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Iliofemoral Deep Venous Thrombosis (During Pregnancy) 449
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fered a symptomatic pulmonary embolism. Alternatively, suprathrombus balloon
occlusion during the caval thrombectomy can be performed. This patient also
underwent a preoperative echocardiogram to evaluate the impact of her presumed
pulmonary embolism on right ventricular function. Echocardiography should be
performed in all patients with pulmonary embolism, since it is a predictor of
chronic thromboembolic pulmonary hypertension, and patients who have rightsided abnormalities should be considered for thrombolytic therapy or mechanical
thromboembolectomy. [Q5: D, E]
During the operative procedure, fluoroscopy is used to guide the placement of the
balloon catheter so as not to dislodge the vena caval filter. Fluoroscopy is also used
to assess the success of thrombectomy and to evaluate for underlying venous lesions
and their correction (Fig. 46b.3). Since the fetus is very well developed, the risk to the
fetus from modest X-ray exposure is low. Fetal monitoring is routinely performed
throughout the procedure. The monitoring devices must be checked so as not to
interfere with appropriate imaging of the venous system during the procedure.
Shielding of the fetus would obscure the iliac veins and distal vena cava. [Q6: B, C]
Previous descriptions of iliofemoral venous thrombectomy focus only on the
iliofemoral venous system. An occluded infrainguinal venous system reduces
venous return through the thrombectomized iliofemoral veins, and leaves substantial thrombus burden infrainguinally with its resultant post-thrombotic sequelae.
Current techniques of infrainguinal venous thrombectomy allow the procedure to
be performed successfully following a cut-down on the posterior tibial vein. [Q7: C]
Therefore, contemporary venous thrombectomy should be viewed much the same
as arterial thrombectomy, that is, removing as much thrombus from the venous circulation as is physically and pharmacologically possible, correcting any underlying
lesion, and perform mechanical and pharmacological maneuvers to avoid recurrent
thrombosis.
An iliac venous stenosis observed on completion phlebography is common.
Correcting the underlying iliac vein stenosis is considered an important part of the
procedure (Fig. 46b.3). This is performed under fluoroscopic guidance and if recoil
occurs, a self-expanding stent is used to maintain unobstructed venous drainage
from the iliac venous system into the vena cava. [Q8: C] Direct endophlebectomy of
the iliac vein lesion and transposition above the right common iliac artery is a large
operation, which has been replaced by the relatively simple balloon dilation and
stenting.
Following successful thrombectomy of the infrainguinal and iliofemoral venous
systems and correction of any underlying iliac vein stenosis, prevention of recurrent
thrombosis is paramount. There are mechanical and pharmacologic measures
which, if used, minimize recurrence. These include the construction of a femoral
AV fistula using the end of the transected proximal saphenous vein (or a large side
branch) anastomosed to the side of the proximal superficial femoral artery (Fig.
46b.4). Frequently, the proximal saphenous vein must undergo a thrombectomy to
restore its patency. The saphenous vein is not a collateral pathway of venous
drainage for patients with iliofemoral venous thrombosis. On occasion, it may be a
collateral drainage pathway for patients with infrainguinal DVT. Since the infrainguinal venous system had patency restored, that is not an issue in this patient. The
AV fistula is constructed to increase venous velocity in the iliofemoral veins;
however, it should not increase venous pressure. Limiting the size of the anastomosis to approximately 4 mm usually accomplishes this goal. Pressure monitoring of
the common femoral vein before and after flow is initiated through the AVF is

