Paediatric lymphoedema · Telehealth Helpdesk 4985 5400 Option 1 6 Primary vs Secondary •Primary:...

2/06/2015

If you experience connection problems or issues during the session please ring HNE

Telehealth Helpdesk 4985 5400 Option 1

1

Welcome to Allied Health Telehealth

To receive an attendance certificate please complete your online evaluation at:

https://www.surveymonkey.com/s/paedlymphoedema

Paediatric lymphoedema A challenge for clinicians and families

Paediatric lymphoedema

A challenge for clinicians and families

Margaret Patterson

Senior Physiotherapist

Sydney Children’s Hospital Randwick

Johanna Newsom

Senior Physiotherapist

Children’s Hospital at Westmead

https://www.surveymonkey.com/s/paedlymphoedema

2/06/2015



2

What is lymphoedema?

• “Swelling of a part of the body caused

by accumulation of interstitial fluid

secondary to a malformation or

malfunction of the lymphatic system” Lymphoedema Framework document

• Low flow, high protein oedema

What is lymphoedema?

2/06/2015



3

Lymphoedema

• Chronic condition

• Not curable

• Alleviated by appropriate management

• If ignored it can progress and become

difficult to manage

Aetiology

• Congenital malformation of the

lymphatic system (primary)

• Damage to lymphatic vessels and /or

lymph nodes (secondary) – Trauma

– Cancer and its treatment

– Infections eg: filiarisis

2/06/2015



4

The Lymphatic System

• A one-way drainage system which

transports lymph from the tissues to the

vascular system – Healthy lymph system will transport 2-4L/day

• Continuous rapid removal of interstitial

fluid, plasma proteins, cells and debris


• Plays an integral role in the immune

functions of the body

• First line of defence against disease

• Organised in groups that drain specific

regions

2/06/2015



5


Incidence

• At birth, about 1 in 6000 will develop

primary lymphoedema

• Overall prevalence estimated as 0.13-2%

• In developed countries, main cause

widely assumed to be treatment for

cancer

2/06/2015



6

Primary vs Secondary

• Primary: 3-10% of all lymphoedemas – Idiopathic, with no identified cause

– Genetic causes

– Associated with a syndrome

• Secondary: very rare in paediatrics – Identified cause eg: infection, surgery, tumours,

radiation, trauma, obesity, filiarisis

Milroy’s Disease

• Visible at birth or soon after

• 5-10% of all primary lymphoedemas

• Usually affects lower limbs

• 2:1 females to males

• Familial pattern of inheritance

2/06/2015



7

Milroy’s Disease

Lymphoedema Praecox

• Approximately 80% of cases of primary

lymphoedema

• Occurs during 2nd and 3rd decade of life

2/06/2015



8

Lymphoedema Tarda

• Occurs after age 35

• Begins in foot and ankle progressing for

months or years

• About 70% experience swelling in one

lower limb

Impacts of lymphoedema

• Lymphoedema may produce significant

physical and psychological morbidity

• Increased limb size can interfere with

mobility and affect body image

• Pain and discomfort are frequent

symptoms

2/06/2015



9

Impacts of lymphoedema

• Increased susceptibility to acute

cellulitis/erysipelas can result in frequent

hospitalisations and long-term

dependency on antibiotics

Cellulitis

• Stagnant oedema fluid provides an ideal

medium for bacterial growth

• Acute, diffuse, spreading, oedematous,

suppurative infection of deeper

subcutaneous tissue and fat.

2/06/2015



10

Lymphoedema with cellulitis

Lymphoedema staging

• Several staging systems have been

devised

• ISL – International Society of

Lymphology

2/06/2015



11

ISL 0

• A sub clinical state where swelling is not

evident despite impaired lymph transport

• This stage may exist for months or years

ISL 1

• Early onset of condition

• Accumulation of tissue fluid

• Subsides with limb elevation

• Oedema may be pitting

2/06/2015



12

ISL 2

• Limb elevation alone rarely reduces

swelling

• Pitting is manifest

Late stage 2 (also called stage 3)

• Tissue fibrosis more evident

• May or may not be pitting

Elephantiasis

• Usually develops in untreated cases of

primary and filiarial lymphoedema

• Gross oedema with loss of limb shape

• Reduced lymphatic transport capacity

• Increase of subcutaneous fat and fibrous

tissue

2/06/2015



13

Elephantiasis

• Poor posture

• Impaired gait

• Lymphorrhoea

Elephantiasis

2/06/2015



14

Diagnosis and Recognition

• Clinical history

– History and behaviour of swelling

– Symptoms such as heaviness, tightness or hardness

– History of skin or nail infections

– Family history

Recognition

• Objective findings:

