Analysis of patients with lymphedema at public institution
(Portuguese PDF version)

Andréa Paula Kafejian-Haddad,1 Fernanda Alves Sanjar,2 Juliana Hiratsuka2, Priscila Gagliardi Kalil Debs2, Marcela Bianca Resende Fernandes2, Ohannes Kafejian3

1. Physician. Assistant professor and Head of the Service of Lymphology, Angiology and Vascular Surgery, Faculdade de Medicina do ABC (FMABC), Santo André, SP, Brazil.
2. Undergraduate student, FMABC, Santo André, SP, Brazil.
3. Professor, Angiology and Vascular Surgery, FMABC, Santo André, SP, Brazil.

Correspondence:
Andréa Paula Kafejian-Haddad
Rua Bom Pastor, 1238
CEP 04203-001 - Ipiranga, SP, Brazil
E-mail: apkh@uol.com.br


ABSTRACT

Objective: To study the incidence and distribution of lymphedema concerning etiology and clinical forms in patients followed-up at the Service of Lymphology of the Angiology and Vascular Surgery Course at the Faculdade de Medicina do ABC.

Method: 115 patients with lymphedema were assessed and classified as to etiology according to Kinmoth's classification, site involvement, evolution time, and clinical form of the disease according to Mowlem's classification.

Results: In our analysis, 43.48% of patients presented primary lymphedema, 56.25% presented secondary lymphedema, which was more frequent as a consequence of erysipelas. Most patients were female (79.14%).

Conclusions: In our study, lymphedema was predominant in female patients and lower limbs were the most affected. Regarding the clinical form, Mowlem type II prevailed. Among the causes for secondary lymphedema, the most frequent was the postinfectious. Outpatient follow-up offers a better assessment of patients with lymphedema in order to control the disease evolution.

Key-words: lymphedema, lower limb, lymphatic system.

J Vasc Br 2005;4(1):55-8


Lymphedema is a chronic disease characterized by the accumulation of interstitial fluid with a high protein concentration, a consequence of the lymphatic drainage insufficiency due to congenital abnormalities or acquired from the lymphatic system.1-7 It usually affects the extremities, especially the lower limbs.2-5,7-9

The edema is discrete, soft and pitting at its beginning, but, as the disease evolves, it becomes hard and non-pitting.2,5,9 Chronically, this volume increase in the limb can cause significant deformities, invalidity and lymphostatic elephantiasis; besides, it can become malignant.2,3,8

Lymphedema can be classified into primary and secondary, according to Kinmoth's classification.1-3,5,7,9-11 Primary lymphedema occurs due to congenital alterations, such as: agenesis, hypoplasia or hyperplasia of the lymphatic vessels, and it can be subdivided into congenital, early and late.3,5,7,9 In the secondary lymphedema, there are alterations acquired from the lymphatic system due to traumas, infections, surgeries, radiotherapy, chronic venous insufficiency, among others.1-3,10

The lymphedema diagnosis is basically clinical; complementary exams can be used for eliminating edemas of other etiologies, for diagnostic confirmation and for determining the involvement level of the lymphatic vessels.2-5,7,9,12,13 The choice examination for assessing the lymphatic system is currently the lymphoscintigraphy, which allows an anatomical and functional assessment.2-5,7,9,12-14

The exact prevalence of the lymphatic disorders has not been well defined in the literature due to rare epidemiological cases, although its occurrence is considered widely underestimated in the clinical practice. It is estimated that there are 450 million people with lymphatic disorders, that is, 15% of the world population.15 In Brazil, the incidence and distribution of the lymphedema is little known. We have not found in the literature a study assessing the disease incidence in our environment.

The objective of this study is to analyze the number of patients receiving care in our Service of Lymphology from January, 2000 to May, 2003, describing factor related to the disease etiology and clinical form, gender, age, and site involvement of the lymphedema.

METHOD

From January, 2000 to May, 2003, 115 patients with lymphedema were retrospectively assessed in the Service of Lymphology of the Angiology and Vascular Surgery Course at the Faculdade de Medicina do ABC, receiving care at the Hospital Mário Covas and at the Hospital Anchieta.

The study was performed through a survey of medical charts and specific forms for patients with lymphedema. These were filled in on the first day of the patient's assessment at the Service. Thus, it was possible to analyze age group, topography, etiology of the lymphedema, evolution time, and clinical involvement level of the disease.

The lymphedema was classified into primary, when the patients presented a history of disease occurrence with no triggering factor; and secondary, when related to several causes (post-episodes of repeated infection, post-surgeries, post-radiotherapy, post-trauma, post-chronic venous insufficiency, among others).1-3

Concerning the clinical form, we used Mowlem's3 classification:

  • STAGE I: reversible lymphedema with elevation of the limb and rest in bed for 24-48 hours: pitting edema with pressure.
  • STAGE II: irreversible lymphedema even with prolonged rest; moderate to severe fibrosis and non-pitting edema with pressure.
  • STAGE III: irreversible lymphedema with severe fibrosis in the subcutaneous tissue and lymphostatic elephantiasis in the limb.

Data were tabled for assessment.