450 Vascular Surgery
important. If the venous pressure increases, one must suspect a proximal (iliac
vein) stenosis or excessive flow through the AVF, either (or both) of which should
be corrected.
An additional, effective adjunctive technique is the placement of a catheter into
the posterior tibial vein, which is used to anticoagulate the patient with unfractionated heparin postoperatively. A pediatric feeding tube is inserted into the posterior
tibial vein and brought out through a separate stab wound in the skin adjacent to
the lower leg incision. This small catheter is used for postoperative anticoagulation
with unfractionated heparin. Targeting a therapeutic PTT ensures a high concentration of heparin in the diseased vein, which should substantially reduce the risk of
recurrence. In the author’s experience, when these adjunctive techniques have been
used, no patient has experienced rethrombosis. [Q9: A, C, E]
Following delivery, women can be anticoagulated with Coumadin, assuming they
do not wish to breastfeed. Warfarin is excreted in the breast milk of women; therefore, those who breastfeed should not be taking warfarin compounds. Among the
options, oral anticoagulation for 6–12 months is the most appropriate. [Q10: B]
While it is true that residual thrombus increases the risk of recurrent thrombosis
[8], it would be inappropriate to treat this patient with less than a full course of anticoagulation. Since this patient had extensive venous thrombosis and a positive
family history, an underlying thrombophilia is suspected and the author would
extend the duration of anticoagulation to 1 year or more.
A thrombophilia evaluation is appropriate in this patient. A complete thrombophilia evaluation cannot be performed while the patient is on anticoagulation,
since antithrombin III, proteins C and S, and factor VIII will be affected. However,
lupus anticoagulant, antiphospholipid antibody, factor V Leiden, prothrombin gene
mutation, and homocysteine levels can be obtained during anticoagulation and, if
positive, may play a role in the subsequent management of this patient. [Q11: D]
References
1. Buller HR, Agnelli G, Hull RD, Hyers TM, Prins MH, Raskob GE. Antithrombotic therapy for venous
thromboembolic disease: the Seventh ACCP Conference on Antithrombotic and Thrombolytic
Therapy. Chest 2004;126:401S–28S.
2. O’Donnell TF, Jr, Browse NL, Burnand KG, Thomas ML. The socioeconomic effects of an iliofemoral
venous thrombosis. J Surg Res 1977;22:483–8.
3. Akesson H, Brudin L, Dahlstrom JA, Eklof B, Ohlin P, Plate G. Venous function assessed during a
5 year period after acute ilio-femoral venous thrombosis treated with anticoagulation. Eur J Vasc Surg
1990;4:43–8.
4. Delis KT, Bountouroglou D, Mansfield AO. Venous claudication in iliofemoral thrombosis: long-term
effects on venous hemodynamics, clinical status, and quality of life. Ann Surg 2004;239:118–26.
5. Partsch H, Kaulich M, Mayer W. Immediate mobilisation in acute vein thrombosis reduces post-
thrombotic syndrome. Int Angiol 2004;23:206–12.
6. Kasirajan K, Gray B, Ouriel K. Percutaneous AngioJet thrombectomy in the management of extensive
deep venous thrombosis. J Vasc Interv Radiol 2001;12:179–85.
7. Monreal M, Rey-Joly BC, Ruiz MJ, Salvador TR, Lafoz NE, Viver ME. Asymptomatic pulmonary
embolism in patients with deep vein thrombosis. Is it useful to take a lung scan to rule out this condition? J Cardiovasc Surg (Torino) 1989;30:104–7.
8. Prandoni P. Risk factors of recurrent venous thromboembolism: the role of residual vein thrombosis.
Pathophysiol Haemost Thromb 2003;33:351–3.

47a. Management of Upper Extremity
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Lymphoedema with Microsurgical
Lymphovenous Anastomosis
Corradino Campisi and Francesco Boccardo
A 59-year-old woman presented with an 8-year history of oedema of the left arm.
Initially, the oedema appeared in the upper arm. The patient was treated with
combined decongestive physiotherapy (manual and mechanical lymphatic
drainage), bandaging and exercises three to four times over a 12-month period.
Despite these measures, the oedema extended as far as the forearm and hand
(Fig. 47a.1), and she had several episodes of erysipeloid lymphangitis. In the
months preceding her admission, she also complained of episodes of lymphangitis and pain. There were no warts or wounds on the skin. Her past medical
history included lumpectomy with axillary lymphadenectomy and radiotherapy
for left breast cancer, although routine follow-up for breast cancer did not
suggest any local recurrence.
Initially, the oedema had a rhizomelic location. It was hard to the touch
without pitting oedema. There were no dystrophic or dyschromic skin lesions,
except for signs of acute reticular erysipeloid lymphangitic attacks caused by
Gram-positive Staphylocci infections promoted by lymph stasis. A lymphangioscintigram was performed, which showed features compatible with lymphatic
circulatory impairment in the left arm (Fig. 47a.2). This was followed by lymphangio-magnetic resonance imaging (MRI) of the left arm and hemithorax,
which showed no signs of locoregional relapse of breast cancer but confirmed
lymph stasis, predominantly in the epifascial compartment. In addition, it
showed dilated medial arm lymphatic collectors interrupted at the proximal
third of the arm. Finally, echo-Doppler of the left subclavian and axillary venous
axis was performed. This did not demonstrate any venous dysfunction. A diagnosis of chronic secondary lymphoedema of the left arm following breast cancer
treatment was made.
Question 1
How do you classify lymphoedema?
453