– Distribution of swelling

– Pitting oedema

– Positive Stemmer’s sign

– Increased skin folds

– Changes to skin texture and quality

2/06/2015



15

Clinical findings

Investigations

• Albumin

• Markers of immune function

• Renal function tests

• Lymphoscintigraphy

• MRI

• CT

• Ultrasound

2/06/2015



16

Assessment of swelling

• Circumferential limb measurements

• Other options: – Water displacement method

– Perometry

– Bioimpedance

Assessment of skin condition

• Dryness

• Fragility

• Pigmentation

• Redness/pallor

• Cyanosis

• Warmth/coolness

• Dermatitis

• Stemmer’s sign

• Fungal infection

• Hyperkeratosis

• Lymphorrhoea

• Cellulitis

2/06/2015



17

Assessment of skin condition

Other assessments

• Pain

• Psychosocial

• Mobility and functional

• Nutritional

2/06/2015



18

Management

• Early and accurate diagnosis is essential

• Life-long condition

• Emphasis on management rather than

cure

• Multidisciplinary input ideal

• Lymphoedema therapist to coordinate

care

Basic principles

• Reduction of swelling and improvement

of shape

• Skin care and treatment of skin problems

• Prevention of infection

• Pain management

• Psychosocial intervention

• Participation with peers

• Education

2/06/2015



19

Treatment decisions

• Holistic approach based on principles

• Specific treatment tailored to individual

Treatment & Management

• Gold standard is “decongestive lymphatic

therapy” – Complex, time-consuming, intensive, expensive

and challenging

• Education

2/06/2015



20

Treatment & Management - cautions

• Drug therapy

– Diuretics

– Antibiotics

• Surgery

Decongestive Lymphatic Therapy

• Phase 1 – initial treatment

– Skin care

– Massage

– Compression (bandaging)

– Exercise

2/06/2015



21

Decongestive Lymphatic Therapy

• Phase 2 – maintenance

– Compression (garments)

– Skin care

– Exercise

– Massage as needed

Skin and limb care

• Daily inspections for cuts/bites/scrapes

• Regular moisturising

• Anti-microbial and anti-fungal washes

• Immediate ABs if any signs of infection

• Nail care

2/06/2015



22

Skin and limb care

Skin and limb care

• “Dos and Don’ts”

– No BP or injections on affected limb

– No sunburn or overheating

– No heavy weights or strains

– Sensible footwear and clothing

2/06/2015



23

Massage

• Sequential massage following the

principles of lymphatic drainage

• Aims to reduce effects of oedema

• Re-route flow of stagnant lymphatic fluid

into centrally located healthy lymphatic

vessels

Multi-layered inelastic lymphatic bandaging

• Firm but flexible to counteract the elastic

insufficiency of the skin

• Increases tissue pressure and assists the

musculo-lymphatic pump

• Soften fibrosis and restore limb shape

2/06/2015



24

Bandaging

Bandaging

2/06/2015



25

Bandaging

Bandaging

2/06/2015



26

Bandaging

Bandaging

2/06/2015



27

Compression garments

• Maintain reduction

• In children may be the only intervention

available

• Custom made

• Graduated compression


2/06/2015



28


Exercise

• Mostly discussed in terms of healthy lifestyle and weight management

• Bandages or garments need to be worn during exercise.

2/06/2015



29

Challenges for clinicians – gaining education

• Literature and professional development

focuses mainly on adults and secondary

• No specific guidelines for paediatric

patients

• Accessing courses: time and money

• Maintaining skills in rare patient

population

Challenges for clinicians - services

• Dedicated services for children with

lymphoedema are rare

• Local services are often unavailable or

private

• Lack of knowledge among other

professionals

2/06/2015



30

Challenges for clinicians - paediatrics

• Effects of compression

• When to start bandaging/garments

• Manual lymphatic drainage - modified

Challenges for clinicians – tricky areas

2/06/2015



31

Challenges for clinicians – tricky areas

Challenges for families

• Obtaining a correct diagnosis

• Accessing clear and correct information

• Accessing treatment and on-going

management

• Lack of local services

2/06/2015



32


• Advocating for child’s needs

• Ensuring adequate supply of garments,

shoes and bandages

• Lack of funding

• Parents as therapists


• Impact on adolescents e.g. body image

• Managing physical and psychosocial

needs

• Allowing children normality

2/06/2015



33

Questions?

• Margaret Patterson:

[email protected]

Ph: (02) 9382 1050

• Johanna Newsom

[email protected]

Ph: (02) 9845 3369

References

• Lymphoedema Framework. Best practice for the

Management of Lymphoedema. International

consensus. London: MEP Ltd, 2006

• International Lymphoedema Framework. Care of

Children with Lymphoedema. Focus Document,

2010

• Textbook of lymphology for Physicians and

Lymphedema Therapists. 5th Edition. Authors –

M.Foldi, E Foldi, S Kubik

mailto:[email protected]

mailto:[email protected]

2/06/2015



34

References

• Connell, Brice, Mansour and Mortimer: The

Presentation of Childhood Lymphoedema. Journal of

Lymphoedema 2009 (4); 65-72

• Moffat and Murray: The experience of children and

families with lymphoedema – a journey within a

journey. Internation Wound Journal 2010 (7);14-26

• Preston, Seers and Mortimer: Physical therapies for

reducing and controlling lymphoedema of the limbs.

Cochrane Database of Systematic Reviews 2004 (4),

republished 2008

Date post:	09-Aug-2020
Category:	Documents
Upload:	others
View:	1 times
Download:	0 times

Paediatric lymphoedema · Telehealth Helpdesk 4985 5400 Option 1 6 Primary vs Secondary •Primary:...

Documents