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- •Lymphedema
- •Foreword
- •Preface I
- •Preface II
- •Contents
- •Contributors
- •Clinical Presentation
- •Lymphedema Staging
- •Diagnosis
- •Therapy
- •Physical and Non-Operative Therapy
- •Operative Therapy
- •Introductory Note
- •Primary Lymphedema
- •Secondary Lymphedema
- •Complications of Lymphedema
- •Conclusions
- •References
- •Embryological Development of the Lymphatic System
- •Lymphedema
- •Lymphangioma
- •Protein-Losing Enteropathy and Intestinal Lymphangiectasia
- •Complex Vascular Malformations
- •Infectious Diseases
- •Lipedema
- •Lymphangioleiomyomatosis
- •References
- •Introduction
- •Molecular Lymphology
- •Work-up
- •Syndromes
- •Chromosomal Aneuploidies and Sporadic Syndromes
- •Conclusion
- •References
- •References
- •Anatomical
- •Functional
- •Lymph Flow Pathways
- •Skin and Subcutaneous Tissue
- •Gut Lymphatics
- •Lung Lymphatics
- •References
- •References
- •References
- •Tissue Fluid
- •Lymph
- •Physiological Observations
- •Proteins in Obstructive Lymphedema
- •Lymph Cytokines in Obstructive Lymphedema
- •References
- •Tissue Fluid Pressure and Flow
- •Pressures in the Normal Limb
- •Pressures in the Lymphedema
- •Normal Tissue Fluid Flow
- •Tissue Fluid Flow in Lymphedema
- •Lymph Pressure and Flow
- •Extrinsic Factors that Propel Lymph
- •Normal Conditions
- •Lymphedema Conditions
- •Intrinsic Factors that Propel Lymph
- •Pressures in Lymphedematous Limbs
- •Lymph Flow in Normal Limbs
- •Lymph Flow in Lymphedematous Limbs
- •General Remarks
- •References
- •Immune processes in lymphatics and nodes
- •Remarks
- •References
- •General Considerations
- •Clinical Diagnosis
- •Associated Disorders
- •When Further Investigation Is Needed
- •References
- •References
- •Conclusion
- •References
- •References
- •Consensus Documents
- •Consensus Documents in the Treatment of Lymphedema
- •International Society of Lymphology
- •International Lymphedema Framework
- •Italian
- •Latin American
- •Australian
- •American Cancer Society
- •National Lymphedema Network
- •Summary
- •Concluding Thought
- •Disclosure
- •References
- •Signs to Look for at Presentation
- •References
- •Introduction
- •Clinical Diagnosis
- •Differential Diagnosis
- •Introduction
- •Differential Diagnosis: Other Reasons for a Swollen Limb
- •Differentiating the Lymphedemas
- •Filarial Lymphedema
- •Malignant Lymphedema
- •Factitious Lymphedema
- •Primary Lymphedema
- •When a Patient Might First Present
- •Risk Factors to Consider at Presentation
- •Laboratory Diagnosis
- •Waist-to-Height Ratio
- •Streeten Test
- •Capillary Fragility Assessment
- •Assessment of Aortic Distensibility and Stiffness in Lipedema
- •Pain Perception Assessment
- •Ultrasound Examination
- •CT and MRI Examination
- •Lymphoscintigraphy and Fluorescent Microlymphography
- •Clinical Management
- •Prognosis
- •References
- •General Considerations
- •When Clinical Examination Should Be Complemented by Imaging
- •Methods to Evaluate Lymph Flow, Lymphatic Vessels, and Lymph Nodes
- •Methods of Evaluating Tissue Changes
- •References
- •Brief Historical Note
- •Materials and Methods
- •Interpretation and Comments
- •Primary Lymphedema
- •Secondary Lymphedema
- •Lymphatic Filariasis
- •Kaposi Sarcoma
- •Klippel–Trenaunay and Other Lymphangiodysplastic/Mixed Syndromes
- •The Future
- •Conclusions
- •References
- •References
- •Introduction
- •Lymphoscintigraphy and/or SPECT-CT Lymphoscintigraphy
- •Lymphoscintigraphy or SPECT-CT Lymphoscintigraphy in Relation to the Clinical Presentation of the “Simple” Lymphedematous Situations
- •In Primary Lower Limb Lymphedemas
- •In Secondary Lymphedemas
- •Lymphoscintigraphy to Demonstrate the Collateralization Pathways
- •Lymphoscintigraphy, Lymphoceles, and Lymphangiomas?