454 Vascular Surgery
Fig. 47a.1.
Patient before treatment.
Question 2
Which of the following statements regarding the diagnosis of lymphoedema are
correct?
A. Lymphangiography is currently the best diagnostic investigation for all kinds of
lymphoedema.
B. The echo-Doppler investigation has an important role in determining the
correct treatment for the patient.
C. Lymphangioscintigraphy is the most popular noninvasive first-line investigation
for lymphoedema.
D. It is difficult to diagnose lymphoedema at an early stage.
E. Lymphangio-MRI offers precise morphological data on oedema distribution
and topography of dilated lymphatic pathways, without requiring contrast.

Lymphoedema 455
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Fig. 47a.2.
Lymphangioscintigram before microsurgery.
The patient underwent microsurgical lymphatic-venous anastomoses in the
proximal third of the volar surface of the left arm using an 8/0 nylon suture material
(Fig. 47a.3).
Fig. 47a.3.
Lymphatic-venous anastomoses seen through the operating microscope (30¥).

456 Vascular Surgery
Question 3
Which of the following statements regarding the management of lymphoedema are
correct?
A. Microsurgery can reduce oedema in all patients, but the best outcome is seen in
patients operated on in the second and third stages.
B. Proper elastic compression garments are an important adjunct to optimise long-
term results.
C. Surgical intervention is not indicated in the advanced stages of lymphoedema.
D. Microsurgical lymphatic-venous anastomoses are used more frequently than
reconstructive microsurgical methods.
E. Microsurgery cannot be applied in primary lymphoedema.
The postoperative recovery was uneventful. The patient was discharged home on the
fifth postoperative day. The incidence of lymphangitic attacks decreased significantly.
Fig. 47a.4.
Long-term clinical outcome after microsurgery.

Lymphoedema 457
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A reduction in arm volume was seen within 3 days of the operation, and further
improvements were observed at medium- and long-term follow-up, particularly
between the first and the fifth years after surgery. From the fifth year onwards, the clinical condition of the arm remained stable with time, even more than 10 years after the
operation (Fig. 47a.4). Lymphangioscintigraphy at this point demonstrated that the
lymphatic-venous anastomoses were still patent (Fig. 47a.5).
Question 4
What are the long-term results of derivative and reconstructive microsurgery for
lymphoedema?
Question 5
In what ways can secondary lymphoedema be prevented?
Commentary
Lymphoedema is a significant worldwide problem. It can be divided into primary
and secondary forms. Primary lymphoedemas do not have any recognisable cause
Fig. 47a.5.
anastomoses more than 10 years after the operation.
Lymphangioscintigram performed after microsurgery shows the patency of the lymphatic-venous

