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Lymphoedema - Clinical features and Management

Lymphoedema is an abnormal limb swelling caused by a buildup of high-protein fluid due to defective lymphatic drainage, affecting about 2% of people and often leading to emotional distress. It can be classified into primary and secondary lymphoedema, with various causes including infections, trauma, and malignancies. Early diagnosis and comprehensive management including compression therapy and possible surgical options are crucial to prevent complications and improve patient quality of life.

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LYMPHOEDEMA Dr R S Dhaliwal MBBS,M.S,DNB(Surg),M.Ch,DNB(CTV Surg) FACS,FCCP,FICA,FNCCP,FIACS Prof &HOD (Former) , CTV Surgery, PGIMER,Chandigarh
LYMPHOEDEMA • It is an abnormal swelling of limb due to the collection of excessive amount of high protein fluid secondary to defective lymphatic drainage in the presence of normal capillary filteration. • Lymphoedema affects around 2% of people and is common cause of limb swelling. It causes significant symptoms & complications It leads to emotional and psychological distress affecting relationships, education and work.Patient feels emberrassed for seeking treatment .It is often misdiagnosed and mistreated by doctors thinking it to be a cosmetic problem only . Early diagnosis and treatment can prevent the development of disabling late problems
Lymphoedema
Symptoms • Constant dull ache or severe pain • Burning and bursting sensations in limb • General tiredness and debility • Sensitivity to heat, cramps, pins and needles • Skin problems- dehydration, flakiness, weeping excoriation and breakdown • Immobility of patient leading to obesity and muscle weakness • Backache and joint problems • Athlete’s foot and acute infective episodes
Classification • Primary lymphoedema – cause not known but presumed to be due to ‘ congenital lymphatic dysplasia’ • Secondary lymphoedema – due to a specific cause like inflamation, malignancy or surgery • Grade (Brunner) Subclinical - No apparent lymphoedema, Excess I S fluid , HP changes in lymphatics and lymph nodes Gr I – Pitting oedema,Swelling subsides on rest or elevation of foot Gr II- Oedema does not pit and does not reduce upon elevation Gr III- Oedema associated with skin changes I e fibrosis, excoriation
Primary lymphoedema
Aetiological Classification • Primary lymphoedema • Congenital -onset <2 yrs Sporadic Familial (Milroy’s disease • Praecox( onset -2-35yrs ) Sporadic Familial(Meige’s dise ) • Tarda – onset – after 35 yrs old ) • Secondary lymphoedema Parasitic – Filariasis Fungus - Tinea pedids Exposure to F B particles silica particles Primary lymphatic malignancy Lymph nodes metastasis Radiotherapy to L N Surgical excision of L N Trauma –Degloving injury Supf. Thrombophlebitis Deep venous thrombosis
Differential Diagnosis • Non vascular or non lymphatics - *General disease states- CHF Liver failure Hypoproteinemia Hypothyroidism Allergic (Angioedema) Prolonged immobilisation –Lower limb * Local disease processes - Arthritis Haemarthrosis Bony or soft tissue tumours Calf mucle hematoma * Retroperitoneal fibrosis – Lymphatic * Drugs – Steroids, Oestrogens, MAO inhib *Trauma * Obesity * Gigantism ( rare, all tissues enlarged)
Secondary lymphoedema
Differential Diagnosis • Venous causes - DVT(Pain & redness absent) -Post thrombotic synderome -External compression- Pelvic or abdml tumour, Retroperitoneal fibrosis, uterus -Reperfusion for lower limb ischemia - Varicose veins (rare) -Rare synderomes • Arterial - A V malformations - Aneurysm - Popliteal femoral False aneurysm
Malignancies associated with lymphoedema • Lymphangiosarcoma ( Stewart Treves Synderme ) • Kaposi Sarcoma ( HIV, HID virus ) • Squamous cell carcinoma • Liposarcoma * Lymphoma • Malignant melanoma • Malignant fibrous histiocytoma • Basal cell Carcinoma
Secondary Lymphoedema • Most common form of lymphoedema caused by infection, inflamation,tumour and trauma • Filariasis -Commonest cause of lymphoedema worldwide, In Africa, India and South America Upto 10% population is affected.Caused by a nematode Wucheria bancrofti , spread by mosquito due to poor sanitation, its only host is man. The parasite enters lymphatics from blood and lodge in lymph nodes causing obstruction due to physical damage and immune response of host. Proximal lymphatics become grossly dilated causing massive oedema termed as Elephantiasis. Eosinophilia is present, Microfilariae enter blood at night and can be seen on a blood smear, urine or lymph itself. A C F test is +ve in present or past infection. Diethylcarbamazine kills parasites but does not reverse lymphatic changes. Treatment is to clear infection by drug and the treat lymphoedema as primary