- •X-Ray Computed Tomography?
- •Positron Emission Tomography or Positron Emission Tomography Combined with X-Ray Computed Tomography?
- •Magnetic Resonance Imaging and/or Lymphangio-MRI with Injection of Contrast Enhancement?
- •Magnetic Resonance Imaging in the Diagnosis of Pathologically Positive Lymph Nodes?
- •Heavily T2-Weighted Imaging or Magnetic Resonance Lymphangiography for Lymphedemas?
- •MRI or MRL in Lymphedemas?
- •MRI and Lymphangiomatosis?
- •MRI and Lymphangiomas?
- •Lymphoscintigraphy and/or MRI?
- •Conclusions
- •References
- •Visual Lymphography and Radiological Lymphography
- •Radiological Lymphography
- •Oil Contrast Lymphography
- •References
- •Microlymphography in Healthy Individuals, in Chronic Venous Disease, and in Lymphedema (Table 23.1)
- •Measurement of Microlymphatic Pressure
- •Lymphatic Vasomotion and Lymphatic Flow Motion
- •References
- •Measurement of Fibrotic Induration
- •Measurement of Fluid Content
- •Measurement of Limb Volume and Circumference
- •Measurement of Functional Status of the Lymphatic System
- •Measurement of the Structural Status of the Lymphatic System and of the Limb
- •Measurement of the Status of the Vascular System
- •Measurement of the Subjective Parameters
- •Treatment Outcomes
- •References
- •General Overview
- •Primary and Secondary Infections
- •Primary Infections
- •Secondary Infections: Dermato-Lymphangio-Adenitis
- •Chronic Dermatolymphangioadenitis
- •Acute DLA
- •Differential Diagnosis of Lymphangitis, Erysipelas and Dermato-Lymphangio-Adenitis
- •Bacteriology of Lower Limb Skin
- •Bacterial Flora of Normal Foot and Calf Skin
- •Bacterial Flora of Normal Leg Lymph
- •Bacterial Flora of Lymphedematous Leg Lymph
- •Sensitivity of Isolates to Antibiotics
- •Prophylaxis of Recurrent DLA
- •Chronic DLA
- •Treatment of Acute DLA Attacks
- •References
- •Introduction
- •Sites of Accumulation of Lymph and Tissue Fluid in Lymphedema
- •Morphological Changes in the Lymphedematous Skin and Subcutis
- •Hydraulic Conditions in the Subcutaneous Tissue
- •Pressures
- •Pressure Gradient Across Skin and Subcutaneous Tissue
- •Conditions for Creating Centripetal Tissue Fluid Flow
- •Manual Massage
- •Indications
- •Advantages and Shortcomings
- •Manual Massage Hydraulics
- •Pneumatic Massage
- •Indications
- •Advantages and Shortcomings
- •Pneumatic Compression Hydraulics
- •Remarks for Users of Compression Devices
- •References
- •Introduction
- •Complete Decongestive Physiotherapy
- •The Use of CDP
- •Long-Term Therapy Results
- •References
- •Introduction
- •Detailed Characterization of MLD According to Dr. E. Vodder
- •Stationary Circle
- •Rotary Stroke
- •Pump Stroke
- •Scoop Technique
- •Additive Manual Techniques
- •Indication and Contraindication
- •References
- •Introduction
- •Investigations
- •References
- •Graduated Compression Garments
- •Multilayered Bandage Compression
- •Intermittent Pneumatic Compression
- •Impact of Compression Therapy upon Lymphedema Outcomes
- •References
- •References
- •Conservative Therapies for Secondary Lymph Edema
- •Contemporary Treatments
- •The Groupings of Contemporary Treatments
- •Methods
- •Pharmacogenomics and Medications Targeting the Lymphatic System
- •Low-Level Scanning and Hand-Held Laser
- •Lymphatic Drainage Massage Delivered by Partners/Carers and Mechanically
- •Mild Exercise (Tai Chi)
- •Moderate Exercise (In and Out of Water)
- •Electro-Stimulation
- •Tissue Manipulation
- •Kinesio-Taping
- •Diet (Mid-Chain Triglycerides) and Abdominal Issues
