Phlebology 2008;23:276-286
doi:10.1258/phleb.2008.008010
© 2008 Royal Society of Medicine Press
Diagnosis and therapy in children with lymphoedema
R J Damstra * and
P S Mortimer 
* Department of Dermatology, Phlebology and Lymphology, Nij Smellinghe Hospital Drachten, The Netherlands;
Department of Cardiac and Vascular Sciences (Dermatology), St George's Hospital Medical School, University of London, London, UK
Correspondence: R J Damstra MD, Department of Dermatology, Phlebology and Lymphology, Nij Smellinghe Hospital Compagnonsplein 1, 9202 NN Drachten, The Netherlands. Email: r.damstra{at}nijsmellinghe.nl
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Abstract
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Introduction: Lymphoedema (LE) is a disorder characterized by persistent swelling
caused by impaired lymphatic drainage because of various aetiologies,
including lymphatic injury and congenital functional or anatomical
defects.
Objective: Literature review and expert opinion about diagnosis and treatment of LE in children.
Results: LE is rare in children, with a prevalence of about 1.15/100,000 persons, 20 years old. The management of LE in children differs considerably from adults in terms of origin, co-morbidity and therapeutic approach. The objective of this presentation is to discuss practical issues related to clinically relevant information on the diagnosis, aetiology, work-up and treatment of LE in children. In contrast to adults, who usually experience secondary LE because of acquired lymphatic failure, most cases in children have a primary origin. The diagnosis can be made mainly on the basis of careful personal and family history, and physical examination. LE in children can be part of a syndrome if there are other concomitant phenotypic abnormalities and if a genetic defect is recognizable. Treatment of LE is mostly conservative utilizing decongestive LE therapy including compression therapy, directed exercises, massage and skincare. In the neonate, initial observation alone may be sufficient, as delayed lymphatic development and maturation can result in spontaneous improvement. The role of parents is crucial in providing the necessary input.
Conclusion: We present a review emphasizing a practical approach to treating a child with LE according to current publications and our own experience.
Key Words: lymphoedema • children • review • diagnosis • treatment
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Introduction
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The main function of the lymphatic system is to drain protein-rich
fluid, macromolecules and cells from the interstitium to lymph
collectors, then to larger collecting lymph vessels, to the
lymph nodes and eventually into the venous circulation. Lymphoedema
(LE) is defined as swelling of a part of the body because of
impairment in lymph transport capacity based on the malfunction
or malformation of the lymphatics. There is usually a pitting
component present. In general, a pitting component arises because
of imbalance between capillary filtration and lymph drainage
capacity. In many types of oedema it is because of an increased
capillary filtration and normal lymph drainage. In early forms
of LE, especially when there were no secondary changes due to
protein accumulation, a pitting component arises because of
lymphatic impairment. However, in long-lasting stages of LE,
more sustained finger pressure to the skin (of at least 20 s)
is required to demonstrate an indentation because of the firmer
and thicker nature of the skin and the subcutaneous compartment.
1 In long-lasting LE, interstitial accumulation of proteins,
2 inflammatory cells, adipose tissue hypertrophy and fibrosis
make pitting less evident.
3 Erysipelas (cellulitis) is common
and, if LE remains untreated, recurrent bouts of infection are
increasingly likely and may lead to greater morbidity. Early
recognition and diagnosis, proper treatment management and long-lasting
follow-ups are crucial for preventing progressive disease and
complications.
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Purpose of review
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The purpose of this review is to provide clinically relevant
information regarding the aetiology, diagnosis, work-up and
treatment of LE in children through a synthesis of the current
literature and our own experience.
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Classification and description of types of lymphoedema in children
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Lymphatic drainage impairment can be either congenitally determined
(primary) or by a consequence of acquired lymphatic failure
because of obstruction or damaged lymphatics (secondary). Primary
LE is considered to be due to an intrinsic or constitutional
fault in lymph drainage that can become manifest at different
ages of life depending on the underlying cause.
