Lymphedema/ Lymphoedema

1. LYMPHEDEMA
Dr Okoye
13/05/2019

2. Outline
 Introduction
– Introduction
– Definition
– Embryology
– Anatomy
– Physiology
– Historical perspective
– Epidemiology
• Statement of surgical importance
 Etiology
 Pathology
 Pathogenesis
 Pathophysiology

3. Outline
 Staging
 Diagnosis
– History
– Examination
– Investigations
 Treatment
– Medical
– Surgical

4. Outline
 Complications
 Follow up
 Prevention
 Peculiarities of our environment
 Advances
 Conclusion
 References

5. INTRODUCTION
• Lymphedema, also known as lymphoedema and
lymphatic edema;
• Is a condition of localized fluid retention and
tissue swelling.
• Caused by a compromised lymphatic system.
• The lymphatic system functions as a critical
portion of the body's immune system and returns
interstitial fluid to the bloodstream.

6. Definition
– Lymphedema is the accumulation of protein rich
interstitial fluid in the subcutaneous tissue and
skin of the extremity. which may be associated
with chronic inflammation and fibrosis or
hyperplasia.
Two broad types
– Medical
– Surgical

7. EMBRYOLOGY
• Closely related to blood vessel development to
understand concept
• Vasculogenesis; building blood vessels from
beginning and mostly completed in-utero (from stem
cells/ progenitors)
• Angiogenesis; new blood vessels grow from
extension or sprouting of existing vessels mostly post
natal
 Wound healing
 Menstrual cycle and pregnancy
 Tumor formation

8. EMBRYOLOGY Vasculogenesis

9. EMBRYOLOGY Lymphangiogenesis
Vascular Endothelial
Growth Factor
(VEGF)
+ Vasculogenesis
+ Angiogenesis
+ Increases vessel
permeability
+ Tumor angiogenesis

10. EMBRYOLOGY Lymphangiogenesis

11. ANATOMY
• Blind-ended lymphatic capillaries arise within the
interstitial spaces of the dermal papillae.
• These unvalved, superficial dermal lymphatics
drain into interconnected subdermal channels,
which parallel the superficial venous system.
• These subsequently drain into the deeper,
epifascial system of valved trunks lined with
smooth muscle cells and located just above the
deep fascia of the extremity.

12. ANATOMY
• A deeper-valved subfascial system of lymphatics is
responsible for the drainage of lymph from the fascia,
muscles, joints, ligaments, periosteum, and bone. This
subfascial system parallels the deep venous system of the
extremity.
• In lymphedema some reversed flow through perforators
from the epifascial to the subfascial system may occur as a
mechanism of decompression of the epifascial system.
• However, the derangement in lymphedema is almost
always exclusive to the epifascial lymphatic system, with
the subfascial system being uninvolved. Thus, the surgical
approaches to lymphedema focus on the epifascial system

13. PHYSIOLOGY
– lymphatic fluid is an ultra filtrate of plasma.
– Has the same electrolyte and some plasma
protein content of the plasma.
– Lymphatic capillaries (Zonal drainage)
– Primary lymph vessels valveless
– Secondary lymph vessels valved
– Sentinel nodes

14. PHYSIOLOGY
– Regional lymph nodes (arranged anatomically) in upper and
lower exteremities
– Watershed area of Sapey
– No communication between deep and superficial lymphatics
except at 2 points in the extremity
– also are aided by skeletal muscle contractions and pulsatile
movements of surrounding blood vessels to assist with lymph
flow

15. HISTORICAL PERSPECTIVE
• The 17th century saw several emerging and almost
simultaneous discoveries in the field of lymphology
by Asselli, Pecquet, Bartholin and possibly Joliffe.
• Lymphatic system was first described by Erasistratus
in Alexandra > 2000yrs ago
• Williams Hunter in 18th century was 1st to describe
the functions of the lymphatics
• Olof Rudbeck (1630-1708) was probably the first
anatomist to consider correctly the lymphatic
circulation as an integrated system of the whole body

16. EPIDEMIOLOGY
• Worldwide, the most common cause of
lymphedema is filariasis infection. More than 100
million people are affected in endemic areas
worldwide
• Prevalence is about 0.13 – 2% globally
• At birth 1 in 60,000 people
• Females account for 70-80% of cases of
congenital lymphedema.
• Apart from its symptoms, it is also a frequent
source of emotional and psychological distress

17. STATEMENT OF SURGICAL IMPORTANCE
• It's important that lymphedema is identified
and treated as soon as possible. If it isn't
treated, it can get worse.