- •Placebo
- •References
- •Antibiotics
- •Conclusion
- •References
- •Introduction
- •General Considerations
- •Intermittent Pneumatic Compression
- •Compression
- •Use of Elastic Bandages
- •Special Compression Material
- •Medical Compression Stockings
- •Exercise
- •Lymphedema Severity-Adapted Forms of CDP
- •Stage I Lymphedema
- •Stages II and III Lymphedema
- •References
- •Introduction
- •Lymphedema of the Arm
- •Considerations in Manual Lymph Drainage
- •General Considerations for Compression
- •Compression Therapy in the Arms
- •References
- •Introduction
- •Physical Treatment of Lymphedema of the Face and Neck
- •Manual Lymph Drainage (Leduc Method)
- •Description of the Maneuvers
- •Protocol for Manual Treatment of Lymphedema of the Face and Neck
- •Multi-Layered Bandaging Leduc Method
- •Stimulation of Muscular Activity
- •Compression Garment
- •Education in Precautions to Apply to Avoid Exacerbation of Symptoms
- •Education in Self-Treatment
- •An Example of Self-Treatment of Head and Neck Lymphedema
- •Rehabilitation to Address Functional Impairments
- •Quality of Life
- •References
- •Introduction
- •Anatomy
- •Etiology
- •Diagnosis
- •Clinical Course
- •Treatment
- •Surgical
- •References
- •References
- •Lymphovenous Microsurgical Shunts in Lower Limbs
- •Lympho-Venous Shunts (1966–2010)
- •Pre- and Post-operative Pharmacological Treatment
- •Postoperative Physiotherapy
- •Postoperative Evaluation Criteria
- •Objective Indirect Methods for the Evaluation of the Function of the Lympho-Venous Shunt
- •Direct Methods for Evaluation of Function of Lympho-Venous Shunt
- •Factors Adversely Affecting the Patency of Lymph-Venous Shunts
- •Local
- •Distant
- •Factors Affecting Evaluation of Clinical Results
- •Results in General
- •References
- •Principles
- •Indications
- •Microsurgical Reconstructions
- •Lymphovenous Anastomosis
- •Lymph Node-to-Vein Anastomosis
- •Technique
- •Results
- •Lymph Vessel-to-Vein Anastomosis
- •Microsurgical Technique
- •Results
- •Lymphatic Grafting
- •Technique
- •Results
- •Lymph Node Transplantation
- •Technique
- •Results
- •Problems with Microvascular Lymphatic Reconstructions
- •Conclusions
- •References
- •General Considerations
- •Clinical Experience and Surgical Techniques
- •Results and Final Considerations
- •References
- •Introduction
- •Correlation With the Pathophysiology of Lymphedemas
- •Experimental Basis
- •Indications for Lymphatic Reconstruction Using Lymphatic Grafts
- •Operative Technique
- •Post-operative Procedures
- •Results
- •References
- •NodoVenal Shunt
- •Indications
- •Surgical Techniques
- •End-to-End Anastomosis
- •End-to-Side Anastomosis
- •Contraindications
- •Complications
- •References
- •Introduction
- •Secondary Lymphedema
- •Lymphedema of the Arm: Upper Extremity
- •Indication for Node Grafting
- •Operative Technique
- •Results
- •Plexopathy
- •Breast Reconstruction Combined with Lymphedema Treatment
- •Lymphedema of the Leg: Lower Extremity
- •Operative Technique
- •Results
- •Primary Lymphedema
- •Indications
- •Operative Technique
- •Results
- •Conclusion
- •References
- •Clinical Experiences (Personal)
- •Conclusion
- •References
- •References
- •Introduction
- •The Morphological Changes in Advanced Lymphedema
- •Indications for Debulking
- •Bacteriology of Skin and Deep Tissues
- •Surgical Technique
- •References
- •References
- •Clinical Experience
- •Conclusion
- •References
- •Excess Subcutaneous Adiposity and Chronic Lymphedema
- •The Outcome of Liposuction