Although LE results from disturbances or dysfunctions in drainage routes at various levels (capillary, collector or main lymphatics levels), lymphatic malformations (LMs) may or may not interfere with lymph drainage and can produce isolated swelling (because of the malformation), and not inevitable to impairment of lymphatic transport capacity. Congenital lymphatic developmental disorders can produce LE, an LM or both. Congenital vascular malformations (CVM) may possess blood, vascular- and LMs. The Hamburg classification of CVM distinguishes two forms of LM: a truncular (LM-T) and an extratruncular (LM-ET) form.4,5 Both results from a developmental defect in lymphangiogenesis, which can be based on a mono- or polygenetic origin. In early embryogenesis, lymphangiogenesis focuses on the formation of lymph sacs and on the migration of lymphatic endothelial cells in the tissues; in later phases of embryogenesis, the differentiation and separation of blood and lymphatic vessels takes place. In the later phase, the emphasis is on the maturation of the lymphatic architecture as valve formation and smooth muscles occur within the lymphatic vessel.6 The embryological stage, at which developmental arrest occurs, determines the type of LM. Early dysadjustment in embryogenesis leads to LM-ET forms of LM, while later arrest exposes more LM-T forms.
In this classification, LM-T is known as primary LE; the LM-ET includes micro/macrocystic or cavenous lymphangioma, and are closely tied to other forms of CVM. LM can be confused with vascular malformations known as haemolymphatic or mixed vascular malformations.7 LM-T and LM-ET need a separate therapeutic approach. In this article, we will focus on primary LE (LM-T).
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Primary lymphoedema
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In children, nearly all LEs are classified as primary. LE in
children is rare, with an estimated prevalence of 1.15/100.000
persons <20 years old.
8 Genetic conditions associated with
LE are reported in frequencies varying from solitary case reports
to estimates of approximating 1/500 birth in case of Klinefelter
syndrome.
9 A study by Dale
10 of 312 index patients younger than
36 years with primary LE reported a frequency of 1/6000 at birth
and a sex ratio of one male to three females.
LE has a wide phenotypic variability, even within the hereditary forms. Usually one or two limbs are affected but other parts of the body can be involved, including visceral organs, such as the heart, lungs and intestines. In a large study from Smeltzer et al.8 analysing 291 children, only 42 (14%) had a family history of LE.
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Secondary lymphoedema
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As determined by the total numbers of patients, infectious (secondary)
LE is most frequent in tropical regions of the world. Endemic
lymphatic filiariasis is a major mosquito-borne tropical disease
that affects more than one billion people living in 83 countries.
In endemic regions, the prevalence of antigenaemia in children
aged 0–5 years is 18.9%.
11 In this review we will focus
on non-filiarial LE.
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Classification
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Classification can be based on several parameters, such as age
of onset, anatomical variations or pathophysiological phenomena.
Primary LE is traditionally classified according to the time
of onset: congenital LE, LE praecox or LE tarda. Congenital
LE is present at birth or soon thereafter. The recent discovery
of disease-causing mutations has helped to reclassify LE according
to the genotype. Other genetic forms of LE in which the genetic
fault is not known must rely on phenotypic classification. LE
can either be the only clinical sign (Milroy disease, see Figure
1)
or can sometimes be a part of a phenotypical complex of defects,
as in LE-distichiasis syndrome. In chromosomal disorders, the
defect is because of a deficiency of genes contained within
a whole chromosome or a chromosome segment, as in Klinefelter
syndrome or Turner syndrome. LE praecox presents between 1 and
35 years of age and is mostly sporadic. Familiar cases of LE
praecox are called the Meige syndrome.
In some cases, primary LE will manifest after 35 years of age.
This is known as LE tarda. This type of primary LE is a developmental
abnormality of the lymphatic system, which becomes clinically
evident only after 35 years of age and is often precipitated
by trauma, an inflammatory event or a physical immobility.
12 Presumably, a pre-existing covert constitutional weakness in
lymph drainage is exposed by an extrinsic event, like infection.
The frequencies of the various types of primary LE vary:13,14 congenital LE (onset <1 year after birth) occurs in 6–12%, LE praecox (onset between 1 and 35 years) occurs in 77–94% and LE tarda frequencies of 11% of primary LE patients are reported.
An anatomical classification using lymphoscintigraphy into aplasia, hypoplasia or hyperplasia does not lead to a change in attitude according to diagnosis and treatment and will burden a child unnecessarily.
Pathophysiological classification depending on origin of the lymphatic impairment can be useful in order to make a therapeutic plan. Lymphatic impairment can be classified as high- and low- out insufficiency (dynamic/static insufficiency) depending on the factor that if there is an overload of the lymphatics (e.g. infection, cardiac failure) or an impairment of the transport capacity of the lymphatic system (‘true LE’), sometimes combination of both types can be observed. Several types of primary LE are categorized in Table 1.