18. ETIOLOGY
• MEDICAL LYMPHEDEMA:
– Increased Production increased vascularity
hydrostatic pressure
– Hypoproteinemia reduced oncotic pressure
– Medication
• SURGICAL LYMPHEDEMA:
– Intrinsic obstruction-
– Primary
– secondary
– Extrinsic obstruction (Abdominal /Pelvic tumors)

19. ETIOLOGY
•Primary lymphedema
• Lymphedema congenita (Milroy’s dx) – present
at birth
• Lymphedema praecox (Meige’s dx)– develops in
childhood or adolescence
• Lymphedema tarda – develops in adulthood

20. Hypotrichosis, lymphedema,
telangectasia syndrome
Early-onset hypotrichosis with absence of
eyebrows
and eyelashes
Lymphedema appears in childhood or
adolescence
PRIMARY HEREDITARY LYMPHEDEMA

21. Secondary Lymphedema
• Infection
• Filariasis
• Traumatic injury –
• Circumferential degloving injuries and avulsions
• Surgery
• Groin LN dissection
• Axillary LN dissection
• Radiation therapy
• Regional radiotherapy after regional node dissection
• Malignancy
– Breast
– Melanoma
– Prostate/Bladder
– Gynecologic Ovarian
– Lymphoproliferative disease

22. • Lymphatic Damage
• Sclerosis
PATHOGENESIS
 Protein/Sugar rich
 accumulates
 Acute/Sub Acute
infection
 cellulitis
 lymphangitis

23. PATHOGENESIS
• Albumin  Smaller
Peptides
 inflammation
 GF- bFGF
 MDGF
 EGF  fibrosis
 Skin
Thickening
 Hyperkeratosis
 Parakeratosis
 papilloma

24. • STARLING FORCES
– Increased capillary filtration
– Decrease interstitial absorption

25. STAGING
ISL/BRUNNER STAGING
sTAGE 0 /LATENT
• Impaired fluid transport
• No evidence of swelling/edema

26. STAGING
STAGE I /EARLY
• Accumulation of protein-rich edema fluid
• Pitting edema
• Reduces with elevation (no fibrosis)

27. STAGING
STAGE II / INTERMEDIATE
􀂙 Accumulation of protein rich edema
􀂙 Pitting becomes progressively more
difficult
􀂙 Connective tissue
proliferation

28. STAGING
• STAGE III / LATE
• Accumulation of protein rich edema fluid
• Non-pitting Fibrosis and sclerosis
(severe induration)
• Skin changes (papillomas, hyperkeratosis, etc.)

29. MANAGEMENT
• History
• Examination
• Investigations
• Treatment

30. •Address of the patient
•Differentiate between unilateral and
bilateral Lymphedema
•Age of the patient
•Duration of swelling
•History of ulcers/Lymphangitis
•Effects of Posture
•How fast did the swelling develop
/progress
•What is the underlying medical
disease
•Previous Surgery
•Family History
•Drug History -Adalat
HISTORY

31. PHYSICAL EXAMINATION
INSPECTION
•Compare the 2 limbs
•Extend of lymphedema with
measurement
•Old scar
•Current Lymphangitis
•Any skin changes
•Collateral veins
•Scars and/or Radiation skin
•Ulcers
•Papillomas

32. PHYSICAL EXAMINATION
PALPATION
􀂙Temperature
􀂙Stemmer sign
􀂙Skin fold
􀂙Pitting
􀂙Fibrosis
􀂙Muscular status

33. PHYSICAL EXAMINATION Circumference
• Most commonly used
method to assess limb
volume
• Measurements taken at
various points of arm or
leg
• Time-consuming
• Requires considerable
experience

34. Diagnosis
• Investigations
• Laboratory
– FBC & differential
– Serum protein and albumin
– U/E,Cr
– LFT
– Urinalysis
– Blood/Skin Snips for Microfilaria

35. Diagnosis
• Imaging
– Plain films
– ECG or & Echocardiogram
– USS
– CT scan
– MRI
– Lymphangiography
– Lymphoscintigraphy
– Perometry
• Biopsy and Histology