- •How to Perform Liposuction for Lymphedema
- •Surgical Technique
- •Postoperative Care
- •Controlled Compression Therapy
- •Volume Measurements
- •When to Use Liposuction to Treat Lymphedema
- •Summary
- •Key Points
- •References
- •Extratruncular Lymphatic Malformation Lesions
- •Truncular Lymphatic Malformation Lesions
- •Clinical Evaluation
- •Clinical Management
- •Conservative (Physical) Therapy
- •Surgical Therapy: Reconstructive Surgery
- •Surgical Therapy: Ablative/Excisional Surgery
- •Liposuction: Circumferential Suction-Assisted Lipectomy
- •Prospect: Primary Lymphedema as Lymphatic Malformation
- •Conclusion
- •References
- •References
- •Diagnosis
- •Management
- •General Considerations
- •References
- •Medical Therapies for Chylorrhea
- •References
- •Introduction
- •Drainage Procedures
- •Image-Guided Approaches
- •Open Surgical Approaches
- •Treatment of Cutaneous Chylorrhea and Chylorrhagia
- •Treatment of Chylothorax
- •Treatment of Chylous Ascites
- •Summary
- •References
- •References
- •Morphology
- •Life Cycle
- •Pathology
- •Gross Pathology
- •Changes Attributed to Filariae
- •Changes Ascribed to Bacterial Infections
- •Immunology
- •References
- •Manifestations

56 Epidemiology
24. Kerketta AS, Babu BV, Rath K, et al. A randomized clinical trial to compare the efficacy of
three treatment regimens along with footcare in the morbidity management of filarial lymphoedema. Trop Med Int Health. 2005;10:698-705.
25. WHO. Informal consultation on preventing disability from lymphatic filariasis, WHO, Geneva,
August 2006. Wkly Epidemiol Rep. 2006;81:373-383.
26. Evans DB, Gelband H, Vlassoff C. Social and economic factors and the control of lymphatic
filariasis: a review. Acta Trop. 1993;53:1-26.
27. Ottesen EA. The global programme to eliminate lymphatic filariasis. Trop Med Int Health.
2000;5:591-594.
28. Ottesen EA, Hooper PJ, Bradley M, et al. The global programme to eliminate lymphatic filari-
asis: health impact after 8 years. PloS Negl Trop Dis. 2008;2(10):e317.
29. Chu BK, Hooper PJ, Bradley MH, et al. The economic benefits resulting from the first 8 years
of the Global Programme to Eliminate Lymphatic Filariasis (2000–2007). PloS Negl Trop Dis.
2010;4(6):e708.
469


Chapter 57
Etiology and Pathophysiology
Waldemar L. Olszewski
Lymphatic filariasis is caused by Wuchereria bancrofti, Brugia malayi, and Brugia
1,2
timori.
primary lesions causing lymph stasis. Lymph stasis predisposes to bacterial infections and host inflammatory response. Adult worms die after some time, but bacterial colonization of tissues continues. The pathological changes in tissues develop
further, leading to an increase in limb volume, hyperkeratosis, and fibrosis.
Morphology
The adult worms live in the lymphatic vessels and are responsible for the
The adult W. bancrofti are 4–10 cm long, whereas B. malayi are shorter (3–5 cm
long). The males are smaller than the females. The life-span ranges from 7 to 15 years.
The female worm discharges eggs (microfilaria) into the lymphatics that circulate in
the blood. The microfilaria vary in length from 200–300 mm. Humans are the only
known definitive hosts for W. bancrofti, but B. malayi is also seen in animals.
Life Cycle
The W. bancrofti microfilariae are ingested by mosquitoes as Culex or Anopheles and
B. malayi by Mansonia and Anopheles. The adult worms do not multiply in the host,
nor do the larvae multiply in the mosquitoes. The microfilariae are ingested by mosquitoes. In the mosquitoes the microfilaria mature to become the infective form.