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Milroy disease
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Persistent, painless swelling, typically of one (or both) lower
limb(s), is usually the first sign of LE. Swelling is often
present at birth or soon afterwards. Due to the accumulation
of protein-rich interstitial fluid, thickening of the skin over
the digits occurs. This phenomenon at the second toe was originally
described by Kaposi, but was reinvented by the French phlebologist
Stemmer and is therefore called a positive Stemmer sign when
there is an inability to pinch up a fold of skin between the
second and third toe on the dorsum of the foot. This sign is
a sensitive test for LE that does not give false positive findings.
15
When the feet are swollen, discrete features in the foot such as swollen toes, upturned nails and non-pitting oedema will be observed.
In a study of 71 patients from 10 families with Milroy disease and VEGFR3 mutations, Brice et al.23 described an onset of LE at birth in 97% of the patients; distal oedema (94%), bilateral (85%), unilateral (14%), hydrocèle in males (37%), prominent veins (23%), cellulitis (20%) and ‘ski jump’ nails (14%) were other phenotypic features that Brice et al. described.
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Lymphoedema-distichiasis
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In patients with LE-distichiasis syndrome and a FOXC2 mutation,
LE usually develops in late childhood, but onset can be delayed
well into adulthood. A failure of differentiation of eyelid
follicles into the Meibomian glands results in distichiasis
(a double row of eyelashes) that leads to eyelash irritation
of the cornea. Other major complications that have been reported
are cardiac defects, cleft palate defects, extradural cysts,
photophobia and other ophthalmic complications.
16 Petrova
et al.17 first reported that FOXC2 is essential for the morphogenesis
of lymphatic valves in FOCC2 –/– mice, which explains
the typical clinical features of this entity. In a study from
Mellor
et al.,
18 all 18 patients with a FOXC2 mutation also
showed venous valve failure and pathological reflux in the superficial
venous system. Deep venous reflux was recorded in 14 of the
18 patients.
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Syndromal lymphoedema
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In syndromal LE, other concomitant features will be present,
such as a dysmorphic phenotype, paediatric defects or mental
disorders. Signs of visceral lymphatic impairment because of
lymphangiectasia can lead to chylothorax. Chylous reflux or
protein-losing enteropathy should be considered if there is
a failure to thrive, diarrhoea or dyspnoea. Clinical signs,
which should be closely examined, are listed in Table
2.
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Diagnosis
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Diagnosis of LE, particularly in more advanced stages can easily
be made by analysing the typical history and clinical signs.
In children, signs are often less pronounced and to determine
the type of primary LE, a full history and physical examination
is obligatory. The history focuses on the antenatal and postnatal
periods, as well as on the family pedigree. The diagnostic process
also includes the clinical investigation of relatives if hereditary
LE is suspected. Physical examination includes assessing the
whole body and looking for the extent of oedema, for cutaneous
birthmarks, for overgrowth and for systemic involvement.
In well-defined cases with suspicion of syndromal LE, paediatric consultation can be helpful to examine systemic involvement. Close follow-up is essential in order to see how the condition evolves, as swelling can resolve and new swelling can appear elsewhere. Volume measurement will not be very beneficial, because a child will grow in length, circumference of the limbs and weight. Therefore photographic documentation can be supportive in evaluating the patient.
Sometimes erysipelas (cellulitis) can be a first sign of pre-existing lymphatic impairment (Figure 2). Patients with erysipelas of unclear origin have a high risk of developing subsequent LE with persisting swelling in up to 55% of cases19 and a high recurrence rate of erysipelas in up to 30–54%.20,21
Recently, we showed that in patients with a first episode of
unilateral erysipelas without signs of LE, 79% had bilateral
lymph transport impairment.
22 Therefore, we consider more than
one attack of erysipelas in children without predisposing factors
as a strong indication of lymph transport impairment.
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Specialized diagnostic procedures
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Venous duplex scanning is easy to perform and allows for a thorough
assessment of superficial and deep venous valves. In a study
of 71 patients with Milroy disease
23 and 18 patients with LE-distichiasis
syndrome,
22 superficial venous reflux was present in 16 of 71
and in all 18, respectively. Duplex scanning can also be useful
to get an idea about the amount of interstitial fluid, visualized
by clefts and splitting in the supra facia compartment.
Performing routine imaging techniques, such as lymphoscintigraphy, magnetic resonance imaging (MRI) or computed tomography scanning is not helpful in the diagnosis of LE.4 In some rare cases, as with early non-pitting LE or with some vascular stigmata like haemangioma and asymmetric leg length, it may be necessary to determine whether the swelling is due to LE, tissue overgrowth or a vascular malformation using MRI. The extent and depth of tissue involvement may also be determined (Figure 3).