36. Goals of treatment
• Improve cosmesis
• Preserve skin integrity
• Soften subcutaneous tissues
• Avoid infection or lymphangitis
• Decrease limb size
• Avoid contracture of the involved limb
• Most importantly: patient and family
education

37. TREATMENT MODALITIES
CONSERVATIVE/MEDICAL
– Foot Care
– Treatment of cuts and infections (Medical)
– Complete Decongestive Therapy
SURGICAL
• Physiologic
– Microsurgical
– Macrosurgical
• Debulking
• Mixed Physiologic and Debulking

38. COMPLETE DECONGESTIVE THERAPY CDT
• Foot Elevation
• Lymph Drainage
– Manual Lymphatic Drainage (MLD)
– Mechanical Lymphatic Drainage Device
• Compression
• Exercise

39. What types of compression are available ?
• Compression stocking
• can be used for treatment and maintenance
• 2 pairs are appropriate to maximize hygiene
• last from 4 to 6 months
• can be difficult to don or doff and keep in position
• Compressive wrapping
• better flexibility for specific problem areas and for
specific patients
• patients and families can learn technique
• allow greater activity level than pumps
• can be time consuming to don
COMPLETE DECONGESTIVE THERAPY

40. Surgery
• Indications
– Failed conservative therapy
– Impaired function
– Gross extremity size and weight
– Recurrent lymphangitis (>3/yr)
– Severe skin changes

41. SURGICAL TREATMENT
• Physiologic
– Micro surgical Procedures (All Physiologic)
1. Lymphnodo-venous
2. Lympho-venous
3. Lympho-lympatic
4. Lympho -Capsular
– Macroscopic
1. Swiss Roll Operation (Thompsons Operation)
2. Omental transposition
3. Deepithelized Dermal Flaps
• Reduction
– Charles’/modified Charles’ procedure
– Miller’s procedure
– liposuction
• Reduction-Physiologic
– Homan (Sistrunk Operation)

42. Complications
• Of lymphedema
– Recurrent cellulitis and/or lymphangitis
– Bacterial and fungal infections
– Lymphangio-adenitis
– DVT
– Severe functional impairment
– Cosmetic embarrassment
– Necessitating amputation
• Of Surgery
– Partial wound separation
– Seroma
– Hematoma
– Skin necrosis
– Exacerbation of edema
– Lymphangiosarcoma

43. Follow up
• Wear compressive garments
• With improvement, do routine limb elevation
and compression therapy
• Document circumferential measurements

44. Prevention
• Avoid extreme temperature changes
• Wear rubber gloves when doing chores
• Always put on shoes even at home
• When traveling by air, patients with lymphedema or those at
risk must wear a compression sleeve or stocking
• Use only electric razors
• Avoid trauma (bruising, cuts, sunburn, insect bites, cat
scratches etc.) to affected area
• Call physician at first sign of infection
• Treat all skin infections aggressively

45. Advances
• Use of silicon tubes
• Stem cell therapy
• VEGF-C therapy

46. Peculiarities of our environment
• Poverty
• Ignorance

47. CONCLUSION
• Lymphedema is a common, complex and
debilitating disorder whose biology is still
incompletely understood.
• Increased interest in, and comprehension of, this
disorder have led to enhanced methods of
diagnostic evaluation and therapeutic
intervention.
• It is anticipated that further investigation into the
cellular mechanisms of lymphedema will lead to
ever more elegant refinements in the ability to
control this disease.

48. •THANK YOU

49. REFERENCES
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disorders of the lymphatic system.Lymphology1993;26(4): 156–68.2
• Cambria RA, Gloviczki P, Naessens JM, Wahner HW. Noninvasive
evaluation of the lymphatic system with lymphoscintigraphy: a pro-
spective, semi quantitative analysis in 386 extremities.J Vasc
Surg1993;18(5): 773–82.3
• BeckerC,VasileJV,LevineJL,etal.Microlymphaticsurgeryforthetreatmentofiat
rogeniclymph- edema. Clin Plast Surg 39:385-398, 2012.
• Partsch H. Assessment of abnormal lymph drainage for the diagnosis of
lymphedema by isotopic lymphangiography and by indirect lym-
phography.Clin Dermatol1995;13: 445–50.4
• Ter SE, Alavi A, Kim CK, Merli G. Lymphoscintigraphy. A reliabletest for the
diagnosis of lymphedema.Clin Nucl Med1993;18(8):646–54.5