An
ethiological study of lymphedema based upon the Kinmonth classification
as modified by Cordeiro
(Portuguese
PDF version)
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: drguedes@drguedes.med.br
J Vasc
Br 2004;3(1):60-4
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, 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).
The types of lymphedema that are of greatest interest to a vascular
surgeon are those affecting the upper or lower limbs, the face and the
penis and scrotum.1
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.
METHODS
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.
RESULTS
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.
DISCUSSION
The lymphatic
system is made up of numerous superficial lymph vessels which follow
the veins and perform lymphatic drainage to superficial and (less common)
deep lymph nodes .
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 .
CONCLUSIONS
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.
REFERENCES
1.
Guedes Neto HJ. Surgical treatment of penile-scrotal lymphedema. Lymphology
1997;29(3):132-3.
2. Board J, Harlow W. Lymphoedema 2: classification,
signs, symptoms and diagnosis. Br J Nurs 2002;11:389.
3. Szuba A, Rockson SG. Lymphedema: classification,
diagnosis and therapy. Vas Med 1998;3:145-56.
4. Griton P. Clinical aspects and etiology of lymphedema
of the lower limbs in adults. Phlebologie 1988;41:325-54.
5. Lazareth I. Classification of lymphedema. Rev Med
Interne Suppl 2002;3:375-8.
6. Miller AJ, Bruna J, Beninson J. A universally applicable
clinical classification of lymphedema. Angiology 1999;50:189-92.
7. Baracat FF, Cordeiro AK. Linfologia. São Paulo:
Byxk-Procienx; 1983.
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.
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