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Figure 3 Clinical signs of lymphoedema (left). Magnetic resonance imaging of transverse and longitudinal sections with pure supramuscular fascia region thickening and trabecular densities, corresponding to fibrosis and an excess of adipose tissue
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The clinical usefulness of lymphoscintigraphy depends upon scrupulous
technique and careful image evaluation.
24 For radionuclide evaluation,
Tc-99 m-labelled microcolloid particles are intradermally or
subcutaneously injected. Qualitative and quantitative assessments
were made to visualize lymphatic clearance and measure uptake
values at regions of interest. A standardized protocol is obligatory.
Qualitative lymphoscintigraphy can be helpful to understand if swelling is (or is not) lymphatic in origin and, if so, what the mechanism might be. Quantitative lymphoscintigraphy in children is difficult to interpret because of a lack of control parameters. No literature is available on this issue.
Nevertheless, lower limb lymph drainage is usually symmetrical and a substantial reduction in ilio-inguinal nodal uptake on one side would indicate LE even in the absence of any qualitative differences. Performing lymphoscintigraphy on a routine basis for the diagnosis LE is absolutely unnecessary if the clinical diagnosis is not in doubt, but it can be helpful in understanding mechanisms. For example, in Milroy disease there is no peripheral uptake of tracer (lower limb), indicating initial lymphatic failure. A fluorescence microlymphangiography study by Bollinger and Amann-Vesti25 showed that Milroy's disease is characterized by an absence of initial lymphatics. However, in LE-distichiasis syndrome, tracer is seen to be taken up and transported by the leg's collecting lymphatics. But, the tracer then refluxes back down the leg because of valve failure.
For special indications, such as chylous reflux, lymphoscintigraphy can be useful (discussed subsequently).26
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Visceral forms of lymphoedema: chylous effusion
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Visceral forms of lymphatic impairment can accompany LE sometimes.
If lymphostasis occurs in the cisterna chyli, the drainage from
the entire intestine can be deficient. Primary chylous ascites
is closely correlated to generalized lymphatic dysplasia. Chylous
ascites is the accumulation of triglyceride-rich, milk-like
peritoneal
uid because of the presence of intestinal lymph in
the abdominal cavity.
27
Chyle can also occur in the thorax (chylothorax), urine (chyluria) or can leak into the gut (protein-losing enteropathy).
Incompetent lymphatics throughout the limbs, abdomen and chest permit reflux and produces reflux syndromes with chyle reflux into the genital region, or even in the leg and foot. Some assume that dilated lymphatics, which can sometimes be visualized in the abdomen and chest, are probably because of upstream congenital hypo- or aplasia, but this is still under discussion.
Chylous lymphangiectasia on the skin can leak a white, milky substance from surface lymph blisters that represent grossly engorged superficial dermal lymphatics. Chylous reflux syndromes are very rare. Diagnosis and treatment of chylous reflux should be centralized with a special experience in this field.
Lymphoscintigraphy is useful to diagnose lymphatic impairment in the legs, but does not give sufficient information about dilated lymphatics. Lymphangiography is in general obsolete to diagnose LE, but is only useful to visualize ectasia via injection in a cutaneous vesicle.28 MRI and videocapsule endoscopy may confirm the diagnosis.29,30
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Family history/genetic counselling
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LE in children is usually based on genetics. A very meticulous
recording of the family history is essential to discover inheritance.
The genetics of LE are highly variable and LE is associated
with a very wide range of penetration, relatives often may not
know if they are affected. Therefore, careful examination of
as many family members as possible is recommended. This is very
important in order to perform genetic counselling to the parents
to help them understand the inheritance of LE. Although a genetic
base is expected, most cases of primary LE are sporadic (90%)
and have a negative family history. Consulting a clinical geneticist
is advised. For probands suspected of Milroy disease or LE-distichiasis
syndrome, testing of mutations in VEGFR3 and FOXC2 is now available.
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Treatment
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Although there are many treatment modalities for LE, a cure
is a not available. The cornerstones of LE therapy are compression
therapy, manual lymph drainage (MLD) and exercise
31,32 to reduce
pitting oedema, enhance lymph transport and maintain mobility.
Parents should be educated to help in the treatment of their
children. The combination of compression therapy, directed exercise,
MLD and skincare is called decongestive lymphoedema treatment
or complex decongestive therapy or complex physical therapy.
As LE is a chronic disease, life-long treatment is essential. Proper monitoring and follow up of the patient will help prevent complications such as deterioration of LE, irreversible skin changes or erysipelas.
