2017 Te management oflipoedema Best Practice Guidelines interventions andsurgical Non-surgical Compression therapy self care and stylesupport Life management Lipoedema andassessment Diagnosis WUK WUK BPG

BEST PRACTICE GUIDELINES: EXPERT WORKING GROUP: THE MANAGEMENT OF Tanya Coppel, Specialist Lymphoedema Physiotherapist, LIPOEDEMA Belfast Health & Social Care Trust, Belfast

PUBLISHED BY: Julie Cunneen, Macmillan Clinical Lead for Wounds UK Lymphoedema Service/Nurse Consultant, Moseley Hall Hospital, Birmingham A division of Omniamed, 1.01 Cargo Works Sharie Fetzer, Chair, Lipoedema UK, London 1–2 Hatfields, London SE1 9PG, UK Tel: +44 (0)203735 8244 Kristiana Gordon, Consultant in Dermatology and Web: www.wounds-uk.com Lymphovascular Medicine, St George’s Hospital, London

Denise Hardy, Lymphoedema/Lipoedema Nurse Consultant, Kendal Lymphology Centre, Kendal, Cumbria; Nurse Adviser, Lipoedema UK/ Lymphoedema Support Network (LSN), Cumbria; Co- Chair of the Expert Working Group © Wounds UK, March 2017 Tis document has been developed Kris Jones, Patient; Joint Managing Director & Nurse by Wounds UK and is supported byActiva Healthcare, BSN Consultant, LymphCare UK; Nurse Consultant, Medical, Haddenham Healthcare, Lipoedema UK Lipoedema UK, medi UK, Sigvaris and Talk Lipoedema. Angela McCarroll, Trustee, Talk Lipoedema; Patient, Northern Ireland

Caitriona O’Neill, Lymphoedema Care Lead Nurse, Accelerate CIC, London

Sara Smith, Senior Lecturer in Dietetics and Nutrition, Queen Margaret University, Edinburgh

Cheryl White, Lymphoedema Specialist Physiotherapist, Cheshire

Anne Williams, Lymphoedema/Lipoedema Nurse Consultant, Lecturer in Nursing, Queen Margaret University, Edinburgh; Trustee, Talk Lipoedema, Edinburgh; Co-Chair of the Expert Working Group Tis publication was coordinated by Wounds UK with the Expert Working Group. Te views REVIEW PANEL: Rebecca Elwell, Macmillan Lymphoedema CNS, Univer- presented in this document are sity Hospitals of North Midlands NHS Trust, Staffordshire the work of the authors and do not necessarily reflect the views of the Peter Mortimer, Professor of Dermatological Medicine, supporting companies. Consultant Dermatologist, St George’s University of London How to cite this document: Wounds UK. Best Practice Alex Munnoch, Consultant Plastic Surgeon and Clinical Guidelines: The Management of Lead, Ninewells Hospital, Dundee Lipoedema. London: Wounds UK, 2017. Dirk Pilat, General Practitioner; Medical Director for Available to download from: ELearning at the Royal College of General Practitioners www.wounds-uk.com (RCGP), London

Melanie Tomas MBE, National Clinical Lead for Lymphoedema, NHS Wales and the Lymphoedema Network Wales INTRODUCTION

Developing best practice guidelines for the management of lipoedema People with lipoedema in the UK face Te meeting participants recognised a significant challenges. Many are not general paucity of clinical evidence relating GUIDE TO USING THIS recognised by healthcare professionals as to the management of lipoedema. Te DOCUMENT Each section of the having the condition or are misdiagnosed. conclusions of the meeting formed the basis document helps Awareness of lipoedema among medical for this document, which draws, where healthcare practitioners practitioners is poor, and little clinical possible, on relevant literature. Where to provide appropriate research is focused on the condition. To evidence is lacking, expert opinion has been support and effective date, no good quality guidelines for the used to inform the guidelines and make treatment and care for management of the disease have been recommendations. Te content was subject patients with lipoedema. published, resulting in inconsistent and to review by the Expert Working Group and Te key points for each frequently inappropriate care for people additional reviewers before being finalised. section summarise with lipoedema. the information most Tis document will be of interest to anyone relevant to clinical Even when lipoedema is diagnosed correctly, involved in delivering support and clinical practice accessing appropriate care within the NHS services to people with lipoedema, including may be difficult because of poor general practitioners, lymphoedema understanding of treatment and referral therapists, community nurses, plastic routes, and geographical variations in clinic surgeons, dietitians, commissioners, availability, funding and capacity. third-sector organisations and more.

Lipoedema is a chronic, incurable disease Tere is still a considerable amount to learn that can have a severe impact on quality of about lipoedema. Undoubtedly, the next few life, and physical and psychosocial years will bring rapid advances in wellbeing. Some patients are so seriously understanding of the pathophysiology of affected that they lead very restricted lives, lipoedema and the most effective ways of sometimes to the extent of being unable to managing the condition. As a result, the leave their homes. Te complexity of the Group recognises that this document is likely issues faced by patients with lipoedema to need to be reviewed within three years. necessitates interprofessional, multidisciplinary care with an emphasis on Te Group hopes that the document will be supporting self management and working in useful to people with lipoedema, and the partnership with the person to identify wide range of professionals who have realistic goals and to manage expectations. contact with them. Tis document is an early step towards achieving tangible Tese best practice guidelines on lipoedema benefits for patients, enhancing recognition were inspired by a group of clinicians who and diagnosis of the condition by first started discussing the need for clear professionals and the public, improving guidance in 2015. Te discussions access to best practice management, and culminated in a meeting in September 2016 providing scope for future development of that had the specific aim of developing lipoedema services in the UK. guidelines on management that improve the lives and outcomes of people with Anne Williams and lipoedema. Te meeting was ground Denise Hardy breaking: not only did it bring together key Co-Chairs opinion leaders and experts involved in the treatment of lipoedema from all around the UK, but, significantly, it also included people with lipoedema representing UK third sector organisations.

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 3 EPIDEMIOLOGY AND PATHOPHYSIOLOGY OF LIPOEDEMA

SECTION 1: EPIDEMIOLOGY AND PATHOPHYSIOLOGY OF LIPOEDEMA

Lipoedema was first described in 1940 and suggests: cases may be ‘hidden’ because of Box 1. Synonyms for is a chronic incurable condition involving a their mild nature or because the person is lipoedema (Schmeller & pathological build-up of adipose tissue reluctant to contact health services. Other Meier-Vollraith, 2007; (Allen & Hines, 1940). It typically affects the cases may be unrecognised or misdiagnosed Langendoen et al, 2009; thighs, buttocks and lower legs, and by health services. Common misdiagnoses Herbst 2012a; Cornely, sometimes the arms, and may, although not include obesity or lymphoedema (Box 2) 2014) always, cause considerable tissue (Goodliffe et al, 2013), although both ■ Adiposalgia enlargement, swelling and pain. It may conditions may co-exist with lipoedema. ■ Adiopoalgesia significantly impair mobility, ability to ■ Lipalgia perform activities of daily living, and Cause ■ Lipedema (American psychosocial wellbeing. Current Te precise mechanisms responsible for the spelling) conservative management involves development of lipoedema are unknown, ■ Lipohyperplasia dolorosa ■ encouraging self-care, managing symptoms, but it is likely that multiple factors are Lipohypertrophy dolorosa improving functioning and mobility, involved (Okhovat & Alavi, 2014). ■ providing psychosocial support, and Lipomatosis dolorosa of the legs preventing deterioration in physical and Lipoedema often first presents during ■ Painful column legs mental health and wellbeing. puberty, although oral contraceptive use, ■ Painful fat syndrome pregnancy and the menopause also appear ■ Riding breeches Lipoedema is predominantly a chronic to be triggers. Tese observations suggest syndrome adipose tissue disorder (the word lipoedema that hormonal change may be involved ■ Stovepipe legs. means ‘fat swelling’), with clinically apparent in initiating the characteristic build-up of oedema due to fluid accumulation in the adipose tissue (Fonder et al, 2007; Bano et tissues occurring as a secondary feature in al, 2010; Godoy et al, 2012). Onset of the some individuals (Todd, 2010; Herbst, disease after periods of significant weight Box 2. Lymphoedema and 2012a; Reich-Schupke et al, 2013; Herbst et gain have also been reported (personal lipoedema (Harwood et al, 2015). Although most commonly called communication, K Gordon). al, 1996; Lymphoedema lipoedema, the condition has a variety of Framework, 2006; Goodliffe et al, 2013) other names (Box 1). Tere is also evidence of a genetic predisposition to lipoedema. A family Patients with lipoedema Prevalence history of the condition has been found may be misdiagnosed as Lipoedema almost exclusively affects in 15%–64% of patients (Harwood et having lymphoedema. women, but a few cases have been reported al, 1996; Child et al, 2010; Schmeller & Lymphoedema results in men (Chen et al, 2004; Langendoen et al, Meier-Vollrath, 2007). Te genetic variants from malfunction of the 2009). Relatively little epidemiological involved have not been identified fully, lymphatic system, whereas research has been carried out on lipoedema but research suggests that autosomal lipoedema is thought to and so it is unclear exactly how many dominance with male sparing is the most primarily be a disorder people are affected and to what extent. likely mode of inheritance (Child et al, of adipose tissue (a Te research so far has produced widely 2010). Investigations into the genetics lipodystrophy). Confusingly, varying figures. In the UK, the minimum of lipoedema are ongoing, and include however, patients with prevalence of lipoedema has been estimated researching whether men may act as lipoedema may develop to be 1 in 72,000 (Child et al, 2010). carriers for the associated genetic factor(s). lymphatic dysfunction. However, the authors noted that this is Tis combination of likely to be an underestimate (Child et al, lipoedema and secondary 2010). In Germany, the prevalence of lymphoedema is lipoedema has been estimated to be 11% in sometimes referred to as women and post-pubertal girls (Földi et al, lipolymphoedema. 2006; Szél et al, 2014).

Further research is needed to establish clearly the proportion of the population affected by lipoedema. It is likely to be more common than the limited evidence available

4 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA VENOUS LEG EPIDEMIOLOGY AND PATHOPHYSIOLOGY ULCERATION OF LIPOEDEMA

Enlargement of fat tissue joint problems, may act to decrease the Te characteristic increase in subcutaneous effectiveness of the venous and lymphatic Key points fat tissue seen in lipoedema may be due to systems (Harwood et al, 1996; Cornely, 1. Lipoedema is adipocyte hypertrophy (increase in size but 2006; Langendoen et al, 2009). As a result, underdiagnosed and not necessarily number of fat cells) and/or the rate of interstitial fluid accumulation almost exclusively hyperplasia (increase in number of fat cells) may exceed the rate of clearance, and affects women (Suga et al, 2009; Schneble et al, 2016) oedema may occur. 2. Although lipoedema is (Figure 1). In addition, there is evidence of often misdiagnosed as an increase in the rate of adipocyte death, In patients with lipoedema who also have simply being obesity, possibly due to hypoxia induced by excessive chronic venous insufficiency (CVI) the lipoedema and obesity tissue enlargement, and infiltration of fat tendency for interstitial fluid accumulation can co-exist tissue by scavenger inflammatory cells may be compounded. 3. Hormonal and (macrophages) (Suga et al, 2009). genetic factors are Age-related changes that cause the likely to contribute By inducing growth of new fragile capillaries lymphatic vessels to harden to the adipose in the fat tissue, it has been suggested that (lymphangiosclerosis) and become less tissue enlargement hypoxia may contribute to the easy bruising effective at removing fluid may also characteristic of often reported by patients with lipoedema contribute to the development of lipoedema (Fife et al, 2010). Other tissue changes that lipolymphoedema (Cornely, 2006). 4. Patients with lipoedema may occur include reduced elasticity of the may develop secondary skin and connective tissue (fascia) Some women with lipoedema report lymphoedema (Jagtman et al, 1984; Herbst, 2012a). premenstrual fluid retention that can have a (lipolymphoedema), considerable cyclical impact on the size and which may be Te cause of the pain and hypersensitivity shape of lipoedematous areas. compounded if chronic often mentioned by patients with lipoedema venous insufficient is is unclear, but may relate to compression of also present. nerve fibres by enlarged fat deposits, inflammation and/or central sensitisation (a process which involves changes in the brain and spinal cord that are associated with the Genetic, hormonal development of chronic pain) (Langendoen and other factors et al, 2009; Peled & Kappos, 2016).

