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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

40 General Principles and Indications
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341


Chapter 41
Lymphatic-Venous Derivative
and Reconstructive Microsurgery
Corradino Campisi and Francesco Boccardo
General Considerations
Lymphedema that is refractory to nonoperative methods may be managed by surgical
treatment. Indications include insufficient lymphedema reduction by well- performed
medical and physical therapy (less than 50%), recurrent episodes of lymphangitis,
intractable pain, worsening limb function, patient who are unsatisfied with the result
obtained by nonoperative methods, and patients who are willing to proceed with
surgical options.
The first microsurgical derivative operations were those using lymph node–
venous shunts. These have been largely abandoned, except in endemic areas of lymphatic filariasis such as India, where thousands of these procedures have been
performed. Lymphatic channels in lymph nodal–venous anastomoses are often
widely dilated because of the high rate of anastomotic closures caused by the thrombogenic effect of lymph nodal pulp on the venous blood and the frequent re-endothelialization of the lymph node surface.1 Because of the difficulties encountered with
lymph nodal–venous shunts by surgeons worldwide, the next approach was to use
lymphatic vessels directly anastomosed to veins.
The technique consists of anastomosing lymphatic vessels to a collateral
branch of the main vein with competent valvular function to secure the proper
continence of the vein segment used for the anastomosis with no reflux. valvular
competence warrants the mandated condition of lymph flow alone and not the
blood within the venous segment, avoiding any risk of thrombosis of the
anastomosis.
3
2
C. Campisi (*)
Department of Surgery, Section of Lymphology and Microsurgery,
University Hospital “San Martino”, Genoa, Italy
B.-B. Lee et al. (eds.), Lymphedema,
DOI 10.1007/978-0-85729-567-5_41, © Springer-Verlag London Limited 2011
343

344 C. Campisi and F. Boccardo
LVA
Fig. 41.1 Lymphatic–venous
multiple anastomosis: several
lymphatics are introduced
inside a valved vein. The blue
dye flowing into the vein
demonstrates the
patency of the vein. The
well-functioning valve
ensures the continence of the
vein, avoiding blood reflux
toward the lymphatics. This
technical trick is important
for the long-term patency of
the anastomosis
Clinical Experience and Surgical Techniques
The operations consisted of multiple microsurgical lymphovenous anastomoses.
Healthy-appearing lymphatics found at the operation site are directly introduced
together into the vein segment by a U-shaped stitch and then further secured to the
vein’s cut–end by means of additional stitches between the vein border and the perilymphatic adipose tissue. With the use of the Patent Blue dye, properly functioning
lymphatics appear blue, and the passage of blue lymph into the vein branch verifies
the patency of the lymphovenous anastomosis under the operating microscope when
the anastomosis is completed (Fig. 41.1).
For patients with lower limb lymphedema, anastomoses are performed at the
subinguinal region. Superficial lymphatic–lymph nodal structures are isolated, and
all afferent lymphatics are used for the operation. Lymph nodes are subjected to
histopathological examination. The usual finding in primary lower limb lymphedemas is a varying grade of nodal fibrosclerosis and thickening of the nodal capsule,
but with normal afferent lymphatic vessels.
For upper limb lymphedema, lymphovenous anastomoses are performed at the
middle third of the volar surface of the arm, using both superficial and deep lymphatic collectors, as demonstrated by the blue dye. Deep lymphatics are found
among the humeral artery, vein, and the median nerve. The vein used for anastomoses is a patent branch of one of the humeral veins, and the technique most frequently
performed is microsurgery (Fig. 41.2).
Primary lymphedemas largely include lymph node dysplasias (LAD II, according to Papendieck’s classification4) consisting of hypoplastic lymph nodes with
sinus histiocytosis and a thick and fibrous capsule with microlymphangioadenomyomatosis. In these cases, lymph flow obstruction is apparent, as seen by alterations
of the afferent lymphatics, which appear dilated and swollen with thickened walls
and where smooth muscle cells are reduced in number and appear fragmented by
associated fibrous elements.