458 Vascular Surgery
(so-called idiopathic), although triggering aetiological factors can often be found.
Lymphoedemas that present at birth (congenital) are included in this category.
These can be hereditary-familial (Nonne–Milroy’s disease), and are often associated
with chromosomal abnormalities. Other primary lymphoedemas may have an early
or late onset, which can be triggered by minor trauma, infection or surgery. In
females, the predisposing factors are often thought to be alterations in neurohormonal
status (neuroendocrine lymphoedema).
Primary lymphoedemas can also be due to lymphatic and/or lymphnodal dysplasia, hypoplasia or even hyperplasia with associated increased lymph production. Lymph nodes and/or lymphatics can be involved in abnormal lymph flow. In
most cases of hypoplasia, lymph node involvement is demonstrated and leads to
the progressive secondary alteration of lymphatic vessels. From pathophysiological and diagnostic points of view, this picture is practically the same as that seen
with secondary lymphoedemas resulting from lymphadenectomy and/or
radiotherapy [1].
Approximately 90 per cent of all primary lymphoedemas are characterised by
hypodysplastic alterations involving lymph nodes and/or lymphatics. This is
characterised by a diminished ability to form and activate a proper collateral
circulation in response to trauma, infection and surgery. In a further 8–10%
of primary lymphoedemas, an increased number and size of lymphatic collectors can be found, associated with structural lymphatic and lymph nodal
dysplasia [2].
Disorders in lymphogenesis also play an important role from a lymphodynamic
point of view. Hyperlymphogenesis may derive from pre-existing regional arterialvenous hyperstomies, arterial-venous fistulae (i.e. in Klippel–Trenaunay’s disease)
or related angiodysplasia. In contrast, reduced or absent production of lymph, agenesis, hypoplasia, or impaired permeability of the initial lymphatics is very rare, if
not exceptional.
Finally, among lymphodynamic abnormalities, apart from insufficient lymph
drainage along anatomically pre-established pathways, gravitational lymph
and/or chylous reflux pathologies should also be mentioned. This top-to-bottom
lymph backflow is caused by insufficient antigravitational structures, normally
represented by valves, the reticular myoelastic layer of the lymphatic walls, and
lymph node architecture (lymphoedemas and chyloedemas due to gravitational
reflux).
The aetiology of secondary lymphoedemas can generally be identified in the
patient’s history or physical examination. This can be secondary to trauma, infection, inflammation, radiotherapy, surgery, paralysis or even neoplasia. Indeed, lymphatic filariasis is endemic in some tropical and subtropical areas of Asia, Africa
and Latin America. However, secondary lymphoedemas often have some congenital
predisposition. For instance, congenital wall-valve dysplasia of the lymphatics is
always found in lymphatic filariasis. Similarly, arm lymphoedema secondary to
breast carcinoma treatment occurs in 5–35% of cases, depending on whether axillary surgery is associated with radiotherapy [3]. However, it is more likely to occur
when there is no deltoid pathway [4]. This lymphatic way drains the lymph coming
from the arm directly into the supraclavicular lymph nodes, thus bypassing the axillary stations. With preventive lymphoscintigraphic studies, comparing the arm ipsilateral to the breast cancer site with the contralateral one, patients with a higher risk
of developing secondary lymphoedema could be identified and could, theoretically,
receive preventive therapeutic treatment. Based on these observations, Tosatti’s

Lymphoedema 459
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Fig. 47a.6.
Classification of chronic lymphoedema of the limb according to Tosatti.
classification of lymphoedemas (Fig. 47a.6), proposed more than 30 years ago [5],
still seems to be valid. [Q1]
Apart from some exceptional cases of acute post-lymphangitis and/or post-traumatic types, lymphoedema is normally a chronic, progressing and disabling condition characterised by a progressive volume increase of the limb(s) involved. This
disease, which evolves in phases, is characterised by frequent lymphangitic,
erysipeloid exacerbations with subsequent lipodermatosclerotic indurated cellulitis,
chronic fibrosclerotic lymphadenitis (in primary lymphoedemas) and lymphostatic
warts. The worst outcome is generally elephantiasis, with severe functional impairment or systemic sepsis. However, degeneration into lymphangiosarcoma
(Stewart–Treves syndrome) is a rare sequel, most likely to occur in post-mastectomy lymphoedemas. It is important not to confuse this with cutaneous local recurrence of breast cancer. Lymphoedema of the leg can also occasionally be associated
with Kaposi’s sarcoma. This is not necessarily human immunodeficiency virus
(HIV)-related.
The differentiation of lymphoedema from phleboedema can be based on a thorough medical history and clinical examination, paying attention to the time and
conditions of onset, location, evolution, extent and volume of the oedema.
Lymphoedema is hard to the touch, while venous oedema is soft and pits under
finger compression. This difference reflects the underlying pathophysiology, in that
stagnant lymph in the subcutaneous connective tissue is an excellent culture
medium for fibroblasts. In this environment, they mature rapidly into fibrocytes,
thus forming fibrosclerotic connective tissue. Lymphoedema typically begins proximally, whereas venous oedema initially affects the distal part of the lower limbs with
the notable exception of phlegmasia dolens, caused by acute deep thrombophlebitis
of the iliofemoral veins. Unlike phleboedema, lymphoedema does not usually
evolve into dystrophic-dyschromic skin lesions or ulcers. It is more likely to be
complicated by acute reticular erysipeloid lymphangitis, caused by Gram-positive
cocci infection in the presence of static lymph. Phleboedema is often associated with
varices and varicophlebitis, and unlike lymphoedema, it is subject to rapid postural
changes and is characterised by abnormal Doppler venous flow rates with