Depending on the temperature and humidity of the environment, this process takes
10–14 days. The infective L3 larvae are 1.4–2.0 mm long. They migrate to the
W.L. Olszewski
Department of Surgical Research and Transplantology,
Medical Research Centre, Warsaw, Poland
B.-B. Lee et al. (eds.), Lymphedema,
DOI 10.1007/978-0-85729-567-5_57, © Springer-Verlag London Limited 2011
471

472 W.L. Olszewski
Fig. 57.1 Lymphoscintigram of a filaria-infected patient
with lymphedema of both lower limbs. The toewebinjected Nanocoll T99 spreads in the dilated calf
lymphatics (left leg). It flows along the superficial
lymphatics to the inguinal lymph nodes and along the
deep vessels, through multiple enlarged popliteal nodes
in both limbs. In normal conditions only 1–2 small nodes
are seen in this region
proboscis to be deposited at the bite wound by the mosquito. The larvae enter the
skin at puncture sites and reach the lymphatics to grow, mature, and mate. Microfilariae
appear in circulation 6–12 months after entry of the larvae. They remain viable for
2–3 months, after which they die and are removed by host macrophages.
Pathology
Gross Pathology
Filarial lymphedema is most commonly seen in the lower limbs (Fig. 57.1). It may
also affect the upper limbs, breast, scrotum, and urinary tract. It is a chronic, progressive condition leading to major tissue changes.
The volume of the limb steadily increases, accumulating tissue fluid in the subcutaneous tissue. Skin growth and deposition of collagen further increase limb
mass. Hyperkeratosis develops, first on the toes, and then on the dorsum of the foot.
Papillomatosis is observed in the late stages. Epidermal vesicles filled with lymph
break and ooze fluid. Maceration of the epidermis develops in the interdigital spaces.

57 Etiology and Pathophysiology
Fig. 57.2 Gigantic lymphedema of the left lower limb of a woman from the filaria-endemic area.
Hyperkeratotic warty foot skin, hypertrophy of the calf skin, and subcutaneous tissue in the swollen part. Duration of lymphedema, 3 years
473
Breaks in the sole skin change into chronic crevices colonized by bacteria and fungi.
The mechanisms of the uncontrolled growth of the epidermis remain unclear.
Microbial etiology is likely because long-term administration of antibiotics mitigates this process.
The movements in ankle joints become limited due to hardening of the skin and
formation of a hanging calf tissue fold. Gradually, the volume of the calf increases
and the skin becomes thicker and harder, limiting at the later stages the knee joint
movements. The inguinal lymph nodes first become enlarged and then decrease in
size and harden.
The changes in the limb superficial lymphatic system can be seen on lymphoscintigrams (Fig. 57.2). At the initial stages, there is dilatation of the lymphatic collectors and enlargement of the lymph nodes. Later, the lymphatics are obliterated.
Recurrent attacks of dermato-lymphangio-adenitis (DLA) accelerate this process.
The lymph nodes shrink, and become impermeable for the radioisotope. Collateral
lymphatics bypassing nodes can be seen.
Ultrasound shows dilated lymphatic trunks in the calf and thigh. In some cases
the “dancing microfilariae” can be seen.
A filaria-infected scrotum enlarges, in some cases to a size limiting walking ability. As a rule, there is leakage of stagnant lymph from multiple epidermal vesicles.
Dysfunction of the lymphatics of the spermatic cord and the para-aortic lymph
nodes leads to accumulation of fluid to the tunica vaginalis testis and hydrocele.
Rupture of dilated mesenteric lymphatics and cisterna chyli results in formation
of chyloperitoneum and transudation of milky lymph to the scrotum and thighs.
Leakage of lymph from the thigh skin occurs frequently.

474 W.L. Olszewski
Rupture of the overloaded retroperitoneal lymphatics to the kidney pelvis leads
to loss of lymph with chyluria.
All tissues and organs affected by lymph stasis are sites for development of
lymphangitis. It is observed in the lower and upper limbs, scrotum, spermatic cord,
testes, inguinal and axillary lymph nodes, intestine, and urinary tract.