Elephantisis duetoFilariasis Post mastectomy lymphedema
Features of Filariasia • Active - Fever Headache Malaise Inguinal and axillary lymphadenitis Lymphangitis Cellulitis,ulceration Abcess formation Epididymo orchitis • Chronic - Lymphoedema of legs, arm, breast Hydrocele Abdominal lymphatic varices Chyluria Lymphuria
Causes of secondary lymphoedema • Trauma & Tissue damage - Lymph node excision Burns Scarring Radiotherapy Varicose vein surgery / vein harvesting Large wounds • Malignant disease- Lymph node metastasis Infilterative carcinoma Lymphoma Pressure by large tumours • Venous disease- Chronic venous insufficiency Venous ulceration Post thrombotic synderome I V drug use • Infection- Cellulitis/ erysipelas Lymphadenitis Tuberculosis Filariasis • Inflamation - Rheumatoid arthritis Dermatitis Psoriasis Sarcoidosis Epidermal dermatosis • Endocrine Disease- Pretibial myxoedema • Immobility & dependency - Bed redden pts Paralysis • Factitious - Self harm
Conditions mimicking lymphoedema • Factitious lymphoedema- Caused by application of a tourniquet ( a start and sharp cut off is seen on examination) or hysterical disuse of limb in pts with psychological or psychiatric problems • Immobility lymphoedema- Generalised or localised immobility of any cause leads to chronic limb swellling e.g.elderly person who spends all day or night sitting in a chair (arm chair legs ), the hemiplegic stroke patient or young patient with multiple sclerosis • Lipoedema- Seen only in women as B/L symmetrical enlargement of legs and sometimes lower half of the body due to abnormal deposition of fat . It may or may not be associated with obesity .It can coexist with other causes of limb swelling.
Complications • Slow wound healing • Infection- Cellulitis – Lymphangitis - Lymphadenitis • Skin Ulcers ,thickening of skin ,follicles forms • Malignancy - Lymphangiosarcoma ( Stewart Treves Synderome) - Retiform haemangioendothelioma (low grade angiosarcoma )
Investigation of Lymphoedema • Are investigations necessary ? It is usually possible to diagnose and manage lymphoedema purely on the basis of history and examination when swelling is mild and there are no complicating features In pts with severe , atypical and multifactorial swelling investigations may help to confirm the diagnosis ,management and prognostic information
Investigations • Routine Tests - TLC,DLC RFT, LFT, Thyroid function tests, total plasma proeins,albumen , fasting blood sugar, urine exam for chyluria , blood smear for microfilariae, X ray chest and ultrasound • Lymphangiography- Direct method involves injection of contrast medium into peripheral lymphatic channel followed by radiographic visualisation of the lymphatic vessels and nodes . It is the gold standard for showing abnormalities of large lymphatics and lymph nodes. It can be technically difficult , is unpleasant for the pt , may cause further injury to lymphatics. As a routine it has become obsolete. Indirect lymphangiography involves intradermal inj. Of water soluble non ionic contrast into a web space it is taken up by lymphatics and is followed radiographically . It will show distal lymphatics but not proximal lymphatics and lymph nodes. • Isotope lymphoscintigraphy - This has become primary diagnostic technique in case of unccertainty. Radioacctve technitium labelled protein or colloid particles are injected into interdigital web space and taken up by lymphatics .Serial films are taken by a gamma camera.It provides a qualitative measure of lymphatic function. Quantitative function is performed using a dynamic ( exercise ) component and provides information on lymphatic transport