Monitoring the effect of treatment in children differs from adults; volumetry is not suitable as a parameter because of natural growth in length and weight, but monitoring of height and weight, and the child's physical and psychological development, is very important.
In general, surgical treatment of LE in children is discouraged. Sometimes, there can be an indication for operation for visceral involvement or ingrown toenails.
Compression
The aim of external compression is to reduce interstitial capillary fluid filtration and therefore the lymphatic preload. On the other hand, compression when combined with exercise leads to increased lymphatic drainage by stimulation of initial lymphatic absorption and lymph propulsion. Compression therapy can be performed in many ways with various pressures: by bandaging with a variety of non-elastic bandages, by using flat-knitted elastic stockings and by using pneumatic compression therapy.33–35 Bandaging and hosiery can be combined with localized padding to raise local pressure on the tissues and to improve the shape and hardness of tissues. In children, many therapies have technical limitations because of the size of the legs or arms, the cooperativeness of the child, and circumstances such as incontinence of urine or stool. As the child grows, the possibilities for treatment options will increase. The child's therapist should try to involve the parents as much as possible.
Manual lymph drainage
MLD is a special massage technique based on the principles described by Winiwarter, Vodder and Földi.36 The goal of the massage is to increase lymphatic drainage by stimulating the existing or collateral lymph drainage routes in order to redirect the lymph flow towards other, less-affected regions. The particular massage technique of MLD is very gentle with stretch and soft pressures applied by a variety of rhythmic hand movements. Some easy massage techniques are suitable for self-management, and can be taught to and performed by the parents. No evidence-based figures are available to describe the method of performing MLD and on the recommended interface pressure of bandages or hosiery in children. They are mostly based on experience and expert opinion. We consider a discussion about this beyond the aim of this article.
Physical exercise
Physical exercise meets several goals: it will stimulate functional rehabilitation and improvement of mobility; it will improve muscular activity, which leads to internal compression of (lymph) vessels under bandages or hosiery. Intermittent pressure changes between muscles and external compression stimulates lymph drainage. Breathing patterns stimulate intrathoracic pressure changes, which lead to a stimulation of lymph flow through the thoracic duct. It is important, especially in children, to integrate these therapeutic options into daily life. Prohibiting physical activities should be discouraged and the child must be allowed to do recreational activities as normally as possible.
Skin care and self-management
One of the main complications in LE is infection. Lymphatics play a crucial role in local immunology. Mallon et al.37 demonstrated an impaired immunological response in the efferent and the afferent pathways in patients with breast cancer-related LE. Local antiseptic soap can be effective in reducing skin colonization, thereby reducing bacterial entry into the affected limb and proper antiseptic wound care can be beneficial. Tinea pedis, fungal infections and onychomycosis are less frequent as in adults, varying with a prevalence in children by about 3–9%.38 Good foot-care with eventual use of antifungal agents is recommended. Dry skin should be avoided by using moisturisers. Obesity must be prevented because this can increase the risk of LE.39 Obese children must be advised to lose weight and do exercise.
To improve the awareness and independence of professional health-care workers, patients and their parents should be educated to learn more about LE and the lymphatic system. Self-management can be taught so that the family is under the control of the treatment.
Psychological aspects
The psychological aspects of swelling are often neglected. Adolescents especially experience the feeling that they are different and cannot fulfil all the demands of their stage of life. LE can affect leisure opportunities,8 as well as social and sexual relationships. Maintaining self-esteem is an important aspect of living and coping with LE.
Treatment of chylous disease
Conservative treatment is based on a diet free of fats containing long-chain fatty acids. These fats are replaced with low-fat medium-chain triglyceride (called as MCT diet) intake in order to reduce the production of chyle. Paracentesis to reduce the intra-abdominal pressure from ascites, or thoracentesis to reduce pleural effusion may be necessary.28 Sometimes total parenteral nutrition is necessary in combination with somatostatin analogues53 to reduce intestinal fluid production. This programme will be combined with other non-operative treatment options for LE, such as MLD and compression.
Browse et al.40 demonstrated, in a series of 45 patients with chylous ascites, a 34% success rate with conservative management. Aalami et al.27 reviewed world literature and reported a 43% success rate with conservative treatment from a total of 156 patients.
