infiltration can injure the lymphatic system (e.g., pelvic masses or node dissection can lead to lymphedema of the lower extremity or genitalia).  Parasitic nematodes, such as Wuchereria bancrofti and Brigia malayi, spread by a mosquito vector cause lymphatic filariasis by obstructing lymphatic channels directly or by regional inflammation.  As many as 120 million people in mosquito-plagued regions develop lymphedema as a result of nematode infection.   Significant penetrating trauma, particularly to the axilla or groin, can cause damage to the lymphatic system with consequent lymphedema. In addition, curvilinear scars may retain lymph fluid, resulting in a raised, swollen area and scar lymphedema. 
Primary lymphedema is likely to account for less than 1% patients with lymphedema and is usually due to hypoplastic lymphatic development.   Most cases are sporadic; however, approximately 10% to 15% of patients have familial or syndromic lymphedema.  A causative mutation has been identified in some lymphedema-associated sydromes: Milroy disease (VEGFR3), lymphedema-distichiasis syndrome (FOXC2), hypotrichosis-lymphedema-telangiectasia (SOX18), and Hennekam syndrome (CCBE1).     
Autosomal-dominant inheritance is most common (e.g., Milroy disease, Meige disease, lymphedema-distichiasis syndrome),    but autosomal-recessive inheritance can also occur (e.g., hypotrichosis-lymphedema-telangiectasia, Hennekam syndrome).   Several other conditions (e.g., Noonan syndrome, Turner syndrome) also have an increased risk of primary lymphedema.  
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