Investigations • Computed tomography - A single axial CT slice through the midcalf is a useful test for lymphoedema (coarse, non enhancing reticular honeycomb pattern in an enlarged subcutaneous compartment), Venous oedema (increased volume of the muscular compartment and lipoedema ( increased sub cutaneous fat). CT will diagnose a pelvic or abdominal mass lesion • MRI – It can provide clear images of lymphatic channels and lymph nodes and is useful in assessment of lymphatic hyperplasis,MRI can differentiate between venous and lymphatic causes of a swollen limb and can detect tumours causing lymphatic obstruction • Ultrasound- It can provide information about venous function like DVT and venous abnormalities • Pathological examination- If malignancy is suspected FNAC , neddle cone biopsy or surgical excision from lymph nodes is useful . Skin biopsy will confirm the diagnosis of lymphangiosarcoma
Management of Lymphoedema • Overview - History ( age of onset, location, progresssion,aggravating and relieving factors ) • Past & Family medical history ( H/O cancer) • Obesity (Diet, Ht and wt, BMI) • Complications –venous,arterial,skin,joint • Relief of Pain *Control of swelling • Skin care * Exercise • Manual lymphatic drainage (MLD) • Multilayer bandaging & compressive garments • Drugs *Surgery
Management • Skin care to treat infections and optimise condition of the skin • Manual Lymphatic Drainage (MLD) to enhance lymph flow.This precedes bandaging and directs lymph fluid to functional territories and helps to form collateral pathways • Application of multi layer compression bandaging • Exercise to increase lymphatic & venous flow- Massage and swimming are beneficial.Avoid vigrous exercises • A compressive garment is used to preserve the reduction acieved by treatment and prevent progression of lymphoedema • Educat ion and Psychosocial support
Management *Compressive Therapy - Use of compression garments is very important in treatment of lymphoedema. They apply medically proper pressure to the swollen region to reduce pooling of fluid. This preserve the limb size and shape , supports and improves lymphatic circulation. These garments are available in a variety of styles,sizes, colours and grades of compression (Class I- IV).More swelling needs stronger support.They come as Pre –sized (ready to wear) or custom made as a circular knit or flat knit design. They are worn during day and removed at night
Surgery • Bypass Procedures- In patients with proximal ilioinguinal obstruction and normal distal lymphatic channels , lymphatics bypass may help * Omental Pedicle * Skin bridge * Ileal mucosal patch * Lymph node to vein anastomosis * Direct lymphaticovenularanastomosis Technically these are demanding operations and there is no conclusive proof that they produce better results than best medical management .
By Pass operations
Surgery • Lymphatic liposuction – Using vibrating canulae excessive fluid, fat and thickened tissue is removed from the limb having advanced lymoedema. It has been shown to be an effetive method to reduce the size and stiffness of the affeted limb. It is less traumatic and a simple method comparing with standard excisional operations Low level Laser therapy- Started in 2008 This has been used in post mastectomy upper arm lymphoedema and hav e shown good results in decreasing size of the involved upper limb
Surgery Excisional Operations • They are indicated when a limb is so swollen that it interferes with mobility and livelihood. They are not ‘ cosmetic’ as they do not create a normally shaped leg and cause significant scarring. Various operations are- • Sistrunk - A wedge of skin and subcutaneous tissue is excised and wound is primarily closed Mainly done to reduce girth of the thigh. • Homans – Skin flaps are elevated then sub cutaneous tissue is excised beneath flaps , these are trimed in size and closed primarily.Good operation for calf . Main complication is skin flap necrosis.There should be 6 months gap between operations on medial and lateral sides of limb .Can be used for upper limb if there is no venous obstruction or malignancy
Surgery • Thompson operation - One skin flap is sutured to the deep fascia and buried under second skin flap (buried dermal flap).Cosmetic results are not better than Homans operation and formation of pilonidal sinus is common. • Charles operation – It was intially designed for filariasis and involved excision of all the skin and subcutaneous tissues down to deep fascia and covering with splitt skin grafts. This gives very unsatisfactory cosmetci result and graft failure is common.Advantage is that it reduces the size of massively swollen limb very much
Thomson operation Charles operation
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