Changes Attributed to Filariae
The adult worms living in the lymphatic collectors are considered to be responsible
for the changes in these vessels. The severity of the pathological condition depends
on the number of adult worms and the host immune response. Not all infected individuals reveal microfilaremia and lymphatic lesions. The largest group of affected
individuals are asymptomatic, despite the presence of microfilariae in the blood.
Live adult worms cause dilatation of the lymphatics. This can be seen using
ultrasound. There is evidence of chronic inflammation of tissues and the draining
lymph nodes.3 On histology, hyperkeratosis is characterized by an increase in the
number of keratinocyte layers (8–10) and desquamation. In the dermis mononuclear
infiltrates around blood capillaries and at the epidermal–dermal junctions are seen.
There is dilatation and increase in the number of lymphatic capillaries of the subepidermal plexus. Lymph nodes are hard and enlarged. The microscopical pictures
show dense cellularity in the subcapsular cortex, enlarged follicles with B cells and
paracortical regions with T cells. Multiple large macrophage-like cells are seen disseminated in all areas, but most densely in the medullary portion of the node.
Changes Ascribed to Bacterial Infections
Skin abrasion, moisture, and barefoot walking predispose to bacterial penetration of
the filarial lymphedematous limb. The common ports of entry are the interdigital
spaces, but any other foot skin damage may also facilitate bacterial colonization.
Dilatation of collecting lymphatic trunks is responsible for the later dysfunction and
subsequent lymph stasis. Stasis predisposes to secondary bacterial infection and tissue colonization. Lack of elimination of microbes brings about host immune response
diagnosed as DLA.4 Acute attacks of DLA are followed by chronic DLA characterized by hyperkeratosis, fibrosis of the skin, and increase in limb volume. The mixed
bacterial flora of the patient’s own skin is presumably responsible for this condition.4
The tissue fluid, lymph and lymph node specimens obtained from lower limbs show
the presence of bacterial isolates in 70% of cases. The dominant strains are coagulase-negative staphylococci and bacilli. Bacterial infections in lymph stasis are easily controlled by antibiotics. Long-term administration of, for example, benzathine
penicillin at a dose of 1,200,000 IU every 21 days has decreased the recurrence rate
of DLA attacks by 80%, without a change in sensitivity to antibiotics.
5,6
The reported

57 Etiology and Pathophysiology
475
presence of endosymbiotic bacteria of the genus Wolbachia in the adult filarial
worms may be an additional indication for antibiotic treatment.
7
Immunology
Filaria evoke the host immune response.
lymphatics for long periods this response is rather weak. Most studies have been
devoted to the humoral reaction, among others, production of IgG4 and its effects
10,11
on IgE
and synthesis of interleukins 4, 5, and 17 by T lymphocytes. The cellular
reaction involves T suppressor cells12 and T regulatory cells (Treg).
8,9
Although the adult worm remains in the
13
References
1. Ottesen EA. The Wellcome Trust lecture: infection and disease in lymphatic filariasis: an
immunological perspective. Parasitology. 1992;104:S71-S79.
2. Report of the Scientific Working Group on Filariasis, 2005 TDR/SWG/057: Addiss DG, Brady
MA. Morbidity management in the global programme to eliminate lymphatic filariasis: a
review of the scientific literature. Filaria J. 2007; 6:2
3. Olszewski WL, Jamal S, Manokaran G, et al. Bacteriological studies of blood, tissue fluid,
lymph and lymph nodes in patients with acute dermatolymphangioadenitis (DLA) in course of
“filarial” lymphedema. Acta Trop. 1999;73:217-224.
4. Olszewski WL, Jamal S, Manokaran G, et al. Bacteriologic studies of skin, tissue fluid, lymph,
and lymph nodes in patients with filarial lymphedema. Am J Trop Med Hyg. 1997;57:7-15.
5. Olszewski WL. Episodic dermatolymphangioadenitis (DLA) in patients with lymphedema of
the lower extremities before and after administration of benzathine penicillin: a preliminary
study. Lymphology. 1996;29:126-131.
6. Olszewski WL, Jamal S, Manokaran G, et al. The effectiveness of long-acting penicillin (peni-
dur) in preventing recurrencies of dermatolymphangiodenitis (DLA) and controlling skin,
deep tissues, and lymph bacterial flora in patients with “filarial” lymphademia. Lymphology.