RESULTS

115 patients were assessed, 91 (79.14%) female and 24 (20.86%) male. Ages varied from 4 to 86 years old (mean of 46.84 years old). Of these patients, 50 (43.48%) had primary lymphedema and 65 (56.52%) had secondary lymphedema.

Concerning the clinical form of the patients with primary lymphedema, three (6%) were stage I, 41 (82%) were stage II, and six (12%) were stage III. Concerning the patients with secondary lymphedema, six (9.23%) were stage I, 46 (70.77%) were stage II, and 13 (20%) were stage III (Table 1).

click hereTable 1 - Sample characterization

Lymphedema n of patients M F Mean age Stage I Stage II Stage III Mean evolution time
Primary 50 10 40 15.35 3 41 6 14.12 years old
Secondary 65 12 53 52.3 6 46 13 8.92 years old

 

Concerning the age at disease manifestation, among the patients with primary lymphedema, five (9.76%) were congenital, 21 (39.02%) were early, and 24 (51.22%) were late. They were also separately assessed as to clinical form (Table 2).

click hereTable 2 - Analysis of patients with primary lymphedema

  n of patients Stage I Stage II Stage III
Congenital 5 --- 4 1
Early 22 1 17 4
Late 23 2 20 1

 

Among the patients with secondary lymphedema, the triggering causes were post-infectious in 37 cases (56.92%); chronic venous insufficiency (CVI) in six cases (9.23%); surgery in seven cases (10.77%); trauma in eight cases (12.31%), and surgery and radiotherapy in seven cases (10.77%). The clinical form was also assessed separately (Table 3).

click hereTable 3 - Analysis of patients with secondary lymphedema

  n of patients M F Stage I Stage II Stage III
Erysipelas 37 11 26 3 25 9
Trauma 8 2 7 --- 5 3
CVI 6 1 4 2 4 ---
Surgery 7 --- 7 1 5 1
Surgery+Radiotherapy 7 --- 7 --- 7 ---

 

Concerning the topography of the disease, most patients, 64 (55.65%) presented bilateral lymphedema of the lower limbs; 44 patients (38.27%) presented unilateral involvement of the lower limb, and seven patients (6.08%) presented unilateral lymphedema of the upper limb.

DISCUSSION

The frequency of lymphopathies is much lower than arteriopathies and phlebopathies.3 However, it is not an uncommon disease. It is essential to know the incidence and the forms of treatment of the lymphedema for an early action, since if not correctly conducted, it can bring several sequels, making it impossible for the patient to lead a normal life.

The Service of Lymphology at the Faculdade de Medicina do ABC, which started operating in January, 2000 has shown that the number of patients with lymphedema in our environment is higher than the predicted number. Patients from our study were having an exclusive follow-up at the Lymphology outpatient clinic. According to previous data, from January, 2000 to May, 2002, the number of patients having a regular follow-up was 53.16 After only 1 year, the number of patients increased to 115.

We believe that this increase in the total of patients is due to the fact that our Service is constantly expanding; the medical care was increased with the inclusion of the Hospital Mário Covas. The lack of specific medical care to patients with lymphedema in the ABC region has also contributed to a higher number of referred patients to our Service, where we provide specialized medical follow-up and specific physiotherapy treatment.

In the present study, the incidence of women with primary lymphedema was high (79.14%). This prevalence had already been observed in our previous study,16 as well as in cases reported in the literature.3

We observed a higher prevalence of secondary lymphedema (56.52%) compared to primary lymphedema. In the study performed at the same institution 1 year ago, when casuistics was only 53 patients, the primary lymphedema was predominant (54.71%). Such change concerning etiology may have occurred only due to the higher number of patients who sought our Service.

In our environment, the infection (post-erysipelas) is considered the most frequent cause of secondary lymphedema.3 In Europe, bacterial and fungal infections represent less frequent etiologic factors.17 In our study, most patients with secondary lymphedema (56.92%) presented a previous infection of the limb.

The oncologic treatment for breast neoplasia, surgery and/or radiotherapy can lead to the lymphedema of the upper limb.6,7,9,18 In the USA and Europe, the most frequent cause of secondary lymphedema is the post-oncologic treatment for breast neoplasia.19,20 In our study, only seven patients had a lymphedema of the upper limb due to post-oncologic treatment for breast neoplasia. The small number of cases found in our study is due to the fact that the outpatient clinic is still recent. We believe that making the clinic more known will increase this demand.

Concerning other causes of secondary lymphedema with lower incidence in our study, there is the chronic venous insufficiency, which was responsible for 9.23% of cases and trauma, accounting for 12.31%. In Europe, such causes are also less frequent.21

Only the awareness of the necessity of early diagnosis and treatment of the lymphedema in the medical environment will improve the quality of life of the affected patients.

Other studies on the incidence of lymphedema will always be necessary, as well as a continuous up-to-dating of this study, in order to detect the real incidence of patients with lymphedema in our environment.

CONCLUSION

The present study of 115 patients with lymphedema in the extremities allowed us to conclude that the secondary lymphedema was predominant related to the primary lymphedema; lymphedema stage II, both in patients with primary and secondary lymphedema, was predominant and the lower limbs were the most affected; there was a higher incidence of female patients; the most frequent cause of secondary lymphedema was the post-infection (erysipelas).

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