Development of oedema In many patients, lipoedema is accompanied by the formation of fluid Hypertrophy and/or hyperplasia Reduced connective oedema. It has been suggested that the of adipose tissue tissue elasticity oedema may result from overloading of an essentially normal lymphatic system (see Appendix 1, page 32 for information on the lymphatic system). Although, changes in Compression the structure and function of the lymphatic of nerve fibres, inflammation, Impaired functioning Increased capillary Impaired system have been observed in some and/or central of the venous and fragility mobility patients, much research is needed to sensitisation lymphatic systems discover whether these changes are a common feature of lipoedema and whether they relate to the pathophysiology of the condition (Amann-Vesti et al, 2001; Increased interstitial fluid Bilancini et al, 1995).

Increased interstitial fluid formation due to Pain Bruising Oedema (lipolymphoedema) capillary fragility and possible mechanical obstruction of small lymphatic vessels by adipose tissue enlargement, combined with reduced skin and connective tissue elasticity, reduced mobility due to pain or Figure 1: Possible pathophysiology of lipoedema

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 5 DIAGNOSIS AND ASSESSMENT ASSESSMENT

SECTION 2 : DIAGNOSIS AND ASSESSMENT Lipoedema is often not recognised in the early stages or in mild forms as the primary care, and awareness and symptoms and signs may be subtle. Te Lipoedema UK and the understanding of the condition among characteristics of lipoedema become more Royal College of General medical professionals is limited (Goodliffe obvious as the disease progresses and in Practitioners (RCGP) et al, 2013; Evans, 2013). more severe forms (Table 4, page 11). have partnered to develop an online course called A diagnosis of lipoedema is made on clinical Although the lower limbs and buttocks are the Lipoedema — An Adipose grounds that are based on the history and most commonly affected areas, it is suggested Tissue Disorder. Te Royal examination of the patient. Currently, there that lipoedema may occur in any part of the College of Nursing (RCN) are no known blood or urine biomarkers, body (Herbst et al, 2015) and there is a great has endorsed the course, nor are there any specific diagnostic tests, deal of variation between individuals in areas which takes about 30 for lipoedema (Herbst, 2012a). affected. In one study, about 30% of patients minutes to complete and with affected lower limbs also had affected can be accessed at: www. In the absence of definitive diagnostic tests, arms (Fife et al, 2010). However, anecdotal elearning.rcgp.org.uk/ clinicians need to have a clear understanding reports suggest the proportion of patients with lipoedema of the unique characteristics of lipoedema and affected lower and upper limbs is much higher, how they differ from other apparently similar particularly in established lipoedema (stage 2 conditions such as lymphoedema and obesity onwards). In about 3% of cases of lipoedema, (Fife et al, 2010) (see pages 8–9). the arms alone are affected, usually with sparing of the hands (Fife et al, 2010). Diagnosis of lipoedema may be delayed due to poor recognition of the condition by In patients with lower limb lipoedema, the health professionals. Making an accurate lower body will often be disproportionately diagnosis may be challenging, particularly in large: individuals may require clothes for the early stages or when a patient has their lower body that are several sizes co-existing obesity. larger than those needed for their upper body (Fife et al, 2010). Te course of lipoedema over time is not fully understood, but is highly variable and Te adipose tissue enlargement may be unpredictable. Te condition may progress accompanied by bruising without apparent relentlessly in some patients, and yet in cause or due to minor trauma only. Many others the only symptom is a relatively patients with lipoedema also often mention minor increase in subcutaneous fat that pain and extreme sensitivity/tenderness to remains stable for many years (Langendoen touch and pressure in the affected areas. et al, 2009; Dutch Guidelines, 2014). Tey also report that the affected areas are cooler than unaffected areas. (Te skin over History and symptoms obese tissue may also feel cooler because of Typically, a patient with lipoedema is the insulating effect of fat.) female and reports onset at puberty or at another time of hormonal change. Only a Patients with lipolymphoedema may handful of male cases have been reported mention that standing for long periods, hot in the literature: all were thought to have environments or weather, and aeroplane developed lipoedema secondary to journeys may exacerbate pain, swelling and hormonal disturbances, with reduced feelings of heaviness in the limbs, probably testosterone levels being a common factor due to fluid accumulation in the tissues. (Child et al, 2010). Mobility may be restricted due to pain, Te development of tissue enlargement is mechanical hindrance, and/or hip and often insidious (Todd, 2016). It is usually knee joint problems, particularly in bilateral and symmetrical, and most patients with severe lipoedema. Tere are commonly affects the legs, thighs, hips anecdotal reports of a possible association and/or buttocks, with sparing of the feet. between lipoedema and hypermobility Diagnosis of lipoedema may be difficult in (Willams & MacEwan, 2016; Lontok et al,

6 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA ASSESSMENT DIAGNOSIS AND ASSESSMENT

Box 3. Areas for discussion with a patient suspected of having lipoedema

■ Age at onset and association with potential hormonal ■ Clothing sizes for upper and lower body triggers, e.g. puberty, oral contraceptive use, pregnancy, ■ Impact on: weight gain - Daily living ■ Areas of the body affected, and whether and how the degree - Mobility (e.g. need for aids such as walking stick or wheel- and extent of enlargement or swelling have changed over time chair) ■ Effect of dieting, calorie restriction and physical activity/ - Personal relationships exercising on weight and limb size - Work ■ Presence and severity of pain, discomfort or hypersensitivity - Emotional state to touch ■ Family history ■ Presence, extent and triggers (if any) of bruising ■ Previous investigations and management (including surgery ■ Presence of knee or hip pain, and related mobility issues such as liposuction) ■ Differences in skin texture and temperature between affected ■ Other medical and surgical history (e.g. comorbidities, and unaffected areas regular medication, allergies, previous episodes of cellulitis ■ Effect of rest or leg elevation on leg size and pain/discomfort and previous surgery) in patients with lower limb enlargement ■ Reasons for presenting now, understanding of disease, and ■ Effect of prolonged standing, heat or hot weather on swelling expectations of treatment outcomes. and pain/discomfort

2017). Muscle weakness may also play a clinicians to examine them. In addition to Box 4. Stemmer’s part: a study in women with lipoedema and characteristic signs such as braceleting at sign (Lymphoedema women with obesity found that those with the ankles, reduced skin temperature and Framework, 2006) lipoedema had statistically significantly lower altered tissue texture may be present and leg muscle strength (Smeenge, 2013). Some require palpation to detect (Table 1, page 8). Stemmer’s sign is people become so restricted that they are Clinicians should check for Stemmer’s sign negative or not present when a fold of skin can housebound or unable to care for themselves. (Box 4), which can assist in differentiating be pinched and lifted up lipoedema from lymphoedema, and for at the base of the second In addition, patients with lipoedema may pitting oedema (Box 5, page 8), which if toe or at the base of the report family history of relatives with similar present may indicate lipolymphoedema. middle finger. tissue enlargement. Tey often mention A positive sign (a) in a repeated attempts to lose weight through Differential diagnosis patient with lipoedema, calorie-restricted diets and exercising that Part of the reason that lipoedema may be when a fold of skin have little or no impact on lipoedema- underdiagnosed is that it may be mistaken cannot be lifted, indicates affected areas and result in weight loss from for other conditions that cause sub- secondary lymphoedema. unaffected areas only (Fife et al, 2010). cutaneous tissue enlargement/swelling or fat Stemmer’s sign is usually deposition. Te two most frequent negative (b) in patients Box 3 lists areas for discussion during history misdiagnoses are generalised obesity with ‘pure’ lipoedema. taking in a patient suspected of having (particularly in young, otherwise well lipoedema. It is important to recognise that patients) and lymphoedema (Table 2, page 9). the patient may be presenting for the first time or may have had investigations and Medical causes of bilateral symmetrical management elsewhere previously. Also, in lower limb swelling are listed in Box 6 (page some cases, the patient may have encountered 10). Infrequent causes of unusual fat dismissive or negative responses during their deposition include Dercum’s disease, contact with health services. Ascertaining the polycystic ovary disease, Cushing’s patient’s reasons for presenting and their syndrome, growth hormone deficiency and hopes for treatment and outcomes will form a lipodystrophies that cause lipohypertrophy good basis for a partnership approach to (e.g. analbuminaemia) (Box 7, page 11). management. Investigations Examination Currently, there are no diagnostic tests for As lipoedema is a clinical diagnosis, lipoedema and the main purpose of examination is particularly important, and investigations is to exclude other diagnoses or individuals appreciate time taken by to inform lipoedema management strategies.

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 7 DIAGNOSIS AND ASSESSMENT ASSESSMENT

Table 1. Characteristic signs of lipoedema that may be found during clinical examination

Sign Description Subcutaneous tissue • Usually bilateral and symmetrical without involvement enlargement of the hands and feet (at least initially) • However, the pattern of areas affected and overall shape may vary between patients

Cuffing or braceleting at the • Te tissue enlargement stops abruptly at the ankles ankles/wrists or wrists so that there is a ‘step’ before the feet or hands which are usually unaffected • May also be called ‘inverse shouldering’

Loss of the concave spaces • Occurs in lower limb lipoedema either side of the Achilles • Te concave areas posterior to the malleoli (retromalleolar sulci) and either side of the Achilles tendon are filled tendon in

Bruising • Bruising may occur anywhere in areas affected by lipoedema, often with no known cause

Altered skin appearance, • Te skin of affected areas may feel softer and cooler than unaffected areas temperature and texture • Te skin may have the texture of orange peel or have larger dimples

Abnormal gait and limited • May be due to bulk of the legs and/or fat pads on the medial aspect of the knees mobility • May include: - Reduced or poor heel to toe strike during walking - Flat feet - Genu valgum (knock knees) • Muscle weakness Stemmer’s sign negative • Usually negative (Box 4, page 7) • A positive Stemmer sign represents failure to pinch a fold of skin at the base of the second toe, and is pathognomonic of lymphoedema

Pitting oedema (Box 5) in • Usually absent in the early stages of the disease patients with lipoedema • Patients with lipoedema may find testing for pitting oedema and secondary lympoedema particularly uncomfortable (lipolymphoedema) • Pitting indicates the presence of excess interstitial fluid and may be and/or chronic venous insuf- present in patients with lipolymphoedema ficiency

Box 5. Pitting oedema (Lymphoedema Framework, 2006)

Pitting oedema is a sign of excess interstitial fluid. It can be detected by applying a thumb or finger to tissues with pressure that is sustained for at least 10 seconds. Oedema is present when a dimple or pit remains in the tissues when the pressure is removed. Te depth of the pit produced may indicate the severity of the oedema. Repetition of the test across the area suspected of involvement can help to determine the extent of the oedema. N.B. Elucidation of this sign may cause discomfort or pain and should be performed gently.

8 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA ASSESSMENT DIAGNOSIS AND ASSESSMENT

Table 2. Differentiating lipoedema from lymphoedema and obesity (Forner-Cordero et al, 2009; Langendoen et al, 2009; Fife et al, 2010; Child et al, 2010; Fetzer & Wise, 2015) Characteristic Lipoedema Lymphoedema Obesity Gender • Almost exclusively female • Male or female • Male or female Age at onset • Usually 10–30 years • Childhood (mainly primary); adult (primary or • Childhood onwards secondary) Family history • Common • Only for primary lymphoedema • Very common Areas affected • Bilateral • May be unilateral or bilateral depending on • All parts of the • Usually symmetrical cause body • Most frequently affects legs, hips • Usually and buttocks; may affect arms symmetrical • Feet/hands spared Effect of dieting on condition • Weight loss will be • Proportionate loss from trunk and affected • Weight reduction disproportionately less from limbs with uniform loss lipoedema sites of subcutaneous fat Effect of limb elevation • Absent or minimal • Initially effective in reducing swelling; may • None become less effective as the disease progresses Pitting oedema (Box 5, page 8) • Absent or minor in the early stages • Usually present but pitting may cease as the • No of the disease disease progresses and tissues fibrose Bruises easily • Yes • Not usually • No Pain/discomfort in affected • Often • May be uncomfortable • No areas • Hypersensitivity to touch in affected • No hypersensitivity to touch areas Tenderness of affected areas • Often • Unusual • No Skin consistency • Normal or softer/looser • Tickened and firmer • Normal History of cellulitis • Unusual (unless lipolymphoedema is • Often • Unusual present) Stemmer’s sign (Box 4, page 7) • Usually negative (unless secondary • Usually positive • Usually negative lymphoedema is present)

Laboratory tests Imaging investigations Routine screening blood tests useful in Imaging investigations such as ultrasound excluding or identifying other or concomitant scans, magnetic resonance imaging (MRI) conditions, especially if weight gain and scans and computed tomography (CT) scans lethargy are present, may include urea and are usually not necessary to diagnose electrolytes (U&Es), full blood count (FBC), lipoedema, but may have a role if there is thyroid function tests (TFTs), liver function diagnostic uncertainty. tests (LFTs), plasma proteins (including albumin), brain natriuretic peptide (BNP – a Lymphoscintigraphy, a method of imaging test for congestive heart failure) and glucose the lymphatic system that involves injection (Forner-Cordero et al, 2012; NVDV, 2014). of radioactive tracers into the skin, should Box 5. Pitting oedema (Lymphoedema Framework, 2006) detect lymphoedema (Keeley, 2006). Even though hormonal factors are thought to contribute to the development of lipoedema, Ultrasound measurement of dermal there is no evidence that endocrinological thickness may help to differentiate tests will detect any abnormalities (NVDV, lymphoedema and lipoedema 2014). Similarly, blood tests to measure the (Naouri et al, 2010). levels of inflammatory markers, such as C-reactive protein (CRP) or erythrocyte Venous duplex ultrasound scanning may sedimentation rate (ESR) are unlikely to be indicated if chronic venous insufficiency provide abnormal results. is suspected (Wounds UK, 2016).