2005;38:66-80.
7. Taylor MJ, Makunde WH, McGarry HF, et al. Macrofilaricidal activity after doxycycline treat-
ment of Wuchereria bancrofti: a double-blind, randomized placebo-controlled trial. Lancet.
2005;365:2116-2121.
8. Allen JE, Maizels RM. Immunology of human helminth infection. Int Arch Allergy Immunol.
1996;109:3-10.
9. Jackson JA, Friberg IM, Little S, Bradley JE. Review series on helminthes, immune modula-
tion and the hygiene hypothesis: immunity against helminthes and immunological phenomena
in modern human population: Coevolutionary legacies? Immunology. 2008;126:18-27.
10. Ottesen EA, Skvaril F, Tripathy SP, et al. Prominence of IgG4 in the IgG antibody response to
human filariasis. J Immunol. 1985;134:2707-2712.
11. Kurniawan A, Yazdanbakhsh M, van Ree R, et al. Differential expression of IgE and IgG4
specific antibody responses in asymptomatic and chronic human filariasis. J Immunol.
1993;150:3941-3950.
12. Piessens WF, Ratiwayanto S, Tuti S, et al. Antigen-specific suppressor cells and suppressor
factors in human filariasis with Brugia malayi. N Engl J Med. 1980;302:833-837.
13. Taylor MD, LeGoff L, Harris A, et al. Removal of regulatory T cell activity reverses hypore-
sponsiveness and leads to filarial parasite clearance in vivo. J Immunol. 2005;174:4924-4933.


Chapter 58
Clinical Overview-Diagnosis
and Management
Gurusamy Manokaran
There are about 120 million people at risk for and 70 million people have established
lymphatic filariasis, of which 40 million are suffering from lymphedema; thus, it is
very important for medical, paramedic and health planners to understand this disease and to provide morbidity control including surgery for these unfortunate
patients. The World Health Organization is working toward elimination of lymphatic filariasis by 2020.
Lymphatic filariasis is a chronic debilitating parasitic disease caused by
Wuchereria bancrofti, Brugia malayi, and Brugia timori. This is transmitted by the
culex mosquito to humans. Prevention and elimination of this disease is vital, but it
will take a long time because the third-world countries are struggling to cope up
with the basic needs of drinking water, food, and shelter (Figs. 58.1 and 58.2).
We have presented the most common manifestation of lymphatic filariasis,
namely, lymphedema and its present management strategies.
1-17
Manifestations
Lymphatic filariasis can manifest as (a) hydrocele, (b) lymphedema of both upper
and lower limbs, (c) chylothorax, (d) chyluria, (e) chylascitis, (f) genital manifestations (filarial scrotum, Ramp horn penis genital vesicles, and edema), and (g) atypi-
cal lymphatic filariasis in the form of fleeting joint pains and lymphangitis (string
sign). It can affect the breast, gluteal region, abdomen, and suprapubic region in the
form of isolated lesions. The lower limb is the commonest manifestation, and
women are more frequently affected than men (Figs. 58.3 and 58.4).
G. Manokaran
Department of Plastic and Reconstructive Surgery and Lymphologist,
Apollo Hospitals, 21, Greams Road, Chennai, India
B.-B. Lee et al. (eds.), Lymphedema,
DOI 10.1007/978-0-85729-567-5_58, © Springer-Verlag London Limited 2011
18-23
477

478 G. Manokaran
Fig. 58.1 Life cycle of Wucheraria bancrofti
Fig. 58.2 Wucheraria
bancrofti
The chemotherapeutic management
of these problems are either diethyl carbamazine [DEC]) alone or in the following combinations – DEC + albendazole,
DEC + ivermectin, along with periodic antibiotics like penicillin, doxycyline,
and sulfonamides. Doxycycline is very useful in symbiotic bacterial infections
called Wolbachia,
25-29
(Figs. 58.5 and 58.6) which reside inside the parasite and
cause resistance to antifilarial drugs. The entire topic of lymphatic filariasis is
beyond the scope of this chapter, which will be restricted to the management of
filarial lymphedema.
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