41 Lymphatic-Venous Derivative and Reconstructive Microsurgery
Fig. 41.2 Primary right upper limb lymphedema in a man treated with derivative lymphatic–
venous anastomosis at the volar surface of the upper third of the arm. Superficial (SL) and deep
(DL) lymphatics are prepared together with a vein (V) branch of one of the brachial veins with
well-functioning valves. The result of the operation is immediate and the technique allowed stable
results to be obtained at long-term follow-up
345
Secondary lymphedemas are largely due to lymphadenectomy and radiotherapy
performed for oncological reasons (carcinoma of the breast, uterus, penis, bladder,
prostate gland, and rectum and seminoma of the epididymis), as well as for complications of minor operations for varicose veins, crural and inguinal hernias, lipomas,
tendinous cysts, or axillary and inguinal lymph node biopsies. Most of the lymphedemas treated by the microsurgery in our experience were at stages II (39%) and
III (52%), whereas 3% of the patients were stage Ib and 6% were stages IV and V.
Lymphoscintigraphy, performed with 99mTc-labeled antimony sulfur colloid,
is employed in the diagnostic work-up of patients with lymphedema and as a test
for selecting patients for derivative microsurgical operations. Lymphoscintigraphy
clearly determines whether or not edema was of lymphatic origin and also provides important data about the etiologic and pathophysiologic aspects of the
lymphedema.
Echo Doppler is performed in all patients to identify any venous disorders possibly associated with lymphedema. In most patients, venous dysfunction is corrected at the same time as microlymphatico-venous anastomoses (i.e., valvuloplasty
in the case of venous insufficiency) is performed. In other cases, the finding of

346 C. Campisi and F. Boccardo
LVLA
Fig. 41.3 Lymphatic–venous–lymphatic anastomoses used in those cases in which, owing to
venous dysfunction, derivative technique are contraindicated. The technique consists in
interposing a vein segment in between lymphatics above and below the obstacle to lymph flow
7
venous dysfunction contraindicates derivative lympho-venous shunts, but at the
same time facilitates referral of the patient for reconstructive microsurgical
operations.
In those cases involving the lower limbs, where surgically uncorrectable venous
disease exists, it is not advisable to use derivative lymphatic–venous techniques,
and accordingly, reconstructive methods are used. The most commonly used technique is the interposition of an autologous vein graft between the lymphatics above
and below the obstacle to lymph flow. Competent venous segments can be obtained
from the same operative site or from the forearm (mostly the cephalic vein). The
length of the graft is variable from 7 to 15 cm, and it is important to collect several
lymphatics to connect to the distal cut end of the vein so as to ensure that the segment is filled with enough lymph and to avoid closure due to subsequent development of the fibrosis. The competent valves of the vein segments are essential for the
correct direction of the lymphatic flow and to avoid gravitational backflow, or reflux.
The technique of anastomosis is the microsurgery with introduction of the lymphatics inside the vein cut ends by a U-shaped stitch, which is then secured by additional
peripheral stitches (Fig. 41.3).

41 Lymphatic-Venous Derivative and Reconstructive Microsurgery
Pre-op Post-op
Fig. 41.4 Right upper limb lymphedema due to breast cancer treatment, managed by derivative
lymphatic–venous microsurgical anastomoses at the arm (long-term follow-up)
347
Results and Final Considerations
Clinical outcome improves the earlier microsurgery is performed, owing to absent
or minimal fibrosclerotic alterations of the lymphatic walls and surrounding tissues. Subjective improvement in our experience was noted in 87% of patients.
Objectively, volume changes showed a significant improvement in 83%, with an
average reduction of 67% of the excess volume. Of those patients followed up,
85% have been able to discontinue the use of conservative measures, with an average follow-up of more than 10 years and average reduction in excess volume of
69% (Figs. 41.4–41.8). There was an 87% reduction in the incidence of cellulitis
after microsurgery.
Lymphoscintigraphy helped in verifying the patency of microanastomoses
long term after operation by direct and indirect findings: reduction of dermal
backflow together with the appearance of preferential lymphatic pathways not
visible before microsurgery; disappearance of the tracer at the site of lymphatic–
venous anastomoses because of direct tracer passage into the blood stream; and
earlier liver uptake compared with pre-operative parameters (indirect patency test;
Figs. 41.9 and 41.10).
Lymphatic microsurgery represents a means of bypassing the obstacle to lymph
flow through lymphatic–venous drainage (lymphatic–venous anastomoses) or by

348 C. Campisi and F. Boccardo
Fig. 41.5 Another case of secondary upper limb lymphedema. Of note is the good result at the
hand and the favorable result from a cosmetic point of view
using venous grafts between lymphatic collectors below and above the obstruction
(lymphatic–venous–lymphatic plasty). Combined physical therapy nonetheless represents the initial treatment of patients affected by peripheral lymphedema and it is
best performed in specialized centers. The surgical timing follows completion of
conservative treatment when further clinical improvement can no longer be achieved
and/or recurrent lymphangitic attacks are not further reduced.
tions can then be performed and provide further improvement in the condition.
5
Microsurgical opera-
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