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 9 DIAGNOSIS AND ASSESSMENT

Hand-held devices Classification and staging Box 6. Other causes Tere is increasing interest in the potential Lipoedema has been classified according to: of bilateral lower limb role of hand-held devices that measure the ■ Distribution of the adipose tissue chronic oedema (Ely et al, electrical properties of skin and superficial enlargement 2006; Trayes et al, 2013) subcutaneous tissues as a way of ■ Te shape of the enlargement (Table 3). differentiating lipoedema and ■ Chronic venous lymphoedema. Te reading obtained (the However, these classifications are of limited insufficiency (CVI) tissue dielectric constant) is a measure of clinical use because neither indicates ■ Congestive cardiac the amount of water in the tissues. Higher severity or disease progression, and neither failure readings indicate higher water content. guides treatment. ■ Dependency or stasis Although patients with lymphoedema have oedema been found to have higher readings than Te first system devised to describe the ■ Obesity patients with lipoedema, further research is severity and progression of lipoedema ■ Hepatic or renal needed to determine the role of this comprised three stages. More recent dysfunction technology in diagnosis and management versions include a fourth stage to account ■ Hypoproteinaemia (Birkballe et al, 2014). for the development of lipolymphoedema ■ Hypothyroidism (Table 4, page 11). However, as oedema can ■ Pregnancy and Another device under development arise at any stage of lipoedema (Fife et al, premenstrual oedema examines the effect of a small area of 2010), inclusion of this fourth stage is ■ Drug-induced swelling, suction over affected tissues. Te suction is potentially confusing. e.g. calcium channel maintained for 30 seconds and an blockers, steroids, associated smartphone app videos the skin Te staging system in Table 4 may indicate non-steroidal anti- being tested (Levin-Epstein, 2016). a patient’s position in the progression of inflammatories. lipoedema. However, it does not take In patients with lipoedema, the suction is account of the severity of symptoms, e.g. N.B. Tese conditions will usually cause pitting oedema, and may thought to produce characteristic skin pain and impact on lifestyle, neither of co-exist with lipoedema changes that do not occur in patients which is necessarily related to the degree without the disease. A pilot trial is of tissue enlargement. underway (Levin-Epstein, 2016).

Table 3. Classifications of lipoedema (Meier-Vollrath & Schmeller, 2004; Földi & Földi, 2006; Langendoen et al, 2009; Herbst, 2012a)

According to the anatomical areas affected Type Anatomical areas affected

Type I Pelvis, buttocks and hips (saddle bag phenomenon)

Type II Buttocks to knees, with formation of folds of fat around the inner side of the knees

Type III Buttocks to ankles

Type IV Arms

Type V Lower leg According to the shape of the tissue enlargement Type Description

Columnar Enlargement of the lower limbs which become column-shaped or cylindrical

Lobar Presence of large bulges or lobes of fat overlying enlarged lower extremities, hips or upper arms Columnar lipoedema is much more common than lobar lipoedema

10 BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA DIAGNOSIS AND ASSESSMENT

Table 4. Lipoedema staging (Schmeller & Meier-Vollrath, 2007; Herbst, 2012a; NVDV, 2014) Box 7. Other diseases that may have unusual Stage Description patterns of fat deposition 1 • Skin appears smooth (Sam, 2007; Florenza et al, 2011; Herbst, 2012a; • On palpation, the thickened subcutaneous Kandamany & Munnoch, tissue contains small nodules 2013; Melmed, 2013; Nieman, 2015)

■ 2 • Skin has an irregular texture that resembles Dercum’s disease — the skin of an orange (‘peau d’orange’) or a mattress individuals have painful • Subcutaneous nodules occur that vary from fatty nodules often the size of walnut to that of an apple in size accompanied by a wide range of other symp- toms including fatigue; 3 • Te indurations are larger and more prominent may be on the ‘lipo- than in Stage 2 edema spectrum’ • Deformed lobular fat deposits form, ■ Multiple symmetrical especially around thighs and knees, lipomatosis (Mad- and may cause considerable distortion elung’s disease) — of limb profile painless symmetrical 4 • Lipoedema with lymphoedema (lipolymphoedema) tumour-like accumula- tions of fat in the sub- cutaneous tissues ■ Polycystic ovary disease — a hormonal disorder with increased production of androgen hormones often accom- panied by generalised obesity Future developments secondary lymphoedema is present ■ Cushing’s syndrome Some studies of the impact of liposuction (i.e. whether lipolymphoedema is present). — due to excess cortisol (see pages 29–31) on patients with production; obesity is lipoedema have used assessments of Such a system would need to be defined one of a wide range of symptoms and functioning to monitor fully and formally validated, but could be symptoms and may be outcomes (Schmeller et al, 2012; based on a scoring system for each of the accompanied by a char- Baumgartner et al, 2016). following items: acteristic dorsal fat pad • Degree of limb enlargement ■ Growth hormone de- Questionnaires were used to grade • Level of pain ficiency — causes may spontaneous pain, pain upon pressure, • Presence and extent of bruising include pituitary disease oedema, bruising, restricted movement, • Presence and extent of lymphoedema or trauma; the accom- cosmetic impairment and reduction of • Alterations in gait panying obesity is often quality of life on a five-point scale. Scores • Restrictions to mobility centralised for individual items as well as a total score • Restrictions to performing activities ■ Lipodystrophies that were compared pre- and post-operatively. of daily living cause lipohypertrophy • Impact on quality of life. (e.g. analbuminaemia) Te Expert Working Group suggested that — rare; may be congen- a similar approach that considers symptoms Te scoring system would need to be clear ital or acquired. and functioning could be developed to and simple. Total scores could be used to indicate non-surgical treatment needs and indicate whether the patient falls into the response in patients with lipoedema. Te mild, moderate or severe grade. In addition, Group also suggested that the terminology the system could be used for monitoring, ‘mild’, ‘moderate’ or ‘severe’ is more intuitive e.g. changes in total or individual item than the use of stages, and that each grade scores could be used to assess the could include scope for indicating whether effectiveness of management approaches.

BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA 11 DIAGNOSIS AND ASSESSMENT

Assessment Assessment of a patient with lipoedema should be holistic and aim to define the Holistic assessment of a person with lipoedema patient’s current disease severity, to indicate suitability for management options and History - including symptoms of lipoedema, medical/surgical history to signal need for referral (Figure 2). In practice, diagnosis and assessment are often Extent, distribution and severity of conducted concurrently and elements of the adipose tissue enlargement two processes often overlap. Pain Degree and extent of adipose tissue enlargement Mobility and gait Measurement of the degree and extent of adipose tissue enlargement in lipoedema Psychosocial assessment is not straightforward and is not used for diagnosis. However, sequential measurements may be useful for Dietary assessment assessment and monitoring purposes. A wide range of types of measurement may be Skin assessment employed, from bodyweight to limb volume measurement (Table 5, page 13). Vascular assessment

In general, simple methods are likely to be the Assessment of comorbidities most useful and the easiest to use consistently. Clinicians may find that they tailor the measurement method used to the needs of individuals. Documentation of the details of Pain Figure 2: Holistic the measurement method used is important Pain is a common and often distressing feature assessment of a patient to ensure that future measurements are of lipoedema that can impact significantly on with lipoedema performed consistently and that changes daily life. Te pain may take several forms, detected are not artefacts of differences in including aching, heaviness, tenderness or measurement location or technique. pain on touch. Te cause of the pain is unclear, but may be related to compression of nerves For some patients, tracking measurements and/or inflammation (Lontok et al, 2017). is highly motivating. However, the distortion and flaccidity of the tissues in patients Pain may also be related to joint problems, with lipoedema may make measurement especially of the knees and hips, arising from impractical. In such situations, serial increased tissue laxity that may cause joint photographs may be useful. misalignment or hypermobility, or from degenerative changes (Hodson & Eaton, 2013). Body mass index (BMI) is a measure of the ratio between weight and height. It is Assessment should aim to determine the used widely to define and diagnose obesity cause, nature, frequency, site, severity and and to monitor efforts to lose weight. In impact of the pain. Rating scales can be lipoedema, however, BMI is likely to be used to ask patients to quantify their pain at high even when the person is not obese and the initial and ongoing assessments. Rating is therefore of limited value (Reich-Schupke scales include: et al, 2013). ■ Numerical rating scale — e.g. individuals are asked to rate their pain on a scale from It should be noted that measurement for 0 to 10, where 0 is no pain and 10 is the fitting compression garments is a separate worst pain imaginable process from measuring for monitoring ■ Visual analogue scale (VAS) — e.g. purposes. Where available, clinicians individuals are asked to mark or indicate should follow the measuring requirements the level of pain on a 10cm line where for compression garments as stipulated by 0cm is no pain and 10cm is the worst pain the manufacturer (see pages 23–27). imaginable (Dansie & Turk, 2013).

12 BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA DIAGNOSISHOSIERY AND CLASSIFICATIONASSESSMENT AND PRODUCT

Table 5. Measurement for assessment and monitoring in lipoedema (de Koning et al, 2007; Langendoen Box 8. Lipoedema UK’s et al, 2009; Lopes et al, 2016; Madden & Smith, 2016) Big Survey 2014 key Weight • Te simplest method of monitoring change in body size findings on quality of life • Not a specific measure of body areas affected by lipoedema (Fetzer & Fetzer, 2016) Waist • Waist measurement provides information about the distribution of body fat Te 250 respondents to • Increased waist circumference can be used to indicate whether a person is Lipoedema UK’s Big Survey overweight or obese, and is associated with increased risk for metabolic syndrome 2014 reported that lipoedema (≥80 cm and ≥94 cm for Caucasian women and men, respectively) had a considerable impact on • Not a specific measure of a body area usually affected by lipoedema, but may be useful their lives: in helping to avoid obesity and to monitor efforts to lose non-lipoedematous fat ■ 95% reported difficulty in Waist to hip ratio • A higher waist-to-hip ratio (waist circumference ÷ hip circumference; using same buying clothes units) is associated with increased risk for metabolic syndrome and cardiovascular ■ 87% reported that disease (≥0.85 for women and ≥0.90 for men) lipoedema had a negative • In lower limb lipoedema waist to hip ratio may be unreliable because of effect on quality of life disproportionate adipose tissue enlargement over the buttocks and upper thighs. A ■ changing ratio may be due to a reduction in waist size or an increase in hip size 86% reported low self esteem Circumferential • For example, in lower limb lipoedema: at ankle, calf, knee, thigh ■ 60% reported restricted • A simple method, but requires consistent use of measurement location for meaningful monitoring over time social life ■ 60% reported feelings of Limb volume • Limb volume measurement is a complicated process hopelessness • Methods include water displacement and the use of computer programs that ■ 51% reported that calculate volume from circumferential limb measurements taken at 4cm intervals with a spring-tension tape lipoedema had an impact on ability to carry out Body mass index (BMI) • A ratio that is calculated by dividing weight by height squared 2 2 their chosen career (weight (kg) ÷ height (m ) ) ■ • Widely used to diagnose obesity (BMI ≥30) and monitor weight change 50% reported restricted • Of limited value in patients with lipoedema sex life ■ 47% reported feelings of self blame Mobility and gait contribute to oedema if present by reducing ■ 45% reported eating Patients with lipoedema should be asked the effectiveness of the foot and calf muscle disorders about mobility and observed when walking pump on venous return. ■ 39% felt that lipoedema so that gait and footwear can be assessed. had restricted their career Shape distortion and fat pads at the inner Psychosocial assessment choices. knee area may alter gait, which in turn may Patients with lipoedema may suffer cause other problems in the legs, knees, considerable psychosocial distress and have hips and back. Lipoedema may hinder significantly reduced quality of life (Box 8 and mobility because of tissue bulk, pain or hip Box 9, page 14). Te initial relief of finding out and knee problems. what is wrong when a diagnosis is received is often followed by feelings of frustration Muscle strength may also be reduced: a and despair when the patient realises that study of quadriceps strength found that treatment may not improve symptoms as patients with lipoedema had significantly much as they had hoped. lower strength than people with obesity (Smeenge, 2013). Te social stigma attached to increased body size and physical restrictions, coupled with Asking whether aids are needed for walking shame and embarrassment can damage and in what circumstances may highlight self-esteem, lead to difficulties with personal issues that may otherwise have gone relationships and work, and cause mental unmentioned. Patients with lipoedema may health issues including anxiety and depression also have flat feet or genu valgum (knock (Hodson and Eaton, 2013; Kirby, 2016; Fetzer knees) and require podiatric biomechanical & Fetzer, 2016). assessment. Restricted ankle mobility (e.g. poor ankle dorsiflexion) and reduced heel to Practical difficulties, such as those due toe movement with reduced heel strike may to reduced mobility and difficulties in induce a laboured or plodding gait. Tis may finding clothes that fit, along with fear of

BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA 13 DIAGNOSIS AND COMPRESSION ASSESSMENT

Box 9. Quality of life assessment in lipoedema

Formal quality of life assessment is usually reserved for For decades, the medical profession was sceptical about research purposes or for health economic evaluations the veracity of a person’s description of their illness. Yet undertaken for regulatory purposes. General tools available the words of the individual are likely to provide the most include the Short-Form (36) Health Survey (SF-36) accurate account of what it is like to live with a condition. (Lins & Carvalho, 2016). Currently, there is no quality of Trough such narratives the complexity of the illness life assessment tool for people with lipoedema, although experience can be seen. As Hyden (1997) stated: “One a tool has been developed for people with lymphoedema of our most powerful forms for expressing suffering and (LYMQOL) (Keeley et al, 2010). A Patient Benefit Index, a experiences related to suffering is the narrative. Patients’ scoring system that evaluates the benefit of treatment from narratives give voice to suffering in a way that lies outside the individual’s perspective, has been developed for people the domain of the biomedical voice.” with lymphoedema and lipoedema (Blome et al, 2014). Gathering information using the illness narrative enables Te illness narrative clinicians to gain a more complete understanding of how An illness narrative (Hyden, 1997) is derived from an the condition is impacting each individual person and individual’s explanation of their struggle with a chronic or therefore how to best meet their needs, in particular, how to disabling illness. It is their story of living with the condition. better address their psychosocial needs.

discrimination or not fitting into seats in have produced weight loss from non- public spaces, may discourage a patient lipoedematous areas, but may also have from leaving their home, resulting in resulted in disordered eating behaviours, social avoidance, withdrawal and isolation. including anorexia nervosa, binge eating and Tese issues may be compounded by lack bulimia (Fife et al, 2010; Forner-Cordero et of understanding and fear expressed by al, 2012; Williams & MacEwan, 2016; Todd, family, friends and colleagues. Patients with 2016; Fetzer & Fetzer, 2016). However, up lipoedema have also reported receiving to half of patients with lipoedema may also verbal abuse from members of the public be overweight or obese (Langendoen et al, (Kirby, 2016). 2009; Fife et al, 2010).

Patients with lipoedema should be Dietary assessment should be approached asked about their home situation sensitively and include: (e.g. accessibility, general living standards, ■ Current diet, eating habits, and fluid and household members, involvement of alcohol consumption carers), activities of daily living, social ■ Previous attempts to lose weight and interactions, recreational/physical activities the effects of these and exercise. Psychological assessment ■ Te patient’s: should include evaluation of mood for signs - Beliefs about eating, weight gain of depression or anxiety, ability to cope, and physical activity energy levels and sleep quality. - Willingness to change - Understanding of the role of diet in Assessment should also include gaining an the management of lipoedema understanding of the patient’s insight into (NICE CG189, 2014). the condition and their personal goals and expectations of the components and Skin assessment outcomes of treatment. Skin should be assessed for general condition and the effectiveness of personal care. Te Dietary assessment skin of patients with lipoedema is soft and Many patients with lipoedema have tried easily damaged and some patients develop repeatedly and often unsuccessfully over ulceration. It is particularly important to many years to reduce the size of the examine any skin folds as these may develop affected areas through dieting and physical friction or moisture-related skin damage, activity or exercise. Tese efforts may and fungal or bacterial infections.

14 BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA COMPRESSION DIAGNOSIS AND ASSESSMENT

Vascular assessment Furthermore, inflation of a cuff around the Compression therapy is an important limb may be very painful for patients with More information on element of the management of lipoedema. lipoedema. the role of ABPI in Patients with lipoedema should undergo determining suitability vascular assessment according to local Comorbidities for compression protocol. Significant arterial disease is a Comorbidities should be identified and therapy can be found contraindication to compression therapy management optimised to minimise impact in the Wounds UK Best (Wounds UK, 2015). on patients with lipoedema. Patients with Practice Statement on lipoedema have self-reported the presence Compression Hosiery, Te vascular assessment should include of several conditions: fibromyalgia, gluten which is available at: consideration of signs, symptoms and allergy (coeliac disease), hypothyroidism, www.wounds-uk.com risk factors for arterial disease. Doppler polycystic ovary syndrome, vitamin D ultrasound to determine ankle-brachial- deficiency and arthritis (Herbst et al, 2015; pressure index (ABPI) is a method often used Smidt, 2015; Williams & MacEwan, 2016). for vascular assessment. However, tissue However, evidence of direct links between enlargement may make it difficult to get an lipoedema and many of these conditions is accurate ABPI in patients with lipoedema. currently very limited.

Key points 1. Te diagnosis of lipoedema is made on clinical grounds: there are no diagnostic tests for the condition 2. Lipoedema is a condition that is distinct from lymphoedema 3. Lipoedema may have a significant impact on a patient’s physical and mental health and wellbeing 4. Patients with lipoedema generally report a history of bilateral symmetrical limb enlargement, with sparing of the hands and feet, which is not responsive to dieting. Tey may also report pain, sensitivity to touch and easy bruising, and a family history of similar tissue enlargement and shape disproportion 5. Affected areas of the body may be softer and cooler, with a texture that is dimpled or resembles a mattress 6. Te presence of pitting oedema in affected areas indicates lipolymphoedema 7. Routine blood tests may be useful to exclude or identify other conditions 8. Imaging investigations are not used routinely 9. Further work is required to develop a classification/staging system for lipoedema that takes into account disease progression along with symptoms such as pain or restrictions to mobility 10. Holistic assessment should include the degree and extent of adipose tissue enlargement, presence and level of pain, mobility and gait, psychosocial assessment, dietary assessment, skin assessment, vascular assessment and assessment of any comorbidities 11. Psychosocial assessment is particularly important in people with lipoedema because of the long-term nature of the disease and the importance of self-management.

BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA 15 PRINCIPLES OF COMPRESSION MANAGEMENT

SECTIONBox 3. Dos and don’ts of hosiery 3 care: PRINCIPLES OF MANAGEMENT

Lipoedema is a long-term condition that has wide-ranging impacts on the health and psychosocial wellbeing of patients. Facilitation and enhancement of the patient’s ability to self-care: Consequently, an interprofessional or including education, healthy lifestyle multidisciplinary approach to management (diet/physical activity/weight reduction) is often required. However, there is currently inconsistency and inequity across the UK in referral patterns and care for patients with lipoedema.

Patients recognised as possibly having Optimisation of health and Management of symptoms: lipoedema in a primary care setting may prevention of progression: including mangement of pain, including weight management, impaired mobility, oedema and be referred to a lymphoedema service, compression therapy, treatment psychosocial issues where available, for investigation, diagnosis, of concomitant conditions management and co-ordination of care. However, there is variation throughout the UK in provision of lymphoedema services, and some services do not have sufficient capacity to manage patients with lipoedema. Where there is no provision of lymphoedema services, a referral to vascular or plastic Figure 3: Principles of lipoedema management surgery services may be appropriate. In keeping with the NHS goal for personalised care for people with long- Even so, the Expert Working Group term conditions, clinicians should take a concluded that lymphoedema services are the collaborative approach to the management most appropriate setting for the management of a patient with lipoedema, providing of patients with lipoedema, not least because individualised care plans according to need of the expertise held within these services and person-centred treatment goals (NHS in differentiating the two conditions and in Outcomes Framework; Coulter et al, 2013; the use of compression therapy. Te Group WHO, 2004; Woods & Burns, 2009; Welsh considers that improved recognition of the Assembly Government, 2007). disease and appropriate referral patterns are reliant on enhancing awareness and Te main components of lipoedema recognition of the disease in primary care management are: settings, and in the wider provision of ■ Psychosocial support, management of lipoedema/lymphoedema services. expectations and education, including family planning, pregnancy advice and Tird sector organisations, such as genetic counselling Lipoedema UK (www.lipoedema.co.uk) and ■ Healthy eating and weight management Talk Lipoedema (www.talklipoedema.org), ■ Physical activity and improving mobility provide help with self-management and are ■ Skin care and protection important sources of peer support. ■ Compression therapy ■ Management of pain. Principles of lipoedema management Te management of lipoedema requires a Each element needs to be tailored according holistic approach (Figure 3) that includes: to the severity of symptoms, degree ■ Facilitating and enhancing the patient’s and complexity of tissue enlargement, ability to self-care and cope with the whether there has been progression to physical and psychosocial impact of the lipolymphoedema, and the psychosocial condition status of the patient. ■ Managing symptoms ■ Optimising health and preventing Patients with lipoedema may be well disease progression. informed about their condition and possible

16 BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA COMPRESSION PRINCIPLES OF MANAGEMENT

management routes following internet Table 6. Involvement of the multidisciplinary team in the management of lipoedema searching and participation in social media. However, the advice and information found Indication Clinician/service → may not be necessarily grounded in evidence. • Tissue enlargement ± oedema Lipoedema/lymphoedema specialist clinician Individuals may be susceptible to • Pain, aching, sensitivity to touch misinformation and may need help in • Abnormal gait → Physiotherapist understanding what is best practice and most • Muscle weakness likely to be of benefit based on current • Joint pain evidence, and what is not yet clear or may be • Mobility problems → Occupational therapist detrimental. Such discussions require a • Difficulty with day-to-day activities sympathetic, non-judgemental approach to → avoid discouraging or offending individuals in • Advice and education about weight Dietitian their efforts to improve their condition. management, healthy eating, disordered eating, nutritional supplements, diabetes Discussions should also bear in mind that • Flat feet → Podiatrist individuals are often very vulnerable and • Abnormal gait sensitive after a long journey to diagnosis, • Unmanageable/chronic pain → Pain clinic which may have included disheartening • Concomitant conditions → Appropriate specialist service (e.g. and upsetting comments from healthcare vascular service, diabetic clinic, professionals seen previously. psychological services) • In carefully selected patients, after non- → Plastic surgeon Support and encouragement alongside → surgical approaches have been implemented: Bariatric surgeon working in partnership with the patient • Severe tissue enlargement causing and their carer(s) with careful management mobility impairment of expectations, including sensitive • Management of severe obesity discussions about the life-long nature of the condition, should underpin the best practice Patient pathway management of lipoedema. Appendix 2, page 33, summarises the patient pathway through assessment and management

■ Clinicians specialising in the management Reduced likelihood of progression to of lipoedema have a key role in providing lipolymphoedema Key points ■ 1. A multidisciplinary education and support around a healthy Where present, reduced severity of approach to the lifestyle, and in implementing and managing lipolymphoedema and reduced risk of management compression therapy. Potential roles for complications such as cellulitis ■ of lipoedema is other members of the multidisciplinary team Minimisation of secondary joint necessary are listed in Table 6. It should be noted that problems, such as knee and hip 2. Management aims to referral may not always be available within osteoarthritis ■ manage symptoms, the NHS; where available, individual services Minimisation of impact on ability to to facilitate and may have specific restrictions and criteria for perform daily activities, including work ■ enhance the patient’s referral. Private referrals may be possible for Enhanced ability to self-care ■ ability to self-care and patients with sufficient financial resources. Improved psychosocial wellbeing. optimise health and Primary care and community-based services to prevent disease have an important role in supporting and Tese effects are also likely to result in wider progression enabling self-care and ensuring referral when benefits to the healthcare system including 3. Te main appropriate (Todd, 2016). an overall reduction in healthcare utilisation components due to lipoedema and for obesity-related of lipoedema conditions such as diabetes. Benefits of lipoedema management management are: Lipoedema is a long-term condition that psychosocial support is not curable. However, management of Although there is currently no evidence and education, lipoedema according to best practice has that early treatment improves prognosis healthy eating, the potential to produce benefits including: in lipoedema, the Expert Working Group weight management, ■ Reduction in pain considers that early diagnosis, intervention physical activity, skin ■ Improved limb shape and initiation of self-care would produce the care, compression ■ Avoidance of impairment or greatest health and economic benefits. As yet, therapy and improvement in mobility no formal health economic analyses have been management of pain. ■ Management or avoidance of obesity done on the impact of lipoedema management.

BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA 17 PSYCHOSOCIAL SUPPORT AND SELFCARE

SECTION 4: PSYCHOSOCIAL SUPPORT AND SELFCARE Patients with lipoedema may experience Support can be provided in several ways similar psychological and social challenges to including discussions during clinic or Box 10. Self-efficacy those faced by people with other long-term health centre visits, and the provision of (Frei et al, 2009; Lorig et al, conditions. Recently published research written materials or links to websites, and 2001; Adams et al, 2010; on the psychosocial impact of lipoedema information on support organisations Hoffman, 2013) found that psychological flexibility and social (Box 11, page 19) and education connectedness were associated with higher programmes. Peer-led groups may have Self-efficacy (the extent or quality of life irrespective of severity of a particular role in reducing feelings of strength of a person’s belief symptoms (Dudek et al, 2016). isolation. Potential barriers to self-care in their own ability to (Box 12, page 19) should be identified and produce specific goals) Psychological flexibility was defined as addressed where possible. influences health outcomes willingness to contact difficult, unpleasant and may also affect level of thoughts, feelings and sensations in order Many patients with lipoedema adjust well, healthcare utilisation. to engage in a valued activity; social and may even be able to identify positive Although self-efficacy has connectedness was defined according to a outcomes of their diagnosis, such as a not been measured in scale that measured feelings of belonging greater appreciation for life and reassessed patients with lipoedema, and connection with friends and society. life priorities. However, some patients research in other chronic may benefit from interventions such as diseases indicates that high In addition, research into a range of other group/individual counselling, cognitive self-efficacy is an important chronic diseases has concluded that: behavioural therapy (CBT) (Box 13, page 19) mediator in altering health ■ Psychological adjustment has a major or mindfulness (Mantzios & Wilson, 2015). behaviours. In addition, impact on chronic disease outcomes and Unfortunately, access to these interventions improvements in self- can be aided by encouraging people to within the NHS is variable. efficacy generated through stay as active as possible, to express their self-management or emotions and to engage in self-care Mental health issues such as depression educational programmes ■ People who have a healthy diet, engage in or anxiety may need treatment according have been associated with physical activity/exercise or employ other to National Institute of Health and Care reductions in healthcare aspects of self-management have fewer Excellence (NICE) guidance (NICE CG91, utilisation and symptom symptoms, better functional capability 2009; NICE CG113, 2011). Furthermore, burden. and fewer complications than those who clinicians should be alert to hidden mental do not health issues in carers (Turner & Kelly, 2000). ■ Mental health issues, such as depression, anxiety or psychological distress, can Patients with lipoedema who have or are compromise the ability to self-manage planning to have children may have concerns which in turn may lead to feelings of and anxieties about genetic and hereditary helplessness and hopelessness (de Ridder aspects of lipoedema. Tese concerns may et al, 2008; Dekker & de Groot, 2016). impact their ability to accept the condition and the treatment strategies that may be Recognition of the importance of and need used. Clinic appointments can provide an for emotional and psychological support opportunity to discuss these worries and to that encourages self-efficacy (Box 10) is put in place referrals for genetic counselling crucial to the successful management or to a lymphoedema clinic as appropriate of lipoedema. In providing person- for the patient or other family members. centred, empathetic care that is realistic but positive in managing expectations, clinicians play a key role in supporting and empowering patients to adjust to their new circumstances, engage with self-care and seek additional help when needed.

18 BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA PSYCHOSOCIAL SUPPORT AND SELFCARE

Box 11. UK lipoedema support organisations Key points 1. In common with ■ Lipoedema UK* – www.lipoedema.co.uk other chronic ■ Talk Lipoedema – www.talklipoedema.org conditions, ■ British Lymphology Society (BLS) – www.thebls.com psychosocial ■ Lymphoedema Support Network – www.lymphoedema.org support underpins the management N.B. A number of overseas and international organisations also exist; their websites can be accessed via of lipoedema and internet searching is important in *Lipoedema UK has produced an advocacy pack, which is available on request, that contains information encouraging self- on accessing services management and realistic expectations 2. Clinicians need to identify and help patients to deal with Box 12. Potential barriers to self-care in patients with lipoedema potential barriers to self-care ■ Previously ineffective care and long duration of the disease that has entrenched a standpoint that nothing can be done to improve the situation 3. Mental health issues may affect carers as ■ Lack of knowledge and skills to support treatments and behaviour changes, e.g. lack of understanding of the role of compression therapy in improving symptoms and of the role of well as patients with physical activity and healthy eating in improving symptoms and overall health, inability to lipoedema. apply compression garments correctly and safely ■ Severity of the condition, e.g. increased bulk, the presence of pain, reduced mobility or concomitant conditions, may hinder physical activity or ability to self-apply compression garments ■ Poor relationship with healthcare professionals may result in a lack of trust with unwillingness to take healthcare advice or to seek help when needed ■ Low self-esteem, self-efficacy or social/emotional support may hinder ability to take responsibility and implement self-care ■ Financial restrictions, e.g. difficulty paying for transport to appointments may prevent opportunities for education and encouragement ■ Inaccurate or misleading information, e.g. from social media sites or the internet may cause confusion or reinforce misconceptions.

Box 13. Cognitive behavioural therapy (CBT) in lipoedema

■ CBT is a type of psychotherapy that can help people by changing the way that they think and behave ■ CBT is recommended by the National Institute for Health and Care Excellence (NICE) for patients who have a mental health condition, an eating disorder, body dysmorphic disorder, or a chronic physical health problem with depression (NICE CG31, 2005; NICE CG9, 2004; NICE CG91, 2009; NICE CG113, 2011) ■ In patients with lipoedema, CBT has the potential to help with a range of issues, including encouraging realistic but optimistic attitudes, treating depression, encouraging self- management and improving functioning (de Ridder et al, 2008; Deter, 2012; Fetzer, 2016).

BEST PRACTICE STATEMENT: THE MANAGEMENT OF LIPOEDEMA 19 HEALTHY EATING AND WEIGHT MANAGEMENT

SECTION 5: HEALTHY EATING AND WEIGHT MANAGEMENT Nutrition plays an important role in the Key to effective weight management is management of lipoedema, not just in weight support and advice enabling patients to management but also in engaging the patient, find a nutritional plan that suits their providing a sense of control over the evolution food preferences and lifestyle. Despite of lipoedema, and reducing the risk of obesity- the plethora of dietary advice available on related conditions such as diabetes and joint the internet and other media, there is no degeneration (Todd, 2010; Todd, 2016). clinical evidence to support the use of a particular dietary plan in lipoedema. All patients with lipoedema who are not overweight or obese should be encouraged Some patients advocate the Harvie and to eat healthily to avoid weight gain through Howell diet which involves a restricted accumulation of non-lipoedema fat. Avoidance calorie intake for two days each week of weight gain will prevent deleterious and eating a Mediterranean-style diet on effects on general health and possibly slow the other days (Harvie & Howell, 2014). progression of the condition (Todd, 2010). Anecdotally, this regimen appears to result in easier, longer-lasting weight loss (Fetzer Patients with lipoedema are likely to have & Wise, 2015; Todd, 2016). tried a variety of diets and may have a complex relationship with food. In Lipoedema Te Rare Adipose Disorders (RAD) diet UK’s Big Survey 2014, 98% of participants has also been advocated for patients with reported trying to lose weight: 82% had lost lipoedema (Todd, 2016). Te basis of this some weight, but this was lost evenly across diet is reduced consumption of pasteurised the body in only 5% (Fetzer & Fetzer, 2016). dairy products, animal fats, simple sugars, carbohydrates, salt and artificial Failure to lose weight and feeling unattractive preservatives, flavours and sweeteners may lead to a cycle of emotional or comfort (Herbst, 2012b). However, as with other eating and further weight gain. Denial that diets, evidence of effectiveness in patients obesity is present, the use of the diagnosis with lipoedema is awaited. of lipoedema as an explanation for weight gain due to overeating, and fixed ideas about Where weight loss is advisable, patients diet and ‘good’/’bad’ foods may complicate with lipoedema are likely to benefit attempts to discuss diet and weight loss. from referral to a dietetic-led service. A stepwise approach to goal setting is Anecdotally, weight loss programmes have important to enable success and prevent little or no effect on the amount of tissue discouragement. A non-prescriptive, enlargement in lipoedema (Todd, 2010; Fife sympathetic approach that supports et al, 2010). Any weight loss that does occur healthy eating and maintains a dialogue is likely to be disproportionately lower in about food and diet is very important. lipoedema-affected areas than in unaffected areas. However, a significant proportion of Some patients with lipoedema find patients with lipoedema also have obesity attending proprietary weight loss (Langendoen et al, 2009). Tese individuals programmes that involve weekly meetings should be encouraged to reduce the amount with weigh-ins and discussions motivating. of non-lipoedema fat tissue through diet and When discussing healthy eating, the focus physical activity. Benefits include improved should be on encouraging sustainable general health with a likely reduction in risk healthy, balanced changes in eating habits. for cardiovascular disease and diabetes, less strain on joints and muscles with potential Referral according to local protocol for benefits for mobility, and a probable reduction eating disorders may be necessary if there in the risk of developing lipolymphoedema is evidence of a problem, such as anorexia, (Fonder et al, 2007; Langendoen et al, 2009; bulimia or binge eating (NICE CG9, 2004). Todd, 2010).

