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).
 Table
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).
Table
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).
Table
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).
REFERENCES
1.
Roesler R, Araujo LRR, Rippel R, et al. Doença de Meige (linfedema
precoce) - relato de caso e revisão da literatura. Rev Soc Bras
Cir Plast 1999;14:69-78.
2.
Guedes HJ, Andrade MFC. Diagnóstico e tratamento do linfedema
periférico. Cir Vasc Angiol 1996;12:6265.
3. Guedes Neto HJ. Linfedemas - Classificação,
Etiologia, Quadro Clínico e Tratamento não Cirúrgico.
In: Brito CJ. Cirurgia Vascular. 1ª ed. Rio de Janeiro: Revinter;
2002. p. 1228-35.
4. Kinmonth JB, Wolfe JN. Fibrosis in the lymph nodes
in primary lymphedema. Ann R Coll Surg Engl 1980;62:344-54.
5.
Cordeiro AK, Baracat FF. Linfologia. São Paulo: Byk - Procienx;
1983.
6.
Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable
site for development of neoplasms. J Am Acad Dermatol 2002;47:124-7.
7. Thomaz JB, Herdey ADC. Fundamentos da Cirurgia Vascular
e Angiologia. 1ª ed. São Paulo: Fundo Editorial BYK; 1997.
p. 581-597.
8. Kinmonth JB, Wolfe JN. Fibrosis in the lymph nodes
in primary lymphedema. Ann R Coll Surg Engl 1980;62:344-54.
9. Isselbacher KJ, Braunwald E, Wilson JD, Martin JB,
Fauci AS, Kasper DL. Medicina Interna Harrison. 12º ed. Rio de
Janeiro: Guanabara Koogan; 1992.
10.
Bernas MJ, Witte CL, Witte MH. The diagnosis and treatment of peripheral
lymphedema: draft revision of the 1995 Consensus Document of the International
Society of Lymphology Executive Committee for discussion at the September
3-7, 2001, XVIII International Congress of Lymphology in Genova, Italy.
Lymphology 2001;34:84-91.
11.
Kinmonth JB, Taylor GW, Tracy GD, Marsh JD. Primary lymphoedema. Br J Surg 1957;45:1-10.
12.
Cambria RA, Gloviczki P, Naessens JM, Wahner HW. Noninvasive evaluation
of the lymphatic system with lymphocintigraphy: a prospective, semiquantitative
analysis in 386 extremities. J Vasc Surg 1993;18:773-82.
13.
Collins OS, Villavicencio JL, Gómez ER, et al. Abnormalities
of lymphatic drainage in lower extremities: a lymphocintigraphic study.
J Vasc Surg 1989;9:145-52.
14.
Cestari SCP, Petri V, Castiglioni MLV, Lederman H. Linfedema dos membros
inferiores: estudo linfocintilográfico. Rev Assoc Med Bras 1994;40:93-100.
15.
Andrade MFC. Linfedema: epidemiologia, classificação e
fisiopatologia. In: Maffei FHA, Lastoria S, Yoshida WB, Rollo HA, editores.
Doenças vasculares periféricas. 3ª ed. São
Paulo: Medsi; 2002. p. 1641-6.
16.
Kafejian-Haddad AP, Galego SJ, Correa JA, et al. Linfedema - Análise
inicial dos pacientes acompanhados no ambulatório de Linfologia
da disciplina de angiologia e cirurgia vascular da Faculdade de Medicina
do ABC. Arquivos Médicos do ABC 2003;28(1):41-9.
17.
Smith RD, Spittell JA, Schirger A. Secondary lymphedema of the leg:
its characteristics and diagnostic implications. JAMA 1963;185:80-2.
18.
Herd-Smith A, Russo A, Muraca MG, Turco MR, Cardona G. Prognostic factors for lymphedema after primary treatment of breast carcinoma. Cancer 2001;92:1783-7.
19.
Lobb AW, Harkins HN. Postmastectomy swelling of the arm with note on
effect of segmental resection of axillary vein of radical mastectomy.
West J Surg Obstet Gynecol 1949;57:550-7.
20.
Kissin MW, Querci della Rovere G, Easton D, Westbury G. Risk of lymphoedema
following the treatment of breast cancer. Br J Surg 1986;73:580-4.
21.
Milroy WF. Chronic hereditary edema: Milroy's disease. JAMA 1928;91-1172.
|