Operative modalities include laparotomy with ligation of refluxing (mega)lymphatics/fistulae, resection of the affected part of the small bowel or peritoneo-venous shunt in order to create a new drainage route. In chylothorax, thoracocentesis and diet management should be performed. When chyle leak in children continues with 100 mL per year of age per day, surgical intervention, such as thoracocentesis, thoracotomy, fistula closure or pleurodesis is indicated.41
Microsurgical procedures
Extensive experimental work has been performed on the microsurgical reconstruction of lymphatic impairment with bypass surgery, lymphovenous anastomosis (LVA) or lymph vessel transplantation. Browse et al.42 comment that microsurgical techniques are challenging and require a dedicated microsurgeon. Long-lasting follow-up or controlled studies are not performed and many questions require answering. Although microsurgery studies in primary LE are not available, Damstra et al.43 studied 10 patients with breast cancer-related LE and reviewed literature. They found a minimal reduction in volume of LE following LVA; in the literature there was no convincing evidence of the success of LVA. Some publications suggest a better chance of success when microsurgical procedures are performed in the early stages of obstructive LE.44 Fortunately, at this early stage, conservative treatment is often very effective to treat and control LE.
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Complications
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LE can lead to complications, especially when not treated properly.
Deterioration of LE can lead to immobility, progressive skin
changes, recurrent infections as well as psychological impairment,
social impairment and disability. The most common complication
is erysipelas/cellulitis, which gives rise to high fever, vomiting,
a painful redness of the skin and sometimes blister formation.
The infection causes further lymphatic impairment, resulting
in further increase of swelling and yet more infection leading
to a dangerous vicious cycle.
In general the recurrence rate of erysipelas is high, up to 30–54%, after two years.22 In breast cancer-related LE, Mozes et al.45 reported an infection rate up to 41%. Figures in primary LE are controversial. In the original paper from Milroy,46 no attacks of erysipelas are described in families with congenital LE. Others reported that rates of infection are up to 31% in primary LE.28 If recurrent bouts of infection occur, prophylactic antibiotics (penicillin) in preventing erysipelas appear to contribute to a reduction of infections when combined with foot-care.52 In our experience, when LE has been treated effectively, the chance of infection is diminished.
In very rare cases, long-lasting LE can be complicated by the development of malignant tumours. Increased frequencies of tumours are reported in LE patients, including Kaposi's sarcoma, malignant lymphoma, squamous and basal cell carcinoma and melanoma.47 Stewart-Treves lymphangiosarcoma is the most aggressive tumour, presenting as multicentric red-brownish patches before tumour formation in the later stages. This neoplasm is very aggressive and patients have a five-year survival rate varying from 5% to 29%.48 It has been documented in both primary49,50 and secondary LE.51
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Flowchart for work-up
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LE in children is rare, and it requires a specialized, multidisciplinary
approach and an experienced physician. The aim of the diagnostic
process is to categorize the LE and to evaluate the pre-existence
of syndromic or familial forms. A flowchart is presented to
facilitate the diagnostic process, the treatment programme and
the follow up (Figure
4).
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Conclusions
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The diagnosis and management of LE in children differs significantly
from adults in terms of origin, co-morbidity and approach. Most
LE in children occurs as primary LE. Close and meticulous clinical
assessment can often lead to a final diagnosis. Additional paediatric
expertise is necessary when there are systemic co-morbidity,
such as cardiac, neurological or intestinal defects. A detailed
family history is essential to precisely determine the inheritance
of LE although more often LE is sporadic. Nevertheless, an increasing
number of cases because of
de novo mutations are being reported,
hence careful phenotyping is crucial.
Treatment will focus on non-operative options, such as MLD, compression therapy and exercises with the close involvement of the parents. Training parents or relatives to perform several components of LE therapy, such as putting on garments, can enhance independence from professional health-care workers. In puberty, psychosocial guidance is essential for supporting the process of growing up with this disfiguring illness and teaching patients to cope with their disability. Because LE is a chronic disease, life-long follow up and control will be useful for preventing complications and monitoring the effects of treatment.
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Conflict of interest statement
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Both the authors declare that they have no conflict of interest.
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Acknowledgements
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RJD was the lead author in the overall conceptualization, design
and review of literature for this article. PSM participated
in discussion and editing the manuscript.
Accepted March 23, 2008
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C Bellini, E Bonioli, and F Boccardo
Lymphoscintigraphy in paediatric patients
Phlebology,
October 1, 2009;
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237 - 237.
[Full Text]
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R J Damstra and P S Mortimer
Reply to letter 'Lymphoscintigraphy in paediatric patients'
Phlebology,
October 1, 2009;
24(5):
238 - 238.
[Full Text]
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Abstract
Introduction