20 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA HEALTHY EATING AND WEIGHT MANAGEMENT

Physical activity

Increases energy Enhances venous and Improves muscle strength expenditure lymphatic return

Reduces strain on and Aids weight maintenance Reduces oedema Mental health benefits or loss stabilises joints

Improved mobility Psychosocial benefits

Figure 4: Potential benefits of physical activity in patients with lipoedema

Dietary supplements undertaken by bus or car (Davies et al, 2011). Some healthcare practitioners recommend Physical activity in patients with lipoedema Key points dietary supplements for people with can also have a number of benefits including 1. Although attempts lipoedema. Currently, there is no robust weight maintenance or loss and improved to lose weight may clinical evidence supporting the use of dietary mobility (Figure 4) (Fetzer & Wise, 2015). not have an impact supplements and further work is needed to Te psychological benefits of physical on enlarged adipose identify which may be of benefit and in what activity may help to combat negative feelings tissue, preventing ways. Discussions around supplements need associated with lipoedema. or reducing obesity to be approached with sensitivity to prevent in other parts of the alienation and loss of trust. Patients with lipoedema who have severely body through healthy impaired mobility, abnormal gait and/or eating and physical Physical activity and pain/joint problems should seek the advice of activity will help to improving mobility a physiotherapist on suitable types of activity. prevent deterioration Increased physical fitness and activity in the in general health general population has been demonstrated Patients with lipoedema embarking on increased 2. Tere is no to have numerous health benefits, including physical activity should start to increase activity clinical evidence reduced mortality, reduced rates of obesity, levels slowly, aiming for some form of physical supporting the use diabetes, cardiovascular disease and cancer, activity every day. Te apparently small gains of a particular diet. and improved mental health and quality of made will increase confidence. Patients should be life (Salmon, 2001; Penedo & Dahn, 2005; encouraged to find Bishop-Bailey, 2013). High intensity exercise or activities that a healthy, balanced cause or aggravate pain or bruising should diet that suits their Current UK guidelines recommend that be avoided (Fetzer & Wise, 2015). Low needs and lifestyle adults aged 19 to 64 years should be active intensity activities include walking, water- 3. Patients with daily and each week undertake at least based exercises, yoga, pilates and the use of lipoedema should 2.5 hours of moderate intensity activity. resistance bands. However, patients already be encouraged to be Tey also recommend that adults should involved in a high intensity form of exercise physically active and undertake physical activity to improve should not be discouraged from participating, undertake activities muscle strength at least two days each but may need to consider how to minimise that suit their needs week (Davies et al, 2011). In addition, the the risks of joint strain and bruising. and lifestyle, while guidelines strongly recommend minimising taking into account sedentary behaviour, e.g. by reducing the Exercising in water, e.g. water aerobics, may that some patients amount of time spent watching television be particularly beneficial in patients with may have individual or using a computer, taking regular breaks lipoedema as the support provided by the limitations. at work, and walking for part of journeys water reduces strain on and aids the range of

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 21 HEALTHY EATING RECURRENCE AND WEIGHT PREVENTION MANAGEMENT

motion of joints, reduces pain, and improves restricted mobility. Use of the term ‘physical venous and lymphatic return (Fetzer & Wise, activity’ and reassurance that increasing 2015). Over 75% of people with lipoedema activity does not necessarily need to involve who answered the question about exercising exercise classes may be more successful in in water in Lipoedema UK’s Big Survey 2014 encouraging increased levels of movement. found it to be helpful (Fetzer & Fetzer, 2016). Suggestions could include home-based exercise, chair exercises, walking, using the Exercise in water can take the form of stairs rather than a lift, or parking further swimming or formal exercise classes from the supermarket door. (aqua-exercise/aerobics). Patients who cannot swim need not be deterred: simply Feeling self-conscious and embarrassed, walking in water is good exercise and the along with difficulties finding sports pressure exerted by the water on the tissues garments and swimwear that fit, can be is beneficial. major barriers to exercising in public or participating in exercise classes for some Overcoming barriers to exercise people with lipoedema. Solutions may and physical activity include enrolling in single sex classes, Te term ‘exercise’ may be worrying and wearing a sarong between the changing imply high intensity exercise in a gym, rooms and pool, and asking a friend or particularly for people who are mainly relative to attend, especially when starting a sedentary, have severe lipoedema and/or new class.

SKIN CARE AND PROTECTION

SECTION 6: SKIN CARE AND PROTECTION Patients with lipoedema should be a spreading bacterial infection of the skin encouraged to pay particular attention to and subcutaneous tissues (Al-Niami & Key points gently drying any skin folds after washing, Cox, 2009). Local signs include warmth, 1. Skin folds may be and to applying appropriate emollients on swelling, erythema, pain and lymphangitis prone to fungal a daily basis, particularly when the skin is (inflammation of the lymphatic vessels that infections and dry (Williams & MacEwan, 2016). Folds may be seen as red streaks), and are often should be washed in the skin may be prone to irritation and accompanied by raised body temperature and dried with care the development of fungal infections that and feeling unwell. Skin necrosis and abscess 2. Patients with require treatment with antifungal agents formation can occur (Morris, 2008). Prompt lipolymphoedema (Langendoen et al, 2009). treatment with antibiotics is required: severe are at increased risk cases may require intravenous antibiotics of cellulitis. Where feasible, patients may prefer to avoid (Al-Niami & Cox, 2009). procedures such as taking routine blood samples, injections and blood pressure in More information about the treatment of lipoedema-affected areas (Todd, 2016). cellulitis in lymphoedema, which would be relevant to those with lipolymphoedema, Patients with lipolymphoedema are at can be found in the recently updated increased risk of cellulitis and should be guidelines from the British Lymphology advised to protect themselves from insect Society and Lymphoedema Support bites, burns, scratches and other skin Network (BLS/LSN, 2016). injuries in the affected areas. Cellulitis is

22 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA REFERENCES COMPRESSION THERAPY

SECTION 7: COMPRESSION THERAPY

As the names suggests, compression severe peripheral neuropathy and uncontrolled therapy is designed to exert pressure heart failure (Lymphoedema Framework, on body tissues. In lipoedema, the use 2006; Wounds UK, 2015). of compression therapy has three main purposes: Careful assessment of a patient with ■ To reduce discomfort, aching and pain lipoedema is required to determine which by supporting the tissues type(s) of compression therapy are indicated. ■ To support tissues and streamline Assessment should include the severity uneven, distorted limb shape and so and extent of the lipoedema, including the by reducing mechanical impairment to presence of skin folds or fat lobes, whether movement, improve mobility oedema is also present, the presence of pain, ■ To reduce oedema in lipolymphoedema the goals of treatment and the patient’s ability by reducing interstitial fluid formation to tolerate and self-manage compression and encouraging venous and lymphatic garments. In practice, availability on return (Reich-Schupke et al, 2013; NVDV, prescription is a major influencing factor in 2014; Fetzer & Fetzer, 2015). the type of compression therapy selected.

Compression therapy will not reverse the Clinicians will need to explain the rationale adipose tissue enlargement of lipoedema for the use of compression therapy and the (Fetzer, 2016). Terefore, unless there is need for daily wear and long-term use to oedema present, compression therapy maximise concordance. will not produce a reduction in limb size. Apparent decreases in limb size may occur Types of compression therapy while wearing compression therapy, however, Tere are several types of compression due to the streamlining effect of compression therapy (Table 7, page 24). Te type used therapy in limbs that are considerably most commonly for patients with lipoedema distorted in shape by tissue lobes or pads. is the compression garment (called compression hosiery when used on lower Compression therapy may also prevent limbs). In patients with lipolymphoedema, lipoedema worsening and decrease the multi-layer inelastic bandaging may risk of progression to lipolymphoedema, be used initially to reduce the oedema although evidence for such effects is not (Lymphoedema Framework, 2006). Compression or currently available (Fonder et al, 2007; containment? Langendoen et al, 2009; Todd, 2010). By Compression garments can be bought For patients who find aiding mobility, patients with lipoedema who ready-to-wear (‘off-the-shelf’) or can compression therapy wear compression therapy may be able to be be custom-made to an individual’s difficult to tolerate and who more active (Reich-Schupke et al, 2013). requirements. Te fabric used in have ‘pure’ lipoedema (i.e. compression garments may be: do not have lipolipoedema), Over time, consistent use of compression ■ Circular knit — garments are produced the concept of containment therapy may reshape limbs to a degree; by knitting on a round knitting cylinder may be helpful. Te in particular it may help to reduce ankle to produce a shaped fabric tube that does word containment may cuffing (Reich-Schupke et al, 2013; Hodson not have a seam; they tend to be thinner communicate better the intended purpose of the & Eaton, 2013). Once lipolymphoedema has than flat knit garments, but more likely to compression garments developed, compression therapy becomes cut in to soft skin or around lobes ■ in these patients, i.e. especially important (Todd, 2010). Flat knit — garments are usually supporting the tissues produced by knitting a flat shaped piece to improve the shape, Assessment and contraindications of fabric that is then stitched together contour and also possibly Before the selection of compression therapy, with a longitudinal seam; some garments the function of the affected vascular assessment according to local protocol have seam-free sections; they tend to areas, while not implying is essential to determine whether arterial be thicker and firmer than circular knit that the volume or condition compromise is present and to what extent. garments and more suitable when there of the affected area will be Compression therapy is contraindicated in is uneven or distorted limb shape (Clark improved. patients with severe peripheral arterial disease, & Krimmel, 2006).

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 23 COMPRESSION THERAPY

Table 7. Compression types and roles in the management of lipoedema (Lymphoedema Framework, 2006; Hodson & Eaton, 2013; Reich-Schupke et al, 2013; Wounds UK, 2015; Williams and MacEwan, 2016; Todd, 2016; Williams, 2016; Fetzer, 2016)

Type Description Notes and role in the management of lipoedema Compression garments • Available ready-to-wear or custom-made • Custom-made flat knit garments may allow more accurate fitting • Available in a range of styles, e.g. hosiery and accommodate uneven or distorted limb shape better than circular (below knee, thigh length, leggings, tights, knit garments capri-style) with or without feet, and arm • In patients who have skin folds, fat lobes and soft tissues, ready-to-wear sleeves circular knit garments may be prone to cutting into tissues and may cause • Often available in a limited colour selection distal oedema; custom-made flat knit garments may be more appropriate only (beige or black), although some • If the feet are not involved, there is no oedema and no risk factors for oedema, companies provide a wider range of colours footless garments can be prescribed • Garments may be available with hook and loop (VELCRO®) or zip fastenings • Applicators may be required to assist with donning and doffing

Adjustable compression • Sections of inelastic fabric joined together • Designed to allow easy application and removal by the patient or carer; often wraps that wrap around the limb and are secured easier to use than compression garments by straps with hook and loop fixings • Mainly used to reduce oedema • More rigid than compression garments • More rigid than compression garments and so less likely to cut in • Available in a variety of styles for the upper • Level of compression for different sections can be adjusted easily and lower limbs, including below knee/ • More durable than compression garments elbow +/- foot/hand; full-length limb length • May be used post-operatively following liposuction +/- hand/foot; thigh Compression bandaging • Multi-layer inelastic bandaging systems • Used to reduce oedema, particularly in severely distorted and painful limbs (N.B. In the USA, bandages usually comprise tubular bandage, a padding • Generally not used for pure lipoedema are sometimes called wraps) layer and inelastic bandages • Can be applied to legs or arms • May need to be reapplied daily especially during initial use as the oedema reduces • Bandaging of toes or fingers may also be required if affected by oedema • Often need to be applied by a clinician; self-application is not easy, but can be taught

Intermittent pneumatic • An inflatable plastic garment with one or • Main indication is reduction of oedema, which is achieved through the compression (IPC) more chambers that are inflated and deflated peristaltic massaging effect produced by the inflation/deflation cycles cyclically by an electrical air pump; sessions • May help to reduce pain, even in the absence of oedema last 30–120 minutes

Compression therapy selection Informed choice and shared Selection of compression therapy (Figure 5, decision making page 25 and Table 7) for patients with A patient’s willingness to wear and ability to lipoedema needs to take account of a wide tolerate compression therapy is key to ensur- range of factors including: ing concordance. Engaging the person in ■ Location, extent and severity of: the decision-making process, ensuring that - Tissue enlargement they understand why compression therapy - Shape distortion is being prescribed and what the benefits are - Deep skin folds and fat lobes likely to be, as well as empowering them to ■ Presence and degree of accompanying: manage the garments/devices themselves are - Pain or tenderness important for successful implementation. - Secondary oedema (lipolymphoedema) ■ Lifestyle, mobility and preference Despite the recommendation that patients ■ Access to and availability of compression with lipoedema wear compression as much as type, and to the expertise needed to possible, every day and during exercise (Fetzer apply/fit and use the compression safely & Wise, 2015; Hardy, 2015), Lipoedema UK’s and optimally (particularly important for Big Survey 2014 revealed that only 55% of made to measure garments) respondents did so ‘most days’ or ‘every day’ ■ Availability on prescription/cost. (Fetzer & Fetzer, 2016). Te main barriers to

24 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA COMPRESSION THERAPY

wearing compression were reported to be ■ Several options may need to be tried before Useful resources discomfort and difficulty putting it on (Fetzer finding the products and treatment regi- ■ Selection and use of & Fetzer, 2016). mens that best suit the patient. compression hosiery — see: Wounds UK A recent survey revealed that 50% of Finding products that are acceptable to the Best Practice Statement patients who used compression garments patient and providing effective symptom relief Compression Hosiery found them unhelpful, most often is critical to a good long-term outcome and (2nd edition). London, due to poor fit. Patients who received to ensure cost-efficiency of care (Williams & Wounds UK, 2015. garments from specialist clinicians MacEwan, 2016). Available from: www. based in a lymphology clinic had a wounds-uk.com better experience (Fetzer & Wise, 2015). Compression garments and ■ Use of compression Individual preference, accurate fitting adjustable wraps therapy, including and the provision of advice or devices to For patients with mild to moderate tissue bandaging, in the aid donning and doffing should therefore enlargement and no obvious oedema, management of be given high priority when planning a circular knit, ready-to-wear compression lymphoedema — see: compression regimen. garments are usually the first choice. Best Practice for Where there is more significant tissue the Management of Clinicians need to discuss options after enlargement with soft skin, deep skin Lymphoedema. London, ascertaining the patient’s priorities. Devising folds and fat lobes, circular knit garments MEP Ltd, 2006. personalised strategies that meet a patient’s are likely to cut in to tissues. For these Available from: www. needs may require a creative and flexible patients, flat knit custom-made garments woundsinternational. approach, e.g.: are more suitable because the fabric is com and www.lympho. ■ Starting at low levels of compression and more rigid and able to bridge skin folds org/publications/ building gradually may improve tolerance without cutting in.