460 Vascular Surgery
significant increase in venous pressure when the patient is standing up. However,
mixed types of lymphophleboedema also exist (as in stage III postphlebitic syndrome), with predominance of either the venous or lymphatic component. These
include the complex conditions of angiodysplasia with arterial-venous hyperstomy,
as seen in Mayall’s syndrome [6], or congenital arteriovenous macro- and
microfistulas, as seen in Klippel–Trenaunay’s disease. (The latter condition is recognised by gigantism with elongation of the affected limb, varying degrees of foot dysmorphism, flat or map-like “port-wine” angioma, and hyperhydrosis of the sole of
the foot.) There are also some spurious forms of lymphophleboedema, which are
masked by prevailing lymphoedema and therefore more difficult to recognise. In
these cases, if angiodysplasia is suspected, then routine investigations such as
Doppler venous pressure measurements may be insufficient, and further investigations, including phleboscintigraphy, phlebography or digital arteriography, may be
required. For the time being, lymphangioscintigraphy and conventional oil-contrast
lymphography are the most suitable investigations of lymphatic and chylous
oedemas. Lymphangioscintigraphy is the most popular method used in the rapid
screening of lymphoedemas [7, 8] as it is a noninvasive way of imaging both
superficial and deep lymphatic circulations. Since it is noninvasive, it can be
repeated easily in patient follow-up, especially after microsurgery. A small tracer
dose of
99m
technetium adsorbed in colloid spherules (colloid sulphide, rhenium,
dextran) is used. The lymphotropic nature of these substances permits display of the
preferential lymphatic pathways with a gamma camera, and allows measurement of
the flow rate and lymph node uptake. A tracer clearance measurement is a useful
parameter from a lymphodynamic viewpoint. However, lymphoscintigraphy is most
useful in the study of lymphoedemas at early stages [9]. Direct lymphangiography
[10] is preferred in the study of gravitational reflux lymphatic and chylous oedema of
the lower limb and external genitalia before surgical intervention [11, 12]. In this
examination, ultrafluid “Lipiodol” is injected into a lymphatic collector, isolated with
microsurgical technique, of the dorsum of both feet. This type of investigation is
minimally invasive and, if performed according to well-established standards, has
minimal complications. However, rare adverse reactions have been reported. These
include general complications such as pulmonary microembolism in the presence of
peripheral lymphovenous fistulas or allergy to contrast medium. Local complications
may also occur in the form of infection on the site of the skin incision, acute lymphangitis or lymphorrhoea. Direct lymphangiography can also be performed in children. It enables a morphofunctional study of the superficial and, with the use of
proper technical support, the deep circulation [12].
Computed tomography (CT), ultrasonography and lymphangio-MRI may also
provide important preoperative data on lymphatic and chylous dysfunction.
Indirect lymphangiography [13] performed with dermo-hypodermic injection of
a water-soluble contrast medium (“Iotasul”) is useful to clarify aetiopathological
aspects of primary lymphoedemas, and fluorescent microlymphography [14] can
be helpful in assessing the status of the superficial dermis lymphatic web, which
reflects the functional condition of the peripheral lymphatic circulation. The conventional Houdack–McMaster dye test with the injection of highly lymphotropic
vital stain (Patent Blue V) is used today as a preliminary investigation in direct
lymphangiography and microsurgery for a better and faster assessment of lymphatics. Recent studies by Olszewski [15] and Campisi et al. [16] have developed
a system to measure endolymphatic pressure and lymphatic flow rate. These
parameters, together with venous pressure assessment, help to measure the
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