N.B. This algorithm is a guide Patient with lipoedema - the compression regimen for a particular patient should be individualised to take account of all of their needs

No oedema Oedema present (lipolymphoedema)

Mild to moderate Moderate to severe Mild to moderate Moderate to severe enlargement enlargement enlargement enlargement No deep skin folds or fat Fat lobes and deep skin folds No deep skin folds or fat Fat lobes and deep skin folds lobes lobes

• Class 1 ready-to-wear • Class 1 or 2 made • Minor oedema: • Multi-layer bandaging circular knit, made- to measure flat knit Class 1 or 2 ready- until oedema, and to-measure, or sports garments to-wear circular knit/ pain if present, is skins/compression • Adjustable compression made-to-measure or sufficiently reduced to clothing or burns wrap if patient has adjustable compression a level where Class 1 garments difficulty applying wrap if problems with or 2 made to measure • If pain or tissue flat knit garments or toleration or donning/ flat knit garments tenderness make is hindered because doffing or adjustable donning the garment of pain or tissue • More extensive compression wraps difficult or hinders tenderness oedema and/or severe are appropriate and the patient from pain: tolerable tolerating it, adjustable Consider course of compression wraps may multi-layer bandaging to provide the patient with reduce oedema to level additional control where compression garments or wraps are appropriate

Figure 5: Compression therapy choice in lipoedema

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 25 COMPRESSION THERAPY

Adjustable compression wraps may be easier as standing, walking, exercising or travelling and less painful to apply than compression (Fetzer & Wise, 2015). garments. Tese also have the advantage of allowing the patient to adjust the degree Lipolymphoedema of compression to some extent, and are Te type of compression therapy suitable for less likely to cut in to soft tissues. Another the treatment of lipolymphoedema is option may be garments with hook and dependent on the degree of tissue eye fastenings intended for wear after enlargement and shape distortion and pain. liposuction. Multi-layer bandaging may For patients without significant shape be helpful for particularly painful limbs as distortion, compression garments may be the level of pressure can be adjusted and sufficient to reduce and control oedema. additional padding added where needed. However, for more severe oedema and in patients with significant shape distortion, Alternatives to prescribed compression multi-layer bandaging may be necessary to garments for patients with mild lipoedema reduce oedema to the point where without significant shape distortion or reassessment for compression garments is oedema include sports skins/compression feasible. clothing or burns garments, both of which may be softer but provide support. Some patients with lipolymphoedema and However, such garments are not always moderate/severe oedema may need to available on prescription and advice from a receive therapy in line with best practice specialist practitioner may be required. for the management of lymphoedema (Lymphoedema Framework, 2006; Fonder et Combining compression al, 2007). Tis may include manual lymphatic therapy types drainage (MLD) (see page 28), multi-layer For some patients, it may be possible to bandaging, skin care, and exercise and combine types of compression therapy. movement. For example, for a patient with severely enlarged thighs but lower legs that are Measuring for and prescribing affected to a lesser extent, an adjustable compression garments and adjustable compression wrap for the thighs or compression devices compression shorts (Bermuda-style or Measuring for compression garments and longer Capri-style) could be combined with adjustable compression devices is a complex hosiery for the lower leg (Fetzer, 2016). task and should be undertaken by a clinician Similar combinations may also be helpful with specialist training and experience in for patients who find it difficult to put on selecting the most appropriate type, style, full-length stockings. fastening type and compression strength of garment or device. Ease of application and It may also be appropriate for patients to removal should be given high priority in have different types of compression therapy the decision process. Ensuring an accurate for different activities or situations, e.g. to fit is essential to encouraging wear and to use adjustable wraps when at home and preventing problems. circular knit garments when going out or exercising (Williams & MacEwan, 2016). Once a compression garment or adjustable compression device has been selected and is Compromise available, an experienced clinician will need Despite these options, therapeutic to assess fit, teach the patient/carer how to compromise may sometimes be necessary don and doff, and explain care and the review/ to ensure that the patient is wearing at least replacement process (Box 14, a low level of compression and does not page 27). Te need for garment/device disengage with treatment (Hodson & Eaton, renewal provides an opportunity to review 2013). Patients who are very reluctant to wear progress and outcomes, and to check compression therapy, could be encouraged to concordance, suitability of style and level of wear it during activities that are more likely to compression, fit and the patient’s/carer’s ability cause discomfort or additional swelling, such to put on and take off the garment.

26 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA COMPRESSION THERAPY

Box 14. Tips for the session for the first fitting of a compression garment or adjustable compression device (Hardy, 2015; Fetzer & Wise, 2015; Wounds UK, 2015) ■ Check that the garment fits well, e.g. does not dig into tissues, particularly at the ankles and knees ■ Demonstrate to patients and carers how to don and doff the garment or device including how to spread the fabric evenly, and help them to practise doing the same ■ Advise on application and removal aids — the use of an applicator, along with closely fitting rubber gloves and non-slip matting, is often invaluable; many application and removal aids are available on prescription ■ Educate the patient and carers about when to remove the garment and who to contact if there are problems — signs that indicate compression should be removed immediately include increased pain, numbness, pins and needles or discoloured digits ■ Manage expectations — explain that compression/containment is not a cure and, unless oedema is present will not reduce limb size, but may improve limb outline and improve symptoms ■ Explain care of the garments — the manufacturer’s recommendations should be followed: some garments/devices can be machine washed but others need to be hand washed; in general, harsh detergents and fabric softener should be avoided, and the garment/device should be air dried rather than tumble dried ■ Discuss short-term and long-term review and renewal schedules — generally compression garments need to be replaced on average every 6 months.

Intermittent pneumatic compression do not want to use compression garments Te main use of intermittent pneumatic or devices (Fetzer, 2016). Patients also compression (IPC) (Table 7, page 24) is as report that IPC helps to reduce pain and an adjunct to other forms of compression discomfort even in the absence of oedema therapy to reduce oedema in patients with (Reich-Schupke et al, 2013). lipolymphoedema (Rapprich et al, 2015). IPC is thought to reduce swelling due to oedema Patients with lipolymphoedema who in two ways: to reduce oedema formation by have pain and tenderness may find opposing capillary filtration and to encourage IPC difficult to tolerate unless used at oedema resolution by increasing venous and very low pressures. Home use of IPC is lymph flow (Feldman et al, 2012). possible after careful assessment: some clinics will loan IPC devices, alternatively IPC is also sometimes used as an alternative individuals can also buy devices in patients with lipo-lymphoedema who independently.

Key points produces a thicker more rigid fabric. Tese garments may 1. Compression therapy is used in lipoedema to reduce be more suitable if there is considerable limb distortion pain and support tissues. In lipolymphoedema it is 8. Adjustable compression wraps may be useful for also used to reduce swelling due to oedema patients who find applying garments difficult or 2. Compression therapy does not reverse adipose tissue painful, and can be used alongside compression enlargement garments applied to other body areas 3. Patients being considered for compression therapy 9. Multi-layer bandaging may be useful in patients with should undergo arterial assessment to exclude lipolymphoedema as an initial step to reduce oedema peripheral arterial disease and/or pain to a level where garments become 4. Choice of compression therapy depends on a wide manageable range of factors, including individual choice and 10. Measurement and fitting of compression garments ability to manage should be undertaken by appropriately trained and 5. Te main type of compression therapy used in competent clinicians lipoedema is compression garments 11. Garments generally need to be replaced every 6. Most ready-to-wear garments are circular knit, 6 months which produces a thinner fabric that may be more 12. Intermittent pneumatic compression (IPC) may prone to cutting into tissues be used as an adjunct to compression therapy in 7. Most custom-made garments are flat knit, which patients with lipolymphoedema.

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 27 OTHER NON SURGICAL APPROACHES

SECTION 8: OTHER NONSURGICAL APPROACHES

Tere is no definitive evidence that Manual lymphatic drainage Box 15. Finding an MLD kinesiology taping provides benefits to Manual lymphatic drainage (MLD) is a practitioner very specific but gentle type of massage patients with lipoedema as very little carried out by qualified specialists/ research has been conducted so far. ■ Further details about practitioners (Box 15). MLD moves the However, there are anecdotal reports that MLD and a list of skin in order to stimulate the activity of kinesiology taping improves symptoms such registered MLD lymph vessels to remove interstitial fluid as knee pain and plantar fasciitis. It may practitioners can be and relieve oedema. also help to streamline limb shape. found at: www.mlduk. org.uk/therapists/ Studies of the effect of MLD have found Patch testing is required to ensure no skin ■ Te British Lymphology that the therapy reduced sympathetic sensitivity to the tape. Once applied, the Society has a directory nervous system activity (involved in tape is left in place for up to 3–4 days. of lymphoedema ‘fight or flight’ reactions) and increased Patients can be taught the technique for use treatment services, parasympathetic nervous system activity at home (Fetzer, 2016). including MLD, at: (involved in the ‘rest and digest’ state) www.thebls.com/ (Kim et al, 2009; Kim, 2013). A review Other treatment modalities directory/ of evidence for the effect of MLD in Two other approaches used by some in lymphoedema concluded that there is the management of lipoedema may act by some evidence that MLD reduces pain stimulating lymphatic drainage. Tere are and discomfort and promotes physical and anecdotal reports that these methods help psychological functioning (Haesler, 2016). to manage symptoms and reduce oedema. However, no research is yet available MLD can be used as part of the treatment to demonstrate benefit in patients with regimen for patients with lipoedema to help lipoedema. manage symptoms and when compression ■ Electrostatic massage therapy (Deep therapy does not control oedema Oscillation®) — a device is applied to sufficiently (Rapprich et al, 2015). However, the skin in a massaging movement by a Key points patients with pure lipoedema report that therapist holding the device with a glove 1. Manual lymphatic MLD may also help to relieve pain and hand while the patient holds a linked drainage (MLD) discomfort (Todd, 2016). electrode; this creates an electostatic stimulates the activ- effect in the tissues that is suggested may ity of the lymphatic Although little research has been done reduce pain and inflammation (Teo et al, system and may be specifically on the use of MLD in the 2016) used in conjunction treatment of lymphoedema and it is ■ Self-lymphatic drainage or dry skin with compression not generally available via the NHS brushing — stroking movements using therapy to reduce (Langendoen et al, 2009), many patients feel the hand or a very soft dry bristled brush oedema and control that they derive psychological benefits from may be used; to promote lymphatic symptoms such as the opportunity to relax. drainage centrally, the patient may use pain in lipolymph- breathing techniques or massage on the oedema Kinesiology taping trunk followed by stroking movements 2. Some patients with Kinesiology taping involves the application of on the limb, always moving towards the pure lipoedema a series of narrow strips of stretchable adhesive centre of the body. Care must be taken find MLD helps to tape to the skin over the area to be treated. not to traumatise the skin (Fetzer, 2016; reduce pain and It was first developed in Japan and has been Williams & MacEwan, 2016). discomfort used most widely to treat sports and other 3. Kinesiology taping soft tissue injuries (Kalron & Bar-Sela, 2013). may help to improve It is thought that the tape moves and lifts the blood and lymph skin and subcutaneous tissues to improve circulation and to blood circulation and lymph drainage (Wu et stabilise and realign al, 2015), and may help to stabilise and realign tissues and joints tissues and joints (Kurt et al, 2016).

28 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA SURGICAL MANAGEMENT

SECTION 9: SURGICAL MANAGEMENT

Surgical options that may be appropriate with pre-operative scores (p<0.001 for for some patients with lipoedema include change in each item score) (Rapprich et Box 16. Definitions of liposuction (to treat the tissue enlargement) al, 2015). Patients received a mean of 2.61 liposuction and bariatric and bariatric surgery (to treat obesity) (Box (range 1 to 6) sessions of liposuction. surgery (Shridharani et al, 16). However, while both types of surgery 2014; Albaugh et al, 2016) may help with symptoms, neither has been Tere is also evidence of longer-term ■ Liposuction: shown to be curative of lipoedema itself. benefits of liposuction. A study sent a the removal of questionnaire to 112 patients who had subcutaneous adipose Liposuction undergone tumescent liposuction between deposits via a cannula Liposuction (Box 16) should be carried out 5 and 11 years previously and who had also attached to a suction by a surgeon who is appropriately qualified been evaluated by questionnaire four years device that is inserted to treatment someone with lipoedema and before (Schmeller et al, 2012; Baumgartner through small incisions who works as part of a multidisciplinary et al, 2016). Responses were received from in the skin. Large team. 76% of patients. Changes over time in volumes of fatty tissue seven parameters (including pain, bruising, can be removed from areas such as the Access to liposuction within the NHS is oedema, mobility and quality of life) that legs, hips and arms. often limited and where available may be contributed to an overall impairment score Liposuction is one of classified as a cosmetic procedure for which were examined. the most commonly patients with lipoedema do not qualify. performed cosmetic Advocacy groups such as Lipoedema UK Te significant reductions in pre-operative surgical procedures. are endeavouring to change the situation and post-operative scores for each item Tere are numerous and to have liposuction recognised as an and for overall score (all p<0.001) noted types of liposuction, effective surgical treatment for patients at 4 years were also present after 8 years including tumescent with lipoedema. (Schmeller et al, 2012; Baumgartner et al, and water-jet assisted. 2016). However, studies are awaited that Te type used in the Due to lack of NHS provision and/or long present data for the longer-term outcomes treatment of lipoedema may relate to the waiting lists, patients may decide to source that are relevant for this patient group. preferences of the liposuction privately in the UK or abroad. individual surgeon. Patients should research clinics carefully Advising patients with lipoedema ■ Bariatric surgery: to ensure an adequate standard of care and Patients with lipoedema considering gastrointestinal surgery to establish that the procedures on offer liposuction should be advised and used to treat obesity are appropriate for their individual needs. encouraged to undertake non-surgical that aims to reduce Tey need to be aware that liposuction treatment for at least 6-12 months as a first intake of food or reduce procedures are not without risk in the step. Box 17, page 30, lists factors that may be absorption of food immediate post-operative period and may considered by surgeons when assessing a from the gut. A wide cause long-term complications (Stutz & patient’s suitability for liposuction. range of procedures Krahl, 2009; Rapprich et al, 2015). is available including gastric banding, partial Pre-operative counselling is very important gastrectomy and gastric Effects of liposuction in lipoedema to ensure that the patient has realistic bypass. Overall, liposuction in patients with expectations of what can be achieved, lipoedema reduces tissue bulk, pain understands the procedure and the and bruising, and improves mobility, importance of post-operative care (including functioning and quality of life (Reich- compression therapy), and comprehends Schupke et al, 2012; Peled & Kappos, 2016). that there is no evidence that liposuction is curative (Box 18, page 31). Provision of such A study of tumescent liposuction in 85 advice is highly variable. Consequently, it patients with lipoedema found that six may fall to lipoedema clinic staff to ensure months after surgery patients’ scores for a that patients have had an opportunity to wide range of symptoms, including pain, discuss these issues. bruising, swelling and impaired mobility, were all significantly reduced in comparison

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 29 SURGICAL MANAGEMENT

Box 17. Factors that may be considered by surgeons when assessing the suitability of a patient with lipoedema for liposuction

■ Lipoedema stage: individual surgeons indicate a high risk of bleeding during use different lipoedema stage criteria for surgery; if the risk is severe surgery may be indicating suitability for liposuction contraindicated ■ Concomitant conditions: patients should ■ Ability and willingness to tolerate not have medical conditions that increase compression therapy: compression the risk of complications from anaesthesia therapy is very important post-operatively ■ Weight management: non-lipoedema ■ Psychological status: liposuction for fat should have been reduced as much as lipoedema has a long recovery period and possible before surgery; patients need a so psychological resilience and mental good understanding of nutrition and the wellbeing are important to cope with need to avoid weight gain post-operatively and motivate self-care and ongoing wear ■ Condition of the skin and tissues: these of compression garments; patients with will indicate how well the patient will heal body dysmorphic disorder (BDD) may and the likelihood of being left with excess require psychological treatment before skin consideration for surgery ■ Presence of oedema: a course of ■ Pain management requirements: patient’s decongestive therapy and compression current pain levels and management therapy may be required prior to surgery if strategies will help to inform discussions oedema is present about post-operative requirements. ■ Bleeding tendency: the presence of varicose veins or bleeding disorders may

Care after liposuction beyond the NICE has published guidelines on the immediate post-operative period may fall criteria for considering bariatric surgery on lymphoedema clinics, and may prove (NICE CG189, 2014). Tese include challenging if the patient has been abroad patients with BMI ≥40 kg/m2 or 35-40 kg/m2 and returns with little information about with type 2 diabetes or hypertension who the procedure that has been performed and have tried all appropriate non-surgical required aftercare. measures to achieve weight loss.

Patients need to be advised to continue wearing the compression garments prescribed and may need advice on pain management, garment application and care, and who to contact if there are problems. Psychological support and encouragement may also be needed.

Te post-operative swelling and pain take at least several months to resolve and may be perceived by the patient as signs of deterioration.

Bariatric surgery Bariatric surgery is not in itself a treatment for lipoedema, but as described previously weight reduction from areas of the body not affected by lipoedema or prevention of further weight gain in patients who are obese may be beneficial.

30 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA SURGICAL MANAGEMENT

Box 18. Pre-operative counselling for patients with lipoedema undergoing liposuction

■ Liposuction aims to reduce fat tissue, improve limb/body shape and mobility, and reduce symptoms such as pain, but there is no guarantee that the condition will not deteriorate later ■ A series of liposuction sessions may be necessary over several months, and plastic surgery may be required if large amounts of lax skin remain ■ Liposuction can be carried out under general or local anaesthetic ■ Risks of surgery include haemorrhage, infection, scarring, wound healing problems, altered sensation, deep vein thrombosis (DVT), pulmonary embolus, fat embolus and loose skin ■ Liposuction is not a ‘quick fix’: pain, swelling and bruising will be marked for several months after surgery; it may take months for post-operative swelling and numbness to resolve fully; full recovery can take up to 12 months. ■ Made-to-measure flat knit compression garments or adjustable wraps are applied immediately after surgery, and need to be worn for at least several months afterwards, if not on an ongoing basis (‘for life’) ■ A patient choosing to have private surgery needs to understand that any pre- and post- operative care, e.g. MLD, bandaging and compression garments, may not be available in NHS lymphoedema clinics, and may need to be sourced privately ■ Professional measurement for and fitting of compression garments is necessary to ensure correct fit and function; self-measurement is not likely to be accurate ■ Weight management and physical activity, e.g. walking, continue to be very important post-operatively.

Key points 1. Tere is no evidence that liposuction cures lipoedema, but it may reduce limb bulk and so improve functioning and mobility 2. Patients should be advised to try at least 6-12 months’ non-surgical treatment before undergoing liposuction 3. Pre-operative counselling is important to ensure patients understand the non-curative nature of liposuction, the long often painful post-operative course, and the need for ongoing wear of compression therapy 4. Bariatric surgery may be indicated for some patients with lipoedema who are also obese.

BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA 31 APPENDICES

APPENDIX 1: THE LYMPHATIC SYSTEM Function Structure Te lymphatic system plays an essential role in fluid Lymphatic vessels and lymph nodes form a network that balance, the immune system and nutrition. Fluid leaks into returns lymph eventually to the blood circulatory system tissue spaces from the blood in capillaries to provide cells via the subclavian veins. Tere are three main types of with nutrients, oxygen and fluid. Formerly, it was thought lymphatic vessel: that most of this interstitial fluid was reabsorbed into the ■ Initial lymphatics – blind-ended, non-contractile vessels venous end of the capillary. However, it is now known that that absorb lymph and drain into pre-collectors most of the interstitial fluid is taken up into the lymphatic ■ Pre-collector lymphatics – vessels that contain valves to system and eventually drains back in to the venous prevent back flow of lymph and that drain into collector circulation (Mortimer & Rockson, 2014). lymphatics ■ Collector lymphatics – vessels that contain one-way Te fluid in lymphatic vessels is known as lymph. In valves and that can contract (Adamczyk et al, 2016). addition to interstitial fluid, it contains immune cells and proteins (Adamczyk et al, 2016). Lymph draining from the Collector lymphatics contract rhythmically in response to gut also contains fat (Lasinski, 2015). distension to pump the lymph towards the venous system via the lymph nodes, the thoracic duct and right lymphatic Tissue oedema occurs when the amount of interstitial fluid trunk. Lymph flow is also assisted by pulsation of nearby formed exceeds the amount removed by the lymphatic arteries, skeletal muscle contraction and variations in system. Tis may be due to increased leakage from intrathoracic pressure during breathing (Adamczyk et al, capillaries, e.g. as may occur in inflammation, and/or 2016). inadequate removal by the lymphatic system (Mortimer & Rockson, 2014).

32 BEST PRACTICE GUIDELINES: THE MANAGEMENT OF LIPOEDEMA APPENDICES

APPENDIX 2: PATIENT PATHWAY

Patient presents with bilateral tissue enlargement

General practitioner suspects or diagnoses lipoedema Initiate further investigation, treatment • Initial routine blood tests, e.g. urea and electrolytes, thyroid function tests, or referral as appropriate and/or as plasma proteins, glucose, brain natriuretic peptide (BNP) indicated by results of blood tests • Referral to lipoedema/lymphoedema service/clinic

Referral as appropriate, e.g.: Lipoedema/lymphoedema service/clinic • Pain management • Confirmation of diagnosis and further investigations if required • Dietitian • Initial assessment, including: • Physiotherapy - Site, extent and shape/disproportion of tissue enlargement; weight • Occupational therapy - Presence of oedema/test for Stemmer’s sign • Counselling/psychological therapy - Assessment for chronic venous insufficiency (CVI) • Leg ulcer management - Pain and psychological assessments • Dermatology - Assessment of functioning and mobility

• Education • Skin care • Healthy eating/weight management (diet) • Treatment of concomitant conditions • Physical activity • Support with and encouragement of self management

No oedema Oedema ( lipolymphoedema)

Mild to moderate Moderate to severe Mild to moderate Moderate to severe enlargement enlargement enlargement enlargement No deep skin folds or fat Fat lobes and deep skin folds No deep skin folds or fat Fat lobes and deep skin folds lobes lobes

• Minor oedema • Class 1 ready-to-wear • Class 1 or 2 made- • Multi-layer bandaging Class 1 or 2 ready-to-wear circular knit or sports to-measure flat knit until oedema, and circular knit/made-to- skins/compression garment pain if present, is measure or adjustable clothing or burns • Adjustable compression sufficiently reduced to compression wrap if garments wrap if patient has a level where Class 1 problems with toleration or • If pain or tissue difficulty applying or 2 made-to-measure donning/doffing tenderness make flat knit garments or flat knit garments • More extensive oedema donning the garment is hindered because or adjustable and/or severe pain: difficult or hinders of pain or tissue compression wraps Consider course of multi- the patient from tenderness are appropriate and layer bandaging to reduce tolerating it, adjustable • Consider MLD tolerable oedema to level where compression wraps may • Consider IPC • Consider MLD compression garments or provide the patient with • Consider IPC wraps are appropriate additional control • Consider kinesiology • Consider MLD • Consider MLD taping • Consider IPC • Consider kinesiology taping

Monitor outcomes regularly, aiming for outcomes as agreed with the patient which may include: • Reduced pain For patients with moderate to severe lipoedema, consider • Reduced oedema referral for liposuction after 6-12 months of non-surgical • Improved mobility and functioning management • Enhanced self management

N.B. This algorithm is a guide - the compression and treatment regimen for a particular patient should be individualised to take account of all of their needs IPC: intermittent pneumatic compression; MLD: manual lymphatic drainage

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